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Background. Liver transplantation (LT) is a well-established, life-saving treatment for children with irreversible acute and chronic liver failure (LF). We aimed to evaluate the factors associated with morbidity and mortality in the early period of LT in children by reviewing our pediatric intensive care unit (PICU) experience. Methods. We reviewed children's medical records followed in the PICU after LT between May 2015-August 2021, including demographic parameters, indications for LT, operative variables, respiratory and circulatory support requirements, LT-related complications and survival. Results. During this period, 40 pediatric patients who underwent LT were evaluated. LT was performed in 35 (87.5%) cases of chronic liver disease and 5 (12.5%) cases of acute liver failure. Twenty-four patients had chronic liver failure due to cholestatic liver disease. The patients' Pediatric Risk of Mortality (PRISM) III score was 18.82±SD (2-58) at PICU admission. 1-year survival was 87.5%, and overall survival was 85%. Younger age, low body weight, preoperative pediatric end-stage liver disease (PELD), and model for end-stage liver disease (MELD) values of 20 and higher were important risk factors for unfavorable outcomes after living donor liver transplantation (LDLT). These risk factors are both associated with technically more challenging vascular and bile duct reconstruction and higher complication rates, and increased mortality during the early period after LT. Conclusions. The early period of optimum PICU management in pediatric LT recipients is crucial for successful outcomes, which is also related to the patients' characteristics, disease severity scores, and surgical procedures. [ABSTRACT FROM AUTHOR]

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Spontaneous extrahepatic bile duct perforation presents in infants with minimal or no jaundice, abdominal distension, pale stools and biliary ascites and requires prompt diagnosis and surgical intervention. The association of epidermolysis bullosa simplex, congenital adrenal hyperplasia and spontaneous perforation of extrahepatic bile duct has not been reported previously. The diagnosis of spontaneous extrahepatic bile duct perforation should be suspected when there is bilious ascites; the majority of the patients need surgical intervention, which is beneficial for both diagnosis and treatment. [Extracted from the article]

Objective: Data for the drugs used in maintenance therapy of functional constipation is limited in childhood. We aimed to evaluate the efficacy of senna, trimebutine, and lactulose in children with functional constipation (FC) or functional fecal incontinence (FFI). Material and Methods: Patients (2-18 years old) diagnosed as FC or FFI according to Rome III criteria who were treated with lactulose (n=36), senna (n=29), and trimebutine (n=33)were included. Patients completed a 0-5 Likert scale questionnaire about their symptoms. Stool consistency was evaluated by the Bristol Stool Chart. Compliance and tolerance to treatment and side effects were recorded. Clinical improvement and pharmacoeconomic evaluation were performed. Results: Clinical improvement in the senna group was significantly higher (p=0.036). Senna showed the highest improvement in stool consistency (p<0.001) and decreased fecal incontinence (p=0.039). Abdominal distention, abdominal pain, nausea/vomiting, and anal fissure were improved by trimebutine (p<0.05). Both senna and trimebutine decreased painful defecation and stool withholding. Lactulose improved anorexia and anal fissure. Senna was the most cost-effective drug. Conclusion: Treatment options for constipation should be tailored according to the predominant symptom. Lactulose may be recommended as the initial treatment for children with anorexia or anal fissure, trimebutine for abdominal distention, abdominal pain, nausea/vomiting, or anal fissure, respectively. Senna may be prescribed for the cost-effectiveness with high clinical improvement and less unpleasant taste. [ABSTRACT FROM AUTHOR]

Transaldolase (TAL) is an enzyme in the pentose phosphate pathway (PPP) that generates NADPH for protection against oxidative stress. While deficiency of other PPP enzymes, such as transketolase (TKT), are incompatible with mammalian cell survival, mice lacking TAL are viable and develop progressive liver disease attributed to oxidative stress. Mice with homozygous or heterozygous TAL deficiency are predisposed to cirrhosis, hepatocellular carcinoma (HCC) and acetaminophen (APAP)‐induced liver failure. Both mice and humans with complete TAL deficiency accumulate sedoheptulose 7‐phosphate (S7P). Previous human studies relied on screening patients with S7P accumulation, thus excluding potentially pathogenic haploinsufficiency. Of note, mice with TAL haploinsufficiency are also predisposed to HCC and APAP‐induced liver failure which are preventable with oral N‐acetylcysteine (NAC) administration. Based on TALDO1 DNA sequencing, we detected functional TAL deficiency due to novel, heterozygous variations in two of 94 healthy adults and four of 27 subjects with APAP‐induced liver failure (P =.022). The functional consequences of these variations were individually validated by site‐directed mutagenesis of normal cDNA and loss of activity by recombinant enzyme. All four patients with TAL haplo‐insufficiency with APAP‐induced liver failure were successfully treated with NAC. We also document two novel variations in two of 15 children with previously unexplained liver cirrhosis. Examination of the National Center for Biotechnology Information databases revealed 274 coding region variations have been documented in 1125 TALDO1 sequences relative to 25 variations in 2870 TKT sequences (P <.0001). These findings suggest an unexpected prevalence and variety of genetic changes in human TALDO1 with relevance for liver injury that may be preventable by treatment with NAC. [ABSTRACT FROM AUTHOR]

Objective: To present the mesalamine-induced acute exacerbation of symptoms and inflammatory markers in children with Crohn's disease (CD).Clinical Presentation and Intervention: Three children who presented with CD had acute exacerbation of colitis symptoms or elevated inflammatory markers when mesalamine was added to treatment while tapering/ceasing steroid treatment. While on steroid treatment, the patients maintained clinical and laboratory remission, but with the initiation of mesalamine treatment, they had abdominal pain and bloody mucoid diarrhoea and/or elevation of white blood cell count, C-reactive protein level and erythrocyte sedimentation rate. Bacterial pathogens were excluded from the urine, throat and blood cultures, parasites with stool examination, viral pathogens with serology. Within 3-7 days after the mesalamine treatment had been stopped, the patients showed improvement of colitis symptoms and normalisation of white blood cell count, C-reactive protein level and erythrocyte sedimentation rate.Conclusion: In this study mesalamine mimicked CD relapse in children with CD while tapering or after stopping steroid treatment. Awareness of this side effect of mesalamine could prevent a misdiagnosis of steroid dependency. [ABSTRACT FROM AUTHOR]

Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and jaundice decreases due to regression of the mass, without any surgical procedure. The authors report the case of a 16-year-old girl who presented with biliary obstruction due to a neoplasm involving the duodenum. Histological examination of the specimen, which was taken from the mass by endoscopic biopsy, revealed Burkitt lymphoma infiltrating the duodenum. Chemotherapy including cyclophosphamide was started immediately. In a few days, jaundice decreased rapidly by the shrinkage of the mass. Neither surgery nor percutaneous drainage were needed. In conclusion, biliary tract obstruction due to non-Hodgkin lymphoma can be effectively treated with chemotherapy alone without any surgical procedure. [ABSTRACT FROM AUTHOR]

Gastric carcinoid tumors (GCT) are rare lesions that constitute 2.6-8.7% of all gastrointestinal carcinoids, mostly affect middle-aged females but the incidence in children is unknown. We present a 14-year-old girl, with GCT. She was treated with recombinant human growth hormone (GH) for complete GH deficiency, and endoscopy was performed to identify iron-deficiency anemia. Upper gastrointestinal endoscopy revealed a gastric polyp, and biopsies were compatible with GCT. [ABSTRACT FROM AUTHOR]

DOCK8 deficiency is a rare inherited combined immunodeficiency, caused by mutations in the DOCK8 gene. We describe a case with DOCK8 deficiency associated with severe CLD in whom orthotopic LT was performed successfully after allogeneic HSCT. A 5 year‐old girl with DOCK8 deficiency presented with mild direct hyperbilirubinemia and abnormal GGT level and without a previous history of jaundice. She had severe growth retardation, hepatosplenomegaly and generalized eczema. Progressive worsening of CLD was observed within 4 months. Investigations for etiology of liver disease were negative. Liver biopsy showed bridging necrosis, cholestasis and, cirrhosis. Recurrent immune hemolytic crisis and several viral infections developed in follow‐up. She underwent whole cadaveric LT for end‐stage liver disease (ESLD) 1 year after allogenic HSCT from a full matched related donor. The postoperative course was uneventful. The patient is alive with normal liver function and moderate skin graft versus host disease for 36 months after LT. In conclusion DOCK8 deficiency can be associated with severe CLD. Successful LT following HSCT is possible in patients with ESLD in DOCK8 deficiency. The timing of LT is challenging in patients requiring both HSCT and LT since conditioning regimens for HSCT can be highly hepatotoxic and the patients with suboptimal liver function can become decompensated during HSCT. [ABSTRACT FROM AUTHOR]

Milk leakage from the breast, which is known as galactorrhea, can be caused by a number of pharmacological, physical, and tumoral factors. Galactorrhea is a well-known side effect of domperidone, and is usually associated with hyperprolactinemia. However, euprolactinemic galactorrhea secondary to domperidone is very rarely seen and has yet to be reported in children before. Here, we report an adolescent with euprolactinemic galactorrhea caused by domperidone. [ABSTRACT FROM AUTHOR]

Abstract: We present a 15-year-old girl with congenital absence of the portal vein, pulmonary arterial hypertension and multiple liver lesions proven to be nodular regenerative hyperplasia with biopsy. Ultrasonography, computed tomography, and magnetic resonance imaging findings of the liver lesions and Type I portosystemic shunt are presented. [Copyright &y& Elsevier]