We analyzed the mRNA expression patterns of major potassium channel genes to determine the mechanism of hypokalemia in familial hypokalemic periodic paralysis. We used quantitative RT-PCR to examine the mRNA levels of both inward (KCNJ2, KCNJ6, and KCNJ14) and delayed rectifi er (KCNQ1 and KCNA2) potassium channel genes in skeletal muscle cells from both normal and patient groups, prior to and after exposure to 4 mM and 50 mM potassium buffers. Quantitative RT-PCR analysis revealed no changes in the mRNA levels of these genes in normal and patient cells on exposure to 4 mM potassium buffer. However, after exposure to 50 mM potassium buffer, which was used to induce depolarization, normal cells showed a signifi cant decrease in KCNJ2, KCNJ6, and KCNJ14 expression, but no change in KCNQ1 and KCNA2 expression. In contrast, patient cells showed no change in KCNJ2 and KCNJ6 expression, but an increase in KCNJ14 expression. Furthermore, KCNQ1 and KCNA2 showed decreased expression. We found that the expression levels of both inward and delayed rectifi er potassium channel genes in patient cells differ from those in normal cells. Altered potassium channel gene expression in patient cells may suggest a possible mechanism for hypokalemia in familial hypokalemic periodic paralysis.

Unilateral adsence of a pulmonary artery is frequently undiagnosed. Unless this entity is recognized, a patient with a potentially curable lesion may become inoperable. The importance of considering unilateral absence of a pulmonary artery in the diffirential diagnosis of cyanotic congenital heart disease and pulmonary hypertension has become clear. We exeperienced a case of congenital isolated unilateral absence of it. pulmonary artery confirmed by clinical features, lung scan, echocardiogram and angiogram.
Diagnosis ; Heart Defects, Congenital ; Humans ; Hypertension, Pulmonary ; Lung ; Pulmonary Artery*

Animals ; Bone Marrow ; Bone Regeneration ; Bone Transplantation ; Cartilage ; Cell Culture Techniques ; Fibrin Tissue Adhesive ; Lifting ; Mesenchymal Stromal Cells ; Osteogenesis ; Rabbits ; Stem Cells ; Tendons ; Tissue Engineering ; Transplants

No abstract available.
Cardiomyopathies* ; Peripartum Period*

In the spring of 2011, a cluster of lung injuries caused by humidifier disinfectant (HD) usage were reported in Korea. Many patients required mechanical ventilation, extracorporeal membrane oxygenation, and even lung transplantation (LTPL). However, the long-term course of HD-associated lung injury remains unclear because the majority of survivors recovered normal lung function. Here we report a 33-year-old woman who underwent LTPL approximately four years after severe HD-associated lung injury. The patient was initially admitted to the intensive care unit and was supported by a high-flow nasal cannula. Although she had been discharged, she was recurrently admitted to our hospital due to progressive lung fibrosis and a persistent decline in lung function. Finally, sequential double LTPL was successfully performed, and the patient's clinical and radiological findings showed significant improvement. Therefore, we conclude that LTPL can be a therapeutic option for patients with chronic inhalation injury.
Adult ; Catheters ; Disinfectants ; Extracorporeal Membrane Oxygenation ; Female ; Fibrosis ; Humans ; Humidifiers* ; Inhalation ; Inhalation Exposure ; Intensive Care Units ; Korea ; Lung Injury* ; Lung Transplantation* ; Lung* ; Oxygen Inhalation Therapy ; Respiration, Artificial ; Survivors

PURPOSE: Familial hypokalemic periodic paralysis (HOKPP) is an autosomal dominant channelopathy characterized by episodic attacks of muscle weakness and hypokalemia. Mutations in the calcium channel gene, CACNA1S, or the sodium channel gene, SCN4A, have been found to be responsible for HOKPP; however, the mechanism that causes hypokalemia remains to be determined. The aim of this study was to improve the understanding of this mechanism by investigating the expression of calcium-activated potassium (KCa) channel genes in HOKPP patients. METHODS: We measured the intracellular calcium concentration with fura-2-acetoxymethyl ester in skeletal muscle cells of HOKPP patients and healthy individuals. We examined the mRNA and protein expression of KCa channel genes (KCNMA1, KCNN1, KCNN2, KCNN3, and KCNN4) in both cell types. RESULTS: Patient cells exhibited higher cytosolic calcium levels than normal cells. Quantitative reverse transcription polymerase chain reaction analysis showed that the mRNA levels of the KCa channel genes did not significantly differ between patient and normal cells. However, western blot analysis showed that protein levels of the KCNMA1 gene, which encodes KCa1.1 channels (also called big potassium channels), were significantly lower in the membrane fraction and higher in the cytosolic fraction of patient cells than normal cells. When patient cells were exposed to 50 mM potassium buffer, which was used to induce depolarization, the altered subcellular distribution of BK channels remained unchanged. CONCLUSION: These findings suggest a novel mechanism for the development of hypokalemia and paralysis in HOKPP and demonstrate a connection between disease-associated mutations in calcium/sodium channels and pathogenic changes in nonmutant potassium channels.
Blotting, Western ; Calcium ; Calcium Channels ; Channelopathies ; Cytosol ; Humans ; Hypokalemia ; Hypokalemic Periodic Paralysis* ; Large-Conductance Calcium-Activated Potassium Channels ; Membranes ; Muscle Weakness ; Muscle, Skeletal ; Paralysis ; Polymerase Chain Reaction ; Potassium ; Potassium Channels ; Potassium Channels, Calcium-Activated* ; Reverse Transcription ; RNA, Messenger ; Sodium Channels

PURPOSE: We aimed to investigate the extent of heterogeneity in medical terminology between South and North Korea by comparing medical terms related to the colorectal system. METHODS: North Korean medical terms were collected from the sections on diseases of the small intestine and colon in a surgery textbook from North Korea, and those terms were compared with their corresponding terms in a South Korean medical terminology textbook. The terms were categorized as either identical, similar, showing disparity, or not used in South Korea. In a subsection analysis, the terms were allocated to pathophysiology, diagnosis, symptoms and examination, drugs, testing, treatment, or others according to the categorization used in the textbook. RESULTS: We found 705 terms in the North Korean textbook, most of which were pathophysiological terms (206, 29.2%), followed by diagnostic terms (165, 23.4%) and symptom and examination terms (122, 17.3%). Treatment-, drug-, and testing-related terms constituted 15.5%, 5.8%, and 4.1% of the 705 terms, respectively. There were 331 identical terms (47.0%) and 146 similar terms (20.7%); 126 terms (17.9%) showed disparity. Another 102 terms (14.5%) were not used in South Korea. The pathophysiological terms were the least heterogeneous, with 61.2% being identical terms used in both countries. However, 26.8% of the terms in the drug category were not used in South Korea. CONCLUSION: The present study showed that less than 50% of the terms for the colorectal system used in South and North Korea were identical. As the division between South and North Korea persists, the heterogeneity of medical terminology is expected to increase.
Colon ; Colorectal Surgery* ; Democratic People's Republic of Korea* ; Diagnosis ; Intestine, Small ; Intestines ; Korea ; Population Characteristics

Bacterial endocarditis has been well recognized as an important complication of congenital heart disease, such as ventricular septal defect, patent ductus arteriosus or pulmonary stenosis. The incidence of right sided bacterial endocarditis is lesser than left sided bacterial endocarditis. Also, pulmonic valve vegetation has been thought to be relatively uncommon. So in a patient with fever and evidence of recurrent pulmonary infarction, changing heart murmurs and scattered pneumonic infiltrates, one should direct attention to the heart as a possible source of the infection. Echocardiography with M-mode, 2-D and Doppler mode represents the only noninvasive technic available for detecting vegetations in bacterial endocarditis. In fact, the technic is more sensitive in identifying these lesions than angiography. We experienced a case of ventricular septal defect with bacterial endocarditis, pulmonic valve vegetation and multiple pulmonary embolism diagnosed with Echocardiogram and lung scan, and confirmed by operation. Patch repair of ventricular septal defect, resection of pulmonic valve and vegetation and artificial valve formation with pericardium were done.
Angiography ; Ductus Arteriosus, Patent ; Echocardiography ; Endocarditis, Bacterial* ; Fever ; Heart ; Heart Defects, Congenital ; Heart Murmurs ; Heart Septal Defects, Ventricular* ; Humans ; Incidence ; Lung ; Pericardium ; Pulmonary Embolism ; Pulmonary Infarction ; Pulmonary Valve Stenosis

Coarctation and occlusion of the aorta is a rare condition that typically presents with hypertension or cardiac failure. However, neuropathy or myelopathy may be the presenting features of the condition when an intraspinal subarachnoid hemorrhage has compressed the spinal cord causing ischemia. We report two cases of middle-aged males who developed acute non-traumatic paraplegia. Undiagnosed congenital abnormalities, such as aortic coarctation and occlusion, should be considered for patients presenting with nontraumatic paraplegia in the absence of other identifiable causes. Our cases suggest that spinal cord ischemia resulting from acute spinal subarachnoid hemorrhage and can cause paraplegia, and that clinicians must carefully examine patients presenting with nontraumatic paraplegia because misdiagnosis can delay initiation of the appropriate treatment.
Aorta ; Aortic Coarctation* ; Congenital Abnormalities ; Diagnostic Errors ; Heart Failure ; Humans ; Hypertension ; Ischemia ; Male ; Paraplegia* ; Spinal Cord ; Spinal Cord Diseases ; Spinal Cord Ischemia ; Subarachnoid Hemorrhage

The Bentall-DeBono operation is the technique of choice for aortic root replacement. When the patients have contraindications for lifelong anticoagulation, the biologic Bentall operation may be a good option. However, complex reoperation may be required when bioprosthetic degeneration occurs. For this reason, a new technique for simple reoperation after the Bentall operation has recently been performed by some surgeons. We performed a similar technique in two patients with aortic root dilation and for whom aortic valve sparing techniques could not be performed because of an unrepairable valve contour, we think this modification seems to be simple and reproducible for reoperation.
Aneurysm ; Aorta ; Aortic Valve ; Bioprosthesis ; Cardiovascular Diseases ; Humans ; Reoperation