Sema Aydogdu

Treatment of Helicobacter pylori gastritis improves dyspeptic symptoms in Turkish children.

Background In adults, the treatment of Helicobacter pylori infection is only recommended for patients with active gastric or duodenal ulcers. It is not known whether similar guidelines can be applied to children because the prevalence of peptic ulcer disease in childhood is estimated to be much lower than in adults. The purpose of this study was to determine whether treatment of H. pylori gastritis would improve symptoms of dyspepsia in children. Methods Sixteen patients (5 boys, 11 girls) aged 14 ± 1.2 years who had symptoms of dyspepsia were evaluated using upper gastrointestinal endoscopy with biopsies to establish the diagnosis of H. pylori gastritis. They were treated for 2 weeks with clarithromycin, amoxicillin, and a proton pump inhibitor. Dyspepsia symptoms were evaluated by a questionnaire before and after treatment of the infection. The effect of H. pylori treatment on the total symptom score was analyzed with use of the Student t test. Values are presented as mean ± SEM. Results All patients had antral nodularity and chronic active gastritis with spiral-shaped organisms but no evidence of peptic ulcer disease. Mean total symptom score decreased significantly at 2 to 4 weeks after treatment (12.6 ± 0.9 vs. 2.1 ± 0.5 P < 0.001), and it remained low (2.9 ± 0.7) at follow-up 9.7 ± 1.4 months (range, 2–24 months later). Conclusion These results suggest that the treatment of H. pylori gastritis can improve dyspeptic symptoms in children.

Our Experience with Fulminant Hepatic Failure in Turkish Children: Etiology and Outcome

Fulminant hepatic failure is a rare and devastating event during childhood. The etiology of liver failure is reported to change according to age and geographical location. We aimed to investigate, retrospectively, causes and outcome of fulminant hepatic failure in Turkish children. Thirty-four children with fulminant hepatic failure were analysed by means of etiology and outcome. Etiological factor, clinical presentation, encephalopathy stage and biochemical parameters were correlated with outcome. Acute viral hepatitis was detected in 12 cases (35.2 per cent) and hepatitis A was the most commonly detected cause among cases with fulminant hepatic failure (n = 9, 26.4 per cent). Hepatitis B and non A-E infection were diagnosed in two (5.8 per cent) and one (2.9 per cent) cases, respectively. Wilsons disease was defined in four patients (12.5 per cent). Budd-Chiari syndrome (2.9 per cent), autoimmune hepatitis (2.9 per cent) and mushroom poisoning (2.9 per cent) were other detected causes of fulminant hepatic failure in this group. No viral, metabolic, toxic or anatomic reason could be detected in the remaining 15 (44.1 per cent) patients and they were evaluated as cryptogenic. Mortality was 67.6 per cent (23 cases). Encephalopathy grade, total and indirect bilirubin levels were found to be significantly higher in patients who died (p = 0.004, p = 0.03, p = 0.04). Seven patients could have been transplanted (two cadavaric, five living related) and the mortality of this group was 28.5 per cent (n = 2). It was concluded that fulminant hepatitis A virus (HAV) infection is the most common detectable cause of fulminant hepatic failure in Turkish children.

Vascular complications in living-related and deceased donation pediatric liver transplantation: Single center's experience from Turkey

Abstract:  The aim of the study was to assess early and long‐term incidence of venous complications, in both deceased donation (DD) and living‐related (LR) liver transplantation (LT) in a pediatric population. Seventy‐five liver transplants performed in 69 (39 boys, 30 girls) children at Ege University Hospital between 1997 and 2004 were prospectively monitored and reviewed. Age, sex, primary diagnosis, graft type, vascular complications and their management were evaluated. All patients received Doppler ultrasonographic examination both during operation and daily for the first three postoperative days and when necessary thereafter. The complications were classified as early and late presented. Thirty‐three grafts (47.8%) were from DD and 36 (52.2%) were from LR donors. Recipients of DD were older than LR donors (mean age 10.5 ± 5.1 and 5.0 ± 0.7, respectively) (p < 0.05). Vascular complication occurrence was not statistically different between DDLT and LRLT recipients (p = 0.2), and between infants and children (p = 0.9). Overall, stenosis was more common than thrombosis. We observed hepatic artery (HA) thrombosis, in five of 75 (6.7%) transplants within 30 days post‐transplant. Portal vein (PV) thrombosis and hepatic vein (HV) thrombosis were detected in six and one patients (8.7% and 1.3%), respectively. Six PV stenosis were identified (8.7%), while HA and HV‐VC (vena cava) stenosis occurred in one and six patients (1.4% and 8.7%), respectively. All PV stenosis (6/33, 18.2%) and one PV aneurysm occurred in DDLT recipients while HV‐VC stenosis were detected almost equally in LRLT and DDLT recipients (4/36 vs. 2/33). Except one, all PV stenosis were detected as a late complication and no intervention were needed. Stenosis of HV‐VC was more common in girls (5/30 vs. 1/39) (p < 0.05) and the incidence was not different in DDLT and LRLT recipients (p = 0.8). In conclusion, overall incidences of thrombosis and stenosis formation after orthotopic liver transplantation (OLT) were 17.4% and 18.8%, respectively in our center. We suggest that in the cases with HA thrombosis manifested intra‐operatively or within the early postoperative period, graft salvage was successful. Thrombosis of HA causes significant mortality. Thrombosis of PV was among the causes of mortality and morbidity. Stenosis of HV‐VC could be managed by angioplasty and endovascular stenting with no significant effect to mortality.

Current therapeutic approaches in childhood chronic hepatitis B infection: A multicenter study

Background and Aim:  The aim of the present study was to compare the therapeutic efficacy of three different regimens in childhood chronic hepatitis B (CHB) infection.

Subclinical Neurological Abnormalities in Children With Celiac Disease Receiving a Gluten-free Diet

Objectives: Because clinically evident manifestations are frequent in adults with celiac disease (CD), we aimed to investigate whether early neurological abnormalities may be detected in children with CD. Methods: Electroencephalography, electromyography, and somatosensory evoked potentials were performed in children with CD receiving a gluten-free diet. Results: The neurophysiological tests revealed subclinical neurological abnormalities associated with CD in 3 (11%) of 27 children: 2 had peripheral polyneuropathy documented with electromyography, and 1 had prolonged latencies in somatosensory evoked potential. Magnetic resonance imaging showed abnormalities in 2 (7.4%) of children: pontine demyelinization in 1 and cortical atrophy in the other. Conclusions: Because the rate of neurological problems is increased in children with CD, neurological abnormalities should be carefully investigated early after the diagnosis of CD is made.

Live donor liver transplantation for acute liver failure.

Background. Acute liver failure (ALF) carries a high mortality unless urgent orthotopic liver transplantation (OLT) is performed on time. Live donors are utilized to treat this irreversible condition first in pediatric cases and then in adults. Herein, we aimed to report our experience with live donors for ALF in a country of a deceased donor organ donation rate is only 1.5 per million people. Methods. Among the 245 live donor liver transplantations (LDLT) performed from June 1999 to December 2005, 14 of them (6%) were performed for ALF in 8 pediatric and 6 adult cases. Right lobes were harvested for the adult cases whereas left lateral segments were harvested for pediatric cases, except one child transplanted with a right lobe graft. The etiology of the disease was; acute hepatitis B in four cases, hepatitis A in three cases, Wilson disease two cases, autoimmune hepatitis in two cases, and was unknown in three cases. Results. Three-year graft and patient survival is 79% for these series. Five of the six adult patients and six of the eight pediatric cases survived after transplantation. There was not any donor mortality or major morbidity. Conclusions. LDLT offers a safe and effective modality of treatment for ALF for both pediatric and adult patients to overcome the problem of organ shortage especially in countries where the chance of receiving an organ from a deceased donor is low.

Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growth

Abstract:  In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score <2), and four patients had weight SD score <2. Liver transplantation were performed at the age of 43.2 ± 27 months (range 9 to 96 mfonths), 6.5 ± 3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score <2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r = 0.588 (p = 0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis.

Hepatocellular carcinoma in children and effect of living-donor liver transplantation on outcome

Abstract:  Hepatocellular carcinoma (HCC) is primarily observed in the older children and in most cases it develops in association with liver cirrhosis. Liver transplantation offers a good chance for long‐term cure. To evaluate the outcome of children with HCC and the impact of living‐donor orthotopic liver transplantation (OLT) on survival a retrospective review of radiographic, laboratory, pathologic, and therapeutic data in 13 children (six female and seven male) with chronic liver disease accompanied with HCC were studied. The patients were divided into two groups according to therapeutic modality: transplanted and non‐transplanted patients. Kaplan–Meier survival curves in various therapeutic groups were plotted. The mean age of patients was 6.4 ± 4.8 yr. Pediatric end‐stage liver disease score was adapted to model for end‐stage liver disease score for HCC and ranged between 1–44 and 18–44, respectively. The underlying liver diseases were tyrosinemia type 1 (n = 6), chronic hepatitis B infection (n equals;6), glycogen storage disease type 1 (n = 1). Alfa‐feto protein levels were elevated in all patients except one. Median number of tumor nodules was three (1–10), median maximal diameter of tumor nodules was 3.4 cm (0.5–8). Eleven patients were eligible for OLT whereas two patients were not eligible. Seven of the 11 patients considered for transplantation underwent living‐donor OLT. Remaining four patients died while waiting on cadaveric transplant list. Overall 1 and 4‐yr survival rates for all patients were 53.3 and 26.6%, respectively, and were found significantly higher in transplanted children than non‐transplanted children (72%, 72% vs. 33% and 16.6%). No patient had tumor recurrence at median of 36‐month follow‐up after OLT. OLT is a life‐saving procedure for children with chronic liver disease accompanying with HCC. Living‐donor OLT avoids the risk of tumor progression and transplant ineligibility in these children.

Inflammatory myofibroblastic tumor of small bowel wall in childhood: Report of a case and a review of the literature

Benign intestinal tumors are rare in children, however we describe an inflammatory myofibroblastic tumor (IMT) of the jejunum in a 2‐year‐old girl who presented with an intestinal obstruction. During laparotomy, an annular mass around the jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare entity emphasizes the importance of histological confirmation of its benign nature. Because of the risk of local recurrence, IMT cases should have a long‐term follow up.

Positive association of macrophage migration inhibitory factor gene-173G/C polymorphism with biliary atresia.

Background: Macrophage migration inhibitory factor (MIF) is a pleiotrophic lymphocyte and macrophage cytokine; it is likely to play an important role in innate immunity. Its expression was increased in several inflammatory diseases, and MIF gene polymorphisms have an effect on disease outcome and response to glucocorticoid treatment. Aim: To investigate the role of the 173G/C polymorphism of the MIF gene for susceptibility to biliary atresia (BA). Method: Between February 2002 and November 2004, 18 patients (mean age 1 ± 0.4 years) diagnosed as having BA were studied. After informed consent, blood was collected, and DNA was obtained. MIF 173C/G polymorphism was detected using the polymerase chain reaction-restriction fragment length polymorphism based method. BA patients were compared with a group of chronic liver disease patients (CLD) (n = 36) and a group of unrelated healthy controls (n = 103). Results: MIF-173C allele frequency was significantly higher than both the CLD and healthy control groups (P = 0.03, odds ratio [OR] 4.4, 95% confidence interval [CI] 1.3-15.1; P = 0.000, OR 4.1, 95% CI 2.3-7.6, respectively). Univariate analysis showed that MIF-173G/C polymorphism was significantly associated with BA (for GC genotype, OR = 6, 95 % CI 2.8-11.5, P = 0.000). There was no significant correlation between pediatric end stage liver disease score and MIF genotypes both in BA and CLD groups. Conclusion: Our results suggest that the -173C allele of the MIF gene might be associated with the susceptibility to BA.