Clinical Course of Childhood and Adolescence Onset Type 2 Diabetes Mellitus / 대한소아내분비학회지

PURPOSE:Worldwide incidence of type 2 diabetes mellitus in youth is increased for last 3 decades. We reviewed the clinical characteristics of the patients and their long term complications. METHODS:We reviewed the medical data of 69 patients who were diagnosed type 2 diabetes mellitus at Seoul National University Children's Hospital before the age of 18 years between January 1991 and December 2007. RESULTS:Forty seven patients (68.1%) developed after 2000. The mean age of onset was 13.1+/-2.0 years. Forty seven (69.1%) patients had family history of diabetes mellitus and forty six (66.6%) patients had overweight or obesity. Thirteen (22.4%) patients were small for gestational age but their body mass index wasn't increased compared to that of appropriate for gestational age at the onset of disease. There were 7 patients who had microvascular complication and the mean disease duration was 9.1+/-3.3 years. Hemoglobin A1c was not increased in the complication group compared to the complication-free group. CONCLUSION:The number of childhood and adolescence onset type 2 diabetes mellitus was increased. The proportion of small for gestation age was high and the long term complication developed in adolescence or early adult life. Early intervention of the risk factors and regular evaluation of the complications are needed and systematized follow-up of small for gestational age is also necessary.

A case of testicular adrenal rest tumor in a male child with congenital adrenal hyperplasia / 소아과

Testicular adrenal rest tumors are a well-known complication in male patients with congenital adrenal hyperplasia. Corticosteroid suppressive therapy usually results in the regression of these tumors. We describe a patient with 21-hydroxylase deficiency who developed bilateral testicular masses. Despite steroid suppressive therapy, the tumors did not regress and hormonal control was poor. Consequently, bilateral partial orchiectomies were performed.

Interpretation of screening for congenital adrenal hyperplasia in preterm infants / 소아과

PURPOSE: This study was undertaken to identify factors that influence 17-OHP levels in preterm infants and to suggest a reasonable follow-up schedule of screening for congenital adrenal hyperplasia (CAH) in preterm infants. METHODS: The 17-OHP concentrations in filter paper blood spots of 427 preterm infants were obtained. The effects of gestational age (GA), systemic diseases, and antenatal dexamethasone on screening and follow-up 17-OHP values were investigated. RESULTS: The screening 17-OHP values were markedly variable (range: 0.1-143.3 ng/mL). The screening 17-OHP levels were negatively correlated with GA (r=-0.535, P or =20 ng/mL, the intervals until rescreening 17-OHP <10 ng/mL or serum 17-OHP <20 ng/mL were negatively correlated with GA (r=-0.541, P<0.01) and were prolonged in infants with bronchopulmonary dysplasia (P<0.01). None of the preterm infants were confirmatively diagnosed with CAH. CONCLUSION: The 17-OHP values of preterm infants were influenced by GA, prenatal dexamethasone, and postnatal diseases. Because the 17-OHP vlues of preterm infants were markedly variable, a follow-up schedule should be developed considering both 17-OHP values and clinical status.

A study and the growth and the development of microvascular complications in patients with type 1 diabetes mellitus / 소아과

PURPOSE: Reduced growth and microvascular complications have been recognized as consequences of type 1 diabetes mellitus (T1DM). We assessed the effect of T1DM on growth and factors associated with the development of microvascular complications. METHODS: We conducted a retrospective longitudinal evaluation of 154 patients above 16 years of age. We analyzed factors which affect final height standard deviation scores (SDS) and development of microvascular complications. RESULTS: Final height SDS was -0.11+/-1.15 (-0.26+/-1.33 in females, 0.04+/-0.91 in males). Final height SDS was significantly lower than midparental height SDS and height SDS at diagnosis. There was no difference in final height SDS according to age at onset, existence or nonexistence of complications, or average HbA1C. Height SDS at onset of puberty, midparental height SDS and pubertal growth gain affected final height SDS. The number of patients with complications was 37 (24 percent). Microvascular complications developed at a younger age and after longer duration of diabetes in patients with a prepubertal onset of T1DM compared to patients with pubertal onset. Patients with complications had a higher level of average HbA1C than patients without complications. Patients whose microalbuminuria regressed had lower levels of average HbA1C, systolic BP, second 24h urine microalbumin than patients with persistant or progressed microalbuminuria. CONCLUSION: The results suggest that degrees of glycemic control don't affect final height, but various factors associated with T1DM can impair growth potential. Additionally, the degrees of glycemic control and puberty affect the development of microvascular complications.

Factors affecting the final adult height in survivors of childhood brain tumors / 소아과

PURPOSE: Short stature is an important complication that impairs the quality of life in survivors of childhood brain tumors. We studied their final adult height (FAH) to evaluate risk factors for short stature. METHODS: We reviewed the medical data of 95 survivors of childhood brain tumors (64 males and 31 females) who had been followed up from 1982 to 2006, reached FAH, and had a more than five year-disease-free survival. RESULTS: Final adult height standard deviation score (FAHTSDS: mean+/-SD) of the patients was lower than those of general population (-1.15+/-1.72), HTSDS at diagnosis (-0.13+/-1.57), and target HTSDS (-0.49+/-0.69). FAHTSDS of craniopharyngioma patients did not decrease (0.57+/-1.17), but those of germ cell tumor and medulloblastoma patients were significantly reduced (-1.20+/-1.45, -2.70+/-1.46; P<0.05). The patients treated with craniospinal radiation or chemotherapy had lower FAHTSDS (-1.93+/-1.58, -2.27+/-1.44; P<0.01). In the spinal irradiation group, the younger the age at diagnosis was, the more the loss of FAH (r=0.442, P<0.01). Growth hormone replacement (GHR) didn't improve FAHTSDS, but starting GHR under 12 years was an independent factor for improving FAH once treatment methods were taken into account (P=0.01). CONCLUSION: The younger age at diagnosis, spinal radiation and chemotherapy were all important risk factors of height loss, and height gain was expected in patients who received GHR under the age of 12 years. Therefore, regular check-ups of growth and early intervention with growth hormones are needed for high risk groups to improv

A Case of Orbital Myositis Secondary to Systemic Lupus Erythematosus / 대한류마티스학회지

A 17-year-old girl with 12-year history of systemic lupus erythematosus (SLE) was presented with one month history of diplopia and headache. She had experienced acute cerebral infarction due to multiple cerebral arterial stenosis one year before, and fully recovered except right-side central facial nerve palsy. When she visited pediatric emergency room, ophthalmologic examination showed ophthalmoplegia of the left eye ball; limitation of medial gaze, supra-adduction and infra-adduction. Neurologic examination didn't show any newly developed neurologic defect. There was no newly developed intra-cranial lesion on the brain MRI. But, the brain MRI revealed irregularly enhanced thickened left medial rectus muscle, and that was compatible with orbital myositis. There was no definite evidence of infection or other autoimmune disease. Her condition responded to high dose intravenous methylprednisone therapy (1 g/day for 3 days) and continued oral prednisolone.

An Enterococcus gallinarum Strain Carrying Both vanA and vanC1 Genes / 대한임상병리학회지

Enterococcus gallinarum carrying both vanA and vanC1 genes were detected from a surveillance culture from a patient staying at the surgical intensive care unit for a few years. E. gallinarum, SI04, was highly resistant to vancomycin (MIC of >or=256ng/mL) and teicoplanin (MIC of >or=256ng/mL). Multiplex PCR for vanA, vanB, vanC1 and vanC2/3 genes revealed SI04 to be positive for both vanA and vanC1 genes. This finding supports the fact that genotyping is needed to classify vancomycin-resistant enterococci (VRE). This is the first report on VanC VRE accompanying vanA gene in Korea.

A Case of Septicemia Caused by Yersinia pseudotuberculosis / 대한임상미생물학회지

Yersinia pseudotuberculosis is a relatively infrequent cause of human infections, mostly as intestinal yersinosis. A septicemic form of Y. pseudotuberculosis infection has been reported only rarely. It is usually seen in patients with underlying disorders such as diabetes, hepatic cirrhosis or iron overload. A 63-year-old man with diabetes mellitus and liver fibrosis was admitted to Asan Medical Center via emergency department because of epigastric pain, fever and watery diarrhea; he was septic. The stool culture did not grow Salmonella, Shigella, or Yersinia. But, in the blood culture Y. pseudotuberculosis grew from one anaerobic vial among two sets of aerobic and anaerobic blood cultures. Serotype of Y. pseudotuberculosis strain was could not be determined because it was a rough type. The isolate was positive in the autoagglutination test and polymerase chain reaction for the virF gene. The serum levels of iron, TIBC and ferritin were within normal range. The patient received ceftriaxone therapy for 3 days and was discharged with a clinical improvement.

The Performance Evaluation of Yeongdong URiSCAN GEN 10SGL Urine Dipstick Strip Using Other Quantitative, Microscopic, and Culture Methods / 대한임상병리학회지

BACKGROUND: The previous performance tests of URiSCAN GEN 10SGL dipstick reagent strip (Yeongdong pharmaceutical Co., Seoul, Korea) were mainly done by comparison with the approved urine strips. However, adequate comparison was inavailable because the grading systems were different among the manufacturers. We evaluated the correlation of new generation URiSCAN GEN 10SGL urine strip with known quantitative, microscopic, and culture methods. METHODS: We used urine specimens which were collected for the urinalysis and culture from November 2000 to Mars 2001. We evaluated the correlation between the results of URiSCAN GEN 10SGL and the quantitative methods by comparing the mean of change of reflectance rate (change %R) with the result of the corresponding quantitative method for protein, glucose, bilirubin, urobilinogen, pH, and specific gravity. To calculate the sensitivity and specificity, we used microscopic examination for leukocytes and erythrocytes, and used urine culture for nitrite test. RESULTS: The correlation coefficients between the change %R of URiSCAN GEN 10SGL and the corresponding quantitative method exceeded 0.81, except bilirubin and specific gravity (P<0.01; respectively). The agreements of identical or neighboring concentration block were more than 90%, except urobilinogen and specific gravy. The sensitivity and specificity of URiSCAN GEN 10SGL were 63.6% and 94.2% for leukocytes; 92.8% and 74.1% for erythrocytes; 74.4% and 85.0% for nitrite producing organisms. CONCLUSTIONS: URiSCAN GEN 10SGL had acceptable accuracy and agreement compared with the corresponding quantitative methods and culture result. Also, it had improved sensitivity and specificity of leukocytes and erythrocytes detection compared with previous URiSCAN urine dipstick strip.

Evaluation of Vironostika HIV Uni-Form II Ag/Ab Enzyme Immunoassay Kit for Simultaneous Detection of Antigen and Antibody of HIV / 대한수혈학회지

BACKGROUND: It has been estimated that 1 unit per 493,000 blood donations could transmit HIV by infected persons in seroconversion window in America. The 4th generation enzyme immunoassay (EIA) kits which are designed to detect HIV antigen and antibody simultaneously, could reduce the period of seroconversion window and thereby increase the safety of donated bloods without additional expenses. METHODS: Sensitivity and specificity of Vironostika HIV Uni-Form II Ag/Ab (Organon Technika, Boxtel, Netherlands) were evaluated using 53 samples (11 western blot positive samples and 42 sera from BBI panel) and 391 samples (282 healthy donors, 20 anti-HCV positive sera and 89 FANA positive sera), respectively. Seroconversion window was compared with that of COBAS CORE Anti-HIV-1/HIV-2 EIA DAGS (Roche Diagnostics, Basel, Switzerland) using 6 kinds of BBI seroconversion panel. RESLUTS: Sensitivity was 100% (53/53). Aggregated specificity was 99.5% (389/391), while all the samples from the healthy donors showed negative (282/282). COBAS CORE Anti-HIV-1/HIV-2 EIA DAGS also showed 100% sensitivity (53/53) and 100% specificity (391/391). Vironostika HIV kit detected HIV-1 infection earlier about 9.8 days than COBAS CORE Anti-HIV-1/HIV-2 EIA DAGS. CONCLUSION: Sensitivity and specificity of Vironostika HIV kit were as good as those of COBAS CORE kit. Vironostika HIV kit, however, could get more safety of donated bloods than the 3rd generation EIA such as COBAS CORE Anti-HIV-1/HIV-2 EIA DAGS, in that it can reduce seroconversion window of HIV infection.