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Pulmonary Involvement (pulmonary + involvement)
Selected AbstractsValue of fractional exhaled nitric oxide (FENO) for the diagnosis of pulmonary involvement due to inflammatory bowel diseaseINFLAMMATORY BOWEL DISEASES, Issue 4 2010Ezgi Ozyilmaz MD Abstract Background: Pulmonary involvement due to inflammatory bowel disease (IBD) is frequent when evaluating a patient with IBD and pulmonary involvement remains complicated. Most of the patients are asymptomatic and the methods used are mostly invasive or expensive procedures. The aim of this prospective study is to evaluate the value of the fractional exhaled nitric oxide (FENO) level for the diagnosis of pulmonary involvement due to IBD and to investigate any correlation between FENO level and disease activity. Methods: Thirty-three nonsmoker patients with IBD (25 ulcerative colitis [UC] and 8 Crohn's Disease [CD]) who were free of corticosteroid treatment and 25 healthy subjects as a control group were enrolled in this study. All patients with IBD were investigated for pulmonary involvement with medical history, physical examination, chest roentgenogram, oxygen saturation, blood eosinophil levels, pulmonary function tests (PFTs), high-resolution computed tomography (HRCT), and FENO level. Results: Pulmonary involvement was established in 15 patients (45.5%) with IBD. The FENO level was higher in patients with pulmonary involvement than without pulmonary involvement and healthy controls independent from the pulmonary symptoms, eosinophil count, duration of disease, activity of disease, and surgery history (FENO: 32 ± 20; 24 ± 8; 14 ± 8 ppb, respectively) (P < 0.05). In addition, diffusion capacity (DLCO) was found to be significantly lower in patients with CD compared with UC (P < 0.05). Conclusions: This study showed that an increased FENO level may be used for identifying patients with IBD who need further pulmonary evaluation. Inflamm Bowel Dis 2009 [source] Pulmonary involvement in Sweet's syndrome: a case report and review of the literatureINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 6 2006Leonardo Astudillo MD Pulmonary involvement in Sweet's syndrome (SS) is rare. We report a case of SS with severe respiratory involvement responding to corticosteroid therapy. A 82-year-old man presented fever of 39 °C associated with cough and dyspnea, and crackles in the left lung. The infection work-up was negative. Chest X-ray showed cardiomegaly and left lower lobe pulmonary infiltrates. Pulmonary signs did not improve on treatment with antibiotics, and after 1 week maculopapular lesions appeared, localized on the knees, the periombilical area and the back. The antibiotics were changed without improvement. A skin biopsy revealed infiltration by neutrophilic granulocytes and marked edema in the dermis, consistent with SS. The patient's condition progressively worsened, requiring high oxygenotherapy, and he was transferred to an intensive care unit. Chest X-ray revealed an important alveolar and interstitial syndrome. Bronchoalveolar lavage found 170 leukocytes with 30% neutrophils (N < 5%), 7% lymphocytes and 63% macrophages. A search for bacteria, viruses or parasites in bronchoalveolar lavage was negative. The patient was treated with antibiotics, a high dose of furosemide and steroids for 4 days. Because the patient improved dramatically within 5 days, with a negative infection work-up and a dramatic decrease of C-reactive protein, the antibiotics were stopped. Steroids were secondarily tapered very slowly. A chest computed tomography (CT) scan showed a substantial improvement of pulmonary lesions. We also review the 22 cases of pulmonary involvement of SS reported in the literature. [source] Pyoderma gangrenosum: a report of 21 casesINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 2 2002Rym Benmously Mlika MD Background Pyoderma gangrenosum (PG) is an uncommon, destructive, cutaneous ulceration, belonging to the neutrophilic disease spectrum. It is associated with systemic disease in 50% of cases. Methods We report a retrospective study of 21 cases of PG. All cases studied fulfilled the following criteria: (i) clinical features of PG; (ii) histopathology consistent with a diagnosis of PG, and excluding other specific dermatoses. Results The average age of our patients was 41.8 years. The male to female ratio was 1.1. The typical ulcerative variant was found in 17 patients, bullous PG in two patients, and the granulomatous variant in two patients. Sixty-two per cent of our patients had lesions on their lower legs. Two patients had neutrophilic pulmonary involvement concurrent with the ulcers. An association with other internal diseases was noted in 12 patients. Histopathologic study showed vasculitis in 13 patients. Of these, 11 were leukocytoclastic and the others predominantly lymphocytic. Conclusions PG is a rare disease, with the ulcerative variant being most frequent. The lower legs are the most commonly affected sites. The recurrence rate in our study was about 46% regardless of the treatment prescribed. Pulmonary involvement was fatal in two patients. [source] Pathogenesis of multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis and association with tuberous sclerosis genes TSC1 and TSC2PATHOLOGY INTERNATIONAL, Issue 8 2001Hiroshi Maruyama Tuberous sclerosis (TSC) is a rare, genetically determined disorder / familial tumor syndrome, currently diagnosed using specific clinical criteria proposed by Gomez, including the presence of multiorgan hamartomas. Pulmonary involvement in TSC is well known as pulmonary lymphangioleiomyomatosis (LAM), which has an incidence of 1,2.3% in TSC patients. LAM has immunohistochemical expression of both smooth-muscle actin and a monoclonal antibody specific for human melanoma, HMB-45. It has recently been reported that multifocal micronodular pneumocyte hyperplasia (MMPH) associated with TSC should be considered as a distinct type of lung lesion, whether it occurs with or without LAM. Two predisposing genes have been found in families affected by TSC; approximately half of the families show linkage to TSC1 at 9q34.3, and the other half show linkage to TSC2 at 16p13.3. TSC genes are considered to be tumor suppressor genes, and mutations in them may lead to abnormal differentiation and proliferation of cells. Tuberin, the TSC2 gene product, has recently been found to be expressed in LAM and MMPH. In this article we discuss the histogenesis and genetic abnormalities of neoplastic lesions associated with TSC, and we review the current understanding of the pathogenesis of pulmonary hamartomatous lesions such as LAM and MMPH in TSC. [source] Early pulmonary involvement in Niemann-Pick type B disease: Lung lavage is not usefulPEDIATRIC PULMONOLOGY, Issue 2 2005Z.S. Uyan MD Abstract Niemann-Pick disease (NPD) is a rare, autosomal-recessively inherited lipid storage disease which is characterized by intracellular deposition of sphingomyelin in various body tissues. The disease is heterogeneous and classified into six groups. Pulmonary parenchymal involvement may be a feature of several subtypes of NPD, including type B. Progressive pulmonary involvement in NPD type B is a major cause of morbidity and mortality. It is usually diagnosed at older ages. Only a few cases with early pulmonary involvement have been reported. In this report, a patient with NPD type B, hospitalized with the diagnosis of pneumonia at age 3 months, is presented. Following treatment for pneumonia, she continued to have persistent respiratory symptoms and became oxygen-dependent. High-resolution computed tomography of the chest revealed diffuse interstitial changes. During follow-up, the patient developed hepatosplenomegaly. Lung, liver, and bone marrow biopsies showed characteristic findings for NPD. Biochemical studies also confirmed the diagnosis, and the sphingomyelinase enzyme level of the patient was low. Unilateral lung lavage was performed in order to decrease lipid storage as a treatment modality. However, there was no clinical or radiological improvement. The patient died at age 15 months due to progressive respiratory failure. Pulmonary involvement is a rare entity in early childhood in patients with NPD type B, but should be considered in the differential diagnosis of persistent interstitial lung disease. It may cause progressive respiratory failure, but the treatment options remain limited. Pediatr Pulmonol. 2005; 40:169,172. © 2005 Wiley-Liss, Inc. [source] Pulmonary involvement in a patient with dyskeratosis congenitaPEDIATRICS INTERNATIONAL, Issue 6 2003Sebnem Kilic No abstract is available for this article. [source] An open randomized controlled trial of desmopressin and pulse dexamethasone as adjunct therapy in patients with pulmonary involvement associated with severe leptospirosisCLINICAL MICROBIOLOGY AND INFECTION, Issue 8 2010K. Niwattayakul Clin Microbiol Infect 2010; 16: 1207,1212 Abstract Pulmonary involvement in leptospirosis is emerging as a common complication of severe leptospirosis. A prospective randomized controlled trial of desmopressin or high-dose (pulse) dexamethasone as adjunctive therapy in 68 patients with pulmonary involvement associated with severe leptospirosis was conducted between July 2003 and October 2006 at five hospitals in Thailand. There were 23 patients in the desmopressin group, 22 in the pulse dexamethasone group, and 23 in a control group who received standard critical care alone. The diagnosis of leptospirosis was confirmed in 52 patients (77%). There were 15 deaths (22%), of which eight patients received desmopressin, four patients received pulse dexamethasone, and three patients received critical care alone (p 0.19). Eight patients with confirmed leptospirosis died (five patients in the desmopressin group, one in the pulse dexamethasone group and two in the control group). The mortality was not significantly different in the desmopressin group or pulse dexamethasone group compared to the control group in both intention-to-treat patients, and in patients with confirmed leptospirosis. There were no serious events associated with desmopressin treatment, although pulse dexamethasone treatment was associated with a significant increase in nosocomial infection. The results of logistic regression analysis revealed that serum bilirubin level was the only significant risk factor associated with mortality (OR 0.759, 95% CI 0.598,0.965, p 0.024). The results obtained in the present study do not support the use of either pulse dexamethasone or desmopressin as adjunct therapy for pulmonary involvement associated with severe leptospirosis. [source] Pulmonary involvement in Fabry diseaseACTA PAEDIATRICA, Issue 2002F Barbey No abstract is available for this article. [source] Smoking preceded pulmonary involvement in adults with Langerhans cell histiocytosis diagnosed in childhoodACTA PAEDIATRICA, Issue 11 2000C Bernstrand ABSTRACT. Two patients with childhood Langerhans cell histiocytosis (LCH) (aged 2 and 6 y at diagnosis) in whom pulmonary involvement was diagnosed in adulthood, 23 and 12 y later, respectively, are presented. In each patient, smoking preceded the diagnosis of pulmonary involvement by 3 y, providing further evidence that smoking is a risk factor in the development of pulmonary LCH. ,Langerhans cell histiocytosis, lungs, smoking [source] Value of fractional exhaled nitric oxide (FENO) for the diagnosis of pulmonary involvement due to inflammatory bowel diseaseINFLAMMATORY BOWEL DISEASES, Issue 4 2010Ezgi Ozyilmaz MD Abstract Background: Pulmonary involvement due to inflammatory bowel disease (IBD) is frequent when evaluating a patient with IBD and pulmonary involvement remains complicated. Most of the patients are asymptomatic and the methods used are mostly invasive or expensive procedures. The aim of this prospective study is to evaluate the value of the fractional exhaled nitric oxide (FENO) level for the diagnosis of pulmonary involvement due to IBD and to investigate any correlation between FENO level and disease activity. Methods: Thirty-three nonsmoker patients with IBD (25 ulcerative colitis [UC] and 8 Crohn's Disease [CD]) who were free of corticosteroid treatment and 25 healthy subjects as a control group were enrolled in this study. All patients with IBD were investigated for pulmonary involvement with medical history, physical examination, chest roentgenogram, oxygen saturation, blood eosinophil levels, pulmonary function tests (PFTs), high-resolution computed tomography (HRCT), and FENO level. Results: Pulmonary involvement was established in 15 patients (45.5%) with IBD. The FENO level was higher in patients with pulmonary involvement than without pulmonary involvement and healthy controls independent from the pulmonary symptoms, eosinophil count, duration of disease, activity of disease, and surgery history (FENO: 32 ± 20; 24 ± 8; 14 ± 8 ppb, respectively) (P < 0.05). In addition, diffusion capacity (DLCO) was found to be significantly lower in patients with CD compared with UC (P < 0.05). Conclusions: This study showed that an increased FENO level may be used for identifying patients with IBD who need further pulmonary evaluation. Inflamm Bowel Dis 2009 [source] Pulmonary involvement in Sweet's syndrome: a case report and review of the literatureINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 6 2006Leonardo Astudillo MD Pulmonary involvement in Sweet's syndrome (SS) is rare. We report a case of SS with severe respiratory involvement responding to corticosteroid therapy. A 82-year-old man presented fever of 39 °C associated with cough and dyspnea, and crackles in the left lung. The infection work-up was negative. Chest X-ray showed cardiomegaly and left lower lobe pulmonary infiltrates. Pulmonary signs did not improve on treatment with antibiotics, and after 1 week maculopapular lesions appeared, localized on the knees, the periombilical area and the back. The antibiotics were changed without improvement. A skin biopsy revealed infiltration by neutrophilic granulocytes and marked edema in the dermis, consistent with SS. The patient's condition progressively worsened, requiring high oxygenotherapy, and he was transferred to an intensive care unit. Chest X-ray revealed an important alveolar and interstitial syndrome. Bronchoalveolar lavage found 170 leukocytes with 30% neutrophils (N < 5%), 7% lymphocytes and 63% macrophages. A search for bacteria, viruses or parasites in bronchoalveolar lavage was negative. The patient was treated with antibiotics, a high dose of furosemide and steroids for 4 days. Because the patient improved dramatically within 5 days, with a negative infection work-up and a dramatic decrease of C-reactive protein, the antibiotics were stopped. Steroids were secondarily tapered very slowly. A chest computed tomography (CT) scan showed a substantial improvement of pulmonary lesions. We also review the 22 cases of pulmonary involvement of SS reported in the literature. [source] Cerebral and Oculorhinal Manifestations of a Limited Form of Wegener's Granulomatosis With c-ANCA,Associated VasculitisJOURNAL OF NEUROIMAGING, Issue 1 2001Peterus Thajeb MD ABSTRACT The authors report on cerebral and oculorhinal manifestations in a patient with a cytoplasmic pattern of antineutrophil cytoplasmic autoantibody (c-ANCA),associated vasculitis. Recurrent Tolosa-Hunt syndrome, cavernous sinus syndrome, Raeder's paratrigeminal neuralgia, and seizures were the major clinical manifestations. Brain MRI showed localized enhancing lesions initially in the cavernous sinus and later in the convexity pachymeninges. The lesions disappeared following 9 months of oral prednisolone (15 mg/day) and cyclophosphamide (100 mg/day) therapy. The presence of c-ANCA, demonstration of vasculitis, and depositions of immunoglobulin G (IgG) and fibrinogen in the vessel walls of pachymeninges of the patient confirmed an immune-mediated cause of the vasculitis. Cranial pathology without renal and pulmonary involvement suggests a variant of Wegener's granulomatosis, which is called the "limited" form of Wegener's granulomatosis. [source] Posaconazole as first line treatment for disseminated zygomycosisMYCOSES, Issue 6 2008Trisha Peel Summary We describe the first case report of posaconazole use as first line agent in the treatment of disseminated zygomycosis with prosthetic hip joint and pulmonary involvement due to Rhizopus microsporus. This infection occurred in a heavily immunosuppressed patient with systemic lupus erythematosus. [source] Non-specific interstitial pneumonia as a manifestation of graft-versus-host disease following pediatric allogeneic hematopoietic stem cell transplantationPATHOLOGY INTERNATIONAL, Issue 2 2010Aya Miyagawa-Hayashino Bronchiolitis obliterans (BO) is generally believed to be a marker of pulmonary manifestation of graft-versus-host disease (GVHD) in patients who have undergone bone marrow transplantation for hematological malignancy. Pulmonary manifestations reported as GVHD (other than BO) include lymphocytic bronchiolitis with cellular interstitial pneumonia, lymphoid interstitial pneumonia, veno-occlusive disease, and diffuse alveolar damage. Morphological reactions in the lungs of bone marrow transplant recipients associated with interstitial pneumonia have not been described systematically. Reported herein is a fibrosing non-specific interstitial pneumonia (NSIP) pattern together with BO in both lungs in an 8-year-old girl following a second allogeneic hematopoietic stem cell transplantation for relapsed neuroblastoma of adrenal origin. The course was complicated by bilateral pneumothoraces, and the patient underwent lung transplantation 3 years after the second stem cell transplantation. Because the patient had chronic GVHD of the skin and the liver preceeded by the development of pulmonary involvement, NSIP may represent one of the facets of pulmonary GVHD. [source] Solitary squamous cell papilloma of the lung in a 40-year-old woman with recurrent laryngeal papillomatosisPATHOLOGY INTERNATIONAL, Issue 5 2000Hidekazu Harada A rare case of recurrent respiratory papillomatosis (RRP) is reported with a review of the literature. A 40-year-old Japanese woman had suffered from RRP since 1 year of age. She developed a pulmonary squamous papilloma with a thin-walled cavity, which was suspected as being lung carcinoma. The trachea and bronchi around the tumor were intact, and no malignant transformation was present. Two types of human papillomavirus, 6 and 16, were detected, both in the laryngeal and pulmonary papillomas by in situ hybridization and the polymerase chain reaction method. To date, only 40 cases of juvenile laryngeal papilloma with pulmonary involvement have been reported in the English literature. [source] Early pulmonary involvement in Niemann-Pick type B disease: Lung lavage is not usefulPEDIATRIC PULMONOLOGY, Issue 2 2005Z.S. Uyan MD Abstract Niemann-Pick disease (NPD) is a rare, autosomal-recessively inherited lipid storage disease which is characterized by intracellular deposition of sphingomyelin in various body tissues. The disease is heterogeneous and classified into six groups. Pulmonary parenchymal involvement may be a feature of several subtypes of NPD, including type B. Progressive pulmonary involvement in NPD type B is a major cause of morbidity and mortality. It is usually diagnosed at older ages. Only a few cases with early pulmonary involvement have been reported. In this report, a patient with NPD type B, hospitalized with the diagnosis of pneumonia at age 3 months, is presented. Following treatment for pneumonia, she continued to have persistent respiratory symptoms and became oxygen-dependent. High-resolution computed tomography of the chest revealed diffuse interstitial changes. During follow-up, the patient developed hepatosplenomegaly. Lung, liver, and bone marrow biopsies showed characteristic findings for NPD. Biochemical studies also confirmed the diagnosis, and the sphingomyelinase enzyme level of the patient was low. Unilateral lung lavage was performed in order to decrease lipid storage as a treatment modality. However, there was no clinical or radiological improvement. The patient died at age 15 months due to progressive respiratory failure. Pulmonary involvement is a rare entity in early childhood in patients with NPD type B, but should be considered in the differential diagnosis of persistent interstitial lung disease. It may cause progressive respiratory failure, but the treatment options remain limited. Pediatr Pulmonol. 2005; 40:169,172. © 2005 Wiley-Liss, Inc. [source] Inflammatory pulmonary nodules in Kawasaki diseasePEDIATRIC PULMONOLOGY, Issue 2 2003Alexandra F. Freeman MD Abstract Symptomatic pulmonary manifestations of Kawasaki disease (KD) are uncommon. However, epidemiologic, radiologic, and histologic studies have indicated that respiratory symptoms and findings occur in KD and suggest that the KD agent may have a respiratory portal of entry. We report on three young infants with KD who developed pulmonary nodules, in addition to coronary artery aneurysms. Two patients had pathologic specimens available, one from biopsy and the other from autopsy. The nodules had predominantly mononuclear cell infiltrates, which were within the lung parenchyma and infiltrating vessel walls. Immunohistochemical studies of the nodules, using antibodies to common leukocyte antigen (LCA) and factor VIII-related antigen, confirmed the inflammatory nature of the lesions and showed capillary proliferation. IgA plasma-cell infiltration was observed in the nodule, consistent with previous KD findings of IgA plasma-cell infiltration in the vessel walls, kidneys, pancreas, and upper respiratory tract. The two patients with nonfatal KD were treated with intravenous immunoglobulin and aspirin, with resolution of the nodules. We propose that when pulmonary involvement occurs in KD, it ranges from subclinical interstitial micronodular infiltrates to larger inflammatory pulmonary nodules. These pulmonary infiltrates and nodules likely reflect the host response to the etiologic agent of KD, and may resolve with the disease process. Recognition of this pulmonary complication of KD may enable cautious observation of such lesions for spontaneous resolution. Pediatr Pulmonol. 2003; 36:102,106. © 2003 Wiley-Liss, Inc. [source] Hodgkin's disease and ataxia telangiectasia with pulmonary cavitiesPEDIATRIC PULMONOLOGY, Issue 5 2002Bilgehan Yalçin MD Abstract Ataxia telangiectasia (AT) homozygotes have an increased risk for development of Hodgkin's disease (HD). Parenchymal lung involvement is not uncommon in HD; however, cavitary pulmonary lesions are quite unusual. We report on 3 cases of AT with HD who had mediastinal disease and parenchymal pulmonary involvement with cavitation. Of 6 AT patients in our HD series, 3 developed pulmonary cavities. The patients displayed pulmonary infiltration, cavitation in the lung parenchyma, and mediastinal enlarged lymph nodes on both plain chest X-rays and thoracic computed tomographies. No infectious etiologies were established for the pulmonary findings. Histopathological examination of open lung and mediastinal biopsies revealed HD, and all patients received multiagent chemotherapies. The outcome was fatal in all 3 patients. Respiratory infections are the principle cause for morbidity and mortality in AT patients. Reports on cavitating pulmonary lesions in HD are quite rare. Furthermore, data regarding the patterns of pulmonary involvement in AT patients with or without HD are lacking. The increased incidence of malignancies in AT patients may relate to immunodeficiency and to the chromosomal alterations identified. Pediatr Pulmonol. 2002; 33:399,403. © 2002 Wiley-Liss, Inc. [source] Clinical spectrum of tuberculous pleural effusion in childrenPEDIATRICS INTERNATIONAL, Issue 3 2007CHIH-YUNG CHIU Abstract Background: The aim of this study was to describe the clinical characteristics and potentially diagnostic specimens of pediatric patients with tuberculous pleural effusion (TPE) to make a prompt diagnosis. Methods: Children who had TPE from September 1997 to December 2003 were retrospectively reviewed at a tertiary pediatric facility in northern Taiwan. Results: There were seven boys and six girls and their ages ranged from 10 to 17 years (average, 14.6 years). Tuberculosis contact history was identified in only six patients (46%). Fever (12/92%), cough (9/69%) and malaise (6/46%) were the most common symptoms. Normal leukocyte count was found in 12 patients (92%). Chest radiograph review showed unilateral pleural effusion in 12 patients (92%) but parenchymal involvement was found in nine patients (69%). Most of the pleural fluid analysis showed a lymphocytic exudative effusion (5/6). The acid-fast bacilli (AFB) stain of sputum, gastric washing, and pleural aspirate was positive in six of 11 (55%), two of seven (29%), and one of five (20%) patients, respectively. Culture of sputum, gastric washing, and pleural aspirate yielded Mycobacterium tuberculosis in four of 11 (36%), two of seven (29%), and two of five (40%) patients, respectively. A total of 6 to 9 months of multiple-drug therapy for tuberculosis was successful without sequale. Conclusions: Tuberculous pleural effusion usually presents as an acute illness and should always be considered in the differential diagnosis for older children and adolescents with pneumonia. A normal leukocyte count with a lymphocytic exudative effusion may provide a clue to the correct diagnosis of TPE. Diagnostic specimen of sputum seems more effective and sensitive in childhood TPE, especially those having pulmonary involvement. [source] Granulocytic sarcoma with pulmonary involvementAMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2007Shaul Avraham No abstract is available for this article. [source] Recurrent respiratory papillomatosis with pulmonary involvement: A case report and review of the literatureRESPIROLOGY, Issue 1 2009Sheng-Yuan RUAN ABSTRACT Recurrent respiratory papillomatosis (RRP) is a viral infection that usually affects the upper airways. Although it can spread throughout the respiratory tract, involvement of the lung parenchyma is quite rare. Radiographic images of RRP with lung involvement have been mainly presented in case reports of paediatric patients. We present the case of an adult patient with RRP and lung involvement, and detail the serial radiographic manifestations. CT and reconstructed images were obtained. The literature was also reviewed and radiographic features of RRP with lung involvement are summarized. [source] Peripheral T-cell lymphoma with diffuse pulmonary infiltration and an increase in serum KL-6 levelRESPIROLOGY, Issue 3 2007Tomoyuki FUJISAWA Abstract: Peripheral T-cell lymphoma is a subtype of non-Hodgkin's lymphoma. A case of peripheral T-cell lymphoma showing diffuse pulmonary involvement together with a marked increase in the level of serum KL-6 is presented. CXR and CT revealed reticular and ground-glass opacities, which mimicked interstitial pneumonia. Immunopathological findings and an analysis of T-cell receptor gene rearrangements of the lung biopsy specimen led to a definite diagnosis of peripheral T-cell lymphoma. In addition, the extensive proliferation of type II pneumocytes, which stained strongly positive for anti-KL-6 antibody suggested that the pneumocytes were the source of serum KL-6. [source] Langerhans cell histiocytosis in a premature baby presenting with skin-isolated disease: case report and literature reviewACTA PAEDIATRICA, Issue 12 2008Shraga Aviner Abstract Langerhans cell histiocytosis (LCH) in premature babies is extremely rare as is a vesicular skin rash, while gastrointestinal involvement is associated with a poor outcome. We report a case of LCH in a premature baby presented with isolated vesiculo-papulo-macular skin lesions and insidiously developed gastrointestinal symptoms, haematological and severe pulmonary involvement. We also reviewed a few cases of LCH in premature babies in the English language medical literature. LCH in preterm babies appears to be a severe systemic disease, usually lethal in-utero or post delivery. Conclusion: Careful observation should be applied to newborns with skin-only Langerhans cell histiocytosis in order to identify in time progression to potentially fatal systemic disease. [source] Anti-topoisomerase II , autoantibodies in systemic sclerosis,association with pulmonary hypertension and HLA-B35CLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2000B. Grigolo We have previously detected autoantibodies against topoisomerase II , (anti-topo II ,) in sera from patients with idiopathic pulmonary fibrosis. To determine whether anti-topo II , is also present in systemic sclerosis (SSc) patients with pulmonary involvement, we screened sera from 92 patients and 34 healthy controls. Presence of anti-topo II , was investigated with respect to clinical and serological features, including the frequencies of HLA class I and II alleles. Anti-topo II , was detected in 20/92 (21.7%) patients. No association was found with either anti-topoisomerase I (Scl-70 or anti-topo I) or anti-centromere antibodies. However, anti-topo II , was associated with the presence of pulmonary hypertension (PHT) (as opposed to pulmonary fibrosis), and with a decrease of carbon monoxide diffusing capacity. Anti-topo II , was strongly associated with the presence of the class I antigen HLA-B35. No significant association was found with HLA class II antigens. HLA-B35 also turned out to be associated with the presence of PHT. These results indicate that in SSc patients, the presence of anti-topo II , is associated with PHT, and that the simultaneous presence of HLA-B35 seems to add to the risk of developing PHT. [source] An open randomized controlled trial of desmopressin and pulse dexamethasone as adjunct therapy in patients with pulmonary involvement associated with severe leptospirosisCLINICAL MICROBIOLOGY AND INFECTION, Issue 8 2010K. Niwattayakul Clin Microbiol Infect 2010; 16: 1207,1212 Abstract Pulmonary involvement in leptospirosis is emerging as a common complication of severe leptospirosis. A prospective randomized controlled trial of desmopressin or high-dose (pulse) dexamethasone as adjunctive therapy in 68 patients with pulmonary involvement associated with severe leptospirosis was conducted between July 2003 and October 2006 at five hospitals in Thailand. There were 23 patients in the desmopressin group, 22 in the pulse dexamethasone group, and 23 in a control group who received standard critical care alone. The diagnosis of leptospirosis was confirmed in 52 patients (77%). There were 15 deaths (22%), of which eight patients received desmopressin, four patients received pulse dexamethasone, and three patients received critical care alone (p 0.19). Eight patients with confirmed leptospirosis died (five patients in the desmopressin group, one in the pulse dexamethasone group and two in the control group). The mortality was not significantly different in the desmopressin group or pulse dexamethasone group compared to the control group in both intention-to-treat patients, and in patients with confirmed leptospirosis. There were no serious events associated with desmopressin treatment, although pulse dexamethasone treatment was associated with a significant increase in nosocomial infection. The results of logistic regression analysis revealed that serum bilirubin level was the only significant risk factor associated with mortality (OR 0.759, 95% CI 0.598,0.965, p 0.024). The results obtained in the present study do not support the use of either pulse dexamethasone or desmopressin as adjunct therapy for pulmonary involvement associated with severe leptospirosis. [source] | |||||||||||||||||||||||||