Nurten Koçak

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.

Peptic Ulcer Disease in Children Without Helicobacter pylori Infection

W e read with interest the study by Elitsur and Lawrence [1], in which they investigated the prevalence of peptic ulcer disease in children who were Helicobacter pylori negative and nonsteroidal anti-inflammatory drugs (NSAIDs) negative. In this study a total of 622 upper endoscopic procedures was carried out and 11 [1.8%] of them had mucosal ulceration. Duodenal ulceration was detected in 10 and 3 [30%] were associated with H. pylori infection. They concluded that H. pylori infection and/or NSAIDs are not the major etiologic factors for the development of peptic ulcer disease in children in developed countries. We have also evaluated the frequency of H. pylori infection in children with duodenal/ gastric ulcer. During the period January 1999 to September 2001, a total of 324 children underwent upper gastrointestinal endoscopy. The presence of H. pylori was determined by culture, rapid urease test, and histology. The main symptoms of the patients were abdominal pain, vomiting, nausea and hematemesis. No patient had a history of NSAID using. Out of 324 children, eight [2.5%] patients had duodenal/gastric ulcer. Six patients (four boys; mean age, 12.8 ± 2.1 years; range, 9–15 years) were positive for H. pylori and two patients (one boy; mean age, 10.5 ± 0.7; range, 10–11 years) were negative. Five of seven [71%] children with duodenal ulcer were H. pylori positive. Three of them also had antral nodularity, whereas all five had gastritis. Two patients who had peptic ulcer and were H. pylori negative did not have associated gastritis. The only patient with gastric ulcer was also H. pylori positive with antral nodular gastritis. H. pylori infection in children is associated with gastritis and peptic ulcer disease [2]. It has been suggested as a major cause of peptic ulcer disease [3,4]. Prieto et al. [3] showed that 90.9% of patients with duodenal ulcer were H. pylori positive. Similarly, we found H. pylori positivity in 71% of the patients with duodenal ulcer. Our results indicate that H. pylori infection should be considered as the main cause of duodenal ulcer in children, especially, in developing countries with a higher prevalence of H. pylori infection. However, as H. pylori infection was not found in 29% of duodenal ulcers, other causes should be looked for.

Is There Any Relation Between Helicobacter pylori Infection and Iron Deficiency Anemia in Children with Celiac Disease

Celiac disease is a permanent gluten intolerance characterized by histopathologic abnormalities in the proximal intestine. It has a large spectrum of gastrointestinal and extra-intestinal manifestations. Iron deficiency anemia caused by malabsorption is common and may be the only presenting manifestation of celiac disease [1]. Helicobacter pylori infection in children is associated with gastritis and peptic ulcer disease [2]. It has been suggested that H. pylori infection may lead to iron deficiency anemia [3]. The mechanisms by which H. pylori infection can cause iron deficiency anemia are still unclear [4]. In this study, we wanted to investigate the association between H. pylori infection and iron deficiency anemia in patients with celiac disease. Thirty-six children (17 boys, 47%) with untreated celiac disease were evaluated. The mean age of the patients was 11.4 ± 3.1 years, ranging from 2 to 17 years. Diagnosis of celiac disease was made on the basis of the presence of positive anti-gliadin immunoglobulin A (IgA) and IgG antibodies, and anti-endomysium antibodies, and was confirmed by histological findings. The presence of H. pylori infection was determined by culture, rapid urease test (Dio-Helico; Diomed, Nürnberg, Germany), and histology. Iron deficiency anemia was defined as a hemoglobin concentration < 11.5 g/dl aged 2–12 years, < 12 g/dl in girls aged 12–18 years and < 13 g/dl in boys aged 12–18 years, in the presence of low serum iron levels (normal range, 22–184 μg/dl) and high iron-binding capacity (normal range, 250– 400 μg/dl) [5]. In the 36 children with celiac disease, 15 (42%) had H. pylori infection and 21 (58%) did not. Of the 15 H. pylori-positive patients, 7 (47%) had iron deficiency anemia while of the 21 H. pylorinegative patients, 10 (48%) showed iron deficiency anemia (p > .05) (Table 1). Mean values of hemoglobin concentrations were 10.7 ± 1.6 and 10.7 ± 2.1 in H. pylori-infected and noninfected children with celiac disease, respectively ( p > .05). In a recent study, a relation between H. pylori infection and iron deficiency anemia has been suggested in patients with celiac disease. However, iron deficiency anemia does not develop in all H. pyloriinfected patients [6]. We did not find any significant association between H. pylori infection and iron deficiency anemia in our patients with celiac disease. Our findings suggest that celiac disease itself plays a major role, rather than H. pylori infection, in the development of iron deficiency anemia.

Cholesteryl Ester Storage Disease: Case Report During Childhood

ABSTRACT 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 3½-year-old child with CESD. The diagnosis was indicated by liver biopsy and confirmed by reduced acid lipase activity in leukocytes.

Caroli's syndrome in two siblings.

sible susceptibility of patients with hepatitis C to this adverse effect of ibuprofen is dependent rather on specific etiology of the liver disease than on the type and degree of histological damage. On the contrary, no other NSAIDs have been involved in increased hepatotoxicity of patients with chronic hepatitis C, suggesting that this phenomenon is drug specific. The incidence of acute liver injury in patients with chronic hepatitis C exposed to ibuprofen remains to be determined. We did not find any other similar case among the 421 patients with chronic hepatitis C observed in our liver clinic since 1992. However, this could be due in part to the fact that our policy is to strongly discourage the use of NSAIDs by patients with chronic hepatitis C. In conclusion, our report provides further evidence for the predisposition of patients with chronic hepatitis C to the hepatotoxicity of ibuprofen. A warning for the use of ibuprofen in this subset of patients should probably be considered.

Long-term prognosis of interferon nonresponder children with hepatitis B.

TO THE EDITOR: We read with interest the article by Mazzellaet al. (1) reporting long-term results in 64 adult patients with chronic hepatitis B (CHB). Patients were randomized into two groups; 33 of them received lymphoblastoida-interferon (IFN, 5 MU/m of body surface areat.i.w. for 6 months) and 31 were not treated. The two groups were prospectively followed for a mean of 86.4 6.9 and 79.76 6.8 months, respectively. IFNa was found to accelerate hepatitis B virus (HBV) DNA, HBeAg, and HBsAg clearance rates (78.9% vs 58.1%, 90.9% vs 61.2%, and 36.4%vs 9.8%, respectively) in treated patientsversus controls. Four treated patients (three of them nonresponders) and six controls developed cirrhosis at the end of follow-up. Among these patients with cirrhosis, one responder and two controls also developed hepatocellular carcinoma (HCC) after 60, 48, and 98 months of follow-up, respectively. We also want to report the follow-up findings with respect to long-term complications of CHB in children who have not responded to different protocols of IFNa therapy (5 or 10 MU/m of body surface area, s.c., t.i.w. for 24 or 48 wk). At the beginning of therapy, all 23 children (16 male, 69%) had abnormal or fluctuating transaminases for

PREVALENCE OF HEPATITIS A ANTIBODIES IN CHILDREN WITH CHRONIC LIVER DISEASE AND OTHER GASTROINTESTINAL DISEASES

6 months and were positive for serum HBeAg and HBVDNA. The mean age of the patients was 11.3 6 3 yr (median 11 yr, range 7–17). The patients were followed by periodic biochemical and serological tests; in addition, abdominal ultrasonography was performed for long-term complications of CHB such as cirrhosis, portal hypertension, and HCC, every year. At the end of therapy all patients were nonresponders and were followed for a mean of 54.3 6 12.3 months (median 50 months, range 46 –96). During this period, no physical or biochemical sign of cirrhosis, portal hypertension, or HCC was detected. Repeat ultrasonograms were all normal. Although the long-term prognosis of hepatitis B carrier children with normal liver enzymes is good (2), the data on the long-term results in IFN-nonresponder children with CHB are rare, especially with respect to complications. In the study by Mazzella et al., three of the IFN-nonresponders developed cirrhosis after a follow-up period of 7 yr. In our study, after 4.5 yr, we have not seen cirrhosis or other long-term complications in 23 IFNnonresponder children with CHB. Controversial data exist concerning a possible prophylactic effect of IFN against HCC in adult patients with chronic HBV hepatitis (3). Although larger controlled studies and longer follow-up is necessary, it may be speculated that CHB is relatively benign in IFN-nonresponder children, compared with adults.

SEA BLUE HISTIOCYTE SYNDROME

position. Another explanation might be catheter extravasation into the retroperitoneal space and, with ongoing parenteral feeding, retrograde catheter migration along the femoral nerve into the L4 foramen and eventually the spinal canal. All reported patients became lethargic, hypotonic and presented with clinical deterioration resembling septicaemia (Table 1). Neurological symptoms (segmental myoclonus, seizures and ̄accid quadriplegia) were noticed in two patients [1±4, 6]. During lumbar puncture milky white ̄uid was obtained or extreme hyperglycorrhachia was noticed. Prognosis of this serious complication seems to be good when recognized and treated immediately; four out of six patients had an uneventful recovery [1±4, 6]. As catheter position should be routinely checked, using a catheter with clear marks, with an anteroposterior supine abdominal ®lm, a doubtful or puzzling catheter position should be promptly analysed with a lateral abdominal ®lm in order to reduce the incidence of this potentially serious complication. In case of suspected septicaemia, lumbar puncture should be performed looking for a high level of protein, hyperglycorrhachia or milky white ̄uid. If present, parenteral feeding should be discontinued immediately, the catheter position radiographically documented and the catheter removed.

Benign Recurrent Intrahepatic Cholestasis

Three children with congenital form of sea blue histiocyte syndrome are reported. Two of them had liver involvement and the third one was mentally retarded. In all these cases, marked growth retardation was documented and the two had frequent epistaxis. The diagnosis was made by the demonstration of sea blue histiocytes in the bone marrow. These cells were also shown in the liver and spleen needle biopsy specimens, whichever was obtained.

Detection of Helicobacter pylori with stool antigen test in children with gastroesophageal reflux disease

From Hacettepe University School of Medicine, Department of Pediatrics, and Hacettepe Children’s Hospital Medical Center, Ankara, Turkey. * Professor of Pediatrics, Hematologist and Head of the Liver Clinic. ** Pediatrician. INTRAHEPATIC cholestasis due to neonatal hepatitis, intrahepatic biliary atresia and erythroblastosis fetalis is fairly frequent in the first months of life. It is not unusual in liver disease, and may be observed following the use of some drugs such