PURPOSE:Varying clinical phenotypes are associated with the chromosome 22q11.2 microdeletion syndrome. The endocrine manifestation are latent or overt hypoparathyroidism, thyroid dysfunction and short stature. This study was undertaken to investigate frequencies of endocrine abnormalities and short stature in patients with the chromosome 22q11.2 microdeletion syndrome. METHODS:Forty three unrelated patients were diagnosed having chromosome 22q11.2 microdeletion syndrome. Chromosomal microdeletion was confirmed by fluorescent in situ hybridation (FISH) with DNA probe (22q11.2 LSI TUPLE1 from Vysis). Serum total calcium and intact parathyroid hormone (PTH) were measured in all patients. Thyroid function tests including free thyroxine(T4), thyroid stimulating hormone (TSH) and thyroid autoantibodies were performed in all patients. Insulin-like growth factor-1 (IGF-1) was measured in 10 patients. Height, weight and body mass index were compared with chronological age in all patients. RESULTS:Seven patients (16%) had an overt hypoparathyroidism, presenting with hypocalcemic tetany. Thirteen patients (31%) showing hypocalcemia with normal PTH were regarded as having latent hypoparathyroidism since their PTH secretion response was blunted. Out of 2 patients with thyroid diseases, one patient had Graves disease and the other had Hashimoto thyroiditis. Five patients (12%) were below the 3rd percentile in height at evaluation. The BMI was below the 5th percentile in 23% of patients. CONCLUSION: Twenty patients (47%) presented with overt and latent hypoparathyroidism. Interestingly, autoimmune thyroid diseases such as Graves disease and Hashimoto thyroiditis were associated in patients with chromosome 22q11.2 microdeletion, indicating predisposition to autoimmune disorders. Therefore, a careful endocrine and growth evaluation is needed in these patients.
Autoantibodies ; Body Mass Index ; Calcium ; DNA ; Graves Disease ; Hashimoto Disease ; Humans ; Hypocalcemia ; Hypoparathyroidism ; Parathyroid Hormone ; Phenotype ; Tetany ; Thyroid Diseases ; Thyroid Function Tests ; Thyroid Gland ; Thyrotropin

BACKGROUND: According to the 1992 version of ACLS guideline, adenosine is recommended as the first line drug far the treatment of paroxysmal supraventricular tachycardia(PSYT). But adenosine is not used frequently in our country, despite currency proven effect and safety. Therefore we tried to compare the efficacy and safety of adenosine with verapamil for the treatment far PSVT. MATERIALS AND METHODS: We prospectively reviewed charles of PSVT patients admitted to YongDong Severance Hospital from Jan. 1995 to Dec. 1996. These patients were randomly divided into two groups. The flat group was given 6mg of adenosine initially, and another 12mg was given within 5 minutes if fast dose failed. The other group was given 5mg of verapamil initially, and if failed, 10mg was given within 10 minutes. The results were analyzed by Chi-square test and student-t method. RESULTS: In converting PSVT to normal rhythm, adenosine and verapamil showed similar results and there was no difference between the two Groups in frequency of side elects, but serious arrhythmia such as ventricular fibrillation and ventricular tachycardia was not seen in the adenosine group. CONCLUSION: Adenosine not only showed comparable effect and safety, but also had shorter action time than verapamil. Therefore we recommand adenosine, as a safe and effective fort line drug for PSVT.
Adenosine* ; Arrhythmias, Cardiac ; Humans ; Prospective Studies ; Tachycardia, Supraventricular* ; Tachycardia, Ventricular ; Ventricular Fibrillation ; Verapamil*

OBJECTIVE: We analyzed the clinical features of amniotic fluid embolism confirmed by autopsy or clinical dicision for making a plan of rapid diagnosis and intensive emergency care. STUDY DESIGN: We experienced 2 clincal cases in Ewha Mokdong Hospital and Severance Hospital at 1995, and reviewed the medical record & autopsy report of 15 cases in National Institute of Scientific Investigation from Jan, 1991 to Dec, 1995. RESULTS: The mean maternal age was 31+/-3 years. The mean gestational age was 40+/-1 weeks. The number of delivery were 1.4+/-0.8. The number of abortion history. were 2.6+/-2. The sexuality of fetus were six males (35 %), five females (29%), and unknown six cases (35 %) and the fetal mortality rate was 29 % (5 cases). The initial clinical symptoms and signs were hypotension (12 patients, 71%), vaginal bleeding(ll patients, 65%), cardiac arrest (6 patients, 35 %), dyspnea (5 patients, 29 %), and seizure (2 patients, 12%). In the case of normal spontaneous vaginal delivary (NSVD), the symptoms occurred during induction in 3 (18%), during delivery in 1 (6%), and after delivery in 9 (53%). In the case of Cesarean section, the symptoms occurred during section in 2 (12%), and after section in 1 (6%). There are one case whose symptoms occurred during 3rd gestational period. CONCLUSIONS: We should alert for the amniotic fluid embolism in the clinical findings of acute collapse and vaginal bleeding, respiratory symptoms, and seizure at any peripartum. With eary suspicion of this disease and aggressive intensive care we can reduce amniotic fluid embolism mortality rate and it's legal problem.
Abortion, Induced ; Amniotic Fluid* ; Autopsy ; Cesarean Section ; Diagnosis ; Dyspnea ; Embolism, Amniotic Fluid* ; Emergency Medical Services ; Female ; Fetal Mortality ; Fetus ; Gestational Age ; Heart Arrest ; Humans ; Hypotension ; Critical Care ; Male ; Maternal Age ; Medical Records ; Mortality ; Peripartum Period ; Pregnancy ; Seizures ; Sexuality ; Uterine Hemorrhage

BACKGROUND: Calcipotriol ointment has been reported to be a safe and effective treatment for patients with psoriasis vulgaris. Scalp involvement of psoriasis is extremely common and in some patients the scalp may be the only affected site. OBJECTIVE: Our purpose was to assess the efficacy, safety and tolerability of calcipotrial solution(50 u g/ml) in the treatment of scalp psoriasis. METHODS: Twenty five patients with mild to moderate scalp psoriasis were treated with calcipotriol solution(50 u g/ml) twice daily for 8 weeks. Efficacy as measured by erythema, thickness and scale of the lesions and safety were assessed every 2 weeks. RESULTS: Reduction of scores of erythema, thickness and scale of the lesions was statistically significant through the treatment. The extent of psoriasis and itching also decreased. An analysis of the overall assessment by investigator and patients at the end of the treatment showed cleared or marked improvement in 80% and 68% individually. Some patients developed a mild and transient burning sensation(2%) or lesional irritation(2%). Laboratory tests including serum calcium levels did not show any significant changes. CONCLUSION: Calcipotriol solution can be used as an effective and safe treatment method for scalp psoriasis.
Burns ; Calcium ; Erythema ; Humans ; Pruritus ; Psoriasis* ; Research Personnel ; Scalp*

No abstract available.
Drug Therapy*

Primary disorders of the nervous system admitted to the Pediatric ward of Seoul National University Hospital during 10 years from January 1969 till December 1978 were analysed to observe particularly the annual change of the incidence. Total number of the diseases of the nervous system was 1522, about 20.2% of the total admitted patients during this period. Annual incidence of the diseases of the nervous system was decreasing. It is mainly due to decreased incidence of the infectious diseases, such as tuberculous meningitis, encephalitis and encephalopathy. The most common deseases of the nervous system during the 10 years were tuberculous meningitis 370 cases (24.3%), aseptic meningitis 122 cases (8.0%), grand mal epilepsy 102 cases (6.7%), unspecified convulsion 79 cases (5.2%), bacterial meningitis 77 cases (5.1%), infectious polyneuritis 68 cases (4.5%), encephalitis 63 cases (4.1%), febrile convulsion 59 cases (3.9%), and unspecified encephalopathy 57 cases (3.7%) in the order of frequency. The incidences of tuberculous meningitis was noted in March, April and May, aseotic meningitis in June, July, August and September, encephalitis in September and October, encephalopathy in June, July, and August, and infectious polyneuritis, in July and August. Mumps meningo encephalities predominantly affected male children. Bacterial meningitis was most common before 2 years of age, especially before 6 months, and tuberculous meningitis during second and third year of life. Aseptic meningitis and mumps meningitis wee mots common at 7 years of age. Encephalopathy occured commonly before one year of age, and Reye's syndrome before four years of age. Unspecified convulsion was commonly observeb before 6 months of age, and febrile convulsion before one year. Infectius ployneuritis was common due to meninitis (76 cases) particularly tuberculous meningitis (50 cases), the next was encephalopathy, unspecified (31 cases).
Child ; Communicable Diseases ; Encephalitis ; Epilepsy, Tonic-Clonic ; Humans ; Incidence* ; Male ; Meningitis ; Meningitis, Aseptic ; Meningitis, Bacterial ; Mumps ; Nervous System* ; Neuritis ; Reye Syndrome ; Seizures ; Seizures, Febrile ; Seoul ; Tuberculosis, Meningeal

This study is to find out changes in medical practice at a university hospital before and after covering intraocular lens (IOL) from the health insurance benefit. The coverage started on March 1, 1993 and a total of 596 cases who were discharged from July 1 to December 31, 1992 and 580 cases who were discharged from July 1 to December 31, 1993 were analyzed. Since the standard reimbursement scheme was changed from March 1, 1993, the charges for 1992 were transformed into 1993 scheme. Major findings are as follows: Average length of stay was statistically significantly decreased from 8.24 days in 1992 to 6 86 days in 1993. Charges except IOL has been statistically significantly decreased from 501,000 won in 1992 to 444,000 won in 1993. Charges for drugs and injection have been reduced. However, charge per day for them was not much different. This is due to decrease in length of stay. Charges for laboratory tests and radiologic examination were quite the same. charges which are not covered by the insurance remained the same. The revenue of the hospital was reduced as expected. However, the hospital reduced the length of stay and increase the turnover rate in order to compensate the potential loss of revenue due to the difference of reimbursement between the out-of-pocket expense and the insurance coverage. By introducing the IOL benefit in the insurance, the insured pays less, hospital generates more revenue through shortening the hospital stay, and the total medical care cost becomes less nationwidely.
Health Care Costs ; Insurance ; Insurance Coverage ; Insurance, Health* ; Length of Stay ; Lenses, Intraocular*

No abstract available.
Cell Line* ; Humans*

BACKGROUND: The pediatric CPR is rather uncommon practice than adult CPR in emergency room. Therefore, few interest and studies were focused in pediatric CPR. The object of this study is to make an analysis about pediatric CPR performed within hospital setting. METHOD: From January 1. 1990 to December 31. 1996, a total of 59 patients, less than 15-year-old, who received cardiopulmonary resuscitation were subject of this study. The study was done retrospective chart analysis for sex, age, weight, the place of cardiac arrest, initial EKG rhythms, endotracheal tube size, the causes of arrest, CPR time, ROSC, the amount and types of CPR drugs used during resuscitation. Wilcoxon rank sum test and chi2 test were used to compare the dose of drugs during CPR in each group. RESULTS: There were no statistical difference between ROSC and non-ROSC group in place of arrest, initial EKG rhythms, CPR time, dosage of CPR drugs. CONCLUSION: The uniform reporting guideline of pediatric advanced life support should be used for future pediatric CPR study, such as Utstein style guideline in pediatric CPR.
Adolescent ; Adult ; Cardiopulmonary Resuscitation* ; Electrocardiography ; Emergency Service, Hospital ; Heart Arrest ; Humans ; Resuscitation ; Retrospective Studies

Adrenal cortical carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as virilization, Cushing syndrome, hyperaldosteronism or feminization. We experienced a case of adrenal cortical carcinoma in a 7yr old boy who showed features of virilization such as rapid growth rate, penile enlargement, and pubic hair. This case was diagnosed with typical hormonal findings and abdominal MRI and confirmed by pathologic findings. He was successfully treated by total left adrenalectomy and has been followed up without problem over eight months.
Adrenalectomy ; Adrenocortical Carcinoma* ; Adult ; Child ; Cushing Syndrome ; Feminization ; Hair ; Humans ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Rare Diseases ; Virilism