A Case of Juvenile Dermatomyositis

Juvenile dermatomyositis is an uncommon autoimmune disease with classic heliotrope discoloration of eyelids, erythematous skin rash of joints and proximal muscle weakness. Quite different from adults, malignancy is rarely accompanied in juvenile dermatomyositis. However vasculitis, muscle atrophy, calcification and gastrointestinal involvement are often observed in juvenile dermatomyositis. A six year old boy was admitted with chief complaints of general weakness and skin rash. Muscle biopsy was performed which was consistent with dermatomyositis. The patient was treated with intravenous immunoglobulin, steroid, methotrexate and physiotherapy. We report a case of juvenile dermatomyositis.

Subacute Sclerosing Panencephalitis: Clinical Experience of 6 Cases

PURPOSE: Subacute sclerosing panencephalitis(SSPE) is a severe and usually fatal neurodegenerative disorder of childhood and adolescence. The etiology is related to previous measles infection especially during the first 2 years of life. Since recent measles epidemics in Korea may increase the late risk of SSPE, the authors investigated the clinical characteristics of SSPE focusing on brain MRI. METHODS: Six cases(4 males, 2 females) of SSPE patients were retrospectively reviewed for clinical, EEG, laboratory and brain MRI findings. RESULTS: Four of 6 had a history of measles infection in the first year of life. Clinical manifestations were as follows:myoclonus(6), falling(4), ataxia(4), dysarthria(3), seizures (2), involuntary movements(2), tremor(2), head drop(1), sleep disturbance(1). In all cases, CSF IgG, CSF IgG/albumin ratio, and CSF/serum IgG index increased, oligoclonal bands were positive, and CSF antimeasles antibodies were positive. Frontal high amplitude sigma activities and anteriorly-accentuated multifocal epileptiform discharges were noted on EEG. Brain MRI revealed T2-weighted high signal intensity of the deep white matter. CONCLUSION: The diagnosis of SSPE depends on characteristic clinical features and elevation of measles antibodies in CSF, supported by others including EEG, CSF and brain MRI findings. We hope the clinical characteristics we mentioned may be useful for the early diagnosis and active management of SSPE in Korea.

A Case of Neuromyelitis Opitica (Devic Disease)

Neuromyelitis optica (Devic disease) is a rare demyelinating disorder of unknown etiology in which unilateral or bilateral optic neuritis and transverse myelitis occur within 8 weeks. The disease has no clinical involvement beyond the spinal cord or optic nerves. This illness occurs more commonly in adults than in children. The prognosis is better in children than in adults. The authors experienced a case of neuromyelitis optica (Devic disease) in a 5-year-old female who developed acute visual loss of the left eye, paraplegia, sensory change above the 4th thoracic dermatome, nuchal rigidity and fever. We diagnosed this case through neurologic features, magnetic resonance imaging, ophthalmoscopy and cerebrospinal fluid findings. The patient improved with conservative treatment without sequelae.

Immunogenicity of Haemophilus influenzae PRP-D Conjugate Vaccine in Korean Infants

PURPOSE: Infection by Haemophilus influenzae Type B(Hib) occurs most often in infants and children who are 5 years old and less. The incidence is highest around 6 months of age, and then decreases thereafter as infants gain natural immunity. Hi PRP-D conjugate vaccine is poorly immunogenic in infants. However, immunogenicity PRP-D vaccine in Korean infants is unknown. This study was carried out to evaluate the immunogenicity of PRP-D in Korean infants. METHODS: Forty-two normal infants who were born in Hanil General Hospital from February 1994 to July 1995 were vaccinated at 2, 4, 6, 15 months after birth. The serum antibody level was measured at 2, 6, 7, 15, 16 months of age. The infants also received DTaP, TOPV at 2, 4, 6 months and MMR at 15 months. Anti-PRP antibody level was measured by radioimmnune assay at Dana Faber Cancer Institute in Boston, U.S.A. RESULTS: The geometric mean of Anti-PRP antibody titers were 0.074microgram/ml at 2 months, 0.185 microgram/ml at 6 months, 1.390microgram/ml at 7 months, and then fell to 0.501microgram/ml at 15 months(Mean values are geometric mean; L=512, P=0.0001). Anti-PRP antibody titers increased in proportion to the number of vaccinations. The proportion of infants with anti-PRP antibody titers> OR =0.15microgram/ml were 15.8% at 2 months, 44.7% at 6 months, and 94.7% at 7 months. The proportion of infants with anti-PRP antibody titers> OR =1.0microgram/ml were 2.6% at 2 months, 26.3% at 6 months, and 57.9% at 7 months(X2 test; total: X2=27.64, P<0.01; male: X2=23.05, P<0.01; female: X2=48.55, P<0.01). CONCLUSION: The immunogenicity of PRP-D conjugate vaccine may be more effective in young Korean infants than in other population published in studies. The clinical implication of good responsiveness of Korean infants to PRP-D vaccine needs further evaluations.

A Case of Intestinal Amebiasis with Protein Losing Enteropathy

Amebiasis is an infectious disease caused by Entameba histolytica. Amebiasis remains an extremely important consideration in the differential diagnosis of diarrhea, especially when there is associated bleeding. It is imperative that appropriate studies to establish or exclude the diagnosis of amebiasis be carried out in all patients who present with a clinical and sigmoidoscopic picture of colitis, and that patients treated with metronidazole for amebiasis have adequate clinical and parasitological follow-up. We have experienced one case of intestinal amebiasis with protein losing enteropathy in 30month-old boy whose chief complaint was mild fever, vomiting and blood tinged diarrhea. His laboratory findings were compatible with protein losing enteropathy. The diagnosis of amebiasis is confirmed by observation of trophozoite of E. histolytica in the stools. A brief review with related literatures is also presented.

A Case of anhidrotic Ectodermal Dysplasia / 대한피부과학회지

Anhidrotic ectodermal dysplasia is inherited as an X linked recessive trait. This disor der is characterized by hypotrichosis, hypodontia and hypohidrosis. The diagnosis is often delayed until the first or second year of life, after repeated episodes of potentially damaging high fever. In the newborn period, the diagnosis is more difficult, but early diagnosis is of importance in ensuring that the appropriate enivironment and medical measures be taken to avoid uncontrolled hyperthermia. We have experienced a case of anhidrotic ectoclermal dysplasia in an8-day-old male patient who showed charecteristic features including hypotrichosis, peeling or scaling of the skin, recurrent fever and a characteristic face. A skin biopsy from the right palm revealed no sweat gland strutures. A brief rview with related literature is also presented.

Effects of Nebulized Budesonide in the Treatment of Patients with Mild to Moderate Croup

PURPOSE: Dexamethasone intramuscular injection and oral prednisolone have been known to be effective in the treatment of croup. The aim of this study was to determine whether nebulized budesonide leads to clinically important improvement in respiratory symptoms within four hours for child with mild to moderate croup. METHODS: Patients with croup visited to the department of Pediatrics, Han Il General Hospital from March 1995 to June 1996 were enrolled in this study. Patients were eligible if their age was between six months and six years, their total croup score was higher than 2 after breathing humidified air for at least 15 minutes. 28 patients were treated with 2mg (8ml) of nebulized budesonide and 8 patients with 8ml of normal saline as control group over the 30 minutes. Croup score, heart rate and respiratory rate were then assessed hourly for up to four hours. Side effects were also observed. RESULTS: 1) The croup score was improved from 3.6+/-0.8 to 1.9+/-0.9 at 4 hours after the nebulized budesonide treatment. 2) No significant differences in changes in heart rate were observed until 4 hours after both the nebulized budesonide group and control group. 3) Significant decrease in respiratory rate was observed at 4 hours after the nebulized budesonide group. 4) No specific side effects were noted during and 4 hours after the nebulized budesonide treatment. CONCLUSIONS: We concluded that nebulized budesonide leads to a prompt and important clinical improvement in children with mild to moderate croup without any specific side effects.

A Case of 17alpha-Hydroxylase Deficiency

A 12-year-old girl with hypertension, hypokalemia, cystic ovaries and absence of secondary sexual development is presented. Hormonal study revealed very low levels of cortisol, testosterone, estrogen, and high levels of progesterone, deoxycorticosterone, corticosterone, FSH and ACTH. Following treatment with dexamethasone and estrogen, the levels of the latter group remarkably decreased. Serum potassium level and blood pressure also became normal. With all the above hormonal profile and clinical findings, we reached the diagnosis of 17alpha-hydroxylase deficiency and report this case with the review of literature.

Clinical Observation of Neonatal Group B B-Hemolytic Streptococcal Meningitis

Neonatal meningitis caused by the Group B beta-hemolytic streptococcus was first reported in 1958. In recent years this organism has assumed major importance in the etiology of neonatal infection. In Korea, this organism is likely to play an increasing role in neonatal infection. So this investigation was designed to report the 9 cases of Group Bbeta-hemolytic streptococcus infected neonates admitted to the NICU, department of Pediatrics, Hanil Hospital from January 1990 to June 1993. The results were as followings. 1) Onset of disease was between 12days and 32 days of age and male to female ratio is 1:2. 2) Two cases were delivered by c-section, seven cases were by NFSD and all of the infants had normal birth weight with full term at delivery. Maternal obstetric and neonatal complications were not detected in all cases. 3) The common presenting symptoms were fever in all cases, seizure in 6 cases, feeding difficulty, lethargy, vomiting, etc. 4) Group Bbeta-hemolytic streptococcus was isolated in CSF of all cases and in blood of 6 cases. 5) There were abnormal brain CT findings in 4 cases of group Bbeta-hemolytic streptococcal meningitis. 6) The sensitive antibiotics were Cefotaxime, Cefazoline, Erythromycin, Ampicillin, Chloramphenicol, Clindamyucin and Penicillin. 7) All cases were reconvered except one case that expired on the 1st day of admission and 6 cases were followed up for the period of 2months to 33months without neurologic sequelae, of which 1 case revealed decreased wave on Lt. hemisphere in ABR.