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A 2006 study by Koy and Coons on the incidence of chronic conditions reported that approximately threefifths (62. Nasal congestion secondary to sinusitis and other conditions is a relative contraindication to air travel. In studies by Chester and others, and by Soler et al, all but the following symptoms were clinically and statistically improved by sinus surgery in patients who failed to respond to medical therapy: A. However, the words rhinitis, sinusitis and rhinosinusitis are often used interchangeably. Typically, acute bacterial sinusitis resolves with medical therapy, resulting in no permanent mucosal damage. However, recurrent acute episodes of sinusitis require further workup to uncover anatomic or systemic underlying factors. This can represent acute sinusitis that has either been inappropriately treated or not treated at all. Generally, adequate medical treatment of subacute sinusitis should lead to complete resolution with no resultant mucosal damage. Because recurrent infections occur in these patients despite medical treatment, this subset of patients is more likely to undergo primary endoscopic sinus surgery. Patients with recurrent acute rhinosinusitis used significantly fewer sinus medications after endoscopic sinus surgery. Patients with recurrent acute rhinosinusitis were more often primary surgical patients and underwent less extensive surgery than their chronic rhinosinusitis counterparts. The signs and symptoms of chronic sinusitis have been detailed in the previous chapter. They include major and minor symptoms, which vary slightly depending on the source. Minor criteria include headache, fever, halitosis, fatigue, dental pain, cough, and ear pain, pressure, or fullness. There are some criteria more specific to children, and these include cough and irritability. A strong history consistent with a diagnosis of sinusitis is indicated by the presence of either two major criteria or one major and two minor criteria. A suggestive history is indicated by the presence of one major or two minor criteria (Table 1). Chronic sinusitis is most commonly defined as persistent signs and symptoms for more than 3 months, with or without a constant need for antibiotics. This condition requires evaluation by an otolaryngologist, possibly by an allergist, and possibly by an infectious disease specialist to identify causative factors. Next the doctor will perform a careful examination, including nasal endoscopy (Fig. An assessment is made of mucosal appearance; size, shape, and angulation of the turbinate; obstruction secondary to a deviated septum; and crosssectional airway competence at the external and internal nasal valve. Hyperemia, edema, crusting, polyps, and purulence in the nasal cavity are also noted and may be indicative of chronic sinusitis. Rigid nasal endoscopy in the office after appropriate topicalization by a skilled endoscopist may be helpful in identifying these physical findings (11, 12). Factors that lead to nasal obstruction, and more specifically to obstruction of the narrow sinus drainage pathways, are identified. These include viral upper respiratory tract infection, allergic rhinitis, vasomotor rhinitis, barotrauma, and mucosal hypertrophy. Furthermore, mechanical obstruction can be caused by nasal polyps, deviated nasal septum, foreign body, trauma, choanal atresia, and tumors. Instrumentation of the nose in the hospital, such as a nasogastric tube, can be a contributing factor. Typically, the patient has a previous upper respiratory viral infection whose symptoms have failed to clear after numerous overthe 33 counter and home remedies. Communityacquired bacterial sinusitis is relatively common as a complication of a viral upper respiratory infection. Patients with acute bacterial sinusitis complain of facial pain aggravated by bending over, a yellowish/ greenish nasal discharge, nasal obstruction, unpleasant breath and taste, increased postnasal mucus (especially in the upright position) headache, and cough. Because purulent nasal discharge and pain are the most common clinical findings of acute bacterial sinus infections, the location of the facial pain may suggest which sinuses are involved. Pain in the cheeks suggests maxillary sinusitis, whereas pain in the forehead or medial orbit suggest frontal sinusitis. Pain between the eyes suggests ethmoid sinus and pain behind the eyes and also occipital pain is associated with sphenoid sinusitis. It is not at all surprising that there is confusion about differentiating the common cold from sinusitis, because the symptoms are very similar in the first week. However, patients who develop bacterial sinusitis typically seek help because of fever, headache, facial pain, or nasal obstruction that interferes with sleep. Symptoms of bacterial sinusitis are generally not relieved with overthecounter preparations. Chronic sinusitis is present when there are persistent signs and symptoms of sinusitis for 12 weeks or more. There is a more scientific definition of chronic sinusitis: chronic sinusitis is a disease in which the mucosal damage is no longer reversible despite appropriate medical therapy (46). In these cases, a definitive cure will most likely require surgery that addresses the "main" sinus drainage pathways. Endoscopic examination enables the doctor to identify specific areas of blockage, to detect the presence of polyps, and to obtain cultures at specific drainage sites (Fig. Endoscopic examination enables the doctor to identify specific areas of blockage, to detect the presence of polyps, and to obtain cultures at specific drainage sites. The flexible fiberoptic endoscope is useful in certain circumstances because its flexibility allows examination of difficulttoexamine structures. Examination of the ear, nose, and throat all the way down to the vocal cords can be undertaken with a flexible scope. The rigid endoscopes are advocated for diagnostic purposes by most otolaryngologists. Spraying the nose with 1% phenylephrine and 2% tetracaine (pontocaine) often is sufficient to make the patient comfortable for a complete office exam with nasal endoscopy. If necessary, additional comfort can be effectively achieved by placing a cotton pledget with 4% topical anesthetic agent into the nasal cavity for 5 minutes. Proper diagnosis of intranasal and sinus disease can only be maximally achieved with this type of endoscopic examination (5, 1113). Also, sweat chloride testing may be performed to rule out cystic fibrosis, especially in children with nasal polyps or chronic or recurrent sinusitis. These tools are an immense improvement over plain film sinus depiction and can give reliable reproducible information. This information includes the status of the bony walls, the nature of material within the sinuses, and the status of the adjacent normal structures such as the eye, brain, and midface. The status of the bony walls of the sinuses is important both in benign sinus disease and also in sinus tumors. It takes newergeneration scanners only minutes to provide highresolution images of tissue slabs that are 36 only a few millimeters thick. Patients undergo medical therapy to address acute infections, shrink inflamed mucosal membranes, and reduce hyperplastic mucosa. The scan technique results in relatively low radiation exposures and generates image contrast that is diagnostic for definition of anatomic structures. These images are adequate for evaluation of various densities within the sinus contents, which can indicate fungal sinus disease or concretions in the sinuses. Although some clinicians advocate additional windowing to increase sensitivity for extra 37 sinus pathology, this has not routine. It is notable that plain films of the sinuses in children can be especially misleading. In children, computer tomography will offer an improved sensitivity and specificity, but it has its drawbacks, namely higher cost, somewhat increased radiation exposure, and the frequent necessity of sedation to perform these exams in children.
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Similarly, delayed language cannot be attributed to sex; boys are not significantly behind girls in language development. Young children may have a maturational or developmental delay in speech or language that resolves with time. However, a speech or language disorder exists when difficulties in learning language or developing speech skills persist and cause impairment. About half of "late talkers" continue to have language difficulties at 4 years of age. Language disorders are present in about 2% to 3% of school-age children and speech disorders are present in about 3% to 6%. Speech disorders include phonologic disorders (articulation problems) and stuttering. Stuttering occurs in 1% of children and is characterized by impaired speech fluency. Speech sounds are prolonged, parts of words are repeated, pauses are present, and facial tension can be apparent when the child attempts to speak. The 3-year-old boy in the vignette demonstrates a significant delay in his expressive language and decreased intelligibility, even to his mother. His language is still at the mature jargoning stage, as compared to the expected 3-word utterance stage. While it is possible that his language and speech may improve, his delay in expressive language is not mild and a "wait and see" approach would be of disservice to this child. This child will not necessarily have problems with language-based learning, but as a child with language delay, he is at risk. Therefore, this child should be monitored for possible development of those issues in school. He may not develop speech intelligible to unfamiliar adults by 4 years of age as expected without further evaluation and intervention. This child and any other child in whom a speech/language delay or disorder is considered should be referred for an audiology evaluation and speech/language evaluation. Evaluation of other developmental domains should be considered, as speech/language delay may be the presenting sign for other conditions such as global developmental delay, intellectual disability, or autism spectrum disorder. Evaluation and treatment may be accessed through Early Intervention programs if the child is younger than 3 years of age and through the school district if older than 3 years of age. She has felt nauseous and has had 8 episodes of nonbilious vomiting since the pain began. She had a cough and nasal congestion over the past 2 days, but denies any other associated symptoms, including fever and diarrhea. She "felt fine" earlier in the evening when she went out to dinner with her mother and older sister. On physical examination, she appears to be in moderate distress due to pain, but her mental status is normal. Her abdomen is tender to palpation over the right lower quadrant and suprapubic region, but she displays no peritoneal signs. As you are completing your physical examination, the adolescent reports increasing nausea and has another episode of nonbilious emesis. Based on her history and physical examination findings, her most likely diagnosis is ovarian torsion. It is important for all pediatric providers to recognize the clinical findings associated with ovarian torsion. Ovarian torsion had been estimated to account for nearly 3% of all cases involving acute abdominal pain in children. Pediatric patients account for an estimated 15% of all ovarian torsion cases, with major centers reporting between 0. Ovarian torsion has been described in all ages, occurring at an average age of 10 years among children. While ovarian torsion is more common following menarche, it may affect children in the prepubertal period as well. Ovarian torsion begins when an ovary twists on its pedicle, resulting in obstruction of venous outflow and lymphatic drainage, leading the ovary to become engorged and edematous. If not corrected, the persistent increase in ovarian parenchymal pressure may result in occlusion of arterial blood flow and infarction of the affected ovary. Clinical findings of ovarian torsion include abrupt onset of severe, constant, unilateral pain located in the pelvis or lower abdomen. In patients presenting with suspected ovarian torsion, pelvic ultrasonography should be obtained. Acute appendicitis with perforation is less likely to be the diagnosis for the patient in the vignette than ovarian torsion. While there can be considerable overlap in the clinical findings of ovarian torsion and acute appendicitis, patients with ovarian torsion (as noted in the girl in the vignette) are much less likely to have fever, migratory pain, or peritoneal signs such as rebound tenderness on examination. Furthermore, the onset of symptoms of acute appendicitis (especially acute appendicitis complicated by perforation) would typically be expected to be less abrupt than the sudden onset of symptoms that occurs with ovarian torsion. Although acute food poisoning could certainly lead to acute onset of nausea and vomiting, patients with this diagnosis would not be expected to have localized abdominal tenderness on physical examination, as is noted in the adolescent in the vignette. Children with lower lobe pneumonia may present with abdominal pain because of visceral innervation. However, associated symptoms including fever, cough, and tachypnea are typically present in these children, and these findings are not present in the adolescent in the vignette. Abdominal or pelvic pain due to ovarian cysts is much less likely to be associated with nausea and vomiting, which are prominent symptoms displayed by the patient in the vignette who is presenting with acute ovarian torsion. Associated symptoms include nausea and vomiting, as well as urinary tract symptoms such as dysuria and frequency. If ovarian torsion is highly suspected clinically, laparoscopy may be required to both diagnose and treat the condition. Contraindications to pertussis vaccination include anaphylaxis after a previous dose of pertussis-containing vaccine and encephalopathy within 7 days of receipt of pertussis vaccine without another identifiable cause. In order to avoid ascribing symptoms to vaccine, it is recommended that vaccine be deferred in patients with an evolving neurologic condition. Additionally, a family history of a severe reaction to a pertussis-containing vaccine would not be considered a contraindication. Other than in settings where pertussis vaccination is contraindicated, Td can be used for tetanus prophylaxis in wound management and for routine decennial booster when the individual has previously received Tdap. It can also be used for catch-up vaccinations in individuals 7 years of age or older after Tdap has been given. The girl told her mother she thought it looked like a little baby bottle when she was discovered holding it to her lips. The bottle is now empty, and the mother is unsure of the quantity of liquid it had contained. Since these preparations are readily available in most households, there are significant safety concerns about adverse drug effects and the risk of unintentional ingestions or overdoses. A minimum toxic dose of the topical imidazolines has not been established, but ingestion of as little as 2. Onset of symptoms can be rapid and generally occurs within 4 to 6 hours after ingestion. Admission and close monitoring is warranted for all symptomatic children who have ingested topical imidazolines. Symptoms should be managed with supportive treatment and usually resolve within 24 hours. Children who are asymptomatic 6 hours after ingestion may be discharged from the hospital if continued close supervision for 24 hours can be assured, as well as ready access to return for care if needed. The girl in the vignette is already exhibiting bradycardia, so close observation at home is not appropriate. In addition, because the interval from ingestion is uncertain, she should be transported to an emergency center via ambulance with hemodynamic monitoring. For gastrointestinal decontamination, activated charcoal must be administered within 1 to 2 hours of ingestion (< 1 hour for rapidly absorbed toxins).
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High Performer Middle Performer 22 Next Steps the Department has several important initiatives to refine and enhance the utility of this data for improvement. These include, but are not limited to, innovative approaches to early identification of high risk patients, rapid response of early interventions, mobilization of clinical, laboratory, and pharmacy resources within the institution, sepsis protocol content, quality improvement activities, use of clinical decision support through electronic medical records, workforce sepsis training and education, and more. Both the Advisory Group and the P4P provide a forum for discussion and dissemination of these findings. Data Collection Improvement and Alignment the data dictionary will continue to be streamlined and improved so that hospitals and their data collection staff can completely and accurately report all data elements needed for valid and reliable quality measurement. On-going data audits provide information to both hospitals and to the Department that serve to identify variables requiring further elaboration. Future Measurement: Pediatrics and Morbidity the Department plans to explore the ability to evaluate other important pediatric sepsis outcomes beyond the one hour bundle including risk adjusted mortality. While the number of pediatric cases for each hospital will not permit statistically valid comparisons these results could be used for quality improvement and internal hospital benchmarking. Last, there are other outcomes in addition to survival that are important to clinicians and patients related to serious and long lasting organ or tissue damage that can result from sepsis. While there are currently no standardized metrics or data to capture this information the Department is committed to exploring ways to develop new and innovative measures in this important area. In the first part of the process, a mortality model estimates the probability of in-hospital mortality for each patient with sepsis. This estimate is based on patient demographic, comorbidity, and severity of illness characteristics. Multivariable logistic regression was used to determine which variables are important and accurate in estimating the probability of mortality for each patient. Treatment variables, within the control or influence of the providers and hospital, are not included in the prediction model. Table A1 shows the thirteen (13) variables in this model that are used to estimate the probability of mortality as well as the overall performance of the model. The risk adjusted model in this report makes use of the most recent complete and audited data from four quarters of patient data submission in 2015. Patients with advanced care directives in place prior to the episode of sepsis who declined sepsis protocol interventions, or who refused sepsis protocol interventions at the time of presentation, were removed from the data set. Patients admitted more than once in 2015 for sepsis are represented only once for purposes of development of the risk adjusted model (using their last admission only). To assess hospital performance, the probability of hospital mortality is calculated for every patient from that hospital using the logistic regression model. These probabilities are summed over all the patients at that hospital to calculate the expected number of deaths for that hospital. The actual number of deaths is determined for all patients in that hospital as well. The highest performing hospitals are displayed in blue and the lowest performing hospitals are displayed in gold. Table B1 contains performance and outcome measures for inpatient adult (age 18) sepsis care for New York State Hospitals. The Protocol Initiated measure (N2) includes all patients except for those excluded from the protocol or who died within six hours. This measure is only reported for those hospitals with greater than 10 adult sepsis cases in 2015. Table B2 contains the performance measures for inpatient pediatric (age < 18) sepsis care for New York State Hospitals. This table includes the following performance measures: protocol initiated and 1-hour bundle. The Protocol Initiated measure (N2) includes all patients except for those excluded from the protocol or those who died within one hour. This measure indicates the percentage of patients in the denominator for whom a protocol was initiated. This measure is only reported for those hospitals with greater than 10 pediatric sepsis cases in 2015. In both tables, the highest performers are highlighted in blue and the lowest performers are highlighted in gold. Lukes Mount St Marys Hospital and Health Center Nassau University Medical Center Nathan Littauer Hospital New York Community Hospital of Brooklyn, Inc 4 150 151 84 14 86 325 337 124 18 90 N. Marys Hospital Staten Island University HospNorth Staten Island University HospSouth Strong Memorial Hospital Syosset Hospital the Unity Hospital of Rochester Tri Town Regional Healthcare United Health Services Hospitals Inc. Roohan, Director Anne Schettine, Deputy Director Office of the Medical Director Dr. The score is calculated at admission and every 24 hours until discharge, using the worst parameters measured during the prior 24 hours. This information can then be used in various ways, such as to provide the family with a prognosis, for clinical trials, and/or for quality assessment. On behalf of the Working Group on Sepsis-Related Problems of the European Society of Intensive Care Medicine. Crossvalidation of a Sequential Organ Failure Assessment score-based model to predict mortality in patients with cancer admitted to the intensive care unit. It may help increase suspicion or awareness of a severe infectious process and prompt further testing and/or closer monitoring of the patient. These patients should be more thoroughly assessed for evidence of organ dysfunction. It is still not clear how it will be used in the sequence of events from screening to diagnosis of sepsis to the triggering of sepsis-related interventions. The management of sepsis is continuously evolving and is detailed in the 2016 Surviving Sepsis Campaign: International Guidelines for the Management of Sepsis and Septic Shock (Rhodes 2017). Assessment of clinical criteria for sepsis: for the third International Consensus definitions for sepsis and septic shock (Sepsis-3). Prognostic accuracy of Sepsis-3 criteria for in-hospital mortality among patients with suspected infection presenting to the emergency department. The Third International Consensus definitions for sepsis and septic Shock (Sepsis-3). Surviving Sepsis Campaign: international guidelines for management of sepsis and septic shock: 2016. The authors themselves have explicitly objected to the score being used in this way, and analysis has shown that patients with the same total score can have huge variations in outcomes, specifically mortality. A comparison of the Glasgow Coma Scale score to simplified alternative scores for the prediction of traumatic brain injury outcomes. Validation of the Simplified Motor Score for the prediction of brain injury outcomes after trauma. Validation of the Simplified Motor Score in the out-of-hospital setting for the prediction of outcomes after traumatic brain injury. Both companies are dedicated to providing evidence-based clinical decision-making support for emergency medicine clinicians. Hand washing Catheter care Barrier precautions Airway management Elevation of head of bed Subglottic suctioning Transfuse for hgb < 7. Special Articles Surviving Sepsis Campaign: International Guidelines for Management of Severe Sepsis and Septic Shock: 2012 R. Design: A consensus committee of 68 international experts representing 30 international organizations was convened. Nominal groups were assembled at key international meetings (for those committee members attending the conference). A formal conflict of interest policy was developed at the onset of the process and enforced throughout. A stand-alone meeting was held for all subgroup heads, co- and vice-chairs, and selected individuals. Teleconferences and electronic-based discussion among subgroups and among the entire committee served as an integral part of the development.
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Second branchial arch dysplasia results in anomalies of the hyoid, styloid, stylohyoid ligament, and stapes. Cochlear anomalies may be classified according to the stage of developmental arrest. Complete labyrinthine aplasia (Michel deformity) results in a single small cystic cavity. Other anomalies include a large common cavity (common chamber anomaly), cochlear aplasia or hypoplasia, and incomplete partition (Mondini syndrome-small cochlea with incomplete septation, i. Inner Ear Anomalies Congenital sensorineural hearing loss is commonly associated with inner ear anomalies. A common anomaly is vestibular aqueduct dysplasia (ranging from obliteration to dilatation; Fig. Facial Nerve Anomalies Aberrant course of the facial nerve is usually associated with an anomaly of the external, middle, or inner ear. Dehiscence of the facial nerve canal most often occurs in its tympanic portion at the level of the stapes and results in a conductive hearing loss (Fig. Neck, Oral Cavity, and Jaw Normal Development the branchial apparatus, which contributes to formation of the head and neck, consists of paired branchial arches, pharyngeal pouches, branchial grooves, and branchial membranes. Each arch consists of a mesenchymal core (containing neural crest cells and arterial, nerve, cartilage, and muscular elements). Each arch is separated by branchial membranes and covered externally by surface ectoderm (branchial grooves) and internally by endoderm (pharyngeal pouches). The primitive mouth (stomodeum) arises from the surface ectoderm in contact with the amniotic cavity externally and the primitive gut internally via the esophagus (after rupture of the primitive buccopharyngeal membrane). The developing thyroid gland is a diverticulum connected by the thyroglossal duct ventral to the hyoid to the tongue base at the foramen cecum. The laryngotracheal groove and tracheoesophageal folds form to become the ventral laryngotracheal tube and dorsal esophagus. Three layers of deep cervical fascia divide the suprahyoid neck into eight compartments (parapharyngeal space, pharyngeal mucosal space, masticator space, parotid space, retropharyngeal space, perivertebral space, and posterior cervical space). The sternocleidomastoid muscle divides the infrahyoid neck into anterior and posterior triangles. The layers of the deep cervical fascia permit further subdivision of the infrahyoid neck into five major spaces that are continuous with corresponding spaces in the suprahyoid neck (carotid, visceral, posterior cervical, retropharyngeal, and perivertebral spaces). The adenoids become conspicuous within the nasopharynx by 2 to 3 years of age and regress during adolescence. If no adenoidal tissue is seen in a young child, and in the absence of prior adenoidectomy, the possibility of immunodeficiency should be considered. The lymph nodes of the neck occur in contiguous groups and may be classified according to various systems. Contrast enhancement of lymph nodes is abnormal and may be seen in a variety of inflammatory and neoplastic processes. The major vessels of the head and neck include the common carotid arteries, which bifurcate into internal and external carotid arteries, the external jugular veins, the anterior jugular veins, and the internal jugular veins. The oral cavity contains the tongue and is bound inferiorly by the mylohyoid muscle. Within the oral cavity are the submandibular and sublingual spaces (separated by the mylohyoid muscle). The major salivary glands consist of the paired parotid, submandibular, and sublingual glands. Thyroid Anomalies Thyroglossal duct cyst arises from thyroglossal duct remnants and often occurs in childhood. They are usually midline, or paramedian, and occur at any site from the tongue base to the suprasternal region. Off-midline cysts often occur near along the outer thyroid cartilage and deep to the neck muscles. The differential diagnosis includes dermoid, teratoma, vallecular cyst, mucous retention cyst, laryngocele (see Fig. These anomalies are therefore classified according to the level (arch, cleft, or pouch) of origin. Defects include branchial cysts, aberrant tissue, branchial sinus (incomplete tract usually opening externally that may communicate with a cyst), and branchial fistula (epithelial tract with both external and internal openings). Wall thickness, enhancement, content, and surrounding edema often increase with inflammation. The differential diagnosis includes an inflammatory cyst, lymphatic malformation, and necrotic adenopathy. It usually manifests as a mass at the mandibular angle but may occur at any site along a line from the tonsillar fossa to the anterior margin of the sternocleidomastoid muscle to the supraclavicular region (Figs. The differential diagnosis includes vascular anomaly, suppurative adenopathy, paramedian thyroglossal duct cyst, laryngocele, and necrotic metastatic adenopathy. The third branchial sinus/fistula arises from the inferior pyriform sinus and extends between the common carotid artery and vagus nerve to the lower lateral neck. The fourth branchial sinus/fistula usually arises from the left inferior pyriform sinus, looping beneath the aortic arch (or subclavian artery if on the right) and then upward via the carotid bifurcation to the lateral neck. Recurrent neck abscess or suppurative thyroiditis, particularly if it contains air, should raise the possibility of a pyriform sinus/ fistula (Fig. After treatment of the infection, a swallowing study using the appropriate contrast medium is performed to demonstrate the sinus/fistula. Other branchial anomalies are exceedingly rare but include anomalies of the thymus, thyroid (see later), and parathyroid glands. Other thyroid anomalies include hypogenesis (partial or complete) and ectopic thyroid tissue (usually near the foramen cecum at the tongue base; Fig. Such tissue may be shown by thyroid scintigraphy to be nonfunctional or the only functioning thyroid tissue. They usually involve the parotid gland and include branchial cysts and dermoid cysts. Laryngocele A laryngocele results from obstructive dilatation of the laryngeal ventricle and may be aerated or fluid-filled. The differential diagnosis includes thyroglossal duct cyst and laryngeal mucosal cyst (see Fig. Jaw Anomalies Mandibular and maxillary hypoplasia may be seen with a number of craniofacial syndromes (see earlier discussions). Cherubism is a benign hereditary condition misnamed "congenital fibrous dysplasia. The mandible and maxilla are often both involved by multiple expansile fibroosseous lesions (Fig. Anomalies of the Oral Cavity, Tongue, and Salivary Glands Congenital and developmental abnormalities of the oral cavity previously described include lingual thyroid (see Fig. Agenesis of the major salivary glands is rare, causes xerostomia, and may be associated with absence of the lacrimal glands. B 312 Pediatric Radiology: the Requisites ocular rupture, enophthalmos, optic nerve avulsion, vascular occlusion, pseudoaneurysm, and carotid-cavernous fistula. Penetrating orbit injury may result in retained foreign body and secondary infection. Trauma Orbit and Globe Blunt and penetrating impact injuries are common in childhood. Orbit floor and inferior rim fractures rarely occur prior to maxillary pneumatization. Frontal impact may result in a blow-out fracture of the orbital floor near the infraorbital canal (Fig. Rarely is there upward displacement of the orbital floor fragments (blow-in fracture) with impingement on the muscles or globe. Medial orbital wall fracture into the ethmoid may be isolated or may be associated with an orbital floor fracture (see Fig.
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The Senate recedes with an amendment that would authorize the Secretary of Defense, acting through the Under Secretary of Defense for Research and Engineering, to carry out a research program on foreign malign influence operations as part of the university research programs of the Department of Defense. Further, the provision would require the Secretary to submit to the congressional defense committees a notification not less than 30 days prior to initiating such a program. The Senate recedes with an amendment that would: (1) Include support from the Under Secretary of Personnel and Readiness for the assessment; (2) Specify the scope of the assessment as the research and engineering workforce of the Department of Defense; (3) Modify the elements required in the assessment; and (4) Require consultation with the Secretaries of the military departments in the development of the plan. Policy on the talent management of digital expertise and software professionals (sec. The Senate recedes with an amendment that would authorize the Secretary of Defense to appoint a Chief Digital Engineering Recruitment and Management Officer. The amendment would also require an implementation plan describing how the Department of Defense will execute its policy to promote and maintain digital expertise and software development as core competencies of the civilian and military workforce. The conferees encourage the Secretary of Defense to include in the implementation plan required by this section the following: (1) An assessment of progress made in recruiting an individual to serve as the Chief Digital Engineering Recruitment and Management Officer; (2) A timeline for implementation of the policy required by this section; and (3) Recommendations for any legislative or administrative action needed to meet the requirements of this section. The Senate recedes with amendments that would further elaborate the governance process and that would further define the scope of the demonstration and selection of programs to participate, as well as clarifying amendments to the roles and responsibilities of officials and organizations and technical amendments to the reporting requirements. The conferees believe that establishing a digital engineering capability is critical to accelerating the adoption of best practices in the use of software to model and simulate complex system behavior to assess potential effects of proposed hardware or software engineering changes on system performance. Further, the conferees note the significant potential value of digital engineering capability for automating developmental and operational test and evaluation, and especially where a weapon or business system is software-defined and is developed using agile or secure continuous development/continuous delivery methods. The Senate recedes with an amendment that would: (1) Modify the elements required for the process; (2) Modify the required report to a briefing; and (3) Modify definitions. The House recedes with an amendment that would modify the use of funds for fiscal year 2020 and clarify that the account be executed through the Under Secretary of Defense for Acquisition and Sustainment. The amendment would also require that the Secretary of Defense submit to the congressional defense committees a notification identifying the military services or agencies that will be responsible for the conduct of air and missile defense in support of joint campaigns as it applies to defense against current and emerging missile threats, including against each class of cruise missile. The Senate recedes with an amendment that would: (1) Require the Secretary of Defense and each of the Secretaries of the military departments to develop the master plan; (2) Modify the elements required in the plan; and (3) Modify the reporting requirements. The Senate recedes with an amendment that would: (1) Require consultation with the Secretaries of the military departments; (2) Modify the elements of the plan; and (3) Require prioritization of unfunded requirements for laboratory military construction projects. Strategy and implementation plan for fifth generation information and communications technologies (sec. The Senate recedes with an amendment that would: (1) Modify elements of the required strategy; and (2) Add periodic briefings on the development and implementation of the strategy. This provision would also require that the designated senior official or entity develop a strategy for research and development of the next generation software and software intensive systems and submit the strategy to the congressional defense committees not later than 1 year after the date of the enactment of this Act. The Senate recedes with an amendment that would expand the scope of the activities assigned under a senior official and associated scope of the strategy, to include foundational research, technical workforce and infrastructure, software acquisition, and software dependent missions; and further an amendment that would expand the strategy to incorporate activities in certain organizations to include universities, federally funded research and development centers and other entities. The Senate recedes with an amendment that would require the Secretary of Defense to develop a strategy and implementation plan for educating servicemembers in relevant occupational fields on matters relating to artificial intelligence. The House recedes with an amendment that would add the rest of the Federal government to the list of entities to be consulted. Commercial edge computing technologies and best practices for Department of Defense warfighting systems (sec. The Senate recedes with an amendment that would combine the reports into a single report and modify the required elements therein. The Senate recedes with an amendment that would modify the provision to require the Secretary of Defense to commission an independent National Academies of Sciences, Engineering, and Medicine study to review the state of defense research at covered institutions. The provision would also require the Department of Defense to develop an implementation plan in response to the recommendations of the study. Study on national security emerging biotechnologies for the Department of Defense (sec. The House recedes with an amendment that would change the provision into a requirement for the Secretary of Defense to direct the Defense Science Board to study the national security aspects of emerging biotechnologies. The provision would also require a briefing on emerging biotechnology-based threats. Sense of Congress on the importance of continued coordination of studies and analysis research of the Department of Defense the House amendment contained a provision (sec. The conferees note the responsibilities of the Under Secretary of Defense for Research and Engineering in supervising all defense research and engineering, technology development, transition, prototyping, experimentation, and development testing activities, including unifying these efforts across the Department and the Services. The conferees understand the importance of coordinating these activities to prevent duplication of effort while also preserving the service-specific applications of research and engineering activities. Musculoskeletal injury prevention research the House amendment contained a provision (sec. These jobs exist with the organic industrial base, research, development, and engineering centers, life-cycle management commands, and logistics centers of the Department. Briefing on cooperative defense technology programs and risks of technology transfer to China or Russia the Senate bill contained a provision (sec. Increase in funding for basic operational medical research science the House amendment contained a provision (sec. Funding for the Sea-Launched Cruise Missile-Nuclear analysis of alternatives the Senate bill contained a provision (sec. Increase in funding for university research initiatives the House amendment contained a provision (sec. Review and assessment pertaining to transition of Department of Defense-originated dual-use technology the Senate bill contained a provision (sec. No later than January 1, 2021, the university business school or law school should submit a report on the assessment to the Secretary of Defense with any recommendations for changes to statute, regulations, or policy. No later than February 1, 2021, the Secretary of Defense shall deliver the report to the Committees on Armed Services of the Senate and House of Representatives along with any relevant Department of Defense comments or recommendations. Quantum Information Science Innovation Center the House amendment contained a provision (sec. The provision would also increase the funding table authorization for research, development, test, and evaluation, Air Force, applied research, dominant information sciences and methods, line 014, by $10. The conferees note the modification of the existing authorization of a Defense Quantum Information Science and Technology Research and Development Program elsewhere in this bill. The conferees note that the Defense Quantum Information Science and Technology Research and Development Program includes the authority for each of the Secretaries of the military departments to establish or designate a Quantum Science Research Center. Increase in funding for Naval University Research Initiatives the House amendment contained a provision (sec. Increase in funding for university and industry research centers the House amendment contained a provision (sec. Increase in funding for national security innovation capital the House amendment contained a provision (sec. Increase in funding for Air Force University Research Initiatives the House amendment contained a provision (sec. Independent study on threats to United States national security from development of hypersonic weapons by foreign nations the House amendment contained a provision (sec. The conferees note that the development of hypersonic weapons is a critical national interest as highlighted in the John S. Report on innovation investments and management the House amendment contained a provision (sec. The conferees direct the Under Secretary of Defense for Research and Engineering, not later than December 31, 2019, to provide to the Committees on Armed Services of the Senate and House of Representatives a report on the efforts of the Department of Defense to improve innovation investments and management. The report shall include an explanation of the following: (1) How incremental and disruptive innovation investments for each military department are defined; (2) How such investments are assessed; and (3) Whether the Under Secretary has defined a science and technology management framework that emphasizes the greater use of existing flexible approaches to more quickly initiate and discontinue projects to respond to the rapid pace of innovation, incorporates acquisition stakeholders into technology development programs to ensure that they are relevant to customers, and promotes advanced prototyping of disruptive technologies within the defense labs so that the science and technology community can evaluate the effectiveness of these technologies and applications in future acquisition programs. Increase in funding for Army University Research Initiatives the House amendment contained a provision (sec. Funding for anti-tamper heterogenous integrated microelectronics the House amendment contained a provision (sec. Briefing on use of blockchain technology for defense purposes the House amendment contained a provision (sec. The conferees direct the Under Secretary of Defense for Research and Engineering to provide, not later than 180 days after the date of the enactment of this Act, to the congressional defense committees a briefing on the potential use of distributed ledger technology for defense purposes. This briefing shall include an explanation of how distributed ledger technology may be used by the Department of Defense to: (1) Improve cybersecurity, beginning at the hardware level, of vulnerable assets such as energy, water, and transport grids through distributed versus centralized computing; (2) Reduce single points of failure in emergency and catastrophe decisionmaking by subjecting decisions to consensus validation through distributed ledger technologies; (3) Improve the efficiency of defense logistics and supply chain operations; (4) Enhance the transparency of procurement auditing; and (5) Allow innovations to be adapted by the private sector for ancillary uses. The briefing shall also include any other information that the Under Secretary of Defense for Research and Engineering determines to be appropriate. Efforts to counter manipulated media content the House amendment contained a provision (sec. The conferees direct the Secretary of Defense not later than 180 days after the date of the enactment of this Act to provide a briefing to the congressional defense committees on initiatives of the DoD to identify and address, as appropriate and as authorized in support of DoD operations, manipulated media content, specifically "deepfakes. Additional amounts for research, development, test, and evaluation the Senate bill contained a provision (sec.
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Prognosis for recovery is good, although at times return of function is not complete. Reevaluate after two years for return of function, at which time it may be amenable for a schedule loss of use of the arm. Lower brachial plexopathy can be associated with surgery or falls on the abducted arm. Persistent severe weakness and intractable pain might necessitate considering a partial disability which might lead to a classification. An anomalous cervical rib arising from the 7th cervical vertebra can extend laterally between the anterior and medial scalene muscles disturbing the outlet and compressing the brachial plexus. Five tenths percent of the population have cervical ribs, ten percent of which are symptomatic. The technique of performance of the test for obstruction of the subclavian artery by the scalenus anticus muscle is as follows: claimant is seated with elbows at sides and neck extended. During deep inspiration the chin is turned downwards towards the affected side while the radial pulse is palpated and there may be total obliteration. It can be confused with cervical discs, carpal tunnel syndrome or ulnar nerve compression at the elbow. Occasionally there is a retrograde distribution of discomfort extending to the neck and shoulders, which may present a problem in differential diagnosis from cervical radiculitis. Prolongation of median motor latencies is found when the median nerve is stimulated at the wrist. Conservative measures may be use of wrist support and splints, anti-inflammatory medication, use of whirlpool and/or paraffin bath, and stretching exercises of the transverse carpal ligament. The usual surgical treatment in cases with progression of symptoms such as numbness, weakness and muscle atrophy is a transection of the transverse carpal ligament which decompresses the median nerve. Carpal Tunnel Syndrome with or without decompression are usually given a schedule loss of the hand which usually averages 10-20% loss of use. Pressure may occur during anesthesia but more commonly the nerve is injured by being drawn tightly against the ulnar groove. Entrapment of the ulnar nerve at the elbow is usually given a schedule loss of use of the arm if accompanied with defects at the elbow. Wrist Wrist injury of the ulnar nerve: the palmar trunk and superficial branches are subject to direct trauma by force directed against the base of the hypothenar eminence as the bone rests on the thinly padded bone. The force may be a repetitive one as from use of a particular tool or instrument in industry such as pliers or a screwdriver. The most significant symptom at this level is weakness of the pinch power of the thumb and sensory loss occurs in the ring and small fingers. Occult trauma such as forceful repeated pronation accompanying forceful finger flexion causes a hypertrophy of the pronator muscle which tautens the sublimis edge and compresses the median nerve. In the Pronator Teres Syndrome, thenar atrophy is not as severe as in carpal tunnel syndrome. The traumatic injury may be a dislocation of the elbow, fracture of the ulna with dislocation of the radial head and radial head fractures. The posterior interosseous nerve can be injured by the compression plates used in the open reduction of fractures of the proximal radius. If the examiner finds a defect of the elbow joint that is causally related, the schedule loss of use is given to the arms. This usually manifests into two distinct entities: a motor syndrome, and a rarer entity, a pain syndrome. The pain syndrome is also called radial tunnel syndrome, resistant tennis elbow and clinically resembles a painful tennis elbow. The ensuing neuropathy causes the burning type pain over the anterolateral thigh with some hypaesthesia. Pressure over the nerve may cause pain into the distribution of the posterior tibial nerve. In severe cases, the claimant may be crippled and demoralized (temporary total disability). Trophic changes are common: red and glossy skin, excessive or diminished sweating, and osteoporosis. The mechanism of causalgia is unknown, although it most often occurs in partially injured nerves suggesting a transient demyelinization between the nerve fibers wherein the short circuiting sympathetic impulses activate pain fibers. A delay in surgery is generally advisable in peripheral nerve and plexus injuries that are closed. It is important to review medical records, hospitalization and diagnostic tests (i. The examining physician must perform a thorough examination of affected areas and describe active and residual lesions. Common disfigurements of the eye include corneal scarring; defects of the iris and in some instances total loss of the eye with use of a prosthesis. Common disfigurements of the lips include loss of soft tissue, enlargement, and alteration of normal contour of the lips. Common disfigurements of the ear include loss of tissue and alteration of normal contour of the ear. Permanent scars and disfigurement of the face and neck are usually evaluated one year post-injury and/or one year after the last surgical procedure was performed. The scar and disfigurement should be described accurately, using such parameters as length, width, color, contour, and exact location. Physical Examination: Note general appearance, weight, habitus, type of breathing, blood pressure, pulse rate, heart sounds, lung sounds, signs of heart failure, and edema. Assess and review functional capabilities, physical restriction, level of activity causing symptoms, and ability to perform activity of daily living. Dyspnea is a major criterion in the assessment of the severity of respiratory impairment. Listed below are key parameters that should be considered in the review of medical records. The claimant has a causally related respiratory disorder and/or impairment with pulmonologist documentation and an appropriate diagnostic test. The claimant is asymptomatic and stable, takes little or no medication and has complaints. The claimant is able to perform usual tasks and activities of daily living without dyspnea. Dyspnea occurs on minimal physical exertion such as usual housework and activities of daily living, walking one block on level ground and/or climbing one flight of stairs. The claimant has a causally related respiratory disorder and/or impairment with a pulmonologist documentation and an appropriate diagnostic test. The claimant is symptomatic, under active respiratory care, may be confined to a chair or bed, may be O2 dependent, has multiple complaints and needs medication to control symptoms. There are positive findings on physical examination such as cyanosis, clubbing of the digits and positive lung findings. Other cardiovascular diseases are seldom claimed to be work related and may be found to be preexisting conditions, i. The claimant has a history of documented myocardial infarction, myocardial ischemia and/or angina. The claimant has a history of documented myocardial infarction myocardial ischemia and/or angina that occurred at least one year before the time of evaluation. There are complaints of recurrent angina and/or shortness of breath on mild exertion. The claimant needs medication to control angina, congestive heart failure and/or arrhythmias. The claimant may have physical findings of congestive heart failure and/or arrhythmias. The claimant becomes symptomatic when walking one block and/or climbing one flight of stairs. There is a history of documented myocardial infarction, myocardial ischemia and/or unstable angina. Claimant is partially recovered or may be failed and required continuous coronary care.
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The Senate recedes with an amendment that, among other changes clarifying the process of preparing and delivering the aforementioned report, would require the Comptroller General of the United States to provide the report to the congressional committees described. The conferees note that Japan and the Republic of Korea have made significant contributions to common security, including through direct, indirect, and burden-sharing contributions. Therefore, the conferees believe that upcoming negotiations concerning new Special Measures Agreements with Japan and the Republic of Korea should be conducted in a spirit consistent with prior negotiations on the basis of common interest and mutual respect. The House recedes with an amendment that would express the sense of Congress that the United States strongly encourages strengthened bilateral security ties between Japan and the Republic of Korea as well as deeper trilateral defense coordination and cooperation, including through expanded exercises, training, senior-level exchanges, and information sharing. The amendment would also express that the following bilateral and trilateral agreements are critical to regional security, and should be maintained: the bilateral military intelligence-sharing pact between Japan and the Republic of Korea, signed on November 23, 2016; and the trilateral intelligence sharing agreement among the United States, Japan, and the Republic of Korea, signed on December 29, 2015. The Senate recedes with an amendment that would express the sense of Congress that, among other things, a sustained credible diplomatic process based on concrete measures to achieve the denuclearization of North Korea and an eventual end to the Korean War should be pursued. Statement of policy and sense of Congress on, and strategy to fulfill obligations under, Mutual Defense Treaty with the Republic of the Philippines (sec. The provision would also express the sense of the Senate that the Secretary of State and the Secretary of Defense should: affirm the commitment of the United States to the Mutual Defense Treaty between the United States and the Republic of the Philippines; preserve and strengthen the alliance of the United States with the Republic of the Philippines; prioritize efforts to develop a shared understanding of alliance commitments and defense planning; and provide appropriate support to the Republic of the Philippines to strengthen the self-defense capabilities of the Republic of the Philippines, particularly in the maritime domain. The House recedes with an amendment that would include the statement of policy from the Senate provision, express the sense of the Congress with the respect to the objectives of the Secretary of State and Secretary of Defense as described in the Senate provision, and require, not later than 1 year after the date of enactment of this Act, the Secretary of Defense, in consultation with the Secretary of State, to submit to the appropriate committees of Congress a report that sets forth the strategy of the Department of Defense for achieving such objectives. The Senate recedes with an amendment that would require, not later than 150 days after the date of the enactment of this Act, the Secretary of Defense, in concurrence with the Secretary of State, to submit to the appropriate congressional committees a report concerning security sector assistance programs with the Philippine National Police. The provisions would also modify the specified congressional committees that receive the annual report, and add certain elements to the annual report. The Senate recedes with an amendment that would add certain elements to the annual report. The provision would direct a report to be delivered to the appropriate committees of Congress on the results of the review. The House recedes with an amendment that would make clarifying changes to the content of the required report. The House recedes with an amendment that would express the sense of the Congress that Taiwan is a vital partner of the United States, and that the United States should continue to strengthen defense and security cooperation in support of Taiwan maintaining a sufficient self-defense capability. In light of the fortieth anniversary of the Taiwan Relations Act (Public Law 96-8), the conferees encourage the Department of Defense to focus attention and resources on the future of the United States-Taiwan defense relationship, particularly in relation to implementation of the National Defense Strategy and strategic competition with China. The Senate recedes with an amendment that would strike the findings in the provision. The amendment would call for efforts to resolve the remaining demands raised by protestors, who represent a broad cross-section of Hong Kong. The House recedes with an amendment that would express the sense of the Congress that robust defense and security cooperation between the United States and the Republic of Singapore is crucial to promoting peace and stability in the Indo-Pacific region. The House recedes with an amendment that would require the Secretary of Defense to notify the congressional defense committees prior to such a transfer of funds. The Senate recedes with an amendment that would modify the conditions for removal from the entity list and add a reporting requirement for licenses issued for exports to Huawei. The provision would also require the Comptroller General to submit to the congressional defense committees a report containing an analysis of the current status of the distributed laydown. The House recedes with an amendment that would add certain elements of the report to focus its contents on the implementation of the planned distributed lay-down of U. Marines in the Indo-Pacific region and strikes the requirement for a Comptroller General report. The conferees note that nothing in this provision shall be construed to change the current distributed lay-down of U. Any potential changes to the distributed lay-down should be considered only after consultation and agreement of impacted countries, especially the Government of Japan. The provision would make an exception for a country that already had in effect an agreement under section 123 as of June 19, 2019. The provision would allow for a waiver of such prohibition if the President submits a report to the appropriate congressional committees, and the Congress enacts a joint resolution of approval of the waiver. The Senate recedes with an amendment that would remove the requirement for a joint resolution of approval of the waiver. The waiver would instead take effect 90 days after the submission of the report to the appropriate congressional committees. The Senate recedes with an amendment that would modify the elements of the required assessment. The Senate recedes with an amendment that, among other changes, would require the Secretary of Defense to coordinate with the Secretary of State on the report and add additional elements to the report, including U. The Senate recedes with an amendment that would require that prior to the transfer of vehicles by the Department of Defense to a joint task force of the Ministry of Defense or the Ministry of Interior of Guatemala during fiscal year 2020, the Secretary of Defense must certify to the appropriate congressional committees that such ministries have made a credible commitment to use such equipment only for the uses for which they were intended. Briefing on strategy to improve the efforts of the Nigerian military to prevent, mitigate, and respond to civilian harm (sec. The provision would also require the President to provide annual updates on progress made with respect to the plan contained in such report. The Senate recedes with an amendment that would require not later than 180 days after the date of enactment of this Act the Secretary of Defense and the Secretary of State to jointly provide a briefing to specified congressional committees on the current strategy to improve defense institutions and security sector forces in Nigeria. The conferees expect the briefing to discuss steps, if any, that the Departments are taking to increase the ability of the Nigerian military to minimize civilian harm when using equipment provided by the United States, to include the A-29 Super Tucano and related munitions. Rule of construction on the permanent stationing of United States Armed Forces in Somalia (sec. The Senate recedes with an amendment to include a rule of construction that states that nothing in this Act may be construed to authorize the permanent stationing of members of the United States Armed Forces in Somalia. The Senate recedes with an amendment that modifies the elements of the required strategy. Reports on expenses incurred for in-flight refueling of Saudi coalition aircraft conducting missions relating to civil war in Yemen (sec. Extension and modification of authority for United States-Israel anti-tunnel cooperation activities (sec. The provision would remove countering unmanned aerial systems from the section 1279 authority. Elsewhere in this Act, the committee recommends a provision that would establish a separate authority for United States-Israel cooperation regarding countering unmanned aerial systems. The provision would also authorize the Secretary of Defense to use amounts available under the section 1279 authority, which are in excess of the amount contributed by the Government of Israel, for costs associated with unique national requirements identified by the United States with respect to anti-tunnel capabilities. The House recedes with an amendment that would extend the section 1279 authority through December 31, 2024. The House recedes with an amendment that, among other clarifying changes, would require the Secretary of Defense to consult with the heads of other Federal departments and agencies as appropriate in the preparation of the report. McCain National Defense Authorization Act for Fiscal Year 2019 to specify that the training and support provided under such program shall emphasize best practices for the protection of sensitive national security information and include the dissemination of unclassified publications and resources. The House recedes with an amendment that would: (1) Combine sections 1285 and 6219 with minor modifications; (2) Amend section 1286 of the John S. McCain National Defense Authorization Act for Fiscal Year 2019 to require the Secretary of Defense to establish enhanced information sharing procedures to collect appropriate information on any personnel participating in defense research and development activities other than basic research and to maintain appropriate security controls over research activities, technical information, and intellectual property; and (3) Amend the required report in the same section to be an annual report. The conferees note the important impact that United States academic institutions have had advancing emerging technologies and contributing to the defense research enterprise. The conferees, however, note the efforts undertaken by foreign adversaries and competitors to exploit the open academic environment through the theft of intellectual property, improper technology transfer, and espionage. The conferees encourage the Secretary of Defense to establish a memorandum of understanding with the Secretary of Homeland Security in order to coordinate the implementation of the enhanced information sharing required in this provision. The conferees note, that to greatest extent possible, the Secretary of Defense should streamline information sharing procedures and leverage existing government information systems and repositories, including the Student Exchange Visitor Information System, to reduce the burden on universities and the Department of Defense. McCain National Defense Authorization Act for Fiscal Year 2019 (Public Law 115-232) relating to civilian casualty matters. McCain National Defense Authorization Act for Fiscal Year 2019 (Public Law 115-232) to modify the responsibilities of the senior civilian official of the Department of Defense designated to develop, coordinate, and oversee compliance relating to civilian casualties. The conferees direct the senior designated official to coordinate with other relevant U. The Senate recedes with an amendment that would establish that nothing in this Act or any amendment made by this Act may be construed to authorize the use of military force, including the use of military force against Iran or any other country. Reports and briefings on use of military force and support of partner forces (sec. The Senate recedes with an amendment that would require the President not later than 180 days after the date of the enactment of this Act, and every 180 days thereafter, to submit to the congressional defense committees, the Committee on Foreign Relations of the Senate, and the Committee on Foreign Affairs of the House of Representatives a report on actions taken pursuant to the Authorization for Use of Military Force (Public Law 107-40) against those countries or organizations described in such law, as well as any actions taken to command, coordinate, participate in the movement of, or accompany the regular or irregular military forces of any foreign country or government when such forces are engaged in hostilities or in situations where imminent involvement in hostilities is clearly indicated by the circumstances, during the preceding 180-day period. The conferees are aware that the lack of data would result in incomplete information on recipients of security cooperation training that have been designated for human rights abuses or terrorist activities.
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Patients often have an enlarged liver, which may be discovered by physical exam, and elevated blood levels of the liver enzyme aminotransferase. Fibrosis may occur earlier than usual in patients with viral hepatitis (particularly hepatitis C), non-alcoholic fatty liver disease, and/or alcohol abuse. Heart disease may include cardiomyopathy (weakening and enlargement of the heart muscle), irregular heartbeats, or heart failure. Screening is performed using blood tests to measure transferrin saturation, ferritin, and unsaturated iron binding capacity. Patients who develop iron overload at an early stage in their blood transfusion history or who have a family history of primary iron overload should undergo genetic testing for hemochromatosis, an inherited disorder that causes the body to absorb too much iron. Oral chelation should be chosen and monitored in consultation with a physician with some experience with these agents. Many families view food, and by extension, dietary supplements, vitamins, and micronutrients, as "natural" and thus safe. Many complementary/alternative nutritional regimes and supplements are directly harmful or, by displacing standard medical therapy, indirectly harmful. Controlled clinical trials of 94 Chapter 4: Gastrointestinal, Hepatic, and Nutritional Problems supplements are necessary to demonstrate effectiveness and limit the risk of toxicity. Establishing a non-judgmental, but candidly informative discussion of complementary and alternative therapies offers the physician a chance to educate parents about their choices. Physicians and families can access information about complementary/alternative nutritional therapies at the website of the Office of Complementary and Alternative Medicine of the National Institutes of Health, available at. Pediatric neurogastroenterology: gastrointesinal motility and functional disorders in children. Rodriguez L, Diaz J, Nurko S (2013) Safety and efficacy of cyproheptadine for treating dyspeptic symptoms in children. Westaby D, Portmann B, Williams R (1983) Androgen-related primary hepatic tumors in non-Fanconi patients. A so-called "floating" thumb that lacks bones and is composed of skin and soft tissue. This physician should be comfortable with and proficient in the diagnosis and management of congenital limb anomalies. Furthermore, radial deficiency- incomplete formation of the radius-is associated with numerous syndromes, further emphasizing the need for a thorough investigation (Table 1). This deficiency is more involved and is characterized by a narrowing of the web space between the thumb and index finger, 102 Chapter 5: Hand and Arm Abnormalities absence of the thenar (thumb) muscle at the base of the thumb, and instability of the metacarpophalangeal joint in the middle of the thumb (Figures 1A and B). A) Absent thenar muscles; B) Narrowed thumb-index web space with instability of the metacarpophalangeal joint. These abnormalities usually involve tendons that arise within the forearm and travel into the thumb. The thumb classifications listed above can guide treatment recommendations, as shown in Table 2 (3,4, 5). A) Z-plasty of the narrowed thumb-index web space; B) tendon transfer to overcome the deficient thenar muscles; C) ligament reconstruction to stabilize the metacarpophalangeal joint instability. In cases with severe instability, fusion of the joint may be the best option to provide a stable thumb for firm grasps. The main distinction between a thumb that can be surgically reconstructed and a thumb that requires amputation is the presence or absence of a stable base. The decision to remove a hypoplastic thumb without a stable base is often a difficult process for parents and caregivers. Discussions with the surgeon and conversations with families who have made similar decisions are often helpful to parents tasked with making this decision for their child (Video 1 in online supplementary information). Because an opposable thumb is critical for manipulating many objects, a functional replacement can be constructed by surgically moving the index 106 Chapter 5: Hand and Arm Abnormalities finger and its nerves, arteries, tendons, and muscles to the thumb position. Good results shortly after pollicization have been shown to persist into adulthood (6,7). Figure 8A Figure 8B (see Figure legend on next page) 108 Chapter 5: Hand and Arm Abnormalities Figure 8. A) Thumb used for grasping large objects; B) mobile thumb incorporated into fine pinch. The alignment and length of this type of thumb must be monitored until the bones have finished growing. A small wedge-shaped bone can be surgically removed and the ligaments of the remaining bones can be reconstructed to form a functional joint. A large wedge-shaped phalanx will cause the thumb to curve and become excessively long, but removal is not recommended because joint instability is common after surgery. A better option involves removing only the wedge-shaped portion of the abnormal phalanx and fusing the remainder to an adjacent thumb bone. A) Clinical appearance with mild angulation; B) X-rays show an extra phalanx that is triangular in shape causing the angulation. The thumbs may be partial and appear fused together, or they may be complete and separate from each other. Thumb duplications have been classified into various types depending on the degree of skeletal replication (Table 3) (8, 9). The soft tissues from the amputated thumb, including the skin, nail, ligaments, and muscle, should be used to augment the retained thumb. Radial Deficiency Radial deficiency is a condition in which the radius-the bone that runs along the thumb side of the forearm-develops abnormally. The severity of radial deficiency is variable and can be determined through X-rays and clinical examination. These are the mildest forms and are charac terized by little or no shortening of the radius and negligible curvature in the ulna. The hand may be tilted slightly inward toward the thumb side of the arm, a condition known as a radial deviation of the wrist, and substantial thumb hypoplasia may be present that requires treatment. A) Xray reveals complete absence of the radius; B) hand with a perpendicular relationship with the forearm. The maturation of the radius takes more time than usual in patients with radial deficiency; therefore, the differentiation between total and partial absence (Types 3 and 4) cannot be determined until the child is approximately 3 years of age. Functional consequences of radial deficiency the outcome of radial deficiency depends on the severity of the abnormality. In a patient with a Type 4 deficiency, the humerus (the bone between the elbow and shoulder) may be shorter than expected and the elbow may not be able to bend properly. In cases of partial or complete absence of the radius, the forearm will not be able to rotate, although some rotation may occur through the wrist or carpal bones. The wrist may be shifted a variable amount towards the deficient radius, a condition known as a radial deviation. The radial artery and nerve are also often absent, although the ulnar nerve and artery are normal (13). An enlarged median nerve substitutes for the absent radial nerve and communicates with its dorsal nerve branch, which is positioned in the fold between the wrist and forearm, to provide sensation to the thumb side of the hand. Partial or complete absence of the radius is more common (Types 2, 3, and 4) and is entirely more difficult to treat because the wrist has shifted toward the thumb side of the arm, shortening an already undersized forearm, placing the forearm flexor and extensor tendons at a awkward angle, and producing functional deficits. Children who have radial deficiency on only one arm (known as a unilateral deficiency) may be able to compensate for any functional deficits using their unaffected limb and thus have a lower overall degree of functional impairment than children who have radial deficiency on both arms (known as a bilateral deficiency). Finger and thumb abnormalities, if present, also require consideration during the formulation of a treatment plan, as stiff fingers and a deficient thumb will further hamper pinch and grasp. This treatment is typically performed both by a physical or occupational therapist and the caregiver. Surgical centralization requires placing the wrist on top of the ulna to realign the carpus onto the distal ulna. This procedure is known as "centralization" or "radialization" depending on the exact position in which the wrist is placed, and remains the standard treatment for realigning the wrist (14,15). Centraliza tion involves releasing and reorganizing the tight muscles and tendons of the wrist, and positioning the hand over the end of the ulna (Figure 12). One end 115 Fanconi Anemia: Guidelines for Diagnosis and Management of a functioning tendon is then shifted from its original attachment site to the wrist to rebalance the forces acting on the wrist, a procedure known as tendon transfer.
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Subcutaneous infusions pose a risk of bleeding or infection in patients with thrombocytopenia or neutropenia. Patients who develop a fever should immediately cease deferoxamine therapy and undergo medical evaluation. Deferasirox is conveniently administered orally once a day as a slurry with a variety of palatable beverages, however more palatable preparations are forthcoming. The optimal dose of deferasirox is between 20-40 mg/kg, which can maintain iron balance in most patients, but unlike deferoxamine, may not be sufficient to reduce iron overload. However, the utility of deferiprone is limited by its side effects, which include neutropenia and fatal agranulocytosis, a particular concern in individuals with bone marrow failure, and arthralgias and arthritis. If transplant is not pursued, then thrombocytopenia should be treated with androgens as the platelet count declines toward 30,000/mm3. As noted above, a long trial of oxymetholone or danazol (up to 6 months) is required before treatment is considered unsuccessful due to the lack of a platelet response or unacceptable side effects. Platelet transfusion is indicated in patients with severe bruising or bleeding, or who are undergoing invasive procedures. Drugs that inhibit platelet function, such as aspirin, non-steroidal anti-inflammatory drugs. Supplements and foods such as omega 3s, flax seed and green tea are associated with increased bleeding and should be avoided in thrombocytopenic individuals and in anyone anticipating surgery. Activities carrying a high risk of significant trauma (particularly to the head or trunk) should be avoided. Patients with fever and neutropenia should have a thorough examination, 67 Fanconi Anemia: Guidelines for Diagnosis and Management have samples of their blood cultured in a lab, and should receive broadspectrum antibiotics until the blood cultures test negative for infection and the fevers resolve. Such practices may lead to increased risks of fungal infections and antibiotic resistance. The use of local anesthetic alone may be insufficient to alleviate the anxiety and pain that is associated with frequent, repeated bone marrow procedures. Huck K, Hanenberg H, Gudowius S, Fenk R, Kalb R, Neveling K, Betz B, Niederacher D, Haas R, Gobel U, Kobbe G, Schindler D (2006) Delayed diagnosis and complications of Fanconi anaemia at advanced age-a paradigm. Parmentier S, Schetelig J, Lorenz K, Kramer M, Ireland R, Schuler U, Ordemann R, Rall G, Schaich M, Bornhauser M, Ehninger G, Kroschinsky F (2012) Assessment of dysplastic hematopoiesis: lessons from healthy bone marrow donors. Masserot C, Peffault de Latour R, Rocha V, Leblanc T, Rigolet A, Pascal F, Janin A, Soulier J, Gluckman E, Socie G (2008) Head and neck squamous cell carcinoma in 13 patients with Fanconi anemia after hematopoietic stem cell transplantation. Scheckenbach K, Morgan M, Filger-Brillinger J, Sandmann M, Strimling B, Scheurlen W, Schindler D, Gobel U, Hanenberg H (2012) Treatment of the bone marrow failure in Fanconi anemia patients with danazol. Respiratory problems, including cough, pneumonia, and wheezing may suggest the need for bronchoscopy, a procedure that enables clinicians to look inside the airways. Approximately 90% of infants survive the surgical repair of the intestines, and will grow normally and develop few symptoms. Management of these complications requires a multidisciplinary 77 Fanconi Anemia: Guidelines for Diagnosis and Management approach. Long-term problems may include fecal incontinence, occasional soiling, and constipation with or without encopresis (involuntary leakage of stool) (6). During routine clinic visits, clinicians should encourage patients and their families to report gastrointestinal symptoms, as patients often do not spontaneously disclose these concerns. Nausea is usually temporary, resolving once the infection has been cured or the medication stopped. Psychological stress, anxiety, and depression can also lead to nausea and abdominal pain, and may worsen existing gastrointestinal complaints. Short bowel syndrome occurs when nutrients from food are not properly absorbed because a large segment of the small intestine is non-functional or has been surgically removed. Constipation with accidental leakage of stool may be mistaken by some families for diarrhea. If the patient has non-specific poor food intake, with or without nausea and abdominal pain, evaluation for evidence of an unobvious infection may be useful. Patients with diarrhea should have stool examination for ova and parasites, giardia and cryptosporidia antigen, and other opportunistic agents. In some cases, digital radiographs may deliver less radiation than conventional techniques and are thus preferred. Gastritis and other peptic diseases should be diagnosed by a procedure called endoscopy with biopsies without radiographic imaging. Evaluation of gastric emptying delay Gastric emptying delay should be suspected in patients who experience nausea, feel full sooner than usual, and vomit food eaten several hours earlier. Delayed gastric emptying in the general population is commonly diagnosed using the nuclear medicine gastric emptying study, which involves radiation. Ultrasound-based diagnosis of delayed gastric emptying may be available at some clinics. If the diagnosis of delayed gastric emptying is entertained, the patient should undergo dietary counseling with a dietitian to adjust meal content and frequency; small and frequent meals that restrict fats and nondigestible fibers while maintaining adequate caloric intake should be favored. The use of metoclopramide is not recommended because of potentially dangerous side effects including irreversible tardive dyskinesia, a movement disorder characterized by repetitive and involuntary movements. Amoxicillin/clavulanic acid has been shown to improve small intestine motility and may be prescribed when the above 80 Chapter 4: Gastrointestinal, Hepatic, and Nutritional Problems medications have failed or if a patient is not tolerating jejunal feeds (feeding directly into the small intestine) (20 mg/kg amoxicillin and 1 mg/kg clavulinate twice a day, with a maximum of 250 mg of amoxicillin 3 times a day) (8, 9). These patients may benefit from treatment with the medication cyproheptadine, given 30 minutes before meals. In cases of severe, uncontrollable nausea without a detectable cause, a trial of the medication ondansetron may be warranted if there is no improvement with cyproheptadine or domperidone. Parents should be encouraged to accept as normal a child whose weight is appropriate for their somewhat short height. Children who are "picky eaters" and their families may benefit from behavioral therapies to increase the variety of foods eaten. This strategy involves delivering a liquid food mixture directly into the bloodstream, stomach, or small intestine, thereby bypassing appetite and food interest. In this way, supplemental feeding allows the child to achieve normal growth to meet his/her genetic potential, have the energy to meet the demands of daily living, and store adequate nutritional reserves to face short-term malnourishment during acute illness. Supplemental feeding via feeding tube, known as enteral supplementation, is preferable to supplementation by intravenous infusion, known as parenteral nutrition. Enteral supplementation may be delivered by feeding tubes inserted into the nose, such as a nasogastric tube or nasojejunal tube, or by a tube surgically inserted into the abdomen, known as a gastrostomy tube. Most patients tolerate nasal tubes well; the major objection, particularly among older children, is the unattractive nature of a visible tube in the nose. Nonetheless, for patients who need supplemental feedings for less than 3 months, the nasal route is the best. It should be noted, however, that nasal tubes increase the risk of sinus infection. Dislodged tubes must be replaced by a radiologist using an X-ray-based imaging technique known as fluoroscopy. Gastrostomy tubes provide more permanent access to the gastrointestinal tract for administration of enteral feedings. Placement requires a brief surgical procedure, generally performed by endoscopy, in which a small camera on the end of a thin, flexible tube is inserted into the gastrointestinal tract. In general, complications are limited to local irritation and/or infection, which can be treated with antibiotic ointments applied directly at the site of infection, rather than oral antibiotics that act on the whole body. Rarely, the gastrostomy tube can become dislodged, increasing the risk of infection. To improve daytime appetite, supplemental feedings can be given over a period of 8-10 hours at night, using a high-calorie formula, if possible; patients may still refuse breakfast, but are generally hungry by lunch. Once an appropriate weight-for-height has been attained, it may be possible to reduce the number of days of the week supplementation is given. Usually, a 84 Chapter 4: Gastrointestinal, Hepatic, and Nutritional Problems dietitian or physician can make simple modifications to the therapy that will alleviate these symptoms. It is also advisable that patients monitor blood sugar levels regularly when on a high-calorie diet. Appetite stimulants Several medications have alleged appetite-stimulating side effects. Before prescribing appetite stimulants, physicians must first investigate and appropriately manage diagnosable causes of poor appetite and inadequate growth. Cyproheptadine, an antihistamine used to treat allergic reactions, is a popular appetite stimulant because it has few side effects besides temporary sleepiness. Patients may benefit from cyproheptadine, as it improves gastric accommodation to reduce retching (14). While a full discussion of the management of overweight and obesity is beyond the scope of this chapter (see references 16-18 for a review), some useful starting points can be offered.
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Reattachment of hand, reattachment of avulsed kidney Freeing a body part Eliminating an abnormal constraint of a body part by cutting or by use of force. Some of the restraining tissue may be taken out but none of the body part is taken out. Drainage tube removal, cardiac pacemaker removal Restoring, to the extent possible, a body part to its normal anatomic structure and function Used only when the method to accomplish the repair is not one of the other root operations Herniorrhaphy, suture of laceration Putting in or on biological or synthetic material that physically takes the place of all or a portion of a body part the biological material is non-living, or the biological material is living and from the same individual. If the body part has been previously replaced, a separate Removal procedure is coded for taking out the device used in the previous replacement. Total hip replacement, free skin graft Moving to its normal location or other suitable location all or a portion of a body part the body part is moved to a new location from an abnormal location, or from a normal location where it is not functioning correctly. Reposition of undescended testicle, fracture reduction Cutting out or off, without replacement, all of a body part None Total nephrectomy, total lobectomy of lung Partially closing an orifice or the lumen of a tubular body part the orifice can be a natural orifice or an artificially created orifice. Esophagogastric fundoplication, cervical cerclage Correcting, to the extent possible, a malfunctioning or displaced device Examples Removal Definition Explanation Examples Repair Definition Explanation Examples Replacement Definition Explanation Examples Reposition Definition Explanation Examples Resection Definition Explanation Examples Restriction Definition Explanation Examples Revision Definition A. Adjustment of pacemaker lead, adjustment of hip prosthesis Moving, without taking out, all or a portion of a body part to another location to take over the function of all or a portion of a body part the body part transferred remains connected to its vascular and nervous supply. Tendon transfer, skin pedicle flap transfer Putting in or on all or a portion of a living body part taken from another individual or animal to physically take the place and/or function of all or a portion of a similar body part the native body part may or may not be taken out, and the transplanted body part may take over all or a portion of its function. The purpose of the alphabetic index is to locate the appropriate table that contains all information necessary to construct a procedure code. If the documentation is incomplete for coding purposes, the physician should be queried for the necessary information. Medical and Surgical section (section 0) - Body system guidelines B2. In the root operations Change, Removal and Revision, when the specific body part value is not in the table. Example: Esophagus is a general body part value; Esophagus, Upper is a specific body part value. Three body systems contain body part values that represent general anatomical regions, upper extremity anatomical regions, and lower extremity anatomical regions respectively. These body part values are used when a procedure is performed on body layers that span more than one body system. Example: Debridement of skin, muscle, and bone at a procedure site is coded to the anatomical regions body systems. Exception: Composite tissue transfers are coded to the specific body systems (Muscles or Subcutaneous Tissue and Fascia). Body systems designated as upper or lower contain body parts located above or below the diaphragm respectively. Example: Upper Veins body parts are above the diaphragm; Lower Veins body parts are below the diaphragm. In order to determine the appropriate root operation, the full definition of the root operation as contained in the Tables must be applied. Components of a procedure necessary to complete the objective of the procedure specified in the root operation are considered integral to the procedure and are not coded separately. The same root operation is performed on different body parts as defined by distinct values of the body part character. The same root operation is repeated at different body sites that are included in the same body part value. Example: Excision of the sartorius muscle and excision of the gracilis muscle are both included in the upper leg muscle body part value, and multiple procedures are coded. Destruction of separate skin body sites on the face are all included in the body part value Skin, Face, and multiple procedures are coded. Multiple root operations with distinct objectives are performed on the same body part. Example: Destruction of sigmoid lesion and bypass of sigmoid colon are coded separately. The intended root operation is attempted using one approach, but is converted to a different approach. Example: Laparoscopic cholecystectomy converted to an open cholecystectomy is coded as endoscopic Inspection and open Resection. If the intended procedure is discontinued, code the procedure to the root operation performed. If a procedure is discontinued before any other root operation is performed, code the root operation Inspection of the body part or anatomical region inspected. Example: Ureteroscopy with unsuccessful extirpation of ureteral stone is coded to Inspection of ureter. Bypass procedures are coded according to the direction of flow of the contents of a tubular body part: the body part value identifies the origin of the bypass and the qualifier identifies the destination of the bypass. Example: Bypass from stomach to jejunum, stomach (origin) is the body part and jejunum (destination) is the qualifier. If multiple coronary artery sites are bypassed, a separate procedure is coded for each coronary artery site that uses a different device and/or qualifier. Example: Aortocoronary artery bypass and internal mammary coronary artery bypass are coded separately. If an attempt to stop postprocedural bleeding is unsuccessful and requires performing Bypass, Detachment, Excision, Extraction, Reposition, Replacement, or Resection to stop the bleeding, then that root operation is coded instead of Control. Example: Resection of spleen to stop postprocedural bleeding is coded to Resection instead of Control. If a diagnostic excision (biopsy) is followed by a therapeutic excision at the same procedure site, or by resection of the body part during the same operative episode, code only the therapeutic excision or resection. Example: Biopsy of breast followed by partial mastectomy at the same procedure site, only the partial mastectomy procedure is coded. Inspection of a body part(s) integral to the performance of the procedure is not coded separately. Example: Fiberoptic bronchoscopy with irrigation of bronchus, only the irrigation procedure is coded. If multiple body parts are inspected, the body part character is defined as the general body part value that identifies the entire area inspected. If no general body part value is provided, the body part character is defined as the most distal body part inspected. Example: Laparoscopy of pelvic organs is coded to the pelvic region body part value. Cystoureteroscopy with inspection of bladder and ureters is coded to the ureter body part value. If the sole objective of the procedure is separating a nontubular body part, the root operation is Division. If the sole objective of the procedure is freeing a body part without cutting the body part, the root operation is Release. In the root operation Release, the body part value coded is the body part being freed and not the tissue being manipulated or cut to free the body part. Example: Lysis of intestinal adhesions is coded to one of the intestine body part values. If multiple vertebral joints included in the same body part value are fused, a separate procedure is coded for each joint that uses a different device and/or qualifier. Example: Fusion of C-4/5 with fixation device and C-5/6 with bone graft are coded separately. Example: Putting a pin in a nondisplaced fracture is coded to the root operation Insertion. Casting of a nondisplaced fracture is coded to the root operation Immobilization in the Placement section. Putting in a mature and functioning living body part taken from another individual or animal is coded to the root operation Transplantation. Putting in autologous or nonautologous cells is coded to the Administration section. Example: Putting in autologous or nonautologous bone marrow, pancreatic islet cells or stem cells is coded to the Administration section. If a procedure is performed on a portion of a body part that does not have a separate body part value, code the body part value corresponding to the whole body part. Example: A procedure performed on the alveolar process of the mandible is coded to the mandible body part. If the prefix "peri" is used with a body part to identify the site of the procedure, the body part value is defined as the body part named. Example: A procedure site identified as perirenal is coded to the kidney body part.