Plasma Levels of High Sensitivity Cardiac Troponin T in Adults with Repaired Tetralogy of Fallot

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Congenital myopathies: characteristic and subtypes in Hong Kong

This journal suppl. entitled: 20th International Congress of The World Muscle SocietyCongenital myopathies are a group of childhood onset neuromuscular disorder with the diagnosis mainly based on genetic and pathological features. This is a unique group with phenotypic, genotypic and pathological heterogeneity, so the confirmation of an underlying diagnosis is often challenging. This is the first congenital myopathy case series in Hong Kong. A total of 15 patients have been diagnosed to have congenital myopathies with 11 patients had the genetic mutations being identified (4 patients had RYR1 mutations, 3 patients had ACTA1 mutations, 2 patients had KLHL40 mutations, 1 patient had MTM1 mutation and 1 patient had DNM2 mutation).postprin

Lactobacillus rhamnosus hepatic abscess associated with Mirizzi syndrome: a case report and review of the literature

The clinical significance of Lactobacillus spp. isolated from clinical specimens has often been overlooked due to its low virulence. We report the first case of life-threatening bacteremic liver abscess due to Lactobacillus rhamnosus associated with Mirizzi syndrome in a 74-year-old Chinese man. Literature on sporadic reports of Lactobacillus liver abscess is reviewed. © 2010 Elsevier Inc. All rights reserved.link_to_subscribed_fulltex

Treatment outcome of Paediatric Hepatoblastoma in Hong Kong

The meeting is organized by the comity of Pediatric Liver Tumors of the French Society of Childhood Cancer (SFCE) and the Institute CurieBackground and aims: The Hong Kong Paediatric Haematology and Oncology Study Group had formulated a consensus protocol (HB/HCC 1996) based on results published by the Children’s Oncology Group (COG) for treatment of paediatric hepatoblastoma (HB). We review the treatment outcome of HB in Hong Kong over the past 2 decades and investigate the role of PRETEXT staging and risk stratification. Methods: Review of population-based paediatric oncology database for patients (100ng/ml) in 58 (97%) patients. Epithelial type was the commonest subtype occurring in 39 (65%) patients. Five (8%) had lung metastases at presentation. Treatment details were not available in 3 patients and 1 patient died of tumor rupture before treatment. Twenty-nine patients (48%) received treatment with first-line chemotherapy; 23 (38%) required alternative agents. Three patients did not require chemotherapy after surgery; 1 died of liver failure post-operatively before initiation of adjuvant treatment. Surgical resection could be performed in 48 (80%) patients whilst 8 patients had unresectable (+/- metastatic) disease among which 3 (5%) underwent upfront liver transplantation. There were 14 deaths (disease relapse in 6; disease progression in 5 and tumor rupture in 3) and 11 relapses (native liver in 6; transplanted liver in 1; lung in 3 and bone in 1). The 5-year OS and EFS rates were 77.6% (±5.5%) and 69.2% (± 6.1%), respectively. Predictors of inferior outcome included advanced COG staging, unresectable or bilobar disease, tumour rupture, low AFP and suboptimal response to first-line chemotherapy. Long term sequalae included hearing loss (Grade 3 in 4) and renal tubular dysfunction (Grade 2 in 2). Radiological review for PRETEXT staging could be performed in 29 of 60 subjects yielding PRETEXT I in 3, II in 8, III in 14 and IV in 4. Eighteen patients belonged to standard risk (SR) and 11 to high risk (HR) according to the SIOPEL risk criteria. 5y OS and EFS rates were significantly better in the SR patients than the HR patients (89.9%±6.8% vs 42.2%±15.6% [p=0.017]; 84.7%±8.2% vs 13.6%±11.7% [p<0.001], respectively). Conclusions: Although treatment with the HB/HCC 1996 protocol resulted in cure of about three-quarters of HB patients, it is clear that we need to identify high risk patients upfront by a robust risk stratification system and improve their treatment outcome by intensifying chemotherapy regimen and consolidating surgical treatment plan (including selection criteria for total hepatectomy and liver transplantation)

Cardiac Magnetic Resonance Assessment of myocardial iron load and fibrosis in adult survivors of childhood leukaemias

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