Ascites related to cytomegalovirus infection.
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Ascites↗
Biomedical subjects
A Yüce
Publications and source records attributed to A Yüce.
Propranolol therapy for portal hypertension in children.
Administration of propranolol to 13 children with portal hypertension reduced splenic pulp pressure by greater than 50 mm H2O (P less than 0.01) in approximately 2 weeks, when the pulse rate became three fourths the initial rate. The influence was found to be greater in compensated than in decompensated portal hypertension. This observation might be interpreted to mean that the effect of propranolol in the reduction of portal venous pressure results not only from decreased intestinal blood flow secondary to decreased cardiac output but also to the stimulation of sympathetic nervous system alpha-adrenoreceptors of the portal tract. Although arterial blood pressure changes were not significant, peripheral venous pressure was reduced significantly (P less than 0.01). We conclude that propranolol has considerable usefulness in treating portal hypertension in children.
Adolescent↗
[Dental restoration using intubation anesthesia].
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Adolescent↗
[Chronic apical periodontitis: borderland between surgical and conservative treatment].
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Humans↗
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↗
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↗
Cutis laxa acquisita: is there any association with Borrelia burgdorferi?
We report the first case of an acquired form of generalized cutis laxa which has positive serology and a positive polymerase chain reaction (PCR) result for lyme borreliosis. A 44-year-old man complained of excessively loose skin for four years and had no family history of any skin disease. Dermatological examination showed lax and wrinkled skin all over the body (especially on the cheeks and the intertriginous areas). Positive serology for lyme borreliosis and the presence of Borrelia burgdorferi DNA which was demonstrated by nested PCR in this acquired form of cutis laxa is interesting since it has not been reported in literature previously.
Adult↗
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↗
Stenotrophomonas maltophilia pneumonia in a premature infant.
Stenotrophomonas (Xanthomonas) maltophilia is an aerobic, non-fermentative, gram-negative bacillus that is generally considered an opportunistic pathogen. Infections due to S. maltophilia have become increasingly important in the hospital environment. Patients compromised by debilitating illnesses, surgical procedures or indwelling vascular catheters are most prone to S. maltophilia infections. To our knowledge, we report the first case of S. maltophilia pneumonia in a premature infant of 31 weeks gestational age. Although the therapy of choice for severe infections caused by S. maltophilia remains to be decided, this patient was successfully treated by amikacin.
Amikacin↗
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↗
Recombinant interferon-alfa therapy in children with chronic hepatitis B and cured cancer.
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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↗
Concurrent presence of HBsAg and anti-HBs in children.
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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↗
Primary hemophagocytic lymphohistiocytosis in Turkish children.
Nineteen children with hemophagocytic lymphohistiocytosis (HLH) were studied in the Department of Pediatric Hematology, Hacettepe University. Patients were divided into two groups. Group 1: Thirteen patients were classified as having a genetic etiology (7 familial, 6 presumed familial) on the basis of an affected sibling and consanguinity. There was a history of consanguineous marriage in 13 of the families. Seven of them had a history of a sibling with HLH. Group 2: Six patients were diagnosed with sporadic HLH. The age at presentation for familial patients was 0.7-84 months (mean 21.9 +/- 24.9 months), and for sporadic cases it was 2.5-48 months (mean 22.7 +/- 19.8 months). The clinical and laboratory data of these two groups were similar at diagnosis. Thirteen cases were diagnosed premortem by bone marrow aspiration. Splenic biopsy was performed in 2 patients. Four patients were diagnosed by postmortem examination. Elevated LDH levels were found in all patients tested. No significant differences for clinical and laboratory data were found between the two groups.
Bone Marrow↗
Congenital generalized lipodystrophy: Berardinelli syndrome. Report of two siblings.
Two successively born infants with Berardinelli syndrome, an unusual lipodystrophic disease, are reported. In addition to hepatomegaly, accelerated growth, muscle hypertrophy, lack of adipose tissue, hirsutism and hypertriglyceridemia, which are the characteristics of the syndrome, these brothers demonstrated bilateral, symmetrical, renal medullary hyperechogenicity, which has not before been reported in association with generalized lipodystrophy. Although more than 25 cases have been recorded, the metabolic defect responsible for this inborn error of metabolism has not yet been determined.
Abnormalities, Multiple↗