A family demonstrating autosomal dominant thrombocytopenia is described. A 28-year-old Malay housewife was found to have a platelet count of 40 x 10(9)/l with a low mean platelet volume (6.8 fl) while being investigated prior to ovarian cystectomy. The bone marrow was consistent with immune thrombocytopenia but she failed to respond to appropriate therapy. Five siblings, one parent and one nephew have easy bruising and platelet counts of 39-82 x 10(9)/l. Platelet aggregation studies excluded a major functional defect. Survival of homologous platelets in the circulation was normal. Familial thrombocytopenias are rare but important to differentiate from the common acquired thrombocytopenias in order to spare the patient unnecessary treatments.
A case of non-fatal strangulation of the neck by rigging lines of a parachute during military training is presented. It is an unusual but potentially life-threatening injury. Probable factors leading to such injury are discussed.
A 26-year-old male presented with claw toes and Factor XIII deficiency. Correction for his deformity was undertaken. Pre, intra and post-operative transfusions of plasma and blood prevented any haemorrhagic complications.
A six-year-old boy, a known case of acute lymphoblastic leukaemia (ALL) on remission since 1991 presented with leukocoria and poor vision of the left eye for two days' duration. Examination revealed endophthalmitis in the left eye with raised intraocular pressure. Anterior chamber paracentesis with vitreous biopsy confirmed a diagnosis of ocular involvement. Further investigation revealed that he also had bone marrow and central nervous system relapse. Clinical manifestation and treatment modalities of ocular involvement in leukaemia are discussed.
Law enforcing authorities need to provide a scientific basis for the identification of any unknown individual. In recent years, dental records comparison has developed into one such credible method of confirming the identity of a deceased. This method is however restricted as dentists are not making and maintaining adequate records of their patients. Fortunately the advent of inexpensive cameras and print processing procedures has enabled the availability of ample antemortem photographs. Photographs in which a person expresses his/her teeth 'gleefully' has provided a sound scientific basis for the identification by comparing dental characteristics of the deceased.
A 36-year-old man presented with haemoptysis and his admission chest radiograph showed a large thin walled cystic lesion with an air-fluid level in the left lower lobe. The pathological diagnosis of the lesion, which was removed by a left lower lobe resection, was an intrapulmonary bronchogenic cyst.
Intensive chemotherapy has prolonged survival in cancer patients. Unfortunately it has also predisposed them to unusual infections because of their immunocompromised state. We report a case of fungal septicaemia caused by Geotrichum candidum, an imperfect yeast of low virulence in a young girl with acute lymphoblastic leukaemia. It was successfully treated with amphotericin B. The morphological characteristics of this fungus leading to its identification are described.
A 16-year-old female from Rantau Panjang, Kelantan reported having diarrhoea for three months. During this period, she lost 15 lb in weight and was treated with antibiotics and anti-spasmodic tablets with no improvement. Stool examinations by private laboratories revealed "worm-like eggs". She was treated for worms with mebendazole which helped to reduce the symptoms but not completely. The patient continued passing out the abnormal "worm-like eggs" which were later identified as pollen grains.
A patient on oral contraceptives over several years developed unilateral proptosis, haemorrhagic retinopathy and increase in intraocular pressure. An orbital vein venogram confirmed the diagnosis of right superior ophthalmic vein thrombosis. There was complete resolution of thrombosis and eye signs and symptoms with discontinuation of the oral contraceptive.
We report two patients who had cerebral malaria, heavy parasitemia, hyperbilirubinemia, hypercatabolism with rapid rises of blood urea and serum creatinine and acute renal failure. There was no evidence of intravascular hemolysis. Renal biopsy was consistent with acute tubular necrosis. Both patients responded to treatment with intravenous quinine and dialysis.
Herpes gestationis is a rare disease in pregnancy. A 35-year-old G3P2 presented during her third trimester with erythematous pruritic papular eruptions. Direct immunofluorescence of a skin biopsy confirmed the diagnosis of herpes gestationis. The patient responded well to predniosolone treatment.
This paper reviews the subject of tuberous sclerosis complex and presents data in eight cases of this condition, admitted to Hospital Universiti Sains Malaysia over a period of 8.5 years. The average age at presentation was 53 months. Seizures were the most common presenting feature. Male to female ratio was 3:1. Family history was present in four patients. All of the patients had one or more skin lesions. Six of them had retinal in six patients. Two patients had renal angiomyolipomas. A high index of hamartomas. Cardiac tumours were found in two patients. Multiple subependymal hamartomas were detected in six patients. Two patients had renal angiomyolipomas. A high index of suspicion and a detailed physical examination is required to diagnose this rare disorder.
Five hundred and sixty-two consecutive attenders at an urban general practice were studied using the Consumption Index which has been successfully used in a general hospital sample. Seventy per cent of Chinese, 11% of Malays and 42% of Indians have used or are currently using alcohol-a vast majority of them were social drinkers. Among those who ever drank, 6% of Chinese and 22% of Malays drank more than 14 units per week. The hypotheses that more Indians used alcohol and used it more heavily were not supported in this urban sample. Possible explanations for these findings and the limitations of this study are discussed.
The training of doctors in therapeutics has created interesting discussions internationally. A survey of senior hospital pharmacists currently practising throughout West Malaysia was embarked on during a recent postgraduate seminar. About sixty per cent said prescribing errors were common amongst doctors. Sixteen per cent of the prescribing errors were potentially serious. Most of the time errors were due to carelessness, lack of knowledge on drug action or a combination of both. Nearly 35% of prescribing errors were not acknowledged by doctors. Most doctors did not give reasons for not acknowledging pharmacists' intervention. About half (46.5%) of the respondents thought that doctors were not adequately trained in the use of drugs.
In view of controversial reports about the role of prelabour rupture of foetal membranes (PROM) in neonatal morbidity and to study the association of PROM with infections and meconium aspiration syndrome (MAS), a prospective case control study was conducted in a level II nursery of Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan. Of the 111 neonates with PROM studied, 18 developed clinical problems (septicaemia and other specific problems such as pneumonia, omphalitis, skin infection and MAS) while 5/56 of the control group developed similar problems. The difference between the two groups was not significant (p < 0.30). There was no neonatal death. It is concluded that PROM is not associated with neonatal morbidity. Neonates with this problem alone do not need to be admitted to the neonatal nursery.
A 66-year-old man sustained an injury to his right foot while gardening. Despite receiving tetanus toxoid one hour later and adequate wound toilet, he developed severe tetanus complicated with autonomic dysfunction six days later. He died 20 days after admission. This case shows that tetanus toxoid alone may not be sufficient to prevent tetanus in wounded patients. Careful consideration must be given to the immune status of the patient and to the nature of the wound sustained. Incompletely immunised patients or patients with unknown immune status who sustain a tetanus prone wound should be protected with both tetanus toxoid and tetanus immunoglobulin.