Paroxysmal nocturnal hemoglobinuria: Kidney biopsy and magnetic resonance imaging

No abstract available.

Is Omalizumab a Problem-Solving Remedy in Severe Asthma?

No abstract available.

Methazolamide-induced toxic epidermal necrolysis confirmed by lymphocyte activation test

Among various dermatological entities, toxic epidermal necrolysis (TEN) is a rare but potentially fatal delayed hypersensitivity reaction to numerous medications. A 38-year-old male presented with systemic hypersensitivity reaction, such as high fever, pain in the eyes, and diffuse pruritic erythematous maculopapular eruptions with multiple targetoid plaques that became vesicular and bullous. Oral mucosa and conjunctivae were involved. The first sign appeared about 1 week after taking methazolamide (50 mg twice a day) for the management of glaucomatous eyes. Although methazolamide was discontinued, blistering and skin denudation progressed to affect up to 80% of the body surface area and a positive Nikolsky sign was noted. High fever also persisted. Skin lesions started to improve after 2 weeks of management and fever subsided. Cutaneous lesions improved with minimal permanent sequele 2 months later. HLA-B*5901 was found by high-resolution genotyping. The lymphocyte activation test performed 6 months after remission showed a positive response to methazolamide challenge. This is the first case of methazolamide-induced TEN in which methazolamide was confirmed as a culprit drug by the lymphocyte activation test.

Experience with Elizabethkingia meningoseptica Infection in Adult Patients at a Tertiary Hospital / 대한구급학회지

BACKGROUND: Few reports have documented the clinical characteristics and treatment outcomes of adult patients with Elizabethkingia meningoseptica infection. METHODS: Medical records of patients over 18 years of age and suspected of having an E. meningoseptica infection from March 1, 2006 to February 28, 2013 were reviewed retrospectively. Their clinical characteristics, antimicrobial susceptibility results, and treatment outcomes were analyzed. RESULTS: E. meningoseptica was isolated from 30 patients. Median age was 68.5 years, and infections were more frequent in males (17, 56.7%). The most common isolation source was sputum (23, 76.7%), and pneumonia was the most common condition (21, 70%) after excluding two cases of colonization. This bacterium was most susceptible to minocycline (27, 90%) and fluoroquinolones, including levofloxacin (20, 66.7%) and ciprofloxacin (18, 60%). The mortality rate due directly to E. meningoseptica infection was 20% (6/30), and uncontrolled pneumonia was the only cause of death. After isolating E. meningoseptica, the numbers of patients with pneumonia (9/9, 100% vs. 12/21, 57.1%), history of hemodialysis (5/9, 55.6% vs. 3/21, 14.3%), tracheostomy (8/9, 88.9 vs. 10/21, 47.6%), and median Charlson comorbidity index score (6 [range, 3-9] vs. 4 [range, 0-9]) were significantly higher in non-survivors than those in survivors (p < 0.05, for each). However, only 12 (40%) patients received appropriate antibiotics. CONCLUSIONS: E. meningoseptica infection most commonly presented as pneumonia in adults with severe underlying diseases. Despite the high mortality rate, the rate of appropriate antibiotic use was notably low.

Experience with Elizabethkingia meningoseptica Infection in Adult Patients at a Tertiary Hospital / 대한중환자의학회지

BACKGROUND: Few reports have documented the clinical characteristics and treatment outcomes of adult patients with Elizabethkingia meningoseptica infection. METHODS: Medical records of patients over 18 years of age and suspected of having an E. meningoseptica infection from March 1, 2006 to February 28, 2013 were reviewed retrospectively. Their clinical characteristics, antimicrobial susceptibility results, and treatment outcomes were analyzed. RESULTS: E. meningoseptica was isolated from 30 patients. Median age was 68.5 years, and infections were more frequent in males (17, 56.7%). The most common isolation source was sputum (23, 76.7%), and pneumonia was the most common condition (21, 70%) after excluding two cases of colonization. This bacterium was most susceptible to minocycline (27, 90%) and fluoroquinolones, including levofloxacin (20, 66.7%) and ciprofloxacin (18, 60%). The mortality rate due directly to E. meningoseptica infection was 20% (6/30), and uncontrolled pneumonia was the only cause of death. After isolating E. meningoseptica, the numbers of patients with pneumonia (9/9, 100% vs. 12/21, 57.1%), history of hemodialysis (5/9, 55.6% vs. 3/21, 14.3%), tracheostomy (8/9, 88.9 vs. 10/21, 47.6%), and median Charlson comorbidity index score (6 [range, 3-9] vs. 4 [range, 0-9]) were significantly higher in non-survivors than those in survivors (p < 0.05, for each). However, only 12 (40%) patients received appropriate antibiotics. CONCLUSIONS: E. meningoseptica infection most commonly presented as pneumonia in adults with severe underlying diseases. Despite the high mortality rate, the rate of appropriate antibiotic use was notably low.

Incidence of and Risk Ractors for Zolpidem-Induced Delirium / 대한내과학회지

BACKGROUND/AIMS: Zolpidem is a safe and effective drug for the treatment of insomnia. However, there are some reports of adverse effects, such as delirium, after administration of zolpidem. The aim of this study was to evaluate the incidence of and risk factors for zolpidem-induced delirium. METHODS: This retrospective study enrolled 481 patients who were admitted to hospital and received zolpidem between January and May 2011. We analyzed the incidence and risk factors associated with zolpidem-induced delirium. RESULTS: Zolpidem-induced delirium occurred in 19 of 481 (4.0%) patients. Zolpidem-induced delirium was significantly associated with old age (> or = 65 years; odds ratio [OR] = 4.35, 95% confidence interval [CI] = 1.52-12.44, p = 0.006) and co-administration of benzodiazepine (OR = 4.30, 95% CI = 1.52-12.12, p = 0.006). When males > 65 years-old took both benzodiazepine and zolpidem simultaneously, the incidence of delirium was notably elevated (OR = 6.04, 95% CI = 1.80-20.20, p = 0.003). Other factors, including dosage, did not influence the occurrence of delirium. CONCLUSIONS: Old age and co-administration of benzodiazepine were independent risk factors for zolpidem-induced delirium. Therefore, a detailed medical history should be taken before prescribing zolpidem to an older person, and zolpidem should be used cautiously, with careful monitoring, in these patients.

An Unusual Case of Superior Vena Cava Syndrome Caused by the Intravascular Invasion of an Invasive Thymoma / 결핵및호흡기질환

Superior vena cava syndrome (SVCS) is usually caused by extrinsic compression or invasion of the superior vena cava (SVC) by malignant tumors involving mediastinal structures. Although thymomas are well-known causes of SVCS, cases of SVCS caused by malignant thymomas protruding into adjacent vessels draining the SVC with thrombosis have been very rarely reported worldwide. We experienced a 39-year-old female patient with SVCS that developed after the direct invasion of the left brachiocephalic vein (LBCV) and SVC by an anterior mediastinal mass with a high maximum standardized uptake value on the chest computed tomography (CT) and positron emission tomography-CT. Based on these results, she underwent en bloc resection of the tumor, including removal of the involved vessels, and was eventually diagnosed as having a type B2 thymoma permeating into the LBCV and SVC. We present this case as a very rare form of SVCS caused by an invasive thymoma.

Everolimus-Induced Interstitial Pneumonitis in a Patient with Metastatic Renal Cell Carcinoma: a Case Report / 대한내과학회지

Everolimus, an inhibitor of the mammalian target of rapamycin, is an active agent against metastatic renal cell carcinoma. Treatment with everolimus prolongs progression-free survival in patients with clear cell-type renal cell carcinoma that has progressed on vascular endothelial growth factor receptor tyrosine kinase inhibitors, such as sunitinib and/or sorafenib. Everolimus-induced interstitial pneumonitis is not rare and is sometimes fatal. Due to the potential for pulmonary toxicity due to everolimus, it is recommended that pulmonary complications be periodically evaluated. We report a case of everolimus-associated interstitial pneumonitis in a patient with metastatic renal cell carcinoma.

A Case of Interstitial Pneumonitis and Pancytopenia Following the Combination Therapy of Pegylated Interferon and Ribavirin / 결핵및호흡기질환

The combination therapy of pegylated interferon and ribavirin is the mainstay of treatment for chronic hepatitis C patients. Anti-viral therapy is commonly associated with side effects such as headache, fever, myalgia, and arthralgia. However, anti-viral therapy can continue because these side effects are mostly mild and can be improved with supportive management. Anti-viral therapy should be stopped promptly if serious side effects, such as interstitial pneumonitis or hemolytic anemia occur, although those serious side effects are rare. There were a few case reports of interferon-related interstitial pneumonitis worldwide. In Korea, one atypical case report of interstitial pneumonitis has been reported, which followed the combination therapy of interferon-alpha and ribavirin in a patient with chronic hepatitis C. We present a case of interstitial pneumonitis and pancytopenia following the combination therapy of pegylated interferon and ribavirin in a patient with chronic hepatitis C.

A Case of Interstitial Pneumonitis and Pancytopenia Following the Combination Therapy of Pegylated Interferon and Ribavirin / 결핵및호흡기질환

The combination therapy of pegylated interferon and ribavirin is the mainstay of treatment for chronic hepatitis C patients. Anti-viral therapy is commonly associated with side effects such as headache, fever, myalgia, and arthralgia. However, anti-viral therapy can continue because these side effects are mostly mild and can be improved with supportive management. Anti-viral therapy should be stopped promptly if serious side effects, such as interstitial pneumonitis or hemolytic anemia occur, although those serious side effects are rare. There were a few case reports of interferon-related interstitial pneumonitis worldwide. In Korea, one atypical case report of interstitial pneumonitis has been reported, which followed the combination therapy of interferon-alpha and ribavirin in a patient with chronic hepatitis C. We present a case of interstitial pneumonitis and pancytopenia following the combination therapy of pegylated interferon and ribavirin in a patient with chronic hepatitis C.

Endobronchial Stent Insertion to Manage Hemoptysis caused by Lung Cancer

Hemoptysis in patients with lung cancer is not uncommon and sometimes have dangerous consequences. Hemoptysis has been managed with various treatment options other than surgery and medicine, such as endobronchial tamponade, transcatheter arterial embolization and radiation therapy. However, these methods can sometimes be used only temporarily or are not suitable for a patient's condition. We present a case in which uncontrollable hemoptysis caused by central lung cancer was successfully treated by inserting a covered self-expanding bronchial stent. The patient could be extubated and was able to undergo further palliative therapy. No recurrent episodes of hemoptysis occurred for the following three months. As our case, airway stenting is a considerable option for the tamponade of a bleeding lesion that cannot be successfully managed with other treatment methods and could be used to preserve airway patency in a select group of patients.

A Case of Hypersensitivity Pneumonitis Following Placenta Extract Injection / 결핵및호흡기질환

Human placenta contains various kinds of nutritional elements essential for embryonic development. Currently, human placenta extracts are widely overused in Korea to improve certain health conditions (postmenopausal syndrome, liver function, and cosmetic purposes) without scientific evidence that they actually work. The use of placenta extracts should be restricted, due to a lack of systematic research on the therapeutic effectiveness and adverse results from these treatments. While the common adverse effects that have been reported are fever, rash, itching, nausea, vomiting, breast pain, and rare cases of anaphylactic shock, there have been no reports of pulmonary complications such as hypersensitivity pneumonitis. Recently, we experienced a patient with hypersensitivity pneumonitis following a placenta extract injection. To our knowledge, this is the first case of hypersensitivity pneumonitis associated with placenta extract use.

Repression of TNF-alpha-induced IL-8 expression by the glucocorticoid receptor-beta involves inhibition of histone H4 acetylation

Increased expression of a number of proinflammatory genes, including IL-8, is associated with inflammatory conditions such as asthma. Glucocorticoid receptor (GR)beta, one of the GR isoforms, has been suggested to be upregulated in asthma associated with glucocorticoid insensitivity and to work as a dominant negative inhibitor of wild type GRalpha. However, recent data suggest that GRbeta is not a dominant negative inhibitor of GRalpha in the transrepressive process and has its own functional role. We investigated the functional role of GRbeta expression in the suppressive effect of glucocorticoids on tumor necrosis factor (TNF)-alpha-induced IL-8 release in an airway epithelial cell line. GRbeta expression was induced by treatment of epithelial cells with either dexamethasone or TNF-alpha. GRbeta was able to inhibit glucocorticoid-induced transcriptional activation mediated by binding to glucocorticoid response elements (GREs). The suppressive effect of dexamethasone on TNF-alpha-induced IL-8 transcription was not affected by GRbeta overexpression, rather GRbeta had its own weak suppressive activity on TNF-alpha-induced IL-8 expression. Overall histone deacetylase activity and histone acetyltransferase activity were not changed by GRbeta overexpression, but TNF-alpha-induced histone H4 acetylation at the IL-8 promoter was decreased with GRbeta overexpression. This study suggests that GRbeta overexpression does not affect glucocorticoid-induced suppression of IL-8 expression in airway epithelial cells and GRbeta induces its own histone deacetylase activity around IL-8 promoter site.

Repression of TNF-alpha-induced IL-8 expression by the glucocorticoid receptor-beta involves inhibition of histone H4 acetylation

Increased expression of a number of proinflammatory genes, including IL-8, is associated with inflammatory conditions such as asthma. Glucocorticoid receptor (GR)beta, one of the GR isoforms, has been suggested to be upregulated in asthma associated with glucocorticoid insensitivity and to work as a dominant negative inhibitor of wild type GRalpha. However, recent data suggest that GRbeta is not a dominant negative inhibitor of GRalpha in the transrepressive process and has its own functional role. We investigated the functional role of GRbeta expression in the suppressive effect of glucocorticoids on tumor necrosis factor (TNF)-alpha-induced IL-8 release in an airway epithelial cell line. GRbeta expression was induced by treatment of epithelial cells with either dexamethasone or TNF-alpha. GRbeta was able to inhibit glucocorticoid-induced transcriptional activation mediated by binding to glucocorticoid response elements (GREs). The suppressive effect of dexamethasone on TNF-alpha-induced IL-8 transcription was not affected by GRbeta overexpression, rather GRbeta had its own weak suppressive activity on TNF-alpha-induced IL-8 expression. Overall histone deacetylase activity and histone acetyltransferase activity were not changed by GRbeta overexpression, but TNF-alpha-induced histone H4 acetylation at the IL-8 promoter was decreased with GRbeta overexpression. This study suggests that GRbeta overexpression does not affect glucocorticoid-induced suppression of IL-8 expression in airway epithelial cells and GRbeta induces its own histone deacetylase activity around IL-8 promoter site.

A Case of Isolated Congenital Absence of Right Pulmonary Artery / 결핵및호흡기질환

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

A Case of Wernicke's Encephalopathy Occurring in a Patient with Aspiration Pneumonia: A Case Report / 대한구급학회지

Wernicke's encephalopathy is a neurologic complication of thiamine deficiency, presenting with acute confusion, oculomotor dysfunction, and gait ataxia. While most often associated with chronic alcoholism, Wernicke's encephalopathy occasionally occurs in the setting of poor nutritional status, such as malabsorption, increased metabolic requirements, or increased loss of the water-soluble vitamins. Patients with critical illnesses can present with excessive catabolic status because of activation of the sympathetic nervous system and the pituitary-adrenal axis. In addition, inappropriate nutritional evaluation and lack of concerns for adequate nutrient support can increase the morbidity and mortality in such patients. However, the importance of adequate nutritional support is often disregarded during treatment of the patient's primary illness. We have recently managed a patient with Wernicke's encephalopathy and pneumonia who did not receive adequate nutritional support during hospitalization. We report this case to call attention to the importance of nutritional support in critically ill patients.

A Case of Pulmonary Trichomoniasis in a Young Healthy Male / 결핵및호흡기질환

Bronchopulmonary infections caused by trichomonads have been reported principally in patients with pre-existing pulmonary diseases, such as bronchial carcinoma, lung abscess, or bronchiectasis. Pulmonary trichomoniasis is most often caused by Trichomonas tenax, which is usually regarded as a harmless commensal organism of the human mouth. However, pulmonary infection may rarely be caused by other trichomonas species, including Trichomonas vaginalis from the genitourinary tract and Trichomonas hominis from the intestines. Because of the rarity of trichomonas pulmonary infection, and because of its close association with underlying lung and systemic disease, pulmonary trichomoniasis is considered an opportunistic infection. We recently treated a case of pulmonary trichomoniasis occurring in a young, healthy male without obvious underlying pulmonary or systemic illness. To our knowledge, there has been only one reported case of pulmonary trichomoniasis in Korea, and there have been only two reported cases of pulmonary trichomoniasis occurring in normal lung worldwide.

The Effects of On-site Measured Ozone Concentration on Pulmonary Function and Symptoms of Asthmatics

Most studies on the effects of ambient ozone on asthmatics have been based on ozone concentration measurements taken by air monitors in downtown areas. Using a passive ozone sampler, we investigated the effects of on-site ozone concentrations on the pulmonary function and symptoms of asthmatics. Twenty moderate to severe asthmatics who had been managed for at least 2 months without changes of their medication were enrolled from 3 June to 18 July 2005. Respiratory, nasal and ocular symptoms, peak expiratory flow (PEF), which was measured twice a day, and medication use were recorded on a daily basis during the study period. Data for 17 subjects were analyzed. The average ozone exposure level was 28.2+/-23.6 ppb (3.4-315.3 ppb). There was no significant correlation between PEF and ozone concentration (p>0.05) on the same day or 1-, 2-, or 3-day lags. Interestingly, the degree of asthma symptoms was influenced by the ozone concentration (rho=0.303, p<0.001), even at concentrations less than 80 ppb (p=0.298, p<0.001), but the correlation between ozone exposure and the frequency of reliever medication use was not statistically significant (p=0.99). Our results suggest that exposure to relatively low concentrations of ozone influences the symptoms of moderate to severe asthmatics regardless of changes in pulmonary function or medication use.

Clinical Characteristics of Tuberculosis in North Korean Refugees / 결핵및호흡기질환

BACKGROUND: North Korea's economic and public health problems began in the early 1990s as a result of the gradual loss of economic support from its communist allies, combined with an inordinate number of natural disasters. The decline in public health has increased the incidence of tuberculosis in North Koreans and refugees. This study investigated tuberculosis situation in North Korean refugees in order to prepare for the future impact of tuberculosis control in Korea. MATERIAL AND METHODS: From 2001 to 2005, tuberculosis patients among North Korean refugees who were diagnosed before or after arriving in South Korea, based on the official records of OO hospital, were enrolled in this study. The demographic and clinical data of the cases were evaluated retrospectively. RESULTS: A total of 42 TB cases were reviewed during the study period. Of these, 37 (88.1%) cases were pulmonary TB. based on the cases identified among the number of North Korean refugees' arriving each year, the annual incidence of pulmonary TB were 900 per 100,000 in 2004, 700 in 2003, The number of smear-positive patients was 20 (47.6%) and the number of culture-positive patients was 18 (42.9%). Of the M. tuberculosis isolates, 2 cases were found to be susceptible to all anti-TB drugs available, 4 were resistant to isoniazid, and 3 were multi-drug resistant. CONCLUSION: The prevalence of pulmonary TB in North Korean Refugees is high. In addition, North Korean refugees suffer from more severe tuberculosis in bacteriological and radiological aspects.

First Male Case of Lymphocytic Hypophysitis in Korea

Lymphocytic hypophysitis is a rare inflammatory disorder which is caused by autoimmune destruction of the pituitary gland. Almost all reported cases have been in women and the disease is often associated with pregnancy. We describe here the first male case of lymphocytic hypophysitis in Korea. The patient presented with headache, impotence, decreased libido, and deteriorated vision. Endocrinologic studies showed panhypopituitarism, and pituitary MRI imaging revealed a homogeneously enhanced pituitary mass with a thickened stalk. Treatment with prednisolone and thyroid hormone for five months was ineffective. Transsphenoidal resection of the pituitary mass was performed successfully with normalization of the visual field defect. Histologic examination revealed diffuse lymphocytic infiltration with dense collagenous fibrosis, consistent with lymphocytic hypophysitis. Lymphocytic hypophysitis should be considered in differential diagnosis even in men with hypopituitarism and an enlarged pituitary gland.