No abstract available.

Two cases of multiple epiphyseal dysplasia in a 3 years old boy and a 10 years old girl are reported. Since Fairbanks description of the disease in 1935, it has been a well recognized entity which is a rare congenital developmental error characterized by mottling or irregularity in density and outline of several of the developing epiphyses, and dwarfism. Many reports indicate a definite hereditary and familiar distribution, particularly one series in which there were 10 cases in a single family, But in this report parent and siblings, all of whom were examined clinically, and roentgenographically showed no demonstrable stigmata of any growth disturbance. As the child develops, a subnormal growth rate becomes more obvious. The vertebrae are usually not affected so that dwarfing is confined to the extremity. The process rarely comes to attention of the physician until the child begin to walk, and then it is noted that one has a waddling gait and short stubby digits of the hands and feet. Intelligence is unaffected. Prognosis as to life expectancy and range of activity is good.
Child ; Christianity ; Dwarfism ; Epiphyses ; Extremities ; Female ; Foot ; Gait ; Hand ; Humans ; Intelligence ; Life Expectancy ; Male ; Osteochondrodysplasias ; Parents ; Prognosis ; Siblings ; Spine

PURPOSE: To evaluate the clinical characteristics, treatments and outcome of patients with hemorrhagic shock and encephalopathy(HSE) syndrome. METHODS: We performed a clinical study on 14 patients who were diagnosed as hemorrhagic shock and having encephalopathy syndrome in the Department of Pediatrics, from 1984 to 1998. Age, sex, clinical symptoms and physical findings at admission, the most deranged laboratory findings, radiologic findings, treatments and outcome were analyzed. RESULTS: The age of onset was 1.0+/-0.9 years and the male to female ratio was 1: 1.8. At admission, clinical findings included dehydration in 85.7%, shock in 85.7%, fever in 71.4%, vomiting in 71.4%, diarrhea in 64.3%, GI bleeding in 50%, convulsion in 42.9%, and edema in 35.7%. Altered mental state was found in 100%, hepatomegaly in 64.3%, and splenomegaly in 21.4%. Laboratory findings revealed D-dimer positive in 92.9%, the mean hemoglobin level 8.2+/-2.1g/dL, BUN 35.7+/-24.0mg/dL, creatinine 1.9+/-1.5mg/dL, AST 561.0+/-1,412.1IU/L, ALT 858.9+/-1,649.8IU/L, blood glucose 229.5+/-197.4mg/dL, ammonia 195.4+/-129.7pg/dL, and total bilirubin 4.9+/-8.2mg/dL. On serologic tests, rotavirus and Epstein-Barr virus was found in 1 patient(7.1%), respectively. The mortality rate was 78.6%. CONCLUSION: We found that shock and disseminated intravascular coagulation(DIC) played important roles in the pathogensis of HSE syndrome, and encephalopathy, hepatic and renal insufficiency, and respiratory failure were secondary complications resulting from shock and DIC. Despite vigorous treatment, the prognosis was very poor. We feel more efforts should be focused on investigating the etiology and pathophysiology of HSE to prevent as well as develop a specific therapy. (J Korean Pediatr Soc 2000;43:814-819)
Age of Onset ; Ammonia ; Bilirubin ; Blood Glucose ; Creatinine ; Dacarbazine ; Death, Sudden* ; Dehydration ; Diarrhea ; Edema ; Female ; Fever ; Hemorrhage ; Hepatic Encephalopathy ; Hepatomegaly ; Herpesvirus 4, Human ; Humans ; Infant* ; Male ; Mortality ; Pediatrics ; Prognosis ; Renal Insufficiency ; Respiratory Insufficiency ; Rotavirus ; Seizures ; Serologic Tests ; Shock ; Shock, Hemorrhagic* ; Splenomegaly ; Vomiting

BACKGROUND: The prevention of preterm deliveries still remains a major problem in ob-stetrics. The cause of preterm labor is poorly understood. Our purpose was to determine the risk factors for preterm labor associated with specific clinical and environmental factors. METHODS: Using a case-control design, 54 women with preterm labor and 134 controls with term labor, who were admitted to the department of Obstetrics and Gynecology, Dan- kook University Hospital from January, 1996 to August, 1996. The study groups were inte- rviewed and their medical records were reviewed. RESULTS: 1. There were no significant differences of maternal age, maternal weight at 20 weeks of gestation and maternal weight gain at the time of delivery. 2. There were no significant differences between housewives and working women. But the physical workload of the preterm labor group was significantly higher than the control group ( 16.0% vs. 4.0% ). 3. There were no significant differences in the smoking habit of mother or her husba- nd. The passive smoking of the preterm labor group was significantly higher than the con- trol group ( 43.8% vs. 26.8% ). 4. In vaginal bleeding at pregnancy, the preterm labor group was significantly higher than the control group ( 15.7% vs. 2.3% ). Especially, the third trimester vaginal bleeding of the preterm labor group was significantly higher than the control group. 5. There were no significant differences in the number of previous fullterm deliveries, previous abortions, and living children between two groups. The experience of the previous preterm deliveries of the preterm group was significantly higher than the control group ( 11. 5% vs. 0.8% ). 6. There were no significant differences of medications, consumption of alcohol, coffee, tea, green-tea, cola and nutritional beverages, the experiences of coitus, defecation numbers and accidents during pregnancy between two groups. 7. There were no significant differences in the plasma concentrations of hemoglobin, hematocrit, white blood cells, and in the blood pressure, and the body temperature between two groups. CONCLUSION: The risk factors of preterm labor were severe physical workload, vaginal bleeding at pregnancy, especially, the third trimester vaginal bleeding, and the experience of previous preterm deliveries.
Abortion, Induced ; Beverages ; Blood Pressure ; Body Temperature ; Case-Control Studies* ; Child ; Coffee ; Coitus ; Cola ; Defecation ; Female ; Gynecology ; Hematocrit ; Humans ; Leukocytes ; Maternal Age ; Medical Records ; Mothers ; Obstetric Labor, Premature* ; Obstetrics ; Plasma ; Pregnancy ; Pregnancy Trimester, Third ; Risk Factors* ; Smoke ; Smoking ; Tea ; Tobacco Smoke Pollution ; Uterine Hemorrhage ; Weight Gain ; Women, Working

Relapsing polychondritis is an uncommon disease manifested by episodes of progressive inflammation and destruction of cartilage. While the cause remains unknown, an autoimmune pathogenesis appears likely. Characteristic features include auricular chondritis, arthritis, nasal chondritis, ocular inflammation, respiratory tract involvement and audiovestibular damage. The clinical course of patients may vary from a relatively benign illness free of clinically evident visceral involvement, to one of episodic or smoldering activity with variable intensity, to a f ulminant illness and death within months of diagnosis. Dapsone, corticosteroid and immunosuppressive agent remain the effective therapies. We have experienced a case of relapsing polychondritis and active pulmonary tuberculosis in a 69-year-old man. He suffered from arthralgia and swelling of joints for several months on the first admission and initially was diagnosed as pseudogout based on polyarthritis and chondrocalcinosis of right knee joint. During follow-up, chondritis of both auricles and nose, which was proved by the biosy of right auricle, and relapse of polyarthritis were developed. His spu tum smear for acid-fast bacillus was postive on the second admission. Then he was diagnosed as relapsing polychondritis and active pulmonary tuberculosis and has achieved improvement with medication of dapsone and antituberculosis drugs according to susceptability test during followed-up. Thus we report this case with literature review.
Aged ; Arthralgia ; Arthritis ; Bacillus ; Cartilage ; Chondrocalcinosis ; Dapsone ; Diagnosis ; Follow-Up Studies ; Humans ; Inflammation ; Joints ; Knee Joint ; Nose ; Polychondritis, Relapsing* ; Recurrence ; Respiratory System ; Tuberculosis, Pulmonary

No abstract available.
Thyroid Diseases* ; Thyroid Gland*

No abstract available.
Antibodies, Monoclonal* ; Blood Cells*

No abstract available.
Hepatolenticular Degeneration*

PURPOSE: H. pylori infection was recently reported to be associated with unexplained iron-deficiency anemia(IDA) in children and adolescents. H. pylori-related IDA was thought to occur due to GI blood loss, scavenging of iron by H. pylori and iron malabsorption. The aim of this study was to examine how the status of H. pylori infection and age of children affected RBC indices, serum iron level and TIBC. METHODS: We performed esophagogastroduodenoscopy, and examined RBC indices, serum iron and TIBC on 178 pediatric patients with recurrent abdominal pain. H. pylori infection was assessed by CLO test and silver stain of gastric biopsy specimen. RESULTS: H. pylori infection was found in 42 of 178 patients(23.6%). In children with H. pylori infection, the prevalence of iron deficiency(ID) and IDA(39.0% and 10.3%, respectively) was higher than in children without H. pylori infection(29.6% and 4.1%, respectively), but there was no statistically significant difference. Serum iron level was lower(69.5+/-32.7 vs 77.3+/-34.1g/dL; P= 0.08) and TIBC was higher(380.8+/-50.4 vs 366.9+/-47.0g/dL; P=0.09) in children with H. pylori infection than in children without H. pylori infection. All RBC indices and iron saturation were lower in children with H. pylori infection than in children without H. pylori infection, but there was no significant difference between the two groups. In correlation analysis, serum iron, Hgb, Hct, MCV and MCH were significantly increased by age in children without H. pylori infection (P<0.05), but MCH and MCHC were decreased significantly(P<0.05) by age in children with H. pylori infection. In multiple regression analysis, the change of serum iron, MCH and MCHC by age was significantly influenced by the status of H. pylori infection(P<0.05). CONCLUSION: H. pylori infection decreases RBC indices and serum iron and increases TIBC in children. These changes become prominent as age increases. This age effect rnay be related to the duration of H. pylori infection. (J Korean Pediatr Soc 2000;43:755-762)
Abdominal Pain ; Adolescent ; Anemia, Iron-Deficiency ; Biopsy ; Child* ; Endoscopy, Digestive System ; Erythrocytes* ; Helicobacter pylori* ; Helicobacter* ; Humans ; Iron* ; Prevalence ; Silver

No abstract available.
Phenylketonurias*