INTRODUCTIONSarcosinaemia is a rare metabolic disorder which has not been reported in Asia.

CLINICAL PICTUREThe urine samples of 2 patients were screened as a routine metabolic screening offered for patients with mental retardation in our hospital. We used gas chromatography-mass spectrometry (GC-MS) which is capable of detecting abnormal pattern in amino acids and organic acids. Plasma sarcosine level was further quantified by GC-MS. The same methods were used in the investigations of asymptomatic family members. Urine examination by GC-MS revealed excessive amount of sarcosine in urine (normally undetectable) and their plasma sarcosine levels were raised. The 2 differential diagnoses of presence of sarcosine in urine--glutaric aciduria type II and folate deficiency--were ruled out by the absence of abnormal organic acids in the initial urine screen and by normal serum folate level respectively. Screening of the 2 families identified excessive sarcosine in urine in 2 siblings, one from each family. However, these 2 siblings of indexed patients thus identified have no neurological or developmental problem.

CONCLUSIONOur finding was consistent with the notion that sarcosinaemia is a benign condition picked up coincidentally during screening for mental retardation.

Amino Acid Metabolism, Inborn Errors ; complications ; diagnosis ; Child ; Child, Preschool ; China ; ethnology ; Family Health ; Female ; Gas Chromatography-Mass Spectrometry ; Hong Kong ; Humans ; India ; ethnology ; Intellectual Disability ; complications ; Sarcosine ; blood ; urine ; Sarcosine Dehydrogenase ; deficiency

No abstract available.
Collagen Type I* ; Skin* ; Wound Healing* ; Wounds and Injuries*

Adult ; Charcot-Marie-Tooth Disease ; etiology ; genetics ; Female ; GTP Phosphohydrolases ; Humans ; Membrane Proteins ; genetics ; Mitochondrial Proteins ; genetics ; Mutation ; Sequence Analysis, DNA

OBJECTIVETo measure Derp1 and Blot5 allergen levels in asthmatics' homes in Hongkong.

METHODSSeventy houses were enrolled for a mite indoor environment study. Dust samples were obtained from two sites of each patients' house: bed and floor. Derp1 and Blot5 levels were quantified by a two-site monoclonal antibody-based ELISA technique.

RESULTSThe levels of Derp1 allergens found in bed (geometric mean (GM) 3.43 microg/g of dust; 95%CI, 1.89-4.96 microg/g) and on the floor (GM 1.12 microg/g of dust; 95%CI, 0.71-1.53 microg/g) indicated significant differences (P=0.005). However, the levels of Blot5 allergens found in bed (GM 19.00 microg/g of dust; 95%CI, 0.89-38.90 microg/g) and on the floor (GM 6.14 microg/g of dust; 95%CI, 0.40-11.90 microg/g) showed no statistically significant difference. In addition, in regards to the exposure index for Derp1 and Blot5 allergens found in bed and on the floor, 17.6% in bed and 8.6% on the floor had levels of Blot5 > or = 10 microg/g of dust, higher than those obtained for Derp1 (7.2% and 0% in bed and on the floor respectively, P<0.05); higher percentages in bed and on the floor (25.0% and 35.7%) were observed for levels of Blot5 = 0 microg/g of dust as compared with Derp1 in bed and on the floor (4.3% and 14.5% respectively, P<0.05).

CONCLUSIONSDerp1 and Blot5 are the major allergens found in this regional study, Blot5 is a more potent allergen in Hongkong, probably reflecting the high level of exposure to Blomia tropicalis (Bt). Bt and Dermatophagoides pteronyssinus (Dp) allergens should be included for precise diagnosis and effective immuno-therapeutic treatment of mite allergy in Hongkong.

Allergens ; analysis ; Animals ; Antigens, Dermatophagoides ; analysis ; Arthropod Proteins ; Asthma ; immunology ; Bedding and Linens ; Cysteine Endopeptidases ; Dermatophagoides pteronyssinus ; immunology ; Dust ; analysis ; Environmental Exposure ; Floors and Floorcoverings ; Hong Kong ; Housing ; Humans ; Humidity ; Mites ; immunology

OBJECTIVETo define the prevalence, severity and risk factors for lead poisoning in new immigrant children from the mainland of China to Hong Kong, China.

METHODSNew immigrant children from the mainland of China under 18 years of age were invited to join the study. Their growth parameters and venous blood lead levels (BLL) were measured within 7 days of arrival. Those with elevated BLL i.e. > 10 micrograms/dl (0.48 mumol/L) were assessed for signs, symptoms and risk factors of lead poisoning. Education on the prevention of lead poisoning and follow up BLL measurements were offered until their BLL normalized.

RESULTSFour hundred and fifty-seven children were recruited. Among them, 18.1% and 2.6% had BLL > 0.48 and 0.71 mumol/L, respectively. None had BLL > 0.96 mumol/L. Possible risk factors included contaminated drinking water (19%), herb ingestion (17.5%), pica, playing in dumping grounds, residing near paint factories or highways, habitual inhalation of car exhaust and cooking with petroleum. Symptoms and signs included abdominal pain, headache, short stature, and learning difficulties but did not correlate with BLL. None required treatment except for counseling on the avoidance of risk factors. About 94.7% showed a reduction in BLL 2-3 months after arrival and before counseling. All had normalized BLL by 9 months except 1 who had an X-ray feature of lead line.

CONCLUSIONSLead poisoning is common in new immigrant children from the mainland of China. Environmental factors were most important while behavioral factors like regular herb ingestion might have contributed to the persistence of high BLL in the minority.

Adolescent ; Child ; Child, Preschool ; China ; Emigration and Immigration ; Female ; Hong Kong ; epidemiology ; Humans ; Infant ; Infant, Newborn ; Lead Poisoning ; epidemiology ; etiology ; Male ; Prevalence ; Risk Factors

OBJECTIVETo evaluate serum cytokine concentrations in children with and without obstructive sleep apnoea (OSA) and to investigate the effects of OSA treatment on cytokines.

MATERIALS AND METHODSConsecutive children with habitual snoring and symptoms suggestive of OSA were recruited. They completed a sleep apnoea symptom questionnaire, underwent physical examination and overnight polysomnography (PSG). OSA was diagnosed if obstructive apnoea index (OAI) >1. A blood sample was collected for analysis of IL-6, IL-8, and TNF-alpha after PSG.

RESULTSOne hundred forty-two children (97 males) with a median (IQR) age of 11.1 years (9.0-12.8) were recruited. The commonest presenting symptoms were nocturnal mouth breathing, prone sleeping position and poor attention at school. Forty-seven children were found to have OSA and they had higher serum IL-6 [0.1 (0.1-0.4) vs 0.1 (0.1-0.1) pg/mL, P = 0.001] and IL-8 [1.7 (1.0-2.3) vs 1.3 (0.9-1.7) pg/mL, P = 0.029] concentrations compared to their non-OSA counterparts. Multiple regression analysis indicated that OAI was significantly associated with both IL-6 (r = 0.351, P <0.001) and IL-8 (r = 0.266, P = 0.002). Sixteen children underwent treatment and there was significant reduction in mean (SD) serum IL-8 after intervention [pre vs post levels of 1.9 (1.0) vs 1.1 (0.6) pg/mL, P = 0.001] independent of weight loss.

CONCLUSIONChildren with OSA had elevated levels of pro-inflammatory cytokines that normalised following treatment suggesting that the inflammatory response is potentially reversible. Early detection and intervention may be beneficial.

Child ; Cytokines ; blood ; Female ; Humans ; Interleukin-6 ; blood ; Interleukin-8 ; blood ; Male ; Polysomnography ; Sleep Apnea, Obstructive ; blood ; therapy ; Tumor Necrosis Factor-alpha ; blood

Salmonella are causative agents of gastroenteritis and systemic disease in animals. The invA gene was selected as a target sequence of loop-mediated isothermal amplification (LAMP) assay for diagnosis of Salmonella infection. The detection limits for broth dilution, spiked feces and enrichment were 10(4), 10(5) and 10(2) CFUs/mL, respectively. The LAMP assay developed in the present study may be a reliable method for detection of Salmonella spp. in pig feces.
Animals ; Diagnosis ; Feces* ; Gastroenteritis ; Limit of Detection ; Salmonella Infections ; Salmonella*

Objective: To study the application of associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) in hepatocellular carcinoma with mild-to-moderate liver cirrhosis. Methods: There are 14 patients with hepatocellular carcinoma underwent ALPPS at the Department of Hepatobiliary and Pancreatic Surgery, Hong Kong University-Shenzhen Hospital from April 2014 to December 2017. The clinical data was retrospectively studied. The studying objects consisted of 9 males and 5 females, aged from 26 to 71 years old with the average age of 51, all cases were of Child-Pugh grade A. The degree of liver cirrhosis, operation and postoperative complications were analyzed. Results: All 14 patients completed the ALPPS, 1 patient died post stage 2 operation with liver failure. Comparing the groups with no liver cirrhosis (n=4) with the groups of mild liver cirrhosis (n=5) and moderate liver cirrhosis (n=5), the future liver remnant liver volume growth rates were 58%, 46% and 45.6%, respectively. The average operation intervals were 9.0, 11.2 and 12.8 days, respectively. Postoperative complications occurred in 4 patients: 2 patients with liver failure, 1 patient with intestinal obstruction, and 1 patient with hepatic ascites. Conclusion ALPPS for Child-Pugh grade A, hepatocellular carcinoma with mild-to-moderate liver cirrhosis treatment is safe and feasible.

Objective:To study the safety and efficacy on timing of associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) stageⅡbased on increase in remnant liver volume.Methods:19 patients (male: female 13: 6; average age 53 years) with liver tumors treated by ALPPS from April 2014 to December 2020 were retrospectively studied. Patients with FLV/ESLV (future liver volume/ estimated standard liver volume) increase of more than 50% within 1 week followed by stageⅡALPPS were included into the rapid group ( n=8). Those who failed to have 50% increase in FLV/ESLV within 1 week were included into the control group ( n=11). The two groups were compared in the ALPPS stage II in operating time, blood loss, postoperative complications, mortality rate and hospital stay. Results:All 19 patients underwent ALPPS stage II uneventfully. One patient in the control group died from liver failure within 30 days of operation. The operation time (3.2±1.8)h, blood loss (554±227) ml and postoperative hospital stay (12.6±2.4) d in the rapid group were significantly better than those in the control group (4.7±2.2) h, [(760±314) ml, (18.2±6.4) d (all P<0.05)]. The two groups had similar complication rates in both post stageⅠ[37.5%(3/8) vs. 45.4%(5/11)] , or stageⅡ [37.5%(3/8) vs. 36.4%(4/11)] (both P>0.05). Conclusion:Rapid increase in FLR volume of more than 50% within a week was safe and feasible to proceed to ALPPS stage II. This conclusion needs to be confirmed by further studies using large sample sizes.

PURPOSE: 83b1 is a novel quinoline derivative that has been shown to inhibit cancer growth in human esophageal squamous cell carcinoma (ESCC). This study was conducted to comprehensively evaluate the cytotoxic effects of 83b1 on a series of ESCC cell lines and investigate the mechanisms by which 83b1 suppresses cancer growth based on molecular docking analysis. MATERIALS AND METHODS: A series of ESCC and nontumor immortalized cell lines were exposed to 83b1 and cisplatin (CDDP) in a dose-dependent manner, and the cytotoxicity was examined by a MTS assay kit. Prediction of the molecular targets of 83b1 was conducted by molecular docking analysis. Expression of cyclooxygenase 2 (COX-2) mRNA and COX-2–derived prostaglandin E₂ (PGE₂) were measured by quantitative real-time polymerase chain reaction and enzymelinked immuno-sorbent assay, respectively. In vivo anti-tumor effect was determined using a nude mice xenografted model transplanted with an ESCC cell line, KYSE-450. RESULTS: 83b1 showed the significant anti-cancer effects on all ESCC cell lines compared to CDDP; however, 83b1 revealed much lower toxic effects on non-tumor cell lines than CDDP. The predicted molecular target of 83b1 is peroxisome proliferator-activated receptor delta (PPARδ), which is a widely known oncoprotein. Additionally the expression of COX-2 mRNA and COX-2–derived PGE2 were down-regulated by 83b1 in a dose-dependent manner in ESCC cell lines. Furthermore, 83b1 was shown to significantly reduce the tumor size in nude mice xenograft. CONCLUSION: The results of this study suggest that the potential anti-cancer effects of 83b1 on human esophageal cancers occur through the possible oncotarget, PPARδ, and down-regulation of the cancer related genes and molecules.
Animals ; Carcinoma, Squamous Cell* ; Cell Line ; Cisplatin ; Cyclooxygenase 2 ; Dinoprostone ; Down-Regulation* ; Epithelial Cells* ; Esophageal Neoplasms ; Heterografts ; Humans* ; Mice ; Mice, Nude ; Molecular Docking Simulation ; PPAR delta ; Quinolines ; Real-Time Polymerase Chain Reaction ; RNA, Messenger*