Utility of the Pediatric Allergic Disease Quality of Life Questionnaire for childhood eczema.

BACKGROUND: Childhood atopic dermatitis (AD) is a chronic inflammatory disease associated with pruritus and sleep loss. It is important to evaluate quality-of-life (QoL) impairment objectively in atopic diseases in children. OBJECTIVES: To investigate the utility of the Pediatric Allergic Disease Quality of Life Questionnaire (PADQLQ) in children with eczema. METHODS: PADQLQ, Patient-Oriented Eczema Measure (POEM, a short-term subjective symptom score), Nottingham Eczema Severity Score (NESS, a long-term subjective symptom score) and Children's Dermatology Life Quality Index (CDLQI, a short-term subjective symptom score) were compared and correlations evaluated. RESULTS: PADQLQ, POEM, NESS and CDLQI correlated well with each other (n = 132 sets; Spearman correlations: rho = 0·48-0·70, P < 0·001). A Bland-Altman plot showed a reasonably good agreement between CDLQI and PADQLQ. PADQLQ showed that symptoms of asthma, allergic conjunctivitis and allergic rhinitis were present in 20-30%, 45-71% and 58-67% of children with AD, respectively. Nevertheless, there was no association of eczema symptomatology by POEM or NESS with the severity of other allergic diseases. CONCLUSIONS: PADQLQ correlates well with AD-specific severity and QoL scores and reflects all allergic symptoms that holistically influence QoL in children with AD. PADQLQ is hence a composite severity score in terms of clinical symptomatology and QoL impairment for AD.

Epidemiology and risk factors for severe respiratory syncytial virus infections requiring pediatric intensive care admission in Hong Kong children.

PURPOSE: Respiratory syncytial virus (RSV) is a common cause of lower respiratory tract infection in young children. However, there are limited data on severe RSV infection requiring pediatric intensive care unit (PICU) admission. This retrospective study described features of RSV-associated PICU admissions in Hong Kong and investigated factors for mortality and duration of PICU stay. METHODS: Children with laboratory-confirmed RSV infection and admitted to the PICUs of all eight government hospitals in Hong Kong between January 2009 and June 2011 were identified from computerized auditing systems and PICU databases. RSV in respiratory samples was detected by direct immunofluorescence and/or viral culture. The relationships between mortality and PICU duration and demographic and clinical factors were analyzed. RESULTS: A total of 118 (2.4 %) PICU admissions were identified among 4,912 RSV-positive pediatric cases in all hospitals. Sixty-five (55.6 %) patients were infants. PICU admissions were higher between October and March. Eight (6.8 %) patients died, but only two were infants. RSV-associated mortality was related to prior sick contact, presence of older siblings, neurodevelopmental conditions, chromosomal and genetic diseases, and bacterial co-infections, but none was significant following logistic regression analyses (odds ratio 9.36, 95 % confidence interval 0.91-96.03 for prior sick contact, p = 0.060). Chronic lung disease was the only risk factor for the duration of PICU admission (ß = 0.218, p = 0.017). CONCLUSIONS: The majority of RSV-infected children do not require PICU support. There is winter seasonality for RSV-associated PICU admission in Hong Kong. Prior sick contact is the only risk factor for RSV-associated mortality, whereas the presence of chronic lung disease is associated with longer PICU stay. The current risk-based approach of RSV prophylaxis may not be effective in reducing severe RSV infections.

Severe neonatal manifestations of Costello syndrome.

BACKGROUND: Costello syndrome (CS) is due to mutations in HRAS, with the most common mutation being c.34G>A (p.G12S), found in most patients in all the published series. A small number of less common mutations have been reported. POPULATION STUDIED: HRAS mutation analysis has been undertaken in 74 predominantly British patients with a possible diagnosis of CS. A HRAS mutation was found in 27 patients, 15 of whom have been previously reported. PHENOTYPE ANALYSIS: Four cases had an unusually severe phenotype, associated in three cases with two unusual mutations, c.35G>A, p.G12D in two cases and c.34G>T, p.G12C in the other. Hypoglycaemia, renal abnormalities, severe early cardiomyopathy, congenital lung and airway abnormalities, pleural and pericardial effusion, chylous ascites and pulmonary lymphangectasia are confirmed as part of the clinical spectrum seen in CS. A lung pathology resembling alveolar capillary dysplasia is reported in one case. CONCLUSION: These cases illustrate that the diagnosis of CS may be difficult in the newborn period, and should be considered in the differential diagnosis of the sick newborn infant with multisystem disease. Study of more cases will be required to establish if there is a definite association between severe disease and less common mutations.

Pharyngomalacia as a cause of severe neonatal stridor.

A male neonate presented at 24 hours with stridor and respiratory distress. Flexible bronchoscopy showed pharyngomalacia, i.e. complete pharyngeal wall collapse during inspiration. Assessment of upper airway dynamics is emphasised. Pharyngomalacia seems to be a self-limiting condition in our case.

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.

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.

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.

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.

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.

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.

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.

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.