BACKGROUNDTo establish the prevalence of hearing deficit in children with Down syndrome (DS) in Hong Kong as measured by brainstem auditory evoked potentials (BAEP). The secondary objective is to examine the agreement between BAEP and clinical questioning in detecting hearing deficit in DS.

METHODSConsecutive DS patients attending the Down's Clinic in a regional pediatric referral center were recruited into this cross-sectional study. BAEP data performed within 12 months were retrieved. The care-taker was interviewed with a structured questionnaire to detect any symptom of hearing impairment. BAEP findings and clinical questionings were compared in an agreement analysis using quadratic weighted kappa statistics.

RESULTSFifty DS patients (35 male, 15 female, mean age 11.70 years ± 5.74 standard deviation) were recruited. Eighteen patients (36.0%) were identified having hearing deficit by BAEP. Among patients with hearing impairment, 13 patients (72.2%) had a conductive deficit, and most have mild to moderate hearing loss. Five patients (27.8%) had sensorineural deficit and most have moderate to severe degree. Eight (44.4%) had bilateral hearing deficit. Care-takers of 13 patients (26.0%) reported symptoms of hearing impairment, with 9 (69.2%) having mild symptoms, 3 (23.1%) had moderate symptoms and 1 (7.7%) had severe symptoms. The weighted kappa was 0.045 (95.0% confidence interval - 0.138-0.229), indicating very poor strength of agreement between BAEP and clinical questioning. For patients with conductive hearing impairment, only 1 patients (7.7%) recalled history of otitis media.

CONCLUSIONSThe estimated point prevalence of hearing impairment in Chinese DS children in Hong Kong is 36%. Our finding of poor strength of agreement between objective testing and symptom questioning reflects significant underestimation of hearing impairment by history taking alone. In view of the high prevalence and low parental awareness, continuous surveillance of hearing is mandatory for DS patients throughout childhood and adolescence.

Adolescent ; Child ; Cross-Sectional Studies ; Down Syndrome ; epidemiology ; physiopathology ; Evoked Potentials, Auditory ; physiology ; Female ; Hearing Loss ; epidemiology ; etiology ; Humans ; Male ; Prevalence

INTRODUCTIONThe angiographic findings and prognosis of patients with complete atrioventricular block (AVB) complicating acute inferior myocardial infarction (MI) remain unclear.

MATERIALS AND METHODSThe clinical and angiographic findings of 70 consecutive patients with complete AVB were compared with those of 319 patients with inferior MI without AVB (control group) admitted within the same study period.

RESULTSPatients with complete AVB were older (68 +/- 12 vs 63 +/- 13 years; P = 0.004) and clustered with clinical features indicative of larger infarct size, such as right ventricular infarction, cardiogenic shock, or low left ventricular ejection fraction (LVEF). The onset of the complete AVB was observed within 24 hours in 62 (88.6%), preceded by second-degree AVB in 26 (37.1%) and the escape QRS complex was wide in 8 (11.4%) patients. In patients with complete AVB, a dominant right coronary artery occlusion was found in >95% of cases and in-hospital mortality was increased (27.1% vs 10.7%; P = 0.000), especially in those with widen QRS escape rhythm (75.0%). Reperfusion therapy had a positive impact on the natural course of complete AVB.

CONCLUSIONSComplete AVB in acute inferior MI was associated with advanced age and larger infarct size. Complete AVB was virtually always caused by dominant right coronary artery occlusion. The in-hospital mortality was significantly higher, but improved by reperfusion therapy. No permanent pacemaker is performed at a mean follow-up of 47 months.

Age Factors ; Aged ; Aged, 80 and over ; Atrioventricular Block ; complications ; diagnostic imaging ; mortality ; Coronary Angiography ; Electrocardiography ; Female ; Hong Kong ; epidemiology ; Hospital Mortality ; Humans ; Inferior Wall Myocardial Infarction ; complications ; diagnostic imaging ; mortality ; Kaplan-Meier Estimate ; Male ; Middle Aged

OBJECTIVETo report our experience of 200 endoscopic totally extraperitoneal inguinal hernioplasties utilizing reusable instruments.

METHODSBetween August 1999 and June 2000, 200 endoscopic totally extraperitoneal hernioplasties were performed on 163 patients. The mean age of the study population was 63 years with a male to female ratio of 157:6. Perioperative details and postoperative outcomes were prospectively evaluated and analyzed.

RESULTSA total of 196 (98%) endoscopic extraperitoneal inguinal hernioplasties were successfully performed. Conversion rates to transabdominal preperitoneal and open repairs were 1.5% (n = 3) and 0.5% (n = 1), respectively. There were no other intraoperative complications. Postoperative morbidity included retention of urine (n = 7), wound bruising (n = 2), atelectasis (n = 2) and gouty arthritis (n = 1). The mean visual analogue pain scores at rest were 2.3, 1.6 and 1.9 on postoperative days 0, 1 and 2, respectively. The mean length of hospital stay was 1.9 days. 113 patients (69%) returned to normal activities within one week. Of the 35 patients who experienced both open and laparoscopic repair, 80% expressed preference for endoscopic hernioplasty in the event of future recurrence.

CONCLUSIONSEndoscopic extraperitoneal inguinal hernioplasty can be safely performed utilizing reusable trocars. Substantial reduction of operative cost could be achieved by the elimination of disposable instruments. Deficiencies of the reusable metallic trocar, namely peri-cannula air-leak and sliding movements of the trocar, can be overcome by purse-string suture of the fascial opening.

Cost Control ; Endoscopy ; Female ; Follow-Up Studies ; Health Care Costs ; Hernia, Inguinal ; surgery ; Humans ; Male ; Postoperative Complications ; etiology ; Surgical Instruments

Food Addiction (FA) is a concept of dependence on substance related to the consumption of palatable foods high in fat, sugar, and salt. This dependence may influence dietary modification advice as dietary modification is an essential component in managing people with diabetes. The objective of this study is to determine the prevalence of FA among people with diabetes. A cross-sectional study was conducted between June and August 2021 at Klinik Primer, Pusat Perubatan Universiti Kebangsaan Malaysia (Cheras, Kuala Lumpur) involving 142 people with diabetes. FA was assessed using the validated Bahasa Melayu version of Yale Food Addiction Scale 2.0. The median age of participants was 58 years (IQR=10), and the majority (88%, n=125) of them were above the age of 45 years. A large majority) had central obesity (90.1%, n=128) and were overweight and obese (91%, n=129). The median duration of diabetes was 10 years (IQR=11) with more than two-thirds (63.4%, n=90) of them having diabetes for more than 5 years. Majority (73.9%, n=105) also had poor glucose control with median HbA1c level of 7.5% (IQR =2). Our study found that the prevalence of FA among people with T2DM was low (2.8%, n=4). This could be because the participants were mostly from the older age group with long standing disease. These two features may have influenced their eating behaviour, becoming more compliant and adapted to diabetic diet advice received over the long duration of the condition. A similar study among young diabetics and those with new onset disease may better reflect FA among this group of people.

Hyperphenylalaninemia is one of the commonest inborn errors of metabolism affecting approximately 1 in 15,000 livebirths. Among Chinese, BH4 deficiency leading to hyperphenylalaninemia is much commoner than in Caucasians. Exact diagnosis is important for the treatment and genetic counseling. In 2000, newborn screening for phenylketonuria is mandatory by law in China throughout the whole country. However, it is not yet included in the newborn screening program of the Hong Kong Special Administrative Region, China. Published data on hyperphenylalaninemia among HongKong Chinese are largely lacking. We report a 1-year-old Hong Kong Chinese girl with severe 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency. The patient presented with infantile hypotonia and was misdiagnosed as cerebral palsy. She had very mild hyperphenylalaninemia (95 μmol/L), significantly high phenylalnine-to-tyrosine ratio (3.1), and elevated prolactin of 1109 mIU/L. Genetic analysis confirmed a homozygous known disease-causing mutation PTS NM_000317.1:c.259C>T; NP_000308.1: p.P87S in the proband. In our local experience, while the estimated prevalence of hyperphenylalaninemia due to PTPS deficiency was reported to be 1 in 29,542 live births, not a single case of phenylalanine hydroxylase deficiency has been reported. Furthermore, there is a general lack of awareness of inherited metabolic diseases in the community as well as among the medical professionals. Very often, a low index of clinical suspicion will lead to delay in diagnosis, multiple unnecessary and costly investigations, prolonged morbidity and anxiety to the family affected. We strongly recommend that expanded newborn screening for hyperphenylalaninemia should be implemented for every baby born in the Hong Kong Special Administrative Region, China.
Asian Continental Ancestry Group ; China ; Female ; Hong Kong ; Humans ; Infant ; Mass Screening ; Phenylketonurias ; diagnosis

OBJECTIVETo further investigate the {ptin vitro} effects of an osteoprotective herbal formula "ELP" (Herba Epimedii, Fructus Ligustri Lucidi and Fructus Psoraleae) using seropharmacological approach.

METHODSRats were fed with ELP or its individual component herbs for 2 days. The serum containing the postabsorbed ingredients of the herbal items were collected for cell culture using UMR106 cell, RAW264.7 cell and mesenchymal stem cell (MSC) isolated from the bone marrow of the rats. The effects of the herbal-containing serum on cell toxicity were detected by 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyltetrazolium bromide assay; bromodeoxyuridine assay was conducted to measure the cell proliferation of UMR106 cell and MSC; cell activity was measured using colorimetric method, and mRNA expression of runt-related transcription factor 2 (Runx2), alkaline phosphatase (ALP) and osteopontin (OPN) of UMR106 and MSC as well as matrix metalloproteinase 9 (MMP-9), tartrate-resistant acid phosphatase (TRAP) and cathepsin K of RAW264.7 were analyzed using real-time reverse-transcription polymerase chain reaction.

RESULTSELP and its component serum exhibited no cytotoxic effects on the cells. The ELP-containing serum increased the proliferation of UMR106 cell and MSC by 25.7% and 14.4 %, respectively and the alkaline phosphatase activity of MSC was increased by 42.6%. On the contrary, it inhibited the RAW264.7 cell differentiation by 29.2 %. ELP serum upregulated the Runx2 expression of UMR and MSC by 1.18 fold and 1.27 fold, respectively. It also upregulated ALP and OPN expression in MSC by 1.69- and 2.12-fold, respectively. On the other hand, ELP serum down-regulated MMP-9 and cathepsin K expression of RAW264.7 cell by 0.46- and 0.36-fold, respectively.

CONCLUSIONSThe serum of the animals fed with ELP contains active ingredients which are effective in promoting osteogenesis and inhibiting osteoclastogenesis.

Absorption, Physiological ; drug effects ; Animals ; Bone and Bones ; drug effects ; pathology ; Cell Differentiation ; drug effects ; Cell Proliferation ; drug effects ; Cell Survival ; drug effects ; Drugs, Chinese Herbal ; pharmacology ; Male ; Mice ; Osteoclasts ; drug effects ; metabolism ; pathology ; Osteogenesis ; drug effects ; Protective Agents ; pharmacology ; RAW 264.7 Cells ; RNA, Messenger ; genetics ; metabolism ; Rats ; Rats, Sprague-Dawley ; Real-Time Polymerase Chain Reaction ; Serum ; metabolism

Aims: The gut microbiota is referred to as an ‘extra organ’ and is critical in assisting the host in terms of nutrition and immunity. Environmental stressors could alter the gut microbial community and cause gut inflammation. This study aimed to investigate and compare the gut microbiota community between healthy and diseased Tor tambroides. Methodology and results: In this study, such gut microbial alterations were explored using NGS-based 16S rDNA targeted sequencing on the Malaysian mahseer (T. tambroides). Three healthy adult and three diseased adult Malaysian mahseers (showing signs of exophthalmia, coelomic distension and petechial haemorrhage) were obtained from LTT Aquaculture Sdn Bhd. Our results revealed significant differences in microbial diversity, composition and function between both populations of T. tambroides. Alpha diversity analysis depicts lower diversity of gut microbiota composition in diseased T. tambroides as compared to the healthy group. In particular, Enterobacteriaceae, Aeromonas, Bacteroides, Vibrio and Pseudomonas were found within gut microbiota of the diseased fishes. In addition, cellulosedegrading bacteria and protease-producing bacteria were identified from the gut of T. tambroides. Conclusion, significance and impact of study Thus, our findings emphasized on the association between the alteration in gut microbiota composition and infectious abdominal dropsy (IAD) in T. tambroides. This finding is important to provide basic information for further diagnosis, prevention and treatment of intestinal diseases in fish.
Gastrointestinal Microbiome ; Cyprinidae