Szu-Hung Chu

Specific IgE to 5 different major house dust mites among asthmatic children.

Asthma is one of the most commonly occurring manifestation of allergy in Taiwan. Sensitivity to house dust mites is closely related to childhood asthma. This study was designed to investigate sensitized rates and average concentrations of specific IgE antibodies to 5 major house dust mites (HDMs) among asthmatic children. A total of 93 asthmatic children aged from 3 to 15 years were enrolled to measure their specific IgE concentrations in response to 5 different species of mites: Dermatophagoides pteronyssinus (Dp), Dermatophagoides farinae (Df), Dermatophagoides microceras (Dm), Euroglyphus maynei (Em), and Blomia tropicalis (Bt). The severity of hypersensitivity was classified based on the concentration of specific IgE as mild (0.35-3.5 kuA/L), moderate (3.5-50 kuA/L), and severe (> 50 kuA/L). Sixty-three asthmatic children were found to have specific IgE to at least one mite. The percentage of these 63 children who had specific IgE to Dp, Df, Dm, Em and Bt were 87%, 85%, 84%, 77%, and 65%, respectively. Patients with specific IgE to Dp, Df, Dm, and Bt, had a high percentage of moderate and severe hypersensitivity (83.6%, 83.4%, 81.4%, 70.6%, respectively). However, patients sensitized to Em have relatively lower concentration of specific IgE Ab, with 75% of them in the mild range. Some patients had positive IgE antibody to Em (3.2%), and Bt (3.2%) even though they had none to Dp and Df. We conclude that Dm and Bt are also important mite allergens in atopic children. Conventional testing that assays only for sensitivity to Dp and Df would fail to demonstrate 6.4% of mite sensitized asthmatic children.

Pachydermodactyly: Three new cases in Taiwan

Pachydermodactyly (PDD), Greek for thick-skin-finger, is an infrequently recognized benign disorder characterized by painless fusiform swelling of the soft tissues around the proximal interphalangeal joints of the hands. Histopathologic features include increased dermal accumulation of collagen fibers. Young males are predominantly affected. PDD is quite rare with approximately 90 cumulative cases reported worldwide. We report three new cases of PDD in Taiwan, including two female patients. Except for patient 1 having the habit of cracking the knuckles, and patient 2 having a history of patent ductus ateriosus post catheterization, the histories of all three patients were unremarkable. X-ray of bilateral hands revealed no abnormal finding except for soft tissue swelling around proximal interphalangeal joints. Laboratory examinations all showed negative results. No local or systemic treatment was given to these patients to treat PDD, with the exception of non-steroidal anti-inflammatory drugs prescribed to one patient for a short period. Skin care with local irritation avoidance was explained to all three patients. The long-term outcome of PDD was benign.

Cystic fibrosis: Experience in one institution

Cystic fibrosis (CF) is one of the most common autosomal recessive inherited disorders among Caucasians. Comparatively, it is considered to be a rare disease among Asians. To date, only a few cases of Taiwanese CF have been published. We report four CF cases from three families. Case 1 was the first report of CF associated with a homozygosity for the CF transmembrane conductance regulator gene (CFTR gene) mutation 3849+10kb C->T in a Taiwanese patient. Cases 2 and 3 had heterozygous c. 1898+5 G->T and heterozygous p. I1023R (novel mutation) for the CFTR gene mutation. Case 4 was homozygous for the CFTR gene mutation R553X being reported in 2005 and complicated with cor pulmonale. These four patients had received 300 mg bid aerosolized tobramycin treatment every other month.

Juvenile Scleroderma in Taiwanese Children-Experience of one Institution in Taipei

Scleroderma is rare in children who are more likely to have localized cutaneous scleroderma. In this study, we retrospectively reviewed the cases of eleven children with systemic and localized scleroderma. Materials and Methods. We reviewed the records of eleven children with systemic or localized scleroderma seen from March 1993 to June 2006 in the Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan. They were diagnosed according to American College of Rheumatology criteria and clinical manifestations of hard skin involvement. Data extracted from the records included gender, age at onset, age at diagnosis, clinical manifestations, laboratory data, family history, trauma history, treatment, and outcome. Results. The mean age at diagnosis was 9.2 years (range, 3 to 12 years). The mean age at onset was 6.5 years (range, birth to 11.7 years). Two children (1 girl and 1 boy) had systemic scleroderma (both with pulmonary involvement and 1 with renal involvement) and the other 6 girls and 3 boys had localized scleroderma, either morphea or a linear pattern. Antinuclear antibodies were positive in 10 at titers of 40x to 640x; 6 had a speckled pattern, 2 had a homogenous pattern, and 2 had a speckled-to-homogenous pattern. Tests for anti-Scl-70 antibodies were all negative. Serum levels of rheumatoid factor ranged from ＜20 to 60.2 IU/ml with only 3 children having positive levels (60.2 IU/ml in 1 girl with systemic scleroderma; 45.6 and 58.2 IU/ml in 1 girl and 1 boy with localized scleroderma, respectively). All patients had skin tightening but none had subcutaneous calcification. Raynauds phenomenon with digital pitting was present only in the 2 patients with systemic scleroderma. Skin biopsy specimens from 2 boys and 1 girl showed hypertrophic collagen bundles with atrophic skin appendages and lymphocytic infiltration. All patients were treated with D-penicillamine with or without steroids and methotrexate. Only 1 boy with localized scleroderma recovered completely while the others with localized disease had a benign course with some skin softening. None of those 9 children with localized scleroderma progressed to systemic disease. Conclusion. Childhood scleroderma is more likely to be a localized cutaneous disease which, while it may not resolve, does not appear to progress to systemic disease.

Clinical Spectrum and Laboratory Characteristics of Neonatal Lupus Erythematosus in a Single Institute

Background: Neonatal lupus erythematosus (NLE) is a passive autoimmune disease in which maternal autoantibodies are transferred across the placenta. These anti-Ro/SSA and/or anti-La/SSB or anti-U1RNP antibodies can be detected in the affected infant for the first few months of life. Common clinical manifestations of NLE include cardiac disease, notably congenital heart block and cutaneous lupus lesions. The other features of NLE including hematologic abnormalities, hepatobiliary disease, and, rarely, CNS involvement are usually underestimated. We reviewed our experience with NLE and examined the relationship between the serology of the mothers and disease in their infants. Methods: We reviewed the records in 12 cases of NLE seen at Mackay Memorial Hospital from 1984 through 2003. Results: The female to male ratio was 1.4:1. Five of the 12 patients had congenital heart block, and 7 had cutaneous lesions. Other noncutaneous manifestations included thrombocytopenia in 2, anemia in 2, and elevated aminotransferases in 3, 1 of whom also had cholestasis without evidence of other metabolic, infectious or inherited causes. One infant had a right-sided focal seizure. Anti-SSA/Ro antibodies were detected in all 12 patients, a positive ANA in 10, and anti-SSB/La antibodies in 5. The last were among the 7 who had cutaneous NLE. Anti-SSB/La antibodies were not present in any of the infants with congenital heart block. Conclusions: In pregnant women with these antibodies, fetal echocardiographic screening is useful to look for atrioventricular heart block. Asymptomatic infants borne to these mothers should be followed carefully during the first 6 months of life, as babies with NLE may initially be asymptomatic. Pediatricians caring for such children should familiarize themselves with the typical features of the disease so that they can recognize it promptly if it occurs.