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.

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.

Eosinophilic appendicitis caused by Schistosoma japonicum: a case report and review of the literature.

Parasitic appendicitis is uncommon. The authors reviewed the pathology of 4,130 appendices resected over the past 10 years (2000 to 2009). Only one case of eosinophilic appendicitis caused by Schistosoma japonicum was identified. The overall prevalence of schistosomal appendicitis was 0.024%. The case was a 61-year-old woman who presented with right lower quadrant abdominal pain. She had been a farmer in Chumphon and Surat Thani Provinces, which are endemic for schistosomiasis in Thailand. Physical, laboratory and ultrasound examinations were suggestive of acute appendicitis. She underwent emergency appendectomy. Intraoperative findings revealed a ruptured appendix with a fecalith in the appendiceal lumen. The histopathologic diagnosis was suppurative eosinophilic appendicitis with schistosomal ova in the mucosa, submucosa, muscular layer and vascular lumens, identified as S. japonicum eggs. The patient was treated for the parasite with praziquantal. We briefly review the clinicopathologic features and pathogenesis of schistosomal appendicitis.

Dysplastic nodules and small primary carcinoma of the liver: a study detecting the early morphological changes during hepatocarcinogenesis.

OBJECTIVE: Detect the early histological changes relating to human hepatocarcinogenesis in three nodular hepatocellular lesions. MATERIAL AND METHOD: Three cases of dysplastic nodules and one of small hepatocellular carcinoma were obtained from the authors' surgical-pathology file during 2000-2005 for a histopathological study in relevance to the early changes during hepatocarcinogenesis by employing hematoxylin and eosin stain, as well as some immunohistochemical staining. RESULTS: One nodular hepatocellular lesion, diagnosed as a complex lesion of focal nodular hyperplasia contained a microscopic focus (1.5 mm in diameter) of combined hepatocellular and cholangiocarcinoma. CONCLUSION: The small dysplastic hepatocytes subjected to neoplastic transformation combined hepatocellular and cholangiocarcinoma and are the precursorial cells of hepatocellular carcinoma. Chronic viral hepatitis B or C, aflatoxin B, and nitrosamine(s), as well as some nodular hepatocellular lesions share distinct roles in the complex process of hepatocarcinogenesis pertaining to this Southeast Asian country.

Preminary report: initial experience of endovascular laser therapy for varicose veins due to greater saphenous vein incompetence in Thailand.

BACKGROUND: Surgical venous stripping (SVS) is a standard treatment for varicose veins (VV) due to greater saphenous vein incompetence (GSVI) but there are some disadvantages to and risks. Endovascular laser (EVL) has been introduced to overcome these disadvantages. The present study was designed to determine the effectiveness of EVL treatment for these patients. MATERIAL AND METHOD: The patients with VV due to GSVI diagnosed by duplex scan were recruited in the present study. The EVL-procedure was percutaneously approached guiding by ultrasound under monitor anesthetic care (MAC). Postoperative clinical and imaging assessment was assessed. RESULTS: There were 17 limbs with symptomatic VVs in 11 patients. Two patients were admitted for a reason not related to surgery. The others were day cases. There was no postoperative complication except a large echymosis in one case. At 3-month follow-up, no recanalization or recurrence was detected. CONCLUSION: The authors' early results demonstrated that EVL could obliterate VVs due to GSVI and further showed some benefits over SVS. More studies with a longer period of follow-up are needed to further confirm the efficacy of EVL.

Transplant renal artery stenosis in Thailand.

Transplant renal artery stenosis (TRAS) is one of the common vascular complications post kidney transplantation. A retrospective study of TRAS among transplant recipients at a single transplant center in Thailand was performed from February 1986 to December 2002. Among 750 cases, 16 cases (2.1%) of TRAS were identified. Twelve cases (3.3%) were from cadaveric donors and four cases (1%) were from living-related donors (p-value = 0.034). Most cases presented with progressive deterioration of kidney graft with or without refractory hypertension. Doppler ultrasonography was used for initial screening followed by renal angiography. Fifteen cases were treated by Percutaneous Transluminal Angioplasty (PTA) with a 73 per cent success rate. Five cases underwent surgical revascularization with an 80 per cent success rate. Two cases (13%) of successful PTA showed recurrent stenosis with 46 months follow-up which were successfully treated by repeated PTA with stents.