Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirrned by kidney biopsy due to her mother's refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome. She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. (J Korean Pediatr Soc 2000;43-837-841)
Acceleration ; Adolescent ; Biopsy ; Blood Glucose ; Cataract ; Diabetes Mellitus* ; Diet, Diabetic ; Diabetic Nephropathies ; Diabetic Retinopathy ; Disulfiram ; Education ; Female ; Glycogen ; Hepatocytes ; Hepatomegaly ; Humans ; Insulin ; Kidney ; Liver ; Obesity, Abdominal ; Physical Examination ; Sexual Maturation

Postpartum thyroid dysfunction occurs in 5-10% of women within one year after delivery. Women with hypothyroidism antedating pregnancy are at high risk for postpartum thyroiditis and should be closely monitored during the first year post-partum. Here, we report a case of recurrent hyperthyroidism between two episodes of postpartum thyroiditis in a woman diagnosed with subclinical hypothyroidism prior to pregnancy. It is of particular interest that spontaneously remitting hyperthyroidism as a sequela of postpartum thyroiditis can occur.
Female ; Humans ; Hyperthyroidism* ; Hypothyroidism ; Postpartum Period* ; Postpartum Thyroiditis* ; Pregnancy ; Thyroid Gland* ; Thyroiditis*

OBJECTIVE: To investigate dose-related effects of repeated intraarticular steroid injection on the experimental arthritis. METHOD: Twenty-four adult male rabbits received Zymosan A into their right knee joint for experimental arthritis. After a week, they were injected with 2 mg (group I; n=9), 10 mg (group II; n=7) or 20 mg (group III; n=8) triamcinolone acetonide into their right knee weekly interval for 4 weeks. We measured weekly changes of the weight and the mediolateral diameter of both knees for the calculation of edema index. 99mTechnetium pertechnate (99mTc) uptake measurement were performed before the first steroid injection and a week after the final steroid injection. All rabbits were sacrificed and histologic examinations of their proximal tibia were performed. RESULTS: A progressive weight loss and changes of edema index were evident for all rabbits (p<0.05) and difference among the groups was not found (p<0.05). 99mTc uptake significantly decreased after the steroid injection in group I (p<0.05) but it was not profound in others (p>0.05). In the histological findings, fibrillation-frayings and fissures of the surface were similar in all rabbits, but loss of nuclear stains and cyst formations were increased prominently in group III than group I (p<0.05). CONCLUSION: Low-dose repeated steroid intraarticular injections are safe and effective treatment in arthritis though no definite evidence of chondroprotection, and high-dose steroid injections accelerate degeneration of the arthritic cartilage.
Adult ; Arthritis* ; Cartilage ; Coloring Agents ; Edema ; Humans ; Injections, Intra-Articular ; Knee ; Knee Joint ; Male ; Rabbits* ; Tibia ; Triamcinolone Acetonide ; Weight Loss ; Zymosan

The Dia and Mia antigens have been detected in Koreans with a frequency of 6.4∼14.5% and 0.9%, respectively. This study evaluated the effectiveness of different screening cells using the cells with Diaand Mia antigens for unexpected antibody screening. An unexpected antibody-screening test was performed separately using different screening cells, including the Dia antigen (Panel D) and Mia antigen (Panel M). A total of 2,077 specimens from 1,847 patients were collected, among which 49 (2.32%) and 43 (2.08%) were positive using Panel D and Panel M, respectively. Twenty-seven patients were positive with both panels, 2012 were negative with both panels, and thirty-eight patients showed a discordant result. The suspected anti-Diaand anti-Mia were detected in 4 (0.19%) and 5 (0.24%) patients, respectively. Therefore, the frequency of anti-Dia and anti-Mia antibodies in this study may be helpful for selecting unexpected antibody screening reagents.

The anti-K antibody has a role in transfusion reactions or hemolytic disease of the fetus and newborns. The K antigen is rarely expressed in Koreans. On the other hand, the distribution of blood group antigens has changed in recent years owing to the increased number of immigrants. This study examined the prevalence of the Kell group antigens according to their parents’ birth region. In this study, 4% of participants whose parents were born in America, Europe, South Asia, and other countries expressed the K antigen. No participants whose parents were born in Korea or East Asia expressed the K antigen. The frequency of the anti-K is expected to increase because of blood donors with the K antigen. Therefore, it is necessary to consider the K antigen frequency and anti-K in transfusions and pregnancy cases in Korea.

Osteonecrosis of the femoral head usually affects young or middle-aged adults and frequently leading to femoral head collapse and hip arthritis. This review reports the updated treatments of the disease.

The anti-K antibody has a role in transfusion reactions or hemolytic disease of the fetus and newborns. The K antigen is rarely expressed in Koreans. On the other hand, the distribution of blood group antigens has changed in recent years owing to the increased number of immigrants. This study examined the prevalence of the Kell group antigens according to their parents’ birth region. In this study, 4% of participants whose parents were born in America, Europe, South Asia, and other countries expressed the K antigen. No participants whose parents were born in Korea or East Asia expressed the K antigen. The frequency of the anti-K is expected to increase because of blood donors with the K antigen. Therefore, it is necessary to consider the K antigen frequency and anti-K in transfusions and pregnancy cases in Korea.

PURPOSE: Porphyromonas gingivalis fimA is a virulence factor associated with periodontal diseases, but its role in the pathogenesis of peri-implantitis remains unclear. We aimed to evaluate the relationship between the condition of peri-implant tissue and the distribution of P. gingivalis fimA genotypes in Koreans using a new primer. METHODS: A total of 248 plaque samples were taken from the peri-implant sulci of 184 subjects. The control group consisted of sound implants with a peri-implant probing depth (PD) of 5 mm or less with no bleeding on probing (BOP). Test group I consisted of implants with a peri-implant PD of 5 mm or less and BOP, and test group II consisted of implants with a peri-implant PD of more than 5 mm and BOP. DNA was extracted from each sample and analyzed a using a polymerase chain reaction (PCR) with P. gingivalis-specific primers, followed by an additional PCR assay to differentiate the fimA genotypes in P. gingivalis- positive subjects. RESULTS: The Prevalence of P. gingivalis in each group did not significantly differ (P>0.05). The most predominant fimA genotype in all groups was type II. The prevalence of type Ib fimA was significantly greater in test group II than in the control group (P<0.05). CONCLUSIONS: The fimA type Ib genotype of P. gingivalis was found to play a critical role in the destruction of peri-implant tissue, suggesting that it may be a distinct risk factor for peri-implantitis.
DNA ; Genotype* ; Hemorrhage ; Peri-Implantitis ; Periodontal Diseases ; Polymerase Chain Reaction ; Porphyromonas gingivalis* ; Porphyromonas* ; Prevalence* ; Risk Factors ; Virulence ; Virulence Factors

BACKGROUND: Thalassemia patients are treated with chronic blood transfusion to relief symptoms. The prevalence of thalassemia in Korea has been increasing recently in response to an increase in the number of immigrants from Southeast Asian countries. Currently, there is no guidelines of blood transfusion outlined by the policy of transfusion of thalassemia, like other inherited hemolytic anemia. Herein, we survey the opinion of transfusion specialists about the policy of blood transfusion of thalassemia in Korea. METHODS: E-mail survey was conducted between July and September of 2017; a total of 40 laboratory medicine specialists working at a general hospital and participating transfusion medicine were included. The questionnaire consisted of blood transfusion experience and blood transfusion policy in patients with thalassemia. RESULTS: Finally, 36 (90%) responders answered the questionnaire; of them, 32 (88.9%) agreed on the necessity of establishing a transfusion guideline for thalassemia patients, and 2 (5.6%) conditionally agreed. Thirty-five (97.2%) respondents agreed on the need for extended matching of RBC antigens for blood transfusion in patients with thalassemia. Thirty-five (97.2%) specialists agreed on the need for extended matching of RBC antigens beyond ABO and RhD to include C, c, E, and e. CONCLUSION: In conclusion, the transfusion guideline for thalassemia, including other inherited hemolytic anemia, is required in Korea. Extended matching of RBC antigens should be considered when developing such guidelines, at least C, c, D, E, e antigens of the Rh blood group are essential for extended matching of RBC antigens.
Anemia, Hemolytic ; Asian Continental Ancestry Group ; Blood Transfusion ; Electronic Mail ; Emigrants and Immigrants ; Hepatitis B e Antigens ; Hospitals, General ; Humans ; Korea* ; Prevalence ; Specialization* ; Surveys and Questionnaires ; Thalassemia* ; Transfusion Medicine

No abstract available.
Adolescent* ; Humans ; Korea* ; Oxidoreductases* ; Prevalence*