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Biomedical subjects

D K K Ng

Publications and source records attributed to D K K Ng.

14 recordsLinked to original sources

Triple-A syndrome--the first Chinese patient with novel mutations in the AAAS gene.

We report on the first Chinese patient with triple-A syndrome, who presented at 22 months with status epilepticus secondary to hyponatraemia and hypoglycaemia. Subsequent endocrine investigations confirmed primary adrenal insufficiency and aldosterone deficiency. In the presence of achalasia and alacrima, this patient satisfies the diagnostic criteria of triple-A syndrome. Further molecular testing detected compound heterozygous mutations in the AAAS gene: a c.580C --> T transition in exon 7 and a c.771delG single nucleotide deletion in exon 8. Testing of parents and brother confirmed their heterozygous carrier status.

Adrenal Cortex Diseases↗

Congenital nasal pyriform aperture stenosis with semilobar holoprosencephaly.

We describe a child who has congenital nasal pyriform aperture stenosis with single maxillary central incisor, holoprosencephaly and central diabetes insipidus without any apparent anterior pituitary dysfunction. Conservative management of the congenital nasal pyriform aperture stenosis is adopted and management of diabetes insipidus is described. A literature review is undertaken.

Abnormalities, Multiple↗

Assessment of the Pediatric Index of Mortality (PIM) and the Pediatric Risk of Mortality (PRISM) III score for prediction of mortality in a paediatric intensive care unit in Hong Kong.

OBJECTIVE: To compare two models (The Pediatric Risk of Mortality III score and Pediatric Index of Mortality) for prediction of mortality in a paediatric intensive care unit in Hong Kong. DESIGN: Prospective case series. SETTING: A five-bed paediatric intensive care unit in a general hospital in Hong Kong. PATIENTS: All patients consecutively admitted to the unit between April 2001 and March 2003. MAIN OUTCOME MEASURES: Scores for both models compared with observed mortality. RESULTS: A total of 303 patients were admitted to the paediatric intensive care unit during the study period. The median age was 2 years, with an interquartile range of 7 months to 7 years. The male to female ratio was 169:134 (55.8%:44.2%). The median length of hospital stay was 3 days. The overall predicted number of deaths using The Pediatric Risk of Mortality III score was 10.2 patients whereas that by Pediatric Index of Mortality was 13.2 patients. The observed mortality was eight patients. The area under the receiver operating characteristics curve for the two models was 0.910 and 0.912, respectively. CONCLUSION: The predicted mortality using both prediction models correlated well with the observed mortality.

Child↗

Obstructive sleep apnoea syndrome and obesity in children.

OBJECTIVE: To review data on children who have both obesity and obstructive sleep apnoea syndrome. DATA SOURCE: Pubmed and MEDLINE (Ovid) literature search using the following key words: obstructive sleep apnea syndrome, obesity, and children. STUDY SELECTION: Literature and data on obesity-associated obstructive sleep apnoea syndrome in children. DATA EXTRACTION: Review of relevant information and data. DATA SYNTHESIS: Different definitions of obesity and obstructive sleep apnoea syndrome in children were used in different studies, which made it difficult to compare results from different studies conducted in different countries. Nonetheless, obstructive sleep apnoea syndrome was found to be moderately prevalent among obese children-namely, 13% to 36%. The severity of obstructive sleep apnoea syndrome was positively related to the degree of obesity. Blood pressure was found to be elevated in obese children with obstructive sleep apnoea syndrome. Weight reduction is an effective treatment. CONCLUSION: Children with obesity and obstructive sleep apnoea syndrome face a double challenge. A holistic approach to management requires a clear understanding of how both problems interact.

Child↗

Hepatotoxicity and persistent renal insufficiency after repeated supratherapeutic paracetamol ingestion in a Chinese boy.

Paracetamol has always been regarded as a useful and safe drug. The risk of toxicity with repeated supratherapeutic paracetamol is an underrecognised condition. We report on a 12-month-old boy who presented with hepatotoxicity, disseminated intravascular coagulation and persistent renal insufficiency 4 days after repeated ingestion of a supratherapeutic dosage of paracetamol. To the best of our knowledge, this is the first reported case of paediatric chronic paracetamol poisoning among the Chinese population. In addition, persistent renal insufficiency has not been a previously reported feature of chronic paracetamol poisoning. We propose that renal damage is the result of the synergistic effect of hypoperfusion and paracetamol overdose.

Acetaminophen↗

Chronic cough in children.

Chronic cough, defined as coughing for more than four weeks, is a common childhood complaint. With careful history-taking and appropriate investigations, a single cause can be found in most cases--these can be successfully treated. Although we have some understanding about the cough reflex through animal studies, the full mechanism and exact location of the responsible neurons in the human brain have not been completely elucidated. There are many causes for it but asthma, chronic rhinosinusitis and gastroesophageal reflux disease account for most of the cases. Other causes, such as dysfunctional swallowing, congenital anomalies and cigarette smoking, are also important. All children with chronic cough deserve a thorough and proper evaluation. The current review provides essential information for medical practitioners to approach this problem. An algorithm is provided to aid the diagnostic process.

Child↗

Clinical risk factors for obstructive sleep apnoea in children.

OBJECTIVE: To identify the clinical factor(s) that identify obstructive sleep apnoea syndrome (OSAS) in children. METHODS: A prospective study of children referred to the sleep clinic of the paediatric department was conducted in a public non-teaching regional hospital in Hong Kong. A standard questionnaire was administered and overnight sleep polysomnography was performed in a consecutive series of patients. Logistic regression analysis was performed to obtain significant risk factors for prediction of OSAS in this series of patients. RESULTS: Sixty-two children were enrolled into the study and 22 were diagnosed to have OSAS. Logistic regression analysis showed that, among all the answers, 'snoring every night' is the single most significant risk factor (p<0.0001) to predict OSAS. 'Snoring every night' has a sensitivity of 91% and specificity of 75% for OSAS patients. It also has a positive predictive value of 67% and negative predictive value of 94%. CONCLUSION: Snoring every night is an important risk factor in identifying OSAS in children. Priority for an overnight sleep polysomnogram should be given to those with this symptom.

Adolescent↗

Childhood fever revisited.

The academic study of body temperature began in 1868. Since then, a variety of thermometers have been developed for clinical use. A working knowledge of these different thermometers, the various sites for taking temperature, and the normal range of body temperatures, is essential. As the pathogenesis of fever is being elucidated, and the cytokines involved identified, the risks and benefits of fever to the organism are becoming better understood. The importance of recognising diagnostic fever patterns should be stressed. Acetaminophen (paracetamol) and ibuprofen are accepted as the standard medical treatments for fever.

Acetaminophen↗

Review of children with severe trauma or thermal injury requiring intensive care in a Hong Kong hospital: retrospective study.

OBJECTIVE: To study the injury pattern of children admitted for management of severe trauma or thermal injury. DESIGN: Retrospective review. SETTING: Paediatric intensive care unit of a regional hospital, Hong Kong. PATIENTS: Twenty-eight children were admitted under this category from July 1996 to December 1999. MAIN OUTCOME MEASURES: Mechanisms, severity, and circumstances of injury. RESULTS: Road traffic accident was the most common cause of admission, followed by thermal injury, accidental fall, and non-accidental injury. However, children with non-accidental injury were admitted in a significantly more severe condition, as measured by the paediatric risk of mortality score, than those admitted for the other three reasons. Non-accidental injury was also associated with significantly higher morbidity and mortality than the other causes of admission. CONCLUSIONS: During the 42-month study period, trauma and thermal injury accounted for 7% of all admissions to the paediatric intensive care unit. Road traffic accident was the most common reason, while non-accidental injury accounted for the most serious injury. Detailed analysis of these cases identified certain preventable risk factors.

Accidental Falls↗

Habitual snoring and sleep bruxism in a paediatric outpatient population in Hong Kong.

OBJECTIVE: To determine the prevalence of habitual snoring and sleep bruxism in children attending the out-patient clinics of a paediatric department. METHODOLOGY: A cross-sectional survey of parents was conducted with questionnaire administered by paediatric nurses. Parents were recruited when they brought their children to the out-patient clinics. Sex and age were recorded. Presence and absence of habitual snoring and sleep bruxism were noted. Types of diseases that brought the children to the out-patient clinics were also noted. RESULTS: Twenty-nine of the 200 recruited children were noted to have habitual snoring (14.5%, 95% C.I. 10%-20%). The mean age of these habitual snorers was 6.2 +/- 3.1 years. For habitual snorers, male to female ratio was 1.4 to 1. Sixteen of these 28 children accepted a sleep polysomnographic examination. Eleven children were found to have snoring during the night of study. Two were found to have obstructive sleep apnoea syndrome. Sleep bruxism was found in 17 children (8.5%, 95% C.I.5%-13%). Sleep bruxism was closely related to habitual snoring as 16 out of the 17 children with sleep bruxism were also habitual snorers (p < 0.0001). CONCLUSION: Habitual snoring and sleep bruxism were commonly found in children attending paediatric clinics. Paediatricians should be aware of these problems and be prepared to deal with them. Habitual snoring and sleep bruxism were closely related. Further studies into this relationship is needed.

Ambulatory Care Facilities↗