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Biomedical subjects

C Aschenbrener

Publications and source records attributed to C Aschenbrener.

10 recordsLinked to original sources

Childhood brain tumor: presentation at younger age is associated with a family tumor history.

In a registry-based sample of 361 children with a brain tumor, those whose grandparents and great-grandparents had a history of any kind of tumor were younger at the time of presentation than were those who lacked this family history (p = 0.1). In post hoc analyses, the age difference was most apparent among children with cerebral tumors, and when family history was limited to brain tumors and to great-grandparents. These findings are in keeping with the hypothesis that a familial tumor diathesis contributes to an early age at onset of a brain tumor in some children.

Age Factors

Inherited metabolic myopathy with storage of glycoproteins and glycosaminoglycans.

Two related patients (mother and daughter, ages 28 and 5 years) showed mild to moderate weakness and atrophy of facial and shoulder muscles with congenital onset and minimal progression. Serum creatine kinase was elevated in the child. Muscle biopsy showed normal light-microscopic and histochemical findings, but scattered sarcoplasmic vacuoles with storage of granular material were evident on electron microscopy. Storage of granular material was also identified in fibroblasts which were weakly PAS-positive, stained metachromatically with toluidine blue and orthochromatically with alcian blue. Muscle glycogen values were low-normal. Repeated biochemical studies of cultured fibroblasts identified excessive storage of glycosaminoglycans and glycoproteins. The uptake of 3H-glucosamine in cultured fibroblasts was 1.7-3.4 times greater in the patients than in control individuals, while the rate of turnover of the radioisotope was normal. These findings suggest that the genetic defect in this inherited metabolic myopathy is related to excessive synthesis of glycosaminoglycans and glycoproteins.

Adult

Primary rhabdomyosarcoma of the tentorium with peculiar angiographic findings.

Primary intracranial rhabdomyosarcoma is extremely rare, only nine cases have been reported in the world literature. To differentiate it from medullomyoblastoma, rhabdomyosarcoma should be devoid of neuroblastic elements. A case of rhabdomyosarcoma of the tentorial region in a 17-year-old boy is reported. Carotid angiograms demonstrated a peculiar neovascularity. Multiple cytologic preparations of CSF repeatedly contained rhabdomyosarcoma cells and CSF dissemination in the spinal subarachnoid space was documented at autopsy. The literature is reviewed.

Adolescent

Ganglioneuroblastoma in adults.

Ganglioneuroblastoma is a rare neoplasm of adults. Some information about the natural history and response to therapy can be obtained from two cases seen recently at our hospital and 17 cases in the literature. These tumors occur in adults of any age and are usually located in the retroperitoneum, mediastinum, or neck. Evidence of the tumor may be present years before diagnosis. Elevations of urinary catecholamines were documented in three cases and the levels correlated with progression or resection of the neoplasm. The tumor spreads either by local invasion or distant metastasis. Attempts at radiotherapy and chemotherapy in the treatment of unresectable or disseminated tumor has not resulted in objective improvement. This contrasts with the widely reported objective successes and cures of ganglioneuroblastomas occurring in childhood. Surgical excision of localized disease was the only curative therapeutic modality in the cases we reviewed.

Adult

Renin as a risk factor for atherogenesis. Effects of hypercholesterolemia and two-kidney--one-clip hypertension in the rabbit.

Four groups of New Zealand rabbits were used to study the effect of plasma renin activity (PRA) on atherogenesis. Control groups were fed normal rabbit chow (Group I) or chow supplemented with 0.25% cholesterol and 0.75% corn oil (Group II). The two-kidney--one-clip (2K-1C) hypertensive model was produced in 2 additional groups; Group III (normal diet) and Group IV (atherogenic diet). The latter 2 groups were subgrouped according to PRA levels. Each group was examined over a 7-month period. Group II became hyperlipidemic and developed extensive lipoidal vascular lesions. Mean arterial pressure remained normal throughout the experimental period; PRA fell below normal. Group III and Group IV rabbits developed sustained hypertension irrespective of circulating PRA. The atheromas of Group III were predominantly microscopic and fibromuscular; the extent of aortic and coronary artery involvement was independent of renin response. The most extensive and complicated atheromas were seen in the 2K-1C rabbits consuming the atherogenic diet (Group IV). The lesions were mostly lipoidal, although some were fibromuscular. These results demonstrated that cardiovascular lesions and atherogenesis were exacerbated in the 2K-1C rabbits on a high cholesterol diet; however, PRA was excluded as the cause.

Adrenal Glands

Histopathology of neurosensory deafness in sarcoidosis.

Sarcoidosis is an idiopathic systemic granulomatous disease which occasionally causes fluctuating auditory and vestibular dysfunction. The temporal bones from a 32-year-old man deaf for 5 years from CNS sarcoidosis were examined histologically and compared with other nervous system tissues. It was found that the acoustic, vestibular, and facial nerves were involved in a striking perivascular lymphocytic infiltration resulting in myelin and axonal degeneration. The cochlear and labyrinthine neuroepithelium and stria vascularis had degenerated. It is hypothesized that neurosensory deafness and vestibular dysfunction in sarcoidosis starts as a reversible neuropathy. In some patients, an ischemia secondary to the vasculitis results in irreversible damage to the inner ear neuroepithelium.

Adult