Prenatal diagnosis and assessment of facial clefts: where are we now?

ABSTRACT

Hong Kong Med J 2012;18:146-52 | Number 2, April 2012
MEDICAL PRACTICE
Prenatal diagnosis and assessment of facial clefts: where are we now?
William WK To
Department of Obstetrics and Gynaecology, United Christian Hospital, Kwun Tong, Kowloon, Hong Kong
 
 
Orofacial clefts are one of the most common non-syndromic congenital structural abnormalities. Prenatal diagnosis of such defects has traditionally been made by ultrasound examination. With the advent of routine second-trimester ultrasound screening for morphological abnormalities in the recent two decades, the prenatal detection rate of such abnormalities has progressively increased. While conventionally, two-dimensional scanning has been used for screening of lip clefts, the development of three-dimensional ultrasound scanning technology has allowed more easy visualisation of the defects, as well as more accurate evaluation of palatal clefts. Various three-dimensional scanning techniques to assess such defects have been advocated in the recent 5 to 6 years, but as yet there is no consensus as to the most effective and practical methods. As fetal magnetic resonance imaging gradually becomes an accessible modality of imaging in modern obstetrics, it is likely to become an additional tool to assess these defects.
 
Key words: Cleft lip; Cleft palate; Karyotyping; Pregnancy; Ultrasonography, prenatal
 
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Rehabilitation in older people: know more, gain more

ABSTRACT

Hong Kong Med J 2012;18:56-9 | Number 1, February 2012
MEDICAL PRACTICE
Rehabilitation in older people: know more, gain more
James KH Luk
Department of Medicine and Geriatrics, Fung Yiu King Hospital, 9 Sandy Bay Road, Pokfulam, Hong Kong
 
 
Functional decline is common among older patients during or even prior to hospitalisation, in spite of treatment of acute illnesses. An effective rehabilitation programme is vital to improve the motor and functional states of the older patients. Knowing factors affecting rehabilitation of older persons is important for their effective and efficient rehabilitation. This article, with special reference to local studies, examines age, gender, cognitive function, and serum albumin levels as to how they influence rehabilitation in the elderly. The conclusion is that we should not exclude patients of advanced age from rehabilitation programmes, based on poor cognitive function and low serum albumin levels as they can achieve similar gains in motor and functional outcomes. A nutritional programme to improve the albumin level of older persons could speed up the rehabilitation progress, rendering it more efficient. A gender-specific rehabilitation programme is needed to improve functional outcome in men and motor outcome in women. Moreover, knowing the factors influencing residential care home placement affords an opportunity to reduce reliance on institutionalisation after rehabilitation.
 
Key words: Activities of daily living; Geriatric assessment; Mobility limitation; Rehabilitation; Self care
 
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The use of sex hormones in women with rheumatological diseases

ABSTRACT

Hong Kong Med J 2011;17:487-91 | Number 6, December 2011
MEDICAL PRACTICE
The use of sex hormones in women with rheumatological diseases
Raymond HW Li, Ailsa E Gebbie, Raymond WS Wong, Ernest HY Ng, Anna F Glasier, PC Ho
Department of Obstetrics and Gynaecology, The University of Hong Kong, Queen Mary Hospital, Pokfulam, Hong Kong
 
 
A number of rheumatological diseases predominantly affect women of reproductive age. There has always been concern that the use of oestrogen-containing agents such as combined hormonal contraception and hormone therapy might aggravate these conditions. This article reviews the up-to-date evidence regarding the safety of using these agents in women with various rheumatological diseases, with emphasis on systemic lupus erythematosus and rheumatoid arthritis. In the absence of antiphospholipid antibody or other prothrombotic risk factors, combined hormonal contraception is not contra-indicated in most rheumatological conditions including inactive systemic lupus erythematosus. Moreover, hormone therapy is generally not contra-indicated except for women with active systemic lupus erythematosus disease where its effect on disease flare is less clear and individual judgement is required.
 
Key words: Arthritis, rheumatoid; Contraceptives, oral, hormonal; Hormone replacement therapy; Lupus erythematosus, systemic; Rheumatic diseases
 
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Enzyme replacement therapy for mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): experience in Hong Kong

ABSTRACT

Hong Kong Med J 2011;17:317-24 | Number 4, August 2011
MEDICAL PRACTICE
Enzyme replacement therapy for mucopolysaccharidosis VI (Maroteaux-Lamy syndrome): experience in Hong Kong
WM But, MY Wong, Jasmine CK Chow, Winnie KY Chan, WT Ko, SP Wu, ML Wong, TY Miu, WY Tse, Winnie WY Hung, TW Fan, CC Shek
Department of Paediatrics, Queen Elizabeth Hospital, 30 Gascoigne Road, Hong Kong
 
 
Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) is a very rare inherited lysosomal storage disease. We evaluated the efficacy and safety of weekly infusions of recombinant human arylsulfatase B as enzyme replacement therapy for two patients in whom this condition was advanced. The primary outcome variables were the distance walked in a 6-minute walk test, forced vital capacity, and ejection fraction. The secondary outcome variables were the number of stairs climbed in a 3-minute stair climbing test, joint mobility, urinary glycosaminoglycan excretion, auto-continuous positive airway pressure study and liver size. After 24 weeks of treatment, patient A walked 40 m (36%) and patient B walked 66 m (58%) more in the walk test than at baseline. After 48 weeks, in patient A the corresponding improvements were 142 m (129%) in the walk test and 33 stairs (60%) in the 3-minute stair climbing test, and in patient B the respective improvements were 198 m (174%) and 77 stairs (140%). There was a significant decline in urinary glycosaminoglycan excretion and improvement in range of motion of joints in both patients. The auto-continuous positive airway pressure study revealed improvements in patient A, while other efficacy variables remained static. There were no drug-related adverse events or allergic reactions reported during and after the infusions of recombinant human arylsulfatase B. Recombinant human arylsulfatase B significantly improves endurance and reduces urinary glycosaminoglycan excretion. The drug is generally safe and well tolerated.
 
Key words: Enzyme replacement therapy; Mucopolysaccharidosis VI; N-acetylgalactosamine-4-sulfatase; Recombinant proteins; Walking
 
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Journey of a Hong Kong public teaching hospital in preparation of hospital accreditation

ABSTRACT

Hong Kong Med J 2011;17:231-6 | Number 3, June 2011
MEDICAL PRACTICE
Journey of a Hong Kong public teaching hospital in preparation of hospital accreditation
Alexander Chiu, WH Seto, Lawrence Lai
Quality and Safety, Hong Kong West Cluster, Hospital Authority, Hong Kong
 
 
Hospital accreditation is a new concept for Hong Kong Hospital Authority hospitals. Queen Mary Hospital has been engaged as one of the hospitals in a territory-wide Pilot Scheme of Hospital Accreditation. In preparation for accreditation, Queen Mary Hospital has undergone the process of self-assessment, staff engagement, and service improvements which all require well-planned strategies to achieve successful outcomes. In this article, we highlight the journey of preparation and the staff engagement exercise we conducted to attain full accreditation. We also highlight the obstacles, conundrums, and pitfalls we encountered, along with successful overcoming strategies and countermeasures we adopted, and quandaries to be avoided. Throughout the preparation, the hospital’s senior executives insisted that achieving hospital accreditation was not the main focus, but rather an emphasis on how the Pilot Scheme would bring about organisational transformations in our culture, and thus foster quality, safety, effectiveness, and reliability of services. We hope our experience can provide a reference and be of value to other hospitals that will go through the journey in the future.
 
Key words: Accreditation; Efficiency, organizational; Organizational innovation; Quality assurance, health care
 
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Minimally invasive chest drain insertion in children

ABSTRACT

Hong Kong Med J 2011;17:137-40 | Number 2, April 2011
MEDICAL PRACTICE
Minimally invasive chest drain insertion in children
Lilian PY Lee, WK Chiu, HB Chan, Kelvin KW Liu
Department of Paediatrics and Adolescent Medicine, United Christian Hospital, Kwun Tong, Hong Kong
 
 
A minimally invasive technique of chest drain insertion using the Mini Step bladeless trocar is described. Thirty-one chest drain insertions were performed with this technique between January 2007 and December 2009. It is a safe, efficient, fast, and easily acquired means of chest drain insertion in children, which has a high success rate and minimal morbidity. Overwhelmingly positive responses were obtained from doctors of all levels who used this technique, which is highly recommended for chest drain insertion in conscious children.
 
Key words: Chest tubes; Child; Drainage; Pneumothorax; Surgical instruments
 
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Training in emergency obstetric skills: is it evidence-based?

ABSTRACT

Hong Kong Med J 2011;17:141-6 | Number 2, April 2011
MEDICAL PRACTICE
Training in emergency obstetric skills: is it evidence-based?
William WK To
Department of Obstetrics and Gynaecology, United Christian Hospital, Kwun Tong, Hong Kong
 
 
In recent years, training in emergency obstetric skills has moved from the traditional clinical teaching in the labour ward to specific, targeted skills and scenario-based courses utilising mannequins and simulators. Such changes have been necessary, largely as a result of the limitations of traditional clinical teaching when it comes to genuine patient encounters in an emergency situation. The range of simulations now available extends from simple pelvic models to sophisticated computer-based birth simulators. Evidence for the effectiveness of such simulated training is gradually emerging, and in general, transfer of knowledge, improvement of clinical skills and teamwork are all enhanced. However, unequivocal evidence that such teaching improves clinical outcomes is still lacking. Further prospective research on the impact of emergency obstetric training courses as a means of reducing adverse perinatal outcomes is warranted.
 
Key words: Clinical competence; Computer simulation; Emergency medicine; Professional practice
 
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Reversible posterior leukoencephalopathy syndrome in Chinese children induced by chemotherapy: a review of five cases

ABSTRACT

Hong Kong Med J 2011;17:61-6 | Number 1, February 2011
MEDICAL PRACTICE
Reversible posterior leukoencephalopathy syndrome in Chinese children induced by chemotherapy: a review of five cases
Kiran M Belaramani, Vincent Lai, CH Li, Anselm CW Lee, NS Kwong
Department of Paediatrics and Adolescent Medicine, Tuen Mun Hospital, Tuen Mun, Hong Kong
 
 
This is a retrospective review of the clinico-radiological features and neurological outcomes of reversible posterior leukoencephalopathy syndrome episodes in Chinese cancer children receiving chemotherapy in a regional hospital in Hong Kong from 1998 to 2008. Five children (3 males and 2 females) with a mean age of 7 years were identified, four of whom had acute lymphoblastic leukaemia and one had a central nervous system germ cell tumour. Presenting symptoms included seizures (100%), altered mental function (100%), headache (40%), and visual disturbance (60%). The mean systolic blood pressure at presentation was 158 mm Hg. Approximately 80% had typical radiological features of reversible posterior leukoencephalopathy syndrome. All showed complete recovery after the acute stage, but one subsequently developed epilepsy. Two patients ultimately died of refractory malignant disease. Two others were followed up for a mean of 6 years, and remained neurologically normal. This report was the first case review documenting reversible posterior leukoencephalopathy syndrome in Chinese cancer children. The clinico-radiological features and neurological outcomes were similar to those reported in western series. Early recognition of the syndrome is important to facilitate appropriate treatment. The central nervous system damage may not be reversible and thus long-term follow-up is warranted.
 
Key words: Antineoplastic combined chemotherapy protocols; Child; Magnetic resonance imaging; Posterior leukoencephalopathy syndrome; Seizures
 
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Osteomalacia: a case series of patients with atypical clinical orthopaedic presentations

ABSTRACT

Hong Kong Med J 2010;16:476-9 | Number 6, December 2010
MEDICAL PRACTICE
Osteomalacia: a case series of patients with atypical clinical orthopaedic presentations
August WM Fok, TP Ng
Department of Orthopaedics and Traumatology, Queen Mary Hospital, Pokfulam, Hong Kong
 
 
Osteomalacia is uncommon in an affluent subtropical city like Hong Kong, where sunlight exposure is adequate and nutritional support is good. We present three patients who had osteomalacia with different presentations. A 74-year-old male with oncogenic osteomalacia presented with multiple bone pain. His biochemical markers returned to normal 4 days postoperatively after resection of a second toe giant cell tumour of tendon sheath. A 62-year-old woman with a history of liver problem and proximal muscle weakness was admitted with atraumatic fracture of the left distal humerus due to osteomalacia. An 81-year-old vegetarian woman with inadequate sun exposure complained of multiple bone pains. Subsequent investigation revealed dietary- and sunlight-deficient osteomalacia with multiple bony abnormalities including marked femur bowing.
 
Key words: Calcium, dietary; Osteomalacia; Rickets; Sunlight; Vitamin D deficiency
 
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Safe use of local anaesthetics: prevention and management of systemic toxicity

ABSTRACT

Hong Kong Med J 2010;16:470-5 | Number 6, December 2010
MEDICAL PRACTICE
Safe use of local anaesthetics: prevention and management of systemic toxicity
KC Lui, YF Chow
Department of Anaesthesiology and OT Services, Queen Elizabeth Hospital, 30 Gascoigne Road, Kowloon, Hong Kong
 
 
Local anaesthetic administration is a common clinical practice not only in the operating theatres, but also in other clinical settings. Relevant monitoring and resuscitation facilities in certain clinical settings outside the operation rooms may not be optimal. Despite the low incidence of severe complications associated with administration of local anaesthetics, their safe use is very important since systemic toxicity can be fatal. This article reviews the pharmacology of local anaesthetics, clinical features of systemic toxicity resulting from their local use, and necessary preventive measures and management. The role of lipid emulsion therapy is also discussed.
 
Key words: Analgesics/pharmacology; Anesthetics, local; Fat emulsions, intravenous; Resuscitation
 
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