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Pemphigus vulgaris is an autoimmune vesiculobullous disease of the skin and the mucous membrane. It most commonly involves the population of the fifth and sixth decades, and is extremely rare in children. We report a case of this disease in a 13-year-old boy, who was documented to have pemphigus vulgaris with historical, histopathological, and immunological criterion. He received corticosteroid and immunosuppressive drugs and a good response was achieved. We concluded that pemphigus vulgaris is a rare disease in children, but it should be kept in mind as a differential diagnosis of oral ulceration along with skin manifestation in children.

We report a 12-year-old girl with hyperimmunoglobulin E (HIE) syndrome who presented with genu vulgus of left knee, joint deformities involving both hands, and frequent fractures. She had had chronic eczema and recurrent skin and soft tissue infections since infancy, and was found to have a pneumatocele during admission. Immunologic abnormalities included extremely elevated serum IgE levels (18989 IU/ml) and cutaneous anergy to candida, purified protein derivative, and tetanus toxoid. The results of polymorphonuclear leukocyte function tests including the nitroblue tetrazolium test and chemotaxis were normal. A high index of suspicion for HIE syndrome should be given in patients with recurrent skin infections and orthopedic complaints. The physician should anticipate orthopedic problems in caring for patients with HIE syndrome, and optimal antibiotics prophylaxis should be used.

The treatment of Sjögren's syndrome (SS) is very controversial, though several therapeutic regimens have been proposed. Cyclophosphamide pulse therapy has been widely used in many disease entities. However, reports concerning its clinical application in SS were very rare. We report a 17-year-old girl presenting with lupus nephritis and SS, which was refractory to corticosteroid therapy but successfully treated with cyclophosphamide pulse therapy. The improvement of clinical features was confirmed by Schirmer's test and minor salivary gland biopsy.

A retrospective study was conducted to analyze the clinical and laboratory features, results of imaging studies, and course and treatment in 27 children with Henoch-Schönlein purpura (HSP) who initially presented with only abdominal symptoms. There were 17 boys and 10 girls, aged 6.7 +/- 0.5 years. The abdominal symptoms preceded the purpura for 10.2 +/- 1.9 days, ranging from 3 to 48 days. The main abdominal symptoms were periumbilical pain (77.8%); vomiting (51.9%); diarrhea (29.6%); pain mimicking appendicitis (22.2%) and bloody stool (14.8%). Laboratory findings revealed leukocytosis (88.9%), thrombocytosis (77.8%), positive stool guaiac tests (77.8%), and elevation of serum C-reactive protein (71.4%). Plain film is of limited use, but emergent abdominal sonography can be helpful in patients suspected of appendicitis. Unnecessary laparotomy was performed in three patients, whose pain persisted after the operation. Corticosteroid, given upon the appearance of rash, alleviated the abdominal pain in 2.4 +/- 0.2 days. All patients had recovered completely at six-month follow-up, except that three had persistent microscopic hematuria. A high index of suspicion and early diagnosis of HSP based on clinical features, laboratory data and the findings from diagnostic imaging may avoid unnecessary surgery. Early use of corticosteroid may reduce the suffering in these children.

The clinical and laboratory features of 72 children with Henoch-Schönlein purpura (HSP) were examined to determine if there were associations between the laboratory indices--including white blood cell (WBC) counts, serum C-reactive protein (CRP) levels, platelet (PLT) counts--and the clinical manifestations of acute HSP. Marked leukocytosis (WBC > 15,000/mm3), elevation of serum CRP levels (> 10 mg/L) and thrombocytosis (PLT > 400 x 10(3)/mm3) were associated with gastrointestinal (GI) bleeding, but not associated with abdominal pain in the absence of GI bleeding. None of the three parameters was associated with arthritis and nephritis. Elevated serum CRP levels alone was associated with prolonged hospital course, and may serve as an indicator of disease severity in patients with HSP. GI bleeding did not occur in patients with normal WBC counts, serum CRP levels, PLT counts (0/19), but occurred in 21.7% (5/23), 50% (8/16), and 85.7% (12/14) in patients with only one, two of the three and all three laboratory abnormalities, respectively. Patients with GI bleeding had higher mean WBC, PLT counts and serum CRP levels than those with abdominal pain in the absence of GI bleeding, and than those without abdominal pain. Routine measurement of these parameters is warranted to monitor children with acute HSP.

Osteocalcin is mainly secreted by osteoblasts, and then diffuses into blood which can be detected by several experimental methods. This study determined the osteocalcin level by solid phase fluorescent immunosorbent assay (Pharmacia CAP; Sweden), a well-established and accurate laboratory method for determining the minor concentration of substances in blood. A total of 332 healthy children were enrolled in the study, including 176 boys and 156 girls ranging in age from one to fifteen years. It was concluded that (1) quantitative osteocalcin (OCs) value varies between 30.2 to 41.0 ng/ml; (2) there is an incremental tendency in both sexes until puberty; (3) girls generally have a higher osteocalcin level than boys before mid-puberty (2-12 years old). The differences are statistically significant.

From 1982 to 1997, 12 Chinese children were admitted to Chang Gung Memorial Hospital with acetaminophen overdose. Six subjects (one young child, and five adolescents) developed liver damage which was severe in three cases (AST > 1000 IU/L). Acetaminophen-induced liver function abnormalities were characterized by elevation of transaminase levels with ALT higher than AST(6/6), coagulopathy(5/6), thrombocytopenia (1/6), but absence of jaundice(6/6). Fortunately, none of the six patients with liver damage developed fulminant liver failure, and all recovered completely. Acetaminophen overdose can cause significant morbidity in children and adolescents. Caretakers should be well instructed to give the drug correctly. So far, acetaminophen is still considered as the drug-of-choice for antipyresis in pediatric practice. However, multicentered collaborative study is necessary to determine whether acetaminophen intoxication causes less hepatic failure in Chinese children than in Western children.

Oxatomide is an orally active H1-histamine receptor antagonist. It has been demonstrated to have therapeutic efficacy in the treatment of allergic diseases in adults. The aim of this study was to evaluate the efficacy and safety of oxatomide in the treatment of asthma in children. Sixty-four asthmatic children of both sexes, aged between 5 and 16 years, were enrolled in this double-blind, placebo-controlled trial with a duration of 4 months. Patients were randomized chosen to receive either oxatomide with a daily dose of 1 to 2 mg/kg body weight or a placebo twice daily. Clinical evaluations including pulmonary function tests and immunological studies. The patients' impression on the effect of treatment also were recorded during the study. The effects of bronchodilatation and normalizing pulmonary function were observed 2 months after oxatomide treatment. The levels of eosinophil cationic protein and total asthma symptom scores were significantly reduced during treatment with oxatomide. There was no significant change in total IgE or IgG4 before or after treatment in either the treatment or control groups. During the study, two (5.8%) oxatomide treated patients reported slight drowsiness and one (2.9%) reported body weight gain. Routine laboratory tests showed no significant alterations. In conclusion, oxatomide was generally well tolerated in this study and may have the potential of being an effective treatment for childhood asthma.

Henoch-Schönlein purpura (HSP) is a systemic vasculitis of unknown cause and abdominal pain is usually complained. Although intussusception and intestinal bleeding may occur, ileal perforation is rare. We present a case of ileal perforation following HSP in which our patient received corticosteroid on the 8th day of clinical course. Attention is focused on assessing the effect of corticosteroid and the relationships among ischemic enteritis, ileal perforation and the usage of steroid.

Poststreptococcal reactive arthritis (PSRA) is a clinical syndrome of reactive arthritis. It is associated with recent streptococcal infections, but could not fulfill the revised Jones criteria for acute rheumatic fever (ARF). The incidence of PSRA cardiac complications to develop was as high as the ARF's developing into rheumatic heart disease. A 9-year-old boy presented with limping gait. He had pain in his left knee for 4 days but no fever. His right knee was swelling with a limitation of movement. A throat culture showed positive growth for group A streptococcus, and consequently antistreptolysin-O serum titer and C-reactive protein were elevated. A synovial fluid examination was turbid but sterile. After 3 days the arthralgia subsided rapidly. A cardiac color Doppler and electrocardiogram showed no evidence of valvular disease. Under the threat of high incidence of rheumatic heart disease in PSRA, we treated this patient with prophylactic antibiotics as acute rheumatic fever. A clinic follow up one year later showed neither sequels nor heart murmur on physical examination.

Sinus disease has been assumed to exist in children with respiratory tract allergies. We consecutively evaluated 375 patients (245 male, 130 female) with childhood onset asthma, between 5 and 15 years of age (mean 7.8 years of age). Abnormal Waters radiographs were found in 205 patients (54.7%) which included mucosal wall thickening greater than 6 mm (67.3%), complete opacification (22.9%) and air-fluid levels (9.8%). The level of immunoglobulin (Ig) E was found to be much higher in non-sinusitis asthmatic children than in asthmatic children with sinusitis (1207 IU/mL vs. 644 IU/mL). The IgG, IgA, IgM and erythrocyte sedimentation rate (ESR) values were not significantly different in the two groups. All patients were treated with antibiotics for 3 to 6 weeks. Five patients required surgical intervention after antibiotic treatment had little success. Streptococcus viridans was isolated from 4 patients following surgical aspirates and Peptostreptococcus anaerobius was isolated in only one patient. In conclusion, sinusitis in children may be an aggravating factor for chronic reactive lower airway disease. Optimal treatment may decrease the need of asthmatic medication.

From January 1, 1981 to December 31, 1992, we experienced nine patients with childhood onset of dermatomyositis and polymyositis. The mean age of disease onset was 12 years (range 7 to 16 years). Seven of them fulfilled the criteria of dermatomyositis, the remaining two were polymyositis. Girls were more predominant than boys in 6:3 ratio. The clinical features included extremities muscle weakness, skin rash, periorbital swelling and dysphagia. Increased muscle enzymes including creatine phosphokinase (CPK) or lactic dehydrogenase (LDH) were all positive in nine patients. All of our nine patients were treated with prednisolone after the diagnosis was established. The duration of treatment ranged from 3 to 65 months (mean: 25.3 months). Two of the nine patients also received immunosuppressive agents, hydroxychloroquine and azathioprine respectively. At present six patients survive without treatment. Two patients continue with corticosteroid and immunosuppressive therapy. One patient died from primary peritonitis, six months after being diagnosed with JDMS. In conclusion our study shows there is a female dominance; monocyclic clinical course is more common; and the prognosis is good in general, in the cases of juvenile dermatomyositis and polymyositis.

Five episodes of fungemias are described; all had occurred in children with leukemia or lymphoma between January 1, 1978 and December 31, 1990. These fungemias comprised 3.4% of the total septicemias encountered during that period. Three episodes occurred during the induction phase and two during relapse. All patients had fever of varying degree and duration. In addition to steroids, all were receiving combination antibiotics before the fungemia had occurred. All patients had severe neutropenia lasting more than one week. Bacteremia preceded fungemia in four patients. Two episodes were diagnosed antemortem. The same species were isolated from other sites in three cases. Fever, chills and gastrointestinal symptoms were the most common clinical features; other symptoms included cough, dyspnea, oliguria and azotemia. One patient experienced skin lesion, dysphagia, hoarseness and hemiparesis. Only one patient survived. The prognosis from fungemia in leukemia and lymphoma patients is very poor. Empiric antifungal therapy is indicated in neutropenic patients who have recurrent or persistent fever despite one week of broad spectrum antibiotics. Early diagnosis and treatment will aid in improving the overall poor outcome of this disease.