A 39-year-old man was diagnosed with von Hippel-Lindau syndrome, which was associated with retinal haemangioblastoma, cervical cord haemangioblastoma and bilateral renal cell carcinoma. He subsequently underwent an arterial embolisation and cervical laminectomy, following a spinal angiogram of the cervical lesion. He also had a right radical nephrectomy, with no perioperative complications. However, on admission for the left radical nephrectomy, he was noted to have preoperative hypertension. Further investigation revealed an enlarged left adrenal gland on abdominal computed tomography scan and raised urinary catecholamines. We discuss the risk of renal cell carcinoma and phaeochromocytoma arising concomitantly in von Hippel-Lindau syndrome, and how best to investigate and manage them.
INTRODUCTION: This study aims to determine the prevalence and the types of menstrual disorders among patients with systemic lupus erythematosus (SLE) and to identify factors that influence their development.
METHODS: 61 patients with SLE were enrolled into a cross-sectional, observational study at the medical outpatient clinic, Hospital Universiti Sains Malaysia. A total of 120 healthy women were selected randomly to act as the control group. A questionnaire was administered, vital signs were recorded, and blood was evaluated for routine investigations. A review of past medical records was also undertaken.
RESULTS: The mean age and standard deviation for the study group was 33.23 +/- 10.96 years, the majority being ethnic Malays. 75 percent had a severe SLE disease activity index score on initial presentation, and 59 percent were on cyclophosphamide. 49 percent of the study population had menstrual irregularities, of which 60 percent had sustained amenorrhoea. Nine patients with sustained amenorrhoea had hormonal assays, which confirmed the diagnosis of premature menopause.
CONCLUSION: This study showed that SLE patients had a higher risk of developing menstrual irregularities compared to the normal/healthy population. The risk was higher in the older age group (greater than 30 years old) and those on cyclophosphamide therapy, especially those with a cumulative dose of more than 10 g. Sustained amenorrhoea was the commonest irregularity and a majority of them had confirmed premature menopause.
Study site: medical outpatient clinic, Hospital Universiti Sains Malaysia (HUSM), Kelantan, Malaysia
Congenital coronary artery anomalies are rare, with an incidence of about 0.06-1.3 percent of all patients undergoing cardiac catheterisation. They are commonly asymptomatic, but potentially serious lesions may lead to myocardial ischaemia, infarction and/or sudden cardiac death. The occurrence of a concomitant stenotic lesion is exceedingly rare. We report an 80-year-old man who presented with acute anterior myocardial infarction. Coronary angiography revealed severe proximal left anterior descending (LAD) and arteriovenous malformation (AVM) from the first septal branch of the LAD. The LAD stenosis and the AVM were successfully treated with two Jomed covered stents.
Rhinocerebral mucormycosis is an invasive fungal sinusitis with a high mortality rate, especially in immunocompromised patients. A 70-year-old woman, with uncontrolled type 2 diabetes mellitus, presented with a one-month history of non-specific headaches associated with progressive swelling of her left eye. Computed tomography of the brain and orbits showed the extensive involvement of bilateral intranasal sinuses, orbits, extraocular muscle and soft tissues. The diagnosis of invasive mucormycosis was confirmed from a tissue biopsy taken from the internasal septum. Despite the extensive mucormycosis invasion, she was successfully treated with intranasal and systemic amphotericin B and minimal adjunctive intranasal sphenoidotomy.
Bronchoscopy is an essential investigative tool in many respiratory complaints. The procedure can be unpleasant for both bronchoscopists and patients. To the best of our knowledge, there are only a few studies that correlate the bronchoscopist's satisfaction with that of the patient's during bronchoscopy. The aim of our study is to assess whether or not a bronchoscopist could reliably assess a patient's satisfaction during bronchoscopy.
INTRODUCTION: Malaysia has a high rate of smoking prevalence and the figure is increasing. Although there has been many local and regional studies on the prevalence and symptomatology of chronic obstructive pulmonary disease patients, data is lacking on the degree of compliance to national management guidelines in the treatment of chronic obstructive pulmonary disease.
METHODS: 86 patients who attended the respiratory outpatient clinic of the Hospital Universiti Kebangsaan Malaysia were enrolled into a prospective, observational study.
RESULTS: 88 percent of the patients were male and the majority was ethnically Chinese (65 percent). The majority of patients were in the moderate to very severe categories, with a mean FEV1 of 0.97 +/- 0.56 L/sec and predicted mean FEV1 percentage of 43.1 +/- 21.3 percent. 58 percent of the patients were on long-acting beta-agonist, 65 percent were on inhaled steroids, and only 16 percent were on scheduled pulmonary rehabilitation.
CONCLUSION: The low uptake rate for long-acting beta-agonist and pulmonary rehabilitation could be attributed to several factors. Financial cost, the need for strict compliance to a structured rehabilitation regime, lack of significant social support and clear up-to-date guidelines are possible reasons.
Study site: Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
Three hundred thirty-one consecutive patients presenting with hypertension to the outpatient medical clinic of Tengku Ampuan Afzan Hospital, Kuantan, Malaysia were screened and 150 patients with concurrent diabetes were enrolled into a cross-sectional study. The majority of patients were male (60.6%) with a mean age of 60.0 +/- 11.0 years. The mean systolic blood pressure (SBP) was 140.9 +/- 20.1 mmHg and the mean diastolic blood pressure (DBP) was 81.7 +/- 9.8 mmHg. Only 38.0% (57/150) of patients had blood pressures within recommended guidelines (130/80 mmHg). The mean blood pressure in this group was 123.7 +/- 8.5/76.4 +/- 5.6 mmHg. The majority of patients were on either 2 (41.3%) or 3 (31.3%) anti-hypertensives. Females had a significantly higher SBP 145.4 +/- 22.7 vs. 138.0 +/- 17.8 mmHg in males (p = 0.026). The level of blood pressure control in diabetics was unsatisfactory, especially in females and the elderly. A reassessment of priorities in the management of patients with concurrent hypertension and diabetes is therefore, urgently needed.
Study site: outpatient medical clinic of Tengku Ampuan Afzan Hospital,