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Biomedical subjects

J Stern

Publications and source records attributed to J Stern.

At least 217 records · Page 12Linked to original sources

Sacral agenesis: an analysis of 11 cases and review of the literature.

Sacral agenesis is an uncommon condition associated with familial diabetes mellitus and complicated pregnancy. The condition is characterized by gluteal skin dimpling, loss of gluteal fold, myelomeningocele, sacral lipoma, as well as numerous orthopedic, anorectal and other widespread anomalies. The motor nerve deficit, for example poor anal sphincter tone, is more pronounced than the sensory nerve deficit, for example perianal anesthesia. Diagnosis is made by anteroposterior and lateral spinal x-rays. All of our patients had bladder or external sphincter dysfunction, while 91 per cent had vesicoureteral reflux. Early diagnosis and conservative management with intermittent catheterization and uropharmacological agents are now advocated.

Abnormalities, Multiple↗

Neurofibromatosis and optic glioma: clinical and morphological correlations.

Although an association between optic glioma and neurofibromatosis is well recognized, there has been no previous analysis of cases relating the locus of tumor to the presence or absence of von Recklinghausen's disease. This paper presents the results of such an analysis as well as a description of the comparative histology of optic gliomas in patients with and without neurofibromatosis. We conclude that optic gliomas in patients with neurofibromatosis present preferentially as multicentric lesions or as lesions affecting the optic nerve alone without invasion of the chiasm, that these tumors frequently differ morphologically, and that patients with neurofibromatosis and optic glioma may have a clinical course different from that of patients with optic glioma unassociated with neurofibromatosis. Previously published reports are reviewed in light of these observations.

Adolescent↗

Management of extracranial carotid stenosis and intracranial aneurysms.

Twenty patients with extracranial carotid stenosis and intracranial aneurysms are reviewed. Fifteen of these patients had transient ischemic attacks (TIA's) and incidental aneurysms. The other five presented with symptoms referrable to an aneurysm, and angiography revealed significant carotid stenosis. None of the patients who presented with TIA's and underwent endarterectomy suffered subarachnoid hemorrhage. However, those patients who presented with symptoms referrable to an aneurysm and underwent endarterectomy seem to be at greater risk for subarachnoid hemorrhage.

Adult↗

Coarctation of the aorta: additional signs.

Rib notching and the figure-three configuration and the most often cited signs of coarctation of the thoracic aorta in the adult. In our experience, these so-called classic signs are absent in many patients. Abnormalities of the ascending aorta, aortic arch, and superior mediastinum are also major findings and indeed may be the only findings in coarctation. This has not been sufficiently emphasized in standard radiology texts. In order to elucidate these points, we reviewed the chest radiographs of 13 patients with adult-type coarctation of the aorta presenting to the Jewish General Hospital over a 10-year period. In addition, the chest radiographic findings of five patients with pseudocoarctation were compared to those of true coarctation.

Adult↗

Non-ketotic hyperglycinaemia in a family with an unusual phenotype.

A 10-year-old girl, one of three affected sisters, with non-ketotic hyperglycinaemia is described. In contrast to other reported cases, the course of the disorder was comparatively mild in this family. The only clinical signs were mental retardation and abnormalities in the EEG; blood glycine levels were 2-3 times normal. In the propositus, the formation of 14CO2 from glycine-1-14C and of FH414CH2OH from glycine-2-14C were impaired, shown by the decreased 14CO2 content of expired air and diminished labelling of carbon 3 of serine. However, the biochemical defect was no less than that seen in patients with much more severe clinical effects.

Amino Acid Metabolism, Inborn Errors↗

Long acting contraceptive implants. An analysis of menstrual bleeding patterns.

The menstrual patterns of women using subdermal implants of levonorgestrel and norgestrienone included in a double-blind clinical trial was evaluated by the method developed by the International Committee on Contraception Research. Women using Copper T200 randomly selected in the same clinics were used as controls. Both implant regimens were associated with a high frequency of reduced bleeding and norgestrel patients had also a high proportion of increased and irregular bleeding. Termination of use was associated to increased bleeding but not so much to reduced bleeding.

Adolescent↗

Acute neonatal and benign citrullinaemia in one sibship.

Citrullinaemia was diagnosed in an infant who died at age 8 days. The clinical picture was of the disease in its acute neonatal form. A sib has a blood citrulline of 100 times normal and about 10% of normal argininosuccinic acid synthetase activity in cultured fibroblasts. Clinically he is normal with an IQ of 94 on the Wechsler Intelligence Scale for Children.

Amino Acid Metabolism, Inborn Errors↗

Adrenal steroidogenesis in methylandrostenediol-induced hypertension.

Adrenal vein catheterizations were done in rats made hypertensive by administration of methylandrostenediol (MAD; 17alpha-methyl-5-androstene-3beta,-17beta-diol), and in control rats at intervals during treatment. All MAD-treated rats were hypertensive by 7 weeks. Secretion of corticosterone was consistently decreased at all times in MAD-treated rats. 18-Hydroxy-11-deoxycorticosterone secretion and 11-deoxycorticosterone (DOC) secretion decreased and increased, respectively, compared to controls at 2, 4, and 6 weeks. Aldosterone secretion was decreased at 2 and 4 weeks. This study shows an in vivo block of adrenal 11- and 18-hydroxylation. Transient DOC accumulation by treatment with MAD produced hypertension, though DOC oversecretion and other changes in steroidogenesis were waning by the time hypertension developed.

18-Hydroxydesoxycorticosterone↗

[On the stress state of bone cement fixing a hip joint prosthesis (author's transl)].

Using a simplified model, tests were made to investigate the shear stress distribution at a hip joint prosthesis. A marked increase of stress at the tip of the prosthesis was found through photoelasticity. This peak stress resulted in a safety factor of 3 against exceeding the shear strength of bone cement. Since the calculation of the stress is based on unfavourable assumptions, the actual factor of safety should show a higher value still.

Bone Cements↗

Sodium exchange between blood, brain, and CSF in normal and hydrocephalic cats.

The exchange of sodium between blood, brain, and cerebrospinal fluid was studied in normal and kaolin-induced hydrocephalic cats. The ventricles were perfused to measure fluid formation and Na+ exchange rates. 22Na was added to the perfusion fluid or injected intravenously as a tracer for Na+ movement. Na+ and 22Na were also measured in cortical gray and white matter. Na+ relative specific activities were calculated for brain, effluent fluid, and serum. With 22Na in the perfusion fluid, Na+ exchange was not different from nascent Na+ influx for both normal and hydrocephalic cats. Na+ relative specific activities of cortical gray and white matter were 10 times greater in hydrocephalic than in normal cats. This difference in Na+ relative specific activity for brain may be due to a higher diffusion constant or to a lower brain capillary permeability. When 22Na was given intravenously, the Na+ diffusional exchange for normal cats was less than that measured when 22Na+ was in the perfusion fluid. In hydrocephalic cats, the Na+ diffusional exchange was effectively zero. Na+ relative specific activities of cortical gray and white matter were the same for normal and hydrocephalic cats. These findings suggest that the impaired Na+ diffusional exchange may be due to pathological changes in the choroid plexus.

Animals↗

Identifying patients at risk for thromboembolism. Use of 125I-labeled fibrinogen in patients with acute myocardial infarction.

Fibrinogen labeled with iodine 125 was used to detect deep vein thrombosis (DVT) in 35 patients during their course and convalescence from acute myocardial infarction. Clinical status was assessed and scored with the use of a modified coronary prognostic index. According to the prognostic scores, patients were allocated to one of two groups. Of 27 patients in good clinical condition, DVT developed in only one patient, whereas thromboembolic complications occurred in seven of eight patients who were severely ill--a highly significant difference. Prophylactic anticoagulation is advisable in patients at risk.

Acute Disease↗