Pseudohypoparathyroidism(PHP) is an inherited metabolic disorder characterized by hypocalcemia, hyperphosphatemia and an impaired phosphaturic response to exogenous parathormone(PTH), which are caused by end organ resistance to the action of PTH. Most of these patients have, in addition, the skeletal abnormalities of Albright hereditary osteodystrophy. We report two cases of PHP, suspicious type Ia, in sibling who were presented with multiple subcutaneous soft tissue calcification similar to that seen in tumoral calcinosis and had short stature, round face, brachydactyly and metabolic abnormalities(hypocalcemia, hyperphosphatemia, increased serum PTH, and decreased 24hr urinary basal cAMP)
Brachydactyly ; Calcinosis ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Pseudohypoparathyroidism* ; Siblings*

No abstract available.

PURPOSE: Chronic urticaria is a disorder characterized by the appearance of wheals for more than 6 weeks; in most cases, the etiology is unknown. This study was aimed to discover the clinical aspects, the etiologic factors, and the course of chronic urticaria. METHODS: 51 children who were diagnosed with chronic urticaria in the past 4 years, and who had had follow-ups more than 6 months after diagnosis in the pediatric department of Soonchunhyang University Hospital in Bucheon, were enrolled in the study. The laboratory findings, clinical aspects, and courses were retrospectively investigated by medical record review and telephone interview. RESULTS: The median age of children with chronic urticaria was 4 years (8 months to 16 years) and the ratio of male to female was 1.4:1. Of the total, 39.2% of patients had a history of atopy. Angioedema occurred concurrently with urticaria in 11.8% of patients, and dermographism was seen in 41.2%. Results of thyroid function tests were normal and thyroid autoantibodies were absent in all cases. Regarding etiology, most cases (74.5%) were forms of idiopathic urticaria. Urticaria was induced by physical factors in 19.6% of patients. Open challenge tests revealed that 3 patients were allergic to food additives (glutamate 2, glutamate, and sulfite 1). In this study, most of the patients reported good response after medication of 1st- or 2nd-generation antihistamines alone. Follow-up at 6 months revealed that 70.6% of patients had experienced remission, and 84.8% of children who had follow-up at 1 year presented remission. CONCLUSION: Chronic urticaria in most patients was idiopathic. Remission occurred within 1 year of diagnosis, in most cases so chronic urticaria in children seems to have good prognosis.
Angioedema ; Autoantibodies ; Child ; Female ; Follow-Up Studies ; Food Additives ; Glutamic Acid ; Histamine Antagonists ; Humans ; Interviews as Topic ; Male ; Medical Records ; Prognosis ; Retrospective Studies ; Thyroid Function Tests ; Thyroid Gland ; Urticaria

Fifteen cases of genital warts were examined for the presence of human papillomavirus (HPV) using polymerise chain reaction (PCR). HPV6/11 DNA were found in all cases of genital warts. The PCR based methods described here provide a sensitive, accurate means of detecting genital HPVs.
Condylomata Acuminata ; DNA ; Humans* ; Papillomaviridae* ; Polymerase Chain Reaction*

We present a case of perforating granuloma annulare which appeared as multiple small papular lesions with central umbilication on the dorsum of both foot and sides of foot, The patient was a 12 year-old female child who had slightly tender lesions of one month's duration. Histologic study showed perforation of the epiderrnis and release of the abnormal necrobiotic material transepiderrnally. The epidermal perforation communicated with area of granuloma annulare in the dermis that consist of necrobiotic collagen surrounded by palisading mononucler phagocytes in the dermis. All the lesions resolved spontaneously with a slight hyperpigmentation in 4 months. She also had erythematous tender nodules, which diagnosed as erythema nodosum, on both shins. The lesions responded to potassium iodide 1.0g for 2 weeks.
Child ; Collagen ; Dermis ; Erythema Nodosum ; Female ; Foot ; Granuloma Annulare* ; Granuloma* ; Humans ; Hyperpigmentation ; Phagocytes ; Potassium Iodide

The task for chromosome karyotyping and diagnosis is requiring repetitive, time consuming job and high cost even it is done by well-experienced cytogenetists. Therefore an web based chromosome karyotyping instruction system has been established to be able to analyze chromosomes and obtain necessary advises from the database instead of human experts and the database is including 2 divisions with database and agent.For the first of all, database model was constructed with relational database consisting of Patient_DB, image_DB, Disease_DB and Manage_DB. As the second procedure, knowledge base by IF THEN production rule was implemented to a knowledge domain with normal and abnormal chromosomes. For the last, independent agent with the inference by knowledge base could enter the inference data into the database.Experimental data were composed of normal chromosomes of 2,736 patients' cases and abnormal chromosomes of 259 patients' cases that have been obtained from GTG-banding metaphase peripheral blood and amniotic fluid samples.The completed system provides variously morphological information by analysis of normal or abnormal chromosomes and it also makes users enable to control and search the information in a short period with learning of high amount of knowledge.
Amniotic Fluid ; Diagnosis ; Female ; Humans ; Karyotyping* ; Knowledge Bases ; Learning ; Metaphase

11,609 of childhood new outpatients seen in Department of Dermatology of Seoul National University Hospital from 1975 to 1984 were analyzed statistica,lly. The study results were summarized as follows: l. 11, 609 out of 48, 188 new outpatients were under 15 years old age, amounting to 24. l %. 2. The 10 most common dermatoses were atopic dermatitis, vitiligo, urticari, hemangiorna, contact dermatitis, fungal disease, molluscum contagiosum, scabies, impetigo and nevocellular neuvs. The most freguent age groups were 2 to 6 years amounting to 47 2p, af total outpatients. 4. The peak incidence occurred in August, while the lowest incidence occurred in November and October. 5. Dermatoses which showed increasing tendency were nevocellular nevus and vitiligo, those of decreasing tendency were contact dermatitis and impetigo,
Adolescent ; Dermatitis, Atopic ; Dermatitis, Contact ; Dermatology ; Humans ; Impetigo ; Incidence ; Molluscum Contagiosum ; Nevus ; Outpatients ; Scabies ; Seoul ; Skin Diseases* ; Statistics as Topic* ; Vitiligo

Ultrasonographic and radiographic findings in 72 cases of pelvic masses which have been provedhistopathologically were analyzed. The results were as follows; 1. Among 72 cases, 28 cases were uterine massesand 44 cases were adnexal masses. 2. Pathologic accuracies of ultrasonographic diagnosis were 64.3% in uterinemasses, 70.5% in adnexal masses, and 68.1% in all pelvic masses. 3. Plain or IVP findings were not specific indiagnosis and showed secondary mass effects or functional disturbances. 4. Because of its high accuracy ofpathologic diagnosis and safety, ultrasonography can be regared as the most valuable and preferential study. Butin cases of cystic myomas, endometrioses, cystic missed abortions and huge masses, the accurate diagnoses weredifficult.
Abortion, Missed ; Diagnosis ; Endometriosis ; Female ; Myoma ; Pregnancy ; Ultrasonography

BACKGROUND: Abnormalities of the peripheral blood are frequent and varied in patients with miliary tuberculosis. Anemia,leukopenia, thrombocytopenia, pancytopenia, monocytosis, basophilia, eosinophilia and leukemoid reactions have been reported. These abnormalities are more frequent in patients with positive bone marrow study. In this report, we evaluated clinical, hematological and immunological features in patients with miliary tuberculosis in order to know whether difference is existed between "bone marrow biopsy positive group(pathologically proven to miliary tuberculosis)" and "negative group". METHOD: Clinical evaluation, serum ADA, sIL-2R, and T-lymphocyte subsets were measured in 40 patients with miliary tuberculosis who received bone marrow biopsy. RESULTS: 1) The average age of patients was 39 year-old. There were 23 male and 17 female patients. Associated extrapulmonary tuberculosis are 9 CNS tuberculosis, 6 joint tuberculosis, and 2 tuberculous pleurisy. 2) Sixteen of the 40 patients were positive bone marrow biopsy(60%). 3) Sixteen of the 40 patients(60%) had anemia(ll positive patients: 13 negative patients). Leukopenia occurred in 12 per cent(4:l). Thrombocytopenia was noted in 10%(3:1). 4) The mean value of serum ADA was 83 U/L(90 U/L: 70.6 U/L, p=0.23). 5) The mean activity of Soluble IL-2 receptor was 4,643 pmol/L(6840+/-7446 pmol/L: 1,897 +/- 1,663 pmol/L, p=0.06). 6) In the T lymphocyte subsets, the percent of T-lymphocytes was 64%(62%:73%, p=0.2). In some patients(9), T4 and T8 ratio in BAL fluid(1.97+/-1.2) was higher than that in the peripheral blood(1.16+/-0.5). CONCLUSION: Bone marrow examination are diagnostic in 60% of cases of miliary tuberculosis. Percents of the total T lymphocyte and helper T cell in BAL are more elevated than in peripheral blood. There was no significant difference in peripheral blood abnormalities and marker of T lymphocyte activation between the bone marrow biopsy positive and negative group.
Biopsy ; Bone Marrow Examination ; Bone Marrow* ; Eosinophilia ; Female ; Humans ; Leukemoid Reaction ; Leukopenia ; Lymphocyte Activation ; Lymphocytes ; Male ; Pancytopenia ; Receptors, Interleukin-2 ; T-Lymphocyte Subsets ; T-Lymphocytes ; Thrombocytopenia ; Tuberculosis ; Tuberculosis, Miliary* ; Tuberculosis, Osteoarticular ; Tuberculosis, Pleural

BACKGROUND: The changes of the composition in the T-lymphocyte are important as an immunological abnormality in the pathogenesis of tuberculosis. Previously, the second type of TCR dimer(gamma delta T lymphocyte) that did not express CD4 or CD8 molecules was found. In other reports the presence of this type of lymphocytes was increased in the initial stage of tuberculous infections. METHOD: To determine whether there are some differences in the T-lymphocyte subsets in the peripheral blood or pleural effusion among the forty-nine patients were examined T-lymphocyte subset analysis(CD4+T-cell,CD8+ T-cell,gamma delta T-lymphocytes) with anti-Leu4, anti-Leu3a, anti-Leu2a, anti HLA-DR and anti-TCR-gammadelta-1(Becton & Dickinson Co.). RESULTS: The average age of the patients was 50 years old(17-81year). There were 33 males and 16 female patients. patiensts with tuberculosis are 30cases(tuberculous pleurisy 15), lung cancer 12cases(malignant effusion 9) and pneumonia 7cases(parapneumonic effusion 6cases) In T lymphocyte subsets of pleural effusion, helper T lymphocyte(54.6 + 13.8 %) of tuberculous pleurisy was higher than that(36.2 + 25.3 %) of non-tuberculous pleurisy(p= 0.04). The peripheral blood gammadelta T-lymphocytes in tuberculousis was insignificantly higher than non-tuberculous patients(p= 0.24). The peripheral blood gammadelta T-lymphocytes and pleural gammadelta T-lymphocytes in tuberculous pleurisy was insignificantly higher than in non-tuberculous pleurisy(p= 0.16, p= 0.12). CONCLUSION: The percentage of gammadelta T lymphocytes among the total T-lymphocytes is not significantly increased in the peripheral blood or pleural effusion of the pleural tuberculosis. gammadelta T lymphocytes is less useful as a diagnostic method of pleural tuberculosis.
Female ; HLA-DR Antigens ; Humans ; Lung Neoplasms ; Lymphocytes ; Male ; Pleural Effusion ; Pleurisy ; Pneumonia ; T-Lymphocyte Subsets ; T-Lymphocytes* ; Tuberculosis ; Tuberculosis, Pleural*