Elephantiasic thyroid dermopathy.
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BCG Vaccine↗
Biomedical subjects
S C Tiu
Publications and source records attributed to S C Tiu.
Use of lithium in the treatment of thyrotoxicosis.
OBJECTIVES: To evaluate the efficacy and safety of lithium in the treatment of thyrotoxicosis, and to study the dose and serum levels at which therapeutic response occurs. DESIGN: Retrospective study. SETTING: Thyroid clinic of a regional hospital in Hong Kong. PATIENTS: Thirteen patients with thyrotoxicosis pending therapy with radioiodine or surgery, in whom thionamides were contra-indicated due to adverse reactions or failure of treatment. MAIN OUTCOME MEASURES: Free thyroxine levels, time to euthyroidism, and side-effects of lithium. RESULTS: A satisfactory response, defined as a fall by 40% or more in free thyroxine levels and clinical improvement, was achieved in eight patients within 1 to 2 weeks of lithium therapy. In four others, response occurred in 3 to 5 weeks. Response was slow and inadequate in one patient due to 'escape'. The median dosage of lithium was 750 mg daily, with a range of 500 to 1500 mg daily. The median serum lithium level was 0.63 mmol/L. Lithium toxicity was observed in one patient. CONCLUSIONS: A relatively low dose of lithium offers a safe and effective alternative means of controlling thyrotoxicosis in patients who cannot tolerate or do not respond to thionamides.
Adolescent↗
N-methyl-D-aspartate receptor and apoptosis in Alzheimer's disease and multiinfarct dementia.
This study investigates the role of excitotoxicity in Alzheimer's disease and in multiinfarct dementia by examining, via immunohistochemical methods, the number of cells that are positive for N-methyl-D-aspartate (NMDA) receptor and the degree of colocalization between NMDA receptor and apoptosis markers such as TUNEL or activated caspase-3 in the frontal cortex of individuals with these two conditions, comparing the results with those from subjects who died of normal aging. We showed an increased number of NMDA receptor-positive cells and an increased number of TUNEL-labeled cells in the frontal cortex of subjects with Alzheimer's disease, especially in the deeper layers of the cortex. However, only about 10% of cells showed colocalization of NMDA receptor with the apoptosis markers studied, suggesting that NMDA-mediated excitotoxicity does not play a major role in neuronal apoptosis in Alzheimer's disease or in multiinfarct dementia.
Aging↗
Resistance to thyroid hormone in a Chinese family with R429Q mutation in the thyroid hormone receptor beta gene.
The combination of elevated serum levels of free thyroid hormones with non-suppressed thyroid-stimulating hormone suggests the differential diagnoses of resistance to thyroid hormone or thyroid-stimulating hormone-secreting pituitary tumour. Clinical differentiation of these two conditions can be difficult, because patients with thyroid hormone resistance may exhibit various combinations of hypermetabolic and hypometabolic features, and laboratory results have limited sensitivity and specificity. We report a case of resistance to thyroid hormone in a Chinese family that illustrates this difficulty. The diagnosis could only be confirmed by the identification of a known disease-causing mutation in the thyroid hormone receptor beta gene in peripheral leukocytes. Availability of genetic tests will identify more cases in the future and improve our understanding of this condition.
Adult↗
Medullary thyroid carcinoma in Hong Kong Chinese patients.
OBJECTIVE: To study the clinical parameters and treatment outcome of medullary thyroid carcinoma in Hong Kong Chinese patients. DESIGN: Retrospective study. SETTING: Regional oncology unit, Hong Kong. PATIENTS: Patients with medullary thyroid carcinoma who were identified among 1656 patients with thyroid malignancies seen in a single institute in Hong Kong from January 1960 to June 2003. MAIN OUTCOME MEASURES: Ten-year cause-specific survival, locoregional failure-free survival, and distant metastasis failure-free survival. RESULTS: Twenty-two (1.3%) patients with medullary thyroid carcinoma were identified. The mean age at diagnosis was 43.7 (standard deviation, 16.5) years. The sex ratio was 1:1. The 10-year cause-specific survival, locoregional failure-free survival, and distant metastasis failure-free survival were 75.4%, 82.0%, and 62.4%, respectively. Lymph node metastasis was present in seven (31.8%) patients at diagnosis. Distant metastasis developed in nine (40.9%) patients: lung, 3 (13.6%); bone, 5 (22.7%); liver, 2 (9.1%); mediastinum, 4 (18.2%). Seven (31.8%) patients died of distant metastasis. Mediastinal (n=3) and bone metastases (n=3) were important causes of death. Genetic study confirmed multiple endocrine neoplasia type 2A in 3 (25.0%) of 12 patients who all had bilateral and multifocal diseases. Younger age (<45 years) was associated with better survival, better locoregional control, and less distant metastasis. Patients with pT1N0 disease (n=3) had an excellent prognosis: all were disease-free following total thyroidectomy. Among eight patients who received external radiation therapy, seven achieved good locoregional control. In seven patients with lymph node metastasis, external radiation therapy gave 100% (4/4) locoregional control compared with 33.3% (1/3) in those without external radiation therapy. Chemotherapy using dacarbazine and 5-fluorouracil was tried in three patients with poor response. CONCLUSIONS: Early stage (T1N0) medullary thyroid carcinoma is associated with a very good prognosis. Postoperative external radiation therapy can achieve good locoregional control in patients with lymph node metastasis or locally advanced disease.
Adult↗
Combined orbital irradiation and systemic steroids compared with systemic steroids alone in the management of moderate-to-severe Graves' ophthalmopathy: a preliminary study.
OBJECTIVE: To assess the efficacy and safety of combined orbital irradiation and systemic steroids in the management of moderate-to-severe Graves' ophthalmopathy. DESIGN: Single-blind randomised prospective study. SETTING: Regional hospital, Hong Kong. PATIENTS: Sixteen patients with active moderate-to-severe Graves' ophthalmopathy who were randomly assigned to steroid therapy (ST group) or combination therapy of orbital irradiation and systemic steroids (SRT group) between June 2000 and June 2003. MAIN OUTCOME MEASURES: NOSPECS scoring system, total eye score, subjective eye score, and extra-ocular muscle thickness as determined by either computed tomographic or magnetic resonance imaging scans. RESULTS: The study was completed by 15 of 16 patients. Both groups experienced improvement in total eye score, soft tissue swelling, ocular motility, visual acuity, and subjective eye score at 52-week follow-up. Total eye score improved earlier in the SRT group, achieving statistical significance (P<0.05) at as early as 4 weeks of follow-up. Improvement in ocular parameters was greater and led to a significantly greater reduction in total eye score than in the ST group at weeks 16, 24, and 52. Maximum extra-ocular muscle thickness was significantly reduced in the SRT group only. No change was observed in proptosis in either group. No serious adverse effect was observed with the addition of orbital irradiation to steroid therapy. CONCLUSION: A combination of orbital irradiation and systemic steroids is well tolerated and more effective than steroids alone in the treatment of active moderate-to-severe Graves' ophthalmopathy. It achieves greater and more rapid improvement in soft tissue swelling, ocular motility, and visual acuity.
Administration, Oral↗
Multiple endocrine neoplasia type 1 (MEN1): genetic and clinical analysis in the Southern Chinese.
OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) is characterized by a triad of neoplasia affecting the parathyroid glands, enteropancreatic endocrine tissue and the anterior pituitary gland. DESIGN: In order to define the prevalence of MEN1 germ-line mutations in Southern Chinese patients with MEN1 syndrome, we performed direct sequencing of the entire open reading frame of the MEN1 gene for 12 index patients and their first-degree relatives. RESULTS: Six patients had familial MEN1 syndrome and six had apparently sporadic disease. Nine different germ-line mutations at the MEN1 gene were identified, including three novel mutations [248-249delTT in exon 2, K559X(AAG --> TAG) in exon 10 and IVS 2nt + 2(G --> T) in intron 2]. All patients with familial MEN1 syndrome were heterozygous carriers of a germ-line mutation and MEN1-related disorders were only evident in their first-degree relatives who also carried the mutation. All patients with an enteropancreatic lesion were mutation carriers and the absence of mutation in three apparently sporadic MEN1 patients with only hyperparathyroidism and pituitary microadenoma might represent the presence of MEN1 phenocopy. CONCLUSIONS: The finding of MEN1 germ-line mutation in all patients with familial MEN1 syndrome suggests that genetic screening should be useful in our population to identify affected individuals within a kindred and allow early detection of MEN1-related tumours.
Adult↗
Effects of treatment with Sandostatin LAR on small dense LDL and remnant-like lipoproteins in patients with acromegaly.
OBJECTIVE: Acromegalic patients have been shown to have an increase in the concentrations of small dense low-density lipoprotein (LDL) and remnant-like lipoprotein particles (RLP). These lipoproteins are atherogenic and may contribute to the cardiovascular risk of these patients. The aim of this study was to determine whether treatment of acromegaly using Sandostatin LAR could lower these atherogenic lipoproteins. METHODS: Fourteen patients with active acromegaly were recruited and Sandostatin LAR, a long-acting somatostatin analogue, was given every 4 weeks by intramuscular injection for 6 months. Fasting lipids, lipoproteins, lipolytic enzymes were determined at baseline, 12 and 24 weeks after treatment. Small dense LDL was measured using density gradient ultracentrifugation and RLP-cholesterol (RLP-C) by an immunoseparation assay. RESULTS: There was already a marked reduction in GH and IGF-1 by week 8 and, in all subjects, IGF-1 levels within their respective age-specific normal range were achieved. At week 12, plasma triglyceride significantly decreased (P < 0.01) and both HDL2 (P < 0.01) and HDL3 (P < 0.01) subfractions increased. A reduction was seen in small dense LDL concentration (P < 0.05) and RLP-C (P < 0.05). Lipoprotein lipase (LPL) activity increased (P < 0.01) and the magnitude of the increase in LPL activity correlated with the increase in HDL at 3 months (r = 0.55, P < 0.05) but not with the changes in plasma triglyceride, small dense LDL or RLP-C. The improvement in plasma lipids and lipoproteins persisted until the end of the study. CONCLUSION: Sandostatin LAR is effective in the treatment of acromegaly and is associated with favourable changes in plasma lipids and a reduction in small dense LDL and RLP-C.
Acromegaly↗
Consensus statement on iodine deficiency disorders in Hong Kong.
This article reviews the available data on the study of iodine deficiency disorders in Hong Kong and to discuss the approach towards preventing such disorders in Hong Kong. The importance of iodine and iodine deficiency disorders is described, and the available data on the dietary iodine intake and urinary iodine concentration in different populations of Hong Kong are summarised and discussed. Dietary iodine insufficiency among pregnant women in Hong Kong is associated with maternal goitrogenesis and hypothyroxinaemia as well as neonatal hypothyroidism. Borderline iodine deficiency exists in the expectant mothers in Hong Kong. Women of reproductive age, and pregnant and lactating women should be made aware and educated to have an adequate iodine intake, such as iodised salt, as an interim measure. A steering group involving all stakeholders should be formed to advise on the strategy of ensuring adequate iodine intake, including universal iodisation of salt in Hong Kong. Continuous surveillance of iodine status in the Hong Kong population is necessary.
Deficiency Diseases↗
A Chinese family with familial dysalbuminaemic hyperthyroxinaemia.
We report the results of biochemical and genetic studies in a Chinese family with familial dysalbuminaemic hyperthyroxinaemia. Total thyroxine levels were 1.2 to 1.7 times the upper limit of the reference range and free thyroxine levels were 1.2 to 1.6 times the upper reference limit. Concentrations of thyroid-stimulating hormone (thyrotropin) and free tri-iodothyronine were normal in all family members tested. Overall, thyroid function tests showed high total thyroxine levels in five males and two females over two generations in the family. The diagnosis of familial dysalbuminaemic hyperthyroxinaemia was confirmed by the detection of a guanine to adenine missense mutation in the second nucleotide of codon 218 of the gene encoding human serum albumin, showing that the mutation in this family is the same as that previously found in Caucasian populations.
Adult↗
Use of the low-dose corticotropin stimulation test for the diagnosis of secondary adrenocortical insufficiency.
OBJECTIVE: To assess the clinical utility and safety of the low-dose corticotropin stimulation test in the diagnosis of secondary adrenocortical insufficiency. DESIGN: Prospective study. SETTING: Regional hospital, Hong Kong. PARTICIPANTS: Seventy-two Chinese patients with suspected secondary adrenocortical insufficiency. MAIN OUTCOME MEASURE: Serum cortisol response during the low-dose corticotropin stimulation test, using the insulin tolerance test as the gold standard. RESULTS: The 30-minute cortisol level during the low-dose corticotropin stimulation test was most closely correlated (r=0.79) with the peak cortisol level achieved during the insulin tolerance test. The optimum sensitivity and specificity of the low-dose corticotropin stimulation test were obtained at a cut-off value of 550 nmol/L or more for the 30-minute cortisol level. Using the insulin tolerance test as the gold standard for comparison, the low-dose corticotropin stimulation test had a sensitivity of 97%, a specificity of 78%, a positive predictive value of 81%, and a negative predictive value of 97% at this cut-off value. The positive likelihood ratio was 4.4 and the negative likelihood ratio 0.04. CONCLUSION: The low-dose corticotropin stimulation test, using the cortisol response at 30 minutes after synacthen 1 microg is a safe, convenient, and sensitive method for screening abnormalities of the hypothalamic-pituitary-adrenocortical axis in Chinese patients suspected of having secondary adrenocortical insufficiency.
Adrenal Insufficiency↗
Novel mutation and polymorphisms of the HMBS gene detected by denaturing HPLC.
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Adult↗
Differential expression of S100B and S100A6(1) in the human fetal and aged cerebral cortex.
S100B and S100A6 (calcylin) are two members of the S100 Ca(2+)-binding protein family and have been localized in the mammalian nervous system. However, information on their distribution in the human nervous system, especially in the developing human fetal brain, is scarce. In the present study, an immunocytochemical method was used to examine the spatio-temporal protein expression patterns of S100B and S100A6 in normal human fetal hippocampus, entorhinal cortex and occipital cortex. Normal aged adult human brain specimens were also included for comparison. From week 15 onwards, an increase with advancing gestation age in both the number and staining intensity of S100B positive, astrocyte-like cells was found in the pyramidal layer of the hippocampus, while both the molecular and polymorphic layers showed similar S100B immunoreactivities at all stages examined. A decrease in the immunoreactivities was found in the molecular layer of the aged adult hippocampus while other layers exhibited immunoreactivities similar to those of the late fetus. At week 15, the molecular, pyramidal and ganglionic/multiform layers of the entorhinal cortex also showed positive S100B immunoreactivities which were maintained throughout the rest of the gestation and in adult specimens. In the occipital cortex, the numbers of positive cells for all layers were about twofold higher than those found in the hippocampus and entorhinal cortex, and immunoreactivities detected in the granular layer increased from week 21, reaching a plateau at around week 27. S100B positive fibers were also found at week 30 but were not observed in aged adult specimens. S100A6 positive cells were on the whole fewer in number than those of S100B in the brain regions examined. The S100A6 immunoreactivities which were localized in some pyramidal neuron-like and some glial-like cells of the pyramidal and molecular layers of the hippocampus increased by midgestation and became weak in the late fetus and in aged adult specimens. Weakly stained S100A6 positive cells were also observed in the entorhinal cortex throughout the gestation and in aged adult cortex. S100A6 immunoreactivities were weak in the fetal occipital cortex. They were also localized in the glial-like cells of the aged adult occipital cortex. The differential spatio-temporal expression of S100B and S100A6 proteins suggests that the proteins play different roles in different brain regions during development and in adulthood.
Aged↗
A case of giant malignant phaeochromocytoma.
Malignant phaeochromocytoma is defined as the presence of tumour deposits at sites that are normally devoid of chromaffin cells. We report on a 63-year-old man who had a giant malignant phaeochromocytoma of the right adrenal gland that encased the inferior vena cava. The urinary excretion rates of catecholamines and their metabolites were normal, except for normetanephrine, which was excreted at a higher rate than normal. The tumour was surgically unresectable by laparotomy. Postoperatively, the patient was given a 4-month trial of subcutaneous octreotide and intravenous meta-iodobenzylguanidine I 131. Occult lung secondary tumours were first detected by meta-iodobenzylguanidine scintigraphy after 2 years, and the patient died of bone and lung metastases 1 year later. Because phaeochromocytoma is rare, local experience in managing this disease is limited. This report alerts physicians of the methods of diagnosing and managing surgically unresectable malignant phaeochromocytoma.
3-Iodobenzylguanidine↗
Physicians' practice patterns of treating Helicobacter pylori-associated peptic ulceration in public hospitals in Hong Kong: questionnaire survey. Coordinator Group of the Quality Assurance Subcommittee (Medicine), Hospital Authority, Hong Kong.
To survey the practice patterns of physicians in public hospitals in Hong Kong when treating Helicobacter pylori-associated peptic ulceration, the records of all patients from 22 medical units who had new peptic ulcers that had been diagnosed endoscopically during August 1996 were examined systematically. Patient data were entered on a one-page questionnaire. Five hundred and twelve patients with peptic ulceration were studied; 173 (34%) of whom had presented with gastro-intestinal bleeding. The Helicobacter pylori status had been determined in 449 (88%) patients, 280 (62%) of whom had subsequently tested positive for Helicobacter pylori. The biopsy urease test or histological examination had been performed for more than 95% of patients. Of 260 patients who had tested positive for Helicobacter pylori, 244 (94%) had received eradication therapy to eliminate this organism; a total of 291 patients, however, were receiving eradication therapy. The most commonly used regimen was proton pump inhibitor triple therapy (151 [52%] of 291 patients). Confirmation of the eradication of Helicobacter pylori had been planned for 152 (52%) of the 291 patients, whereas ulcer-healing drugs--mainly H2-receptor antagonists--had been prescribed for 87 (30%) patients after eradication. Curing Helicobacter pylori infection is therefore widely accepted in the management of peptic ulcer disease among physicians working in Hong Kong public hospitals.
Journal Article↗
Acarbose in NIDDM patients with poor control on conventional oral agents. A 24-week placebo-controlled study.
OBJECTIVE: To determine the efficacy of acarbose, compared with placebo, on the metabolic control of NIDDM patients inadequately controlled on maximal doses of conventional oral agents. RESEARCH DESIGN AND METHODS: In this three-center double-blind study, 90 Chinese NIDDM patients with persistent poor glycemic control despite maximal doses of sulfonylurea and metformin were randomly assigned to receive additional treatment with acarbose 100 mg thrice daily or placebo for 24 weeks, after 6 weeks of dietary reinforcement. Efficacy was assessed by changes in HbA1c, fasting and 1-h postprandial plasma glucose and insulin levels, and fasting lipid levels. RESULTS: Acarbose treatment was associated with significantly greater reductions in HbA1c (-0.5 +/- 0.2% vs. placebo 0.1 +/- 0.2% [means +/- SEM], P = 0.038), 1-h postprandial glucose (-2.3 +/- 0.4 mmol/l vs. placebo 0.7 +/- 0.4 mmol/l, P < 0.001) and body weight (-0.54 +/- 0.32 kg vs. placebo 0.42 +/- 0.29 kg, P < 0.05). There was no significant difference between the two groups regarding changes in fasting plasma glucose and lipids or fasting and postprandial insulin levels. Flatulence was the most common side effect (acarbose vs. placebo: 28/45 vs. 11/44, P < 0.05). One patient on acarbose had asymptomatic elevations in serum transaminases that normalized in 4 weeks after acarbose withdrawal. Another patient on acarbose developed severe hypoglycemia; glycemic control was subsequently maintained on half the baseline dosage of sulfonylurea. CONCLUSIONS: In NIDDM patients inadequately controlled on conventional oral agents, acarbose in moderate doses resulted in beneficial effects on glycemic control, especially postprandial glycemia, and mean body weight. Additional use of acarbose can be considered as a useful alternative in such patients if they are reluctant to accept insulin therapy.
Acarbose↗
Primary hyperparathyroidism in Hong Kong: an analysis of 44 cases.
Primary hyperparathyroidism is increasingly being diagnosed subsequent to the detection of hypercalcaemia using multichannel auto-analyser screening. This paper provides a local picture of the clinical presentation and management of primary hyperparathyroidism. A retrospective review was conducted of 44 patients with primary hyperparathyroidism who were treated at the Queen Elizabeth Hospital between January 1987 and July 1996. Twenty-five (56.8%) of the patients were asymptomatic. Only three (6.8%) patients gave radiograms that had features indicating primary hyperparathyroidism; seven (15.9%) had renal stones. The patients underwent one or more of the following localisation procedures: computerised tomography, ultrasonography, thallium-technetium subtraction scanning, and technetium Tc 99m sestamibi scanning. The latter method was the most sensitive (64.3%). Ten adenomas that could not be localised by any of these procedures were successfully removed during surgery. Surgery was successful in 94.4% of cases and surgical outcome was comparable to that reported in the literature.
Journal Article↗
Habenulo-interpeduncular descending pathways and their relationship to enkephalin- and somatostatin-immunoreactive neurons in the interpeduncular nucleus of human fetuses.
The interpeduncular nucleus of six human fetuses aged 15 (one specimen), 26 (one specimen), 38 (one specimen) and 40 (three specimens) gestation weeks was studied by immunohistochemistry for enkephalin and somatostatin localization and immunohistochemistry coupled with silver staining. Enkephalin-positive and somatostatin-positive cells were detected, the former initially at 15 weeks gestation and the latter at 26 weeks gestation. They appeared to receive long afferents from the habenular region and projected short efferents to adjacent cells devoid of enkephalin and somatostatin positivity. We postulate that these enkephalin- and somatostatin-positive neurons function as modulatory interneurons in the habenulo-interpeduncular and related pathways.
Enkephalins↗