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Biomedical subjects

R S Dubois

Publications and source records attributed to R S Dubois.

At least 19 recordsLinked to original sources

Infantile hypertrophic pyloric stenosis: a clinical review from a general hospital.

A review of 212 cases of infantile hypertrophic pyloric stenosis (IHPS) in a general hospital during an 8.5 year period documents clinical features and suggests refinement of treatment. Features such as sex, age at onset and presentation were similar to other studies. There was a preponderance of infants born in the summer. Premature infants represented 10% of the series, and the most premature (< 33 weeks gestation) presented later after birth (40 vs 25 days). Diagnosis was clinical in 65% of cases, but 4% presented before physical examination and diagnostic tests were positive. At the time of admission only 15% had significant abnormalities of serum electrolytes (chloride < 85 mmol/L). The periumbilical incision resulted in a hidden scar. Duodenal perforation (3.3%) and wound dehiscence (1.4%) are related to technical factors and can be avoided. The timing of commencement of postoperative feeds did not influence the rate of vomiting. The average length of stay was 3.5 days. These results are discussed with suggestions about how to minimize diagnostic tests and length of hospital stay.

Female↗

Treatment of Wilson's disease with triethylene tetramine hydrochloride (Trientine).

Penicillamine is the drug of choice for the treatment of Wilson's disease, whatever the stage of the illness. Toxic manifestations may preclude the use of this life-saving drug in some patients and discontinuation of penicillamine therapy usually leads to death. We report our experience with Trientine in seven patients, aged 13 to 33 years, with Wilson's disease who developed toxic manifestations with penicillamine that required discontinuation of therapy. These include two with nephrosis, one with neutropenia, two with thrombocytopenia, and one each with a SLE-like and a Henoch-Schonlein-like syndrome. The patients were treated for periods from 6 weeks to 16 years with a dose of 0.5 to 2 g/day. Trientine proved to be an effective alternative copper chelating agent in the treatment of Wilson's disease in patients with penicillamine-induced neutropenia, thrombocytopenia, SLE, and nephrosis. No serious untoward side effects were noted.

Administration, Oral↗

Hepatotoxicity due to pemoline (Cylert): a report of two cases.

Pemoline (Cylert) is an agent used to treat attention deficit disorders and other behavioral syndromes. There have, however, been three published reports of mild hepatic dysfunction in five patients coincident with pemoline therapy. We report two further cases of probable pemoline hepatotoxicity. One case involved a mild aminotransferase elevation in a 6-year-old who was on pemoline for 5 months. The second case, in an 11-year-old, developed hepatic failure with marked prolongation in prothrombin time nonresponsive to parenteral vitamin K, deep jaundice, and submassive hepatic necrosis. This patient had a long history of pemoline usage. Pharmacokinetics are briefly discussed. A spectrum of hepatic disease due to pemoline is considered and the importance of obtaining aminotransferase values before, during initiation, and throughout treatment is stressed.

Attention Deficit Disorder with Hyperactivity↗

Role of 99mTc-disofenin in duodenal and gastric aspirates in the evaluation of persistent neonatal cholestasis.

A prospective study was undertaken on 49 infants with prolonged neonatal cholestasis to determine whether the specificity, sensitivity, and accuracy of 99mTc-disofenin scintigraphy can be improved by performing simultaneous radioactivity measurements on duodenal and gastric aspirates along with routine imaging. Specificity of routine scintigraphy was improved by this modification from 45.7 to 76.4%. Accuracy of the test was also superior in improving from 55% with routine scintigraphy to 78.4% by counting additional radioactivity on both duodenal and gastric aspirates. We suggest that this modified technique be applied in the evaluation of infants with conjugated hyperbilirubinemia so that invasive procedures such as open liver biopsy and operative cholangiogram may be avoided.

Duodenum↗

Comparison of radionuclear scintigraphy and liver biopsy in the evaluation of neonatal cholestasis.

Twenty-eight patients with neonatal cholestasis were given appropriate tests to arrive at a definitive diagnosis. Comparison of the results showing total obstruction on scintigraphy but not on liver biopsy is presented. It is suggested that liver biopsy be performed to confirm the diagnosis prior to undertaking exploratory laparotomy when radionuclear scan suggests extrahepatic biliary atresia.

Bile Ducts↗

Pancreas divisum: a cause of pancreatitis in childhood.

The anatomic congenital anomaly pancreas divisum has recently been implicated as a cause of acute pancreatitis. The associated stenosis of the minor papilla has been postulated to cause pancreatitis by producing a relative obstruction to the high volumes of dorsal pancreatic secretion. We present a 10-year-old boy with acute pancreatitis associated with pancreas divisum to illustrate this as a cause of pancreatitis in childhood.

Acute Disease↗

Use of Golytely in children and adolescents.

Proper cleansing of the bowel is essential to ensure an adequate colonoscopic examination. The experience of 24 children and adolescents with Golytely, a new colonic lavage solution, is presented. Oral Golytely was found to be less inconvenient for patients, safe, and more cost-effective because it decreased hospitalization time.

Adolescent↗

Peptic ulcer disease in children and adolescents. A ten-year experience.

We report a retrospective review of peptic ulcer disease (PUD) in children seen at Children's Hospital of Michigan, over 10 years (1971-1980). Of the 61 included, 31 had primary ulcers and 30 had stress ulcers. The diagnosis was made by either barium meal or endoscopy, at surgery, or at autopsy. Stress ulcers were commoner in children less than 4 years of age and were precipitated by various conditions leading to shock. The majority of these patients required surgery for perforation or bleeding. Primary ulcers were more common in children older than 4 years. The most common presenting symptoms in this group were abdominal pain and gastrointestinal bleeding. The majority of patients with primary PUD received medical therapy in the form of antacids and/or cimetidine. In one-third of these patients, surgery was necessary for complications such as intractable pain, perforation, or massive recurrent hemorrhage.

Adolescent↗

Chronic active hepatitis and pregnancy: a report of two cases in adolescence.

The course and outcome of pregnancy in two adolescents with chronic active hepatitis is described. Pregnancy was uneventful in both patients apart from mild biochemical deterioration in one patient. Both infants are entirely normal. One mother died 4 1/2 months after delivery from primary pulmonary hypertension, an extrahepatic manifestation of her chronic active hepatitis. The second patient is asymptomatic but has evidence of ongoing liver disease 26 months postdelivery.

Adolescent↗

Lactobezoar in prematurity. A case with prolonged resolution.

Lactobezoar is a foreign body produced in the gastrointestinal tract by the presence and accumulation of undigested milk curds. It is seen most commonly in low-birth-weight, premature infants fed with 24-calorie formulas. Conventional therapy includes withholding feedings, repeated gastric lavage, and maintenance of nutrition and hydration by the parenteral route. Most cases respond to this regimen within 48 to 72 hours. We present a case of gastric lactobezoar in a premature, small for gestational age infant who was fed a 24-calorie formula but failed to respond to conventional therapy for more than a week. Historical background and pathophysiology of lactobezoar are discussed.

Bezoars↗

Sonography in the diagnosis and management of hydrops of the gallbladder in children with mucocutaneous lymph node syndrome.

Acute hydrops of the gallbladder is described in four patients who had mucocutaneous lymph node syndrome. Diagnosis was readily made with gray-scale ultrasound, and the patients were followed with this technique. The gallbladder returned to normal size in all of the patients within 15 days, and none of the patients needed surgery. The role of surgery in acute hydrops of the gallbladder is for the complications of the hydrops and not for distention alone.

Child↗

The pediatric corner: the varied manifestations of Crohn's disease in children and adolescents.

Thirty children less than 15 years of age with Crohn's disease are reported. The mean age of onset was nine years and five months. Delay in diagnosis may stem from the varying modes of presentation. The mean delay in diagnosis was two years. Chronic diarrhea and/or recurrent abdominal pain associated with extraintestinal manifestations, particularly abnormalities of growth and development suggests the possibility of Crohn's disease in the pediatric age group. Involvement of the terminal ileum occurred in 29 of the 30 patients. In addition, colonic disease occurred in 13 and jejunal involvement in one. Four patients presented with an acute onset of their disease. A favorable response to either medical or surgical therapy occurred in all four. Twenty-six patients had an insidious onset and tended to have a chronic course despite either medical and/or surgical therapy. Thirteen patients had an intestinal resection, with recurrence occurring in three from 1-4 years postoperatively. To date there has been one death.

Adolescent↗