Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

PURPOSE: Exercise is frequently recommended in the management of diabetes mellitus(DM) and can improve blood control by increasing insulin sensitivity and psychological benefits. Exercise can result in good glycemic control in type 2 DM, when combined with diet and drug therapy. However, in type 1 DM, the expected improvement in glycemic control with exercise have not been clearly established. Effects of exercise on glycemic control in patients with type 1 DM were investigated. METHODS: 20 patients with of type 1 DM, who were no retinopathy, neuropathy, nephropathy and cardiac disorders, were enrolled and exercised for 30min. with 50% of individualized maximum VO2. Blood sugar concentration were measured before, immediate and 15min after exercise. The results were evaluated with HbA1c, C-peptide and DM duration. RESULTS: Blood sugar concentration were significantly decreased from pre-exercise 198+/-9.7mg/dL to immediate 145+/-7.1mg/dL and at 15min. Post-exercise 134+/-4.0mg/dL(P<0.05). In case with HbA1c<10%, there was significantly decreased in blood sugar level from pre-exercise 176+/-9.7mg/dL to immediate 123+/-63.2mg/dL and at 15min post-exercise 113+/-1.9mg/dL(P<0.05). In case with HbA1c>10% there was also significantly decreased in blood sugar levels 222+/-64.7mg/dL, 169+/-6.1mg/dL, 157+/-1.2mg/dL respectively(P<0.05). Group with moderate decreased blood sugar(40-99mg/dL) was 8 patients(42.1%), group with mild decreased blood sugar(<40mg/dL) was 7 patients(36.8%) and group with decreased more than 100mg/dL was 4 patients(21.5%). There was no correlation in degree of decreased blood sugar concentration among HbA1c, C-peptide, DM duration. CONCLUSION: Proper exercise in type 1 diabetic children can decrease blood sugar level significantly and make good glycemic control and can decrease DM microvascular complications in addition to motivating physically active lifestyle.
Blood Glucose ; C-Peptide ; Child ; Diabetes Mellitus ; Diabetes Mellitus, Type 1* ; Diet ; Drug Therapy ; Humans ; Insulin Resistance ; Life Style

OBJECTIVES: The myelodysplastic syndromes (MDS) are a group of acquired clonal hematopoietic disorders characterized by peripheral cytopenias and a hypercellular or normocellular dysplastic bone marrow. The mechanisms responsible for development of MDS are not known. We performed this study to evaluate the hematopoietic abnormalities and apoptosis in MDS. METHODS: Long-term bone marrow culture (LTBMC) was performed for colony assays, cobblestone area assay, stromal morphologic changes from 7 patients with MDS and 7 normal controls. In situ nick end labeling (ISNEL) method was performed for detection of apoptosis from LTBMC in 7 patients with MDS and 7 normal controls. ISNEL method also performed in bone marrow cell bloc samples in 36 patients with MI3S. RESULTS: Viability of nonadherent cells from LTBMC of patients with MDS was not decreased compared with normal controls at 1 week, but significantly decreased at 2 and 3 weeks compared with normal controls (P<0.0001). Formation of the cobblestone areas from patients with MDS was slightly decreased compared with normal controls at 1st week, but significantly decreased at 2nd and 3rd weeks compared with normal controls (P<0.0001). Slightly decreased compared with normal controls at 1 week, but significantly decreased at 2 and 3 weeks compared with normal controls (P<0.0001). Stromal layers produced in LTBMC of normal controls and 1 patient with MDS were detected at 1 week and were formed confluent stroma from 3 weeks, but another patients with MDS who did not form a confluent stroma. Patients with MDS had significantly lower colony forming unit granulocyte-macrophage (CFU-GM) compared with normal controls at 1 (P<0.01) and 2 weeks (P<0.001) of LTBMC. Two weeks of LTBMC resulted more profound inhibition of CFU-GM formation than 1 week (P<0.0001). Apoptotic cell death was absent in adherent and non adherent cells from normal controls at 1 and 2 weeks, but massive apoptotic cell death was found in adherent and non adherent cells from patients with MDS at 1 and 2 weeks and the degree of apoptosis was profound at 2 weeks compared with 1 week. Among the 36 patients, fifteen patients demonstrated varying degrees of apoptosis positive cells, 4 having low, 8 intermediates, and 3 high scores. Remaining 21 patients showed absent apoptosis or only occasional positive cells. CONCLUSION: Hematopoietic abnormalities such as a failure of differentiation are caused by the stromal defects and the biologic basis of the apparent paradox of peripheral cytopenias in the face of hypercellular (or normocellular) marrow is related by intramedullary apoptotic cell death of the stromal and hematopoietic cells.
Apoptosis* ; Bone Marrow Cells ; Bone Marrow* ; Cell Death ; Granulocyte-Macrophage Progenitor Cells ; Humans ; In Situ Nick-End Labeling* ; Myelodysplastic Syndromes* ; Stem Cells

No abstract available.
Acute Kidney Injury* ; Lymphoma*

Purpose: High-risk pregnancy, resulting in major complications, such as abortion, gestational hypertension, and preterm labor, are on the rise, requiring intervention. Increased concentration of particulate matter that is 10 μg per cubic meter or less in diameter (PM10) due to increased air pollution and industrialization has recently been noted as a risk factor for health problems, and prior studies have reported that PM10 is related to abortion, gestational hypertension, and preterm labor. Methods: This study used data from the Korean medical panel between 2008 and 2016 to identify 879 women with normal pregnancies and 301 women diagnosed with high-risk pregnancies. The association between high-risk pregnancy and PM10 was analyzed using the annual average regional concentration during the same period by the Korea Environment Corporation. Results: At 35 years of age or older, the risk of abortion (p<0.001; 95% confidence interval [CI], 1.43–2.71) and preterm labor increased (p<0.05; 95% CI, 0.33–0.90). Married pregnant women were at a high risk of preterm labor (p<0.05; 95% CI, 0.25–0.98), and the prevalence of gestational hypertension was high in the metropolitan area (p<0.05; 95% CI, 12.09–25.93). On comparing the average annual concentration of particulate matter according to the level of atmospheric ventilation of the Korea Environment Corporation using a cutoff of ≤50 μg/m3, the probability of preterm labor was 2.2 times higher in groups exposed to particulate matter concentration of ≥50 μg/m3 (p<0.01; 95% CI, 1.49–3.25). Conclusion PM10 was related to preterm labor, and the risk of preterm labor was high if the average annual concentration of PM10 in the individual’s residential area exceeded 50 μg/m3.

No abstract available.
Fetus* ; Humans ; Male* ; Mesothelioma*

Background: Dipeptidyl peptidase-4 inhibitor (DPP-4i) and renin-angiotensin system (RAS) blockade are reported to affect the clinical course of coronavirus disease 2019 (COVID-19) in patients with diabetes mellitus (DM). Methods: As of May 2020, analysis was conducted on all subjects who could confirm their history of claims related to COVID-19 in the National Health Insurance Review and Assessment Service (HIRA) database in Korea. Using this dataset, we compared the short-term prognosis of COVID-19 infection according to the use of DPP-4i and RAS blockade. Additionally, we validated the results using the National Health Insurance Service (NHIS) of Korea dataset. Results: Totally, data of 67,850 subjects were accessible in the HIRA dataset. Of these, 5,080 were confirmed COVID-19. Among these, 832 subjects with DM were selected for analysis in this study. Among the subjects, 263 (31.6%) and 327 (39.3%) were DPP4i and RAS blockade users, respectively. Thirty-four subjects (4.09%) received intensive care or died. The adjusted odds ratio for severe treatment among DPP-4i users was 0.362 (95% confidence interval [CI], 0.135 to 0.971), and that for RAS blockade users was 0.599 (95% CI, 0.251 to 1.431). These findings were consistent with the analysis based on the NHIS data using 704 final subjects. The adjusted odds ratio for severe treatment among DPP-4i users was 0.303 (95% CI, 0.135 to 0.682), and that for RAS blockade users was 0.811 (95% CI, 0.391 to 1.682). Conclusion This study suggests that DPP-4i is significantly associated with a better clinical outcome of patients with COVID-19.

Background: Dipeptidyl peptidase-4 inhibitor (DPP-4i) and renin-angiotensin system (RAS) blockade are reported to affect the clinical course of coronavirus disease 2019 (COVID-19) in patients with diabetes mellitus (DM). Methods: As of May 2020, analysis was conducted on all subjects who could confirm their history of claims related to COVID-19 in the National Health Insurance Review and Assessment Service (HIRA) database in Korea. Using this dataset, we compared the short-term prognosis of COVID-19 infection according to the use of DPP-4i and RAS blockade. Additionally, we validated the results using the National Health Insurance Service (NHIS) of Korea dataset. Results: Totally, data of 67,850 subjects were accessible in the HIRA dataset. Of these, 5,080 were confirmed COVID-19. Among these, 832 subjects with DM were selected for analysis in this study. Among the subjects, 263 (31.6%) and 327 (39.3%) were DPP4i and RAS blockade users, respectively. Thirty-four subjects (4.09%) received intensive care or died. The adjusted odds ratio for severe treatment among DPP-4i users was 0.362 (95% confidence interval [CI], 0.135 to 0.971), and that for RAS blockade users was 0.599 (95% CI, 0.251 to 1.431). These findings were consistent with the analysis based on the NHIS data using 704 final subjects. The adjusted odds ratio for severe treatment among DPP-4i users was 0.303 (95% CI, 0.135 to 0.682), and that for RAS blockade users was 0.811 (95% CI, 0.391 to 1.682). Conclusion This study suggests that DPP-4i is significantly associated with a better clinical outcome of patients with COVID-19.

OBJECTIVES: From February 1995 to April 1998, those patients who had visited for evaluation of infertility, shown abnormal endometrial pattern on hysterosalpingography underwent resectoscopic operation. We evaluated about its therapeutic effect, recurrence rate of uterine adhesion and effectiveness of its assisted method. METHOD: We have reviewed 45 cases of intrauterine adhesion, classified as a central type, marginal type and multiple type. We used 26F resectoscope made in Storz for operation and inserted Lippes loop or pediatric foley catheter for prevention of readhesion. For promoting reepithelialization, conjugated estrogens(premarin) 5mg daily for 30 - 50 days were given and then 10mg of medroxyprogesterone acetate(provera) were added daily for the last 10 days. We evaluated the uterine cavity condition preoperatively and postoperatively by using hysterosalpingography. RESULT: In 45 cases, 41 cases were followed up postoperatively. 21 cases were markedly improved, 12 cases were improved and 8 cases were not improved or recurred on hysterosalpingography. In 41 cases, for prevention of readhesion 20 cases were used pediatric foley catheter and 5 cases(25%) were recurred. 21 cases were used Lippes loop and 3 cases(14%) were recurred. Pregnancy outcome was as follows;15 cases were pregnant and 10 cases delivered a viable infant, 3 cases aborted spontaneously, 1 case was ectopic pregnancy and laparoscopic salpingectomy was done, 1 case was ongoing pregnancy. CONCLUSION: This report suggests that resectoscopic operation is very effective in the treatment of intrauterine adhesion(therapeutic rate of 81%) and that the use of Lippes loop seems like to have the less recurrence rate than the use of pediatric foley catheter in prevention of postoperative readhesion, but more experience and further follow-up are necessary to obtain more detailed conclusions.
Catheters ; Female ; Follow-Up Studies ; Humans ; Hysterosalpingography ; Infant ; Infertility ; Medroxyprogesterone ; Pregnancy ; Pregnancy Outcome ; Pregnancy, Ectopic ; Recurrence ; Salpingectomy

BACKGROUND: There are only a few studies regarding the causes of treatment failure for tuberculosis. Therefore, this study aimed to determine the causes of intractable tuberculosis. METHODS: M.tuberculosis, differentiated MOTT (Tycobacterium Other Than Tuberculosis) were isolated, and the RFLP (Restriction fragments length polymorphisms) pattern was analyzed from 204 patients with pulmonary tuberculosis and 53 suffering from neck tuberculosis. The IL-1β, IL-12, *1 IFNγ and *2 TNFαblood levels were measured. All patients were regularly followed for 18 months after treatment. RESULTS: There was no correlation between the RFLP patterns of M.tuberculosis treatment failure. From the 204 cases, 31.9% were intractable. The characteristics of patients with intractable tuberculosis were old age, being male and recurrent cases. The causes of treatment failure were identified as follows ; a decrease in the IL-12(59.4%) concentration, drug resistant strain(54.7%), irregular medication(15.4%), MOTT(6.2%) and a heavy infection(4.6%). The causes of all cases of intractable tuberculosis could be investigated. The IL-12 concentration in the blood was significantly lower in the intractable cases, where it disclosed a maximum sensitivity(64.7%) and specificity(75.4%) at 165.0 pg/ml. Most of the 53 cases on neck node tuberculosis were treated successfully. Therefore, we were unable to analyze the cause of treatment failure. CONCLUSION: A decrease in the blood IL-12 concentration and drug resistant strains were identified as the most significant causes of treatment failure for tuberculosis. In Korea, infection by clusters were prevalent, but no difference in the clinical course between clusters and non-clusters could be found.
Humans ; Interleukin-12 ; Korea ; Male ; Neck* ; Polymorphism, Restriction Fragment Length ; Treatment Failure* ; Tuberculosis* ; Tuberculosis, Pulmonary