Prevalence of growth and endocrine disorders in Malaysian children with transfusion-dependent thalassaemia

INTRODUCTION@#Endocrine dysfunction due to iron overload secondary to frequent blood transfusions is a common complication in children with transfusion-dependent thalassaemia (TDT). We ascertained the prevalence of endocrine dysfunction in children with TDT seen in a hospital setting in Malaysia.@*METHODS@#We reviewed all patients with TDT who had ≥ 8 blood transfusions per year. Patients who had a history of stem cell transplantation, concurrent autoimmune diseases or were newly diagnosed to have TDT were excluded. Standard diagnostic criteria were used in the diagnosis of various endocrine dysfunctions.@*RESULTS@#Of the 82 patients with TDT, 65% had at least one endocrine dysfunction. Short stature was the commonest (40.2%), followed by pubertal disorders (14.6%), hypoparathyroidism (12.3%), vitamin D deficiency (10.1%), hypocortisolism (7.3%), diabetes mellitus (5.2%) and overt hypothyroidism (4.9%). Subclinical hypothyroidism and pre-diabetes mellitus were seen in 13.4% and 8.6% of the patients, respectively. For children aged 10 years of age. Close monitoring for endocrine dysfunction and hormonal therapy is essential to prevent long-term adverse outcomes.

Fatal infections in older patients with inflammatory bowel disease on anti-tumor necrosis factor therapy

Anti-tumor necrosis factor (anti-TNF) is highly effective in inflammatory bowel disease (IBD); however, it is associated with an increased risk of infections, particularly in older adults. We reviewed 349 patients with IBD, who were observed over a 12-month period, 74 of whom had received anti-TNF therapy (71 patients were aged <60 years and 3 were aged ≥60 years). All the 3 older patients developed serious infectious complications after receiving anti-TNFs, although all of them were also on concomitant immunosuppressive therapy. One patient developed disseminated tuberculosis, another patient developed cholera diarrhea followed by nosocomial pneumonia, while the third patient developed multiple opportunistic infections (Pneumocystis pneumonia, cryptococcal septicemia and meningitis, Klebsiella septicemia). All 3 patients died within 1 year from the onset of the infection(s). We recommend that anti-TNF, especially when combined with other immunosuppressive therapy, should be used with extreme caution in older adult patients with IBD.

Health-Related Quality of Life in Children with Biliary Atresia Living with Native Livers

<p><b>INTRODUCTION</b>This study aimed to quantify and investigate factors affecting the health-related quality of life (HRQoL) in children with biliary atresia (BA) living with their native livers.</p><p><b>MATERIALS AND METHODS</b>A cross-sectional study on the HRQoL using the PedsQL4.0 generic core scales in children with BA aged between 2 to 18 years followed up at the University Malaya Medical Centre (UMMC) in Malaysia was conducted. Two groups, consisting of healthy children and children with chronic liver disease (CLD) caused by other aetiologies, were recruited as controls.</p><p><b>RESULTS</b>Children with BA living with their native livers (n = 36; median (range) age: 7.4 (2 to 18) years; overall HRQoL score: 85.6) have a comparable HRQoL score with healthy children (n = 81; median age: 7.0 years; overall HQRoL score: 87.4; P = 0.504) as well as children with CLD (n = 44; median age: 4.3 years; overall score: 87.1; P = 0.563). The HRQoL of children with BA was not adversely affected by having 1 or more hospitalisations in the preceding 12 months, the presence of portal hypertension, older age at corrective surgery (>60 days), a lower level of serum albumin (≤34 g/L) or a higher blood international normalised ratio (INR) (≥1.2). Children who had liver transplantation for BA did not have a significantly better HRQoL as compared to those who had survived with their native livers (85.4 vs 85.7, P = 0.960).</p><p><b>CONCLUSION</b>HRQoL in children with BA living with their native livers is comparable to healthy children.</p>

Immune response in infants after universal hepatitis B vaccination: a community-based study in Malaysia

<p><b>INTRODUCTION</b>This study aimed to assess the immune response in infants who received the three-shot hepatitis B vaccine in Malaysia.</p><p><b>METHODS</b>Consecutive infants born between March 2002 and April 2010 who received three doses of hepatitis B vaccine at a community clinic in Malaysia were enrolled in the study. Screening for hepatitis B surface antigen (HBsAg) and antibody against HBsAg (anti-HBs) was performed after the completion of primary immunisation, at approximately one year of age.</p><p><b>RESULTS</b>A total of 572 infants (median age 9.3 ± 2.7 months; range 6.3-48 months) were screened for immune response to hepatitis B vaccination - 553 (96.7%) infants had adequate levels of anti-HBs (≥ 10 IU/L). Of the 440 mothers whose HBsAg status was known, 14 (3.2%) were positive for HBsAg. None of the 14 infants who were born to HBsAg-positive mothers were positive for HBsAg, and all but one infant had anti-HBs level ≥ 10 IU/L. Gender, gestational age and maternal HBsAg status were not found to significantly affect the subsequent immune response in infants following vaccination.</p><p><b>CONCLUSION</b>The proportion of Malaysian mothers who are positive for HBsAg remains high. The three-shot hepatitis B vaccine, given as part of universal vaccination against hepatitis B, provides adequate anti-HBs in the vast majority of infants in a community setting in Malaysia.</p>

Regular paracetamol in severe dengue: a lethal combination?

An eight-month-old female infant with severe dengue disease, who was repeatedly given therapeutic paracetamol for severe dengue, developed fulminant liver failure with encephalopathy, gastrointestinal haemorrhage and severe coagulopathy. She responded to supportive measures and N-acetylcysteine infusion. This case highlights the potential danger of administering repeated therapeutic doses of paracetamol in childhood severe dengue disease with hepatitis.

Impact on parents during hospitalisation for acute diarrhoea in young children

<p><b>INTRODUCTION</b>This study aimed to determine the emotional impact on parents of young children who require hospitalisation for acute diarrhoea (AD), and the disruption of daily activities experienced and costs incurred by them.</p><p><b>METHODS</b>A prospective study was conducted on children below two years of age with AD admitted to two urban hospitals in Malaysia. Parents were interviewed on the emotional impact and disruption of daily activities experienced by them, as well as the extra costs incurred as a result of the hospitalisation of their child.</p><p><b>RESULTS</b>The parents of 85 children (median age 13 months; boys n = 58, 68%; girls n = 27, 32%) were recruited for the study. The proportions of parents who reported that they were very worried about the symptoms of diarrhoea, vomiting and fever in their child were 82%, 83% and 78%, respectively. Parents also reported being upset, helpless, mentally and physically exhausted, and having experienced loss of sleep and disruption of daily routine during the hospitalisation of their child (median four days). The median extra cost (including out-of-pocket cost and loss of income) incurred by parents as a result of the hospitalisation was USD 252.86, which constituted 16% of the combined monthly family income.</p><p><b>CONCLUSION</b>Although short, AD-related hospitalisation in young children has considerable emotional impact and creates significant distress, in addition to causing significant financial burden for parents.</p>

Clinical features differentiating biliary atresia from other causes of neonatal cholestasis

<p><b>INTRODUCTION</b>This study determined any clinical features which may help to differentiate biliary atresia (BA) from other causes of neonatal cholestasis (NC).</p><p><b>MATERIALS AND METHODS</b>A prospective and observational study was conducted on consecutive infants with NC referred to the University of Malaya Medical Centre, Malaysia, between November 1996 and May 2004.</p><p><b>RESULTS</b>The 3 most common causes of cholestasis among the 146 infants with NC studied were idiopathic neonatal hepatitis (n = 63, 43%), BA (n = 35, 24%) and congenital cytomegalovirus hepatitis (n = 13, 9%). Common clinical features at presentation were jaundice (100%), hepatomegaly (95%), splenomegaly (52%) and pale stools (47%). Three clinical features noted to be sensitive for BA were the presence of acholic or variably acholic stools on admission, a liver which was firm/hard in consistency and a palpable liver of ≥4 cm (sensitivity of 77%, 80% and 94%, respectively), but the corresponding specificity was poor (51%, 65% and 39%, respectively). The stools of 2 children with BA were pigmented initially but became acholic subsequently.</p><p><b>CONCLUSIONS</b>We did not find any single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of NC. Repeated inspection of stools colour is necessary as occasionally, patients with BA may have initial pigmented stools. Biochemical assessment and imaging studies are important in the assessment of any infant with NC.</p>