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Biomedical subjects

Virginia Wong

Publications and source records attributed to Virginia Wong.

36 records · Page 2Linked to original sources

Carrier incidence for spinal muscular atrophy in southern Chinese.

A real time quantitative PCR (QPCR) method using TaqMan technology was used to assess the copy number of the two survival motor neuron genes (SMN1 and SMN2) on chromosome 5q13. This allows the accurate determination of carriers for spinal muscular atrophy (SMA), with one copy of SMN1. Analysis of 569 normal southern Chinese individuals revealed a carrier incidence of 1.6%, similar to that found in the western society. Study of 42 obligatory carriers showed a (2 + 0) genotype in two (4.8 %). In 27 SMA patients with homozygous deletion of the SMN1 gene, the number of SMN2 gene correlated with disease phenotype, with 68% of type II and III patients carrying three or more SMN2 genes, whilst the incidence of three or more SMN2 genes in the normal population was 1.57%.

Asian People↗

Ultrasound measurement of lumbosacral spine in children.

The aim of this study was to establish normal ultrasound measurements of lumbosacral spine in children as a screening assessment of tethered cord or postoperative retethering of cord. Sonography of lumbosacral spine was performed in 108 neurologically normal children (mean age = 2.1 years) using 5- to 12-MHz linear transducer. M-mode was applied at the posterior and anterior subarachnoid spaces just below the conus medullaris and at the L(5)/S(1) dural sac to document cerebrospinal fluid pulsation of the cauda equina. The oscillation rate and amplitude were measured. Sixteen children with spinal cord anomalies (6 with low tethered cord and 10 postoperative cases of low tethered cord) were also examined. The mean posterior/anterior subarachnoid spaces of normal children were 2.6 mm/1.8 mm at the terminal dural sac. The mean oscillation amplitude and rate of the cauda equina were 0.52 mm and 121/min at the L(5)/S(1) dural sac. The oscillation amplitude at this level demonstrated a statistically significant difference between normal and abnormal groups. In conclusion, we recommend taking the fifth percentile of the normal oscillation amplitude at the L(5)/S(1) dural sac as a reference. Any oscillation amplitude of less than 0.3 mm in a symptomatic patient should alert the clinician to possible cord abnormality, cord tethering, or retethering in postoperative cases.

Chi-Square Distribution↗

Use of developmental language scales in Chinese children.

The Reynell Developmental Language Scale (RDLS) and Symbolic Play Test (SPT) have been useful language tests for assessing the language age of children. Both tests have been validated in English-speaking children. However, there have been no studies conducted for Chinese children, whether Mandarin (Northerners) or Cantonese (Southerners) is used as the main dialect. As the Chinese population is the largest ethnic group in the world, and Chinese emigration occurred to nearly all parts of the world, it is essential to test whether these language tools can be applied for this ethnic group. The objective of this research was to study whether RDLS and SPT are useful in assessing the language age of Chinese children. Both RDLS (Chinese version) and SPT are conducted for 116 Chinese (Cantonese-speaking) children, aged 13-59 months, in Hong Kong. There is a significant positive correlation of the language age using RDLS and SPT with the chronological age of Chinese children. Both RDLS and SPT can be adopted in determining the language/mental age of Chinese (Cantonese-speaking) children.

Age Factors↗

Environmental mercury exposure in children: South China's experience.

BACKGROUND: Environmental mercury levels significantly increased in the past decades following its increase in industrial applications. In spite of an increasing concern on the potential harmful effects of mercury on children, there is no reported data for the Chinese population. The relationship between dietary habit and environmental mercury exposure in Chinese children was studied. METHODS: The hair and blood mercury levels of Chinese children aged above 3 years in 2000 March to September, were studied. Sociodemographic data, dietary habits of the past 6 months, and other risk factors for environmental mercury exposure were collected. Those children with blood mercury levels above the toxic range (i.e. > 45 nmol/L) and their family members were further evaluated and their blood and hair mercury levels were monitored before and after Fishing-Moratorium period (June to August 2000) in South China Sea. RESULTS: Altogether, 137 Chinese children (mean age, 7.2 years) were recruited. The mean hair mercury level was 2.2 p.p.m and the mean blood mercury level was 17.6 nmol/L. There was a strong correlation (r = 0.88) between hair and blood mercury levels in our cohort. Frequency of fish consumption correlated with hair (r = 0.51) and blood (r = 0.54) mercury levels. For those children who consumed fish more than 3 times/week, hair and blood mercury levels were twice as high as those who consumed fish l-3 times/week and threefold of those who never consumed fish. Five children and 12 family members had toxic blood mercury levels. Their blood (P < 0.0001) and hair (P = 0.02) mercury levels dropped significantly after reducing fish consumption during Fishing-Moratorium period. CONCLUSION: Both blood and hair (i.e. Tissue) mercury levels of children in Hong Kong was elevated and correlated with the frequency of fish consumption.

Age Factors↗

Video-fluoroscopic study of swallowing in children with neurodevelopmental disorders.

BACKGROUND: Children with neurodevelopmental disorders can have feeding problems. Malnutrition and recurrent aspiration pneumonia can increase the risk of morbidity and mortality. Video-fluoroscopic study of swallowing (VFSS) is essential in understanding the pathological mechanisms involved during swallowing. METHODS: The aim of the present study was to assess the role of VFSS in assessment and management of four children with various neurodevelopmental disorders in a multidisciplinary feeding team. We describe the team approach, with the participation of child neurologist, radiologist with the rehabilitation team including the speech therapist, occupational therapist and dietician, in the assessment and plan of management. RESULTS: Video-fluoroscopic study of swallowing had been useful in assessing the type of swallowing problems with treatment goals targeted to the basic underlying pathophysiological mechanism. CONCLUSION: A child neurologist should be involved in the multidisciplinary oromotor rehabilitation program for neurologically impaired children with feeding problems.

Adolescent↗

Study of seizure and epilepsy in Chinese children in Hong Kong: period prevalence and patterns.

The Hong Kong Children's Seizure and Epilepsy Registry was set up for all children with a history of seizures or epilepsy followed up in the Epilepsy Clinic of the University of Hong Kong. The aim was to study the pattern of seizures and epileptic disorders in Chinese children. The objective was to study the period prevalence of epilepsy and the pattern of epilepsy in Chinese children. A prospective study of seizure and epilepsy in Chinese children was conducted from 1985 to 1997 in Hong Kong. The population census of 1997 was used to calculate the period prevalence of epilepsy in Chinese children. Altogether, 1103 children aged < 19 years at first assessment with epilepsy were included in the study. The period prevalence rate of epilepsy in 1997 (January to December) is estimated to be 4.5 per 1000 children aged < 19 years. The estimated period prevalence rate of children and adolescents with epilepsy in our children is 4.5 per 1000 children.

Adolescent↗

Mercury exposure in children with autistic spectrum disorder: case-control study.

Although mercury has been proven to be a neurotoxicant, there is a lack of data to evaluate the causal relationship between mercury and autism. We aim to see if there is increased mercury exposure in children with autistic spectrum disorder. We performed a cross-sectional cohort study over a 5-month period in 2000 to compare the hair and blood mercury levels of children with autistic spectrum disorder (n = 82; mean age 7.2 years) and a control group of normal children (n = 55; mean age 7.8 years). There was no difference in the mean mercury levels. The mean blood mercury levels of the autistic and control groups were 19.53 and 17.68 nmol/L, respectively (P = .15), and the mean hair mercury levels of the autistic and control groups were 2.26 and 2.07 ppm, respectively (P = .79). Thus, the results from our cohort study with similar environmental mercury exposure indicate that there is no causal relationship between mercury as an environmental neurotoxin and autism.

Autistic Disorder↗

Spectrum of anticonvulsant hypersensitivity syndrome: controversy of treatment.

An 18-month-old girl presented with a maculopapular rash 10 days after carbamazepine treatment. Initially, she was suspected of having a viral rash owing to associated fever. She deteriorated rapidly and was suspected of having anticonvulsant hypersensitivity syndrome or Stevens-Johnson syndrome. She developed features compatible with toxic epidermal necrolysis rapidly over 24 to 36 hours. Carbamazepine was then stopped. She responded immediately to high-dose intravenous pulse methylprednisone treatment. We discuss the controversy in the management of anticonvulsant hypersensitivity syndrome, toxic epidermal necrolysis, or Stevens-Johnson syndrome with high-dose corticosteroids, intravenous immunoglobulin, and antibiotics.

Anticonvulsants↗

Cerebral palsy: correlation of risk factors and functional performance using the Functional Independence Measure for Children (WeeFIM).

We studied the risk factors affecting the functional status of cerebral palsy. A cross-sectional study of 73 children with cerebral palsy was conducted with the Functional Independence Measure for Children (WeeFIM), which assesses functional skills in the domains of self-care, mobility, and cognition. The mean total Functional Independence Measure for Children quotient was 67.5%. The mean subquotients for self-care, mobility, and cognition were 68.3%, 62.7%, and 69.4%, respectively. The risk factors related to the degree of functional dependency were (1) mental retardation (P = .030), (2) epilepsy (P = .005), (3) type of cerebral palsy (P < .001), and (4) severity of cerebral palsy using the Gross Motor Function Classification System (P < .001) (using univariate analysis). However, when using multivariate analysis, only epilepsy (P = .02) and severity status according to the Gross Motor Function Classification System (P < .001) were significantly related. When the etiology was analyzed, only prematurity was significantly associated with better Functional Independence Measure for Children scores using both univariate (P = .022) and multivariate (P = .007) analyses. The functional status of children with cerebral palsy depends on the severity and the presence of epilepsy. Despite impairment, we found that most children with cerebral palsy could achieve functional independence.

Activities of Daily Living↗

A modified screening tool for autism (Checklist for Autism in Toddlers [CHAT-23]) for Chinese children.

BACKGROUND: There is a recent trend of a worldwide increase in the incidence of autistic spectrum disorder. Early identification and intervention have proved to be beneficial. The original version of the Checklist for Autism in Toddlers (CHAT) was a simple screening tool for identification of autistic children at 18 months of age in the United Kingdom. Children with an absence of joint attention (including protodeclarative pointing and gaze monitoring) and pretend play at 18 months were at high risk of autism. Section A of the CHAT was a self-administered questionnaire for parents, with 9 yes/no questions addressing the following areas of child development: rough and tumble play, social interest, motor development, social play, pretend play, protoimperative pointing (pointing to ask for something), protodeclarative pointing, functional play, and showing. Section B of the CHAT consisted of 5 items, which were recorded with observation of the children by general practitioners or health visitors. The 5 items addressed the child's eye contact, ability to follow a point (gaze monitoring), pretend (pretend play), produce a point (protodeclarative pointing), and make a tower of blocks. A 6-year follow-up study of >16,000 children screened with the CHAT at 18 months in the United Kingdom showed a sensitivity of only 0.40 and a specificity of 0.98, with a positive predictive value (PPV) of 0.26. Rescreening using the same instrument at 19 months for those who failed the 18-month screening yielded a higher PPV of 0.75. Therefore, children were likely to have autism if they failed the CHAT at 18 months and failed again at 19 months. It was estimated that consistent failure in 3 key questions (ie, protodeclarative pointing, gaze monitoring, and pretend play) at 18 months indicated an 83.3% risk of having autism. Because of the poor sensitivity of the original CHAT for autism, a Modified Checklist for Autism in Toddlers (M-CHAT), consisting of 23 questions, with 9 questions from the original CHAT and an additional 14 questions addressing core symptoms present among young autistic children, was designed in the United States. The original observational part (ie, section B) was omitted. The M-CHAT was designed as a simple, self-administered, parental questionnaire for use during regular pediatric visits. The more questions children failed, the higher their risk of having autism. Two criteria were used to measure the sensitivity and specificity of M-CHAT. Criterion 1 used any 3 of the 23 questions, and criterion 2 used 2 of the 6 best questions that could be used to discriminate autism from other groups. The sensitivity and specificity for criterion 1 were 0.97 and 0.95 and those for criterion 2 were 0.95 and 0.99, respectively. M-CHAT had a better sensitivity than the original CHAT, because children up to 24 months of age were screened, with the aim of identifying those who might regress between 18 and 24 months. The 6 best questions of the M-CHAT addressed areas of social relatedness (interest in other children and imitation), joint attention (protodeclarative pointing and gaze monitoring), bringing objects to show parents, and responses to calling. Joint attention was addressed in the original CHAT, whereas the other areas were addressed only in the M-CHAT. To date, there has been no study of the application of either the original CHAT or the M-CHAT for Chinese populations. OBJECTIVES: CHAT-23 is a new checklist translated into Chinese, combining the M-CHAT (23 questions) with graded scores and section B (observational section) of the CHAT. We aimed to determine whether CHAT-23 could discriminate autism at mental ages of 18 to 24 months for Chinese children and to determine the best combination of questions to identify autism. METHODS: A cross-sectional cohort study was performed with 212 children with mental ages of 18 to 24 months. The children were categorized into 2 groups, ie, group 1 (N = 87) (autistic disorder: N = 53; pervasive developmental disorder: N = 33) and group 2 (N = 125) (nonautistic). The checklist included self-ad25) (nonautistic). The checklist included self-administered questionnaires with 23 questions (part A) and direct observations of 5 items by trained investigators (part B). We performed discriminant function analysis to We found that 7 key questions, addressing areas of joint attention, pretend play, social relatedness, and social referencing, were identified as discriminative for autism. For part A, failing any 2 of 7 key questions, ie, question 13 (does your child imitate you? [eg, you make a face; will your child imitate it?]), question 5 (does your child ever pretend, for example, to talk on the phone or take care of dolls, or pretend other things?), question 7 (does your child ever use his/her index finger to point, to indicate interest in something?), question 23 (does your child look at your face to check your reaction when faced with something unfamiliar?), question 9 (does your child ever bring objects over to you [parent] to show you something?), question 15 (if you point at a toy across the room, does your child look at it?), and question 2 (does your child take an interest in other children?), yielded sensitivity of 0.931 and specificity of 0.768. Failing any 6 of all 23 questions produced sensitivity of 0.839 and specificity of 0.848. For part B, failing any 2 of 4 items produced sensitivity of 0.736, specificity of 0.912, and PPV of 0.853. The 4 observational items were as follows: item B1: during the appointment, has the child made eye contact with you? item B2: does the child look across to see what you are pointing at? item B3: does the child pretend to pour out tea, drink it, etc?; item B4: does the child point with his/her index finger at the light? CONCLUSION: We found that integrating the screening questions of the M-CHAT (from the United States) and observational section B of the original CHAT (from the United Kingdom) yielded high sensitivity and specificity in discriminating autism at 18 to 24 months of age for our Chinese cohort. This new screening instrument (CHAT-23) is simple to administer. We found that a 2-stage screening program for autism can offer a cost-effective method for early detection of autism at 18 to 24 months. For CHAT-23, use of both the parental questionnaire and direct observation and use of the criterion of failing any 2 of 7 key questions yielded the highest sensitivity but a relatively lower specificity, whereas use of part B yielded the highest specificity but a lower sensitivity. We recommend identifying the possible positive cases with part A (parental questionnaire) and then proceeding to part B (observation) with trained assessors. The proposed algorithm for screening for autism is as follows. 1) The parents or chief caretakers complete a 23-item questionnaire when their children are 18 to 24 months of age. 2) The parents mail, fax, or hand this 23-item questionnaire to the local child health agency. 3) Clerical staff members check for and score failure, with the criteria of failing any 2 of 7 key questions or failing any 6 of 23 questions; if either criterion is met, then the staff members highlight the medical records of the suspicious cases. 4) Trained child health care professionals observe the children who failed any 2 of 7 key questions or any 6 of 23 questions. These identified patients are observed for 5 minutes for part B of the CHAT-23. 5) Any child who fails any 2 of 4 items requires direct referral to a comprehensive autism evaluation team, for early diagnostic evaluation and early intervention. The high sensitivity and specificity of the criteria observed in our study suggested that CHAT-23 might be used to differentiate children with autism. Additional international collaboration with the use of the CHAT, M-CHAT, and CHAT-23 could provide more prospective epidemiologic data, to establish whether there is a genuine increase in the worldwide incidence of autism.

Attention↗

Pediatric stroke among Hong Kong Chinese subjects.

BACKGROUND: The incidence of pediatric stroke was estimated to be 2.5 to 2.7 cases per 100,000 children per year in North America and 13 cases per 100,000 children per year in France. Stroke is among the top 10 causes of death among children in the United States, with the highest incidence in the first 1 year of life. The annual mortality rate was 0.34 deaths per 100,000 person-years, with an average of 244 deaths per year. Interethnic differences have been demonstrated to be important in pediatric stroke. However, most population-based studies on pediatric stroke were from Europe or North America, and there was a lack of data on the incidence of stroke among Chinese or Asian children. Whether the etiologic patterns and risk factors for death and morbidity among Chinese children with stroke were similar to those described for other ethnic groups was unknown. OBJECTIVES: To calculate the incidence of stroke among Chinese children in Hong Kong and to examine the clinical spectrum, causes, patterns, risk factors, and outcomes of pediatric stroke among Chinese subjects. METHODS: The population of Hong Kong was 6.7 million in 2001, and >98% of our population is Chinese in origin. In Hong Kong, public hospitals under the Hospital Authority provide >95% of the hospital service for the region. We identified children (>1 month to <15 years of age) who were admitted and given a discharge diagnosis of stroke from the Clinical Data Analysis and Reporting System, which is a centralized computerized database for all public hospitals. The discharge coding of stroke used codes from the International Classification of Diseases, 9th Revision, Clinical Modification. Only first admissions during the study period were included. We excluded any subsequent admissions by using multiple demographic characteristics of the patients. The incidence of pediatric stroke was estimated as the number of first hospitalizations divided by the person-years at risk. Since 1991, we had been collecting a database on pediatric stroke (ages of 1 month to 16 years) from a single center (the university-affiliated pediatric unit). The clinical presentation, causes, risk factors, and outcomes for those in the Hong Kong Children's Stroke Registry with follow-up data for > or =2 years were analyzed. Data on outcomes, in terms of survival and neurologic deficits, were studied. For survivors, neurologic deficits were defined as short-term if they resolved within 3 months and long-term if they persisted for >3 months. The severity of deficits was defined as mild when function was minimally affected and the patient remained independent in activities of daily living, moderate when the patient required supervision or partial assistance in activities of daily living or when the deficit caused delay in developmental milestones, and severe when the patient required total or near-total care in activities of daily living. Potential risk factors for death and poor neurologic outcomes, including gender, age at the time of stroke, clinical presentation, causes, and neuroimaging findings, were analyzed. RESULTS: Using projections from census data in 2001, the number of children <15 years of age in Hong Kong from 1998 to 2001 was estimated to be 1,104100 to 1,158800, resulting in 4,545300 person-years. During the same period, 94 children with discharge coding of stroke were identified. Therefore, the estimated incidence of pediatric stroke between 1998 and 2001 was 2.1 cases per 100,000 children-years. The average number of new cases treated annually was 4.5 (0-15 cases/year). Fifty children (28 boys and 22 girls; male/female ratio: 1.27:1) were identified in the 11-year period. The mean age at presentation was 5.6 +/- 4.9 years. Thirty-six strokes (72%) were ischemic and 14 (28%) were hemorrhagic. Despite evaluation for possible underlying causes, 12% (6 cases) remained idiopathic. Eighteen patients with ischemic strokes had cerebral thrombosis, whereas 15 had cerebral embolism. We did not observe any case of sinovenous thrombosis. The 36 cases of ischemic stroke were subtyped according to vascular territories. Eleven cases had infarction involving the middle cerebral artery territory; 2 were limited to the cortical region, 3 were limited to subcortical structures such as the basal ganglia or internal capsule or both, and 6 had complete middle cerebral artery involvement, with cortical and subcortical stroke. Involvement of the anterior cerebral artery occurred in 2 cases, with involvement of cerebellar/basilar artery territories in another 2 cases. The remaining 15 cases had multiple sites of infarction. Three patients experienced secondary hemorrhagic transformation after the initial thrombotic event. Of the 14 patients with hemorrhagic strokes, only 1 had subarachnoid hemorrhage. All others had intracerebral bleeding, at single (N = 9) or multiple (N = 4) loci. Important causes included complications related to congenital heart diseases (N = 15, 30%), vascular diseases (N = 13, 26%), and hematologic diseases (N = 14, 28%). Six cases had no determined causes. One case involved mitochondrial encephalopathy with lactic acidosis and stroke-like episodes and constituted the only case with a metabolic cause. For the 7 patients for whom prothrombotic screening was performed, findings were negative. Seizures (52%) and hemiplegia (34%) were the most common presenting features. Other presenting clinical features included headaches (22%), decreased consciousness (30%), visual field defects (12%), dysphasia (10%), and lethargy (8%). Only 1 patient, with moyamoya disease, had a family history of stroke. The median follow-up time was 8.7 years (range: 2-12.4 years). Nine patients (18%) died, 5 with ischemic stroke and 4 with hemorrhagic stroke. Among the 5 cases of death with ischemic stroke, 3 involved hemorrhagic transformation before death. Seven patients (77%) died within 31 days (range: 2-31 days), whereas the other 2 died 6 months and 2.5 years after the episode. Recurrence occurred in 5 cases (10%). Long-term neurologic deficits occurred among 41% of survivors, including mental retardation (N = 11), epilepsy (N = 7), and hemiplegia (N = 10). The functional deficits were classified as severe in 7 cases, moderate in 3 cases, and mild in 7 cases, for patients with long-term neurologic deficits. Decreased levels of consciousness, hematologic causes, and hemorrhagic transformation (applicable only in ischemic stroke) were significant risk factors associated with high mortality rates. For the 41 patients who survived, the only significant risk factor for long-term neurologic deficits was seizures at the initial presentation. Other factors, such as gender, age, other clinical features, stroke type, vascular territory, other causes, and recurrence of stroke, were all insignificant for both death and long-term deficits. The 3 risk factors identified for death were analyzed in multivariate logistic regression analyses, with adjustment for the confounding variables, and only decreased levels of consciousness remained significant (odds ratio = 15.6). CONCLUSIONS: The incidence of stroke among Chinese children was slightly lower than that in Europe or North America. The etiologic pattern was different in our cohort, and there was no sickle cell anemia, thrombophilia, or sinovenous thrombosis. Despite these differences, however, mortality and long-term neurologic deficit rates were similar.

Adolescent↗

Prevalence of neuromuscular diseases in Chinese children: a study in southern China.

Our objective was to study the prevalence of neuromuscular diseases in Chinese children. A prospective study of neuromuscular diseases in Chinese children was conducted from 1985 to 2001 in Hong Kong, which is a city in southern China. The population census of June 30, 2001, was used to calculate the prevalence of neuromuscular diseases in Chinese children. Altogether, 332 children aged < 19 years at first assessment with neuromuscular diseases confirmed by using electromyography, muscle biopsy, and/or molecular genetic study were included in the study. Of these, 228 (68%) had inherited and 104 (32%) had noninherited neuromuscular diseases. Of the inherited neuromuscular diseases, the most common were the dystrophinopathies, including Duchenne muscular dystrophy (n = 66) and Becker muscular dystrophy (n = 8). Spinal muscular atrophy was the second most common (n = 61). Of the noninherited neuromuscular disorders, myasthenia gravis was the most common (n = 62, 60%). Nearly 88% of the cases of myasthenia gravis were ocular type. The prevalence rate of neuromuscular diseases in June 2001 (n = 291 surviving) is estimated to be 214 x 10(-6). The estimated prevalence rate of neuromuscular diseases in our Chinese children is 1 in 4669.

Adolescent↗

Use of chopsticks in Chinese children.

OBJECTIVE: There has been no study on the developmental stage of acquiring the skill of using chopsticks, which is a common eating tool of the Orientals. We aimed to obtain a developmental profile for achieving the skills of chopsticks manipulation in Chinese children and to assess the correlation between chopsticks manipulation and the level of achieving independence in 'eating' item in the Functional Independence Measure of children (WeeFIM). We also studied the relationship between demographic and environmental factors and the age of achieving chopstick manipulation. METHODS: Direct interview with the mothers of 445 non-disabled children who were randomly selected from the community. A chopsticks score (CS) of 1 was defined as children who could use chopsticks to finish more than half the meal, and a CS of 0 as failure to do so. RESULTS: The mean age of achieving a CS of 1 was 4.6 years. At 6.7 years, all these children achieved this skill. There was a significant relationship between age of achieving the skills of chopsticks and the level of attaining independence in 'WeeFIM eating' score. (P < 0.001) However, there was no correlation of age of achieving CS of 1 with sex, social class or the presence of a domestic helper. CONCLUSION: Most non-disabled Chinese children can achieve the visual motor skill of using chopsticks at 4.6 years. There was also a correlation with the age of achieving independence in the WeeFIM eating score. The skill of using chopsticks should be added as part of the tools, apart from spoons or forks, for scoring WeeFIM in Chinese children.

Child↗

Clara cell secretory protein deficiency alters clara cell secretory apparatus and the protein composition of airway lining fluid.

Clara cells represent the predominant secretory cell within distal conducting airways of mammals and exhibit functional alterations with chronic lung disease. We previously demonstrated that Clara cell secretory protein (CCSP) deficiency results in enhanced susceptibility to environmental agents. The present study was undertaken to define changes in Clara cell secretory function associated with CCSP deficiency in knockout mice. Comparative morphometry of Clara cell ultrastructure revealed dramatic alterations in secretory apparatus between wild-type (WT) and CCSP knockout (CCSP-/-) mice. Secretory granules, which occupy greater than 2% of Clara cell cytoplasmic volume in WT mice, were completely absent among Clara cells of CCSP-/- mice. Moreover, Clara cells of CCSP-/- mice exhibited a > 95% reduction in rough endoplasmic reticulum and alterations to Golgi apparatus, relative to WT controls. Ultrastructural perturbations to Clara cells were associated with altered protein composition of airway lining fluid as revealed by two-dimensional gel analysis of bronchoalveolar lavage proteins, but were not associated with altered abundance or secretion of CC26, another Clara cell secretory protein. We conclude that CCSP is required for the appearance of Clara cell secretory granules and that functional changes to Clara cells that result from CCSP deficiency lead to alterations in the composition of epithelial lining fluid.

Animals↗

Open-label study of botulinum toxin for upper limb spasticity in cerebral palsy.

The objective of this study was to assess the efficacy of botulinum toxin for upper limb spasticity in cerebral palsy. An open-label study was conducted in 11 children with cerebral palsy. Post-botulinum toxin assessment was conducted in weeks 1, 4, and 16, with averaged scores being analyzed. The Clinical Global Impression Scale of the mothers showed marked, moderate, and mild improvement in five, four, and two cases, respectively. The Modified Ashworth Spasticity Scale score showed a statistically significant decrease in the mean spasticity score (P < .003). Other tests were performed depending on the cognition of the child. Increase in joint motion occurred in all five children assessed using web space (P = .043). For the Jebson Hand Function Test, improvement occurred in all five cases assessed (P < .03). Four of five (80%) children could perform tasks that they failed before they were given botulinum toxin. Botulinum toxin is useful in decreasing spasticity and improving the upper limb function of young children with cerebral palsy with normal cognition. Motivated families should be selected with a specific target of using botulinum toxin as an adjunct in a habilitation program.

Adolescent↗

Functional Independence Measure (WeeFIM) for Chinese children: Hong Kong Cohort.

BACKGROUND: The Functional Independence Measure (WeeFIM) for children is a simple-to-administer scale for assessing independence across 3 domains in American children. WeeFIM was based on a conceptual framework by the World Health Organization (1980) of pathology, impairment, disability and handicap, and the "burden of care." WeeFIM is useful in assessing functional independence in children aged 6 months to 7 years. It can be used for children with developmental disabilities aged 6 months to 21 years. Normative WeeFIM data had been validated for American children. Because of cultural and environmental differences among countries, normative data for the Chinese population are needed. With a normative database, the progression of independence at home and in the community can be evaluated. WeeFIM is an 18-item, 7-level ordinal scale instrument that measures a child's consistent performance in essential daily functional skills. Three main domains (self-care, mobility, and cognition) are assessed by interviewing or by observing a child's performance of a task to criterion standards. WeeFIM is categorized into 2 main functional streams: "Dependent" (ie, requires helper: scores 1-5) and "Independent" (ie, requires no helper: scores 6-7). Scores 1 (total assistance) and 2 (maximal assistance) belonged to the "Complete Dependence" category. Scores 3 (moderate assistance), 4 (minimal contact assistance), and 5 (supervision or set-up) belonged to the "Modified Dependence" category. Scores 6 (modified independence) and 7 (complete independence) belonged to the "Independent" category. The WeeFIM is a 7-level criterion-specific ordinal scale. Level 7 requires no assistance for the child and the child completes the task independently without requiring a device. During the task, there is no concern about safety or taking an inordinate amount of time. Level 6 reflects modified independence and includes use of an assistive device or not completing the task in a timely or safe manner. OBJECTIVE: To examine the utility of the WeeFIM in Chinese children and to create a normative WeeFIM profile suitable for Chinese children. METHOD: Direct interviews were conducted for 445 normal Chinese children, aged 6 months to 7 years, in the community. RESULTS: 1. WeeFIM total score and 3 domains subscores versus age. The total WeeFIM scores increased progressively with age, reaching a plateau at 72 months. There was a progressive increase in subscores of self-care, mobility, and cognition independence between 6 to 62 months, especially between 6 to 45 months. Similarly, the WeeFIM self-care subscores increased progressively with age, reaching a plateau at around 72 months. The WeeFIM mobility subscores increased progressively with age, reaching a plateau at around 54 months. The WeeFIM cognition subscores increased progressively with age, reaching a later plateau at around 80 months. 2. WeeFIM subtotal scores versus age. The total WeeFIM score and 3 domain subscores correlated significantly with age. We further classified the 18 items into 3 groups according to the degree of correlation with age. Most items had high correlation with Spearman's correlation coefficient of rho >0.8. Only 1 item (chair transfer) showed moderate correlation with rho = 0.7-0.8. The item "walk" had the lowest correlation with rho = 0.6-0.7. 3. Chronological order for achieving different items. The 50th percentile of age in months for achieving level 6 (modified independence) of the 18 items were compared and ranked according to the age of achieving level 6. In creating a developmental scale of achievement of level 6 (modified independence) for all 18 items, the developmental sequence clustered in the following order: chair transfer (order 1) and walk (order 2) were achieved much earlier (at 18 and 24 months, respectively). Thereafter, there was a clustering of 4 items achieved at around 45 months: toilet transfer (order 3), stair (order 4), expression (order 5), and social interaction (order 6). Then, 9 items were achieved at around 54 to 56 months: tub or shower transfer (order 7), eating (order 8), bathing (order 9), bowel management (order 10), dressing of lower body (order 11), comprehension (order 12), dressing of upper body (order 13), bladder management (order 14), and grooming (order 15). At 60 months, the following ranked in order: memory (order 16), problem-solving (order 17), and toileting (order 18). 4. Impact of sex factor. Girls aged 22 to 45 months had higher scores in self-care subscores and cognition subscores. From 63 months onwards, boys had statistically significant higher scores in mobility subscores. Otherwise, there was no statistically significant difference in WeeFIM scores for different age groups. 5. Impact of domestic helpers. There was also significant difference for self-care subscore with the presence of a maid at home. Those children with a maid at home obtained lower self-care subscores. However, the mobility and cognition subscores were not affected. CONCLUSION: We have created a normative functional independence profile for Chinese children by adapting the American-based WeeFIM. There were cultural differences when compared with American children. Interestingly, Chinese children in Hong Kong scored better than their American counterparts in domain 1 (self-care) in all ages. This might be attributable to early attendance in preschool settings where children are taught to tend to their needs. Even for domain 2 (mobility), the higher scores in younger Chinese children in Hong King (<3 years) might be explained by earlier attendance in preschool settings. The American children did catch up after 3 years. As for domain 3 (cognition), the local educational system emphasized reading, writing, memorizing materials, and social interaction. Thus, Chinese children in Hong Kong had better cognition scores until 42 months, when their American counterparts caught up by attending preschool. There are definitely environmental and cultural practices affecting functional independence in both ethnic groups, especially in the upper age range (>4 years) both in America and Hong Kong. Thus, a locally validated WeeFIM instrument should be adopted for Chinese children. Our study demonstrated that WeeFIM could be used as a functional independence measure for Chinese children. Hong Kong has a different cultural background compared with America; thus, usage of WeeFIM with different age criteria for achieving independence should be adopted.

Activities of Daily Living↗

Evidence-based approach of the use of Botulinum toxin type A (BTX) in cerebral palsy.

A systematic review with the Sachett model of evidence-based medicine of the use of Botulinum toxin type A (BTX) for intervention in children with Cerebral Palsy (CP) is highlighted. Currently, the evidence showed that BTX is useful for treating pes equinus due to spasticity of the gastrocnemius-soleus muscles. However, careful patient selection and goals of treatment have to be addressed. More multi-centre clinical trials with standardized protocols are needed before widespread recommendation of the use of BTX in treating spasticity in CP can be made.

Botulinum Toxins, Type A↗