Parkinson's disease: aetiology, diagnosis, and management

ABSTRACT

Hong Kong Med J 2005;11:476-89 | Number 6, December 2005
REVIEW ARTICLE
Parkinson's disease: aetiology, diagnosis, and management
H Leung, V Mok
Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
OBJECTIVE. To review the aetiology, diagnosis, and management of Parkinson's disease, with a local perspective.
 
DATA SOURCES. Medline from 1966 onwards, and all major neurological journals and movement disorder journals were searched for evidence on the aetiology, diagnosis, and management of Parkinson's disease.
 
STUDY SELECTION. Key words for the literature search were "Parkinson's disease" and "Chinese" or "Hong Kong".
 
DATA EXTRACTION. All relevant articles in English were reviewed.
 
MAIN OUTCOME MEASURES. Reasons for not participating in a phase II cardiac rehabilitation programme.
 
DATA SYNTHESIS. The number of promising genes for familial Parkinson's disease is still expanding rapidly and there has been a wealth of studies on susceptibility genes for Parkinson's disease. Potential treatment choices include the use of agents thought to be neuroprotective, symptomatic treatment with drugs or surgery, and non-pharmacological treatments. Pharmacological treatment using a dopa-sparing strategy and continuous dopaminergic stimulation is now gaining support to address the issue of long-term motor complications. Surgical treatment with deep brain stimulation is safe and effective for refractory cases and has been increasingly utilised locally.
 
CONCLUSIONS. Medical therapy remains the mainstay of treatment and newer agents and treatment approaches are emerging, which will hopefully address the issue of neuroprotection and provide symptomatic treatment with fewer motor complications.
 
Key words: Chinese; Hong Kong; Parkinson disease
 
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Influenza vaccination: options and issues

ABSTRACT

Hong Kong Med J 2005;11:381-90 | Number 5, October 2005
REVIEW ARTICLE
Influenza vaccination: options and issues
SSY Wong, KY Yuen
Department of Microbiology, Centre of Infection, University of Hong Kong, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong
 
 
Currently available vaccines have similar efficacy if they are matched to the most prevalent circulating strains. They also have comparable adverse effect profiles. The choice of a specific preparation of vaccine therefore requires consideration of cost, purity of the vaccine preparation in terms of the amount of egg protein and endotoxin, allergy to different constituents of the vaccine, reactogenicity profiles, as well as the preferred route of administration. Intradermal injection of the vaccine appears to be a viable alternative to the traditional intramuscular administration with the additional benefit of requiring a smaller volume of vaccine. Despite the documented benefits in various community and institutional settings, influenza vaccination has been underutilised by most target groups. A major obstacle to broader coverage of vaccination is the perceived ineffectiveness of the vaccine and the relatively benign nature of the illness in most patients. Uptake of vaccine among target populations, especially health care workers, needs to be improved through a concerted effort between frontline clinicians and health authorities.
 
Key words: Influenza; Influenza vaccines; vaccines, attenuated; Vaccines, inactivated
 
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Genetic markers for retinitis pigmentosa

ABSTRACT

Hong Kong Med J 2005;11:281-8 | Number 4, August 2005
REVIEW ARTICLE
Genetic markers for retinitis pigmentosa
DY Wang, WM Chan, POS Tam, SWY Chiang, DSC Lam, KKL Chong, CP Pang
Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong Eye Hospital, 147K Argyle Street, Hong Kong
 
 
OBJECTIVE. To review recent advances in the molecular genetics of retinitis pigmentosa with emphasis on the development of genetic markers that aids diagnosis and prognosis.
 
DATA SOURCES AND EXTRACTION. Literature search of MEDLINE from 1988 to 2005 using the following key words: ‘retinitis pigmentosa’, ‘rhodopsin’, ‘RP1’, ‘RPGR’, and ‘genetic counseling’. References of two genes—RHO and RP1—causing retinitis pigmentosa in the Chinese population were reviewed.
 
STUDY SELECTION. Literature and data related to genetic markers for retinitis pigmentosa.
 
DATA SYNTHESIS. The genetics of retinitis pigmentosa is complex. It can be sporadic or familial, with heterogeneous transmission modes. Retinitis pigmentosa is associated with nearly 40 chromosomal loci, where 32 candidate genes have been identified. A large number of mutations are known to cause retinitis pigmentosa. But no single mutation alone accounts for more than 10% of unrelated retinitis pigmentosa patients. Genetic tests for retinitis pigmentosa require screening for a consort of mutations in a large number of genes. High throughput screening technology such as denaturing high performance liquid chromatography and automated DNA sequencing should make such tests feasible.
 
CONCLUSIONS. Rapid developments in the understanding of the genetics of retinitis pigmentosa have helped to establish genetic tests of clinical value. The complex mode of inheritance nonetheless makes genetic counselling difficult, even in the presence of positive genetic screening results.
 
Key words: Genetic counseling; Genetic heterogeneity; Retinitis pigmentosa
 
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Human infection by avian influenza A H5N1

ABSTRACT

Hong Kong Med J 2005;11:189-99 | Number 3, June 2005
REVIEW ARTICLE
Human infection by avian influenza A H5N1
KY Yuen, SSY Wong
Department of Microbiology, University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong
 
 
The Southeast Asian outbreak of the highly lethal avian influenza A H5N1 infection in humans is unlikely to abate because of the enormous number of backyard farms providing poultry as the main source of food protein in developing countries. This increases the risk of the emergence of a reassortant pandemic influenza virus with improved human-to-human transmissibility. Currently triage of suspected cases by epidemiological risk factors remains the only practical way of case identification for laboratory investigation and infection control. The clinical usefulness of rapid diagnostic laboratory tests requires more vigorous evaluation. The lethality of this disease may reflect systemic viral dissemination, cytokine storm, or alveolar flooding due to inhibition of cellular sodium channels. The present circulating genotype Z is intrinsically resistant to amantadine and rimantadine. Prognosis may be improved by early treatment with a neuraminidase inhibitor with good systemic drug levels, and post-exposure prophylaxis for health care workers is recommended. The role of immunomodulators and other modalities of therapy requires evaluation in randomised controlled trials, with prospective monitoring of the viral load and cytokine profiles in various clinical specimens. In view of the high fatality of the disease, a combination of contact, droplet, and airborne precautions are recommended as long as resources allow despite the fact that the relative importance of these three modes in nosocomial transmission of avian influenza is still unknown.
 
Key words: Hemagglutinins; Humans; Influenza A virus, avian; Influenza A virus, human
 
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Japanese encephalitis in Hong Kong

ABSTRACT

Hong Kong Med J 2005;11:182-8 | Number 3, June 2005
REVIEW ARTICLE
Japanese encephalitis in Hong Kong
K Lam, OTY Tsang, RWH Yung, KK Lau
Department of Medicine and Geriatrics, Caritas Medical Centre, Shamshuipo, Hong Kong
 
 
OBJECTIVES. To review past and present patterns of occurrence of Japanese encephalitis in Hong Kong and across Asia. A better understanding of the disease should facilitate the formulation of an effective strategic plan to prevent a future epidemic.
 
DATA SOURCES. Report of local cases, and literature search of MEDLINE up to November 2004.
 
DATA SELECTION. Literature and data related to Japanese encephalitis.
 
DATA EXTRACTION. Relevant information and data were reviewed by the authors.
 
DATA SYNTHESIS. Since 16 July 2004, under the ordinance of Hong Kong, Japanese encephalitis has been a notifiable disease. In the past, Japanese encephalitis has reached epidemic proportions in Japan, South Korea, and some areas in China. It has spread globally and has a worldwide incidence of 35 000 to 50 000 cases per year with 10 000 deaths. Mortality is about 30% and survivors often suffer serious long-term morbidity. In 2004, there were five local cases of Japanese encephalitis in Hong Kong. Subsequent serological surveillance of serum samples from 1547 local inhabitants revealed that 37 were positive, ie 2.4% of local inhabitants had been exposed to the Japanese encephalitis virus in the past. Most local inhabitants are immunologically naive to Japanese encephalitis virus. Most infections in endemic areas are asymptomatic. Patients with symptomatic Japanese encephalitis usually present with fever, headache, and confusion. Other signs include neurosis, poliomyelitis, and convulsion. Investigations including magnetic resonance imaging, electroencephalography, and single photon emission computed tomography are not specific. A definitive diagnosis depends on serological studies. Treatment is mainly supportive.
 
CONCLUSIONS. The control of Japanese encephalitis in Hong Kong relies on an accurate surveillance system, vector control, vector avoidance, and vaccination of the at-risk population. At present, vaccination should be limited to travellers to endemic areas who would stay for longer than 1 month.
 
Key words: Asia, Southeastern; Encephalitis, Japanese; Epidemiology; Hong Kong
 
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Childhood obstructive sleep apnoea: an update

ABSTRACT

Hong Kong Med J 2004;10:406-13 | Number 6, December 2004
REVIEW ARTICLE
Childhood obstructive sleep apnoea: an update
AM Li, DFY Chan, TF Fok, YK Wing
Department of Paediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
OBJECTIVE. To review literature on epidemiology, complications, diagnosis, and treatment of childhood obstructive sleep apnoea.
 
DATA SOURCE. Literature search of MEDLINE up to July 2004 using the following key words: ‘obstructive sleep apnoea syndrome’, ‘children’, ‘epidemiology’, ‘complications’, ‘treatment’, and ‘polysomnography’.
 
STUDY SELECTION. Literature and data related to the aspects of childhood obstructive sleep apnoea.
 
DATA EXTRACTION. Relevant information and data were reviewed by the authors.
 
DATA SYNTHESIS. There is a paucity of normal data on childhood obstructive sleep apnoea. Varying definitions and diagnostic criteria have been used in different studies, making direct comparison difficult. However, a small-scale local study found that the prevalence and clinical features of this condition were similar to data published overseas. Increasing evidence suggests that childhood obstructive sleep apnoea is associated with cardiovascular morbidity and neurocognitive dysfunction. Overnight polysomnography has remained the gold standard for diagnosing obstructive sleep apnoea but the diagnostic criteria has not been standardised nor correlated with the long-term outcome. Surgical intervention has remained the treatment of choice, although alternative therapies are being evaluated.
 
CONCLUSION. Consensus on the various important aspects of childhood obstructive sleep apnoea is still limited, especially the definition, diagnosis, and long-term sequelae of this condition. Further advances can only be made with international collaborative research, using evidence-based definitions, standardised techniques, and polysomnographic criteria.
 
Key words: Child; Polysomnography; Sleep apnea, obstructive
 
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An update on the aetiology of orofacial clefts

ABSTRACT

Hong Kong Med J 2004;10:331-6 | Number 5, October 2004
REVIEW ARTICLE
An update on the aetiology of orofacial clefts
FK Wong, U Hagg
Faculty of Dentistry, The University of Hong Kong, Pokfulam Road, Hong Kong
 
 
OBJECTIVE. To review recent data on the aetiology of cleft lip and palate.
 
DATA SOURCES. MEDLINE literature search (1986-2003).
 
STUDY SELECTION. Literature and data on aetiology of cleft lip and palate using the following key words: ‘cleft lip’, ‘cleft palate’, ‘aetiology’, and ‘genetics’.
 
DATA EXTRACTION. Relevant information and data were reviewed by the authors.
 
DATA SYNTHESIS. Cleft lip and palate is one of the most common types of congenital malformation. The aetiology seems complex, but genetics plays a major role. Recently several genes causing syndromic cleft lip and palate have been discovered. Three of them—namely T-box transcription factor-22 (TBX22), poliovirus receptor like-1 (PVRL1), and interferon regulatory factor-6 (IRF6)—are responsible for causing X-linked cleft palate, cleft lip/palateectodermal dysplasia syndrome, and Van der Woude’s and popliteal pterygium syndromes, respectively; they are also implied in non-syndromic cleft lip and palate. The nature and function of these genes vary widely, illustrating high vulnerability within the craniofacial developmental pathways. The aetiological complexity of non-syndromic cleft lip and palate is also exemplified by the large number of candidate genes and loci.
 
CONCLUSIONS. The aetiology of non-syndromic cleft lip and palate is still largely unknown, but mutations in candidate genes have already been identified in a small proportion of cases of non-syndromic cleft lip and palate. Determining the relative risk of cleft lip and palate, on the basis of genetic background and environmental influence, including smoking, alcohol use, and dietary factors, will aid in genetic counselling and the development of future preventive measures.
 
Key words: Cleft lip; Cleft palate; Genetics; Mutation; Syndrome
 
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Gout: a review of its aetiology and treatment

ABSTRACT

Hong Kong Med J 2004;10:261-70 | Number 4, August 2004
REVIEW ARTICLE
Gout: a review of its aetiology and treatment
EK Li
Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
OBJECTIVE. To review the current understanding of the causes and the management of gout.
 
DATA SOURCES. Publications on all peer-review literature from MEDLINE from 1965 to January 2004.
 
STUDY SELECTION. Selected and evaluated by the author.
 
DATA EXTRACTION. Extracted and evaluated by the author.
 
DATA SYNTHESIS. The underlying metabolic disorder in gout is hyperuricaemia. Most patients with hyperuricaemia remain asymptomatic throughout their lifetime. The phase of asymptomatic hyperuricaemia ends with the first attack of gouty arthritis or urolithiasis. The risk of gout and stone formation is increased with the degree and duration of hyperuricaemia. Drugs available for the treatment of acute gouty arthritis, such as non-steroidal anti-inflammatory drugs, selective cyclo-oxygenase 2 inhibitors, systemic corticosteroids, or colchicine, are effective. For periods between attacks, prophylactic therapy, such as low-dose colchicine, is effective. In those with recurrent attacks of more than two to three times yearly, a uric acid–lowering agent as a long-term therapy should be considered to avoid recurrence and the development of tophaceous gout.
 
CONCLUSION. Effective management of gout can be achieved through better understanding of the causes of the condition, preventive measures as well as drug treatment.
 
Key words: Arthritis, gouty; Hyperuricemia
 
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Use of ophthalmic medications during pregnancy

ABSTRACT

Hong Kong Med J 2004;10:191-5 | Number 3, June 2004
REVIEW ARTICLE
Use of ophthalmic medications during pregnancy
CY Chung, AKH Kwok, KL Chung
Department of Ophthalmology, Hong Kong Sanatorium and Hospital, Happy Valley, Hong Kong
 
 
OBJECTIVES. To review potential risks of eye medications to the mother and her foetus.
 
DATA SOURCES. PubMed search for all relevant articles (1966 to 2003).
 
STUDY SELECTION. All types of publication that documented potential risks of eye medications during pregnancy. The following key words were used: pregnancy, fetus, teratogenicity, eye, ocular, ophthalmic, glaucoma, antibiotics, anti-inflammatory, and corticosteroids.
 
DATA EXTRACTION. All relevant articles including original articles, review papers, case studies, and relevant book chapters were extracted and reviewed.
 
DATA SYNTHESIS. Whether ophthalmic medications can be used during pregnancy is a very important issue; yet, limited information on the subject exists in the literature. Topically applied eye medications that give rise to systemic side-effects are of particular concern to both patients and doctors. Various ophthalmic anti-infective preparations and ophthalmic corticosteroids have shown to cause teratogenicity in animal studies. Furthermore, anti-glaucoma drugs pose potential risks to the foetus if they are absorbed systemically. This article examines the association between the main groups of ophthalmic medication and their possible adverse effects on the mother and the foetus. Recommendations for the treatment of pregnant patients with eye diseases are also discussed.
 
CONCLUSION. The risk of giving ophthalmic drugs to pregnant women is low. Doctors should be cautious when prescribing drugs for pregnant women and consult experts in the field when in doubt.
 
Key words: Administration, oral; Anti-bacterial agents; Ophthalmology; Pregnancy complications, infectious/drug therapy; Teratogens
 
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Epilepsy in Hong Kong: a literature review

ABSTRACT

Hong Kong Med J 2004;10:185-9 | Number 3, June 2004
REVIEW ARTICLE
Epilepsy in Hong Kong: a literature review
ACF Hui, P Kwan
Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong
 
 
OBJECTIVE. To review data on the causes, clinical features, and management of patients with epilepsy in Hong Kong.
 
DATA SOURCES. MEDLINE and Chinese Current Medical Contents were used to search the literature. A manual search of the Hong Kong Medical Journal, Hong Kong Practitioner, and Chinese Medical Journal (1982-2002) was also undertaken.
 
STUDY SELECTION. Key words for the literature search were ‘epilepsy’ and ‘Hong Kong’.
 
DATA EXTRACTION. All relevant articles in English or in Chinese language were reviewed.
 
DATA SYNTHESIS. Overall, disease characteristics and the response to both medical and surgical treatments of epilepsy among local Chinese patients with epilepsy was found to be comparable to that reported for patients in western countries. Knowledge of epilepsy among the general population was more limited than expected from the international literature, and attitudes to epilepsy relatively more negative, adding to the psychosocial burden for people with epilepsy.
 
CONCLUSION. Further research in Hong Kong on aspects of epileptology is indicated with a view to developing more innovative and effective therapy.
 
Key words: Chinese; Cross-cultural comparison; Epidemiology; Epilepsy
 
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