Evaluation of nutritional status and related factors in children with cystic fibrosis.

BACKGROUND: This study aimed to evaluate the nutritional status and body composition in children with cystic fibrosis (CF), in accordance with the new nutritional targets defined by European Society for Clinical Nutrition and Metabolism (ESPEN), the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the European Cystic Fibrosis Society (ECFS) 2016. METHODS: In this cross-sectional study, data were collected prospectively in a single centre. A record was made for a total of 95 patients with CF of clinical data. Anthropometric data were evaluated using the World Health Organization growth standards. The bone mineral density (BMD) z-score was adjusted for height by measuring dual-energy X-ray absorptiometry (DXA). The speed of sound z-score values were measured with quantitative ultrasound (QUS). RESULTS: The nutritional status was normal in 37.9% of patients aged < 2 years and 33.3% of patients aged 2-18 years. When the DXA BMD z-score values were corrected for height, it was determined that the BMD deficit was less. The calcaneus QUS SOS z-score mean value was lower than the mean height for age z-score adjusted BMD (BMDHAZ). CONCLUSIONS: The malnutrition rates of CF patients were higher than the rates previously reported in literature. As there are insufficient nutritional data in Turkey, there is a need for multi-centre studies to determine the frequency of malnutrition according to the new classifications. It is clear that QUS measurements cannot replace DXA in the diagnosis of osteopenic bone disease. However, when low values are determined with QUS as the first recommended measurement in the screening of bone status, it can be considered appropriate to confirm the status with DXA.

The Effect of Enteral Nutrition Support Rich in TGF-ß in the Treatment of Inflammatory Bowel Disease in Childhood.

Background and Objective: Malnutrition is a major complication of inflammatory bowel disease (IBD). Our aim of the study was to examine the effects of Modulen IBD supplementation, which was administered to IBD patients without limiting their daily diet in addition to medical treatment, on the clinical, laboratory, anthropometric values, and disease activities of these patients. Materials and Methods: Seventy three children with IBD were evaluated retrospectively. The cases were classified as those who had Crohn disease receiving (CD-M; n = 16) or not receiving Modulen IBD (CD; n = 19) and those who had ulcerative colitis receiving (UC-M; n = 13) or not receiving Modulen IBD (UC; n = 25). Disease activities, laboratory values, remission rates, and anthropometric measurements of the groups were compared. In addition to IBD treatment, Modulen IBD in which half of the daily calorie requirement was provided was given for eight weeks. Results: In the third month of treatment, 14 (88%) patients were in remission in CD-M group and eight (42%) patients were in remission in CD group. The height and weight z scores, which were low at the time of diagnosis, improved in the first week in CD-M group. Inflammatory parameters (UC) were significantly lower in the UC-M group compared to the UC group in first and third months. In the third month, eight (62%) patients in the UC-M group and four (16%) in the UC group were remitted clinically and in terms of laboratory values. Conclusions: TGF-ß-rich enteral nutrition support in children with IBD is an easy, effective, and reliable approach. It was shown that TGF-ß-rich enteral nutritional supplementation enabled the disease to enter the remission earlier, and contributed to the early recovery of weight and height scores.

Budd-chiari syndrome and renal arterial neurysms due to Behcet disease: a rare association.

Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm.

Inflammatory bowel disease in Turkish children.

BACKGROUND: This study was undertaken to evaluate demographics, clinical manifestations, laboratory findings and outcomes of children with inflammatory bowel disease (IBD) in Turkey. METHODS: We analyzed the medical records of 127 children diagnosed with IBD (under 18 years old) between January 2004 and January 2012 in 8 pediatric gastroenterology centers. RESULTS: Of the 127 patients, 90 (70.9%) suffered from ulcerative colitis (UC), 29 (22.8%) from Crohn's disease (CD), and 8 (6.3%) from IBD unclassified. The mean age of the 127 patients was 11.6 ± 4.1 years, and 11.8% of the patients were below 5 years old. Of the patients, 49.6% were male, and males were more predominant in patients with CD than in those with UC (72.4% vs. 42.2%, P = 0.008; a male/female ratio of 2.62 in CD, P = 0.0016). Approximately one fifth of the patients had extra-intestinal manifestations and 13.3% of the patients had associated diseases. Extraintestinal manifestations and associated diseases were more common in early onset disease [P = 0.017, odds ratio (OR) = 4.02; P = 0.03, OR = 4.1]. Of the patients, 15% had normal laboratory parameters including anemia, high platelet count, hypoalbuminemia, hypoferritinemia, and high sedimentation rate. Area under receiver operation characteristics was used to predict pancolitis in patients with UC. The values of C-reactive protein, sedimentation rate and pediatric ulcerative colitis activity were 0.61 (P = 0.06), 0.66 (P = 0.01) and 0.76 (P = 0.0001), respectively. Four (4.4%) patients with UC underwent colectomy, and finally two (1.5%, 95% confidence interval: 0-3.7%) patients died from primary disease or complications. CONCLUSIONS: IBD is an increasing clinical entity in Turkey. Features of IBD are similar to those in other populations, but prospective multicenter studies are needed to analyze the true incidence of IBD in Turkish children.

Essential thrombocythemia with Mpl W515 K mutation in a child presenting with Budd-Chiari syndrome.

Essential thrombocythemia (ET) is an extremely rare childhood disorder characterised by clonal expansion of megakaryocytic lineage in bone marrow, leading to a persistent increase in the number of circulating thrombocytes and thus increased risk for thrombotic and haemorrhagic events. The molecular mechanisms of ET are not fully understood. Most children with ET have the JAK2 V617F somatic mutation; however, another mutation, involving a W to L or K substitution at Mpl codon 515, was reported in a small proportion of adult ET patients that is extremely rare in children. Herein, we describe a Mpl W515K somatic mutation in a paediatric case of ET who presented with Budd-Chiari syndrome. No paediatric patient harbouring a Mpl W515K mutation has been previously reported.

Comparison of standard and standard plus vitamin E therapy for Helicobacter pylori eradications in children.

BACKGROUND/AIMS: Although various drugs can be used in adults for Helicobacter pylori eradication in adults, treatment options are limited in children. The aim of this study was to compare the effects of the standard lansoprazole, amoxicillin, and clarithromycin (LAC) protocol to those of LAC+vitamin E (LACE) combination for H. pylori eradication. MATERIALS AND METHODS: The study included 90 children (age range: 10-17 years) who were admitted to four pediatric gastroenterology centers between March 2011 and November 2012 with dyspeptic symptoms and who had tested positive for H. pylori by 14C-urea breath tests. The patients were randomized into two groups. The LAC group [45 patients (pts)] was treated with a standard regimen consisting of lansoprazole (1 mg/kg/day), amoxicillin (50 mg/kg/day), and clarithromycin (14 mg/kg/day), each of which was given in two equally divided doses every 12 h for 14 days; the LACE group (45 pts) was given the standard regimen and vitamin E at 200 IU/day for 14 days. H. pylori eradication was assessed using the 14C-UBT in the 6th week after the cessation of treatment. RESULTS: H. pylori was eradicated in 21 (46.6%) pts in the LAC group, while it was eradicated in 29 (64.4%) pts in the LACE group. There was no statistical difference between the two groups (p=0.13). CONCLUSION: The eradication rate of H. pylori in children while using the LAC regimen has decreased in the last years. The LACE regimen has been associated with an increased eradication rate but can reach to statistically significance. Further studies with larger cohorts are needed to examine the success of the LACE regimen for H. pylori eradication.

An unexpected cause of pleural effusion in paediatric emergency medicine.

Pancreaticopleural fistula (PPF) is an uncommon complication of chronic pancreatitis leading to a large and recurrent pleural effusion. Since the patients presented predominantly with respiratory symptoms, diagnosis and treatment were often delayed. We describe a child who was admitted to our paediatric emergency department with an acute onset of dyspnoea and unilateral massive pleural effusion caused by PPF. Multidetector CT is an easily accessible method that is able to show both the thoracic and abdominal findings non-invasively. The clinical and imaging features of this unusual entity are discussed.

Liver involvement in children with Familial Mediterranean fever.

AIM: Familial Mediterranean fever is characterised by recurrent, febrile, inflammatory attacks of the serosal membranes. Prolonged inflammatory response is triggered secondary to cytokine stimulation due to reduced activity of pyrin. Inflammatory cytokines play major role in the pathogenesis of acute liver injury; and chronic, recurrent cytokine production may cause chronic hepatitis/cirrhosis. We aimed to analyse liver involvement in children with Familial Mediterranean fever. PATIENTS: The study included 58 patients with Familial Mediterranean fever. Patients with liver involvement were examined in detail. RESULTS: Liver involvement was seen in 11 of 58 patients (18.9%). Two patients (3.4%) had abnormal liver enzymes during the diagnostic evaluation, whilst 9 patients (15.5%) were admitted with the features of liver diseases, and had final diagnosis of Familial Mediterranean fever (2 had Budd-Chiari syndrome, 5 had chronic hepatitis/cirrhosis, 2 had acute hepatitis). None of the demographic factors or laboratory findings was different between the patients with or without liver involvement M694V allele was more common in patients with liver involvement but did not reach significant difference (50% vs. 33.6%, p=0.21). All the patients showed clinical and laboratory improvement after colchicine. CONCLUSION: Paediatric hepatologists must keep Familial Mediterranean fever in mind in the patients with cryptogenic hepatitis/cirrhosis especially in regions where hereditary inflammatory diseases are common.

Peptic ulcer disease in children: an uncommon disorder with subtle symptomatology.

BACKGROUND/AIMS: Data concerning peptic and infectious ulcers in children are limited. The aim of the study was to investigate the prevalence, presenting symptoms and significance of symptomatology in ulcer diagnosis in the pediatric age group. MATERIALS AND METHODS: Between January 2000 and 2009, upper gastrointestinal endoscopy charts were examined retrospectively. All children in whom a diagnosis of ulcer was established were included in the study. Demographic, clinical, endoscopic, and histopathologic data were obtained from the patients' records. Peptic ulcer disease prevalence, presenting symptoms and symptomatology were evaluated. RESULTS: Ulcer disease was observed in 31 (3.4%) of 902 patients. The mean age was 10.85 ± 4.25 (range: 2-17 years), and the male to female ratio was 2:1. The most common symptom was chronic abdominal pain (68%), hematemesis and melena (55%) and vomiting (39%). Helicobacter pylori was identified in 19 patients (61%) with ulcer. In the Helicobacter pylori-positive group, upper intestinal bleeding and pain were the major symptoms. Symptom frequency was not different between Helicobacter pylori-positive and -negative patients (p>0.05). CONCLUSIONS: Ulcer disease is an uncommon disorder in children with nonspecific clinical symptoms. Unlike the adult population, symptoms fail to diagnose peptic ulcer disease before gastrointestinal bleeding occurs.

Late-onset graft-versus-host disease after pediatric living-related liver transplantation for Langerhans cell histiocytosis.

GVHD is the most common and well-known cause of morbidity and mortality following allogeneic BM transplantation. The GVHD following OLT is an uncommon complication but has a high mortality and poses a major diagnostic and therapeutic challenge. We herein discussed a 12-month-old girl with multi-system LCH, who developed end-stage liver disease despite intensive chemotherapy. She underwent ABO-compatible liver transplantation at 28 months while in remission from LCH. The donor was her 26-yr-old father. Post-operative course was uneventful. The GVHD manifested with skin rash and BM suppression on post-transplant day 94 and confirmed by both microchimerism and skin biopsy. Prednisolone, basiliximab, and ATG were administered immediately but the bone marrow suppression was not improved and the patient died because of Candida sepsis at six-month post-transplant. GVHD after OLT should be keep in mind in patients with rash and BM suppression after liver transplantation. In LDLT, a patient who carries risk factors should investigated for optimal HLA matching.

Evaluation of growth after liver transplantation in Turkish children.

AIMS: Currently, the main interest in childhood liver transplantation (LT) is to prevent long-term complications and optimize growth. The aim of this study is to analyze (1) nutritional status in the pretransplantation period, and (2) posttransplantation growth and associated factors in children. PATIENTS AND METHODS: Eighty children were included in the study. Height (Z (H)) and weight (Z (W)) Z scores were calculated before transplantation and postoperatively at the 6th month and 1st, 2nd, 3rd, 4th, and 5th year. RESULTS: Patients' Z (H) and Z (W) scores at LT were -1.6 ± 1.3 and -1.5 ± 1.4, respectively. Both Z (H) and Z (W) scores increased after LT, especially in the first 6 months, and then continued to rise gradually. Both reached beyond -1 Z score at 2nd year and -0.5 at 4th year. Age, primary diagnosis, total steroid dose (<1,000 mg), and absence of rejection episodes had positive impact on posttransplantation growth, whereas gender, immunosuppression type, surgical complications, and presence of tumor had no impact on posttransplantation growth. Age at time of LT was negatively correlated with Z (W) score at 5th year (P = 0.02, r = -0.43). Both Z (W) and Z (H) scores at time of LT were positively correlated with Z (W) and Z (H) scores and negatively correlated with ∆Z (W) and ∆Z (H) scores at 5th year. CONCLUSIONS: LT is not only a modern, life-saving treatment technique but also an efficient method of facilitating growth, an indispensable component of childhood and the best indicator of health.

Therapeutic management of neonatal hemochromatosis: report of four cases and literature review.

Neonatal hemochromatosis (NH) is a rare disease of iron metabolism that starts at intrauterine period causing liver failure and extrahepatic siderozis. The etiology of NH has not been understood exactly, yet it is accepted that a maternofetal alloimmune disorder that leads to liver failure in fetus causes the illness. The prognosis of NH is generally bad and death is inevitable if left untreated. The efficiency of chelation-antioxidant coctail used in medical treatment is between 10% and 20% and these patients frequently need liver transplantation. In our study, we presented four newborn cases diagnosed as NH and treated medically. Of the four patients, one died of pulmonary hemorrhage and another died of multiorgan failure in the first week of hospitalization. The other two patients' clinical status and laboratory parameters recovered with medical treatment. However, since liver transplantation was not carried out, one of these patients died at the age of two and a half months and the other at eighth month due to sepsis. In this study, we would like to emphasize the importance of early liver transplantation in patients recovered with medical treatment.

Intestinal involvement in metachromatic leukodystrophy.

Metachromatic leukodystrophy is a lysosomal storage disease caused by the deficiency of the enzyme arylsulfatase A. If arylsulfatase A is deficient, sulfatide accumulates. Functionally, this accumulation results in progressive neurological deterioration. The reports about the extra nervous system manifestations of metachromatic leukodystrophy are related to the gallbladder involvement such as polyposis. Unexplained vomiting began in a 5½-year-old girl with late infantile metachromatic leukodystrophy. Endoscopy showed multiple polypoid masses in the pylor of the stomach and duodenum. Severe gastrointestinal bleedings occurred during nasogastric feeding. Intestinal intussusception developed later. To the authors' knowledge, intestinal polypoid masses and obstruction with metachromatic leukodystrophy have not previously been reported. The persistent vomiting may be a symptom of intestinal obstruction due to intestinal polypoid masses with metachromatic leukodystrophy. There may be a trend for the development of polypoid masses in intestine as well as in the gallbladder in metachromatic leukodystrophy.

Foreign body ingestion in Turkish children.

Foreign body ingestion (FBI) is a common problem in the pediatric population. Even though morbidity and mortality due to foreign body ingestion are rare in childhood, they may cause serious anxiety in parents. We aimed to analyze the clinical presentation, etiology and management strategy of FBI in children in our country. Records of children admitting with a history of FBI over a three-year period were reviewed retrospectively. Data regarding gender, age, type of the ingested body, management strategy and outcome of the patients were recorded. Of 176 children, 98 (55.6%) were male. Mean age +/- SD of the patients was 3.75 +/- 4.25 years, and most of the patients were below four years of age (71.5%). Most of the children (64.7%) were seen within 48 hours, and most were asymptomatic. Blue beads attached to a safety pin (a cultural good luck charm) (38.6%), coins (27.8%) and turban pins (18.1%) were the most commonly observed foreign bodies. The blue beads/safety pin were found to be ingested primarily by infants, while ingestion of turban pins was mostly seen in adolescent girls who covered their heads. Localization of the foreign bodies was in the distal small intestine, stomach and esophagus in 61.4%, 23.8% and 14.7% of the cases, respectively. Sixty-nine endoscopic interventions were performed in 61 patients (34.6%), and these accounted for 7.3% of all endoscopic interventions during the three-year period. No major complication was observed during the procedure, and none of the patients underwent surgery. The frequently used accessory devices were retrieval net basket (57.9%), snare for pins (17.3%), tripod forceps and rat-tooth forceps. The blue beads/safety pin and turban pin were the commonly ingested foreign bodies in our center due to cultural factors. Education of the parents and of adolescent girls should greatly reduce the incidence of FBI. Endoscopic removal is safe without any major complications.

Chronic constipation in Turkish children: clinical findings and applicability of classification criteria.

We aimed to evaluate general features of children with chronic constipation and classified them according to the Iowa criteria and Rome II criteria in order to analyze applicability of these criteria in our population. The medical records of 485 children who were referred for chronic constipation over a six-year period were evaluated retrospectively. We found that 7.7% of the cases had an organic pathology, and short segment Hirschsprung disease was the leading cause. Other children (92.3%) were classified as functional constipation, with a mean age of 6.4 +/- 4 years and with slight male dominance. Encopresis was found in 117 children (51.7%) aged over four years, and was associated with older age, male predominance and long duration of symptoms. Both of the classification systems showed a similar prevalence of constipation, but 9.9% of the children with pediatric constipation were not recognized by Rome II criteria. Additionally, 1.8% of the children were not recognized by either Iowa or Rome criteria. Functional constipation is common in primary care, and most of the children were school-aged. Constipation associated with encopresis and nutritional problems such as obesity is less common in developing countries. Rome II criteria are too restrictive and do not recognize approximately 12% of the children. A new classification system must be simple, easy to understand especially by the primary care physician, and must include the common features of constipation recognized by the parents.

Liver transplantation for acute liver failure due to toxic agent ingestion in children.

ALF is characterized by sudden onset, impaired liver function, jaundice and encephalopathy, without previous liver disease. We analyzed the patients who underwent LT due to toxic agent induced ALF to raise community awareness about preventing the toxic agent induced ALF. Five children (three boys, two girls) underwent LT due to toxic agent ingestion. Toxic agents were mushroom poisoning (n = 2), Datura stramonium (n = 1), yellow phosphorous (n = 1) and INH (n = 1). On admission, one patient had stage IV, two had stage III and two had stage II hepatic encephalopathy but worsened during the follow-up. One patient had renal failure, and three patients required mechanical ventilation. Three patients underwent LRLT and others from a DD. Post-operative complications were managed by supportive managements successfully, and overall all the patients are alive (100% survival) without any organ sequelae. Although outcome of these patients are excellent, ALF may be prevented in these cases by educating the public about consuming mushrooms and toxic effects of wild plants, prohibiting fireworks and serial liver enzyme measurements after initiating INH.

Long-term effect of gluten-free diet on growth velocity in Turkish children with celiac disease.

We sought to analyze the long-term growth in children with celiac disease (CD) as well as the association of height and weight SD score at the end of 4 years with the demographic and clinical presentation at initial admission. Thirty-four children with CD were enrolled in the study and followed for at least 4 years. Patients were divided into three groups (Group 1: patients 10 years, at the time of diagnosis). Patients' charts were reviewed for demographic and clinical features at initial admission. Anthropometric measurements at initial admission, at 6th months, and 1st, 2nd, 3rd, and 4th years were recorded. Gluten-free diet led to rapid increase in weight SD and height SD score in patients

Helicobacter pylori infection in children with celiac disease.

OBJECTIVE: To analyze the prevalence of Helicobacter pylori (H. pylori) infection in children with celiac disease (CD) and to examine the role of H. pylori infection in clinical, laboratory and histopathological presentations of CD. MATERIAL AND METHODS: Data on 96 children with CD and 235 children who underwent endoscopy were compared for the prevalence and gastric histology pattern of H. pylori. Clinical presentation, laboratory and histological findings of CD children with and without H. pylori infection were compared. RESULTS: Twenty-one subjects (21.8%) in the CD group and 56 subjects (23.8%) in the control group had H. pylori gastritis. Gastric metaplasia is higher in CD patients with H. pylori gastritis (19%) than in patients without H. pylori gastritis (1.3%) and in the control group (3.5%) (p<0.05 for all groups). Abdominal distension is more common at initial admission in CD patients with H. pylori gastritis (57.1% versus 14.6%, p<0.05). No significant difference was found between H. pylori (+) and (-) CD patients in terms of prevalence of anemia, iron deficiency and iron-deficiency anemia. Only mild duodenal histological findings were more common in H. pylori patients (57.1% versus 26.7%, p<0.05). CONCLUSIONS: CD may be associated with H. pylori gastritis, but it does not affect the clinical presentation of the disease, except for abdominal distension; CD is associated with mild duodenal lesions. A gluten-free diet improves the symptoms in all patients independently of the presence of H. pylori gastritis. Gastric metaplasia increases in the presence of H. pylori gastritis. Further prospective studies are needed to examine the clinical and histopathological outcomes of gastric metaplasia associated with H. pylori gastritis in CD patients.