The objective of the study is to determine the proportion and different types of birth defects among the children born in Hospital Kuala Lumpur. A cross-sectional study was conducted for a period of 18 months where all consecutively born infants, dead or alive were included. There were total of 34,109 births recorded during this period. The proportion of birth defects in Hospital Kuala Lumpur was 3.1% (n = 1056). The commonest involved were the hematology system, (157.7 per 10,000 births), the central nervous system, genitourinary system and chromosomal anomalies. The proportion was significantly higher in males and in the Chinese (p < 0.001). The commonest abnormalities are Glucose 6 Phosphate Deficiency (157.7/10000), Down's syndrome (12.6/10000), thalassaemia (8.8/10000), cleft lip and/or palate (7.6/10000) and anencephaly (7.3/10000). Neural tube defect is common and ranked second after G6PD deficiency. There is a need for a birth defect registry to assess the extent of the problem in Malaysia.
The use and reasons for use of Complementary Medicine (CM) amongst hypertensive patients attending the Hypertension/ Diabetes/ Asthma Clinic in Greentown Health Clinic, Ipoh was assessed. One hundred and twenty patients were selected by systematic random sampling (1:5) over a 2-week period commencing 26/04/04. Data was obtained from interviews, questionnaires and medical records. Twenty seven percent were on CM. Most commonly used CM was herbal medicine. Majority of those using CM for BP control were Malays. The Chinese and Indians were using CM mainly for other health problems. Ninety six percent were using both CM and conventional therapy concurrently. Therefore doctors should enquire about CM usage during patient assessment to prevent possible drug interactions.
An audit of hypertension management was done in October 2004 in nine general practice (GP) clinics. Two structure, ten process and two outcome indicators were assessed. Results showed that targets were achieved in only four indicators, i.e., weight recording (89%), BP monitoring (85.8%), follow-up interval not exceeding 6 months (87.9%) and mean diastolic BP (73.9%). The other indicators (hypertension registry, reminder mechanisms for defaulters, recording of smoking, height, fundoscopy, monitoring of lipid profile, blood sugar, ECG, renal function and achievement of target mean systolic pressure) showed adequacy percentages varying from 22.1 to 68.7. Out of the 1260 patients assessed, 743 (59%) achieved a mean BP < or = 140/90 (or < or = 130/80 mmHg with diabetes mellitus / renal insufficiency) in the last 3 recorded readings. There was a vast difference between individual clinics. Reasons for not achieving targets were discussed and remedial measures for implementation were recommended.
In the initial clinical examination of a child with ambiguous genitalia an accurate measurement of the corporeal length is needed. Most often the corporeal length is measured with a ruler from the symphysis pubis to the tip of the glans of a stretched penis. More recently, ultrasound has been successfully used to measure corporeal length. This study aimed to (i) establish normal values for corporeal length in normal male newborns using ultrasound measurement, (ii) compare these measurements to stretched corporeal measurements, (iii) compare the corporeal length of newborns of different races, and (iv) determine the relationship between corporeal length and birth weight, birth length and head circumference. This was a prospective study of 141 newborns. Ultrasound imaging was done in an oblique parasagittal plane such that the corpus could be included in a single image and measured, Stretched corporeal length was measured with the penis stretched alongside a wooden spatula and the length from the pubic bone to the tip of the glans was marked on the spatula and measured. By ultrasound measurement the mean corporeal length of the normal newborn was 3.18 +/- 0.56cm. There was no significant difference in the mean corporeal length when determined by ultrasound and by stretched corporeal measurement. There was no significant difference in the mean corporeal length of the different races when the length was determined by either ultrasound or stretched corporeal measurement. There was a positive correlation between ultrasound length and birth weight and birth length. However, there was no correlation between ultrasound length and head circumference. There was no significant correlation between the stretched length and either birth weight, birth length or head circumference.
Chronic ruptures of the extensor mechanism of the knee are uncommon injuries, and previously reported literature assumes the presence of an intact patella for repair. We present a case of chronic rupture following patellectomy done twelve years previously. The defect in the extensor apparatus was extensive (18cm), and this was bridged using a large fascia lata graft from the opposite thigh, with reasonably successful results.
Vaginal defects can either be congenital or acquired due to tumor or trauma. The reconstructions are aimed in producing a good physical and functional result with the least donor site morbidity. The pudendal thigh flap is a sensate fasciocutaneous flap based on the terminal branch of the superficial perineal artery, the continuation of the internal pudendal artery. Although various flaps have been described for vaginal reconstruction, the pudendal thigh flap offers a very attractive alternative.
We describe a patient with HbE-beta thalassaemia and chronic hepatitis C virus infection (genotype 1a) who was treated successfully with peginterferon alfa-2b and ribavirin, following failure to respond to standard interferon and ribavirin therapy. She had sustained virological response for nearly 24 months after completing peginterferon alfa-2b and ribavirin therapy. Transfusion requirements were significantly increased during combination therapy due to ribavirin-induced haemolysis. The adverse effects of interferon were well tolerated. Combination therapy with peginterferon alfa-2b and ribavirin maybe a feasible treatment option for a subset of thalassaemia/HCV infected non-responders to standard interferon-based therapy.
Neurocysticercosis, infection of the central nervous system (CNS) by larvae of the pork tapeworm Taenia solium, is the commonest neuroparasitic infection in humans. However in countries as in Malaysia it poses a diagnostic problem as the disease in not seen amongst the local population; however with the arrival of immigrant workers, a number of cases have recently been diagnosed. There were 3 cases of neurocysticercosis reported in our centre over the last 5 years.
Large Vestibular Aqueduct Syndrome is a congenital malformation of the temporal bone characterised by early onset of sensorineural hearing loss and vestibular disturbance. Familial large vestibular aqueduct syndrome suggests autosomal recessive or X-linked inheritance and accounts for non-syndromic sensorineural hearing loss in these patients.
Hemangiopericytoma is a very rare angiogenic tumor. In the nasal cavity, it can be considered malignant. It occurs in various parts of the body but those in the nasal cavity account for only 5% of total cases. Less than 200 cases have been reported worldwide involving the nose and paranasal sinuses. Due to its rarity a proper line of management has not been established to tackle this tumour. This article highlights two cases of hemangiopericytoma (HPC), one in an adult and the other in a child, presenting as an intranasal mass.
Asymptomatic simultaneous thrombosis of abdominal aorta and inferior vena cava is a rare complication in advanced malignancy. We described an incidental finding of this clinical entity in our patient who presented with advance stage of malignant fibrous hystiocytoma of soft tissue and pathological fracture. The radiological evaluation with spiral computed tomography scan of abdominal aorta and inferior vena cava are presented and the subsequent management highlighted.
A thirty four year old female presented with upper and lower respiratory symptoms in the third trimester of pregnancy. After the delivery of a healthy baby, the symptoms progressed to involve multiple organ systems and eventually a diagnosis of limited Wegener's Granulomatosis (Carrington-Liebow syndrome) was made. The extremely rare combination of WG and pregnancy, especially the onset of disease in late pregnancy is discussed. The successful outcome of pregnancy even without treatment of WG is the highlight of the case.
We present a case of a foreign body which migrated to the maxillary ostia by mucociliary action from its initial location on the floor of the maxillary sinus where it was traumatically introduced. This report illustrates that a powerful mechanism of mucociliary action can cause relatively heavy objects within the maxillary sinus to migrate naturally to the sinus ostia against gravitational force.
Neonatal stridor resulting from intrinsic or extrinsic aberration in the upper respiratory tract often poses not only a diagnostic problem, but also a difficult airway and a dilemma as to the necessity / timing of surgical intervention. A 45 day old female child with increasing stridor since birth was managed by emergency intubation and CT scan followed by excision biopsy of the cystic left sided parapharyngeal mass via a transcervical approach. On histopathology, the excised specimen was reported as cystic salivary choristoma.
A 36 year-old Malay lady with diabetes mellitus in pregnancy and poorly controlled hypertension developed rapid progression of diabetic retinopathy from no retinopathy to florid proliferative retinopathy over three months in her right eye. She had subsequent loss of vision due to vitreous haemorrhage in the peri-partum period. She had good final visual acuity with quiescent retinopathy following pars planar vitrectomy. A similar course was avoided in the left eye by timely pan retinal photocoagulation.
The use of airway stents for the treatment of benign airway stenosis is increasingly advocated. However, the long-term safety and efficiency of these devices has not been established. We present a case of tracheal stenosis. which persisted despite open surgical and laser correction. The patient required tracheal stent insertion and is currently well with no respiratory difficulty. The use of metallic or silicon intraluminal stent remains appropriate in cases in which there is defined and relative short-term end point of treatment.
The differential diagnosis of a cystic lesion in the submandibular area can be difficult. We report a case of epidermal cyst of submandibular gland which is relatively rare compared to the commoner epidermoid cyst.
Salmonella osteomyelitis of the rib is a rare clinical entity. In our case, a muhidrug resistant Salmonella enterica serotype Typhi was isolated from an immuno-competent patient with osteomyclitis of the ribs, who was treated earlier with ciprotloxacin for typhoid fever. The patient was successfully treated for osteomyclitis with intravenous ceftriaxone.
We report a rare case of a giant extradural and intraabdominal ganglioneuroblastoma in a young Malay girl who presented to a paediatrician initially at 5 days of life with a palpable abdominal mass. Unfortunately, the parents refused any form of surgical intervention until the child was 3 years old. She subsequently underwent vascular embolisation followed by the removal of this large tumour both via the abdomen and through a laminectomy approach and subsequently refused chemotherapy. The c-myc amplication in this patient was absent and there were no chromosomal aberrations, During the 2 year folow-up the patient remained well, and ambulatory with no tumour recurrence.