Over a three-year-period, 310 babies with prolonged jaundice admitted to GHKL were studied, to determine the incidence of alpha-1-antitrypsin deficiency as a cause of the problem. Ninety-two babies (29.7%) were found to be alpha-1-antitrypsin deficient. The percentage incidence was found to be highest in Indians (33.3%), followed by Malays (31.9%) and Chinese (26.7%). There was a male preponderance with a M:F ratio of 1.6:1. Most of these babies presented at the hospital at the age of more than two weeks but less than one month. Apart from the problem of prolonged jaundice and alpha-1-antitrypsin deficiency, 2 had associated bleeding problems, 11 associated infections and 3 respiratory problems. Two babies had clinical features of Down's syndrome, 2 had G6PD deficiency and 1 had congenital hypothyroidism. AST, ALT and ALPO4 were high in 20, 26 and 3 babies respectively.
A study of 1,437 unselected febrile patients in rural Malaysia yielded a diagnosis of leptospirosis in 86 (6.0%). The clinical syndrome was mild to moderate in all cases, jaundice was observed in only 2 (2.3%) and no deaths were documented. The diagnosis was not clinically obvious in most cases, and it is apparent that many infections must be going unnoticed at present.
A descriptive study of full-term neonates with jaundice was carried out to determine factors affecting severity of neonatal jaundice for those infants presenting to the hospital from their homes. Severe jaundice (serum bilirubin >=250 µmol/l) was significantly more likely in infants whose mothers consumed traditional herbs during the postpartum period (p<0.001) and if the jaundice was first detected by the parents or relatives rather than by medical personnel (p<0.05). In addition, the interval between detection of jaundice and presentation to hospital was significantly longer in jaundice that was first detected by parents compared to those detected by medical personnel. In conclusion, factors affecting severity of neonatal jaundice for infants who present from home could be influenced by the socio-cultural practices of maternal postpartum use of herbs, the ability of parents to detect jaundice and the urgency of tlie parents in seeking medical treatment once the jaundice was detected. Further studies need to be done to explore the association of these factors with neonatal jaundice more specifically. KEYWORDS: Hyperbilirubinaemia, socio-cultural practices, herbs.
Study site: Paediatric Institute, Kuala Lumpur Hospital, Malaysia
A 55-year-old immunocompetent woman was presented with features of obstructive jaundice and a clinicoradiological picture suggestive of a hilar cholangiocarcinoma (Klatskin tumour). However, caseating granulomatous lesion associated with miliary nodules were revealed intraoperatively. The lesion responded to standard antituberculous therapy. This unusual presentation highlights the considerable diagnostic challenge in such case.
Tatumella ptyseos, the type species for the genus Tatumella, is a newly established member of the Family Enterobacteriaceae. It is a Gram-negative, oxidase negative, fermentative rod that grows on Mac Conkey agar. This first isolate was obtained from the blood culture of a neonate having neonatal jaundice with presumed sepsis. The organism was in vitro sensitive to Gentamicin, Chloramphenicol, Cotrimoxazole and Ampicillin. The patient was treated with Ampicillin and Gentamicin and recovered uneventfully.
The trait condition for hemoglobin Tak, was found in a 4-day old newborn Malay who suffered from severe neonatal jaundice. The beta chain of the abnormal hemoglobin was elongated by 11 residues at the C-terminus and had the same structure as reported for Hb Tak. The mother was heterozygous for this abnormal hemoglobin, the father was normal. The mother and child, 4 years later, did not show clinical or hematological symptoms except definitely increased resistance of their erythrocytes to hypotonic saline solutions and slight anisopoikilocytosis. The abnormal gene in the two reported Thai families and in our Malay family may have the same origin.
There are significant differences in the prevalence and severity of neonatal jaundice among various populations. Recently, it has been reported that a mutation of the UGT1A1 gene, glycine to arginine at codon 71 (G71R), is related to the development of neonatal jaundice in East Asian populations. However, whether the G71R mutation contributes to the high incidence of neonatal jaundice in different Asian populations remains unknown. The authors screened for this mutation in the Javanese-Indonesian and Malay-Malaysian populations.
Home phototherapy treatment has been available in the Klang Valley (comprising Petaling ]aya, Kuala Lumpur; Shah Alam and Klang) since 2003. This study was conducted to create awareness of the existence of home phototherapy and its usage in Malaysia. This was a retrospective study using 1297 informed consent forms that parents had to read and sign prior to the commencement of home phototherapy. lt was found that the majority ofthe babies was males (41.2%), at or over 36 weelds gestation (97.2%), from areas in Selangor (57.4%), fully breastfed (53 %) and referred by doctors (98%). The mean age of the babies at initiation of lwme phototherapy was 6. 7 days. The mean bilirubin level at the start of home phototherapy was 243.8 umoVL and the mean bilirubin level at the end of home phototherapy 5 was 139.3 umoVL. The mean decrease in bilirubin level was 103.12 umoVL and the mean number of days of usage was 3.5 days which is a daily decrement of about 29 umoVL (29.46 + 13.8). Home phototherapy remains a viable clinical option for full term babies with physiological jaundice.
A study was carried out of 332 babies suffering from severe neonatal jaundice who were admitted to the General Hospital, Kuala Lumpar, Malaysia. Of the 332 neonates, 51 were premature and 281 were full-term babies, 178 (110 Chinese, 58 Malay, 9 Indian and 1 European-Pakistani) had bilirubin levels of 20 mg% or higher, requiring exchange blood transfusion. Of the Chinese neonates, 23 (20.9%) had G6PD deficiency, 9 (8.2%) had Hb Bart's and 2 (1.8%) had an abnormal haemoglobin, one Hb Q and one fetal variant. Among the Malay infants, 10 (17.2%) had G6PD deficiency, 7 (12.1%) had Hb Bart's and 10 (17.2%) had abnormal haemoglobins (four had Hb E trait, one had Hb K and Bart's in addition to Hb E, three had Hb CoSp with Hb Bart's, one had Hb Q and one Hb Tak). One of the nine Indian neonates had G6PD deficiency and one had Hb S trait. The one European-Pakistani baby was a carrier of Hb D Punjab. In addition to G6PD deficiency, abnormal haemoglobins seem to have contributed to the high incidence of severe neonatal jaundice in Malaysia. The mean activities of GP, GR and GR after stimulation with FAD were higher, while the mean activity of PK and mean level of reduced glutathione were lower than in normal cord bloods. The percent increase of GR after FAD stimulation was significantly lower; fewer in this group had increases above 20% than in normal cord blood. The possible significance of the findings is discussed.