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Biomedical subjects

M M Jonas

Publications and source records attributed to M M Jonas.

47 records · Page 3Linked to original sources

Hepatitis C infection in a pediatric dialysis population.

A variable prevalence of hepatitis C (HCV) infection has been reported in adult patients on hemodialysis. We have studied HCV infection and associated risk factors in a pediatric dialysis unit. Sera from all 27 patients undergoing either hemodialysis or peritoneal dialysis in our unit were tested for antibody to HCV by enzyme-linked immunosorbent assay, and seropositives were confirmed by recombinant immunoblot assay. Records were reviewed for demographic, biochemical, and risk factor data. From the total of 27 patients (12 male, mean age 20.9 years, range 7.3 to 28.1 years), five were anti-HCV(+) (18.5%). All the anti-HCV(+) patients had been on hemodialysis (69 to 194 months, mean 105 months), while of the 22 anti-HCV(-) patients, only 14 had been on hemodialysis (5 to 209 months, mean 41.4 months), P less than .005. All the anti-HCV(+) patients had received blood transfusions (10 to 124 units, mean 61.4 units) as had 12 of the anti-HCV(-) patients (1 to 54 units, mean 14 units), P less than .02. Of the 5 anti-HCV(+) patients, only one had prior hepatitis B infection; of the 22 anti-HCV(-) patients, three had hepatitis B surface antigen, and no others had evidence of hepatitis B infection. The most predictive risk factor for HCV infection was length of time on hemodialysis. Eleven of the 27 patients (40.7%) had abnormal alanine aminotransferase values, of whom four were anti-HCV(+), three were hepatitis B surface antigen(+), and one was seropositive for antibody to human immunodeficiency virus.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Congenital diabetes mellitus and fatal secretory diarrhea in two infants.

Two unrelated male infants presented with brittle insulin-dependent diabetes mellitus in the first days of life. Subsequently they each developed severe secretory diarrhea, with stool volumes of more than 100 ml/kg/day. Extensive biochemical and serological investigation failed to reveal the etiology of the diarrhea. The infants, cared for at different institutions, underwent therapeutic trials of various agents including loperamide, cholestyramine, prednisone, indomethacin, and somatostatin analogue, without response. Both infants succumbed to septicemia and malnutrition related to diarrhea and poor control of glycemia. At autopsy, both were found to have absence of islets of Langerhans in the pancreas, and diffuse dysplastic changes in small and large intestinal mucosae. In particular, the entire alimentary tract in each case was lined by epithelia most typical of foregut mucosa: secretory-type glands, absent crypts of Lieberkuhn, and absent villi. These cases are contrasted with previously-reported infants with congenital diabetes mellitus, and the possible interrelation of these two highly unusual findings, congenital diabetes mellitus and diffuse intestinal dysplasia, is examined.

Catheters, Indwelling↗

Hepatitis B infection in a large municipal obstetrical population: characterization and prevention of perinatal transmission.

We have previously described a large municipal obstetrical population in which the carriage rate of hepatitis B (HBV) is 1.2%. The present study was undertaken to determine the effectiveness of our immunoprophylaxis regimen (hepatitis B immune globulin at 36-72 h, hepatitis B vaccine at 36-72 h, 1 month and 6 months) in eliciting protective antibody to hepatitis B surface antigen (anti-HBs) in the infants of these women, the rate of perinatal transmission of HBV in this population prior to vaccination, the prevalence of anti-hepatitis delta antibody (anti-HD), and the prevalence of liver disease in our hepatitis B surface antigen-positive (HBsAg+) population. Four hundred eleven infants of HBsAg+ women were born during the 33-month study period. Of these, only 64 (15.6%) completed the vaccine series and returned for testing at 12 months. Sixty of the 64 had anti-HBs, and one (1.6%) had become HBsAg+. Eighty-nine older siblings of the immunized infants were tested, and 17 (19%) were HBsAg+. Of 54 mothers and eight siblings who were HBsAg+, none had anti-HD. Serum alanine aminotransferase (ALT) levels were normal in 53 of 54 HBsAg+ mothers tested. These data demonstrate 1) reduction of perinatal transmission of HBV from 19% to 1.6% using our protocol, 2) absence of hepatitis delta infection in this population, and 3) high prevalence of asymptomatic carriage of HBV, rather than clinically significant liver disease, in this population. It is imperative to improve compliance in order to maximize the effectiveness of immunoprophylaxis for newborns of HBsAg+ mothers.

Carrier State↗

Histopathologic features of the liver in pediatric acquired immune deficiency syndrome.

Autopsy and liver biopsy specimens from 30 pediatric patients with acquired immune deficiency syndrome (AIDS) or AIDS-related complex (ARC) were retrospectively reviewed. Of 28 cases with histologic abnormalities, the following findings were noted singly or in combination: giant-cell transformation, cytomegalovirus inclusions, Kaposi's sarcoma, diffuse lymphoplasmocytic infiltrate, granulomatous hepatitis, mild portal inflammation, necrosis around central veins, steatosis, and cholestasis. For the most part, abnormalities in the liver were not predictive of those in other organs, but the two children with the diffuse parenchymal lymphoplasmocytic infiltrate also had lymphoid interstitial pneumonitis (LIP). Liver histopathology in pediatric patients with AIDS shares some features with that in adults, but appreciable differences are noted. In particular, these differences include the higher frequency of giant-cell transformation and the lower frequency of granulomas in children and the observation of diffuse lymphoplasmocytic infiltrate associated with LIP.

AIDS-Related Complex↗

Isolated intestinal myopathy resembling progressive systemic sclerosis in a child.

A 15-yr-old girl had a life-threatening episode of toxic megacolon at age 6 yr and a life-long history of constipation and abdominal distention. A diagnosis of chronic intestinal pseudoobstruction was made. Her clinical course was that of repeated bouts of pseudoobstruction, multiple episodes of intestinal volvulus at different sites, and progressive cachexia. Histologic examination of specimens of jejunum, ileum, appendix, and colon revealed progressive fibrotic changes in intestinal smooth muscle. The abnormalities observed are most consistent with those described in progressive systemic sclerosis, but no cutaneous manifestations of this disorder have been noted in this child, and no abnormalities in other organs have been detected. Thus, this patient represents a childhood case of chronic intestinal pseudoobstruction caused by a disorder closely resembling progressive systemic sclerosis confined to the gastrointestinal tract.

Adolescent↗

Failure of Centers for Disease Control criteria to identify hepatitis B infection in a large municipal obstetrical population.

The Centers for Disease Control (CDC) has recommended screening pregnant women from high-risk populations for hepatitis B surface antigen (HBsAg). To assess the adequacy of the risk criteria, all women presenting for delivery to a large municipal hospital were screened. Sera from 5356 women were tested, and questionnaires designed to identify women at high risk were completed by 78% of these patients. Sixty-four women were found to be HBsAg seropositive (1.2%). If the CDC criteria had been applied for screening, 30 of the seropositive mothers (47%) would not have been identified. Women from some Latin American and Caribbean countries not recommended for screening were found to have a relatively high prevalence of hepatitis B infection. Reluctance to give a history of venereal disease or illicit drug use may be another factor in the failure of the CDC screening strategy. To achieve effective immunoprophylaxis of newborns, all pregnant women should be screened for HBsAg carriage.

Carrier State↗

Intestinal lactase synthesis during postnatal development in the rat.

To elucidate the mechanism of the developmental decline in intestinal lactase activity at weaning, we examined lactase synthesis in suckling and adult rats. Lactase was purified to homogeneity from pooled intestines of newborn rats and used to raise a monospecific antibody. Using this antibody, we developed a quantitative immunoprecipitation assay for lactase. Intestinal microvillus membrane proteins were labeled in 15-day and adult rats by intraluminal pulse-chase with 3H-leucine, and newly synthesized lactase quantified by immunoprecipitation. When lactase synthesis was expressed as the quantity of microvillus membrane lactase synthesized relative to total microvillus membrane protein synthesized, a significantly greater proportion of 3H-leucine incorporation into lactase was demonstrated in the suckling animals. No structural differences between newly synthesized suckling and adult lactase were observed when they were compared by SDS-polyacrylamide gel electrophoresis and fluorography. These data suggest that a change in the rate of lactase synthesis plays a role in the postweaning decline in enzyme activity.

Animal Population Groups↗

Propylthiouracil hepatotoxicity: two pediatric cases and review of the literature.

We have observed isolated hepatotoxicity in two children treated with propylthiouracil (PTU) for hyperthyroidism. Neither patient had risk factors for or clinical evidence of preexisting liver disease. In one patient the drug was promptly discontinued when signs of liver disease were noted. This patient quickly recovered. The second patient continued to receive PTU for several days after developing symptoms. Her illness progressed to fulminant hepatic failure with encephalopathy, and she died. These are the third and fourth pediatric cases reported, and there have been 10 cases reported in adults in the English language literature. Thirteen of the 14 patients are female. The literature regarding all these patients is reviewed. Propylthiouracil may cause lethal hepatic damage. This drug should be discontinued immediately if signs or symptoms of hepatic injury are detected.

Adolescent↗

Neonatal hemochromatosis: failure of deferoxamine therapy.

We describe a premature neonate with severe hepatic dysfunction from birth which progressed to fatal hepatic failure at 3 months of age. The diagnosis of a familial disorder, neonatal hemochromatosis, was made, and therapy was attempted with deferoxamine. Features of iron metabolism are presented, and this infant's illness is contrasted with other liver diseases of infancy associated with hepatic iron overload.

Deferoxamine↗