Wilson's disease revisited in the tropics.

The clinical features and investigations of 17 patients were analysed. Thirteen of them were Chinese and the rest Indians. Their ages at presentation ranged from 8 to 63 years (mean 18.35 years). Thirteen patients (76%) were symptomatic; 8 with predominantly hepatic manifestations and 5 with neurological features. Four were asymptomatic siblings. At diagnosis, however, 10(59%) had features of liver involvement singly, 3 (18%) had neurological involvement alone and 4 (27%) had mixed presentations. Family histories were available in 15 patients; 26.9% of siblings had Wilson's Disease. Serum ceruloplasmin was low in 82% of the patients. 24-hour urinary copper was measured in 16 patients and was raised in all of them. About half the patients (41%) had evidence of concomittant renal tubular dysfunction with hypouricaemia and aminoaciduria. Three patients (18%) had joint involvement at presentation. All 17 patients were treated with Penicillamine. Complications due to therapy included pemphigus in one and toxic epidermal necrolysis and later a lupus like syndrome in another. The features of clinical improvement included fading of K-F rings, improvement of neurological signs and the normalisation of serum transaminases. One patient developed primary hepatocellular carcinoma 5 years after presentation. Delay in diagnosis was encountered in half of the patients reviewed. Being a treatable condition, Wilson's Disease, although rare, should always be thought of in patients with haemolysis, liver diseases or extrapyramidal disorders.

Combined team management of diabetes mellitus in pregnancy.

225 women with diabetes in pregnancy were managed by a team of obstetricians, physicians (endocrinologists) and paediatricians from the National University of Singapore. A protocol of management was formulated and followed. The incidence of 1.1% or 1 in 90 pregnancies was found, with significantly higher incidence in Indians and lower in Malays. There were 37 established diabetics and 188 diagnosed during pregnancy. Of these (188), 74 were gestational diabetics. All the women were treated with Insulin and Diet or Diet alone. 177 (79%) were treated with Insulin and Diet. Blood sugar profiles were done for monitoring diabetic control. 72.8% of the women were between para 0 and 1 and 85.2% between the ages of 20 and 34. 72.5% of the women delivered at 38 weeks gestation or later. 48.9% went into spontaneous labour, 32.4% were induced and 18.7% had elective caesarean section. 62.2% of the women had labour of less than 12 hours. The overall caesarean section rate was 41.7%. There were 3 stillbirths and 2 neonatal deaths. The perinatal mortality rate was 2.2%. Thirteen babies had congenital malformations (5.8%). 77.8% of the babies had Apgar score of 7 or more at 5 minutes after delivery. 79.1% of the babies weighed between 2.5 kgm and 3.9 kgm. Pre-eclamptic toxaemia was the commonest complication in pregnancy followed by Urinary Tract Infection and Polyhydramnios. Postpartum complications in the mother were confined to 14 women (6.2%), and wound infection or breakdown was the commonest cause.

Extremes of foetal birthweight for gestation in infants of diabetic mothers.

100 patients were registered at the Diabetic Clinic in 1981, where they were managed by a team of physician, obstetrician and paediatrician, based on a preset protocol. Only 92 patients were eventually analysed. The study showed a 1.3% incidence of pregnancies complicated by diabetes mellitus. The mean birthweights of infants of both gestational and established diabetics were heavier than that of the general population by race and gestation. 25% of the 92 infants of diabetic mothers have birthweight exceeding the 90th centile of population. Further division of the 92 patients into the "true gestational" diabetics, as shown by an oral glucose tolerance test performed 6 weeks post-natally, also showed a 25% incidence of macrosomia. Late antenatal booking, delayed detection of abnormal glucose tolerance and treatment attributed to the high incidence of macrosomia. Only one infant had birthweight below the tenth centile. There were no perinatal mortality in the 92 patients studied. Macrosomia is a common complication in infants of diabetic mothers despite a physician-obstetrician joint-care system. Also, the risk of having macrosomia amongst gestational diabetics is high.

Diabetic amputees in Singapore.

We studied the clinical features of 262 consecutive patients who had 289 limb amputations in the University Department of Orthopaedics, Singapore General Hospital from January, 1978 to June, 1980. Of these, 171 (59.2%) were done for diabetic complications, 43 (14.9%) for trauma and 27 (9.3%) for atherosclerosis. The mean age of the three groups were 65 +/- 10 (S.D.), 32 +/- 17, and 75 +/- 8 years respectively. The indications for amputation in diabetics were gangrene (36%), ulcer (25%), infection (14%), gangrene and ulcer (11%), gangrene and infection (8.2%), and ulcer and infection (6%). The majority of diabetics were treated with oral agents (80.2%) and diet (6.4%), with 13.4% on insulin. Most patients had their diabetes for less than ten years-38.6% less than five years and 30.7% six-ten years. The mean known duration of the lesion was 1.13 +/- 1.42 months, with 38.7% less than one week. To determine the prevalence of peripheral vascular disease in diabetics admitted for amputation, the ankle/arm systolic BP ratio at rest and post-exercise were determined. In the affected leg, 16/19 patients had decreased ankle/arm systolic BP ratio. In another study, 23/26 diabetic amputees had decreased ankle/arm systolic BP ratio in the remaining leg. These data suggest that diabetes is the most common indication for amputation in this study. Most of these patients are Type II diabetics and have peripheral vascular disease.

Successful angioplasty of renal artery stenosis due to aorto-arteritis.

A 24-year-old Chinese female who suffered from hypertension and acute myocardial infarction was found to have aorto-arteritis on angiographic examination, with total occlusion of her right coronary artery and subtotal occlusion of her right renal artery. Transluminal angioplasty of her right renal artery was successfully performed and this normalized her blood pressure from 165/105 to 135/85 mm Hg.

Risk factors for screening potential diabetics in pregnant Chinese patients.

An intensive screening programme for potential diabetics in pregnancy was conducted in the University Department of Obstetrics and Gynaecology, Kandang Kerbau Hospital, Singapore, from June 1978 to December 1980. All patients with one or more risk factors for potential diabetes underwent an oral glucose tolerance tests (OGTT) with 50 g glucose loads. An abnormal OGTT was defined as one in which the second hour venous plasma glucose value was 7.8 mmol/1 (140 mg%) or more. In a group of 789 Chinese patients aged between 20 and 40 years, the overall detection rate of abnormal OGTTs was 15.5%. A significantly high proportion of abnormal OGTTs was found in patients with a direct family history of diabetes associated with random glycosuria during pregnancy and those with a previous history of 2 or more consecutive spontaneous abortions. However, in view of the relatively low detection rate of abnormal OGTTs in pregnant potential diabetics, a preliminary blood screening test would be useful to select those patients who require full OGTTs.

Cardiac amyloidosis--diagnosis by 2-dimensional echocardiography.

In the past, the precise diagnosis of amyloid cardiomyopathy without resort to invasive techniques has been difficult. Recent experience with 2-dimensional echocardiography in cardiac amyloidosis has suggested that there are characteristic, and perhaps unique, features that are regularly imaged. We describe a case where usage of this non-invasive technique allowed accurate definition of cardiac involvement by amyloid.