Intrapulmonary vascular dilation in children with chronic liver diseases: pre- and post-liver transplantation.

BACKGROUND AND STUDY AIMS: Chronic liver disease (CLD) can cause hepatopulmonary syndrome (HPS), defined as triad of liver disease, hypoxemia, and intrapulmonary vascular dilation (IPVD). The aim of this study was to determine the evidence of IPVD in a cohort of pediatric patients with CLD pre- and post-liver transplantation (LT). MATERIAL AND METHODS: All pediatric patients with CLD listed for LT were studied. Pulse oxygen saturation (SpO(2)), technetium-99m-labeled macroaggregated albumin ((99m)Tc- MAA) perfusión scan (positive test: uptake of the isotope ≥ 6% in the brain), and echocardiography with saline bubble test (SBT) were performed. SBT was re-evaluated at 3-6 months after LT. Grading of SBT included grade 0 (no bubble), I (1-9 bubbles), grade II (10-20 bubbles), and grade III (> 20 bubbles). RESULTS: Eighteen patients, median age 22.5 months (8-108), were enrolled. Most had biliary atresia (77.8%). Pre-LT, all patients had SpO(2) of 100% and none had positive (99)mTc- MAA perfusion scan. Two patients (11%) had negative SBT (grade 0), 1 (5.5%) had grade I, 3 (16.5%) had grade II, and 12 (67%) had grade III, respectively. Post-LT SBT became negative in all survivors (n = 16), (p = 0.0001). CONCLUSIONS: Most cirrhotic children in this cohort study had evidence of IPVD by positive SBT. However, none of these met the criteria for diagnosis of HPS. This evidence of IPVD subsided after LT.

Long-term outcome of living donor liver transplantation in a Thai boy with hereditary tyrosinemia type I: a case report.

UNLABELLED: Hereditary tyrosinemia type I (HT-I) is an autosomal recessive inborn error of tyrosine metabolism, caused by mutation(s) in the gene encoding for fumarylacetoacetate hydrolase (FAH) enzyme. The authors report a Thai boy who presented at two months of age with liver failure. HT-I was diagnosed based on the presence of succinylacetone in urine and homozygous R237X mutations of FAH gene. He was started on tyrosine and phenylalanine restricted diet immediately. Due to a limitation of 2-(2-nitro-4-trifluoromethyl benzoyl)-1,3-cyclohexanedione (NTBC) therapy in Thailand, it was commenced at eight months old and used as a bridging therapy before liver transplantation. He had a good response to NTBC therapy with an improvement in liver chemistries and synthetic functions. Subsequently, living donor liver transplantation (LDLT) was performed at 15 months old Long-term follow-up for 6.3 years following LDLT revealed normal growth, good school performance, normal liver, renal tubular, and glomerular functions, and without urinary excretion of succinylacetone. CONCLUSION: Liver transplantation is a promising treatment for patients with HT-1 when NTBC is unavailable, resulting in a good long-term outcome.

Testicular enlargement in a pre-pubertal boy with adrenocortical tumour.

Adrenocortical tumours are rare in children. Virilisation caused by overproduction of adrenal androgens is the most common presentation. The testes of pre-pubertal boys with this tumour are usually small or of pre-pubertal size. A 4.8-year-old boy with an adrenocortical tumour and symmetrical pubertal-sized testes is reported. The serum testosterone level was 204 nmol/L (<0.7), dehydro-epiandrosterone-sulphate 56.7 µmol/L (<1.5) and luteinising and follicle-stimulating hormones were at suppressed levels. Histology demonstrated a diffusely increased mean tubular diameter of 90 µm (the size in a 12-year-old boy) and hyperplasia of Sertoli cells. There were no Leydig cells in the interstitial area. Prolonged exposure to an extraordinarily high testosterone level could have had stimulating effects on the seminiferous tubules and Sertoli cell growth and thus contributed to testicular enlargement.

Cardiac abnormalities in cirrhotic children: pre- and post-liver transplantation.

BACKGROUND/AIM: Liver cirrhosis is associated with several cardiac abnormalities. There have been few studies of these abnormalities in cirrhotic children post-liver transplantation (LT). The purpose of this study was to evaluate cardiac abnormalities in cirrhotic children pre- and post-LT. METHODS: All cirrhotic children <15 years of age on a waiting list for LT underwent pre-LT echocardiography to evaluate left ventricular (LV) dimension, mass, and function. Repeated studies were performed at 1-2 and 3-6 months post-LT. RESULTS: A total of 20 cirrhotic children (median age 21.5 months [8-108 months], 11 female [55 %]) were enrolled in the study. Most patients had biliary atresia (75 %) and decompensated cirrhosis, with a median pediatric end-stage liver disease score of 19.5 (14-28). Two patients subsequently died, at 1 and 4 months post-LT. Echocardiography was re-evaluated in 17 and 18 patients at 1-2 months and 3-6 months post-LT, respectively. Prior to transplant, most patients had cardiac abnormalities, including LV enlargement (50 %), increased LV mass (95 %), abnormal LV geometry (95 %), hyperdynamic LV systolic function (60 %), LV diastolic dysfunction (60 %), and high cardiac index (75 %). At 3-6 months post-LT, no significant decrease in cardiac abnormalities was noted; however, cardiac parameters including LV dimension in diastole index and z-score, LV mass index, and relative wall thickness were significantly decreased. CONCLUSIONS: Most cirrhotic children had cardiac abnormalities, including LV enlargement, increased LV mass, abnormal LV geometry, and LV dysfunction. These abnormalities tended to improve post-LT. We suggest that echocardiography should be performed in all cirrhotic children.

Recombinant-activated factor VII for control and prevention of hemorrhage in nonhemophilic pediatric patients.

A total of 108 episodes among 103 nonhemophilic pediatric patients (nine newborns, 16 infants and 78 children) treated with recombinant factor-activated VII (rFVIIa) were evaluated retrospectively. These episodes were divided into two groups: group 1 included 86 occurrences for hemorrhagic control of ongoing massive bleeding due to thrombocytopenia and coagulopathy unresponsive to blood component therapy in patients with dengue hemorrhagic fever, life-threatening, intraoperative and postoperative bleeding; group 2 included 22 episodes for prevention of hemorrhage with invasive procedures in patients with chronic liver disease and associated coagulopathy, and patients without preexisting hemostatic disorder but at high risk due to their underlying diagnosis and required surgical intervention. The effective control of hemostasis response rate in group 1 was significantly lower than in group 2. The median total dose per kilogram of rFVIIa group 1 was twice that of group 2. The overall case-fatality rate related to bleeding or underlying conditions was 31.1% (32/103). Adverse events were observed in three patients (2.9%) receiving rFVIIa for control of intraoperative and postoperative bleeding in the setting of corrective cardiac surgery. These results support the safety and potential benefit of rFVIIa for control and prevention of hemorrhage in pediatric patients without congenital hemophilia.

Transanal one-stage endorectal pull-through for Hirschsprung's disease in infants and children.

PURPOSE: This report presents the technique and results of transanal one-stage endorectal pull-through procedure in children with rectosigmoid lesions from Hirschsprung's disease. METHODS: Eight children aged one month to 6 years with frozen section biopsy-proven Hirschsprung's disease underwent transanal one-stage endorectal pull-through procedures during a 12-month period. A rectosigmoid transitional zone was suggested by contrast enema in 7 patients; rectal manometry was done to confirm the diagnosis in one patient. Preoperative colonic irrigation to evacuate feces out of the dilated colon was done in the hospital. Bowel preparation was the same as conventional colorectal surgery. Full-thickness rectal biopsy at 1 to 2 cm above the dentate line was submitted for pathologic diagnosis. A rectal mucosectomy dissection was started 0.5 cm proximal to the dentate lines and was extended into the intraperitoneal rectum. The muscular sleeve was divided circumferentialy at 3 to 4 cm proximal to the dentate line, exposing the intraperitoneal rectum and allowing full-thickness mobilization of the rectosigmoid colon out of the anus. Aganglionic colon segment was resected, and the normal colon was pull down to anastomose with the distal end of anorectal mucosa. RESULTS: Operating time, including taking frozen sections, ranged from 110 to 180 minutes. The length of bowel resections ranged from 9 to 25 cm. The length of hospital stay depended on the amount of fecal impaction in the colon. Older children with substantial fecal impaction required 2 weeks of preoperative saline enema. One infant needed 3 days for bowel preparation, the same as for conventional colorectal surgery. The hospital stay ranged from 6 to 7 days in children younger than 2 years and 10 to 28 days in older children. There were no intraoperative or postoperative complications related to the pull-through procedure. One case of colitis occurred in the 6-year-old child, which required rectal tube decompression one week after the operation. Seven patients passed stool within 24 hours after surgery. All patients had normal bowel movements within 3 weeks. There was no rectal cuff stricture or enterocolitis during one year of follow-up. CONCLUSIONS: Transanal one-stage endorectal pull-through operations for rectosigmoid lesions from Hirschsprung's disease can be performed successfully in all ages of children with good results, avoiding transabdominal exploration. The early postoperative enterocolitis in the older children might occur and should be treated urgently. The partial coloanal anastomosis obstruction found in older children could be treated by placing a rectal tube into the anus to decompress the dilated pull-through colon. The limitation of this approach is that retroperitoneal fixation of the descending colon could not be dissected by the transanal route.