PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver

Diagnostic hematology subcommitee of The Korean Association of Quality Assurance for Clinical Laboratory performed laboratory proficiency testing for blood cell count, cell morphology and coagulation tests in 2013. Four trials for blood cell count and cell morphology and 2 trials for coagulation tests were executed. Average 1,308, 494, and 558 laboratories participated in the surveys of blood cell count, cell morphology and coagulation tests, respectively. The overall reply rates were 95.78%, 97.75%, and 97.38%, respectively. The homogeneity of external quality materials was stable (less than 3%) and status of use of the instrument and reagents was similar to those of the previous year. The CVs in white blood cell count, red blood cell count, platelet count, hemoglobin, and hematocrit were 3.15%, 2.00%, 5.10%, 1.81%, and 2.71%, respectively. For cell morphology, most showed concordant rate >80%. CVs of coagulation tests showed difference depending on instruments or reagent groups. An educational workshop on hands-on experience in diagnostic hematology was held in July. In 2013, the number of participating laboratories is more increased and the performance of surveys of hematology tests is similar performance compared to previous year. In addition, the revision in the way of evaluation of coagulation tests is needed.
Blood Cell Count ; Education ; Erythrocyte Count ; Hematocrit ; Hematology* ; Indicators and Reagents ; Korea ; Laboratory Proficiency Testing ; Leukocyte Count ; Partial Thromboplastin Time ; Platelet Count ; Prothrombin Time

No abstract available.

During 2015, the Diagnostic Hematology Subcommittee of Korean Association of External Quality Assessment Service performed laboratory proficiency testing for blood cell count, cell morphology, and coagulation tests. Four trials for blood cell count and cell morphology tests each and two trials for coagulation tests were performed. The trials for blood cell counts had a reply rate of 97.2% among 1,352 laboratories, compared to 99.0% among 503 laboratories for cell morphology and 98.6% among 574 laboratories for coagulation tests. The homogeneity of the external quality materials was stable (<3%), and the use of instruments and reagents was similar to that observed during the previous year. The coefficients of variation (CVs) for white blood cell counts, red blood cell counts, platelet counts, hemoglobin tests, and hematocrit tests were 4.13%, 1.89%, 1.92%, 5.02%, and 8.10%, respectively. For cell morphology tests, concordant rates were >80% for most of the participating laboratories. The CVs for the coagulation tests varied according to the specific instruments or reagents that were used. An educational workshop was held in November to provide hands-on experience in diagnostic hematology. During 2015, the number of participating laboratories increased, while the performance of hematology tests was similar to that observed in the previous year.
Blood Cell Count ; Education ; Erythrocyte Count ; Hematocrit ; Hematology* ; Indicators and Reagents ; Korea* ; Laboratory Proficiency Testing ; Leukocyte Count ; Partial Thromboplastin Time ; Platelet Count ; Prothrombin Time

During 2014, the Diagnostic Hematology Subcommittee of the Korean Association of Quality Assurance for Clinical Laboratories performed laboratory proficiency testing for blood cell count, cell morphology, and coagulation tests. Four trials for blood cell count and cell morphology tests and 2 trials for coagulation tests were performed. The trials for blood cell counts had a reply rate of 96.8% among 1,343 laboratories, compared to 99.3% among 489 laboratories for cell morphology and 98.6% among 565 laboratories for coagulation tests. The homogeneity of the external quality materials was stable (<3%), and the use of instruments and reagents was similar to that observed during the previous year. The CVs for white blood cell counts, red blood cell counts, platelet counts, hemoglobin tests, and hematocrit tests were 4.46%, 2.12%, 2.21%, 5.08%, and 8.31%, respectively. For cell morphology tests, concordant rates were >80% for most of the participating laboratories. The CVs for the coagulation tests varied according to the specific instruments or reagents that were used. An educational workshop was held in July to provide hands-on experience in diagnostic hematology. During 2014, the number of participating laboratories was increased, while the performance of hematology tests was similar to that observed in the previous year.
Blood Cell Count ; Education ; Erythrocyte Count ; Hematocrit ; Hematology* ; Indicators and Reagents ; Korea ; Laboratory Proficiency Testing ; Leukocyte Count ; Partial Thromboplastin Time ; Platelet Count ; Prothrombin Time

Primary fetal hydrothorax presents a wide spectrum of severity ranging from small harmless effusions to life-threatening thoracic compression. Management of this condition is controversial. We experienced a case of non-chylous primary fetal hydrothorax not associated with hydrops or other malformations. A predominantly unilateral hydrothorax in a fetus was diagnosed by sonography at 31 weeks gestation. Repeated ultrasonographic examinations to detect development of hydrops or progression of intrapleural effusion were used to evaluate the need for intrauterine thoracentesis and to decide the optimal time for delivery. Two thoracenteses were performed. The baby was delivered at 34 weeks. Postnatal intubation was carried out in the baby, and then chest tube placement was performed and resulted in the collection of 805ml of serous fluid. Sustained intubation was necessary for 16 days because of developing respiratory distress as a result of prematurity and recurrent pleural fluid accumulation. The baby did well after postnatal evacuation of the intrapleural fluid. Pre and postnatal investigation to find the pathophysiological mechanism leading to hydrothorax were negative. We conclude that non-chylous primary fetal hydrothorax is generally associated with a favorable outcome.
Chest Tubes ; Edema ; Fetus ; Hydrothorax ; Intubation ; Pregnancy

Sarcoidosis is a multisystem granulomatous disorder commonly affecting young adults. Diagnosis is confirmed by evidence of non-caseating granuloma in more than one organ. A case is presented where the diagnosis was made on a bone marrow biopsy, the first case in Korea. A 54-year-housewife was admitted because of multiple variable sized, skin papules and macules, who showed pancytopenia and evidence of liver cirrhosis on ultrasound. We performed skin biopsy and bone marrow biopsy, which showed non-caseating granulomas. The chest CT, opthalmologic examination was performed and showed the involvement of sarcoidosis of mediastinal lymph nodes and uvea. Multiple joints of both hands and feet also seemed to be involved in view of simple X-ray. By this case, we emphasize the significance of bone marrow biopsy in the sarcoidosis with pancytopenia.
Biopsy ; Bone Marrow* ; Diagnosis ; Foot ; Granuloma ; Hand ; Humans ; Joints* ; Korea ; Liver Cirrhosis ; Liver* ; Lymph Nodes ; Pancytopenia ; Sarcoidosis* ; Skin* ; Tomography, X-Ray Computed ; Ultrasonography ; Uvea* ; Young Adult

Hepatopulmonary syndrome (HPS) is characterized by a defect in arterial oxygenation that's induced by pulmonary vascular dilatation in the setting of liver disease. Some studies have shown the relationship between the presence of the HPS and the severity of liver disease, but there are only rare cases of HPS inpatient with Child-Pugh class A liver cirrhosis. We report here on a case of a 58 years-old male who suffered from progressive dyspnea for the previous few years. He was diagnosed with alcoholic liver cirrhosis 5 years previously. There was no significant abnormality on the chest radiograph and transthoracic echocardiography, but the arterial blood gas analysis revealed severe hypoxemia. Contrast-enhanced transesophageal echocardiograpy with agitated saline demonstrated a delayed appearance of microbubbles in the left cardiac chambers. Thus, he was finally diagnosed with HPS. This case suggests that we should consider HPS when a patient with compensated liver cirrhosis has unexplained dyspnea.
Anoxia ; Blood Gas Analysis ; Dihydroergotamine ; Dilatation ; Dyspnea ; Echocardiography ; Hepatopulmonary Syndrome ; Humans ; Inpatients ; Liver ; Liver Cirrhosis ; Liver Cirrhosis, Alcoholic ; Liver Diseases ; Liver Transplantation ; Male ; Microbubbles ; Oxygen ; Thorax

Necrotizing sarcoid granulomatosis was first described by Liebow, who included it in the category of pulmonary angiitis and granulomatosis. Wegener's granulomatosis and limited Wegener's granulomatosis are also included in this category. Necrotizing sarcoid granulomatosis is characterized by histologically sarcoid-like granulomata, prominent and granulomatous vasculitis, and varying degrees of necrosis, radiographically pulmonary nodules but no enlarged hilar lymph nodes, clinically benign course. Whether this entity is a variant of necrotizig vasculitis with sarcoid reaction or sarcoidosis with prominent vascultic features and necrosis is not yet, clear, but many authors suggest the relationship with sarcoidosis from the observation of the similarity of histologic features and clinical course between the two entities. Patients are asymptomatic in about one fourth. Even symptomatic patients have vague symptoms such as cough, chest pain. Extrapulmonary involvement is rare. The prognosis is good even without therapy or with steroid alone, if necessary. We report a case that was diagnosed by open lung biopsy as necrotizing sarcoid granulomatosis, and it is assumed to be the first report of this entity in Korea.
Biopsy ; Chest Pain ; Cough ; Humans ; Korea ; Lung ; Lymph Nodes ; Necrosis ; Prognosis ; Sarcoidosis ; Vasculitis ; Wegener Granulomatosis

The majority of cardiac myxoma occur sporadically as isolated lesions in the left atrium of middle-aged women. However, a familial form and a syndrome form of this lesion have also been identified. The syndrome myxoma can present itself with pigmented skin lesions and peripheral or endocrine neoplasms. The familial and syndrome forms of cardiac myxomas can usually be distinguished from the sporadic form by their occurrences at younger ages, their unusual locations, the multicentricity of the lesions, and the presence of rare pathological conditions. In addition, a higher rate of recurrent lesions is usually associated with the familial and syndrome forms of this disease. We present a case of complex cardiac myxoma with pigmented skin lesions and breast myxoma and report it with a review of literature.
Breast ; Female ; Heart Atria ; Humans ; Myxoma* ; Nipples* ; Skin