Pulmonary vascular sling with tracheobronchial stenosis and hypoplasia of the right pulmonary artery.

We present a case of pulmonary sling associated with tracheobronchial stenosis, and with hypoplasia of the right lung and right pulmonary artery. Radiologic studies showed evidence of pulmonary sling and hypoplasia of the right lung; associated hypoplasia of the right pulmonary artery was also present, but not recognized initially. Narrowing of the distal trachea and right main bronchus was present, not due to compression by the anomalous left pulmonary artery, but to congenital stenosis of the trachea and right main bronchus associated with complete cartilaginous tracheobronchial rings.

Diffuse metanephric adenoma after in utero aspirin intoxication. A unique case of progressive renal failure.

Diffuse persistent glomerular immaturity and focal proximal tubular ectasia were seen in bilateral open renal biopsy specimens for an infant with fluid and salt depletion and slowly progressive renal failure. Subsequently, diffuse tubulopapillary renal adenoma subtotally replaced each kidney, thereby, necessitating renal transplantation. Origin of diffuse metanephric adenoma from persistent primitive epithelium of the proximal nephron is postulated and partly substantiated.

Visualization of alcohol-induced rhabdomyolysis: a correlative radiotracer, histochemical, and electron-microscopic study.

Technetium-99m diphosphonate was used to visualize the extent of alcohol-induced rhabdomyolysis and its resolution. Transient secondary hyperparathyroidism was documented. Histological and biochemical analyses of skeletal muscle obtained at biopsy 6 days postscan and 9 days after the onset of the illness did not show abnormal calcium content.

Antrochoanal polyps.

The antrochoanal polyp, a benign solitary polypoid lesion, usually arises in a maxillary sinus, opacifying and enlarging the sinus cavity without bone destruction. It passes through the ostium of the sinus into the choana, and from there into the posterior nasopharynx. The soft tissue mass does not erode or destroy contiguous soft tissue or bony structures.

Multifocal nephroblastic neoplasia.

Among 23 patients with multifocal metanephric neoplasia were 14 patients with 43 grossly visible subcapsular tumorlets, 0.3-3.5 cm in diameter, that were apparently derived from nodular renal blastema.

Rhabdomyosarcoma of lung arising in congenital cystic adenomatoid malformation.

An asymptomatic congenital cystic adenomatoid malformation of lung discovered at age 18 months, and unchanging for 14 months prior to its removal, was found to harbor a small embryonal rhabdomyosarcoma following excision. With lobectomy and chemotherapy, the tumor-free interval has now exceeded three years. The case represents a well-documented occurrence of malignant tumor arising within a congenital malformation, an association that is unlikely to be the result of chance.

Acute respiratory illness in children with acute lymphoblastic leukemia.

Ten of 70 children (14%) with acute lymphoblastic leukemia developed severe interstitial pneumonitis within three weeks after induction of central nervous system prophylactic therapy. The clinical picture was characterized by fever, cough, progressive dyspnea, and hypoxemia with complete resolution in one to three weeks, except in one patient who died during the acute illness from respiratory failure. P.

Phytosterols in aortic tissue in adults and infants.

Relationships between aortic tissue phytosterols and cholesterol in five adults, five infants, two neonates, and one 30 week abortus were studied. In the normal aortic tissue of the abortus and two neonates, tissue cholesterol levels were 0.67, 0.

Rhabdomyosarcoma in children. A clinicopathologic study of 35 patients.

Thirty-five patients with rhabdomyosarcoma were treated at the Children's Hospital Medical Center from 1953 to 1973. Factors important in diagnosis and prognosis included age, sex, site, stage of tumor, and specific pathologic type. Effects that surgery, radiation, and chemotherapy had on survivals were also analyzed.

Transiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome.

Since Reye's syndrome is associated with hyperammonemia, we measured the urea-cycle enzymes in hepatic tissue of 13 patients. Expressed as nanomoles of citrulline per milligram of hepatic protein per minute, mean activity of carbamyl phosphate synthetase (6.27 +/- 2.

The nephroblastomatosis complex and its relationship to Wilms' tumor: a clinicopathologic treatise.

1. At least one third of all children with Wilms' tumors have evidence of aberrant metanephric differentiation, hamartomas, adenomas and nodules of blastema or Wilms' tumorlets in the subcapsular renal cortex. Only the presence of nodular renal blastema was found to correlate with younger age, suggesting that the other tumorlike lesions may be derivatives.

The left ventricle in anomalous pulmonary venous return. Morphometric analysis of 36 fatal cases in infancy.

The mass of the left ventricle is normal in infants with total anomalous pulmonary venous return, but the ventricular cavity is compromised by leftward displacement of the interventricular septum due to the combined pressure-volume overload of the right side of the heart. Severe septal displacement is associated with distorted myocardial architecture in the region of the septal attachments of the left ventricular free wall. Abnormalities of septal motion are frequently detected in echocardiograms.

The hepatic lesion in Reye's syndrome.

The hepatic lesion in Reye's syndrome (acute encephalopathy with fatty degeneration of viscera) was studied by light microscopy of sequential biopsy specimens obtained in 49 children. The hepatic lesion is a morphologically characteristic, rapidly evolving, and reversible toxic hepatitis. In specimens obtained with 48 hr of onset of neurological deterioration, the severity of the diffuse microvesicular steatosis is best appreciated in frozen sections stained for lipid content.

The association of fixed and dynamic left ventricular outflow obstruction.

Twelve patients were investigated echocardiographically and angiographically and were shown to have severe fixed obstruction to the left ventricular outflow tract. Eight had valvular stenosis, and four had discrete subvalvular membranes. Two of the patients had additional dynamic obstruction of the left ventricular outflow tract.