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Biomedical subjects

H Ozen

Publications and source records attributed to H Ozen.

At least 145 records · Page 8Linked to original sources

Surgical management of vesicoureteral reflux in children.

The authors report on ten years of experience in ureteroneocystostomies on 80 ureters in 50 children suffering from vesicoureteral reflux. Complete follow-up was obtained in all cases. Postoperative radiograms revealed cure of reflux in 85 per cent of the renal units. A slightly modified Politano-Leadbetter procedure was used in all of the cases. Postoperative urinary tract infection occurred in 13 patients. This relatively low success rate is discussed briefly.

Adolescent↗

Biopsy of apparently normal bladder mucosa in patients with bladder carcinoma and its prognostic importance.

A total of 100 cold cup biopsies were obtained from apparently normal vesical mucosa of 25 patients admitted to Hacettepe University Hospital between 1980 and 1981. Dysplasia was found to occur in 40.49, hyperplasia in 17.35, squamous metaplasia in 9.91 per cent. Dysplasia was shown to be increasing parallel with the increase in the grade and stage of the tumour. The frequency of dysplasia and hyperplasia in the recurrent and non-recurrent groups differed significantly (p less than 0.001). In other words, recurrence was seen more frequently in patients with positive random biopsies. Although recurrence phenomena cannot be explained on the basis of mucosal abnormalities alone, this study shows that these subendoscopic lesions do have some prognostic role in recurrences.

Aged↗

Cholesteryl ester storage disease: case report during childhood.

Cholesteryl ester storage disease (CESD) is rare and characterized by accumulation of cholesteryl esters and triglycerides in many tissues due to the deficiency of lysosomal acid lipase. We report a 3(1/2)-year-old child with CESD. The diagnosis was indicated by liver biopsy and confirmed by reduced acid lipase activity in leukocytes.

Biopsy↗

nm23 protein expression in renal cell tumors: the role of the cell type.

This study investigated nm23 protein expression in renal cell carcinomas to determine the relationship between nm23 protein expression and grade, stage, prognosis and the cell type. 89 cases were examined by immunohistochemistry. Tubular epithelia were homogeneously stained. Cytoplasmic nm23 protein levels were reduced in renal cell carcinoma. nm23 protein levels persisted in oncocytomas, which are accepted to be benign. Cytoplasmic nm23 staining intensity did not show any correlation with stage and grade of tumor nor prognosis. Reduction in nm23 protein levels may have a role during renal cell carcinoma pathogenesis but not in progression or metastasis suppression.

Adult↗

Recombinant interferon-alpha-2A with or without steroid pretreatment in children with chronic hepatitis B.

Interferon is the most promising therapeutic agent for the treatment of chronic viral hepatitis. The results of studies suggest that corticosteroid pretreatment may improve the response rate. Twenty-nine children with chronic hepatitis B (CHB) were randomly assigned to receive recombinant interferon alpha (rIFN-alpha) alone (Group 1.5 million units/m2 body surface, 3 times a week for 24 weeks) or to receive oral prednisone (Group 2.2 mg/kg/day for 3 weeks, discontinued by tapering the dose within 1 week) followed by rIFN-alpha (same dose as above). Tests for liver function and hepatitis B virus (HBV) markers including HBV-DNA were done periodically. Overall, 10 patients (34.5%) cleared hepatitis Be antigen and 13 (44.8%) HBV-DNA. Anti-HBe seroconversion was observed in nine patients (31%). Only three patients (10.3%) cleared hepatitis B surface antigen and seroconverted to anti-HBs. No response was obtained in 11 patients (37.9%). There was no statistically significant difference between the two treatment groups regarding response rate. Baseline transaminases levels and HBV-DNA concentrations were predictive parameters for HBeAg clearance. It is concluded that prednisone pretreatment does not have a beneficial effect in children with CHB.

Adolescent↗

Propranolol for primary and secondary prophylaxis of variceal bleeding in children with cirrhosis.

Variceal bleeding due to portal hypertension is a frequent and severe complication of cirrhosis in children as in adults. The prophylactic approach is important for these high mortality bleedings, both for the first and for recurrent attacks. Variceal bleeding/rebleeding rates were evaluated in sixty patients with cirrhosis who received 1-2 mg/kg/day propranolol p.o. for 1-14 years. According to Child-Pugh classification, 33 patients were Class A, 22 Class B, and five Class C. Patients were divided into two groups according to whether they had variceal bleeding before starting propranolol treatment (secondary prevention; 15 patients) or not (primary prevention; 45 patients). Seven (15.6%) of 45 patients experienced bleeding on propranolol therapy in the primary prevention group, while eight (53.3%) of 15 patients bled in the secondary prevention group (p < 0.01). Propranolol was found effective in primary and secondary prevention in Class A patients, while it was effective only for primary prevention in Class B and C patients. Propranolol administration is useful for preventing first and recurrent variceal bleeding in Class A cirrhotic patients. In Class B and C cirrhotic patients, it is effective only for preventing the first bleeding episode.

Adolescent↗

Interferon-alpha treatment for chronic hepatitis C in children.

Interferon-alpha therapy has been proven efficient in chronic hepatitis C infection. Although it has been used as a standard therapy in adults, there are limited data on benefits of interferon treatment in children. We conducted a study of recombinant interferon-alpha therapy in 10 children with chronic hepatitis C. They had high aminotransferase values and positive antibodies to hepatitis C virus and HCV-RNA for at leas six months. Interferon-alpha was given at a dosage of 5 million units/m2 body surface three times a week for six months. At the end of therapy, five (50%) of the patients had complete response and two partial response. Three patients were nonresponders. Eight of the patients could be followed up for six months after stopping therapy, at which point one of the four complete responders and a partial responder relapsed. One of the three nonresponders had complete response at 12 months. Eventually, four (50%) of eight patients were complete responders. All of the nonresponders were the patients with previous malignant diseases. These findings suggest that interferon-alpha has beneficial effects in children with chronic hepatitis C, and a six month therapy seems to be reasonable. Patients with underlying malignant disease are not good candidates for interferon treatment.

Adolescent↗

Hepatitis as the presenting symptom of childhood systemic lupus erythematosus.

We report in this article a girl with an initial diagnosis of autoimmune hepatitis who developed full-blown systemic lupus erythematosus (SLE) at her two-years follow-up. She was formerly considered as HBV-related chronic active hepatitis but due to the persistence of elevated liver enzymes, the reversal of the albumin and globulin ratio and abnormal HBV serology, she was later diagnosed as autoimmune hepatitis. With the clinical findings of arthritis, arthralgia and malar rash and supported by results of laboratory tests, she was diagnosed as a case of unusual SLE presenting with autoimmune hepatitis. We conclude, therefore, that each patient with a diagnosis of autoimmune hepatitis in childhood who exhibits abnormal HBV serology must be evaluated for a possible diagnosis of SLE.

Child↗

Ursodeoxycholic acid therapy in children with cholestatic liver disease.

The beneficial effect of ursodeoxycholic add have been documented in adults but experience with this agent is limited in the pediatric population. The objective of this study was to evaluate ursodeoxycholic acid treatment in children with cholestatic liver disease. Twenty-four patients with intrahepatic cholestasis (neonatal hepatitis 7, Byler disease 7, idiopathic intrahepatic cholestasis 10) whose ages ranged from 1.5 months to 15 years were treated with ursodeoxycholic acid (15-20 mg/kg/day) for 12 months. Liver biopsy was performed initially on all patients and on 17 at the end of the twelve months. The outcome was evaluated by monitoring clinical and biochemical markers of cholestasis, including alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, cholesterol, total serum tasting bile acids and total and conjugated bilirubin at entry and every three months of treatment. Pruritus was ameliorated in all patients; there was complete disappearance of itching in 16.7 percent. There were significant decreases in mean serum levels of alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, total bilirubin and gamma-glutamyl transpeptidase. Liver biopsy specimens showed a significant improvement in the cholestasis but not in fibrosis. No adverse effects of therapy were noted. The improvements in the clinical and biochemical parameters and tolerability of the drug suggest that ursodeoxycholic acid is a safe and effective treatment in children with intrahepatic cholestasis.

Adolescent↗

Wilson's disease patients with normal ceruloplasmin levels.

Wilson's disease, an inborn defect of copper metabolism, is a fatal disease unless specific treatment is given. Hepatic presentation mimics almost all kinds of liver disease and the diagnosis is sometimes problematic. The diagnosis is based on clinical findings, family history, presence of Kayser-Fleischer rings, and results of key laboratory tests such as low serum ceruloplasmin level, increased urinary copper excretion and hepatic copper content. We report four patients with Wilson's disease with hepatic manifestations with unknown there were difficulties in making the diagnosis because of normal serum ceruloplasmin levels. Inspite of normal ceruloplasmin levels and absence of Kayser-Fleischer rings, strong family history suggested Wilson's disease and the diagnosis was confirmed by increased urinary and hepatic copper amounts.

Adolescent↗

Acute lymphoblastic leukemia in a child with Wilson disease.

Wilson disease is an autosomal recessively inherited disease of copper metabolism and is characterized by liver and central nervous system dysfunction. The heterozygote carrier state rate is about one in 90 persons and the incidence of the disease is about 30 in 1,000,000. Although leukemia is the most common form of childhood malignancies, the probability of the presence of Wilson disease and acute lymphoblastic leukemia in the same patient is very low. We report an unusual case of a child with Wilson disease who developed acute lymphoblastic leukemia in three months.

Child↗

Gaucher disease type I: analysis of two cases with thalassemic facies and pulmonary arteriovenous fistulas.

Here we report two unusual patients with Gaucher disease type I. Both girls admitted with hepatosplenomegaly, growth retardation, and anemia at four and 2.5 years of age, and Gaucher cells were seen on bone marrow aspirates. Thalassemic face was first noted at 8 and 11 years of age, respectively, with frontal bossing and maxillary hypertrophia. Although they had unconjugated hyperbilirubinemia, high reticulocytes, polychromasia, and normoblasts on peripheral smear, other laboratory tests for hemolytic disease were negative. Radiological examination revealed typical bone involvement of Gaucher disease, as well as costal enlargement and obliteration of paranasal sinuses, the latter two reported in hemolytic diseases. Cyanosis, digital clubbing and recurrent lung infections led to contrast echocardiography that revealed diffuse pulmonary arteriovenous shunting in both. Diagnosis was confirmed by low leukocyte beta glucosidase levels and mutations N370S7/L444P (Case 1) and N370S/? (Case 2). These features, all reported for the first time, may show a new clinical course in Gaucher disease.

Abnormalities, Multiple↗

Neuroblastoma presenting as protein-losing enteropathy.

Protein-losing enteropathy is often reported to be associated with malignancies such as Hodgkin's disease, non-Hodgkin's lymphoma, and mesenteric mesenchymoma, but it seldom complicates neuroblastoma. In this report, we describe a case of neuroblastoma presenting as protein-losing enteropathy in which neurohumoral mechanisms were involved.

Female↗

Prognostic factors in Salmonella typhimurium septicemia. A 10-year retrospective study.

In this study, 74 S.typhimurium septicemia cases were evaluated retrospectively from their records, and the age and sex distribution, presence of underlying disease, signs and symptoms, complete blood count, liver function tests and case fatality rate were documented and prognostic factors determined. It has been shown that S.typhimurium is the most common strain causing Salmonella septicemia, which is more fatal in the newborn period and in the presence of an associated disease, while hemoglobin and leukocyte counts do not play an important role in the prognosis. In Salmonella septicemia, congenital heart disease was the second-most common associated disease, which may be attributed to probable underlying immunodeficiency.

Age of Onset↗

Typhoid fever with very high transaminase levels.

Typhoid fever is endemic in developing countries and may cause very different clinical findings. Although hepatic involvement and abnormal liver function tests may be seen in 50% of the patients, intravascular hemolysis and renal involvement are rare. In this report, a 10-year-old patient with enteric fever presenting with hepatitis, severe intravascular hemolysis and glomerulonephritis is presented. To see all of these findings together in a patient with typhoid fever is very rare and may cause diagnostic difficulties.

Alanine Transaminase↗