Elevated plasma carnitine in the hepatic form of carnitine palmitoyltransferase-1 deficiency.

In a boy with a defect in fatty acid oxidation due to the hepatic form of carnitine palmitoyltransferase-1 deficiency, plasma carnitine concentrations were found to be twice normal. The elevation in plasma carnitine levels was accompanied by an unusually high renal threshold for free carnitine, suggesting a secondary increase in carnitine transport. Similar to other fatty acid oxidation disorders involving the carnitine cycle, urinary dicarboxylic acids were not abnormally elevated during illnesses.

Computed tomography, management, and follow-up in infantile hemangioendothelioma of the liver in infants and children.

Hepatic hemangioma (hemangioendothelioma) is a rare but most common benign vascular tumor of the pediatric age group. Prompt diagnosis and treatment prevent complications. Familiarity with various CT patterns should facilitate correct diagnosis of hemangioendothelioma of the liver in infants and children and avert angiography.

Gastrointestinal bleeding: an accessory spleen causing a false-positive Tc-99m-sulfur colloid study.

A Tc-99m-sulfur colloid abdominal scan was performed on a 12-year-old girl to localize the site of gastrointestinal bleeding. The study was normal. When bleeding recurred two weeks later, a repeat study revealed a focal abnormality in the upper abdomen.

Primary nonspecific ileal ulceration as a cause of massive rectal bleeding.

A case of primary ulceration of the ileum in the absence of a Meckel's diverticulum or ectopic gastric tissue is described. Although this condition is infrequently observed, it should be considered as a potential cause of massive rectal bleeding, iron deficiency anemia of unknown cause, perforation, or partial small bowel obstruction. The diagnosis is normally made at surgery.