Juvenile dermatomyositis in Thai children.

Juvenile dermatomyositis is a rare, chronic multisystemic inflammatory disorder of unknown etiology, characterized by a typical skin rash and proximal muscle weakness. A retrospective study from the medical records of patients diagnosed as juvenile dermatomyositis was performed at Queen Sirikit National Institute of Child Health from 1988 to 1998. There were seven cases of juvenile dermatomyositis diagnosed according to the criteria of Bohan and Peter. Six cases were female and one case was male. The age of diagnosis ranged from 2.5 years to 11 years. (mean age was 7 +/- 3.6 years). The presenting symptoms were muscle weakness (6 cases), muscle pain (2 cases) and skin rashes (4 cases). All of the patients developed proximal muscle weakness of the lower extremities varying from grade 3 to grade 4. The cutaneous manifestations were heliotrope signs (6 cases), gottron's papules (2 cases), photosensitivity (2 cases) and calcinosis cutis (4 cases). Electromyography (EMG) was performed in 6 cases and revealed typical change of myopathic type. Elevated muscle enzymes were noted in all cases. Muscle biopsy was performed in 6 cases and was compatible with myositis. Oral prednisolone (1-2 mg/kg/day) was given in 6 cases and the muscle weakness improved. There was no mortality in this study. Four cases developed calcinosis cutis 1 to 3 years after muscle weakness and did not respond to any treatment. In conclusion, juvenile dermatomyositis is a disease which causes chronic disability in children. Early diagnosis and treatment can prevent morbidity and mortality. Calcification at the skin usually occurs after the onset of muscle weakness several months to years after diagnosis.

Neonatal varicella: a report of 26 cases.

Varicella infection usually occurs in childhood and is uncommon in neonates. We reported 26 cases of neonatal varicella seen at the Queen Sirikit National Institute of Child Health, Bangkok, from 1988 to 1995. The sex ratio of male to female was equal. The age of onset was between 6 to 27 days. Twelve cases contracted varicella from mothers who were infected between 6 days before delivery to 2 days after delivery (perinatal varicella) and fourteen cases contracted varicella from mothers or siblings in the postnatal period (postnatal varicella). All babies developed vesicular rash. Intravenous acyclovir was given in high risk and severe cases (nine perinatal and three postnatal varicella patients). Complications of neonatal varicella included clinical sepsis 8 cases (30%), pneumonia 7 cases (26%), pyoderma 9 cases (35%) and hepatitis 1 case (4%). There was no statistical difference between the complications of perinatal and postnatal group (p > 0.05). No death was observed during this study. Clinical manifestations of neonatal varicella varied from mild to severe, depending on the onset of rash in the mother and baby and mode of transmission of the disease. Although we have no varicella-zoster immunoglobulin (VZIG), acyclovir therapy is beneficial in the treatment of neonatal varicella.

Harlequin baby: a case report.

Harlequin fetus is a rare and the most severe form of congenital ichthyosis. Most of the infants die within a few weeks after birth due to sepsis and respiratory difficulties. The case of a female harlequin baby is reported. The baby survived because of good neonatal intensive care, topical emollients and oral etretinate. Now she is over three years old and the skin developed into congenital non-bullous ichthyosiform erythroderma. Unfortunately she had delayed growth and development. This is the first case report of a harlequin fetus in Thailand that had prolonged survival.

Epidermolysis bullosa letalis (Herlitz disease): a case report.

A case of junctional epidermolysis bullosa was reported. A four-month-old boy was presented with generalised blisters after minor trauma since birth. There was no family history of blistering diseases or consanguinity. Skin examination revealed blisters primarily on the upper and lower extremities. Erosions were also noted on the face, abdomen and buttocks. The lesions healed without milia or scarring formation. Anonychia of all fingers and toe nails were noted. Skin biopsy from the blisters showed subepidermal bulla with a few inflammatory cells. Electron microscopic examination revealed cleavage plane at the lamina lucida and the absence of hemidesmosomes. These findings supported a diagnosis of junctional epidermolysis bullosa. The patient was treated with topical and systemic antibiotics. However, the blisters gradually increased. He died of uncontrolled sepsis and diarrhea.

Hemogram in normal newborn babies with special reference to platelet count.

A prospective study was performed to verify the hemogram of 318 healthy fullterm newborn babies aged one hour to thirty days. The mean hemoglobin, hematocrit and reticulocyte count were between 17.6-17.9 g/dl, 52.2-53.4% and 5.7-6.7% in the first 72 hours of life. After that, they were decreased gradually approaching adult level at the later half of one month old. The white blood count at 12 to 24 hours was 18,482 +/- 6,600/microliters, gradually decreased to 9,817 +/- 2,496/microliters during 14 to 30 days. The ratio of immature to total neutrophils between 1-12 hours was 0.07 and 12 hour to 30 day was 0.04-0.05. The mean platelet counts in the first 72 hours was between (280 +/- 69) x 10(3)-(285 +/- 93) x 10(3)/microliters, it increased thereafter until 30 days old with a peak of 402 x 10(3)/microliters during 7-14 days. The platelet counts below 150 x 10(3)/microliters in the newborn period should be considered as thrombocytopenia.