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Biomedical subjects

B B Dahms

Publications and source records attributed to B B Dahms.

50 records · Page 3Linked to original sources
PubMed1982

Renal lipidosis associated with arteriohepatic dysplasia (Alagille's syndrome).

We report a 20-year-old patient with arteriohepatic dysplasia (Alagille's syndrome) who developed progressive renal failure associated with extensive renal lipidosis. A renal biopsy showed diffuse thickening of glomerular basement membrane mimicking idiopathic membranous glomerulonephritis on light microscopy. Electron microscopy, however, demonstrated numerous intramembranous and mesangial lipid deposits, quite similar to those described in familial lecithin cholesterol acyltransferase deficiency. We believe the renal lipidosis developed secondary to hyperlipidemia associated with longstanding intrahepatic cholestasis. This case illustrates that extensive lipid deposition in the glomerular basement membrane may occur in patients with arteriohepatic dysplasia, and it may lead to progressive renal failure.

Adolescent↗
PubMed1981

Abdominal lipoblastomatosis: radiographic, echographic, and computed tomographic findings.

Two cases of lipoblastomatosis in infants are described. Radiography, ultrasonography, and computerized tomography showed well-defined soft-tissue densities embedded within large masses of adipose tissue. This appearance, which may be specific for lipoblastomatosis, correlated precisely with the pathologic appearance of lobules of immature adipose tissue surrounding masses of myxoid tissue.

Abdominal Neoplasms↗
PubMed1980

Pulmonary arterial lipid deposit in newborn infants receiving intravenous lipid infusion.

Three newborn infants are described with a distinctive pulmonary arterial lipid lesion, characterized by a wide foamy layer of intima partially occluding the lumen of small muscular pulmonary arteries. Lipid stains indicated lipid infiltration of the walls of these arteries, involving not only the intimal layer, but media and adventitia as well. All three infants had received intravenous lipid infusions. All three infants also had histologic evidence of pulmonary hypertension, one on the basis of chronic lung disease (BPD) and two on the basis of congenital heart disease. A retrospective autopsy study of long-term neonatal ICU dwellers receiving such infusions revealed two additional patients with lipid deposits in pulmonary arterial walls. It is speculated that the pulmonary arterial lipid deposits were derived from the lipid infusions and were dependent on antecedent or concurrent vascular damage from pulmonary hypertension.

Chronic Disease↗
PubMed1980

Nesidioblastosis and other islet cell abnormalities in hyperinsulinemic hypoglycemia of childhood.

The histologic findings are described in 16 subtotal pancreatectomies performed in patients with hyperinsulinemic hypoglycemia over a 17 year period. All patients had nesidioblastosis, defined as the presence of small packets of two to 25 islet cells scattered throughout acinar tissue and separate from islets of Langerhans. The proliferating islet cells were a mixture of beta, alpha, and delta cells. Two histologic subgroups were apparent: Group I patients had diffuse hyperplasia of the islets of Langerhans as well as nesidioblastosis, and group II patients had more subtle nesidioblastosis alone. The patients in groups I and II were remarkably age segregated. All but one of the group I patients were eight months old or younger. Group II patients ranged in age from three to 15 years. The incidence of nesidioblastosis in the patients undergoing pancreatectomy was higher than the incidence in a group of age matched autopsy controls.

Adolescent↗
PubMed1979

Hepatoma in familial cholestatic cirrhosis of childhood: its occurrence in twin brothers.

Obstructive jaundice, pruritus, and malabsorption developed in twin brothers in infancy. Early liver biopsy specimens showed intracellular and canalicular cholestasis with normal bile ducts. By the age of 3 years, both had cirrhosis and portal hypertension. Each died during the teen years from hepatocellular carcinoma. These brothers represent the tenth reported family with familial cholestatic cirrhosis, and they are the first patients with this syndrome in whom hepatoma developed.

Adolescent↗
PubMed1976

The occurrence in a neonate of a pancreatic adenoma with nesidioblastosis in the tumor.

A newborn with persistent hypoglycemia and fixed insulin secretion unresponsive to medical management underwent 80% pancreatectomy. A 1-cm. nonencapsulated islet-cell adenoma near the tail of the pancreas was identified histologically. Islet-cell granules stained with indirect aldehyde fuchsin. Throughout the tumor, areas of nesidioblastosis were identified. The persistence of this embryologic process suggests a basic mechanism for the adenoma formation.

Adenoma, Islet Cell↗
PubMedDate not supplied

Delayed contrast enhancement magnetic resonance imaging in congenital aortic stenosis.

We report a case of a 12-year-old boy with severe congenital aortic stenosis in whom magnetic resonance imaging (MRI) with delayed contrast enhancement demonstrated extensive subendocardial hyperenhancement within the left ventricle. The hyperenhancement was confirmed to be subendocardial infarct and fibrosis by histopathology. This case supports the utility of MRI with delayed contrast enhancement in evaluating myocardial viability in patients with congenital heart disease.

Aortic Valve Stenosis↗