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Introduction: Open disclosure is poorly understood in Malaysia but is an ethical and professional responsibility. The objectives of this study were to determine: (1) the perception of parents regarding the severity of medical error in relation to medication use or diagnosis; (2) the preference of parents for information following the medical error and its relation to severity; and (3) the preference of parents with regards to disciplinary action, reporting, and legal action., Methods: We translated and contextualised a questionnaire developed from a previous study. The questionnaire consisted of four case vignettes that described the following: medication error with a lifelong complication; diagnostic error with a lifelong complication; diagnostic error without lifelong effect; and medication error without lifelong effect. Each case vignette was followed by a series of questions examining the subject's perception on the above areas. We also determined the content validity of the questionnaire. We invited parents of Malaysian children admitted to the paediatric wards of Tuanku Jaafar Hospital to participate in the study., Results: One hundred and twenty-three parents participated in the study. The majority of parents wanted to be told regarding the event. As the severity of the case vignettes increased, the desire for information, remedial action, acknowledgement of responsibility, compensation, punishment, legal action, and reporting to a higher agency also increased. The findings did not have strong evidence of a relationship with subject's demographics., Conclusion: This study gives insights into previously unexplored perspectives and preferences of parents in Malaysia regarding open disclosure. It also highlights the opportunity for more research in this area with potentially broad applications.

Progressive familial intrahepatic cholestasis (PFIC) is characterized by early onset cholestasis, progressive liver cirrhosis, pruritus, poor growth and inexorable progression to liver cirrhosis in early childhood. The serum level of gamma-glutamyl transferase is low or normal, which is discordant with severe cholestasis. Five Malaysian patients with PFIC, who all had typical features of PFIC with early onset of severe and progressive cholestasis, pruritus, cirrhosis and liver failure, were described. Three patients died as a result of the disease, while another one died due to post-liver transplant complication. The only survivor has compensated liver cirrhosis. Patients with severe cholestasis but has spuriously low yGT should be suspected of having PFIC. Liver transplant, which is life-saving in a majority of patients with PFIC, should be considered in all patients with PFIC.

We aimed to determine the epidemiology and burden of rotavirus (RV) gastroenteritis (GE) in children requiring hospital care in an urban setting in Sabah, Malaysia. A prospective study of all patients younger than 12 years of age admitted with acute GE to Queen Elizabeth Hospital, Sabah, over a six-month period (October 2005 to March 2006) was conducted. During the study period, a total of 167 children with acute GE who had stool samples examined for RV were studied. RV accounted for 16% of all diarrhoeal cases, and 1.7% of all admissions to the children's wards during the study period. There was no difference in severity of GE between RV and non-RV groups. RV infection is a common cause of childhood GE requiring hospital care in Sabah.

A five-month-old Indian girl, product of consanguineous marriage, presented with diarrhoea with an onset within two days after birth, severe malnutrition and metabolic acidosis. The diarrhoea persisted even with lactose-free formula, amino acid-based formula and glucose-containing oral rehydration solution, but stopped when fasted. She required prolonged parenteral nutrition. Fructose and glucose tolerance tests were performed, confirming the child was able to absorb and metabolize fructose but not glucose, indicating a diagnosis of glucose-galactose malabsorption. This case illustrate how simple and pertinent clinical observations and laboratory investigations is sufficient to allow a firm diagnosis to be made.

We aimed to determine the outcome of nutritional intervention in children with moderate to severe malnutrition following persistent diarrhoea (PD), referred to a tertiary referral unit in Malaysia. Thirty-one (44%) of the 71 children (median age 19 months) with PD had moderate to severe malnutrition on admission. Fifty-three (75%) required dietary modification and 15 (21%) needed parenteral nutrition (PN, median duration 96 days). Of the 70 patients in whom remission of diarrhoea could be ascertained, 64 (91%) achieved remission. Three required home PN. At three months after discharge, there was a significant improvement in the mean weight-for-height z-score as compared to the original score at initial presentation (from -1.83 +/- -1.77 to -0.80 +/- -1.17; p < 0.001), although 12 (22%) of the 55 patients in whom nutritional status could be ascertained still had moderate to severe malnutrition. In conclusion, moderate to severe malnutrition was a common complication following PD resulting from diverse causes. With appropriate therapy, remission can be achieved in majority of patients, although a small number of patients needed home PN because of persistence of diarrhoea.

Mortality from acute diarrhoea in developed countries is low, but the morbidity and financial cost remains significant. A one-year prospective, descriptive, non-interventional, hospital-based study of acute gastroenteritis (AGE) was conducted in the year 2002 in the paediatric unit of University of Malaya Medical Centre, Kuala Lumpur, an urban hospital in Malaysia, to determine its morbidity and management. During the study period, 393 children with AGE were admitted, utilizing 0.50% of total patient-bed-day of the hospital. The median duration of symptoms before admission was two days. Seventy-seven percent of patients had consulted family physicians before admission. Antidiarrhoeal drugs (57%) and anti-emetics (48%) were commonly prescribed, but oral rehydrating solution (36%) was rarely advised. Upon admission, severe vomiting (24%) and severe diarrhea (24%) were not common, while 17% had moderate or severe dehydration. Rotavirus (22%) was the commonest pathogen identified. Electrolyte derangement, secondary septicaemia and chronic diarrhoea were all rare. Eighty-nine percent of patients received intravenous fluid therapy whilst in the hospital. No death was noted. The morbidity and mortality of children with AGE requiring hospital care in this study was low. However, preadmission management and fluid therapy after admission was not ideal. Efforts to encourage better adherence to established management protocol of AGE among family physicians and hospital clinicians should be instituted.

There is an increasing trend for Shigella isolates worldwide to be resistant to commonly prescribed antibiotics. The species distribution and antibiotic resistance of Shigella species isolated from children in Kuala Lumpur, Malaysia from 1978 to 1997 was reviewed. Three hundred and eighty six isolates were positive for Shigella species, representing 1.4% (95% CI: 1.3%-1.6%) of the 26320 total stool specimens and 13% (95% CI: 11.8%-14.2%) of 2986 isolates positive for bacterial pathogens. Shigella flexneri, constituting 74% of all isolates in the first five years of the study, decreased by 40% during the last five years (95% CI of decrease: 22.1%-57.9%), p-value < 0.0001) to 34%. There was a significant reduction (chi2 for linear trend = 77.6, p-value < 0.001) in the number of Shigella isolates as a percentage of total stool isolates obtained. 58% of the 241 isolates tested for antibiotic sensitivity were resistant to at least one antibiotic, and 42% wEre multi-resistant to three or more antibiotics. Shigella species was not a common pathogen among children admitted with diarrhoea in Kuala Lumpur, and was more likely to be resistant to commonly prescribed antibiotics.

A six-week-old male infant was admitted for investigation of cholestasis and pale stools. He became lethargic and apnoeic with prolonged seizures after a percutaneous liver biopsy. Subsequent investigations showed conjugated hyperbilirubinaemia, elevated liver enzymes, and hypoglycaemia. The radinuclide hepatobiliary scintigraphy was non-excretory. After an operative cholangiogram, the infant developed Addisonian-like crisis with bradycardia, hypotension, respiratory distress, metabolic acidosis, hypoglycaemia, hyponatraemia, and hyperkalaemia. Blood investigations confirmed congenital hypopituitarism. Hormone replacement therapy with L-thyroxine and cortisone acetate resulted in dissolution of jaundice and the reduction of the liver size.

The profile of admissions staying less than 24 hours admitted to the paediatric wards of University Malaya Medical Center, Kuala Lumpur, over a period of six weeks was reviewed to ascertain the need of a short-stay ward. Ninety-three (22%) of the 428 admissions admitted during the study period were discharged within 24 hours, 56 (60%) were discharged within 12 hours. Major categories of admissions were: elective investigative procedures (43%), and emergency admissions (44%). Reasons for emergency admissions: infections 42%, minor trauma/cerebral concussion 25% and febrile/afebrile seizures 11%. Only 20% required percutaneous oximetry monitoring and 2% required observations more frequently than 2 hourly. There may be a case for a short stay ward in a big paediatric unit in Malaysia.

A retrospective review of all stool samples obtained from children aged < 16 years with diarrhoea from University of Malaya Medical Centre (UMMC), Kuala Lumpur, from 1978 to 1997 was undertaken to ascertain the pattern of bacterial pathogens causing diarrhoea in children in an urban area in Malaysia. Of 26444 stool samples processed, 2989 (11%) were positive. The five most common bacterial pathogens isolated were non-typhoidal Salmonella (57%), enteropathogenic E. coli (EPEC) (14%), Shigella spp. (11%), Campylobacter spp. (5%) and Aeromonas spp. (4%). There was a significant reduction in the average percentage of positive isolation during the last 5 years of the study period as compared to the first 5 years (15.0% vs. 7.2%; r = -0.92, p = 0.0001). EPEC and Shigella spp. were less commonly isolated in the last five years compared with the first five years of the study (6% vs 21% p < 0.001 for E. coli; 7% vs 22%, p < 0.001 for Shigella spp.). This information is important for public health education in reducing the incidence of childhood diarrhoea further, and in the selection of appropriate antimicrobials in the management of extra-intestinal complications of childhood diarrhoea.

The risk factors and modes of death following acute diarrhoeal illness in children admitted to University Hospital, Kuala Lumpur between 1982 and 1997 were studied retrospectively. Among 4,689 cases of acute gastroenteritis admitted, ten deaths were noted. The case mortality rate was 2.1/1000 admissions. All deaths were infants below one year, with eight females and two males. Acute renal failure and acute pulmonary oedema were common preceding events. Female sex, infants less than twelve months, the presence of hyper or hyponatraemia and moderate to severe dehydration on admission were risk factors for deaths.

Two hundred and ninety five children admitted with acute gastroenteritis from January 1, 1996 to December 31, 1996 to the Paediatric unit, University of Malaya Medical Centre, Kuala Lumpur, were reviewed. Eighty-nine percent of children received treatment before admission. Information regarding the type of treatment received were available in 152 (52%) cases. Eighty percent of them were prescribed medications, 40% were prescribed glucose-electrolyte mixtures, and 13% were advised a change of formula. Only 18 children (12%) were advised to take glucose-electrolyte mixtures alone. The four most common prescribed drugs were: antibiotics (43%), antipyretics (39%), antidiarrhoeal agents (30%), and antiemetics (24%). The use of antibiotics, antiemetics and antidiarrhoeal drugs for children with acute gastroenteritis among primary care doctors appears to be common. The use of glucose-electrolyte mixtures was uncommon.

From October 1996 to March 1997, 31 children with febrile convulsions were admitted to the University Hospital, Kuala Lumpur. Human Herpesvirus 6 (HHV 6) was virologically and/or serologically confirmed to be the cause of the febrile episode in 5 of these children (16.1%). Age, sex and other associated clinical features (diarrhoea, cough, running nose and type of seizure) were not useful in differentiating cases of febrile convulsion due to HHV 6 from those of other aetiology. However, uvulo-palatoglossal junctional ulcers were noted in children in whom the cause of the seizure could be attributed to HHV 6 but not in the remaining cases in the study group. HHV 6 DNA was detected in peripheral blood mononuclear cells from all patients with febrile convulsions attributed to HHV6, and in patients shown serologically to have already been exposed to the virus by nested polymerase chain reaction amplification. Only genotype HHV 6B was detected from patients with seizure due to HHV 6 but both genotype 6A and 6B were detected in the remaining cases studied.