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Biomedical subjects

J Bernstein

Publications and source records attributed to J Bernstein.

At least 271 records · Page 15Linked to original sources
PubMed1979

Segmental "hypoplasia" of the kidney (Ask-Upmark).

Severe segmental renal atrophy with loss of parenchymal elements in small kidneys is commonly known as segmental hypoplasia. The scars are seen as cortical depressions overlying shrunken medullary pyramids and their dilated calyces, and are characterized histologically by colloid-filled tubular microcysts and a paucity or absence of glomeruli. This lesion has been identified in 17 patients, 11 female and 6 male, between 6 and 23 years of age. Eleven patients had hypertension, which developed in six while they were under observation. Thirteen had histories of urinary tract infection, and 16 had evidence of vesicoureteric reflux. Seven patients had impaired renal function (GFR less than 40 ml/minute/1.73 m2). Abnormal metanephric differentiation (dysplasia) in two specimens, one in association with posterior urethral valves, suggested an occasional intrauterine origin of the abnormality. Twelve patients had radiographic evidence of decreasing renal size over two to five years of observation, even after surgical correction of reflux, in four of them unaccompanied by infection. We conclude that segmental "hypoplasia" is an acquired lesion, although it sometimes has intrauterine origins, and that it is commonly associated with vesicoureteric reflux, even in the absence of demonstrable infection.

Adolescent↗
PubMed1979

Polypoid and papillary epithelial hyperplasia: a potential cause of ductal obstruction in adult polycystic disease.

In experimental models of cystic renal disease, functional studies define conditions that suggest increased resistance to outflow from dilated or cystic nephrons. Morphologically, models exhibit foci of cellular hyperplasia and micropolyp formation along outer medullary collecting tubules. Temporally, cellular proliferation precedes cyst formation. These findings in models have led to a hypothesis that polypoid hyperplasia participates in cyst formation in susceptible kidneys by increasing resistance to the outflow of tubular urine. The present study was undertaken to establish the presence, extent, and distribution of cellular hyperplasia in human adult polycystic kidney disease. Kidneys from four unrelated individuals were studied by light and by transmission and scanning electron microscopy. Foci of hocation of hyperplasia along the nephron were similar to those seen in the models. These findings delineate a heretofore unappreciated morphologic similarity between the models and human disease and add further support to the hypothesis that partial rubular obstruction participates in the pathogenesis of renal cystic disease, whether it be heritable or acquired, in animals and in man.

Animals↗
PubMed1979

Primary Crohn's disease of the appendix: report of 14 cases and review of the literature.

Fourteen patients with primary Crohn's disease of the appendix have been seen in a 12 year period. These patients represent 12.8% of the total number undergoing surgical resection because of Crohn's disease. Twenty-three cases of appendiceal Crohn's disease have been previously reported in the literature. A correct preoperative diagnosis is rarely made; the usual diagnosis is that of acute appendicitis or appendiceal abscess. The removed appendix in twelve of our 14 cases had marked thickening of the wall with transmural fibrosis and often with granulomatous inflammation. The enlarged appendix had an external appearance similar to that of ileal Crohn's disease, and we consider a correct surgical diagnosis might be possible with better awareness of its existence. The diagnosis might be suspected earlier when the clinical course of apparent appendicitis is protracted or atypical. Contrary to the previous estimation of high recurrence rate, this series and the cumulative evidence in the literature show a relatively low rate at 14%. The feared fistula formation following the removal of the appendix has not been seen in either our series or the literature. These patients, however, merit long-term follow-up.

Adolescent↗
PubMed1979

Cell-mediated immune response to bacterial products in human tonsils and peripheral blood lymphocytes.

Lymphoproliferative responses of tonsillar tissue lymphocytes and peripheral blood lymphocytes to phytohemagglutinin and specific bacterial product antigens were studied in children undergoing tonsillectomy and adenoidectomy. Tonsillar tissue lymphocytes responded to optimal concentrations of phytohemagglutinin. Varidase, and streptolysin-O in a manner similar to peripheral blood lymphocytes. Higher base-line mitogenic activity in tonsillar lymphocytes was frequently associated with the presence of Staphylococcus aureus in the tonsils. Tonsillar tissue lymphocytes from 23% of the subjects with the highest base-line mitogenic activity manifested a decreased response to in vitro stimulation with mitogens or antigens. In subjects with such preactivated tonsillar lymphocytes, the proliferative responsiveness of blood lymphocytes to mitogen and antigens was markedly increased after tonsillectomy and adenoidectomy. These observations suggest the existence of in vitro correlates of cellular immunity to bacterial products in the mucosal surfaces. In addition, it is proposed that tonsils may possess immunosuppressive activity for peripheral blood lymphocytes, which may be related to local tonsillar infections.

Adenoidectomy↗
PubMed1979

Localized cystic disease of the kidney: angiographic-pathologic correlation.

Each of the three patients with a type of unilateral, localized cystic disease of the kidney that did not fit into a specific category of existing classifications had numerous cysts involving a part of one kidney. Selective angiography showed stretching of arteries around cysts that remained radiolucent in the nephrographic phase, and microscopic examination demonstrated that septa among the cysts contained compressed renal elements. The condition appeared to be neither progressive nor familial. All three had hypertension. Two patients who had nephrectomy for the cystic lesion became normotensive postnephrectomy. Despite some morphologic resemblances, this condition is believed to be different from adult type polycystic kidney disease in its clinical and genetic aspects.

Angiography↗
PubMed1979

Identification of dense deposit disease: a report for the International Study of Kidney Diseases in Children.

The fluorescent dye thioflavin T stains intensely the deposits in dense deposit disease and can be used for identification purposes. Comparison of thioflavin T staining with electron microscopy was carried out in 25 patients and showed excellent congruity. Thioflavin T is much easier to use, but is less specific than electron microscopy. Cases of light chain disease and of some gammopathies can be a source of confusion. In nearly all other instances, the pattern of staining helps to differentiate dense deposits from other types of deposits and from amyloid.

Basement Membrane↗
PubMed1978

Sarcoidosis of the spinal cord as the presenting manifestation of the disease.

Involvement of the spinal cord by sarcoid is quite rare, with only 11 previous histologically proved cases. One finds an almost uniform increase in the spinal fluid protein level. Even with the presence of other stigmas of sarcoidosis, the diagnosis of spinal cord sarcoidosis is difficult, and biopsy usually must be done to rule out a malignant tumor.

Adult↗
PubMed1977

Conjugated hyperbilirubinemia in infancy associated with parenteral alimentation.

Liver biopsy was performed to exclude anatomic obstruction of the biliary tract in five prematurely born infants who had developed conjugated hyperbilirubinemia during intravenous alimentation with a protein hydrolysate. Each was being treated after having undergone a segmental intestinal resection for necrotizing enterocolitis. Bacterial and viral infections, metabolic disorders, and isoimmune hemolytic disease were excluded as possible causes of jaundice. Light microscopic and ultrastructural analysis disclosed cholestasis and hepatocellular injury without significant inflammatory reaction. Jaundice abated following permanent discontinuation of parenteral alimentation. The jaundice and cholestasis are interpreted to be hepatotoxic effects because of (1) their temporal relationship to the treatment and (2) the presence of hepatocellular damage.

Biopsy, Needle↗
PubMed1977

Upper cervical myelopathy in achondroplasia.

Two achondroplastic infants had small foramina magna with lethal compression of the corresponding upper cervical spinal cords. The damage was histologically comparable to the hyperextension type of spinal cord injury. An anterior displacement of the foramen magnum was demonstrated in one of the two cases. The displacement is considered worthy of future verification as it may be an additional factor in susceptibility to hyperextension injury to the spinal cord. The second patient survived 6 months, demonstrating that not all upper cervical spinal cord injuries are immediately fatal. Patients who have sublethal lesions are potentially salvageable by surgery. It is also speculated that there might be more cases of nonlethal upper cervical spinal-cord damage in achondroplasia, especially in early infancy when infants are unable to hold their heads erect. The true incidence of slight upper cervical spinal cord damage is not known, although it is considered rare at present. As a result of the findings in these two cases, it is suggested that hyperextension of the head be avoided, when possible.

Achondroplasia↗