No abstract available.
Ectromelia*

No abstract available.
Meconium* ; Peritonitis*

No abstract available.
Oxidoreductases*

No abstract available.
Child* ; Diabetes Mellitus* ; Humans ; Insulin*

No abstract available.
Down Syndrome* ; Edema* ; Hydrops Fetalis*

No abstract available.

No abstract available.
Humans

Sjogren-Larsson Syndrome is a rare disorder chracterized by congenital icthyosis, spastic diplegia or tetraplegia and mental retardation. The inheritance is autosomal recessive, due to deficiency of alcohol dehydrogenase activity of fatty acid matabolism. We have experienced a case of Sjogren-Larsson Syndrome in a 16 month-old male children who have dried thick skin, developmental delay, and spastic diplegia. He also has glistening spot on macula area with irregular high voltage slow wave (2-3Hz) on electroencephalography. From Biopsy finding, Lamella ictyosis with acanthosis and hyperkeratosis in epithelium is shown. He was much improved clinically for skin lesion and developmental status with etretinate supplement. We report the case with brief review of related literatures.
Acitretin ; Alcohol Dehydrogenase ; Biopsy ; Cerebral Palsy ; Child ; Electroencephalography ; Epithelium ; Etretinate ; Humans ; Infant ; Intellectual Disability ; Male ; Quadriplegia ; Sjogren-Larsson Syndrome* ; Skin ; Wills

No abstract available.
Measles*

We made a clinical study on 10 cases of histiocytosis syndrome who had been admitted to the pediatric department of Soon Chun Hyang University Hospital from Jan. 1982 to Dec. 1991. The results were obtained as follows 1) The sex incidence revealed male predominance with the ratio 4:1. 2) Among 10 cases, 4 cases were classified as eosinophilic granuloma, 1 case as Letterer-Siwe disease, 1 case as linfection associated hemophagocytic syndrome and 4 cases as malignant histiocytosis. 3) The mean age of symptom onset was 4 and 7/12 years in all disease group. 4) The common clinical symptoms and signs at dignosis were dyspnea, mass, pain on lower extremities and fever. 5) The most common organ involved among 9 organ systems was liver-spleen and the number of organ systems involved were 1 in 3 2 ases(30%), 3 in 2 cases(20%), 8 in 2 cases (20%) and 6, 7 and 10 organs in each 1 case. 6) The abnormal hematologic findings (Hb; 10g/dl and/or WBC; 4,000/mm3 and/or PLT; 100,000/mm3) were found in 6 cases. 7) Low serum albumin was found in 2 cases. Results of blood culture were Pseudomonas aeruginosae and Salmonella typhi in each 1 case. 8) The common findings on tissue biopsy were histiocytic proliferation and infiltration. 9) 4 patients of MH who recieved chemotherapy, a combination of adriamycin, vincristine, cyclophosphamide and prednisone were given in a total of four courses every 2 weeks ad induction therapy. When complete response was attained, a combination of adriamycin, vincristine, prednisone (AOP) and cyclophosphamide, vincristine, prednisone(COP) was administered alternately every 4 weeks as maintenance therapy for 6-64 months. 10) Among 4 patiens of MH who recieved chemotherapy, 1 patient was lost during induction chemotherapy, for 1 day.2 patients expired during induction chemotherapy, for 1 month, 1 patient expired during maintenance chemotherapty, for 8months, Eosinophilic granuloma cases (3) were recieved currettage and no recurrence. IAHS case due to typhoid fever was improved spontaneously.
Biopsy ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Dyspnea ; Eosinophilic Granuloma ; Fever ; Histiocytic Sarcoma ; Histiocytosis* ; Histiocytosis, Langerhans-Cell ; Humans ; Incidence ; Induction Chemotherapy ; Lower Extremity ; Lymphohistiocytosis, Hemophagocytic ; Male ; Prednisone ; Pseudomonas aeruginosa ; Recurrence ; Salmonella typhi ; Serum Albumin ; Typhoid Fever ; Vincristine