A six year old Chinese boy with relapsed Acute Promyelocytic Leukaemia (APML) failed to respond to reinduction with Daunorubicin and Cytarabine infusion. He was successfully treated with all trans-Retinoic Acid (45 mg/m2/day) orally. After four weeks of treatment, he was in complete remission. The side effects of all trans-Retinoic Acid were negligible.
Continent pouch ileostomy is fashioned for patients who need a proctocolectomy. It is usually indicated for cases of ulcerative colitis and familial adenomatous polyposis where the anal sphincter can no longer maintain normal function or has to be removed. A case of familial adenomatous polyposis with features of Gardner's syndrome is reported. The patient presented with carcinoma of the rectum. Abdominoperineal resection followed by completion pancolectomy was performed. A continent pouch ileostomy was fashioned for him. He resumed work as a labourer within six months. The pouch was troublefree, needed to emptied four to six times a day and was fully continent of fluid, flatus and faeces until his demise three years later from liver secondaries. Continent pouch ileostomy is a better alternative than permanent end ileostomy. All care should be taken to rule out the existence of Crohn's disease. It is proposed that continent pouch ileostomy should be offered to patients needing permanent ileostomy if the expertise is available.
The distribution of immunoregulatory cells in the peripheral blood of an individual has now been established as an important tool in helping the management of several diseases. It is necessary to set the normal ranges of these cells for the laboratory. We have undertaken in this study to establish the reference ranges for normal Malaysian adults. We found that the mean percentages of T cells, B cells, T Helper cells (CD4), T suppressor cells (CD8), NK cells and the ratio of CD4/CD8 were 70.91%, 11.38%, 38.15%, 37.76%, 17.45%, and 1.00 respectively. There was no significant difference between the sexes. In certain parameters, there was significant differences between Malay, Chinese and Indians. The Chinese and Indians were significantly different in the distribution of B cells and in the CD4/CD8 ratio. In the case of CD4 and NK cells, the Indians were different from the other two groups.
G6PD deficiency is the most common human enzymopathy and affects 200 million people worldwide. To date more than 400 biochemical variants and at least 60 different point mutations in the G6PD locus have been discovered. In Malaysia the overall incidence of G6PD deficiency among males is 3.1%, being more prevalent among the Chinese and Malays and less common among the Indians. As part of our initial effort to characterise G6PD deficiency in the Malaysian population, we investigated 18 G6PD deficient Chinese male neonates for the G6PD mutation G-->T at nt 1376, a common mutation seen among the Chinese in Taiwan and mainland China. The mutation was detected by a PCR-based technique using primers that artificially create a site for restriction enzyme Xho I. We found 61% (11 out of 18) of the Chinese G6PD deficient male neonates positive for this mutation. Study of enzyme electrophoretic mobility in 7 of the cases positive for this mutation revealed three different patterns of mobility. 107% (5 out of 7), 103% (1 out of 7) and 100% (1 out of 7). This study shows that mutation G-->T at nt 1376 is a common allele causing G6PD deficiency in Malaysians of Chinese origin. The finding of different patterns of electrophoretic mobility among the 7 cases positive for 1376 G-->T mutation supports the notion that diverse biochemical variants may share the same mutation.
Surfactant protein A (SP-A) is one of the four known surfactant-associated proteins found in human lungs. It plays a major role in determining regulation of surfactant uptake and resecretion. Qualitative and quantitative deficiencies of SP-A may contribute to neonatal respiratory distress syndrome. The measurement of its level in amniotic fluid or neonatal tracheal aspirate may be useful in the assessment of replacement therapy using natural or synthetic surfactants. In order to develop an in-house immunoassay to detect the level of SP-A, we used a discontinuous sucrose density gradient to isolate SP-A from amniotic fluid. Polyacrylamide gel electrophoresis was carried out on the isolates with low molecular weight markers. We successfully isolated SP-A from 12 out of 31 samples of amniotic fluid. The isolates were found to be relatively pure and have a molecular weight of about 35 kD. The isolated SP-A were used as immunogens to raise antibodies in rabbits for the immunoassay.
An in-house enzyme-linked immunoabsorbant assay (ELISA) for SP-A was successfully developed using in-house polyclonal anti SP-A and a commercial polyclonal anti-rabbit immunoglobulin horseradish peroxidase conjugate system. The standard curve, generated by using 50 ng of SP-A to coat the plate and 1:500 dilution of polyclonal anti SP-A as a primary antibody, was linear for concentrations of SP-A ranging from 4 micrograms/l to 4000 micrograms/l and reproducible. Results of recovery study of SP-A from a known sample of tracheal aspirate ranged from 94%-114%. Intra- and inter-assay coefficients of variations were 2.7% and 5.6% respectively for a known sample of tracheal aspirate. Interference study showed that tracheal aspirate did not interfere with the assay. The assay developed was intended to be used for SP-A measurement in tracheal aspirates obtained from neonates with and without respiratory distress syndrome.
A young primigravida presented with postpartum haemorrhage with no apparent cause following a low forceps delivery. She was extremely pale with gross hepatosplenomegaly. Hysterectomy was performed following three episodes of disseminated intravascular coagulation. Investigations revealed an extremely high platelet count with poor aggregatory function. A diagnosis of primary thrombocythaemia was made.
Bleeding gastroesophageal varices is associated with a high morbidity and mortality. Forty-four cases of bleeding gastroesophageal varices were treated at the Department of Surgery, Universiti Kebangsaan Malaysia, General Hospital, Kuala Lumpur over four and a half years. Thirty-two of them had liver cirrhosis. Hepatitis B infection was noted in 13 and alcoholic abuse was present in 14 patients. Five patients had associated hepatoma. Thirty-four percent had gastric fundal varices and a third of these bled from them. A total of 179 endoscopic injection sclerotherapy sessions were performed averaging 4 per person. Rebleeding rate was 4% and mortality was high (50%) in these cases. It was concluded that injection sclerotherapy is a safe and effective means of controlling bleeding oesophageal varices. Operative surgery was employed in those who rebled after injection and would be considered in those in Child's A.
In dengue shock syndrome, an acute increase in capillary permeability results in leakage of plasma into the interstitial space. Pleural effusion is commonly seen in dengue shock syndrome. We report three cases of dengue-associated adult respiratory distress syndrome (ARDS) in children, in all of whom dengue haemorrhagic fever, presenting with grade 3 or grade 4 dengue shock syndrome with disseminated intravascular coagulopathy, was confirmed. The criteria for the diagnosis of ARDS were based on the expanded definition of ARDS by Murray et al. Treatment consisted of fluid resuscitation, correction of coagulopathy and mechanical ventilation. All three children had multi-organ impairment, but it was more severe in the two who died. The one survivor was well at discharge.
The use of the colloidal-gold technique in electron microscopy immunocytochemistry has provided important information on the in situ localisation of intracellular antigens. We have developed a post-embedding technique for prolactin localisation on resin-embedded human pituitary tissue sections by the use of the protein-A gold conjugate. Human pituitary tissue obtained at autopsy was processed for electron microscopical study without post-osmication and then embedded in Epon. The indirect immunoperoxidase method was used for light microscopical targetting of lactotroph cells for subsequent electron microscopical antigen localisation. Ultra-thin sections were labelled with human anti-human prolactin followed by protein-A gold conjugate. Specific labelling was observed over secretory granules with a density of 15-30 particles per granule, as determined by the Quantimet 570 image analysis system. This technique provides a means of studying the pathophysiology of hormonal secretion at ultrastructural level and can be a useful tool in diagnostic and research investigations.
Computed tomography of the brain was performed in thirty schizophrenic patients in the age group of 19-45 years and thirty controls matched for age and sex The size of lateral ventricles, the width of the third ventricle and the sylvian fissure were measured in the CT scans. On group comparison, we found that the size of lateral ventricles expressed as ventricular brain ratio, and the width of the third ventricle and sylvian fissure were significantly greater in the schizophrenic patients (P< 0.001) as compared to the control group. No correlation was observed between the duration of illness and the cerebral findings. There was no significant difference between the group of schizophrenic patients with a history of having been treated with electro-convulsive therapy and the group without such a history.
An in-house method which utilizes 14C-thymidine as a substrate was used to assay deoxythymidine kinase in serum. The method is sensitive enough to detect normal levels of serum deoxythymidine kinase and the assay procedure also enables rapid handling of multiple samples. With a total reaction volume of 60 ul, the enzyme reaction was found to be linear with concentrations for up to 650 U/L of TK activity. On studying serum deoxythymidine kinase (s-TK) activity with incubation time, there was a proportional increase in activity with the length of incubation time. "Within-batch" precision showed a coefficient of variation (CV) of 4.7% for serum with extremely high s-TK levels and a CV of 8.8% for serum with normal s-TK levels. S-TK showed a CV of less than 16.0% in its activity when stored at -8 degrees C and at -20 degrees C. The normal reference range obtained for s-TK activity was 8.6 +/- 7.5 U/L.
Immunophenotyping of acute leukaemias has become an important diagnostic tool in haematology laboratories as it is now well recognised that the presence of certain surface markers has prognostic significance. In 1988, we experimented with the alkaline phosphatase anti-alkaline phosphatase (APAAP) method for immunophenotyping of leukaemic cells in our laboratory. 48 cases of peroxidase-negative acute leukaemias were studied. Our study showed that 2 peroxidase-negative cases carried myeloid surface markers, 44% were negative for the markers studied and 5% were unclassified due to technical problems. We concluded that the APAAP method is a useful technique for demonstrating cell markers in leukaemic cells as the reaction is reddish and usually intense. We failed to demonstrate surface markers in 44% of the cases probably because of the choice of a limited panel of monoclonal antibodies.
62 cases of polycystic ovary syndrome (PCO) were reviewed with regards to their clinical and endocrine features. The subgroup of patients with acanthosis nigricans (AN) was further studied in detail. The prevalence of the syndrome was significantly higher in the Indian (35.5% of cases). Obesity, AN, hirsutism, non-insulin dependent diabetes mellitus (NIDDM) and raised level of serum testosterone were present in 77.1%, 74%, 79%, 21% and 48% of the cases respectively. Patients with AN was associated with higher body mass index, serum testosterone level, and prevalence of hirsutism and NIDDM than patients without AN. These observations are in keeping with the hypothesis that hyperinsulinemia may be of importance in the pathogenesis of a sub-group of PCO associated with insulin resistant states.
Clinical studies were carried out on mild Indian sickle cell anaemia in Malaysia, and genetic and fertility studies were carried out on 101 families with and without sickle-cell haemoglobin (Hb S). The Indian sickle cell anaemia patients reached adulthood, and pregnancies and deliveries were uneventful without blood transfusion. There was no foetal wastage and the number of children produced was not significantly different from that in families without Hb S. 28 Indian patients hospitalized with Plasmodium falciparum malaria infection were also examined for their beta S genotype. P. falciparum malaria infection occurred much more frequently in individuals without Hb S than in Hb S carriers.
The Contraves Autolyzer 801 is a 20 parameter cell counter that operates on the principle of impedance. This evaluation study found that the within-batch precision for 5 of its parameters--WBC, RBC, Hb, Hct and Plt--had coefficients of variation below 5%, while the counts for red cell parameters were reproducible for up to 3 days. The analyser exhibited good linearity for all the parameters. When compared with 2 other counters--the Coulter S-Plus and the Coulter M350, some degree of proportional and constant bias due to unsuitable calibration were detected in the WBC, Hct and Plt parameters. Otherwise it showed generally good correlation in all the parameters and also with the manual PCV method. Carry over was negligible in the red cell parameters. The Contraves Autolyzer 801 is easy to operate and readily accepted by operators.
In a prospective study of 300 infants with acute gastroenteritis 150 infants had enteropathogens in the stools, 58 being due to rotavirus, 130 to adenovirus, 32 to Sahnonella, 18 Shigella and 29 E. coli. Hypernatraemic dehydration was present in 11% and acquired carbohydrate intolerance in 30% of the infants. Protracted diarrhoea was observed in 8% of infants and was commoner in the bacterial than viral group. The study shows that clinical features and simple blood tests cannot be used as reliable indices of predicting the aetiology of AGE. Despite the diverse aetiology of acute gastroenteritis, rehydration by the oral or intravenous route remained the mainstay of therapy.
Keywords: Kuala Lumpur, university hospital,