Citations

What disease states are occasionally associ ated with hypogarathyraidisna? Is there any explanation for the association of moniliasis or steatorrhea in the syndrome re ported ? Is there a familial incidence of the syndrome of hypoparathyroidism, Addison's disease and moniliasis?

Medical and surgical emergencies occasionally present as ruptured abdominal aortic aneurysms (RAAA). To assess benefits of laparotomies and adverse effects of unnecessary operations, the authors reviewed their experience. Thirteen patients, 9 women, 4 men (mean age: 72 years, range: 41-85) underwent emergency laparotomy between 1988 and 1996 for presumed RAAA and were found to have other surgical or medical emergencies. All the patients presented with hypotension, 12 had abdominal or back pain, four had pulsatile abdominal mass. Rupture was not excluded by computed tomography scan in three or by ultrasonography in two patients. Laparotomy disclosed intact abdominal aorta in all, but seven patients had abdominal aortic aneurysm. Of five surgical emergencies, laparotomy was indicated in four: three for ruptured visceral artery aneurysms, one for perforated duodenal ulcer. The fifth patient required thoracotomy for ruptured thoracic aneurysm. Four of eight medical emergencies were myocardial infarctions. One iatro genic complication required reoperation for bleeding. Mean hospital stay was 18 days; mean hospital charges were $40,771. Seven (54%) early deaths occurred; none were caused directly by the operation. Laparotomy was indicated in one third of deceptive emergencies that present as RAAA. Although mortality, morbidity, and costs were high, iatrogenic surgical complica tions were rare and deaths were not caused by unnecessary operations.

To test the hypothesis that the frequency of biotinidase deficiency is greater in children with unexplained developmental delay or neurologic abnormalities than in the general population, we studied children seen at a large outpatient clinic over a four-year period who had one or more of these neurologic abnormalities and for whom no specific cause for their abnormalities could be found. The group totaled 274 children (163 boys; 111 girls) whose ages ranged from 2 weeks to 17 years. Characteristics were IQ/DQ, 30 to 70 in the 115 for whom scores were available; 41% had seizures; 15% had sensorineural hearing loss; 54% showed gross motor delay or ataxia; and 27% had decreased muscle tone. One patient with a classical clinical picture of biotinidase deficiency was diagnosed during the study period and was not included in the study. None of the patients with nonclassic findings had a deficiency of biotinidase activity. Our results suggest that biotinidase deficiency does not account for a large proportion of children with unexplained neurologic abnormalities or developmental delay. This does not negate the importance of biotinidase testing in children with clinical patterns specifically suggestive of the deficiency.

Hypersensitivity pneumonitis is infrequently reported in children. This article describes a 15-year-old girl who presented with insidious onset of dyspnea and weight loss. A hamster and doves were housed in her bedroom. Chest radiographs showed bilateral nodular infiltrates. Pulmonary function tests showed a restrictive pattern with O 2 desaturation at rest. The diagnosis was substantiated by the presence of precipitating antibody to dove serum and droppings and by clinical improvement, along with marked improvement in pulmonary function test, after the doves were removed from her environment. The importance of making an early diagnosis to prevent debilitating fibrotic lung disease is emphasized. A detailed environmental history is essential.