Cutaneous Mucormycosis in a Patient with Diabetes Mellitus / 이화의대지

Mucormycosis is a rare disease caused by fungi. Most commonly involved sites of mucormycosis infection are sinuses, lungs, skin and soft tissues. Systemic risk factors for mucormycosis are diabetes mellitus, neutropenia, corticosteroid use, hematological malignancies, organ transplantation, metabolic acidosis, deferoxamine use and advanced age. Local risk factors are history of trauma, burns, surgery and motor vehicle accidents. We present a case of cutaneous mucormycosis in a patient with diabetes mellitus. A 66-year-old female with uncontrolled diabetes mellitus, admitted with necrotizing lesion after minor abrasions on leg. We took a culture of the lesion and it is diagnosed with mucormycosis. Disease progressed despite administration of systemic amphotericin B. We performed above-knee amputation and changed antifungal agents into liposomal amphotericin B. A tissue biopsy showed nonseptate, irregularly wide fungal hyphae with frequent right-angle branching. Our case report suggests that patients with risk factors should be observed carefully.

Testosterone Replacement and Bone Mineral Density in Male Pituitary Tumor Patients

BACKGROUND: Hypopituitarism is associated with osteoporosis and osteopenia especially when hypogonadotropic hypogonadism is present. Despite hypopituitarism being an important cause of secondary osteoporosis, osteoporosis in patients receiving surgery for pituitary tumors in Korea has not been studied. In this study, we evaluated the effects of testosterone replacement therapy (TRT) on bone mineral density (BMD) in postoperative hypogonadal patients with pituitary tumors. METHODS: To examine the effect of TRT on BMD, we performed a retrospective observational study in 21 postoperative male patients who underwent pituitary tumor surgery between 2003 and 2012 at the Ajou University Hospital. Testosterone was replaced in postoperative hypogonadal patients by regular intramuscular injection, daily oral medication, or application of transdermal gel. BMD (g/cm2) measurements of central skeletal sites (lumbar spine, femoral neck, and total femur) were obtained using dual-energy X-ray absorptiometry (GE Lunar). For lumbar spine BMD, L1 to L4 values were chosen for analysis. Femur neck and total femur were also analyzed. RESULTS: During the follow-up period (mean, 56 months; range, 12 to 99 months) serum testosterone levels increased with the administration of TRT (P=0.007). There was significant improvement (4.56%+/-9.81%) in the lumbar spine BMD compared to baseline BMD. There were no significant changes in the femur neck BMD or total femur BMD. We did not find any statistically significant relationships between changes in testosterone levels and BMD using Spearman correlation analysis. CONCLUSION: Our results indicated that TRT used in the postoperative period for hypogonadal pituitary tumor surgery patients may have beneficial effects on the BMD of the spine.

Sleep Duration and Glycemic Control in Patients with Diabetes Mellitus: Korea National Health and Nutrition Examination Survey 2007-2010

Short sleep duration has been reported to increase the risk of diabetes. However, the influence of sleep duration on glycemic control in diabetic patients has not been clarified. In this study we evaluated the association between sleep duration and glycemic control in diabetic patients. We analyzed the data from the Korea National Health and Nutrition Examination Survey (KNHANES) 2007-2010. Sleep duration was classified into five groups: or =9 h/day. Fasting blood glucose and HbA1c showed a U-shaped trend according to sleep duration. Sleep duration of 7 h/day had the lowest HbA1c (7.26%) among the subjects (P=0.026). In the older age group (> or =65 yr), a sleep duration of 6 h/day was associated with the lowest HbA1c (7.26%). The adjusted odds ratio (OR) with a 95% confidence interval (CI) of worse glycemic control (HbA1c > or =7.0%) in group of sleep duration of > or =9 h/day was 1.48 (1.04-2.13) compared with the group of 7 h/day. This relationship disappeared after adjusting duration of diabetes (OR, 1.38; 95% CI, 0.93-2.03). Our results suggest that sleep duration and glycemic control in diabetic patients has U-shaped relationship which was mainly affected by duration of diabetes.

An Unusual Presentation of Diabetic Ketoacidosis in Familial Hajdu-Cheney Syndrome: A Case Report

A 21-year-old man with diabetic ketoacidosis (DKA) displayed short and clubbed fingers and marked eyebrow, which are typical of Hajdu-Cheney Syndrome (HCS). Laboratory findings confirmed type 1 diabetes mellitus (DM). After conservative care with hydration and insulin supply, metabolic impairment was improved. Examinations of bone and metabolism revealed osteoporosis and craniofacial abnormalities. The mutation (c.6443T>G) of the NOTCH2 gene was found. The patient was diagnosed with HCS and DM. There may be a relationship between HCS and DM, with development of pancreatic symptoms related to the NOTCH2 gene mutation.

A Case of CATCH22 Syndrome with Normal Parathyroid Function

CATCH 22 is a medical acronym for cardiac defects, abnormal faces, thymic hypoplasia, cleft palate, and hypocalcemia, and a variable deletion on chromosome 22. It includes DiGeorge syndrome, conotruncal anomaly face syndrome, and velo-cardio-facial syndrome. It has a prevalence estimated at 1:3,000-1:6,000. Most deletions occur at de novo, but autosomal dominant inheritance is observed in 6-10% of cases. Hormonal disorders are common in patients with CATCH22 syndrome. While hypoparathyroidism was the predominant endocrine disturbance that has been documented in the DiGeorge syndrome, other hormonal defects, such as growth hormone deficiency, hypothyroidism, and hyperthyroidism have been occurred in patients with CATCH22 syndrome. The spectrum of parathyroid gland dysfunction in this syndrome ranges from severe neonatal hypocalcemia to normal parathyroid function. Most patients are usually diagnosed in young age, but a few patients with mild abnormality are presented later in life. We report a case of CATCH22 syndrome with normal parathyroid hormone and calcium level in an adult. The diagnosis of CATCH22 syndrome was confirmed by fluorescence in situ hybridization analysis.

Need for Cross-calibration of Body Composition Even with the Same Model of Dual-energy X-ray Absorptiometry / 대한골다공증학회지

OBJECTIVES: The purpose of this study was to compare total body fat mass, lean mass, and bone mineral content in addition to regional fat measured by the same model of equipment installed in different places, and to determine the extent of agreement. MATERIALS AND METHODS: Twenty seven healthy volunteers aged 20 years and over received two consecutive total body scans using the same dual-energy X-ray absorptiometry model of installed in different places. All scans were conducted on the same day. RESULTS: Relatively good agreements were shown in fat mass, the percent of tissue fat, android fat, and gynoid fat. However, there were two outliers each in lean mass and bone mineral content based on the limits of agreement. CONCLUSIONS: These results indicate the need for cross-calibration even with the same model of equipment.

Comparison of the Clinical Characteristics of Bisphosphonate-associated Osteonecrosis of the Jaw with Inflammation-associated Osteonecrosis of the Jaw in Patients with Osteoporosis and Benign Diseases / 대한골다공증학회지

OBJECTIVES: Osteonecrosis of the jaw (ONJ) involving the use of bisphosphonates is referred to as bisphosphonate-associated ONJ (BONJ). Inflammation also can cause ONJ and it may be called inflammation-associated ONJ (IONJ). The aim of this study was to compare clinical characteristics between BONJ and IONJ. MATERIALS AND METHODS: Medical records at the Ajou University Hospital were reviewed using key words "osteonecrosis", "sequestrum", and "dead bone" of the mandible and maxilla. Eighteen patients diagnosed with ONJ were identified from January 2003 to December 2009. Using criteria from the American Association of Oral and Maxillofacial Surgeons, 12 cases were diagnosed as BONJ and six as IONJ. Clinical characteristics and treatment modalities, outcomes of two groups of patients were investigated. RESULTS: Patients of the BONJ group were older than those of IONJ group (median: 76.5 years vs. 60 years). In the BONJ group, mandible was more commonly involved sites and percentage of diabetic patients was higher. Three of 12 (25%) BONJ patients received oral glucocorticoids, while none of the IONJ patients. Ten (83.3%) of the BONJ cases and 2 (33.3%) of the IONJ cases were triggered by dental extractions. Though duration of ONJ treatment was longer in the BONJ group than the IONJ group (6.4+/-5.2 months vs. 2.9+/-1.9 months), both groups eventually showed good prognosis. CONCLUSIONS: ONJ may occur in the absence of bisphosphonate. BONJ occurred in older patients, and needed longer treatment duration compared to IONJ. Prognosis of ONJ was relatively good.

The Relationship between Diabetes Mellitus and Health-Related Quality of Life in Korean Adults: The Fourth Korea National Health and Nutrition Examination Survey (2007-2009)

BACKGROUND: Diabetes is a major health problem in Korea. However, interest in the quality of life in patients with diabetes is low. We examined the effects of diabetes on health-related quality of life (HRQoL) and compared it with HRQoL in the general Korean population using the Fourth Korea National Health and Nutrition Examination Survey (KNHANES IV) (2007-2009). METHODS: Using KNHANES IV data, we compared EuroQol (EQ)-5D and EQ-visual analogue scale (VAS) scores after adjusting for sociodemographic and psychosocial factors as well as for comorbidities (hypertension, heart disease, stroke, arthritis, and chronic renal disease). Logistic regressions were used to explore determinants for the lowest quintile HRQoL scales in the diabetes group. RESULTS: The mean age of the 14,441 enrolled subjects (6,129 men and 8,312 women) was 52.5+/-14.5 years. The mean EQ-5D and EQ-VAS scores were significantly lower in the diabetes group (EQ-5D. 0.87; EQ-VAS, 71.94) than in the non-diabetes group (EQ-5D, 0.94; EQ-VAS, 77.40) (P<0.001). Self-reported depressive symptom had a significant effect on lowering the EQ-VAS (odds ratio [OR], 1.7; 95% confidence interval [CI], 1.1 to 2.6) in the diabetes group. Stress level had a significant effect in lowering both the EQ-5D (OR, 2.0; 95% CI, 1.3 to 2.9) and the EQ-VAS (OR, 1.9; 95% CI, 1.3 to 2.9). HbA1c, diabetes duration, and treatment modalities had no significant effect on lowering HRQoL. CONCLUSION: Diabetes was clearly associated with impaired HRQoL compared with the non-diabetic population regardless of comorbidities. Therapeutic approaches should focus much more on the subjective perception of health in patients with diabetes.

Vancomycin-Associated Spontaneous Cutaneous Adverse Drug Reactions

PURPOSE: With the increase in vancomycin use, adverse drug reactions (ADRs) associated with vancomycin have been reported increasingly more often. However, the characteristics of cutaneous ADRs with and without systemic reactions (SRs) have not been described. This study investigated the characteristics of spontaneously reported and assessed ADRs associated with vancomycin by a pharmacovigilance center. METHODS: ADRs (n=121) associated with vancomycin in 96 patients were collected from 2008 to 2009. Records from physician- and nurse-reported suspected cases of vancomycin ADRs, ADR type, latent period, and laboratory results were compared between cutaneous ADRs with and without SRs. RESULTS: The main vancomycin-related ADRs were skin rashes (47.9%), hematologic abnormalities (17.36%), fever (12.4%), and elevated serum creatinine (12.4%). Significant differences were observed in latent period (days) and the mean change in eosinophils (%) between cutaneous (9.21+/-9.71 and 1.4+/-3.4, respectively) and other ADRs (14.03+/-11.71 and -0.5+/-3.5, respectively). Twelve cases of cutaneous ADRs with SRs had been initially reported as cutaneous ADRs only. Mean changes in the eosinophil count were significantly higher for cutaneous ADRs with SRs compared to those without SRs. CONCLUSIONS: Skin rashes accompanied by peripheral eosinophilia, representing suspected immune-mediated delayed hypersensitivity reactions, are a common vancomycin ADR. For the early and exact detection of ADRs associated with vancomycin administration, close monitoring of laboratory tests, including complete blood counts with differential analysis, is recommended.

A Case Report of Hajdu-Cheney Syndrome

Hajdu-Cheney syndrome (HCS) is a rare skeletal dysplasia that is characterized by acroosteolysis of the distal phalanges, distinctive craniofacial and skull changes, dental abnormalities and generalized osteoporosis. The clinical and radiologic characteristics are variable and these characteristics progress with age. This syndrome shows autosomal dominant inheritance with sporadic cases. The genetic defects or molecular pathogenesis of HCS are still unknown. We experienced a case of Hajdu-Cheney syndrome in a 20-year-old man who had generalized osteoporosis with multiple non-traumatic spine compression fractures. He had acroosteolysis of the hands and feet, wormian bones in the skull, facial dysmorphism (mid-facial flattening, micrognathia and bushy eyebrows), a high arched palate, malocclusion and short dental alveolar processes. HCS was diagnosed based on the clinical and radiologic evidence. For the differential diagnosis, we excluded the other possible causes of the acroosteolysis and wormian bones, including hyperparathyroidism, osteogenesis imperfecta, hypophosphatemia and mandibuloacral dysplasia. The specific treatment of HCS is unknown, but case reports with bisphosphonate treatment have been reported.

Prediction of compensated liver cirrhosis by ultrasonography and routine blood tests in patients with chronic viral hepatitis / 대한간학회지

BACKGROUND/AIMS: Liver biopsy is a standard method for diagnosis of liver cirrhosis in patients with chronic hepatitis. Because liver biopsy is an invasive method, non-invasive methods have been used for diagnosis of compensated liver cirrhosis in patients with chronic hepatitis. The current study was designed to evaluate the usefulness of ultrasonography and routine blood tests for diagnosis of compensated liver cirrhosis in patients with chronic viral hepatitis. METHODS: Two hundred three patients with chronic viral hepatitis who underwent liver biopsy were included in this study and ultrasonography and routine blood tests were analyzed retrospectively. Ultrasonographic findings, including surface nodularity, parenchyma echogenecity, and spleen size, were evaluated. The diagnostic accuracy of ultrasonography and routine blood tests were examined. RESULTS: Discriminant analysis with forward stepwise selection of variables showed that liver surface nodularity, platelet count, and albumin level were independently associated with compensated liver cirrhosis (p95% specificity: platelet count 1.3; and surface nodularity. If at least one of the four variables exists in a patient with chronic viral hepatitis, we can predict liver cirrhosis with 90% specificity and 61% sensitivity. CONCLUSIONS: These results suggest that four variables (platelet count 1.3, and surface nodularity) can be used for identification of liver cirrhosis in patients with chronic viral hepatitis with high specificity.