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Query: UMLS:C0017168 (gastroesophageal reflux disease)
 11,783 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dysfunction of the upper esophageal sphincter was found in five out of 44 children with gastroesophageal reflux. Three of the five children had mental retardation associated with Silver Russell syndrome, 5p syndrome, or minimal change myopathy. The five patients had swallowing disorders, vomiting, and failure to thrive; four also had pulmonary aspiration. Esophageal manometry showed incomplete upper esophageal sphincter relaxation in two patients, upper esophageal sphincter relaxation incoordinated with pharyngeal contractions in two other patients, and both incomplete and incoordinated upper esophageal sphincter relaxation in the last patient. Intensive and successful treatment of gastroesophageal reflux did not improve swallowing or symptoms of pulmonary aspiration in four children. The fifth patient underwent cricopharyngeal myotomy, with complete resolution of respiratory and swallowing symptoms. It is suggested that a dysfunction of the upper esophageal sphincter, either primary or secondary to neuromuscular disorders, may play a role in the swallowing disorders and respiratory symptoms of pediatric patients.
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PMID:Disorders of upper esophageal sphincter motility in children. 368 74

The dermatology staff was called to evaluate abnormal hair on a 22-month-old Hispanic girl whose parents were first cousins. Her medical history was significant for leptomeningitis with subsequent neurologic devastation, gastroesophageal reflux disease, and recurrent respiratory infections. Her hospital course was complicated by sepsis, liver dysfunction, pan-cytopenia, and disseminated intravascular coagulation. She had developed normally for the first year of life. At 13 months she became progressively lethargic and developed floppy muscle tone; a delay in mental and motor milestones was recognized. Results of a metabolic workup were negative. On examination she was noted to have generalized excessively fair skin when compared with her parents. She had silver-gray hair (Figure 1) and white eyebrows and body hair. Her maternal grandfather and granduncles had silver hair since childhood, but were without health problems. A maternal family member was said to have light skin. The presumed diagnosis before pathologic examination was Chediak-Higashi syndrome. Hematoxylin and eosin stain tests revealed prominent melanocytes in the basal layer of the epidermis. The melanocytes were large and distended with a large volume of melanin (Figure 2). The adjacent keratinocytes were completely devoid of melanin. Application of Masson-Fontana ammoniac silver stain highlighted prominent melanocytic melanin and a relative paucity of melanin in the adjacent keratinocytes (Figure 3). Microscopic examination of her hair revealed clumps of melanin of various sizes and shapes irregularly distributed throughout the hair shaft. Ultrastructural examination of the epidermis showed the melanocytes were distended by an accumulation of large stage IV mature melanosomes. Peripheral blood smear failed to show abnormal granules, even after repeated examination. Based on the clinical features and the pathologic findings, a diagnosis of Griscelli syndrome type 2 was made.
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PMID:Griscelli syndrome. 1748 61

Careful selection of bariatric patients is critical for successful outcomes. In 1991, the NIH first established patient selection guidelines; however, some surgeons operate on individuals outside of these criteria, i.e., extreme age groups. We developed appropriateness criteria for the spectrum of patient characteristics including age, BMI, and severity of eight obesity-related comorbidities. Candidate criteria were developed using combinations of patient characteristics including BMI: > or =40 kg/m(2), 35-39, 32-34, 30-31, <30; age: 12-18, 19-55, 56-64, 65+ years old; and comorbidities: prediabetes, diabetes, hypertension, dyslipidemia, sleep apnea, venous stasis disease, chronic joint pain, and gastroesophageal reflux (plus severity level). Criteria were formally validated on their appropriateness of whether the benefits of surgery clearly outweighed the risks, by an expert panel using the RAND/UCLA modified Delphi method. Nearly all comorbidity severity criteria for patients with BMI > or =40 kg/m(2) or BMI = 35-39 kg/m(2) in intermediate age groups were found to be appropriate for surgery. In contrast, patients in the extreme age categories were considered appropriate surgical candidates under fewer conditions, primarily the more severe comorbidities, such as diabetes and hypertension. For patients with a BMI of 32-34, only the most severe category of diabetes (Hgb A1c >9, on maximal medical therapy), is an appropriate criterion for those aged 19-64, whereas many mild to moderate severity comorbidity categories are "inappropriate." There is overwhelming agreement among the panelists that the current evidence does not support performing bariatric surgery in lower BMI individuals (BMI <32). This is the first development of appropriateness criteria for bariatric surgery that includes severity categories of comorbidities. Only for the most severe degrees of comorbidities were adolescent and elderly patients deemed appropriate for surgery. Patient selection for bariatric procedures should include consideration of both patient age and comorbidity severity.
Obesity (Silver Spring) 2009 Aug
PMID:Appropriateness criteria for bariatric surgery: beyond the NIH guidelines. 1934 19