[Combined method for the diagnosis of Hirschsprung's disease].
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Biomedical subjects
Publications and source records attributed to E Rosenmann.
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A case of the Hajdu-Cheney syndrome in a 15 year old boy is described. The manifestations were: 1. isiopathic progressive acro-osteotions; 2. mutiple cranio-skeletal abnormalities; and 3. polycystic kidneys complicated by a rapidly progressive glomerulonephritis.
Saline or BSA were instilled 2, 5, 8 or 11 times at daily intervals into the parotid glands of rats, via Stensen's duct. Hydropic change of the acinar cells developed after 8 and 11 instillations. Histologically, the lesion was characterized by enlargement of the acinar cells, the cytoplasm of which contained vacuoles displacing the nuclei. In view of the negative results of various histochemical reactions the vacuolization was interpreted as hydropic in nature.
Alpha-fetoprotein was detected in the serum and urine of 2- to 9-mth-old rats subjected to protracted CCl4 poisoning. During the first 3 mth of the experiment, urinary excretion of AFP was found in 30-40 per cent. of the animals, increasing to 70 per cent. thereafter. The liver lesions progressed from acute parenchymal necrosis to cirrhosis, but hepatocellular carcinomas did not develop. Uptake of tritiated thymidine by the hepatocytes increased significantly but was constant throughout the experimental period. The findings are compared to the observations made during 3mDAB-induced hepatocarcinogenesis in the rat.
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1. Fructose 1,6-bisphosphatase from the white muscle tissue of the carp, Cyprinus carpio L. was purified. 2. The mol. wt of the enzyme was 145,000. Its subunit mol. wt was ca. 35,000. 3. The enzyme exhibited neutral pH optimum, activation by monovalent cations, and temperature-dependent allosteric AMP inhibition. 4. Carp muscle fructose 1,6-bisphosphatase was 10- to 30-fold more sensitive to AMP inhibition than the carp liver enzyme. 5. The carp muscle enzyme was less sensitive to AMP inhibition than the muscle enzyme from a homeothermic mammal. These results are interpreted as an example of temperature-adaptation of an enzyme regulatory property.
Female rats were maintained on standard laboratory diet, Miller's diet or Miller's diet containing 3'MeDAB. Animals fed standard or Miller's diet did not excrete alpha-foetoprotein (AFP) in their urine. Early appearance of AFP was demonstrated by examining the urine of rats on the 3'MeDAB regimen. The incidence of positive urine samples was high between the 5th and 7th week of the experiment. It thereafter declined, but from the 3rd month it steadily rose and reached a maximum of 80% at about 10 months. Though urinary excretion of AFP was irregular in individual animals, several positive urine samples were obtained from all rats followed for more than a few months. The urine of 90% of hepatoma-bearing rats contained AFP at the time of killing. The incidence of elevated serum AFP levels as determined by immunodiffusion, increased with the duration of the experiment, but was still only 70 percent in rats fed 3'MeDAB for over 34 weeks. The severity of the hepatic alterations, as well as hepatocytic uptake of [3H]thymidine, increased with time. The serum of animals fed the standard diet was negative, whereas AFP was very infrequently detected in the serum of rats given Miller's hypoprotein diet. The results demonstrate that, in a population exposed to hepatocarcinogenic agent, the recurring detection of urinary excretion of AFP is a useful indicator of the high risk of developing hepatomas.
The right parotid duct of rats was instilled five to eight times with bovine serum albumin at daily intervals. Acute parotitis developed in non-sensitized animals, while chronic destructive sialoadenitis ensued in preimmunized rats. The morphological changes were reminiscent of those found in the salivary glands of patients with Sjögren's syndrome.
An unusual case of progressive systemic sclerosis with rapidly progressing renal failure after a successful delivery is described. The presenting syndrome was malignant hypertension. The blood pressure was refractory to the various therapeutic measures which were instituted, including hemodialysis. The patient, therefore, underwent bilateral nephrectomy, following which the blood pressure returned to normal, and she survived on hemodialysis for 17 months. To the best of our knowledge, the patient presented herein is the only reported case of this kind of fetal and maternal salvage.
An Arab patient with a typical nephrotic syndrome associated with the deposition of an amyloid-like material in the glomeruli is described. The deposits consisted of an electron-dense finely granular material which contained immunoglobulins and complement and fibrillar structures which resembled those of amyloid but did not stain with the typical amyloid stains.
A brief description is given of the main clinical, histological and epidemiological circumstances of retinal vein occlusion. In an assessment of the pathogeneis of the condition, primacy is given to the role of arteriolar flow insufficiency and stress is laid on the long-standing capillaropathy which precedes the heamorrhagic phase of the disease. Basic therapeutic considerations are mentioned but stress is laid on the diagnosis of the pre-occlusive stage and on measures of a preventive nature that may be taken. Retinal vein occlusion and haemorrhagic cerebral infarct are compared with regard to their pathogenesis and the advantages are discussed of a common ophthalmoscopic study of retinal vein occlusion with neurologists interested in cerebral vascular disease leading to an interchange of therapeutic experience in both conditions. Finally, there are detailed the studies in retinal vein occlusion being conducted by the Jerusalem Institute for the Prevention of Blindness. These are studies of incidence, natural history and of pathogenesis as observed in the experimental disease produced in rhesus monkey.
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Serum alpha-fetoprotein was detected by immunodiffusion in rats treated with anti-thymocytic serum. Augmented synthesis of AFP is related to compensatory hyperplasia of liver cells secondary to a state of hypoproteinemia in the proteinuric nephritic animals. The nephritis was produced by the anti-basement membrane antibodies present in the thymocytic antiserum.
Sixty-two cases of mesenteric panniculitis collected from the literature and six cases treated by the authors are reviewed. Symptomatology, pathology, treatment, and outcome of this disorder are discussed. It seems that mesenteric panniculitis is a well established clinical entity of unknown etiology, with a benign course and favourable outcome. Radical surgical treatment is not advised. The sole surgical intervention advocated would be deviation of the gastrointestinal tract in patients with symptoms of obstruction.
An unusual case of acute renal failure is described. The patient, who had no previous history or signs of renal impairment, underwent lumbar laminectomy under general anesthesia with trichloroethylene (Trilene) and nitrous oxide. On the fifth postoperative day i.v. administration of 12 million units of penicillin was started. Within 16 h the patient developed oliguria and a clinical picture of acute renal failure. The course of the disease was prolonged and necessitated four hemadialysis treatments. The patient recovered only about 50% of his renal function. A biopsy performed two months after the onset of the acute renal failure was interpreted as indicating tubulo-interstitial nephritis. The role of trichloroethylene in the etiology of acute renal failure is discussed.
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Liver antigens were detected in the urine of 4 of 42 patients with various liver diseases. The urine of 25 healthy subjects and patients with diseases not affecting the liver was devoid of antigens in detectable amounts. The presence of hepatic antigens in the urine did not correlate with severity of jaundice and SGOT levels but correlated with parenchymal necrosis and was associtated with a high mortaltiy.