Early and late neurological complications of liver transplantation in pediatric patients.

NCs occur commonly after solid organ transplantation and affect 15%-30% of liver transplant recipients. The aim of this retrospective study was to evaluate the type and incidence of neurologic events in pediatric patients following LT. Between May 2006 and June 2015, 242 patients (118 females, 124 males) requiring LT for different etiologies at the Inönü University Liver Transplantation Institute were included. The incidence, types, and risk factors of NCs that occurred following LT were evaluated retrospectively. Neurologic events occurred in 57 (23.5%) of the patients. Early NCs were encephalopathy (12.4%), seizures (11.5%), and PRES (7%). Of 57 patients, five (8.7%) experienced NCs at least 1 month after LT; these late NCs included tremor, headaches, encephalopathy, ataxia, and neuropathy. The psychiatric symptoms after LT were noted in 42 patients (17.4%). The mortality rate after LT in those with or without neurological events was not significantly different (P=.73). There was a high incidence of serious neurologic events after LT. The major neurologic manifestation in our patients was encephalopathy followed by seizures.

Evaluation of renal functions in pediatric liver transplantation.

AKI is an important complication after LT. As our LT series contains a quite high number of children with ALF unlike published studies, we aimed to determine pre-LT and long-term renal functions in children both with ALF and with CLD. Demographic and disease-related data of 134 transplanted children were evaluated retrospectively. Pre-LT and follow-up GFR and pediatric RIFLE scores were determined. Mean pre-LT GFR was not dependent on the disease presentation or severity of chronic disease. While there was an initial decline until first week of post-LT in CLD children, an increase was observed in ALF. Neither mean GFR nor the pRIFLE on follow-up was different with respect to the type of LT or disease presentation. Mean GFR at first and sixth months were lower in children on cyclosporine compared to tacrolimus (p = 0.001 and p = 0.002, respectively). In conclusion, GFR-time curve was different in children with or without ALF. Type of LT, and severity of the CLD were not risk factors for CKD in any time, but younger age at LT, CLD, and cyclosporine usage were at sixth months of follow-up.

Virulence factors and antibiotic resistance in children with Helicobacter pylori gastritis.

OBJECTIVES: There are limited data regarding the pattern of Helicobacter pylori (Hp) antibiotic resistance and virulence factors in children. Evaluation of prevalence of drug resistance and virulence-factor genotype in children with Hp gastritis and to investigate whether there is any relation between drug resistance and genotype were our aims in this study. METHODS: Ninety-eight children with polymerase chain reaction-positive Hp gastritis were included. Antimicrobial susceptibility was tested by disc diffusion method and polymerase chain reaction assays were used for the determination of virulence factors. RESULTS: The resistance rates to clarithromycin, metronidazole, and amoxicillin were 23.5%, 11.7%, and 3.9%, respectively. All strains carried vacA genotype, and 51%, 70.4%, 49%, 34.7%, and 25.5% were cagA-, cagE-, babA2-, iceA1-, and iceA2-positive, respectively. Of those 98 specimens, 81.6%, 19.4%, 38.8%, and 63.3% carried vacAs1, vacAs2, vacAm1, and vacAm2, respectively. Dominant vacA type was s1am2 (32.7%), followed by s1am1 (14.3%) and s2m2 (12.2%). Significant rates of clarithromycin resistance were observed in cagE-, iceA1-, babA2-, and vacAs1c-positive groups. In those with metronidazole resistance, vacAs1 and vacAs1c were more common (P < 0.05). CONCLUSIONS: The cagE-positive and vacA s1a/m2 genotypes, which are correlated with increased antibiotic resistance, were predominant in our population. In countries where Hp infection is prevalent, studies focusing on virulence factors and antibiotic susceptibility may provide anticipation of the prognosis and may be helpful to reduce morbidity and mortality.

Single-center analysis of the first 304 living-donor liver transplantations in 3 years.

BACKGROUND/AIMS: Living donor liver transplantations (LDLT) is a definitive treatment for patients with end-stage liver disease (ESLD), especially in the countries with donation problem. Between April 2007 and April 2010, we performed LDLT in 289 patients. Fifteen of the cases required re-transplantations. This study evaluates these 304 consecutive LDLTs donor and recipient outcomes. METHODOLOGY: Complication rates and survival data of the recipients and donors of 304 LDLT cases were analyzed. RESULTS: All donors are alive and well. Overall complication rate was 27%. Early postoperative recipient complication rate was 51%. Most frequent complication was infection. In the long-term there were 57 biliary stricture and 5 chronic bile fistula cases. Chronic and acute rejection attacks developed in 7 and 103 patients, respectively. Hepatic artery thrombosis rate was 8%. One, two and three year survival rates were 82%, 79% and 75%, respectively. Recipient mortality was 25%, mostly due to vascular complications, septic complications, liver dysfunction and chronic rejection. CONCLUSIONS: More than 150 liver tranplantations per year in a single center is a challenge in Turkey, where there is a shortage of deceased donor grafts. LDLT is a safe procedure for donors and effective for ESLD. Improvement in surgical technique would provide better outcomes.

Subclinical diastolic dysfunction in children with Wilson's disease assessed by tissue Doppler echocardiography: a possible early predictor of cardiac involvement.

BACKGROUND: The aim of this study was to evaluate the left ventricular systolic and diastolic functions and cardiac rhythm problems for the early detection of myocardial dysfunction in children with Wilson's disease. METHODS: We compared patients who had Wilson's disease (n = 22) witl Wilson's disease was based on clinical symptoms and laboratory tests (serum ce cardiographic examination, as well as pulsed-wave Doppler, tissue Doppler ech Holter monitoring was also performed in all subjects. RESULTS: All patients were asymptomatic on cardiological examination an tion, fractional shortening, wall thickness and left ventricular mass were similar disease had significantly lower mitral E velocity, mitral E/A ratio (P= 0.046, P= 0.0 as estimated by pulsed wave Doppler echocardiography. Wilson patients had lo pler echocardiography (P=0.006) compared to the controls. On 24-hour ECG cardiac arrhythmia. CONCLUSION: Our study showed results that might be consistent with disease children which probably represents an early stage of cardiac involvem the patients.

The role of fecal calprotectin and lactoferrin in the diagnosis of necrotizing enterocolitis.

BACKGROUND: Early detection of necrotizing enterocolitis can improve the prognosis, however, there is not a reliable laboratory test to detect either newborns at risk for necrotizing enterocolitis development or those at early stages of the disease. Since fecal lactoferrin and fecal calprotectin are inflammatory markers of gastrointestinal diseases, it was hypothesized that both these biomarkers could be successfully used in the diagnosis of necrotizing enterocolitis. METHODS: In a prospective study, fecal lactoferrin and fecal calprotectin concentrations of 14 newborns with necrotizing enterocolitis and consecutively admitted 40 healthy preterm, and 23 healthy full-term newborns were measured with enzyme-linked immunosorbent assay technique. RESULTS: Mean fecal lactoferrin and fecal calprotectin were not different between preterm and full-term newborns (p = .235 and p = .845, respectively), or those who were diagnosed with necrotizing enterocolitis or not (p = .545 and p = .968, respectively). Prevalence of necrotizing enterocolitis was 1.51% (14 of 2734). Stage of the disease did not have a statistical effect on mean levels (p = .694 and p = .267, respectively). Mean fecal lactoferrin and fecal calprotectin levels were not different in the case of breastfeeding (p = .623 and p = .792, respectively). CONCLUSION: Neither fecal lactoferrin nor fecal calprotectin has a role in the identification of necrotizing enterocolitis, especially in early stages of the disease. Further studies on wider necrotizing enterocolitis series are needed for a more definite conclusion.

Hand grip and pinch strength in patients with nocturnal enuresis: is there a role of muscle strength in pathogenesis of enuresis?

AIMS: To examine the association of muscle strength with nocturnal enuresis. SUBJECTS AND METHODS: One hundred sixty-three patients with monosymptomatic nocturnal enuresis (8-14 years old) were recruited from outpatient clinics. Two hundred eight healthy students served as the control group (8-14 years old) from two primary schools. Physical examinations and the age, gender, height, weight, dominant hand, and body mass of these children index were recorded. A calibrated, Jamar dynamometer was used to assess grip strength at the first two settings. A pinch gauge was used to assess the key pinch of the right and left hands of these children. Two measurements of each grip and pinch were obtained at 15 sec intervals and mean values were analyzed. RESULTS: The hand grip strength test and pinch was significantly lower in the enuretic group compared with the control group. These low results of the muscle strength in the enuretic group showed that whole body muscle weakness may contribute to the pathogenesis of enuresis as a possible etiological factor. CONCLUSIONS: In this first study on the association of muscle strength with nocturnal enuresis, it was found that neuromuscular function is abnormal in enuretic patients. In this neuromuscular abnormality, the motor activity is affected.

Atypical presentations of celiac disease.

Celiac disease (CD) has a wide variety of clinical presentations; together with the "classical form", in which the intestinal symptomatology is prevalent, there are "atypical forms" with predominating extra-intestinal clinical features, and the "silent form", with no clinical symptom. The "atypical forms" of the disease are characterized by few or no gastrointestinal symptoms, and predominating extra-intestinal features such as neurologic, dermatologic, hematologic, endocrinologic, reproductive, renal, psychiatric, skeletal, and liver involvement(s). Silent presentation of CD may be identified through screening of high-risk groups. Today, it is well known that CD might account for several chronic health issues, so it is essential for healthcare professionals to have a high level of suspicion for the atypical presentations of CD.

Familial Mediterranean Fever Mutation Analysis in Pediatric Patients With Inflammatory Bowel Disease: A Multicenter Study.

BACKGROUND: The aim of the study was to evaluate familial Mediterranean fever (FMF) mutation analysis in pediatric patients with inflammatory bowel disease (IBD). The relation between MEFV mutations and chronic inflammatory diseases has been reported previously. METHODS: Children with IBD (334 ulcerative colitis (UC), 224 Crohn's disease (CD), 39 indeterminate colitis (IC)) were tested for FMF mutations in this multicenter study. The distribution of mutations according to disease type, histopathological findings, and disease activity indexes was determined. RESULTS: A total of 597 children (mean age: 10.8 ± 4.6 years, M/F: 1.05) with IBD were included in the study. In this study, 41.9% of the patients had FMF mutations. E148Q was the most common mutation in UC and CD, and M694V in IC (30.5%, 34.5%, 47.1%, respectively). There was a significant difference in terms of endoscopic and histopathological findings according to mutation types (homozygous/ heterozygous) in patients with UC (P < .05). There was a statistically significant difference between colonoscopy findings in patients with or without mutations (P = .031, P = .045, respectively). The patients with UC who had mutations had lower Pediatric Ulcerative Colitis Activity Index (PUCAI) scores than the patients without mutations (P = .007). CONCLUSION: Although FMF mutations are unrelated to CD patients, but observed in UC patients with low PUCAI scores, it was established that mutations do not have a high impact on inflammatory response and clinical outcome of the disease.

Celiac disease: prevention and treatment.

Celiac disease is a common multisystemic disorder that may be diagnosed at any age. Owing to the increasing prevalence, prevention strategies, including encouragement of breastfeeding and introduction of small amounts of gluten whereas still on breastfeeding at fourth to sixth month of age, become more important than before. For avoiding complications of celiac disease, a strict gluten-free diet is essential. A lifelong gluten-free diet may bring about difficulties as avoiding gluten completely is problematic owing to the contamination with gluten of presumably gluten free foods. New therapeutic approaches include enzyme supplementation, correction of the intestinal barrier defect against gluten entry, blocking of gliadin presentation by human leukocyte antigen blockers and tissue transglutaminase inhibitors.

The etiology of severe neonatal hyperbilirubinemia and complications of exchange transfusion.

Exchange transfusion (ECT) has an important role in preventing kernicterus in the treatment of indirect hyperbilirubinemia of the newborn. In present study, the etiology of hyperbilirubinemia and complications of ECT were studied over a five-year period in the Eastern Mediterranean region of Turkey. We describe our experience of 89 ECTs performed from 2003-2008 in 79 newborns with hyperbilirubinemia. The mean gestational age was 37 +/- 2.1 weeks and the mean of peak total bilirubin levels was 28.1 +/- 6.4 mg/dl. The most common cause of hyperbilirubinemia was ABO isoimmunization (38%). Complications of ECT developed in 17 neonates (21.5%), the most common being thrombocytopenia and seizure. None of newborns died secondary to ECT. Our data showed higher morbidity rates associated with ECT in the treatment of hyperbilirubinemia in our region. In order to prevent adverse effects of ECT, serum bilirubin levels should be closely monitored in newborns with ABO immunization.

Treatment results of chronic hepatitis B in children: a retrospective study.

In this retrospective study, we aimed to share our experience with different treatment modalities for chronic hepatitis B in a series of children. The study included 126 children (mean: 9.5 +/- 3.8 years). Normalization of alanine aminotransferase (ALT), loss of hepatitis B virus (HBV)-DNA and hepatitis B e antigen (HBeAg), and development of antibody to HBeAg (anti-HBe) altogether at the end of the treatment was considered as end of therapy response (ETR). Seroconversion ongoing one year after the cessation of therapy was considered as sustained response. Of the total children, 90 (71.4%) were treated, whereas the remaining were just followed-up. High-dose interferon (IFN)-alpha (10 MU/m2) alone, standard-dose IFN-alpha (6 MU/m2) plus lamivudine (4 mg/kg/d), high-dose IFN-alpha plus lamivudine, or lamivudine alone was used, IFN-alpha thrice weekly for six months, and lamivudine daily for one year. Of children who had completed their treatment, 34 (37.8%) achieved ETR. Sustained response rate was 36.7%. Response rates were different in the different treatment groups (p: 0.01). The highest response rate was observed in those who received standard-dose IFN-alpha plus lamivudine treatment (61.5%). Of children without treatment, one (2.8%) had anti-HBe seroconversion. Standard-dose IFN-alpha plus lamivudine treatment was found superior to the other treatment modalities. Predictors of ETR were similar to those found in previous studies.

Plasma visfatin concentrations in childhood obesity: relationships with insulin resistance and anthropometric indices.

BACKGROUND: The aim of this study was to investigate the relationships between plasma visfatin, insulin resistance, lipid profile and anthropometric measurements in obese children. SUBJECTS AND METHODS: Plasma levels of visfatin, insulin, glucose, lipid profile and anthropometric indices were determined in 30 obese children and compared with those in 30 age- and gender-matched non-obese children. Visfatin was measured with enzyme-linked immunosorbent assay and logarithmically transformed to log visfatin for parametric comparisons. RESULTS: The obese group had significantly elevated plasma visfatin, fasting glucose and insulin and homeostasis model assessment (HOMA) values, as well as elevated lipid concentrations, compared with non-obese children. In the obese group log visfatin correlated positively with weight (p = 0.007), waist circumference (p = 0.007), hip circumference (p = 0.034), BMI (p = 0.005), insulin (p = 0.041) and HOMA (p = 0.044). No correlation was found between visfatin and lipid profile in obese children (p >0.05). Linear regression analysis revealed significant positive relationships between log visfatin and BMI (p = 0.005), insulin and BMI (p <0.001), and between HOMA and BMI (p <0.001) in the obese group but not in the control group. Multivariate regression analysis with log visfatin as a dependent variable showed that only BMI (p = 0.005) and bodyweight (p = 0.014) correlated positively with log visfatin in obese children. CONCLUSIONS: An increased visfatin concentration may be associated with BMI and insulin resistance in obese children. Although these findings may lay a foundation for further hypotheses, the limited sample size in the present study means that longitudinal studies with more patients are needed.

An outbreak of Serratia marcescens septicemia in neonates.

Serratia marcescens is a well recognized nosocomial pathogen. We report an outbreak with this organism in 8 neonates in a neonatal intensive care unit (NICU). Seven cases were treated successfully with meropenem after the failure of imipenem treatment. Although they have similar anti-microbial effects, meropenem can effectively treat the S. marcescens sepsis resistant to imipenem.

Diffuse cystic bronchiectasis associated with left microtia and external auditory canal atresia: a very rare coincidence.

OBJECTIVE: To present a case in which diffuse cystic bronchiectasis was associated with left microtia/external auditory canal atresia. CLINICAL PRESENTATION: A 10-year-old girl suffering from cough, fever, dyspnea and sputum for 6 months was transferred to our clinic due to the diagnosis of bronchopneumonia. She had recurrent episodes of bronchopneumonia. On examination, left microtia and left external auditory canal atresia were detected. Thorax CT revealed diffuse cystic bronchiectasis on the left lung. Radiological examination showed atresia of the left external auditory canal. We could not find any etiopathological reason causing bronchiectasis. INTERVENTION: The patient underwent left pneumonectomy and postoperative histopathology was reported as bronchiectasis. CONCLUSION: This report shows a unique case in which an association of diffuse cystic bronchiectasis and left microtia/external auditory canal atresia was observed. Hence in newborns with microtia and/or external auditory canal atresia, the probability of development of bronchiectasis should be borne in mind and such patients should be followed up more carefully regarding this rare association.

Oxidative stress and antioxidant status in neonatal hyperbilirubinemia.

OBJECTIVE: To investigate the possible relationships between plasma bilirubin levels and concentrations of nitric oxide (NO), malondialdehyde (MDA), and erythrocyte antioxidant enzyme activities in newborn infants with hyperbilirubinemia. METHODS: Thirty term (gestational age > or = 37 weeks) newborn infants with indirect hyperbilirubinemia aged less than 10 days were prospectively recruited in the Kahramanmaras Sutcu Imam University Neonatal Unit, Kahramanmaras, Turkey, between January and July 2007. Thirty randomly selected healthy newborns who had similar age and without clinical jaundice comprised the control group. Erythrocyte catalase, superoxide dismutase, glutathione peroxidase (GPx), glucose-6-phosphate dehydrogenase, and plasma MDA and NO concentrations were measured. RESULTS: Both MDA and NO concentrations were higher in the study group than the controls (p=0.000). The mean activities of erythrocyte antioxidant enzymes were found to be lower in the study group compared with the controls (p=0.000). Furthermore, plasma bilirubin showed significant negative correlations with antioxidant enzyme activities but positive correlations with MDA and NO. CONCLUSION: In this sample, infants with significant hyperbilirubinemia had elevated oxidative stress and disturbed antioxidant enzyme activity. Since these states have been shown to cause cellular injury in neonatal patients with indirect hyperbilirubinemia, such patients should be followed-up and undergo therapy to prevent the harmful effects of hyperbilirubinemia. Further studies are needed to investigate possible benefits of antioxidants in hyperbilirubinemia.

Subacute sclerosing panencephalitis with generalized seizure as a first symptom: a case report.

Subacute sclerosing panencephalitis (SSPE) can show variations in its clinical course. Typical initial symptoms consist of intellectual decline, deterioration in school performance, and myoclonic jerks. Atypical forms of presentation such as generalized seizures and acute or subacute coma can be observed rarely. This report describes a 5-year-old boy with an atypical feature of SSPE, generalized seizures. For 1 month his only symptom was seizures, followed by personality and behavioral changes and myoclonic jerks. A diagnosis of SSPE was made based on the boy's history of measles, characteristic electroencephalogram changes and compatible magnetic resonance, and elevated anti-measles antibody titers in the cerebrospinal fluid and serum. The case presented in this article is a good example of SSPE in which, at early stages, some of the signs and symptoms can lead to an erroneous diagnosis.

Strain and strain rate echocardiography in children with Wilson's disease.

OBJECTIVE: This study aimed to evaluate strain and strain rate echocardiography in children with Wilson's disease to detect early cardiac dysfunction. METHODS: In this study, 21 patients with Wilson's disease and a control group of 20 age- and gender-matched healthy children were included. All the patients and the control group were evaluated with two-dimensional (2D) and colour-coded conventional transthoracic echocardiography by the same paediatric cardiologist using the same echocardiography machine (Vivid E9, GE Healthcare, Norway) in standard precordial positions, according to the American Society of Echocardiography recommendations. 2D strain and strain rate echocardiography were performed after the ECG probes of the echocardiography machine were adjusted for ECG monitoring. Longitudinal, transverse and radial strain, and strain rate were assessed from six basal and six mid-ventricular segments of the left ventricle, as recommended by the American Society of Echocardiography. RESULTS: Left ventricular wall thickness, systolic and diastolic diameters, left ventricular diameters normalised to body surface area, end-systolic and end-diastolic volumes, cardiac output and cardiac index values were within normal limits and statistically similar in the patient and control groups (p > 0.05). Global strain and strain rate: the patient group had a statistically significant lower peak A longitudinal velocity of the left basal point and peak E longitudinal velocity of the left basal (VAbasR) point, and higher global peak A longitudinal/circumferential strain rate (GSRa) compared to the corresponding values of the control group (p < 0.05). Radial strain and strain rate: end-systolic rotation [ROT (ES)] was statistically significantly lower in the patient group (p < 0.05). Longitudinal strain and strain rate: end-systolic longitudinal strain [SLSC (ES)] and positive peak transverse strain (STSR peak P) were statistically significantly lower in the patient group (p < 0.05). Segmental analysis showed that rotational strain measurement of the anterior and lateral segments of the patient group were statistically significantly lower than the corresponding values of the control group (p < 0.05). Segmental analysis showed statistically significantly lower values of endsystolic longitudinal strain [STSR (ES)] of the basal lateral (p < 0.05) and end-systolic longitudinal strain [SLSC (ES)] of the basal septal segment (p < 0.05) in the patient group. End-systolic longitudinal strain [SLSC (ES)] and positive peak transverse strain (STSR peak P) were statistically significantly lower in the patient group (p < 0.05). Segmental analysis showed statistically significantly lower values of endsystolic longitudinal strain [SLSC (ES)] of the mid-anterior and basal anterior segments (p < 0.05), end-systolic longitudinal strain [STSR (ES)] measurements of the posterior and mid-posterior segments, end-systolic longitudinal displacement [DLDC (ES)] of the basal posterior, mid-posterior and mid-antero-septal segments in the patient group. CONCLUSION: Cardiac arrhythmias, cardiomyopathy and sudden cardiac death are rare complications but may be seen in children with Wilson's disease due to copper accumulation in the heart tissue. Strain and strain rate echocardiography is a relatively new and useful echocardiographic technique to evaluate cardiac function and cardiac deformation abnormalities. Our study showed that despite normal systolic function, patients with Wilson's disease showed diastolic dysfunction and regional deformation abnormalities, especially rotational strain and strain rate abnormalities.