OBJECTIVE. To evaluate the impact of genetic testing in the management of familial multiple endocrine neoplasia 2A patients.

DESIGN. Retrospective study.

SETTING. University teaching hospital, Hong Kong.

PATIENTS. Twenty-two patients from eight multiple endocrine neoplasia 2A families underwent prophylactic total thyroidectomy based on a positive RET mutation genetic testing. All mutations were located at codon 634 of exon 11. Nineteen patients had preoperative basal serum calcitonin measured, and the 12 with normal levels had pentagastrin stimulation tests. Preoperative thyroid ultrasound examination was performed for 17 patients.

RESULTS. There were 13 females and 9 males with a median age of 25.1 (range, 6.1-71.9) years. Histopathology revealed medullary thyroid carcinoma in 17 (77%), C-cell hyperplasia in four (18%), and normal pathology in one (5%) of the patients. Five patients with either C-cell hyperplasia or normal pathology were among the youngest (age range, 6-9 years). The youngest patient with medullary thyroid carcinoma was nearly 9 years old. The median size of medullary thyroid carcinomas was 8.3 (range, 0.1-18) mm, but there were no lymph node metastases. Of 15 patients with normal basal calcitonin levels, 10 had medullary thyroid carcinoma, though two tested negative with the pentagastrin-stimulated calcitonin assay. Five of six patients with normal preoperative ultrasonographic examinations had medullary thyroid carcinoma. Three (14%) of the patients were prescribed long-term calcium and vitamin D supplementation. After a median follow-up of 49 (range, 13-128) months, no patient had recurrence of medullary thyroid carcinoma.

CONCLUSIONS. Genetic testing has replaced conventional biochemical and radiological modalities to identifying multiple endocrine neoplasia 2A carriers, in order to offer them prophylactic thyroidectomy. Chinese multiple endocrine neoplasia 2A patients with codon 634 mutation seem to have less aggressive forms of medullary thyroid carcinoma, for whom prophylactic thyroidectomy can be considered at the age of 8 years.

Key words: Genetic screening; Multiple endocrine neoplasia type 2a; Proto-oncogene proteins c-ret; Thyroid neoplasms; Thyroidectomy

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OBJECTIVES. To compare the effectiveness of two minimally invasive procedures, namely computed tomography-guided aspiration and the key-hole approach, in the neurosurgical management for spontaneous putaminal haemorrhage, and to explore the indications for the two approaches.

DESIGN. A multicentre, single-blinded controlled trial.

SETTING. Hospitals taking part in this trial and the sources for patients were from China. Among others, the hospitals involved in the interventions included: the Beijing Tiantan Hospital (of the Capital University of Medical Sciences), the General Hospital of People's Liberation Army, the Peking Union Hospital, and the Shanghai Huashan Hospital (of the Fudan University medical school).

PATIENTS. From September 2001 to November 2003, data were available for analysis from a total of 841 patients with spontaneous putaminal haemorrhage from 135 hospitals all over China (except Tibet, Hong Kong, Taiwan, and Macao). All follow-up data were for at least 3 months.

MAIN OUTCOME MEASURES. Mortality, Glasgow Coma Scale score, postoperative complications, Kanofsky Performance Scale score, and Barthel Index.

RESULTS. There were 563 patients who underwent computed tomography-guided aspiration, and 165 were treated by the key-hole approach. Respective mortality rates 1 month after the operation were 17.9% and 18.3%; at 3 months they were 19.4% and 19.4%. In those undergoing computed tomography-guided aspiration, mortality rates at 3 months after the operation were 28.2% in patients with Glasgow Coma Scale scores of 8 or below, as opposed to 8.2% in those with higher scores. This amounted to a 3.4-fold difference. In those treated by the key-hole approach, the corresponding rates were 30.2% and 7.6%, which amounted to a 4-fold difference. The corresponding mortality at 3 months in patients with complications was 3.9 times as great as in those without complications. In those with haematoma volumes of 70 mL or greater, it was 2.7 times as much as in those in whom the volumes below 30 mL. The postoperative complication rate of computed tomography-guided aspiration (23.7%) did not differ significantly from that in those having the key-hole approach (25.7%) [P=0.420].

CONCLUSIONS. Computed tomography-guided aspiration is not superior to the key-hole approach for treating spontaneous putaminal haemorrhage in terms of favourable outcomes, mortality, and morbidity. However, it could be the first-choice approach for those with bleeds of 50 mL or less, while the key-hole approach may be more suitable for those with larger haematomas.

Key words: Hematoma; Intracranial hemorrhage, hypertensive; Neurosurgical procedures; Putaminal hemorrhage; Tomography, X-ray computed

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OBJECTIVE. To review the pattern of ruptured intracranial aneurysms in terms of size, location, and the prevalence of multiple aneurysms in the Hong Kong Chinese population with subarachnoid haemorrhage.

DESIGN. Retrospective study.

SETTING. Three public hospitals in Hong Kong.

PATIENTS. A total of 267 Chinese patients with subarachnoid haemorrhage from ruptured intracranial aneurysms between July 1998 and June 2002 were reviewed retrospectively.

RESULTS. The patients had a mean age of 59 (range, 13-96) years, with a female-to-male ratio of 2:1. Concerning the age at presentation, males presented with ruptured intracranial aneurysms at a younger age (P=0.001) than females. Ruptured aneurysms were more commonly located in the anterior than posterior circulation (84% vs 16%). The posterior communicating artery (26%) and anterior communicating artery (22%) were the most common sites of rupture. As a whole, 64% of the aneurysms had a size of 5 mm or less. The anterior communicating artery had a higher proportion with a size of 5 mm or less compared to other locations (P<0.05). In this cohort, the prevalence of multiple aneurysms was 17%. There was no significant difference in the prevalence of multiple aneurysms between men and women (P=0.30). In patients with multiple aneurysms, the sizes of ruptured aneurysms were greater than those of the largest unruptured aneurysms (P<0.001). When compared with the group with single aneurysms, patients with multiple aneurysms had a smaller proportion of small aneurysms, sized 5 mm or less (P<0.05).

CONCLUSIONS. The pattern of ruptured intracranial aneurysms in the Hong Kong Chinese population was different from western and Japanese populations. Although the distribution of locations for ruptured aneurysms was similar, Hong Kong Chinese had a larger proportion of small aneurysms sized 5 mm or less. The prevalence of multiple aneurysms in Hong Kong is comparable to that in the Japanese population, but lower than that in the western populations.

Key words: Aneurysm, ruptured; Intracranial aneurysm; Subarachnoid hemorrhage

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OBJECTIVES. To define the role of bypass surgery and percutaneous transluminal angioplasty to manage critical limb ischaemia due to infrainguinal arterial occlusive disease.

DESIGN. Retrospective review.

SETTING. Regional hospital, Hong Kong.

PATIENTS. Consecutive patients treated for critical limb ischaemia, for whom data were prospectively collected in those who underwent: (i) infrainguinal percutaneous transluminal angioplasty first, or (ii) infrainguinal bypass surgery.

RESULTS. Among patients with critical lower limb ischaemia, 364 consecutive individuals having infrainguinal bypass operations and 100 having percutaneous transluminal angioplasty first were compared. The latter patients were older (77 vs 74 years, P=0.014) and had more co-morbidities but higher ankle pressure than those having bypass surgery. In the angioplasty-first group, 74% had favourable lesions (classified as TransAtlantic Inter-Society Consensus A/B). In the bypass group, operative mortality was higher (4% vs 1%, P=0.03) than that in the angioplasty-first group. Hospital mortality was comparable (8% vs 3%, P=0.15). In the bypass group, median hospital stay was longer than that in the angioplasty-first group (24 vs 4 days, P<0.001), and postoperatively they also had a higher median ankle-brachial index (0.92 vs 0.70, P<0.001) and superior long-term patency. In the bypass group, American Society of Anesthesiologists class 4 patients suffered very high operative and hospital mortality (15% and 31%, respectively). Long-term patency of percutaneous transluminal angioplasty depended on the TransAtlantic Inter-Society Consensus class of the treated lesion. Limb salvage rates at 3 years were 89% and 78% for percutaneous transluminal angioplasty first and surgical bypass, respectively (P=0.046). Long-term survival was poorer in the percutaneous transluminal angioplasty-first group (21% vs 51% at 5 years, P=0.04).

CONCLUSION. Infrainguinal bypass and percutaneous transluminal angioplasty are complementary. For TransAtlantic Inter-Society Consensus A and B lesions, percutaneous transluminal angioplasty should be offered first. For American Society of Anesthesiologists class 4 patients, percutaneous transluminal angioplasty should be considered first, regardless of the TransAtlantic Inter-Society Consensus class.

Key words: Angioplasty, balloon; Arterial occlusive diseases; Ischemia; Leg

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OBJECTIVES. To describe a cluster of Hong Kong subjects with hypoglycaemia, after they had taken various non-prescription sildenafil products containing glibenclamide.

DESIGN. Retrospective study.

SETTING. A tertiary referral centre for clinical toxicology analysis in Hong Kong.

PATIENTS. All men referred to the laboratory for investigation of suspected drug-induced hypoglycaemia from December 2007 to September 2008.

MAIN OUTCOME MEASURES. The characteristics of these patients, including their clinical presentations, outcomes, drug history, urine toxicology analysis results, and in some instances, analysis results of unused products.

RESULTS. A total of 144 male patients were referred for suspected drug-induced hypoglycaemia. Sildenafil and glibenclamide, or their metabolites, were detected in the urine specimens of 68 (47%) patients, none of whom had been prescribed either drug by a registered medical practitioner. Among these subjects, 24 (35%) denied any use of sexual enhancement products despite repeated questioning. Eight patients had repeated exposure resulting in re-admission. The sources of these sexual enhancement products included pharmacies in Mainland China, friends, local pharmacies, peddlers, or were unknown. Three patients died, one remains in a vegetative state and one suffered cognitive impairment; the remaining 63 recovered fully. Twenty-five unused sexual enhancement products of seven different kinds were recovered for analysis. The median (range) of sildenafil and glibenclamide per unit dose was 64 (0.05-198) mg and 70 (0-158) mg, respectively.

CONCLUSION. These illegal products pose a severe and continued threat to society and therefore deserve widespread vigilance, so that such products can be eradicated at their source.

Key words: Drug contamination; Erectile dysfunction; Hypoglycemia; Nonprescription drugs

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