Acanthosis nigricans in obese Chinese children.

OBJECTIVES. To investigate the demographic characteristics and insulin resistance in local overweight/obese Chinese children with and without acanthosis nigricans, and the associations of acanthosis nigricans with insulin resistance and other cardiometabolic co-morbidities. DESIGN. Case series with cross-sectional analyses. SETTING. A regional hospital in Hong Kong. PATIENTS. Chinese children assessed between January 2006 and December 2010 at Tseung Kwan O Hospital for being overweight or obese. MAIN OUTCOME MEASURES. The demographics, anthropometric data, acanthosis nigricans status, and biochemical results were analysed. RESULTS. A total of 543 overweight/obese children were studied with 64% being boys and 29% had insulin resistance. Adolescents aged 12 to 18 years, compared with children aged 5 to 11 years, were more likely to have acanthosis nigricans (63% vs 47%; P<0.001) and insulin resistance (37% vs 25%; P=0.005). Compared with overweight children, those who were obese were more likely to have the two conditions: acanthosis nigricans (59% vs 44%; P=0.005) and insulin resistance (35% vs 19%; P=0.001). Compared with those without acanthosis nigricans, those with the condition had significantly higher mean values for systolic blood pressures (P<0.001), 2-hour post-oral glucose tolerance test glucose level (P=0.021), fasting insulin level (P<0.001), homeostasis model of assessment-insulin resistance (P<0.001), fasting triglyceride level (P<0.001), and alanine aminotransferase level (P=0.002), but a lower high-density lipoprotein cholesterol level (P<0.001). Those with acanthosis nigricans were also more likely to have insulin resistance (P<0.001), hypertension (P=0.021), fatty liver (P=0.001), and abnormal glucose homeostasis (P=0.003). CONCLUSION. Obese Chinese children and adolescents with acanthosis nigricans had a higher chance of having insulin resistance and cardiometabolic co-morbidities. Acanthosis nigricans is an important clinical feature warranting early attention and evaluation to facilitate timely interventions and monitoring.

A pale Chinese boy with recurrent painful digital swelling.

Thalassaemia is the most common haemoglobinopathy in the Chinese population. However, recurrent painful digital swelling is not a typical manifestation of this well-known hereditary condition. We describe a case of co-inheritance of beta-thalassaemia and sickle cell trait in a Chinese family and a child who suffered from sickle cell/beta-thalassaemia with recurrent dactylitis. This report highlights awareness of this rare condition in the Chinese population, since acute manifestations can be life-threatening and mimic other emergency conditions. Prompt management can prevent further complications and avoid unnecessary interventions due to delay in diagnosis. A detailed family history and examination of the patient's peripheral blood smear is crucial to reach a correct diagnosis.

Consensus statement on iodine deficiency disorders in Hong Kong.

This article reviews the available data on the study of iodine deficiency disorders in Hong Kong and to discuss the approach towards preventing such disorders in Hong Kong. The importance of iodine and iodine deficiency disorders is described, and the available data on the dietary iodine intake and urinary iodine concentration in different populations of Hong Kong are summarised and discussed. Dietary iodine insufficiency among pregnant women in Hong Kong is associated with maternal goitrogenesis and hypothyroxinaemia as well as neonatal hypothyroidism. Borderline iodine deficiency exists in the expectant mothers in Hong Kong. Women of reproductive age, and pregnant and lactating women should be made aware and educated to have an adequate iodine intake, such as iodised salt, as an interim measure. A steering group involving all stakeholders should be formed to advise on the strategy of ensuring adequate iodine intake, including universal iodisation of salt in Hong Kong. Continuous surveillance of iodine status in the Hong Kong population is necessary.

Gaucher disease with pulmonary involvement in a 6-year-old girl: report of resolution of radiographic abnormalities on increasing dose of imiglucerase.

We report resolution of ground-glass appearance in high-resolution computed tomography of chest in a 6-year-old girl who had Gaucher disease with pulmonary involvement. This radiographic abnormality, which developed during the course of enzyme replacement therapy at doses between 20 to 60 U/kg/2 weeks, resolved when the dose was increased to 100 U/kg/2 weeks. This case illustrates the importance of trial of escalating dosage in the face of failure of response at lower doses.

Pathways from emotional adjustment to glycemic control in youths with diabetes in Hong Kong.

OBJECTIVE: To examine factors that influence emotional adjustment, adherence to diabetic care, and glycemic control in Hong Kong youths with insulin-dependent diabetes mellitus (IDDM). METHODS: Seventy youths, their mothers, and matched controls provided information on health beliefs, authoritarian parenting style, parent-child conflict, emotional adjustment, and adherence to medical regimen. Glycosylated hemoglobin levels were obtained to measure glycemic control. RESULTS: Predictors explained 34% of the variance in emotional adjustment and 39% of the variance in glycemic control. The data supported a pathway from emotional adjustment to self-efficacy to adherence behaviors to glycemic control. In contrast to Western culture and consistent with prediction, parenting style did not associate with negative outcomes, and even relatively low levels of parent-child conflict correlated negatively with emotional adjustment in this culture. CONCLUSIONS: Management of conflict and self-efficacy enhancing interactions are suggested interventions to enhance adherence to diabetic care in Hong Kong youths with IDDM.

Umbilical cord blood transplantation for Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI).

Severe Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) is usually fatal by early adulthood. Bone marrow transplantation is the only form of definitive enzyme replacement therapy available. A 5-year-old boy with Maroteaux-Lamy syndrome has successful recovery of bone marrow and enzymatic functions after umbilical cord blood transplant from his unaffected HLA-identical brother. Busulphan (16 mg/kg) and cyclophosphamide (200 mg/kg) were used as preparative chemotherapy with short methotrexate and long cyclosporin as prophylaxis against graft-versus-host disease (GVHD). A total of 6.08 x 10(7)/kg nucleated cells and 2.92 x 10(5)/kg CD34+ cells were transplanted with neutrophil engraftment achieved on day 26. There was no evidence of acute and chronic GVHD. Fifteen months after transplant, a normal level of N-acetylgalactosamine-4-sulphatase activity was achieved despite mixed chimerism. There was clinical improvement of hepatosplenomegaly, facial and skin features, joint mobility and resolution of suppurative middle ear effusion. He returned to school and continued to perform well in academic studies. We report here the first successful umbilical cord blood transplant as treatment of Maroteaux-Lamy syndrome.

Use of oral valaciclovir in a 12-year-old boy with herpes simplex encephalitis.

We report on a 12-year-old boy with herpes simplex encephalitis, in whom a severe localised skin reaction developed following the infusion of intravenous acyclovir. Oral valaciclovir was given as continuation therapy to complete the 3-week course of antiviral treatment and resulted in complete recovery without side effects. This report illustrates the advantage of using the polymerase chain reaction to diagnose herpes simplex encephalitis and the potential use of newer antiviral agents, such as valaciclovir, as continuation therapy in the management of the infection. The higher oral bioavailability of newer antiviral agents allows part of the extended treatment period of patients with herpes simplex encephalitis to be carried out as an ambulatory oral regimen.

Epidemiology of diabetes mellitus in children in Hong Kong: the Hong Kong childhood diabetes register.

OBJECTIVES: To establish a registry for Chinese children with onset of type 1 (insulin dependent) diabetes mellitus before 15 years of age and to determine the incidence of childhood onset type 1 diabetes mellitus in Chinese children in Hong Kong. RESEARCH DESIGN AND METHODS: A registry was established in 1997 to collect childhood diabetes cases retrospectively from all districts in Hong Kong. The study included all newly diagnosed cases of diabetes with onset < 15 yr of age from 1st January 1984 to 31 December 1996. Primary ascertainment was based on review of medical records at all regional public hospitals in Hong Kong and survey of all the registered practitioners in Hong Kong. The secondary source of validation was made impractical, if not impossible, because of the recent implementation of the Personal Data Privacy Ordinance in Hong Kong. RESULTS: A total of 255 diabetic cases were identified, 227 type 1 diabetes mellitus (218 were Chinese), 18 type 2 diabetes mellitus and 11 secondary diabetes. 246 patients were Chinese and 9 non-Chinese. The age-standardized incidence of type 1 and type 2 diabetes mellitus in southern Chinese children in Hong Kong was 1.4/100,000/yr and 0.1/100,000/yr respectively for children < 15 yr of age during the study period. The incidence rates for type 1 diabetes were 0.9, 1.5 and 1.7 per 100,000/yr for 0-4 years, 5 to 9 years and 10 to 14 years age-groups respectively. The incidence for males was 1.2/100,000/yr and for females 1.7/100,000/yr. A significant increase in the incidence was demonstrated during the study period by simple linear regression (slope 0.14/100,000/year, r2 = 0.73, p = 0.0002) CONCLUSIONS: A diabetic registry is established in Hong Kong. This study documents a very low incidence rate of childhood type 1 diabetes mellitus in southern Chinese children in Hong Kong and we have seen an increasing incidence of the disease in the past 13 years.

Secular trend in the sexual maturation of southern Chinese girls.

In 1993, a cross-sectional study of sexual maturation of normal Chinese schoolgirls was performed in Hong Kong. The aim of the study was to obtain an up-to-date reference for normal pubertal development in Chinese girls. Breast development was assessed in 3749 girls aged 7-19 y, and pubic hair rating was assessed in 3745 girls. Menstrual status was recorded in 6467 girls over 6 y of age. The median age of onset of puberty as indicated by breast stage II or above was 9.78 (95% CI 9.70-9.85) y. The median age of onset of pubic hair development was 11.64 (95% CI 11.56-11.72) y. The median age of menarche was 12.38 (95% CI 11.98-12.78) years. Percentile values for the age at which each puberty staging appeared were constructed and incorporated into the height-for-age charts. When comparison is made with similar studies done in 1962 and 1979, a significant downward secular trend in sexual maturation is observed (p < 0.01). Except for breast development the downward secular trend in sexual maturation appears to be diminishing and may be coming to a halt in the Chinese girls in Hong Kong. Their median ages of sexual maturation are now among one of the earliest medians recorded in the world population studied.

Secular changes in standing height, sitting height and sexual maturation of Chinese--the Hong Kong Growth Study, 1993.

In 1993 a territory-wide cross-sectional growth survey on 25,000 Chinese children from birth to 18 years was performed in Hong Kong. Compared to the last growth survey in 1963, definite secular changes were observed. There was an increase of final adult standing height of 3.6 cm in boys and 2.7 cm in girls, in which 1.8 cm and 0.5 cm respectively for boys and girls was accounted for by the sitting height. Thus most of the height increase had occurred in the leg length in girls, but in boys only half of it. The height difference was more marked during the pubertal years because secular change had brought about an earlier sexual maturation, including an advancement of median menarcheal age by 0.5 year, coupled with an earlier growth spurt. This paper also provides the first growth standards for Chinese from birth to 18 years, with percentile charts on both standing height and sexual maturation in boys and girls.

The effect of cyproterone acetate on the growth of children with central precocious puberty.

We have examined the growth and skeletal maturation of 19 children (6 male, 13 female) with central precocious puberty. The aetiology in nine patients (5 male, 4 female) was secondary to a hypothalamic hamartoma. Six children (2 male, 4 female) received no treatment whereas 13 children (4 male, 9 female) were treated with cyproterone acetate in a mean dose of 68 mg/m2 per day (range, 34-260) for a mean duration of 4.5 years (range, 0.8-7.9). There was no significant difference between height SDS for bone age at the beginning and end of observation in either treated or untreated groups. No significant relationship between the mean dose of cyproterone acetate used and change in height SDS for bone age could be determined. We conclude that cyproterone acetate has no beneficial effect on the growth prognosis of children with central precocious puberty.