Portal Hypertension in Children: A Tertiary Center Experience in Turkey.

Purpose: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. Methods: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed. Results: The most common causes of PH were portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%). Among the enrolled patients, 131 (59.0%) were included in the cirrhotic group and 91 (41.0%) in the non-cirrhotic group. Hepatomegaly and increased transaminase levels were more frequent in the cirrhotic group than in the non-cirrhotic group. Additionally, portal gastropathy, esophageal varices, and variceal bleeding were more frequent in the non-cirrhotic group, whereas ascites, hepatopulmonary syndrome and hepatic encephalopathy were more common in the cirrhotic group. The incidence of hepatomegaly was higher in the presinusoidal group than in the prehepatic group (p<0.001). Hyperbilirubinemia was more frequent in the prehepatic group (p=0.046). The frequency of esophageal varices was similar between the prehepatic and presinusoidal groups; however, variceal bleeding was more frequent in the prehepatic group (p=0.002). Conclusion: Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia were the most prevalent causes of PH in our country. In patients with PH, hepatomegaly, increased transaminase levels, and synthesis dysfunction were suggestive of cirrhotic PH. Notably, PH in patients without cirrhosis might be more severe than that in those with cirrhosis.

Food-induced immediate response of the esophagus in pediatric eosinophilic esophagitis.

BACKGROUND: Food-induced immediate response of the esophagus (FIRE) is a new phenomenon that has been described in eosinophilic esophagitis (EoE) patients. It is suspected when unpleasant symptoms occur suddenly on contact of the triggering food with the esophageal surface and recur with repeated exposures. It can often be mistaken for pollen-food allergy syndrome (PFAS) and solid food dysphagia. Data on FIRE is limited to one survey study and case reports, and there are no screening studies conducted on either adults or children with EoE. In this study, we aimed to screen children aged ≥7 years old with EoE for FIRE. METHODS: Demographic data were collected from medical records. A questionnaire about FIRE was applied to all participants. Skin prick tests were done on suspected patients to identify the triggering foods. FIRE is defined as suitable clinical symptoms with suspected food allergen exposure. RESULTS: A total of 78 patients (74.4% male, median age: 13.5 years) were included. Unpleasant and recurrent symptoms distinct from dysphagia with specific foods were reported in 16.7% of the patients, all of whom had concomitant allergic rhinitis (AR). The symptoms described by almost all patients were oropharyngeal itching and tingling (PFAS: 15.3%) excluding only one patient reporting retrosternal narrowing and pressure after specific food consumption (FIRE: 1.2%). CONCLUSIONS: Although definitive conclusions regarding the true prevalence of FIRE cannot be made, it does not seem to be common as PFAS. However, it deserves questioning particularly in the presence of concurrent AR and/or PFAS in children with EoE.

Oropharyngeal Dysphagia in Children with Eosinophilic Esophagitis.

Dysphagia is the most troublesome symptom of eosinophilic esophagitis (EoE). This study aimed to investigate oropharyngeal dysphagia in children with EoE and possible related factors. Children with a definite diagnosis of EoE were included in the study. Medical and feeding histories were recorded. A disease control level was determined for each child. An oral structure examination, the Turkish version of the Mastication and Observation Evaluation (T-MOE), the Pediatric version of the Eating Assessment Tool-10 (PEDI-EAT-10) and the 3-oz water swallow test were applied in screening for oropharyngeal dysphagia. Fifty-two children participated in the study. Oropharyngeal dysphagia took the form of abnormal swallowing (PEDI-EAT-10 score ≥ 4) and increased aspiration risk (PEDI- EAT-10 score ≥ 13) in 51.9% and 25.0% of the children, respectively. Seven children failed the 3-oz water swallow test. Abnormal swallowing and aspiration risk were significantly higher in children with prolonged mealtimes, impaired chewing function, and uncontrolled disease (p < 0.05). Chewing function was the most important risk factor for abnormal swallowing and increased aspiration (R2 = 0.36, R2 = 0.52, p < 0.001, respectively). Oropharyngeal dysphagia is common in children with EoE and associated with increased aspiration risk in a subpopulation. Uncontrolled disease, prolonged mealtimes, and impaired chewing function may provide clues for oropharyngeal dysphagia in EoE.

A possibly new autoinflammatory disease due to compound heterozygous phosphomevalonate kinase gene mutation.

BACKGROUND: Mevalonate kinase (MVK) plays a role in cholesterol and non-sterol isoprenoid biosynthesis and its deficiency-related diseases are caused by bi-allelic pathogenic mutations in the MVK gene, (MVK), which leads to rare hereditary autoinflammatory diseases. The disease may manifest different clinical phenotypes depending on the degree of the deficiency in the enzyme activity. The complete deficiency of the enzyme activity results in the severe metabolic disease called mevalonic aciduria, while a partial deficiency results in a broad spectrum of clinical presentations called hyper-immunoglobulin D syndrome (HIDS). Serum immunoglobulin (Ig) D and urine mevalonic acid levels may be increased during inflammatory attacks of HIDS. CASE PRESENTATION: Herein, for the first time in the literature, we present a 6-year-old male patient who suffered from recurrent episodes of fever, polyarthritis, skin rash, diarrhea, abdominal pain, and inflammatory bowel disease-like manifestations with elevated levels of serum IgD, and urine mevalonic acid. Eventually we detected compound heterozygous mutations in the phosphomevalonate kinase (PMVK) gene coding the second enzyme after mevalonate kinase in the mevalonate pathway. CONCLUSION: For patients presenting with HIDS-like findings, disease exacerbations and persistent chronic inflammation, and having high urinary mevalonic acid and serum IgD levels, raising suspicion in terms of MVK deficiency (MVKD), it is recommended to study all mevalonate pathway enzymes, even if there is no mutation in the MVK gene. It should be kept in mind that novel mutations might be seen such as PMVK gene.

Long-Term Outcomes of Patients With Progressive Familial Intrahepatic Cholestasis After Biliary Diversion.

OBJECTIVES: Progressive familial intrahepatic cholestasis is a heterogeneous group of genetic disorders characterized by disrupted bile homeostasis. Patients with this disease typically present with cholestasis and pruritus early in life and often progress to end-stage liver disease. The clinical symptoms that patients with progressive familial intrahepatic cholestasis encounter are usually refractory to medical treatment. Although the effects of biliary diversion surgery on native liver survival are not exactly known, this procedure may provide a positive impact on pruritus and laboratory parameters in these patients. MATERIALS AND METHODS: We retrospectively evaluated the clinical and laboratory characteristics of patients with progressive familial intrahepatic cholestasis who underwent partial external biliary diversion between 2002 and 2020 at our center. Diagnosis of progressive familial intrahepatic cholestasis was made by clinical, biochemical, and histopathological characteristics as well as genetic testing. RESULTS: Nine patients were included in the study. Five patients required liver transplant during follow-up, with 4 having liver transplant as a result of endstage liver disease (median interval of 5 years). In 1 patient, partial external biliary diversion was performed 1.5 years after liver transplant for severe diarrhea, metabolic acidosis, and hepatic steatosis. Four patients did not require liver transplant during follow-up (median follow-up time of 7.6 years). Pruritus responded well to partial external biliary diversion in all patients. Among laboratory values evaluated 6 months after biliary diversion, only albumin showed significant improvement. CONCLUSIONS: Partial external biliary diversion had favorable results on long-term follow-up. This procedure can provide the relief of pruritus and delay the requirement for liver transplant in patients with progressive familial intrahepatic cholestasis. In our view, partial external biliary diversion should be considered the first-line surgical management for patients with this disease.

Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1.

Progressive familial intrahepatic cholestasis is a heterogeneous group of autosomal recessive disorders, and liver transplant is the only curative treatment. A biliary diversion operation for disruption of enterohepatic circulation in patients with progressive familial intrahepatic cholestasis type 1 without cirrhosis is another option. We present a pediatric patient with progressive familial intrahepatic cholestasis type 1 who underwent liver transplant due to end-stage liver disease. After transplant, diarrhea and growth retardation complications resolved after partial external biliary diversion surgery.

Two Cases With Neonatal Cholestasis and Renal Disorders Due to DCDC2 Mutation.

Ciliopathies are a heterogeneous group of diseases that are observed after deterioration of the ciliary structures on the cell surface that facilitate communication with the environment. Both liver and kidney involvement are frequently observed in this disease. Recently, a doublecortin domain containing protein 2 (DCDC2) mutation in a ciliopathy disease group was identified. Here, we present 2 patients with this mutation and with neonatal cholestasis and renal involvement.

Expected or unexpected clinical findings in liver glycogen storage disease type IX: distinct clinical and molecular variability.

OBJECTIVES: To reveal the different clinical presentations of liver glycogen storage disease type IX (GSD IX), which is a clinically and genetically heterogeneous type of glycogenosis. METHODS: The data from the electronic hospital records of 25 patients diagnosed with liver GSD IX was reviewed. Symptoms, clinical findings, and laboratory and molecular analysis were assessed. RESULTS: Of the patients, 10 had complaints of short stature in the initial presentation additionally other clinical findings. Elevated serum transaminases were found in 20 patients, and hepatomegaly was found in 22 patients. Interestingly, three patients were referred due to neurodevelopmental delay and hypotonia, while one was referred for only autism. One patient who presented with neurodevelopmental delay developed hepatomegaly and elevated transaminases during the disease later on. Three of the patients had low hemoglobin A1C and fructosamine values that were near the lowest reference range. Two patients had left ventricular hypertrophy. Three patients developed osteopenia during follow-up, and one patient had osteoporosis after puberty. The most common gene variant, PHKA2, was observed in 16 patients, 10 variants were novel and six variants were defined before. Six patients had variants in PHKG2, two variants were not defined before and four variants were defined before. PHKB variants were found in three patients. One patient had two novel splice site mutations in trans position. It was revealed that one novel homozygous variant and one defined homozygous variant were found in PHKB. CONCLUSIONS: This study revealed that GSD IX may present with only hypotonia and neurodevelopmental delay without liver involvement in the early infantile period. It should be emphasized that although liver GSDIX is thought of as a benign disease, it might present with multisystemic involvement and patients should be screened with echocardiography, bone mineral densitometry, and psychometric evaluation.

Reliability and Validity Study of Turkish Version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0) Led to Development of a New Pediatric Eosinophilic Esophagitis Scale: GaziESAS.

BACKGROUND/AIMS: There has been no valid and reliable Turkish scale that measures symptoms in children with eosinohilic esophagitis (EoE). The aim of the study is test the validity and reliability of Turkish version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0) Materials and Methods: Relevant forms of Tr-PEESS v2.0 were applied to 2-18 years old children with EoE and to their parents. KINDL QoL patient and parent questionnaires and GaziESAS scale which was developed in this study were used to test convergent validity of Tr-PEESS v2.0. Discriminant validity was evaluated among three EoE treatment groups: under treatment, off treatment due to remission and uncompliant with treatment. Reliability was evaluated by internal consistency, test-retest reliability, and item analysis. RESULTS: Fiftytwo children/teens (mean age 130.2±60.3 months) and 84 parents were interviewed twice one week apart. Mean duration of EoE was 47.2±35.6 months. Tr-PEESS v2.0 reports correlated with GaziESAS (range,0.361-0.855) and KINDL QoL questionnaires (range,-0.316-0.413). Parent report of Tr-PESS v2.0 discriminated children uncompliant with treatment from the ones off treatment and undertreatment. Cronbach's α values and intraclass correlation coeffcients (ICC) values of Tr-PEESS v2.0 ranged from 0.614 to 0. 895 and 0.646 to 0.910, respectively. CONCLUSION: Tr-PEESS v2.0 is a valid and reliable tool to use in Turkish children. GaziESAS is a new parent-proxy pediatric EoE scale with additional adaptive behaviour domain that passed scale developmental stages successfully for Turkish children with EoE.

Primary Eosinophilic Gastrointestinal Diseases Beyond Eosinophilic Esophagitis in Children.

OBJECTIVES: There are many unknowns about primary eosinophilic gastrointestinal disease (EGID) in childhood. The aim of this study is to provide data about the frequency, management, control level, and prognosis of well documented primary EGID in childhood. METHODS: This study was conducted in children who underwent endoscopy and/or colonoscopy at a single center over 10-year period up to August 2018. Primary EGID was diagnosed after exclusion of secondary EGID and classified as eosinophilic gastritis (EG), eosinophilic enteritis (EE), eosinophilic gastroenteritis (EGE: eosinophilic gastritis with eosinophilic enteritis) and eosinophilic colitis (EC) according to histopathological evaluation. The pathological number of eosinophil counts were accepted as >30 hpf for gastric mucosa in 5 hpf area, ≥20/hpf for duodenal, jejunal, and ileal mucosa, >50/hpf for right colonic mucosa, >35/hpf for transverse colonic mucosa, and >25/hpf for left colonic mucosa. Presenting symptoms, signs, management, follow-up, disease control level, and remission were analyzed. Remission is defined if the patient is controlled with all clinical, endoscopic/colonoscopic, and histopathologic parameters without any treatments or diet for at least a year. RESULTS: During the study period, 7457 biopsies were taken in 8262 endoscopy and/or colonoscopy procedures. Primary and secondary EGID frequencies were found 0.23% (n = 17 patients) and 0.1% (n =8 patients) per procedure with biopsy in children, respectively. Endoscopy/colonoscopy procedures were not able to performed in 9 patients because of short follow-up period (n = 6) or patients leaving follow-up (n = 3). Nine of the primary EGID patients had esophageal eosinophilia (EsE) at the time of diagnosis, 5 of them were previously managed as EoE. The median follow-up period of primary EGID patients excluding the ones without a control endoscopy/colonoscopy procedure was 3.35 years (min-max: 1.1-9.0 years). Proton pump inhibitors (PPI) were the most frequently used treatment alone or in combination with diet, systemic and/or topical corticosteroids. Disease control was evaluated in 8 of 17 patients and it was uncontrolled in 4, partially controlled in 1, and controlled in 3 patients. Remission was achieved in 2 patients. CONCLUSIONS: The frequency of primary EGID beyond eosinophilic esophagitis (EoE) in children is low. It may be difficult to achieve control in children with primary EGID in the long-term follow-up.

Predictable and Unusual Adverse Effects of Immunosuppression in Pediatric Liver Transplant Patients.

OBJECTIVES: Our aim was to determine potentially adverse effects of immunosuppressive protocols after liver transplantation in children. MATERIALS AND METHODS: The medical records of 60 children who underwent liver transplant retrospectively analyzed. Corticosteroid, tacrolimus, and mycophenolate mofetil were the primary immunosuppressive agents used in our center. RESULTS: The mean age of children was 6.1 years, ranging from 3 months to 17 years (34 boys, 26 girls). The most common indication for liver transplant was biliary atresia (26.7%). Thirty-nine patients (65%) received livers from living donors, and 21 patients (35%) received from livers from deceased donors. The main complications of immunosuppressive therapy were diarrhea associated with mycophenolate mofetil, hyperglycemia and hypertension associated with corticosteroid, and seizures and tremors associated with tacrolimus. Two patients developed post transplant lymphoproliferative disorder. The diagnosis was based on histologic findings of cervical lymphadenopathy and duodenal biopsy. One patient was diagnosed with acute lymphoblastic lymphoma. In addition to these predictable adverse effects, unusual adverse effects of immunosuppression were also observed. Hemolytic anemia (n = 3) (one was also diagnosed with Evans syndrome), eosinophilic gastroenteritis (n = 2), de novo food allergy (n = 2), posttransplant lymphoproliferative disorder (n = 2), Burkitt lymphoma (n = 1), and renal tubular acidosis (n = 1) were thought to be related to tacrolimus therapy. CONCLUSIONS: Adverse effects of immunosuppression represent a major cause of postoperative morbidity. The common effects of immunosuppression are recognized easily by clinicians. It should be kept in mind that unexpected symptoms and signs may be related to immunosuppression in pediatric liver transplant patients.

The effect of Helicobacter pylori eradication on functional dyspepsia in Turkish children.

BACKGROUND: The aim of this study was to evaluate the effect of Helicobacter pylori (H. pylori) eradication on dyspepsia symptom scores in children with functional dyspepsia (FD). MATERIALS AND METHODS: One hundred and fifty functional dyspeptic children (ages 8-18 years, mean: 13.3 ± 2.84 years; 30% male) were enrolled to this prospective study. Upper gastrointestinal endoscopy was performed on all patients, and the samples from the gastric antrum and corpus were obtained for the existence of H. pylori. 13 Carbon-urea breath test was performed to evaluate the eradication therapy's efficacy. The symptoms were assessed at first visit and at the 8th week and 16th week. RESULTS: Forty-nine (33%) children were in the H. pylori-positive group, and 101 (67%) children were in the H. pylori-negative group. Dyspepsia symptom scores improved at 8th week in both groups (P < .05). Helicobacter pylori was eradicated in 30 patients (61%), while in the H. pylori-eradicated group, all dyspepsia symptoms' scores decreased, and in the H. pylori-uneradicated group, only three symptoms' scores decreased. Symptom scores were lower in H. pylori-eradicated group than H. pylori-uneradicated group. CONCLUSIONS: Although the tests used for the diagnosis of H. pylori in functional dyspeptic patients increased the cost of health care, the dyspepsia symptom scores decreased with the eradication therapy in a high prevalence community. The findings may differ in low prevalence communities where the diagnostic tests for H. pylori infection are not recommended in children in the absence of alarm signs or symptoms.

Differential diagnosis in ulcerative colitis in an adolescent: Chronic granulomatous disease needs extra attention.

Chronic granulomatous disease (CGD) is a primary immune deficiency that is commonly diagnosed under the age of 5 years (95%) and is rarely seen in adulthood. CGD may manifest as inflammatory bowel disease (IBD) in childhood. Without proper diagnosis, these patients may be monitored for years as IBD; some may even be regarded as steroid-resistant ulcerative colitis (UC) and end up having a colectomy. In this case report, we described a patient who had been followed-up for years as UC and subsequently underwent colectomy, but was finally diagnosed in adulthood as primary immune deficiency.

Iron Overload in the Liver of 2 Children: Nonalcoholic Steatohepatitis and Juvenile Hemochromatosis.

BACKGROUND: Iron overload disorders are hereditary hemochromatosis and secondary etiologies other than hereditary hemochromatosis. We describe 2 boys presenting with iron overload. Juvenile hemochromatosis and nonalcoholic steatohepatitis (NASH) related iron overload are the genetic and secondary causes, respectively. OBSERVATIONS: Both patients benefited from phlebotomy even if they had different etiologies. CONCLUSIONS: In childhood, the diagnosis of iron overload syndromes is crucial because they do not confront us with obvious symptoms and findings. Early initiation of a phlebotomy program can prevent mortality. NASH might lead to iron overload and iron overload might aggravate the clinical course of NASH.

Favorable Response to Sirolimus in a Child With Blue Rubber Bleb Nevus Syndrome in the Gastrointestinal Tract.

Recently, sirolimus was demonstrated to be effective in treating vascular lesions and lessening the frequency of bleeding and secondary iron deficiency anemia. We present a child with blue rubber bleb nevus syndrome who had prolonged history of iron deficiency anemia secondary to unrecognized gastrointestinal bleeding. Treatment with propranolol, omeprazole and iron had failed. After 2.5 months of sirolimus therapy (trough levels 1 to 5 ng/mL), his hemoglobin concentration improved into the normal range and remained stable. Vascular malformations on both the patient's tongue and in the fundus of his stomach shrank within 5 months of the initiation of sirolimus. In gastrointestinal involvement of blue rubber bleb nevus syndrome sirolimus was found to be effective even in the tongue's vascular lesions.

Mutant neurogenin-3 in a Turkish boy with congenital malabsorptive diarrhea.

Congenital diarrheal disorders are caused by disruption in nutrient digestion, absorption, or transport, enterocyte development and functioning, or enteroendocrine functioning. Many additional rare forms of congenital diarrhea are expected to be linked to genes associated with appropriate intestinal fluid and electrolyte balance. Neurogenin-3 mutation, a very rare form of congenital diarrhea, disrupts enteroendocrine cell differentiation and is characterized by malabsorption and the absence of pancreatic islet cells. Diabetes mellitus is typically associated with malabsorptive diarrhea at early onset or at later presentation in neurogenin-3 mutation. Here, we describe the case of an infant with homozygous neurogenin-3 mutation who had severe malabsorptive diarrhea and episodes of hyperchloremic metabolic acidosis after birth. Remarkably, cholestyramine was effective at reducing stool volume and frequency and improved the consistency of the stools; diabetes was not present in this patient.

Chanarin-Dorfman syndrome: a novel mutation in a Turkish girl.

Chanarin-Dorfman syndrome (CDS) is an autosomal recessive disorder, characterized by intracellular accumulation of lipid droplets in most tissues. It is very difficult to find a correlation between the phenotypic and genotypic features due to the occurrence of novel ABHD5 [ α/ß hydrolase domain-containing protein-5; originally called CGI-58 (comparative gene identification-58)] mutations and the fact that there are only a few cases in the literature. The protein encoded by this gene is a cofactor for adipose triglyceride lipase (ATGL), which promotes the catabolism of stored fat. The clinical phenotype involves multiple organs and systems. Ichthyosis, nonbullous congenital ichthyosiform erythroderma and cytoplasmic accumulation of lipid droplets in granulocytes (Jordans' bodies) are always present. Peripheral blood smear is an easy method for diagnosing CDS; its use can also avoid unnecessary further testing. Herein, we report a patient with a homozygous mutation in ABHD5 that has never previously been described. Moreover, the case was diagnosed as Chanarin-Dorfman syndrome with only a peripheral blood smear.

Rare disorders can be an underlying cause of cyclic vomiting: Familial Mediterranean fever, Helicobacter pylori gastritis, and cavernous transformation of the portal vein.

BACKGROUND/AIMS: Considering the etiology of cyclic vomiting syndrome (CVS) in childhood, a variety of underlying organic causes has been clearly identified in the literature. The aim of this study was to emphasize that endoscopic evaluation in the first step may help diagnosis and treatment in patients with CVS, unlike the CVS-related "North American Society for Pediatric Gastroenterology, Hepatology and Nutrition" (NASPGHAN) consensus statement in 2008. MATERIALS AND METHODS: The medical files of patients with vomiting complaints admitted to our tertiary center between the years 2007 and 2012 were analyzed retrospectively. Patients were identified according to the International Classification of Diseases (ICD) codes at their initial presentation, including vomiting. RESULTS: A total of 815 patients with vomiting complaints were evaluated. Of the 379 patients who presented with vomiting only, 336 patients were already being followed for chronic vomiting. Cyclic vomiting was detected in 31 out of 336 patients. CONCLUSION: In our series, familial Mediterranean fever (FMF), cavernous transformation of the portal vein, and Helicobacter pylori (HP) gastritis presented with CVS for the first time in the pediatric age group. We emphasize that endoscopic evaluation in patients with CVS should be performed as the first step for appropriate diagnosis and treatment.

Aspartate aminotransferase-to-platelet ratio index in children with cholestatic liver diseases to assess liver fibrosis.

To assess the relationship between aspartate aminotransferase-platelet ratio index (APRI) and liver fibrosis in children with chronic hepatocellular and biliary cholestatic liver diseases. A retrospective review of one hundred children's demographic and laboratory findings concurrent with liver biopsy, who were followed-up with prolonged cholestasis. The diagnostic accuracy of the APRI was assessed by receiver operating characteristic (ROC) curves. Advanced fibrosis was more common in the hepatocellular group. The APRI values of the two groups were similar. The patients with advanced fibrosis had significantly higher APRI values than patients with mild fibrosis, in both the hepatocellular and biliary groups. The areas under the ROC were 0.68 and 0.81 in the hepatocellular and biliary groups respectively. The cut-off values of APRI for discriminating advanced fibrosis were 0.93 (65% sensitivity, 69% specificity) in the hepatocellular group and 2.35 (62% sensitivity, 96% specificity) in the biliary group. APRI may be most efficient for discriminating between advanced and mild fibrosis in biliary cholestatic liver disease patients.

Chylous ascites after a living-donor liver graft, effectively treated in a child with octreotide.

Chylous ascites after a liver transplant is a rare complication of surgery. We report a 11-month-old girl with biliary atresia who was presented with chylous ascites after a liver transplant. On the seventh day after surgery, while being fed, chylous ascites was observed. Besides fasting and diuretics, total parenteral nutrition and somatostatin analogue (octreotide) were initiated. Chylous ascites resolved in 3 weeks. Abdominal distention recurred 1 week later; fasting and total parenteral nutrition, combined with octreotide, were administered again for 2 more weeks. Thereafter, enteral feeding was started without any complications.