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Sample records for bronchiolitis obliterans syndrome

  1. Lung transplantation for bronchiolitis obliterans syndrome after allo-SCT

    DEFF Research Database (Denmark)

    Holm, A M; Riise, Gerdt; Hansson, Leif Helmuth;

    2013-01-01

    Chronic GVHD (cGVHD) associated bronchiolitis obliterans syndrome (BOS) is a serious complication after allo-SCT, and lung transplantation (LTx) may be the ultimate treatment option. To evaluate this treatment, data on all patients with LTx after allo-SCT ever performed in Sweden, Norway, Denmark...... to cGVHD after allo-SCT.Bone Marrow Transplantation advance online publication, 15 October 2012; doi:10.1038/bmt.2012.197.......Chronic GVHD (cGVHD) associated bronchiolitis obliterans syndrome (BOS) is a serious complication after allo-SCT, and lung transplantation (LTx) may be the ultimate treatment option. To evaluate this treatment, data on all patients with LTx after allo-SCT ever performed in Sweden, Norway, Denmark...

  2. Pulmonary Rehabilitation for Bronchiolitis Obliterans Syndrome after Hematopoietic Stem Cell Transplantation

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    Tran, Jerry; Norder, Emily; Diaz, Phil; Gary S Phillips; Elder, Pat; Devine, Steven M; Wood, Karen L.

    2012-01-01

    Bronchiolitis obliterans syndrome (BOS) is a progressive, insidious lung disease affecting allogeneic hematopoietic stem cell transplant (HSCT) recipients. Unfortunately, there is no standardized approach for treatment of BOS in post HSCT patients. Pulmonary rehabilitation is a standard treatment in emphysema, an irreversible obstructive lung disease secondary to tobacco abuse. The National Emphysema Treatment Trial (NETT) demonstrated improved exercise tolerance, decrease dyspnea, and increa...

  3. Phenytoin induced Steven–Johnson syndrome and bronchiolitis obliterans – case report and review of literature

    Science.gov (United States)

    Pannu, Bibek S.; Egan, Ashley M.; Iyer, Vivek N.

    2016-01-01

    Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both rare but serious idiosyncratic drug reactions characterized by diffuse muco-epidermoid injury and high mortality. Keratinocytes in both skin and mucous membranes (including eyes, mouth and genitalia) are injured resulting in a diffuse maculopapular rash, blistering lesions and epithelial detachment with minimal force (Nikolsky's sign). SJS is typically diagnosed when less than 10% of the skin surface is involved and the term TEN is used in cases with more than 30% involvement. Respiratory involvement in SJS-TEN is common with 30–50% of cases demonstrating respiratory epithelial sloughing with severe short and long term complications. Patients who survive SJS-TEN are often left with impaired respiratory function and bronchiolitis obliterans. Cases of bronchiolitis obliterans with SJS/TEN have been very rarely reported. We report a case of phenytoin induced SJS/TEN followed by severe bronchiolitis obliterans in an adult patient. The presentation, pathophysiology and management of SJS/TEN related bronchiolitis obliterans is also reviewed. PMID:27222786

  4. Phenytoin induced Steven-Johnson syndrome and bronchiolitis obliterans - case report and review of literature.

    Science.gov (United States)

    Pannu, Bibek S; Egan, Ashley M; Iyer, Vivek N

    2016-01-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both rare but serious idiosyncratic drug reactions characterized by diffuse muco-epidermoid injury and high mortality. Keratinocytes in both skin and mucous membranes (including eyes, mouth and genitalia) are injured resulting in a diffuse maculopapular rash, blistering lesions and epithelial detachment with minimal force (Nikolsky's sign). SJS is typically diagnosed when less than 10% of the skin surface is involved and the term TEN is used in cases with more than 30% involvement. Respiratory involvement in SJS-TEN is common with 30-50% of cases demonstrating respiratory epithelial sloughing with severe short and long term complications. Patients who survive SJS-TEN are often left with impaired respiratory function and bronchiolitis obliterans. Cases of bronchiolitis obliterans with SJS/TEN have been very rarely reported. We report a case of phenytoin induced SJS/TEN followed by severe bronchiolitis obliterans in an adult patient. The presentation, pathophysiology and management of SJS/TEN related bronchiolitis obliterans is also reviewed. PMID:27222786

  5. A randomised controlled trial of azithromycin therapy in bronchiolitis obliterans syndrome (BOS) post lung transplantation

    OpenAIRE

    Corris, Paul A; Ryan, Victoria A; Small, Therese; Lordan, James; Fisher, Andrew J.; Meachery, Gerard; Johnson, Gail; Ward, Chris

    2015-01-01

    Background We conducted a placebo-controlled trial of azithromycin therapy in bronchiolitis obliterans syndrome (BOS) post lung transplantation. Methods We compared azithromycin (250 mg alternate days, 12 weeks) with placebo. Primary outcome was FEV1 change at 12 weeks. Results 48 patients were randomised; (25 azithromycin, 23 placebo). It was established, post randomisation that two did not have BOS. 46 patients were analysed as intention to treat (ITT) with 33 ‘Completers’. ITT analysis inc...

  6. Lung transplantation: does oxidative stress contribute to the development of bronchiolitis obliterans syndrome?

    Science.gov (United States)

    Madill, Janet; Aghdassi, Ellie; Arendt, Bianca; Hartman-Craven, Brenda; Gutierrez, Carlos; Chow, Chung-Wai; Allard, Johane

    2009-04-01

    Lung transplantation is the ultimate treatment of end-stage lung disease. After transplantation, the 1-year survival rate is 80%. However, 5-year survival rates drop to 50% due to bronchiolitis obliterans syndrome (BOS). Ischemia/reperfusion injury, infections, and acute rejection are major risk factors contributing to the development of BOS. These risk factors are also associated with increased oxidative stress. Oxidative stress is a condition whereby prooxidants overwhelm the antioxidant defense system and may contribute to the pathogenesis of BOS by inducing more tissue injury and inflammation. This article reviews the current state of knowledge on oxidative stress in lung transplantation and BOS. PMID:19298941

  7. Two cases of bronchiolitis obliterans organizing pneumonia syndrome after the radiation of breast-conserving therapy

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    Komuro, Youko; Nakagomi, Hiroshi; Akaike, Hidenori; Chiba, Shigehiro; Miyashita, Yoshihiro; Obu, S.; Yamaguchi, Motoshi; Oyama, Toshio [Yamanashi Prefectural Central Hospital, Kofu (Japan)

    2001-12-01

    Bronchiolitis obliterans organizing pneumonia (BOOP) syndrome is a recently reported complication of the adjuvant radiotherapy of breast-conserving surgery. We report two cases of BOOP syndrome in 100 patients who underwent breast-conserving therapy. A 75 year-old woman had a cough 3 months after radiation therapy for cancer of the left breast. Chest radiography and computed tomography showed consolidation with air bronchograms in the upper left lung. Transbronchial lung biopsy showed lymphocyte infiltration and fibrosis in the alveoli. A 45-year old woman with bilateral breast cancer had a cough and palpitations for 3 month after radiation therapy for cancer of the right breast. Chest radiography and computed tomography showed consolidation in upper and middle fields of the left lung. Transbronchial lung biopsy showed granulation formation in the alveolar duct, which is a typical feature of BOOP. The symptoms and radiographic findings improved with oral administration of prednisolone. BOOP syndrome may occur as a complication of breast-conserving therapy. (author)

  8. Two cases of bronchiolitis obliterans organizing pneumonia syndrome after the radiation of breast-conserving therapy

    International Nuclear Information System (INIS)

    Bronchiolitis obliterans organizing pneumonia (BOOP) syndrome is a recently reported complication of the adjuvant radiotherapy of breast-conserving surgery. We report two cases of BOOP syndrome in 100 patients who underwent breast-conserving therapy. A 75 year-old woman had a cough 3 months after radiation therapy for cancer of the left breast. Chest radiography and computed tomography showed consolidation with air bronchograms in the upper left lung. Transbronchial lung biopsy showed lymphocyte infiltration and fibrosis in the alveoli. A 45-year old woman with bilateral breast cancer had a cough and palpitations for 3 month after radiation therapy for cancer of the right breast. Chest radiography and computed tomography showed consolidation in upper and middle fields of the left lung. Transbronchial lung biopsy showed granulation formation in the alveolar duct, which is a typical feature of BOOP. The symptoms and radiographic findings improved with oral administration of prednisolone. BOOP syndrome may occur as a complication of breast-conserving therapy. (author)

  9. Oxidative stress and nutritional intakes in lung patients with bronchiolitis obliterans syndrome.

    Science.gov (United States)

    Madill, J; Aghdassi, E; Arendt, B M; Gutierrez, C; Singer, L; Chow, C-W; Keshavjee, S; Allard, J P

    2009-11-01

    Survival after lung transplantation is limited by bronchiolitis obliterans syndrome (BOS). Oxidative stress (OxS) can be associated with BOS due to chronic inflammation. The type of fat and antioxidant intakes may also contribute to OxS. Our aim was to compare OxS and nutritional intakes in non-BOS versus various stages of BOS. Fifty-eight lung recipients with versus without BOS were prospectively classified as: non-BOS; BOS Op-1 (mild), and BOS 2-3 (severe). We measured nutritional intake and plasma vitamins A, C, and E. Among a subgroup of 37 patients, OxS was assessed by measuring lipid peroxidation (LPO micromol/L MDA) and oxidized glutathione (GSSG) in bronchoalveolar lavage BAL fluid (BALF). One-way analysis of variance was used to compare groups. Results are reported as mean values +/- standard errors of the mean. There was no significant difference in demographic features on time posttransplant among groups. Although there were comparable cell counts in BALF, severe BOS patients showed significantly higher BALF LPO concentrations when compared with milder stage of BOS or with non-BOS (P = .001, for both). Severe BOS recipients also displayed higher BALF GSSG concentrations compared to milder stage of BOS (P = .001) or non-BOS (P = .007). In conclusion, patients with severe BOS were more oxidatively stressed compared with mild and non-BOS recipients. PMID:19917398

  10. Clinical investigation of predictors of radiation-induced bronchiolitis obliterans organizing pneumonia syndrome after breast-conserving therapy

    International Nuclear Information System (INIS)

    We investigated 710 patients with breast cancer who received radiotherapy after breast-conserving surgery at our institution to evaluate the incidence of radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome focusing on the interval from irradiation to onset and the clinical presentation. The predictive value of age (≤50 or >50), chemotherapy and hormone therapy was statistically analyzed to determine whether these are risk factors for BOOP syndrome. Radiation-induced BOOP syndrome was seen in 1.3% (9/710). In most cases, the symptoms were mild and none of the patients required hospitalization. Eight patients (88.9%) responded well to steroid administration, but 5 of these patients relapsed after or during tapering of steroids. Although we could not detect significant risk factors for BOOP syndrome, a higher patient age was associated with a higher incidence of radiation-induced BOOP syndrome after breast-conserving therapy. (author)

  11. Acute cellular rejection is a risk factor for bronchiolitis obliterans syndrome independent of post-transplant baseline FEV1

    DEFF Research Database (Denmark)

    Burton, C.M.; Iversen, M.; Carlsen, J.;

    2009-01-01

    BACKGROUND: Post-transplant baseline forced expiratory volume in 1 second (FEV(1)) constitutes a systematic bias in analyses of bronchiolitis obliterans syndrome (BOS). This retrospective study evaluates risk factors for BOS adjusting for the confounding of post-transplant baseline FEV(1). METHODS......: A multivariate survival and competing risk analysis of a large consecutive series of patients (n = 389) from a national center 1992 to 2004. Exclusion criteria were patients not surviving at least 3 months after transplantation (n = 39) and no available lung function measurements (n = 4). RESULTS......: The first maximum FEV(1) occurred at a median 183 days post-transplant. Freedom from BOS was 81%, 53%, 38% and 15%, and cumulative incidence of BOS was 18%, 43%, 57% and 77% at 1, 3, 5 and 10 years post-transplantation, respectively. Acute cellular rejection was independently associated with an...

  12. Bortezomib-Induced Bronchiolitis Obliterans Organizing Pneumonia

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    E. Vandeix

    2012-01-01

    Full Text Available Introduction. Bortezomib is a proteasome inhibitor indicated for the treatment of multiple myeloma patients. The most frequent side effects are gastrointestinal and neurological. Serious pulmonary complications have been described rarely. Observation. This case involves a 74-year-old man suffering from IgG Kappa myeloma treated with bortezomib, melphalan, and dexamethasone. After administering chemotherapy, the patient developed an acute respiratory distress syndrome (ARDS. A surgical pulmonary biopsy proved the existence of bronchiolitis obliterans organizing pneumonia (BOOP lesions. Systemic corticotherapy led to a rapid improvement in the patient’s condition. Conclusion. This is the first reported histologically confirmed case of bortezomid-induced BOOP. Faced with severe respiratory symptoms in the absence of other etiologies, complications due to bortezomid treatment should be evoked and corticotherapy considered.

  13. Rapidly progressive Bronchiolitis Obliterans Organising Pneumonia presenting with pneumothorax, persistent air leak, acute respiratory distress syndrome and multi-organ dysfunction: a case report

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    Chakravorty Indranil

    2008-05-01

    Full Text Available Abstract Introduction Bronchiolitis Obliterans Organising Pneumonia (BOOP may often present initially as a recurrent spontaneous pneumothorax and then develop multi-system complications. Case presentation A 17-year-old boy presented with a pneumothorax, which developed into rapidly progressive Bronchiolitis Obliterans Organising Pneumonia (BOOP. He developed multi-organ dysfunction (including adult respiratory distress syndrome, oliguric renal failure, acute coronary syndrome, cardiac failure and a right atrial thrombus which necessitated prolonged intensive care. Diagnosis was confirmed on open lung biopsy and he responded well to treatment with corticosteroids. Conclusion BOOP is exquisitely sensitive to oral corticosteroids but if the diagnosis is not considered in such patients and appropriate treatment instituted early, BOOP may often lead to prolonged hospital admission with considerable morbidity.

  14. Radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome in breast cancer patients is associated with age

    International Nuclear Information System (INIS)

    Radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome is a rarely observed phenomenon characterized by infiltration of the lungs outside of the radiation field, differentiating it from radiation pneumonitis (RP).The risk factors for radiation-induced BOOP (RT-BOOP) remain unclear and controversial. We retrospectively analyzed the incidence and risk factors for RT-BOOP associated with radiation therapy (RT) after breast conserving surgery (BCS) and post-mastectomy radiation therapy (PMRT). We analyzed 1,176 breast cancer patients treated with RT after BCS or PMRT between March 2005 and September2008 at the cancer institute hospital of the Japanese foundation for cancer research. Chest radiographs were routinely obtained every three to six months for at least 12 months after surgery, as well as when the patients experienced respiratory symptoms or fever. RT-BOOP syndrome was diagnosed in 16patients (1.4%), including12BCS patients (1.3%) and four PMRT patients (1.8%). An older age (≥52 years old) was significantly associated with the incidence of RT-BOOP syndrome in a univariate analysis (p =0.023). The type of treatment (BCS or PMRT) and irradiated lung volume at 20 Gy (V20) were not significantly associated with the incidence of RT-BOOP syndrome in the entire patient cohort. In the multivariate analysis, age and smoking were the significant factor associated with RT-induced BOOP syndrome (p =0.044 and 0.049, respectively). RT-BOOP syndrome was a rarity, and the incidence for BCT cases was similar to that for PMRT cases. The irradiated lung volume was not significantly associated with RT-BOOP syndrome. An older age can predict the incidence of RT-BOOP syndrome

  15. Analysis of Factors Associated With Radiation-Induced Bronchiolitis Obliterans Organizing Pneumonia Syndrome After Breast-Conserving Therapy

    International Nuclear Information System (INIS)

    Purpose: To evaluate factors associated with radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome after breast-conserving therapy. Methods and Materials: A total of 702 women with breast cancer who received radiotherapy after breast-conserving surgery at seven institutions between July 1995 and December 2006 were analyzed. In all patients, the whole breast was irradiated with two tangential photon beams. The criteria used for the diagnosis of radiation-induced BOOP syndrome were as follows: (1) radiotherapy to the breast within 12 months, (2) general and/or respiratory symptoms lasting for ≥2 weeks, (3) radiographs showing lung infiltration outside the radiation port, and (4) no evidence of a specific cause. Results: Radiation-induced BOOP syndrome was seen in 16 patients (2.3%). Eleven patients (68.8%) were administered steroids. The duration of steroid administration ranged from 1 week to 3.7 years (median, 1.1 years). Multivariate analysis revealed that age (≥50 years; odds ratio [OR] 8.88; 95% confidence interval [CI] 1.16-67.76; p = 0.04) and concurrent endocrine therapy (OR 3.05; 95% CI 1.09-8.54; p = 0.03) were significantly associated with BOOP syndrome. Of the 161 patients whose age was ≥50 years and who received concurrent endocrine therapy, 10 (6.2%) developed BOOP syndrome. Conclusions: Age (≥50 years) and concurrent endocrine therapy can promote the development of radiation-induced BOOP syndrome after breast-conserving therapy. Physicians should carefully follow patients who received breast-conserving therapy, especially those who are older than 50 years and received concurrent endocrine therapy during radiotherapy

  16. Protection against bronchiolitis obliterans syndrome is associated with allograft CCR7+ CD45RA- T regulatory cells.

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    Aric L Gregson

    Full Text Available Bronchiolitis obliterans syndrome (BOS is the major obstacle to long-term survival after lung transplantation, yet markers for early detection and intervention are currently lacking. Given the role of regulatory T cells (Treg in modulation of immunity, we hypothesized that frequencies of Treg in bronchoalveolar lavage fluid (BALF after lung transplantation would predict subsequent development of BOS. Seventy BALF specimens obtained from 47 lung transplant recipients were analyzed for Treg lymphocyte subsets by flow cytometry, in parallel with ELISA measurements of chemokines. Allograft biopsy tissue was stained for chemokines of interest. Treg were essentially all CD45RA(-, and total Treg frequency did not correlate to BOS outcome. The majority of Treg were CCR4(+ and CD103(- and neither of these subsets correlated to risk for BOS. In contrast, higher percentages of CCR7(+ Treg correlated to reduced risk of BOS. Additionally, the CCR7 ligand CCL21 correlated with CCR7(+ Treg frequency and inversely with BOS. Higher frequencies of CCR7(+ CD3(+CD4(+CD25(hiFoxp3(+CD45RA(- lymphocytes in lung allografts is associated with protection against subsequent development of BOS, suggesting that this subset of putative Treg may down-modulate alloimmunity. CCL21 may be pivotal for the recruitment of this distinct subset to the lung allograft and thereby decrease the risk for chronic rejection.

  17. Predictive Value of Bronchiolitis Obliterans Syndrome Stage 0p in Chronic Graft-versus-Host Disease of the Lung

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    Abedin, Sameem; Yanik, Gregory A.; Braun, Thomas; Pawarode, Attaphol; Magenau, John; Goldstein, Steven C.; Levine, John E.; Kitko, Carrie L.; Couriel, Daniel R.

    2016-01-01

    Bronchiolitis obliterans syndrome (BOS) is a significant post-transplant complication with low survival. BOS stage 0p (BOS 0p) is a parameter detected on pulmonary function tests (PFTs) after lung transplantation to identify patients at risk to develop BOS. We performed a retrospective study on 442 patients who underwent allogeneic stem cell transplant from 2007 to 2011 to evaluate whether development of BOS 0p is a risk factor in this population for BOS. Patients who met criteria for BOS 0p were significantly more likely to develop BOS (hazard ratio [HR], 3.22; P HR, 2.48; P =.001) and chronic graft-versus-host disease (GVHD) outside the lung post-transplant (HR, 23; P < .001). Finally, BOS 0p criteria were adequately sensitive in predicting BOS (85%), with a high negative predictive value (98%). Our findings suggest a routine PFT screening strategy with the intent of detecting BOS 0p, especially among patients with prior lung disease and who developed chronic GVHD, could suitably identify an at-risk population for the development of BOS. PMID:25687798

  18. Bronchial wall measurements in patients after lung transplantation: evaluation of the diagnostic value for the diagnosis of bronchiolitis obliterans syndrome.

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    Sabine Dettmer

    Full Text Available OBJECTIVES: To prospectively evaluate quantitative airway wall measurements of thin-section CT for the diagnosis of Bronchiolitis Obliterans Syndrome (BOS following lung transplantation. MATERIALS AND METHODS: In 141 CT examinations, bronchial wall thickness (WT, the wall area percentage (WA% calculated as the ratio of the bronchial wall area and the total area (sum of bronchial wall area and bronchial lumen area and the difference of the WT on inspiration and expiration (WTdiff were automatically measured in different bronchial generations. The measurements were correlated with the lung function parameters. WT and WA% in CT examinations of patients with (n = 25 and without (n = 116 BOS, were compared using the unpaired t-test and univariate analysis of variance, while also considering the differing lung volumes. RESULTS: Measurements could be performed in 2,978 bronchial generations. WT, WA%, and WTdiff did not correlate with the lung function parameters (r<0.5. The WA% on inspiration was significantly greater in patients with BOS than in patients without BOS, even when considering the dependency of the lung volume on the measurements. WT on inspiration and expiration and WA% on expiration did not show significant differences between the groups. CONCLUSION: WA% on inspiration was significantly greater in patients with than in those without BOS. However, WA% measurements were significantly dependent on lung volume and showed a high variability, thus not allowing the sole use of bronchial wall measurements to differentiate patients with from those without BOS.

  19. Proteomic Characterization Reveals That MMP-3 Correlates With Bronchiolitis Obliterans Syndrome Following Allogeneic Hematopoietic Cell and Lung Transplantation.

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    Liu, X; Yue, Z; Yu, J; Daguindau, E; Kushekhar, K; Zhang, Q; Ogata, Y; Gafken, P R; Inamoto, Y; Gracon, A; Wilkes, D S; Hansen, J A; Lee, S J; Chen, J Y; Paczesny, S

    2016-08-01

    Improved diagnostic methods are needed for bronchiolitis obliterans syndrome (BOS), a serious complication after allogeneic hematopoietic cell transplantation (HCT) and lung transplantation. For protein candidate discovery, we compared plasma pools from HCT transplantation recipients with BOS at onset (n = 12), pulmonary infection (n = 16), chronic graft-versus-host disease without pulmonary involvement (n = 15) and no chronic complications after HCT (n = 15). Pools were labeled with different tags (isobaric tags for relative and absolute quantification), and two software tools identified differentially expressed proteins (≥1.5-fold change). Candidate proteins were further selected using a six-step computational biology approach. The diagnostic value of the lead candidate, matrix metalloproteinase 3 (MMP3), was evaluated by enzyme-linked immunosorbent assay in plasma of a verification cohort (n = 112) with and without BOS following HCT (n = 76) or lung transplantation (n = 36). MMP3 plasma concentrations differed significantly between patients with and without BOS (area under the receiver operating characteristic curve 0.77). Consequently, MMP3 represents a potential noninvasive blood test for diagnosis of BOS. PMID:26887344

  20. Rituximab-Induced Bronchiolitis Obliterans Organizing Pneumonia

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    Ahmet B. Ergin

    2012-01-01

    Full Text Available Rituximab-induced lung disease (R-ILD is a rare entity that should be considered in patients treated with rituximab who present with dyspnea, fever, and cough, but no clear evidence of infection. A variety of pathologic findings have been described in this setting. Bronchiolitis obliterans organizing pneumonia (BOOP is the most common clinicopathologic diagnosis, followed by interstitial pneumonitis, acute respiratory distress syndrome (ARDS, and hypersensitivity pneumonitis. Prompt diagnosis and treatment with corticosteroids are essential as discussed by Wagner et al. (2007. Here we present a case of an 82-year-old man who was treated with rituximab for recurrent marginal zone lymphoma. After the first infusion of rituximab, he reported fever, chills, and dyspnea. On computed tomography imaging, he was found to have bilateral patchy infiltrates, consistent with BOOP on biopsy. In our patient, BOOP was caused by single-agent rituximab, in the first week after the first infusion of rituximab. We reviewed the relevant literature to clarify the different presentations and characteristics of R-ILD and raise awareness of this relatively overlooked entity.

  1. Fluticasone, Azithromycin, and Montelukast Treatment for New-Onset Bronchiolitis Obliterans Syndrome after Hematopoietic Cell Transplantation.

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    Williams, Kirsten M; Cheng, Guang-Shing; Pusic, Iskra; Jagasia, Madan; Burns, Linda; Ho, Vincent T; Pidala, Joseph; Palmer, Jeanne; Johnston, Laura; Mayer, Sebastian; Chien, Jason W; Jacobsohn, David A; Pavletic, Steven Z; Martin, Paul J; Storer, Barry E; Inamoto, Yoshihiro; Chai, Xiaoyu; Flowers, Mary E D; Lee, Stephanie J

    2016-04-01

    Bronchiolitis obliterans syndrome (BOS) after allogeneic hematopoietic cell transplantation (HCT) is associated with high mortality. We hypothesized that inhaled fluticasone, azithromycin, and montelukast (FAM) with a brief steroid pulse could avert progression of new-onset BOS. We tested this in a phase II, single-arm, open-label, multicenter study (NCT01307462). Thirty-six patients were enrolled within 6 months of BOS diagnosis. The primary endpoint was treatment failure, defined as 10% or greater forced expiratory volume in 1 second decline at 3 months. At 3 months, 6% (2 of 36, 95% confidence interval, 1% to 19%) had treatment failure (versus 40% in historical controls, P < .001). FAM was well tolerated. Steroid dose was reduced by 50% or more at 3 months in 48% of patients who could be evaluated (n = 27). Patient-reported outcomes at 3 months were statistically significantly improved for Short-Form 36 social functioning score and mental component score, Functional Assessment of Cancer Therapies emotional well-being, and Lee symptom scores in lung, skin, mouth, and the overall summary score compared to enrollment (n = 24). At 6 months, 36% had treatment failure (95% confidence interval, 21% to 54%, n = 13 of 36, with 6 documented failures, 7 missing pulmonary function tests). Overall survival was 97% (95% confidence interval, 84% to 100%) at 6 months. These data suggest that FAM was well tolerated and that treatment with FAM and steroid pulse may halt pulmonary decline in new-onset BOS in the majority of patients and permit reductions in systemic steroid exposure, which collectively may improve quality of life. However, additional treatments are needed for progressive BOS despite FAM. PMID:26475726

  2. Bronchiolitis Obliterans Organizing Pneumonia Induced by Minocycline

    OpenAIRE

    Shikuwa, Chieko; Kadota, Jun-ichi; Mukae, Hiroshi; Nagata, Towako; Kaida, Hideyuki; Ishii, Hiroshi; Ishimatsu, Yuji; Kohno, Shigeru

    2001-01-01

    We report a case of bronchiolitis obliterans organizing pneumonia (BOOP) caused by minocycline (MINO). A 59- year-old man visited to our hospital because of flu-like symptoms. He had been treated with MINO for a few weeks for the skin eruption. The chest radiograph showed consolidations in both lung fields. He was admitted to our hospital for further examination. An elevation of lymphocyte percentage was seen in his bronchoalveolar lavage and a diagnosis of BOOP was confirmed by video-assiste...

  3. Another Face of Bronchiolitis Obliterans Organizing Pneumonia

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    Mark O Turner

    2003-01-01

    Full Text Available A 47-year-old man presented with an eight-day history of nonproductive cough and constitutional symptoms progressing to respiratory failure. High resolution computed tomography revealed a diffuse micronodular pattern and a ‘tree-in-bud’ pattern in the lower lung zones. Transbronchial biopsy showed features consistent with bronchiolitis obliterans organizing pneumonia (BOOP. After an initially difficult clinical course, the patient responded well to long term treatment with corticosteroids, including improvement of air flow obstruction. This case illustrates a variant of BOOP characterized by a comparatively acute onset, a component of proliferative bronchiolitis, an obstructive rather than restrictive pattern of pulmonary function testing and good clinical response to corticosteroid therapy.

  4. Clinical and radiological features of bronchiolitis obliterans in children

    International Nuclear Information System (INIS)

    Objective: To study the value of chest radiograph and thin-section computed tomography (CT) in diagnosis bronchiolitis obliterans in children, and to determine clinical view of obliterative bronchiolitis in children. Methods: We identified 12 infants, 10 boys, and 2 girls (age range, 5 month to 11 years) with clinical confirmation of bronchiolitis obliterans. Three cases were after Steven-Johnson syndrome, 8 were post-infection (2 adenovirus, 2 measles and 1 Pseudomonas aeruginosa infection, 3 cases were unknown etiology infection); The symptoms lasted for at least 6 weeks. One case had lung ventilation nuclear scan. We evaluated individual bronchoscopy, pulmonary function test, chest radiograph and thin- section CT features and their characteristic appearance. Results: All cases had typical clinical characteristics and pulmonary function testing results that were consistent with nonreversible small airways obstruction. One case had lung ventilation nuclear scan illustrated absent and reduced ventilation of the right lower lobe. Nine cases who underwent bronchoscopy were chronic endobronchial inflammation. Three children had transbronchial biopsy and 1 patient who underwent open pulmonary biopsies were uncertain of histological diagnosis. Chest radiography showed hyperinflation in 8 cases; peribronchial thickening in 6 cases; consolidation/atelectasia in 6 cases; unilateral hyperlucency of a small/normal-sized lung in 4 cases. Thin-section CT/HRCT features included: mosaic perfusion pattern, decreased lung attenuation in 11 cases, pulmonary vascular attenuation in 10 cases; bronchial dilatation in 7 cases; bronchial wall thickening in 9 cases; unilateral hyperlucency of a small/normal-sized lung in 5 cases; consolidation in 6 cases; nodular in 3 cases; mucoid impaction in 5 cases. Conclusions: In our study, correct diagnoses of bronchiolitis obliterans in children were made more special with thin-section CT than with chest radiographs. The diagnosis of BO in

  5. Bronchiolitis obliterans from exposure to incinerator fly ash.

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    Boswell, R T; McCunney, R J

    1995-07-01

    Inhalation of toxic substances in the workplace can result in a variety of respiratory disorders. One relatively rare sequela of the inhalation of toxic fumes is bronchiolitis obliterans, a condition characterized by fibrosis and narrowing of the small airways. Several substances have been reported to cause bronchiolitis obliterans, including ammonia, chlorine, hydrogen fluoride, hydrogen sulfide, nitrogen dioxide, ozone, phosgene, and other irritant fumes. Little has been reported on the pulmonary effects of fly ash produced by the incineration of coal and oil. We report a case of bronchiolitis obliterans with a component of partially reversible airway obstruction in a 39-year-old male occupationally exposed to incinerator fly ash. PMID:7552470

  6. Bronchiolitis Obliterans (BO: HRCT findings in 20 patients

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    Youssriah Yahia Sabri

    2014-03-01

    Conclusion: High-resolution CT is currently superior as an imaging modality in diagnosis of bronchiolitis obliterans, however, diagnosis of BO requires exclusion of other causes of chronic airway obstruction.

  7. Nitrofurantoin-associated bronchiolitis obliterans organizing pneumonia: Report of a case

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    Fenton, Mark E; Rani Kanthan; Donald W Cockcroft

    2008-01-01

    Bronchiolitis obliterans organizing pneumonia due to nitrofurantoin has rarely been reported and is associated with poor outcomes. A case of nitrofurantoin-associated bronchiolitis obliterans organizing pneumonia responsive to drug withdrawal and corticosteroids is presented.

  8. Advances in Understanding Bronchiolitis Obliterans After Lung Transplantation.

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    Verleden, Stijn E; Sacreas, Annelore; Vos, Robin; Vanaudenaerde, Bart M; Verleden, Geert M

    2016-07-01

    Bronchiolitis obliterans syndrome (BOS) remains a major complication after lung transplantation, causing significant morbidity and mortality in a majority of recipients. BOS is believed to be the clinical correlate of chronic allograft dysfunction, and is defined as an obstructive pulmonary function defect in the absence of other identifiable causes, mostly not amenable to treatment. Recently, it has become clear that BOS is not the only form of chronic allograft dysfunction and that other clinical phenotypes exist; however, we focus exclusively on BOS. Radiologic findings typically demonstrate air trapping, mosaic attenuation, and hyperinflation. Pathologic examination reveals obliterative bronchiolitis lesions and a pure obliteration of the small airways (< 2 mm), with a relatively normal surrounding parenchyma. In this review, we highlight recent advances in diagnosis, pathologic examination, and risk factors, such as microbes, viruses, and antibodies. Although the pathophysiological mechanisms remain largely unknown, we review the role of the airway epithelium and inflammation and the various experimental animal models. We also clarify the clinical and therapeutic implications of these findings. Although significant progress has been made, the exact pathophysiological mechanisms and adequate therapy for posttransplantation BOS remain unknown, highlighting the need for further research to improve long-term posttransplantation BOS-free and overall survival. PMID:27212132

  9. The number of FoxP3+ cells in transbronchial lung allograft biopsies does not predict bronchiolitis obliterans syndrome within the first five years after transplantation

    DEFF Research Database (Denmark)

    Krustrup, Dorrit; Iversen, Martin; Martinussen, Torben;

    2015-01-01

    Background: An important limitation to the success of lung transplantation is the development of bronchiolitis obliterans syndrome (BOS). It has been hypothesized that regulatory T lymphocytes (Tregs) are related to the risk of BOS. We aim to evaluate whether the number of forkhead box P3 (FoxP3......+) cells/mm2 in lung allograft biopsies is a predictor of long-term outcome. Materials and Methods: A total of 58 consecutive lung transplant patients were included in the study. For 233 routine surveillance biopsy samples, the numbers of FoxP3+ cells/mm2 were assessed by immunohistochemical staining with...... antibodies against FoxP3. BOS scores were calculated for the first five yr after transplantation. Results: We determined that acute rejection was related to the time elapsed from transplantation to BOS with hazard ratios of 3.18 (p = 0.02) and 3.73 (p = 0.04) when comparing the levels of acute rejection...

  10. [Bronchiolitis obliterans with organized pneumonia: apropos 2 new cases].

    Science.gov (United States)

    Fernández Vázquez, E; Zamarrón Sanz, C; Palacios Bartolomé, A; Valle Vázquez, J M; Alvarez Dobaño, J M; Tumbeiro Novoa, M

    1992-12-01

    We present two cases of bronchiolitis obliterans organizing pneumonia (BOOP) with different clinical and radiological characteristics. In one case, the chest X-ray demonstrated bilateral migrating infiltrates. The pathology showed bronchiolar and intra-alveolar occupation by granulation tissue in both cases. It should be noted that both patients responded well to treatment with prednisone although alterations in gas exchange persisted in one case. PMID:1486169

  11. Thionyl-chloride-induced lung injury and bronchiolitis obliterans

    Energy Technology Data Exchange (ETDEWEB)

    Konichezky, S.; Schattner, A.; Ezri, T.; Bokenboim, P.; Geva, D. (Kaplan Hospital, Rehovot (Israel))

    1993-09-01

    Thionyl-chloride (TCl) is used in the manufacture of lithium batteries, producing SO2 and HCl fumes on contact with water. We report two cases of accidental TCl exposure resulting in lung injury that may vary from a relatively mild and reversible interstitial lung disease to a severe form of bronchiolitis obliterans causing, after a latent period, an acute/chronic respiratory failure as well as other complications (spontaneous pneumothorax and bronchopleural fistula), previously unreported in TCl fume inhalation.

  12. Serum Krebs Von Den Lungen-6 as a Biomarker for Early Detection of Bronchiolitis Obliterans Syndrome in Children Undergoing Allogeneic Stem Cell Transplantation.

    Science.gov (United States)

    Gassas, Adam; Schechter, Tal; Krueger, Joerg; Craig-Barnes, Hayley; Sung, Lillian; Ali, Muhammad; Dell, Sharon; Egeler, R Maarten; Zaidman, Irina; Palaniyar, Nades

    2015-08-01

    Bronchiolitis obliterans syndrome (BOS) is a devastating complication after allogeneic stem cell transplantation (allo-SCT). Early identification of high-risk patients is pivotal for success. Lung proteins, KL-6, CCSP, SP-A, and SP-D, measured in the serum may identify high-risk patients for BOS earlier than pulmonary function tests (PFTs) can identify changes or clinical symptoms. Lung proteins were measured in patients' serum at baseline and at 1, 3, 6, 9, 12, 18, and 24 months after transplantation along with history, clinical examination, and PFTs. Serum levels of lung proteins were also measured in healthy control subjects. The primary endpoint was the development of BOS confirmed by pathological biopsy or National Institutes of Health criteria. Between September 2009 and September 2011, 39 patients were enrolled. Six children developed BOS at a median time of 200 days (range, 94 to 282). KL-6 levels were low in control subjects, at a median of .1 U/mL (range, .1 to 1.5). Pre-SCT and 1-month KL-6 levels were significantly higher in surviving patients who developed BOS (n = 6) versus those who did not (n = 18) (pre-SCT: mean, 32.6 U/mL [IQR, 9.7 to 89.3] versus 5.8 U/mL [IQR, 2.1 to 12.6], P = .03; at 1 month: mean, 52.5 U/mL [IQR, 20.2 to 121.3] versus 11.4 U/mL [IQR, 5.7 to 36.0], P = .04). Three- and 6-month KL-6 levels continued to be higher in BOS group but were not statistically significant. CCSP, SP-A, and SP-D were not predictive. KL-6 measured in the serum of children receiving allo-SCT may identify patients at high risk for the development of BOS. These patients will benefit from intensive surveillance protocol and early therapy before irreversible lung damage. PMID:25963919

  13. Bronchiolitis obliterans organizing pneumonia after radiation therapy for lung cancer: a case report.

    Science.gov (United States)

    Falcinelli, Lorenzo; Bellavita, Rita; Rebonato, Alberto; Chiari, Rita; Vannucci, Jacopo; Puma, Francesco; Aristei, Cynthia

    2015-01-01

    Bronchiolitis obliterans organizing pneumonia (BOOP), also known as cryptogenic organizing pneumonia, has mainly been described in patients with breast cancer who received radiotherapy after breast-conserving surgery. In this rare case, a 70-year-old man with left apical squamous lung cancer developed BOOP after radiotherapy and only one cycle of concomitant chemotherapy.This case report draws attention to the development of this syndrome in the unusual setting of lung cancer, advising prompt steroid treatment when diagnostic images reveal the characteristic signs of the disease. PMID:25908030

  14. Bronchiolitis obliterans organizing pneumonia in patients with autoimmune rheumatic diseases.

    Science.gov (United States)

    Rojas, Carmen Maria Lara; Borella, Elisabetta; Palma, Lavinia; Ragozzino, Silvio; De Ramón, Enrique; Gomez-Huelgas, Ricardo; Punzi, Leonardo; Doria, Andrea

    2015-02-01

    Bronchiolitis obliterans organizing pneumonia (BOOP) is defined by buds of granulation tissue within lung distal airspaces. The diagnosis requires the histopathologic evidence of organizing pneumonia along with a suggestive clinical and radiographic pattern. This disorder is characterized by a good response to corticosteroids and an excellent prognosis. It can occur in association with a broad spectrum of clinical conditions or can be isolated, in this last case named cryptogenic organizing pneumonia. We searched for BOOP in patients with autoimmune rheumatic diseases (ARD) in the literature, and we found 32 well-documented cases. We reported here demographic features, manifestations, treatment and outcome of patients with BOOP associated with ARD. Notably, BOOP can be the presenting feature in some patients with ARD; thus, a close follow-up of patients with BOOP is recommended. PMID:25480740

  15. Bronchiolitis obliterans organizing pneumonia associated with achalasia: A case report.

    Science.gov (United States)

    Malek, Farhad; Nobakhat, Hossein; Hemmati, Hamidreza

    2016-01-01

    There is little mention in the literature about achalasia as an etiologic factor of Bronchiolitis obliterans organizing pneumonia (BOOP). In this study, a case of BOOP, which appeared to be secondary to achalasia is reported. A 35 years old man present with nonproductive cough, chills and fever from two month ago. Due to permanent consolidation in mid zone of right lung and unresponsive to antibiotics, transthoracic needle biopsy was done that showed BOOP. Due to esophageal dilatation in chest computerized tomography (CT) scan, endoscopy and esophagogram was done that showed achalasia. After surgery and a course of corticosteroid the patient condition became well. This report demonstrates that achalasia may be associated with BOOP. PMID:27408784

  16. Bronchiolitis obliterans organizing pneumonia: experience at three hospitals in Riyadh

    International Nuclear Information System (INIS)

    Because reports of bronchiolitis obliterans organizing pneumonia (BOOP) are lacking from the Middle East, we conducted a retrospective review of all histopathologically proven cases of BOOP over 10-year period at three tertiary care hospitals in Riyadh and describe the clinical features and outcome. Charts at the three hospitals were searched using a specific code for BOOP or cryptogenic organizing pneumonia (COP). Lung specimens have to show histological proof of BOOP with a compatible clinical picture. Chest radiographs and high resolution CT scans were reviewed. Twenty cases of biopsy-proven BOOP had well documented clinical and radiograph data. There were 11 males and 9 females (mean age 58 years; range 42-78). The clinical presentation f BOOP was acute or subacute pneumonia-like illness with cough (85%), fever (70%) dyspnea, (85%) and crackles (80%). The most frequent radiological pattern was a bilateral alveolar infiltrate. The most common abnormality on pulmonary function testing (n=14) was a restrictive pattern (11 patients). Most patients (70%) had no underlying cause (idiopathic BOOP). Other associations included thyroid cancer, rheumatoid arthritis, syphilis and Wegner's granulomatosis. Ten patients (50%) had a complete response to steroids, 6 (30%) had a partial response and 3 (15.8) with secondary BOOP had rapid progressive respiratory failure and died. The clinical presentation of BOOP in our patients is similar to other reported series. A favorable outcome occurs in the majority of cases. However, BOOP may occasionally be associated with a poor prognosis, particularly when associated with an underlying disease. (author)

  17. Biopsy-verified bronchiolitis obliterans and other noninfectious lung pathologies after allogeneic hematopoietic stem cell transplantation

    DEFF Research Database (Denmark)

    Uhlving, Hilde Hylland; Andersen, Claus B; Christensen, Ib Jarle;

    2015-01-01

    Bronchiolitis obliterans (BO) is a serious complication of allogeneic hematopoietic stem cell transplantation (HSCT). Lung biopsy is the gold standard for diagnosis. This study describes the course of BO and assesses the congruity between biopsy-verified BO and a modified version of the National...... Institutes of Health's consensus criteria for BO syndrome (BOS) based exclusively on noninvasive measures. We included 44 patients transplanted between 2000 and 2010 who underwent lung biopsy for suspected BO. Of those, 23 were diagnosed with BO and 21 presented other noninfectious pulmonary pathologies...... vital capacity, and maximal mid-expiratory flow throughout follow-up, but there was no difference in the change in pulmonary function from the time of lung biopsy. The BO diagnosis was not associated with poorer overall survival. Fifty-two percent of patients with biopsy-verified BO and 24% of patients...

  18. Bronchiolitis obliterans organizing pneumonia: Pathogenesis, clinical features, imaging and therapy review

    Directory of Open Access Journals (Sweden)

    Al-Ghanem Sara

    2008-01-01

    Full Text Available Bronchiolitis obliterans organizing pneumonia (BOOP was first described in the early 1980s as a clinicopathologic syndrome characterized symptomatically by subacute or chronic respiratory illness and histopathologically by the presence of granulation tissue in the bronchiolar lumen, alveolar ducts and some alveoli, associated with a variable degree of interstitial and airspace infiltration by mononuclear cells and foamy macrophages. Persons of all ages can be affected. Dry cough and shortness of breath of 2 weeks to 2 months in duration usually characterizes BOOP. Symptoms persist despite antibiotic therapy. On imaging, air space consolidation can be indistinguishable from chronic eosinophilic pneumonia (CEP, interstitial pneumonitis (acute, nonspecific and usual interstitial pneumonitis, neoplasm, inflammation and infection. The definitive diagnosis is achieved by tissue biopsy. Patients with BOOP respond favorably to treatment with steroids.

  19. Efficacy of pulse methylprednisolone in a pediatric case of postinfectious bronchiolitis obliterans.

    Science.gov (United States)

    Tanou, Kalliopi; Xaidara, Athina; Kaditis, Athanasios G

    2015-05-01

    Postinfectious bronchiolitis obliterans is a chronic incapacitating disease with persistent airway inflammation. However, the efficacy of systemic corticosteroids has never been studied systematically. In the presented case, serial spirometry, plethysmography measurements, and nocturnal oximetry demonstrated progressive decline in lung hyperinflation and air-trapping and improvement in expiratory function and nocturnal oxygenation during and after six three-day courses of high-dose methylprednisolone (30 mg/kg/day). At four months post treatment, most gain in lung function was lost suggesting the need for sustained immunosuppression. Randomized, controlled trials using serial plethysmography measurements, spirometry, and nocturnal oximetry could provide evidence for the management of postinfectious bronchiolitis obliterans. PMID:25682945

  20. Improving survival outcomes in lung transplant recipients through early detection of bronchiolitis obliterans: Daily home spirometry versus standard pulmonary function testing

    OpenAIRE

    Robson, Kevin S; West, Andrew J.

    2014-01-01

    BACKGROUND: Long-term lung transplant success is limited by bronchiolitis obliterans syndrome (BOS), a form of chronic allograft rejection that manifests in the majority of patients by five years post-transplant. Frequent monitoring of pulmonary function measurements through the use of daily home spirometry may have the capability to detect the onset of BOS sooner than standard pulmonary function testing. Early detection of BOS would confer a treatment advantage that may improve survival outc...

  1. Are we near to an effective drug treatment for bronchiolitis obliterans?

    OpenAIRE

    Verleden, Geert; Vos, Robin; Dupont, Lieven; Van Raemdonck, Dirk; Vanaudenaerde, Bart; Verleden, Stijn

    2014-01-01

    Lung transplantation remains the only effective therapeutic option for well-selected patients with end-stage (cardio) pulmonary diseases such as emphysema, cystic fibrosis, lung fibrosis and pulmonary arterial hypertension. Although the results have improved lately, the long-term survival is still far behind other organ transplantations. This is mainly due to the development of chronic lung allograft dysfunction (CLAD), with bronchiolitis obliterans (BO) being the most frequent manifestation ...

  2. Increased Respiratory Disease Mortality at a Microwave Popcorn Production Facility with Worker Risk of Bronchiolitis Obliterans

    OpenAIRE

    Halldin, Cara N; Eva Suarthana; Kathleen B Fedan; Yi-Chun Lo; George Turabelidze; Kathleen Kreiss

    2013-01-01

    BACKGROUND: Bronchiolitis obliterans, an irreversible lung disease, was first associated with inhalation of butter flavorings (diacetyl) in workers at a microwave popcorn company. Excess rates of lung-function abnormalities were related to cumulative diacetyl exposure. Because information on potential excess mortality would support development of permissible exposure limits for diacetyl, we investigated respiratory-associated mortality during 2000-2011 among current and former workers at this...

  3. Bronchiolitis obliterans after allo-SCT: clinical criteria and treatment options

    DEFF Research Database (Denmark)

    Uhlving, H H; Buchvald, F; Heilmann, C J;

    2012-01-01

    Bronchiolitis obliterans (BO) following allogeneic haematopoietic SCT (HSCT) is a serious complication affecting 1.7-26% of the patients, with a reported mortality rate of 21-100%. It is considered a manifestation of chronic graft-versus-host disease, but our knowledge of aetiology and pathogenes...... treatment options for BO and presents the treatment results with HDPM in a paediatric national HSCT-cohort.Bone Marrow Transplantation advance online publication, 29 August 2011; doi:10.1038/bmt.2011.161....

  4. Bilateral pulmonary nodules in an adult patient with bronchiolitis obliterans-organising pneumonia

    OpenAIRE

    Kopanakis, Antonios; Golias, Christos; Patentalakis, Michalis; Mermigkis, Christos; Charalabopoulos, Alexandros; Peschos, Dimitrios; Batistatou, Anna; Charalabopoulos, Konstantinos

    2009-01-01

    A 58-year-old male ex-smoker was admitted to hospital because of nodular infiltrates on chest x rays. He was complaining of fatigue, dyspnoea with exertion, low grade fever and weight loss. Physical examination was unremarkable. Bronchoscopy was inconclusive but revealed endobronchial lesions of chronic active inflammation. The diagnosis of cryptogenic organising pneumonitis bronchiolitis obliterans-organising pneumonia (COP-BOOP) was established by open lung biopsy. Proliferative bronchiolit...

  5. Ventilation/Perfusion Scintigraphy in Children with Post-Infectious Bronchiolitis Obliterans: A Pilot Study

    OpenAIRE

    Xie, Bo-Qia; Wang, Wei; Zhang, Wen-Qian; Guo, Xin-Hua; Yang, Min-Fu; Wang, Li; He, Zuo-Xiang; Tian, Yue-Qin

    2014-01-01

    Purpose Childhood post-infectious bronchiolitis obliterans (BO) is an infrequent lung disease leading to narrowing and/or complete obliteration of small airways. Ventilation and perfusion (V/Q) scan can provide both regional and global pulmonary information. However, only few retrospective researches investigating post-infectious BO involved V/Q scan, the clinical value of this method is unknown. This preliminary prospective study was aimed to evaluate the correlation of V/Q scan with disease...

  6. Oxygen-sensitive {sup 3}He-MRI in bronchiolitis obliterans after lung transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Gast, Klaus K. [Klinikum der Johannes Gutenberg-Universitaet, Klinik und Poliklinik fuer diagnostische und interventionelle Radiologie, Mainz (Germany); Biedermann, Alexander [Klinikum der Johannes Gutenberg-Universitaet, 3. Medizinische Klinik, Pulmonologie, Mainz (Germany); Herweling, Annette [Klinikum der Johannes Gutenberg-Universitaet, Klinik fuer Anaesthesiologie, Mainz (Germany); Schreiber, Wolfgang G. [Klinikum der Johannes Gutenberg-Universitaet, Klinik und Poliklinik fuer diagnostische und interventionelle Radiologie, MR-Physik, Mainz (Germany); Schmiedeskamp, Joerg [Max-Planck-Institut fuer Polymerforschung, Mainz (Germany); Mayer, Eckhard [Klinikum der Johannes Gutenberg-Universitaet, Klinik fuer Herz-, Thorax- und Gefaesschirurgie, Mainz (Germany); Heussel, Claus P. [Abteilung fuer Radiologie, Thoraxklinik Heidelberg, Heidelberg (Germany); Markstaller, Klaus; Eberle, Balthasar [Inselspital/Universitaetsspital, Klinik fuer Anaesthesiologie, Bern (Switzerland); Kauczor, Hans-Ulrich [Radiologie, Deutsches Krebsforschungszentrum (DKFZ), Heidelberg (Germany)

    2008-03-15

    Oxygen-sensitive {sup 3}He-MRI was studied for the detection of differences in intrapulmonary oxygen partial pressure (pO{sub 2}) between patients with normal lung transplants and those with bronchiolitis obliterans syndrome (BOS). Using software developed in-house, oxygen-sensitive {sup 3}He-MRI datasets from patients with normal lung grafts (n = 8) and with BOS (n = 6) were evaluated quantitatively. Datasets were acquired on a 1.5-T system using a spoiled gradient echo pulse sequence. Underlying diseases were pulmonary emphysema (n = 10 datasets) and fibrosis (n = 4). BOS status was verified by pulmonary function tests. Additionally, {sup 3}He-MRI was assessed blindedly for ventilation defects. Median intrapulmonary pO{sub 2} in patients with normal lung grafts was 146 mbar compared with 108 mbar in patients with BOS. Homogeneity of pO2 distribution was greater in normal grafts (standard deviation pO2 34 versus 43 mbar). Median oxygen decrease rate during breath hold was higher in unaffected patients (-1.75 mbar/s versus -0.38 mbar/s). Normal grafts showed fewer ventilation defects (5% versus 28%, medians). Oxygen-sensitive {sup 3}He-MRI appears capable of demonstrating differences of intrapulmonary pO2 between normal lung grafts and grafts affected by BOS. Oxygen-sensitive {sup 3}He-MRI may add helpful regional information to other diagnostic techniques for the assessment and follow-up of lung transplant recipients. (orig.)

  7. Biopsy-verified bronchiolitis obliterans and other noninfectious lung pathologies after allogeneic hematopoietic stem cell transplantation.

    Science.gov (United States)

    Uhlving, Hilde Hylland; Andersen, Claus B; Christensen, Ib Jarle; Gormsen, Magdalena; Pedersen, Karen Damgaard; Buchvald, Frederik; Heilmann, Carsten; Nielsen, Kim Gjerum; Mortensen, Jann; Moser, Claus; Sengeløv, Henrik; Müller, Klaus Gottlob

    2015-03-01

    Bronchiolitis obliterans (BO) is a serious complication of allogeneic hematopoietic stem cell transplantation (HSCT). Lung biopsy is the gold standard for diagnosis. This study describes the course of BO and assesses the congruity between biopsy-verified BO and a modified version of the National Institutes of Health's consensus criteria for BO syndrome (BOS) based exclusively on noninvasive measures. We included 44 patients transplanted between 2000 and 2010 who underwent lung biopsy for suspected BO. Of those, 23 were diagnosed with BO and 21 presented other noninfectious pulmonary pathologies, such as cryptogenic organizing pneumonia, diffuse alveolar damage, interstitial pneumonia, and nonspecific interstitial fibrosis. Compared with patients with other noninfectious pulmonary pathologies, BO patients had significantly lower values of forced expiratory volume in 1 second (FEV1), FEV1/forced vital capacity, and maximal mid-expiratory flow throughout follow-up, but there was no difference in the change in pulmonary function from the time of lung biopsy. The BO diagnosis was not associated with poorer overall survival. Fifty-two percent of patients with biopsy-verified BO and 24% of patients with other noninfectious pulmonary pathology fulfilled the BOS criteria. Pathological BO diagnosis was not superior to BOS criteria in predicting decrease in pulmonary function beyond the time of biopsy. A lung biopsy may provide a characterization of pathological patterns that can extend our knowledge on the pathophysiology of HSCT-related lung diseases. PMID:25498923

  8. [Bronchiolitis obliterans with organizing pneumonia associated with idiopathic thrombocytopenic purpura].

    Science.gov (United States)

    Presas, J L; Piriz, J; Serra, S L; Paz, E D; Allievi, A; Kartin, D; Olmedo, G

    1998-01-01

    We report a case of a 35 year-old woman with idiopathic thrombocytopenic purpura (ITP) who, under treatment with immunosuppressive drugs, developed bilateral interstitial pulmonary disease. Previously she had been splenectomized and treated with corticosteroids and cyclosporin. During the clinical course, the patient developed alterations of the hepatogram and presented a positive serology for Epstein-Barr virus. The lung biopsy showed the histologic pattern of obliterative bronchiolitis, interstitial inflammatory infiltration and intraalveolar pneumonia (BOOP). We could not find in the literature a previous report in which ITP was associated with BOOP. Of interest was the spontaneous remission of the pulmonary disease after suppression of cyclosporin and positive serology for Epstein-Barr virus. PMID:9674210

  9. Radiological findings in seven patients of bronchiolitis obliterans with organized pneumonia

    International Nuclear Information System (INIS)

    We present seven patients, five women and two men, between 40 and 71 years old, who were diagnosed between 1983 and 1993 as having bronchiolitis obliterans with organized pneumonia (BOOP). Five of the patients were treated at Hospital Virgen del Rocio and two at Hospital Nuestra Se=A4ora de Valme. The histological, clinical and radiological features are described. All the patients responded favorable to corticosteroid therapy, and there have been no recurrences. We point out the predominance of patchy alveolar infiltrates which, in three cases proved to be migratory. We analyze the computerized tomography (CT) findings in three patients, comparing our observations with those reported in the literature. (Author)

  10. Bronchiolitis obliterans organizing pneumonia (BOOP) after thoracic radiotherapy for breast carcinoma

    International Nuclear Information System (INIS)

    Common complications of thoracic radiotherapy include esophagitis and radiation pneumonitis. However, it is important to be aware of uncommon post-radiotherapy complications such as bronchiolitis obliterans organizing pneumonia (BOOP). We report on two patients with carcinoma of the breast who developed an interstitial lung disease consistent with BOOP. BOOP responds to treatment with corticosteroids and the prognosis is generally good despite of the need for long-term administration of corticosteroids as relapses can occur during tapering of steroids. This report provides guidelines for the evaluation and treatment of patients with pulmonary infiltrates after radiotherapy

  11. Persistent high BAL fluid granulocyte activation marker levels as early indicators of bronchiolitis obliterans after lung transplant.

    Science.gov (United States)

    Riise, G C; Andersson, B A; Kjellström, C; Martensson, G; Nilsson, F N; Ryd, W; Scherstén, H

    1999-11-01

    The major cause of mortality in the long-term in lung transplant recipients is chronic rejection. This is a fibroproliferative process in the small airways leading to obliterative bronchiolitis and progressive loss of lung function, both constituting the clinical entity bronchiolitis obliterans syndrome (BOS). Granulocyte activation has been implicated as one factor behind BOS. Granulocyte markers in bronchoalveolar lavage (BAL) fluid were prospectively and longitudinally studied in order to identify possible association with BOS. BAL fluid from 266 bronchoscopy procedures performed in twelve single lung, eight bilateral lung and five heart/lung transplant recipients were analysed. The majority (19 of 25) were studied for a period of 2 yrs after surgery. Myeloperoxidase (MPO), eosinophil cationic protein (ECP) and interleukin-8 (IL-8) levels were used as indirect markers of activation and attraction of granulocytes. Five patients developed BOS. Ninety-eight episodes of acute rejection, nine of bacterial infection, 19 of cytomegalovirus pneumonitis, nine of Pneumocystis carinii infection, two of aspergillus infection and two of respiratory syncytial virus infection were diagnosed. BOS patients had significantly higher mean levels of MPO, ECP and IL-8 compared to patients without BOS, irrespective of acute rejection status. Over time, the five patients with BOS had significantly elevated BAL fluid levels of MPO and ECP as well as neutrophil percentages, and in four patients this increase preceded the clinical diagnosis of BOS by several months. Elevated bronchoalveolar lavage fluid neutrophil percentage as well as levels of the granulocyte activation markers myeloperoxidase and eosinophil cationic protein appear to be early signs of development of BOS in lung transplant recipients. PMID:10596701

  12. Increased respiratory disease mortality at a microwave popcorn production facility with worker risk of bronchiolitis obliterans.

    Directory of Open Access Journals (Sweden)

    Cara N Halldin

    Full Text Available BACKGROUND: Bronchiolitis obliterans, an irreversible lung disease, was first associated with inhalation of butter flavorings (diacetyl in workers at a microwave popcorn company. Excess rates of lung-function abnormalities were related to cumulative diacetyl exposure. Because information on potential excess mortality would support development of permissible exposure limits for diacetyl, we investigated respiratory-associated mortality during 2000-2011 among current and former workers at this company who had exposure to flavorings and participated in cross-sectional surveys conducted between 2000-2003. METHODS: We ascertained workers' vital status through a Social Security Administration search. Causes of death were abstracted from death certificates. Because bronchiolitis obliterans is not coded in the International Classification of Disease 10(th revision (ICD-10, we identified respiratory mortality decedents with ICD-10 codes J40-J44 which encompass bronchitis (J40, simple and mucopurulent chronic bronchitis (J41, unspecified chronic bronchitis (J42, emphysema (J43, and other chronic obstructive pulmonary disease (COPD (J44. We calculated expected number of deaths and standardized mortality ratios (SMRs with 95% confidence intervals (CI to determine if workers exposed to diacetyl experienced greater respiratory mortality than expected. RESULTS: We identified 15 deaths among 511 workers. Based on U.S. population estimates, 17.39 deaths were expected among these workers (SMR = 0.86; CI:0.48-1.42. Causes of death were available for 14 decedents. Four deaths among production and flavor mixing workers were documented to have a multiple cause of 'other COPD' (J44, while 0.98 'other COPD'-associated deaths were expected (SMR = 4.10; CI:1.12-10.49. Three of the 4 'other COPD'-associated deaths occurred among former workers and workers employed before the company implemented interventions reducing diacetyl exposure in 2001. CONCLUSION: Workers

  13. Two cases of bronchiolitis obliterans organizing pneumonia (BOOP) induced radiotherapy after surgery of breast cancer

    International Nuclear Information System (INIS)

    We report two cases of bronchiolitis obliterans organizing pneumonia (BOOP) induced radiotherapy after surgery of breast cancer. One of the patients was a 58-year-old woman. She underwent a conserving surgery for bilateral breast cancers, and received radiation therapy to the remaining part of bilateral breasts. Two months after the termination of irradiation, cough, fever and general fatigue developed. We clinically diagnosed this case as BOOP after radiation therapy. After initiation of oral steroid therapy, the clinical symptoms and radiographic findings disappeared. Another patient was a 57 year-old woman. She underwent radical mastectomy for right breast cancer. A month after the operation, she suffered from local recurrence, so radiation therapy to the thoracic wall was performed. After irradiation, resection of the thoracic wall lesion was performed because of malignancy from local skin biopsy specimen. Two months after the termination of irradiation, cough, dyspnea and fever developed. We clinically diagnosed this case as radiation-induced BOOP by bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) findings. After an initiation of steroid therapy, the clinical symptoms and radiographic findings disappeared. It is important to be aware of BOOP as a complication in the patient who was given radiation after surgery of breast cancer. (author)

  14. Sauropus androgynus (L. Merr. Induced Bronchiolitis Obliterans: From Botanical Studies to Toxicology

    Directory of Open Access Journals (Sweden)

    Hamidun Bunawan

    2015-01-01

    Full Text Available Sauropus androgynus L. Merr. is one of the most popular herbs in South Asia, Southeast Asia, and China where it was known as a slimming agent until two outbreaks of pulmonary dysfunction were reported in Taiwan and Japan in 1995 and 2005, respectively. Several studies described that the excessive consumption of Sauropus androgynus could cause drowsiness, constipation, and bronchiolitis obliterans and may lead to respiratory failure. Interestingly, this herb has been used in Malaysia and Indonesia in cooking and is commonly called the “multigreen” or “multivitamin” plant due to its high nutritive value and inexpensive source of dietary protein. The plant is widely used in traditional medicine for wound healing, inducing lactation, relief of urinary disorders, as an antidiabetic cure and also fever reduction. Besides these medicinal uses, the plant can also be used as colouring agent in food. This review will explore and compile the fragmented knowledge available on the botany, ethnobotany, chemical constitutes, pharmacological properties, and toxicological aspects of this plant. This comprehensive review will give readers the fundamental, comprehensive, and current knowledge regarding Sauropus androgynus L. Merr.

  15. Cell therapy in the treatment of bronchiolitis obliterans in a murine model

    Directory of Open Access Journals (Sweden)

    Julio de Oliveira Espinel

    2015-06-01

    Full Text Available OBJECTIVE: To evaluate the importance of stem cells derived from adipose tissue in reducing graft inflammation in a murine model of allogeneic heterotopic tracheal transplant.METHODS: We performed a heterotopic tracheal allografting in dorsal subcutaneous pouch and systemically injected 5x105 mesenchymal stem cells derived from adipose tissue. The animals were divided into two groups according to the time of sacrifice: T7 and T21. We also carried out histological analysis and digital morphometry.RESULTS: The T7 animals treated with cell therapy had median obstructed graft area of 0 versus 0.54 of controls (p = 0.635. The treated T21 subjects had median obstructed graft area of 0.25 versus 0 in controls (p = 0.041.CONCLUSION: The systemically injected cell therapy in experimental murine model of bronchiolitis obliterans did not reduce the severity of the allograft inflammation in a statistically significant way in seven days; Conversely, in 21 days, it increased the allograft inflammatory process.

  16. Can acute interstitial pneumonia be differentiated from bronchiolitis obliterans organizing pneumonia by high-resolution CT?

    International Nuclear Information System (INIS)

    In the early stages, clinical and chest radiographic findings of acute interstitial pneumonia (AIP) are often similar to those of bronchiolitis obliterans organizing pneumonia (BOOP). However, patients with AIP have a poor prognosis, while those with BOOP can achieve a complete recovery after corticosteroid therapy. The objective of this study was to identify differences in high-resolution CT (HRCT) findings between the two diseases. The study included 27 patients with AIP and 14 with BOOP who were histologically diagnosed [open-lung biopsy (n=7), autopsy (n=17), transbronchial lung biopsy (n=17)]. The frequency and distribution of various HRCT findings for each disease were retrospectively evaluated. Traction bronchiectasis, interlobular septal thickening, and intralobular reticular opacities were significantly more prevalent in AIP (92.6%, 85.2%, and 59.3%, respectively) than in BOOP (42.9%, 35.7%, and 14.3%, respectively) (p<0.01). Parenchymal nodules and peripheral distribution were more prevalent in BOOP (28.6% and 57.1%, respectively) than in AIP (7.4% and 14.8%, respectively) (p<0.01). Areas with ground-glass attenuation, air-space consolidation, and architectural distortion were common in both AIP and BOOP. For a differential diagnosis of AIP and BOOP, special attention should be given to the following HRCT findings: traction bronchiectasis, interlobular septal thickening, intralobular reticular opacities, parenchymal nodules, pleural effusion, and peripheral zone predominance. (author)

  17. Secondary bronchiolitis obliterans organizing pneumonia during treatment of chronic hepatitis C: role of pegylated interferon alfa-2a

    Directory of Open Access Journals (Sweden)

    Ronaldo Soares Martins

    2012-10-01

    Full Text Available The treatment of chronic hepatitis C has frequent side effects such as cytopenias and neuropsychiatric symptoms. However, pulmonary toxicity associated with interferon is rarely described. This paper describes the clinical case of a 67-year-old female patient with chronic hepatitis C who presented an acute onset of dry cough, dyspnoea, and fever 36 weeks after the use of pegylated interferon alfa-2a and ribavirin. The lung biopsy confirmed the diagnosis of a bronchiolitis obliterans organizing pneumonia (BOOP. Corticotherapy was initiated, with clinical and radiological improvement. This paper aims to advise physicians to this occasional, though severe, adverse event related to hepatitis C virus (HCV treatment.

  18. Bronquiolitis obliterante con neumonía organizada y enfermedad de Crohn Bronchiolitis obliterans organizing pneumonia and Crohn’s disease

    OpenAIRE

    P. Gil-Simón; J. Barrio Andrés; R. Atienza Sánchez; L. Julián Gómez; C. López Represa; A. Caro-Patón

    2008-01-01

    Las manifestaciones extraintestinales respiratorias en la enfermedad inflamatoria intestinal (EII) son excepcionales. Presentamos un caso de bronquiolitis obliterante con neumonía organizada (BONO) en paciente con enfermedad de Crohn, en remisión clínica sin tratamientos farmacológicos.Extraintestinal respiratory manifestations in inflammatory bowel disease (IBD) are rare. We present a case of bronchiolitis obliterans organizing pneumonia (BOOP) in a patient with Crohn's disease, with clinica...

  19. Quantitative computed tomography assessment of graft-versus-host disease-related bronchiolitis obliterans in children: A pilot feasibility study

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Gi [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Seoul (Korea, Republic of); Ajou University Medical Center, Department of Radiology, Ajou University School of Medicine, Suwon (Korea, Republic of); Shin, Hyun Joo; Kim, Myung-Joon; Lee, Mi-Jung [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Seoul (Korea, Republic of); Kim, Yoon Hee; Sohn, Myung Hyun; Kim, Kyung Won [Yonsei University College of Medicine, Department of Pediatrics and Institute of Allergy, Severance Children' s Hospital, Seoul (Korea, Republic of); Lyu, Chuhl Joo [Yonsei University College of Medicine, Department of Pediatric Hematology and Oncology, Severance Children' s Hospital, Seoul (Korea, Republic of)

    2015-10-15

    To suggest a simple method that can quantify air trapping from chest CT in children with graft-versus-host disease (GVHD)-related bronchiolitis obliterans (BO). This institutional review board-approved retrospective study included eight GVHD-related BO patients (age, 6 - 17 years) who underwent both 31 CTs of variable settings and pulmonary function tests (PFT). The attenuation values of lung parenchyma in normal (An) and air trapping (Aa) areas were obtained. Individualized threshold [(An + Aa)/2] and fixed threshold of -950 HU were set for air trapping quantification. Spearman correlation analysis and generalized linear mixed models were used for statistical analysis. The mean value of individualized threshold was -830.2 ± 48.3 HU. The mean air trapping lung volume percentage with individualized threshold and -950 HU were 45.4 ± 18.9 % and 1.4 ± 1.9 %, respectively. The air trapping lung volume percentage with individualized threshold showed a significant negative correlation with the PFT of FEV1/FVC% in all data (γ = -0.795, P <.001) and in the correction of repetition (γ = -0.837, P =.010). We suggest a simple and individualized threshold attenuation setting method for air trapping quantification insusceptible to CT imaging protocols or respiratory phase control in children with GVHD-related BO. (orig.)

  20. Lung disease and ulcerative colitis--mesalazine-induced bronchiolitis obliterans with organizing pneumonia or pulmonary manifestation of inflammatory bowel disease?

    Science.gov (United States)

    Moeser, A; Pletz, M W; Hagel, S; Kroegel, C; Stallmach, A

    2015-09-01

    Ulcerative colitis can be associated with numerous extraintestinal organ manifestations. Pulmonary disease in inflammatory bowel disease (IBD) is supposed to be a rare entity and has to be distinguished from infectious complications and side-effects of medications used in the treatment of IBD. We present the case of a 20-year-old male patient with ulcerative colitis and a 4-week history of respiratory symptoms, malaise, fever and respiratory insufficiency under a medication with mesalazine. Computed tomography showed bilateral subpleural consolidations, bronchoscopy revealed signs of acute bronchitis. The diagnostic work-up ruled out an infectious cause. Under the tentative diagnosis of a mesalazine-induced bronchiolitis obliterans with organizing pneumonia (BOOP) the medication with mesalazine was withdrawn and the patient received a corticosteroid trial. The symptoms quickly improved and prednisone was tapered and stopped after 6 months. Unexpectedly, lung function after complete resolution of respiratory symptoms revealed a residual obstructive ventilatory defect that might be due to an asymptomatic pulmonary manifestation of ulcerative colitis. A review of the literature shows that pulmonary manifestations in IBD as well as pulmonary toxicity of mesalazine might not be as rare as expected and should be included as differential diagnoses in the work-up of respiratory symptoms in patients with IBD. A pragmatic therapeutic approach is reasonable in critically ill patients as it is not always easy to distinguish both entities. PMID:26367026

  1. Quantitative computed tomography assessment of graft-versus-host disease-related bronchiolitis obliterans in children: A pilot feasibility study

    International Nuclear Information System (INIS)

    To suggest a simple method that can quantify air trapping from chest CT in children with graft-versus-host disease (GVHD)-related bronchiolitis obliterans (BO). This institutional review board-approved retrospective study included eight GVHD-related BO patients (age, 6 - 17 years) who underwent both 31 CTs of variable settings and pulmonary function tests (PFT). The attenuation values of lung parenchyma in normal (An) and air trapping (Aa) areas were obtained. Individualized threshold [(An + Aa)/2] and fixed threshold of -950 HU were set for air trapping quantification. Spearman correlation analysis and generalized linear mixed models were used for statistical analysis. The mean value of individualized threshold was -830.2 ± 48.3 HU. The mean air trapping lung volume percentage with individualized threshold and -950 HU were 45.4 ± 18.9 % and 1.4 ± 1.9 %, respectively. The air trapping lung volume percentage with individualized threshold showed a significant negative correlation with the PFT of FEV1/FVC% in all data (γ = -0.795, P <.001) and in the correction of repetition (γ = -0.837, P =.010). We suggest a simple and individualized threshold attenuation setting method for air trapping quantification insusceptible to CT imaging protocols or respiratory phase control in children with GVHD-related BO. (orig.)

  2. [A case of bronchiolitis obliterans organizing pneumonia: diagnostic utility of bronchoalveolar lavage and CT scan].

    Science.gov (United States)

    Hyakudo, T; Yoshii, C; Nikaido, Y; Yokosaki, Y; Nagata, N; Nakata, H; Kido, M

    1994-08-01

    A 54-year-old female was admitted to our hospital because of abnormal shadows on chest X-ray at annual checkup. She complained of dyspnea on exertion. Chest X-ray findings showed an increase in density at the bilateral lower lung fields and unclearness of the silhouette of the heart and the diaphragm. CT scan findings revealed irregular opacities of various density with many small cystic changes and air bronchograms and air bronchiolograms. The pulmonary function test showed restrictive ventilatory disturbance and reduced diffusing capacity. BALF findings revealed an increase in the total cell count, an increase in the percentage of lymphocytes and a decrease in the OKT4+/OKT8+ ratio. TBLB specimen showed infiltration of mononuclear cells in alveolar septa and organizing exudate in alveolar ducts. These findings suggested a diagnosis of BOOP rather than IPF, and an open lung biopsy was performed. Open lung biopsy specimen showed obstructive bronchiolitis with polypoid granulation tissue and thickening of alveolar septa with infiltration of mononuclear cells, and she was diagnosed as having BOOP. She responded well to corticosteroid and is free from any abnormalities on chest X-ray, CT scan and pulmonary function test at present. Analysis of BALF and CT scan findings are useful for the differential diagnosis of BOOP and IPF. PMID:7807756

  3. Associação de bronquiolite obliterante pós-infecciosa e hemossiderose pulmonar na infância Postinfectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in childhood

    Directory of Open Access Journals (Sweden)

    Leonardo Araújo Pinto

    2006-12-01

    Full Text Available Descreve-se uma apresentação rara de bronquiolite obliterante associada a hemossiderose pulmonar, em paciente de nove anos com sintomas respiratórios persistentes iniciados após episódio de bronquiolite aguda grave aos sete meses. Após o episódio agudo, apresentou sintomas respiratórios persistentes, piorando significativamente aos sete anos, quando começou a apresentar dificuldade respiratória em pequenos esforços. A tomografia computadorizada de tórax demonstrou achados compatíveis com bronquiolite obliterante. A biópsia pulmonar a céu aberto demonstrou numerosos macrófagos corados com hemossiderina, além dos achados compatíveis com bronquiolite obliterante. O diagnóstico de hemossiderose pulmonar pode estar ocasionalmente associado a bronquiolite obliterante em crianças com seqüela pós-viral grave.In the present report, we describe an unusual presentation of post-infectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in a nine-year-old boy with persistent respiratory symptoms subsequent to an episode of acute bronchiolitis occurring at the age of seven months. After the episode, the persistent respiratory symptoms worsened significantly, and, by the age of seven, the patient began to have difficulty breathing after minimal exertion. Computed tomography of the chest presented findings consistent with bronchiolitis obliterans. Open lung biopsy revealed numerous hemosiderin-laden macrophages, as well as other findings consistent with bronchiolitis obliterans. Pulmonary hemosiderosis can occasionally be accompanied by bronchiolitis obliterans in children with severe sequelae after an episode of viral infection.

  4. Guía clínica para el diagnóstico y cuidado de niños/adolescentes con bronquiolitis obliterante post-infecciosa, 2009 Clinical guide for diagnosis and care of children and adolescents with post-infectious bronchiolitis obliterans, 2009

    Directory of Open Access Journals (Sweden)

    LUIS E VEGA-BRICEÑO

    2009-01-01

    Full Text Available La bronquiolitis obliterante (BO es un síndrome clínico poco frecuente en niños, caracterizado por la obstrucción crónica al flujo de aire asociado a cambios inflamatorios y distintos grados de fibrosis en la vía aérea pequeña. Si bien existen muchas etiologías, la causa más frecuente se asocia a infecciones respiratorias virales, principalmente adenovirus. No existe un consenso para establecer su diagnóstico; sin embargo, se considera un espectro de síntomas persistentes asociados a un patrón en mosaico, bronquiectasias y atelectasias persistentes. El papel de la biopsia pulmonar ha sido cuestionado por su bajo rendimiento, invasividad y complicaciones. No existe un tratamiento específico por lo que el manejo es soporte. Probablemente la mejor estrategia constituya el empleo de antibióticos en forma agresiva, soporte kinésico y nutricional constante y una precoz rehabilitación pulmonar. Estas guías clínicas representan un esfuerzo multidisciplinario, basado en evidencias actuales para brindar herramientas prácticas para el diagnóstico y cuidado de niños y adolescentes con BO post infecciosa.Bronchiolitis obliterans in children is an infrequent clinical syndrome, characterized by chronic airflow obstruction associated to inflammatory changes and different degrees of fibrosis in the small airways. Etiologies are varied but the most frequent one is the association with viral infections, mainly adenovirus. There is no consensus regarding diagnostic criteria, but a spectrum of persistent symptoms together with a mosaic pattern, bronchiectasis and persistent atelectasis is considered useful. Pulmonary biopsy has been questioned because of its low yield, invasiveness and complications. No specific treatment is available, therefore its treatment is supportive. Probably the best strategy is the aggressive use of antibiotics, constant kinesic and nutritional support and early pulmonary rehabilitation. This clinical guide represents

  5. Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus with unusual manifestations and without detectable autoantibodies

    Directory of Open Access Journals (Sweden)

    Jimena Sanz-Bueno

    2014-01-01

    Full Text Available We describe a patient with paraneoplastic autoimmune multiorgan syndrome (PAMS secondary to a lymphoblastic T- cell lymphoma who presented with a lichenoid dermatitis and vitiligo, later developing bronchiolitis obliterans and autoimmune hepatitis. Notably, he had no detectable autoantibodies. The development of vitiligo and autoimmune hepatic involvement probably indicate a role for cytotoxic T- cell lymphocytes in the pathogenesis of this syndrome.

  6. Xenon ventilation CT using dual-source and dual-energy technique in children with bronchiolitis obliterans: correlation of xenon and CT density values with pulmonary function test results

    International Nuclear Information System (INIS)

    Xenon ventilation CT using dual-source and dual-energy technique is a recently introduced, promising functional lung imaging method. To expand its clinical applications evidence of additional diagnostic value of xenon ventilation CT over conventional chest CT is required. To evaluate the usefulness of xenon ventilation CT using dual-source and dual-energy technique in children with bronchiolitis obliterans (BO). Seventeen children (age 7-18 years; 11 boys) with BO underwent xenon ventilation CT using dual-source and dual-energy technique. Xenon and CT density values were measured in normal and hyperlucent lung regions on CT and were compared between the two regions. Volumes of hyperlucent regions and ventilation defects were calculated with thresholds determined by visual and histogram-based analysis. Indexed volumes of hyperlucent lung regions and ventilation defects were correlated with pulmonary function test results. Effective doses of xenon CT were calculated. Xenon (14.6 ± 6.4 HU vs 26.1 ± 6.5 HU; P 25-75, (γ = -0.68-0.88, P ≤ 0.002). Volume percentages of xenon ventilation defects (35.0 ± 16.4%)] were not significantly different from those of hyperlucent lung regions (38.2 ± 18.6%). However, mismatches between the volume percentages were variable up to 21.4-33.3%. Mean effective dose of xenon CT was 1.9 ± 0.5 mSv. In addition to high-resolution anatomic information, xenon ventilation CT using dual-source and dual-energy technique demonstrates impaired regional ventilation and its heterogeneity accurately in children with BO without additional radiation exposure. (orig.)

  7. Bronquiolite obliterante pós-infecciosa: aspectos clínicos e exames complementares de 48 crianças Post-infectious bronchiolitis obliterans: clinical aspects and complementary testing of 48 children

    Directory of Open Access Journals (Sweden)

    Rosaly Vieira dos Santos

    2004-02-01

    obliterans (BO is variable. OBJECTIVE: Substantiate the clinical characteristics, the evolution and the complementary tests of 48 patients with post infectious bronchiolitis obliterans (BO. METHOD: Observational and retrospective study. Diagnosis of BO was based upon clinical criteria, CT scan findings and exclusion of other diseases. History prior to diagnosis and complementary tests were evaluated as well as initial and final values of oxygen saturation. RESULTS: Mean age of patients at the acute stage of the infectious disease was of 9.6 Thirty two of the patients were male. All were hospitalized during the acute stage, 14 of them (29% in the ICU. Four patients died two years after onset of acute bronchiolitis. During evolution all required emergency care due to exacerbation of the pulmonary condition and 24 (50% were hospitalized, 2 of them in the ICU. The majority continued presenting cough, wheezing, crackles and hyperinflation, however to a lesser degree. Mean of initial arterial saturation was of 89% and final of 92%. The most common infectious agents in the sputum samples were H. influenzae, S. pneumoniae and M. caterrhalis. Increased serum IgM and IgG were found in 9 and 7 patients respectively. The most frequent findings at thorax CT scan were mosaic perfusion, bronchioectasis, atelectasis and bronchial wall thickening. CONCLUSION: Post infectious BO is a chronic and severe disease with persistent symptoms that in general affect infants. Positive serum cultures and increased serum immunoglobulins are suggestive of a chronic infectious inflammatory condition.

  8. Xenon ventilation CT using dual-source and dual-energy technique in children with bronchiolitis obliterans: correlation of xenon and CT density values with pulmonary function test results

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo; Yang, Dong Hyun; Seo, Joon Beom; Chae, Eun Jin; Lee, Jeongjin [University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Asan Medical Center, Songpa-gu, Seoul (Korea); Hong, Soo-Jong; Yu, Jinho; Kim, Byoung-Ju [University of Ulsan College of Medicine, Department of Pediatrics, Asan Medical Center, Seoul (Korea); Krauss, Bernhard [Siemens Medical Solutions AG-Computed Tomography, Forchheim (Germany)

    2010-09-15

    Xenon ventilation CT using dual-source and dual-energy technique is a recently introduced, promising functional lung imaging method. To expand its clinical applications evidence of additional diagnostic value of xenon ventilation CT over conventional chest CT is required. To evaluate the usefulness of xenon ventilation CT using dual-source and dual-energy technique in children with bronchiolitis obliterans (BO). Seventeen children (age 7-18 years; 11 boys) with BO underwent xenon ventilation CT using dual-source and dual-energy technique. Xenon and CT density values were measured in normal and hyperlucent lung regions on CT and were compared between the two regions. Volumes of hyperlucent regions and ventilation defects were calculated with thresholds determined by visual and histogram-based analysis. Indexed volumes of hyperlucent lung regions and ventilation defects were correlated with pulmonary function test results. Effective doses of xenon CT were calculated. Xenon (14.6 {+-} 6.4 HU vs 26.1 {+-} 6.5 HU; P < 0.001) and CT density (-892.8 {+-} 25.4 HU vs -812.3 {+-} 38.7 HU; P < 0.001) values were significantly lower in hyperlucent regions than in normal lung regions. Xenon and CT density values showed significant positive correlation for the entire lung in 16 children ({gamma} = 0.55 {+-} 0.17, P < 0.001 or =0.017) and for hyperlucent regions in 13 children ({gamma} = 0.44 {+-} 0.16, P < 0.001 or =0.001-0.019). Indexed volumes and volume percentages of hyperlucent lung regions and ventilation defects showed strong negative correlations with forced expiratory volume [FEV1, ({gamma} = -0.64-0.85, P {<=} 0.006)], FEV1/forced vital capacity [FVC, ({gamma} = -0.63-0.84, P {<=} 0.008)], and forced midexpiratory flow rate [FEF{sub 25-75}, ({gamma} = -0.68-0.88, P {<=} 0.002). Volume percentages of xenon ventilation defects (35.0 {+-} 16.4%)] were not significantly different from those of hyperlucent lung regions (38.2 {+-} 18.6%). However, mismatches between the

  9. Comparison of pulmonary function between bronchial asthma and bronchiolitis obliterans in infants%婴幼儿支气管哮喘和闭塞性细支气管炎患儿肺功能比较

    Institute of Scientific and Technical Information of China (English)

    向莉; 饶小春; 焦安夏; 李珍; 任亦欣

    2012-01-01

    Objective To compare the results of pulmonary function test between bronchia asthma and bronchio-litis obliterans (BO) in infants. Methods Fifty-seven infants with bronchial asthma and 47 infants with BO aged from 0 to 3 years old were selected. Their tidal breathing flow-volume curves were tested by Master Screen Paed and the parameters were recorded including respiratory rate (RR), tidal volume over body weight (VT/kg) , ratio of inspiratory time to expiratory time (TI/TE) , ratio of time to reach tidal peak flow to total expiratory time (TPTEF/TE) , ratio of volume at tidal peak flow to total tidal volume (VPEF/VE). Pulmonary compliance (CrsSO/kg) and airway resistance (Rrs) were examined by the single occlusion technique. Results There was no significant difference in VT/kg between asthma group and BO (P < 0.05). RR and Rrs of BO group were significantly higher than that of asthma group (P < 0.05) . The levels of TI/TE, TPTEF/TE, VPEF/VE, TEF50/TIF50 and CrsSOAg in BO group were significantly lower than those in asthma group respectively (P < 0.05). Conclusions In infants with BO, compare with infants with asthma, the resistance of respiratory system and the degree of small airway obstruction increase, but the respiratory system compliance decreases. The evaluation of lung function can provide certain help to differentiate BO and asthma in infants.%目的 比较婴幼儿支气管哮喘和闭塞性毛细支气管炎(BO)患儿肺功能差异.方法 选择57例婴幼儿支气管哮喘和47例BO患儿,应用Master Screen Paed型肺功能仪测定潮气呼吸流量容积曲线,分析参数包括呼吸频率(RR)、每千克体质量潮气量(VT/kg)、吸呼比(TI/TE)、达峰时间比(TPTEF/TE)、达峰容积比(VPEF/VE)、呼吸气中期流速比值(TEF50/TIF50).单阻断法测定每千克体质量呼吸系统顺应性(CrsSO/kg)和呼吸系统阻力(Rrs).结果 支气管哮喘和BO患儿VT/kg差异无统计学意义(P > 0.05);BO患儿RR、Rrs显著高于支气管

  10. Test de marcha de seis minutos en niños con bronquiolitis obliterante postviral: Correlación con espirometría Six minute walk test in children with post-infectious obliterans bronchiolitis: Its relation with spirometry

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    DANIEL ZENTENO A

    2008-03-01

    Full Text Available la Bronquiolitis Obliterante (BO postviral produce alteraciones en la función pulmonar que pueden repercutir en la capacidad para realizar ejercicio, la cual se puede evaluar con el test de marcha de 6 minutos (TM6. Objetivo: Estudiar con esta prueba a niños con BO postviral y determinar la relación entre variables espirométricas y los resultados del TM6. Veintisiete pacientes, se estudiaron con espirometría basal y TM6. Se relacionaron variables espirométricas con resultados del TM6: distancia caminada, índice de Borg (IB, saturación de oxígeno (Sa0(2 y frecuencia cardíaca (FC, mediante Rho de Spearman utilizando SPSS 11,5. No hubo correlación entre la distancia caminada y ninguna variable espirométrica; la CVF sólo se correlacionó con Sa0(2 final (rho = 0,4; p = 0,02. El resto de las variables espirométricas se correlacionaron con IB, Sa0(2 y FC; destacando VEF1 con: IB (rho = -0,7; p Post infectious obliterans bronchiolitis (OB causes persistent pulmonary function impairment and could affect patient ability to perform exercise. Six minute-walk test (6MWT is a useful tool to study these aspects. Objective: Children with OB were evaluated with spirometry and 6MWT and the relationship between spirometrics measurements and 6MWT were determined. Twenty-seven children with OB were studied with a base line spirometry and 6MWT. Correlation between spirometry and 6MWT: covered distance, Borg index (BI, oxygen saturation (0(2S and heart rate (HR, were carried out using Rho of Spearman with SPSS 11.5. There was not relation between the covered distance and spirometrics values; only FVC correlates with final 0(2S (rho = 0.4; p = 0.02. The other spirometrics variables correlates with BI, 0(2S andHR; Interestingly FEV1correlates with: BI (rho = -0.7; p < 0.01, 0(2S (rho = 0.5; p < 0.01 and HR (rho = -0.5; p < 0.01. BI was significant related to FEV1/FVC (rho = -0.7; p < 0.01 and FEF25-75 (rho = -0.8; p < 0.01. Airflow obstruction

  11. Função pulmonar de crianças e adolescentes com bronquiolite obliterante pós-infecciosa Pulmonary function in children and adolescents with postinfectious bronchiolitis obliterans

    Directory of Open Access Journals (Sweden)

    Rita Mattiello

    2010-08-01

    Full Text Available OBJETIVO: Descrever a função pulmonar de crianças e adolescentes com bronquiolite obliterante pós-infecciosa (BOPI e avaliar potenciais fatores de risco para pior função pulmonar. MÉTODOS: A função pulmonar de 77 participantes, com idades de 8-18 anos, foi avaliada por meio de espirometria e pletismografia. Os seguintes parâmetros foram analisados: CVF, VEF1, FEF25-75%, VEF1/CVF, VR, CPT, VR/CPT, volume de gás intratorácico e specific airway resistance (sRaw, resistência específica das vias aéreas. Foi utilizada a regressão de Poisson para investigar os seguintes potenciais fatores de risco para pior função pulmonar: sexo, idade do primeiro sibilo, idade ao diagnóstico, história familiar de asma, exposição ao tabaco, tempo de hospitalização e tempo de ventilação mecânica. RESULTADOS: A idade média foi de 13,5 anos. Houve uma diminuição importante de VEF1 e FEF25-75%, assim como um aumento de VR e sRaw, característicos de doença obstrutiva das vias aéreas. Os parâmetros mais afetados e as médias percentuais dos valores previstos foram VEF1 = 45,9%; FEF25-75% = 21,5%; VR = 281,1%; VR/CPT = 236,2%; e sRaw = 665,3%. Nenhum dos potenciais fatores de risco avaliados apresentou uma associação significativa com pior função pulmonar. CONCLUSÕES: As crianças com BOPI apresentaram um padrão comum de comprometimento grave da função pulmonar, caracterizado por uma obstrução importante das vias aéreas e um expressivo aumento de VR e sRaw. A combinação de medidas espirométricas e pletismográficas pode ser mais útil na avaliação do dano funcional, assim como no acompanhamento desses pacientes. Fatores de riscos conhecidos para doenças respiratórias não parecem estar associados a pior função pulmonar em BOPI.OBJECTIVE: To describe the pulmonary function in children and adolescents with postinfectious bronchiolitis obliterans (PIBO, as well as to evaluate potential risk factors for severe impairment of

  12. Avaliação do estado nutricional de crianças e adolescentes com bronquiolite obliterante pós-infecciosa Assessment of nutritional status in children and adolescents with post-infectious bronchiolitis obliterans

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    Vera Lúcia Bosa

    2008-08-01

    bronchiolitis obliterans and to analyze associations with clinical and nutritional factors. METHODS: The study included 57 patients. Nutritional status was assessed using z scores for weight/age, stature/age, weight/stature in children, and stature/age and body mass index percentiles in adolescents. Body composition was assessed via tricipital skin folds, subscapular skin folds, and the sum of both plus the muscular circumference of the arm; pulmonary function was also investigated in subjects over 8 years old. RESULTS: The high percentages of malnutrition and risk for malnutrition are noteworthy: 21.7 and 17.5%, respectively. Among children, weight/age and stature/age detected higher percentages of malnutrition (21.6 and 16.2%, while weight/stature underestimated this diagnosis. Among adolescents, body mass index detected a high percentage of malnutrition (25% and of risk for malnutrition (20%. Body composition analysis detected 51% of patients with low muscle reserves, and the majority of patients had normal fat reserves. Compromised pulmonary function was associated with poor performance at exercise (r = 0.434; p = 0.024. Malnutrition and/or nutritional risk and low muscle reserves were significantly associated with the 6-minute walk test (p = 0.032; p = 0.030. There was no association between spirometry and the nutritional variables (p > 0.05. CONCLUSIONS: These results emphasize the need for nutritional intervention, and suggest that, in addition to using weight and height indices for nutritional assessment, it is necessary to combine these with an analysis of body composition, so that a larger number of patients with malnutrition and/or at an increased risk of developing malnutrition may be identified and correctly managed.

  13. Función pulmonar y calidad de vida en niños y adolescentes con bronquiolitis obliterante por adenovirus Pulmonary function and quality of life in children and adolescents with bronchiolitis obliterans post-adenoviral infection

    Directory of Open Access Journals (Sweden)

    Stefhanie Nayar E

    2011-09-01

    Full Text Available Introducción: La infección por adenovirus es una causa importante de neumonía en niños chilenos. La bronquiolitis obliterante (BOPI es la complicación más importante. Existen pocos estudios donde evalúen la función pulmonar y la calidad de vida de los pacientes con BOPI. Objetivo: Evaluar la función pulmonar y la calidad de vida de los pacientes con BOPI y la correlación entre ambas variables. Métodos: Se incluyeron 14 niños con BOPI en elpoliclínico de la unidad de broncopulmonar de un hospital público de niños. Período de estudio: abril de 2009-abril de 2010. La función pulmonar se evaluó en una visita médica por espirometría, curva flujo / volumen y la determinación de volumen de gas intratorácico. Los índices analizados fueron los siguientes CVF, VEF1, FEV25-75, VEF1/CVF, VR,CPT, VR y VR / CPT. En la misma visita se realizó una encuesta auto-administrada de Calidad de Vida (PedsQL, versión 4.0, español de Chile para investigar la calidad de vida global, física y psi-cosocial. Se aplicó la correlación lineal de Pearson entre calidad de vida y los parámetros de función pulmonar. Se consideró estadísticamente significativo unp Introduction: Adenovirus infection is an important cause of pneumonia in Chilean children. Postinfectious Bronchiolitis Obliterans (PIBO is the most important complication. There are few studies assessing pulmonary function and quality of life in PIBO patients. Objective: The aim of this study is to assess the pulmonary function and the quality of life ofpatients with PIBO and the correlation between both variables. Methods: 14 children with PIBO in follow up at the pediatric pulmonology outpatient clinic of a public children hospital were included in this study. Study period: April 2009 - April 2010. Pulmonary function was assessed in a medical visit by spirometry, flow/volume curve and intrathoracic gas volume measurement. The following indices were analyzed FVC, FEV1, FEV25-75, FEV1/FVC

  14. Regional ventilation/perfusion mismatch pattern in patient with Swyer James (MacLeod's) syndrome

    OpenAIRE

    Sait Sager; Sertac Asa; Reşit Akyel; Ersan Atahan; Bedii Kanmaz

    2014-01-01

    Swyer James (McLeod′s) syndrome (SJMS) is an uncommon disease, which occurs as a result of childhood bronchiolitis obliterans. Patients may not be diagnosed until later in their life. A 46-year-old man underwent ventilation/perfusion scintigraphy for acute onset of dyspnea. The scan showed markedly diminished ventilation and perfusion unilaterally on the right middle and inferior lobes. However, mismatched ventilation-perfusion pattern was shown on the upper right lobe, which was consistent w...

  15. Thromboangiitis obliterans (Buerger's disease)

    OpenAIRE

    Sinclair, Nicholas R.; Laub, Donald R

    2016-01-01

    Thromboangiitis Obliterans is a non-atherosclerotic inflammatory disease of unknown etiology, which has a strong association with tobacco. We present current concepts on the pathophysiology and diagnosis, as well as a review in treatments.

  16. Bronquiolite obliterante: perfil clínico e radiológico de crianças acompanhadas em ambulatório de referência Bronquiolitis obliterante: perfil clínico y radiológico de 35 niños acompañados en ambulatorio de referencia Bronchiolitis obliterans: clinical and radiological profile of children followed-up in a reference outpatient clinic

    Directory of Open Access Journals (Sweden)

    Carolina Arcanjo Lino

    2013-03-01

    los pediatras frente a lactantes silbadores graves y perennes.OBJECTIVES: To describe the clinical and radiological characteristics of patients with bronchiolitis obliterans. METHODS: This is a retrospective and descriptive study. Data were collected from patients diagnosed with bronchiolitis obliterans between 2004 and 2008 in the Pediatric Pulmonology Clinic of Hospital Infantil Albert Sabin, in Ceará, Northeast Brazil. Such diagnosis was based on clinical and tomographic criteria. Previous history, clinical findings at the diagnosis, complementary exams, and follow-up data were evaluated. RESULTS: 35 children diagnosed with bronchiolitis obliterans were identified. There was a predominance of male patients (3:1. The mean age at the onset of symptoms was 7.5 months, and bronchiolitis obliterans was diagnosed at a mean age of 21.8 months. The most common clinical findings were crackles/wheezing, tachypnea, dyspnea, and chest deformity. Post-infectious etiology was the main cause of bronchiolitis obliterans. Predominant findings at chest X-ray and high resolution computed tomography were peri-bronchial thickening and mosaic pattern, respectively. The treatment was variable and individualized. The majority of patients improved during follow-up, despite the persistence of respiratory symptoms. CONCLUSIONS: In this study, the predominance of male patients and post-infectious etiology was noted, corroborating scientific literature. The most common tomographic findings were similar to those described in previous studies (mosaic pattern, peri-bronchial thickening, and bronchiectasis. Evidence about the treatment of this disease is still lacking. The diagnosis was delayed, which indicates that clinical suspicion of bronchiolitis obliterans is necessary in children with persistent and severe wheezing.

  17. Radiographic finding in broncholitis obliterans after lung transplantation

    International Nuclear Information System (INIS)

    This paper examines the radiographic findings of bronchiolitis obliterans (BO) after lung transplantation. Among the 55 single- and double-lung transplantations performed at the Toronto General Hospital, 41 have survived longer than 3 months and 7 have developed clinical BO. The chest radiographs, CT scans (where available) and medical records of these 7 patients were reviewed retrospectively, with the use of appropriate controls, and results were correlated with pathologic findings. Pathologic specimens demonstrated widespread BO in 3 patients (open lung biopsy), mild acute rejection with possible BO in 1 (transbronchial biopsy [TBBx]), undefined abnormality with some BO in 1 (TBBx), and acute rejection in 2 (TBBx)

  18. Bronchiolitis obliterans organising pneumonia: a consequence of breast radiotherapy

    OpenAIRE

    Fahim, Ahmed; Campbell, Anne P; Hart, Simon Paul

    2012-01-01

    The authors describe a case of 51-year-old woman who presented with breathlessness following radiotherapy for breast carcinoma. A chest radiograph and thoracic CT scan revealed extensive airspace consolidation affecting right upper and lower lobes. A trans-bronchial biopsy revealed evidence of foamy macrophages and fibroblastic plugs within alveoli, consistent with organising pneumonia. Indirect immunofluorescence microscopy revealed evidence of antiepithelial antibodies. Gradual but complete...

  19. Cryptogenic Organizing Pneumonia - Idiopathic Bronchiolitis Obliterans Organizing Pneumonia

    OpenAIRE

    Bekić, Anto; Mehulić, Muharem; Krmpotić, Diana; Kukulj, Suzana; Gorečan, Marijan; Križanac, Šimun

    2004-01-01

    Cryptogenic organizing pneumonia is a rare pulmonary disease with characteristic clinical, radiologic and histologic features. The radiologic presentation, and ventilatory and respiratory lung functions reflect the presence of intra-alveolar buds of granulation tissue occurring within the alveoli and alveolar ducts but rarely occupying the bronchiolar lumen. Therefore, it has been accepted that the diagnosis of these characteristic but not specific presentations of cryptogenic organizing pneu...

  20. Thromboangiitis obliterans (Buerger disease

    Directory of Open Access Journals (Sweden)

    Jessica Seebald, BS

    2015-10-01

    Full Text Available Thromboangiitis obliterans (Buerger disease is an occlusive, nonatherosclerotic, inflammatory vasculitis that causes ischemia in small and medium vessels. Most commonly, Buerger disease is diagnosed in 40- to 45-year-old men with a heavy smoking history. Our case exemplifies the most common presentation, diagnosis, and treatment in a 53-year-old male smoker who presents with arm pain and dusky cool fingers. A Buerger diagnosis requires exclusion of autoimmune, diabetic, and embolic causes. The only recognized treatment for this disease is smoking cessation.

  1. Regional ventilation/perfusion mismatch pattern in patient with Swyer James (MacLeod's) syndrome.

    Science.gov (United States)

    Sager, Sait; Asa, Sertac; Akyel, Reşit; Atahan, Ersan; Kanmaz, Bedii

    2014-09-01

    Swyer James (McLeod's) syndrome (SJMS) is an uncommon disease, which occurs as a result of childhood bronchiolitis obliterans. Patients may not be diagnosed until later in their life. A 46-year-old man underwent ventilation/perfusion scintigraphy for acute onset of dyspnea. The scan showed markedly diminished ventilation and perfusion unilaterally on the right middle and inferior lobes. However, mismatched ventilation-perfusion pattern was shown on the upper right lobe, which was consistent with pulmonary embolism. Unilaterally matched ventilation/perfusion defect can see in SJMS in lung scintigraphy; however, when pulmoner embolism may accompany, scintigraphy should be carefully examined. PMID:25535507

  2. [Balanitis xerotica obliterans].

    Science.gov (United States)

    Calcagno, C

    2007-01-01

    Balanitis xerotica obliterans (BXO), first described by Stuhmer in 1928, is a chronic, progressive, atrophic, sclerosing process involving prepuce, glans and urethra. Its aetiology is unknown. After a short terminological excursus and a review of the aetiological hypothesis, we have focused on BXO in daily urological practice. We are here describing the clinical presentation and its differential diagnosis with premalignant and malignant lesions of the penis. We tried to define the relationship between BXO and squamous cell carcinoma of the penis. Particular attention was then cast on urethral stenosis. Finally, we focused on the treatment of BXO: corticosteroid local therapy as first line treatment or as adjuvant therapy after circumcision, surgical therapy including circumcision, laser therapy of the glans lesions, meatoplasty in the stenosis of the anterior urethra. We also stressed the need for histological examination of the preputial specimen for a correct follow-up and for medicolegal reasons. PMID:21086381

  3. Aspiration-related pulmonary syndromes.

    Science.gov (United States)

    Hu, Xiaowen; Lee, Joyce S; Pianosi, Paolo T; Ryu, Jay H

    2015-03-01

    Aspiration of foreign matter into the airways and lungs can cause a wide spectrum of pulmonary disorders with various presentations. The type of syndrome resulting from aspiration depends on the quantity and nature of the aspirated material, the chronicity, and the host responses. Aspiration is most likely to occur in subjects with a decreased level of consciousness, compromised airway defense mechanisms, dysphagia, gastroesophageal reflux, and recurrent vomiting. These aspiration-related syndromes can be categorized into airway disorders, including vocal cord dysfunction, large airway obstruction with a foreign body, bronchiectasis, bronchoconstriction, and diffuse aspiration bronchiolitis, or parenchymal disorders, including aspiration pneumonitis, aspiration pneumonia, and exogenous lipoid pneumonia. In idiopathic pulmonary fibrosis, aspiration has been implicated in disease progression and acute exacerbation. Aspiration may increase the risk of bronchiolitis obliterans syndrome in patients who have undergone a lung transplant. Accumulating evidence suggests that a causative role for aspiration is often unsuspected in patients presenting with aspiration-related pulmonary diseases; thus, many cases go undiagnosed. Herein, we discuss the broadening spectrum of these pulmonary syndromes with a focus on presenting features and diagnostic aspects. PMID:25732447

  4. Spontaneous thoracic air-leakage syndrome in patients following allogeneic hematopoietic stem cell transplantation: Causes, CT-follow up and patient outcome

    International Nuclear Information System (INIS)

    Objective: The purpose of this article is to describe and illustrate the acute and follow-up imaging features, clinical constellation and outcome of patients with thoracic air-leakage syndrome following allogeneic hematopoietic stem cell transplantation (allo-HCT). Methods: Patients with evidence of thoracic air-leakage, i.e. spontaneous pneumomediastinum, spontaneous pneumothorax or interstitial emphysema after allo-HCT were retrospectively identified by a chart review. Acute and follow-up morphology, duration and patient outcome were analyzed on CT (HRCT or MSCT with HR-reconstructions). Correlation was made with histological results of transbronchial biopsy. Results: The 6 patients included (3 male and 3 female, 14-64 years old) with thoracic air-leakage after allo-HCT all had histologically proven bronchiolitis obliterans (BO) or bronchiolitis obliterans organizing pneumonia (BOOP). Thoracic air-leakage consisted of spontaneous pneumomediastinum associated with active invasive pulmonary aspergillosis (IPA) in 4/6 and spontaneous pneumothorax or interstitial emphysema each in 1/6 patients. Duration of thoracic air-leakage was 7-135 days. Of the patients with spontaneous pneumomediastinum, 3/4 died of IPA. One patient survived until complete regression of spontaneous pneumomediastinum. One patient died 7 days after spontaneous pneumothorax and one survived developing chronic interstitial emphysema. Conclusion: In all cases, thoracic air-leakage was associated to BO or BOOP. In the majority of cases with additional IPA, thoracic air-leakage is more indicative for severity of pulmonary disease than a life-threatening entity itself

  5. Spontaneous thoracic air-leakage syndrome in patients following allogeneic hematopoietic stem cell transplantation: Causes, CT-follow up and patient outcome

    Energy Technology Data Exchange (ETDEWEB)

    Vogel, Monika [Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Str. 3, 72076 Tuebingen (Germany)]. E-mail: monika.vogel@med.uni-tuebingen.de; Brodoefel, Harald [Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Str. 3, 72076 Tuebingen (Germany); Bethge, Wolfgang [Department of Internal Medicine-Oncology, Eberhard-Karls-University, Ottfried-Mueller-Str. 5, 72070 Tuebingen (Germany); Faul, Christoph [Department of Internal Medicine-Oncology, Eberhard-Karls-University, Ottfried-Mueller-Str. 5, 72070 Tuebingen (Germany); Hartmann, Joerg [Department of Internal Medicine-Oncology, Eberhard-Karls-University, Ottfried-Mueller-Str. 5, 72070 Tuebingen (Germany); Schimmel, Heiko [Department of Pathology, Eberhard-Karls-University, Liebermeisterstrasse 8, 72076 Tuebingen (Germany); Wehrmann, Manfred [Department of Pathology, Eberhard-Karls-University, Liebermeisterstrasse 8, 72076 Tuebingen (Germany); Claussen, Claus D. [Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Str. 3, 72076 Tuebingen (Germany); Horger, Marius [Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Str. 3, 72076 Tuebingen (Germany)

    2006-12-15

    Objective: The purpose of this article is to describe and illustrate the acute and follow-up imaging features, clinical constellation and outcome of patients with thoracic air-leakage syndrome following allogeneic hematopoietic stem cell transplantation (allo-HCT). Methods: Patients with evidence of thoracic air-leakage, i.e. spontaneous pneumomediastinum, spontaneous pneumothorax or interstitial emphysema after allo-HCT were retrospectively identified by a chart review. Acute and follow-up morphology, duration and patient outcome were analyzed on CT (HRCT or MSCT with HR-reconstructions). Correlation was made with histological results of transbronchial biopsy. Results: The 6 patients included (3 male and 3 female, 14-64 years old) with thoracic air-leakage after allo-HCT all had histologically proven bronchiolitis obliterans (BO) or bronchiolitis obliterans organizing pneumonia (BOOP). Thoracic air-leakage consisted of spontaneous pneumomediastinum associated with active invasive pulmonary aspergillosis (IPA) in 4/6 and spontaneous pneumothorax or interstitial emphysema each in 1/6 patients. Duration of thoracic air-leakage was 7-135 days. Of the patients with spontaneous pneumomediastinum, 3/4 died of IPA. One patient survived until complete regression of spontaneous pneumomediastinum. One patient died 7 days after spontaneous pneumothorax and one survived developing chronic interstitial emphysema. Conclusion: In all cases, thoracic air-leakage was associated to BO or BOOP. In the majority of cases with additional IPA, thoracic air-leakage is more indicative for severity of pulmonary disease than a life-threatening entity itself.

  6. A case of bronchiolitis obliterans organizing pneumonia (BOOP) after breast conserving therapy for early breast cancer

    International Nuclear Information System (INIS)

    A case of BOOP occurring after radiotherapy for breast cancer was reported. TBLB and BAL were performed at the time of diagnosis. This case suggested that radiographic changes began in the irradiated area, then later spread to non-irradiated areas. In this article, we discussed the peculiary radiographic image, pathological manifestation, BAL characteristics and clinical course in this sequela. (author)

  7. Acute bronchiolitis in infants, a review

    OpenAIRE

    Øymar, Knut; Skjerven, Håvard Ove; Mikalsen, Ingvild Bruun

    2014-01-01

    Acute viral bronchiolitis is one of the most common medical emergency situations in infancy, and physicians caring for acutely ill children will regularly be faced with this condition. In this article we present a summary of the epidemiology, pathophysiology and diagnosis, and focus on guidelines for the treatment of bronchiolitis in infants. The cornerstones of the management of viral bronchiolitis are the administration of oxygen and appropriate fluid therapy, and overall a “min...

  8. An Approach to Relativity of the Lower Extremity Atherosclerosis Obliterans Syndrome Differentiation Typing and Endothelial Function%下肢动脉硬化闭塞症辨证分型与血管内皮功能相关性探讨

    Institute of Scientific and Technical Information of China (English)

    李建鹏; 王峥

    2011-01-01

    Objective:To research the relativity of the lower extremity atherosclerosis obliterans (ASO) syndrome differentiation typing and endothelial function.Methods: 161 cases of ASO in various typings based on syndrome differentiation were observed, the endothelial function indexes were detected.SPSS11.5 for windows software was adopted for statistical treatment.Results :ASO patients had severe endothelial dysfunction(ED) ,ET- 1 and TXB2 in blood plasma of Damp -heat pouring down syndrome group were significantly higher than other groups' s( P < 0.01 ), and the same result to Blood stasis syndrome group' s.But NO in blood serum and 6 - Keto - PGF1α in blood plasma of Damp - heat pouring down syndrome group and Blood stasis syndrome group were significantly lower than Yin -cold congealing syndrome group and Spleen- kidney yang deficiency syndrome group's(P < 0.05 )Conclusion: With respect to the severity of ED, Damp - heat pouring down syndrome group is highest, Blood stasis syndrome group take second place, Yin -cold congealing syndrome group and Spleen -kidney yang deficiency syndrome group is lower.%目的:探讨不同证型的下肢动脉硬化闭塞症(ASO)与血管内皮功能的相关性.方法:选择ASO患者共161例,经辨证后检测各证型患者血管内皮功能指标,采用SPSS 11.5软件包对数据进行统计学处理.结果:ASO患者存在较为严重的血管内皮功能障碍(ED),湿热下注证组患者血浆ET-1、TXB2水平高于其它证型组(P<0.01),血脉瘀阻证为其次;而血清NO水平在此两证型中低于阴寒结聚证及脾肾阳虚证(P<0.05).结论:下肢动脉硬化闭塞症(ASO)湿热下注证血管内皮功能障碍(ED)最为严重,其次为血脉瘀阻证,阴寒结聚证及脾肾阳虚证内皮功能障碍相对较轻.

  9. Pulse oximetry in bronchiolitis: is it needed?

    Science.gov (United States)

    Hendaus, Mohamed A; Jomha, Fatima A; Alhammadi, Ahmed H

    2015-01-01

    Infants admitted to health-care centers with acute bronchiolitis are frequently monitored with a pulse oximeter, a noninvasive method commonly used for measuring oxygen saturation. The decision to hospitalize children with bronchiolitis has been largely influenced by pulse oximetry, despite its questionable diagnostic value in delineating the severity of the illness. Many health-care providers lack the appropriate clinical fundamentals and limitations of pulse oximetry. This deficiency in knowledge might have been linked to changes in the management of bronchiolitis. The aim of this paper is to provide the current evidence on the role of pulse oximetry in bronchiolitis. We discuss the history, fundamentals of operation, and limitations of the apparatus. A search of the Google Scholar, Embase, Medline, and PubMed databases was carried out for published articles covering the use of pulse oximetry in bronchiolitis. PMID:26491341

  10. The surgical treatment of Balanitis Xerotica Obliterans

    OpenAIRE

    A. Hartley; Ramanathan, C.; Siddiqui, H.

    2011-01-01

    Background: Balanitis Xerotica Obliterans (BXO) is a chronic, often progressive disease, which can lead to phimosis and urethral stenosis, affecting both urinary and sexual function. Steroid creams are usually the first-line treatment but have a limited role and surgical intervention is frequently necessary. Conservative surgical procedures (circumcision) are often preferred in the first instance with the premise that recurrence of disease will require a more definitive reconstruction. This s...

  11. Pulse oximetry in bronchiolitis: is it needed?

    Directory of Open Access Journals (Sweden)

    Hendaus MA

    2015-10-01

    Full Text Available Mohamed A Hendaus,1,2 Fatima A Jomha,3 Ahmed H Alhammadi,1,2 1Department of Pediatrics, General Pediatrics Division, Hamad Medical Corporation, 2Weill-Cornell Medical College, Doha, Qatar; 3School of Pharmacy, Lebanese International University, Khiara, Lebanon Abstract: Infants admitted to health-care centers with acute bronchiolitis are frequently monitored with a pulse oximeter, a noninvasive method commonly used for measuring oxygen saturation. The decision to hospitalize children with bronchiolitis has been largely influenced by pulse oximetry, despite its questionable diagnostic value in delineating the severity of the illness. Many health-care providers lack the appropriate clinical fundamentals and limitations of pulse oximetry. This deficiency in knowledge might have been linked to changes in the management of bronchiolitis. The aim of this paper is to provide the current evidence on the role of pulse oximetry in bronchiolitis. We discuss the history, fundamentals of operation, and limitations of the apparatus. A search of the Google Scholar, Embase, Medline, and PubMed databases was carried out for published articles covering the use of pulse oximetry in bronchiolitis. Keywords: bronchiolitis, children, monitor, oxygen

  12. Local interleukin-10 production during respiratory syncytial virus bronchiolitis is associated with post-bronchiolitis wheeze

    Directory of Open Access Journals (Sweden)

    Hodemaekers Hennie M

    2011-09-01

    Full Text Available Abstract Background Respiratory syncytial virus (RSV is the most common cause of bronchiolitis in infants. Following RSV bronchiolitis, 50% of children develop post-bronchiolitis wheeze (PBW. Animal studies have suggested that interleukin (IL-10 plays a critical role in the pathogenesis of RSV bronchiolitis and subsequent airway hyperresponsiveness. Previously, we showed that ex vivo monocyte IL-10 production is a predictor of PBW. Additionally, heterozygosity of the single-nucleotide polymorphism (SNP rs1800872 in the IL10 promoter region was associated with protection against RSV bronchiolitis. Methods This study aimed to determine the in vivo role of IL-10 in RSV pathogenesis and recurrent wheeze in a new cohort of 235 infants hospitalized for RSV bronchiolitis. IL-10 levels in nasopharyngeal aspirates (NPAs were measured at the time of hospitalization and the IL10 SNP rs1800872 genotype was determined. Follow-up data were available for 185 children (79%. Results Local IL-10 levels during RSV infection turned out to be higher in infants that later developed physician diagnosed PBW as compared to infants without PBW in the first year after RSV infection (958 vs 692 pg/ml, p = 0.02. The IL10 promoter SNP rs1800872 was not associated with IL-10 concentration in NPAs. Conclusion The relationship between high local IL-10 levels during the initial RSV infection and physician diagnosed PBW provides further evidence of the importance of the IL-10 response during RSV bronchiolitis.

  13. Cerebral angiographic findings in thromboangiitis obliterans

    Energy Technology Data Exchange (ETDEWEB)

    No, Young J.; Lee, Eun M.; Kim, Jong S. [University of Ulsan, Department of Neurology, Asan Medical Center, Song Pa, PO Pox 145, Seoul (Korea); Lee, Deok H. [University of Ulsan, Department of Neuroradiology, Asan Medical Center, Song Pa, PO Pox 145, Seoul (Korea)

    2005-12-01

    Transient ischemic attacks (TIAs) or ischemic stroke may complicate thromboangiitis obliterans (TAO). However, there has been debate regarding the mechanism of ischemic stroke in TAO. We report the case of a patient with TAO who developed repeated TIAs. An angiogram showed multiple alternative areas of arterial occlusions in the distal segments of both middle cerebral arteries. Extensive collateral vessels around the occluded segment were also observed, which resembled the ''tree root'' or ''corkscrew'' vessels described in the peripheral arteries in TAO. Our patient illustrates that cerebral manifestations of TAO may occur with vascular changes that are identical with those encountered in the limb arteries in TAO. (orig.)

  14. Penile carcinoma arising in balanitis xerotica obliterans.

    Science.gov (United States)

    Goolamali, S I; Pakianathan, M

    2006-02-01

    Squamous cell carcinoma of the penis is an uncommon cancer, though in one study it accounted for 90% of all penile cancers. Its association with balanitis xerotica obliterans (BXO) is a rare though recognized occurrence. We describe a case of a 46-year-old Caucasian male who first presented to our open-access clinic with a mild phimosis. An elective circumcision was performed and histological examination of the circumcision specimen showed BXO. He was lost to follow-up but re-presented three years later with a persistent tender penile ulcer which on biopsy showed no obvious sinister pathology. He returned a further two years later with a short history of bleeding from the ulcer, and another biopsy now confirmed penile squamous cell carcinoma. Our case emphasizes the importance of regular review of patients with BXO, in particular those with persistent symptoms. PMID:16464280

  15. Pneumothorax complicating bronchiolitis in an infant

    International Nuclear Information System (INIS)

    Pneumothorax is an uncommon complication of bronchiolitis. This case illustrates an unusual pattern of atelectasis of the right lung with pleural air surrounding the right upper lobe and the remainder of the lung expanded. It is thought that the hyperinflated lung is unable to collapse as a result of the 'ball valve' effect of air trapping. (orig.)

  16. Bronchiolitis: what the clinician should know

    Directory of Open Access Journals (Sweden)

    Roberto Antonucci

    2015-10-01

    Full Text Available Bronchiolitis is an acute infection of the lower respiratory tract affecting infants and young children, with Respiratory Syncytial Virus (RSV being the most common pathogen. Bronchiolitis is generally a mild disease, but may present with severe signs and symptoms requiring hospitalization. Risk factors including prematurity, bronchopulmonary dysplasia, immunodeficiency and congenital heart defects may predispose patients to develop a severe disease. The diagnosis should be based on clinical evaluation, without supportive radiographic and laboratory studies. Etiological diagnosis may be helpful to decrease the hospital transmission of virus and to avoid inappropriate use of antibiotics.The mainstay of therapy for bronchiolitis is supportive care, which should be directed at maintaining adequate oxygenation, ensuring a proper respiratory toilet, and meeting the requirements of fluids and nutrition. The use of nebulized hypertonic saline should be limited to hospitalized patients. Severe respiratory failure may require mechanical ventilatory support. Neither corticosteroids nor antibiotics offer consistent benefit in the treatment of bronchiolitis, and thus should not be used. A trial of a bronchodilator may be appropriate, but should be continued exclusively if a prompt favorable response occurs. Effective interventions to prevent the spread of RSV infection include hand washing or disinfection by caregivers and contact isolation. The use of palivizumab, a monoclonal antibody directed against RSV, is a safe prophylactic option, but should be restricted to children at high-risk for severe RSV disease, during the epidemic period. Current evidence suggests that early RSV bronchiolitis predisposes children to recurrent wheezing and asthma in the first decade of life.Proceedings of the 11th International Workshop on Neonatology and Satellite Meetings · Cagliari (Italy · October 26th-31st, 2015 · From the womb to the adultGuest Editors: Vassilios

  17. Surfactant abnormalities in infants with severe viral bronchiolitis.

    OpenAIRE

    Dargaville, P A; South, M; McDougall, P N

    1996-01-01

    To determine whether abnormalities of pulmonary surfactant occur in infants with acute viral bronchiolitis, surfactant indices were measured in lung lavage fluid from 12 infants with severe bronchiolitis and eight infants without lung disease. Compared with controls, the bronchiolitis group showed deficiency of surfactant protein A (1.02 v 14.4 micrograms/ml) and disaturated phosphatidylcholine (35 v 1060 micrograms/ml) which resolved as the disease improved. Surfactant functional activity wa...

  18. Increased T follicular helper cells and germinal center B cells are required for cGVHD and bronchiolitis obliterans

    OpenAIRE

    Flynn, Ryan; Du, Jing; Veenstra, Rachelle G.; Reichenbach, Dawn K.; Panoskaltsis-Mortari, Angela; Taylor, Patricia A.; Freeman, Gordon J.; Serody, Jonathan S.; Murphy, William J.; Munn, David H.; Sarantopoulos, Stefanie; Luznik, Leo; Maillard, Ivan; Koreth, John; Cutler, Corey

    2014-01-01

    T follicular helper cells and germinal center B cells are increased and strongly correlate with the development of cGVHD in a murine model.Blocking mAbs for IL-21, ICOS, and CD40L are potential novel therapeutics for cGVHD.

  19. Envolvimento pulmonar na síndrome de Sweet paraneoplásica Pulmonary involvement in paraneoplastic Sweet's syndrome

    Directory of Open Access Journals (Sweden)

    Marcelo Pavesi

    2006-12-01

    Full Text Available Síndrome de Sweet (dermatose neutrofílica febril aguda é caracterizada por lesões cutâneas inflamatórias acompanhadas por febre, leucocitose e mal-estar, devendo ser reconhecida por hematologistas, já que freqüentemente é paraneoplásica. O envolvimento sistêmico pode estar presente e o comprometimento pulmonar, apesar de raro, tem sido reportado na forma de infiltração pulmonar bilateral, bronquiolite obliterante e derrame pleural. Há infiltração neutrofílica densa na histologia. Apresentamos o caso de uma paciente com leucemia mielóide crônica que desenvolveu a síndrome de Sweet com infiltrações pulmonares não infecciosas, as quais não foram responsivas à antibioticoterapia, porém mostraram melhora clínica com a corticoterapia.Sweet's syndrome (acute febrile neutrophilic dermatosis is characterized by inflammatory skin lesions accompanied by fever, leukocytosis and malaise, it should be recognized by hematologists, because it can be a paraneoplastic manifestation. Systemic involvement may be present and lung lesions, despite of the rarity, have been reported in the form of bilateral pulmonary infiltrations, bronchiolitis obliterans and pleural effusion. Histopathology shows dense neutrophilic infiltrates. We present a patient with chronic myeloid leukemia who developed Sweet's syndrome with bilateral pulmonary infiltrations, which were non-responsive to antibiotics but showed clinical improvement on steroid therapy.

  20. The surgical treatment of Balanitis Xerotica Obliterans

    Directory of Open Access Journals (Sweden)

    A Hartley

    2011-01-01

    Full Text Available Background: Balanitis Xerotica Obliterans (BXO is a chronic, often progressive disease, which can lead to phimosis and urethral stenosis, affecting both urinary and sexual function. Steroid creams are usually the first-line treatment but have a limited role and surgical intervention is frequently necessary. Conservative surgical procedures (circumcision are often preferred in the first instance with the premise that recurrence of disease will require a more definitive reconstruction. This study looked at patients with pathologically proven BXO referred to the Plastic Surgery Unit at James Cook University Hospital between 2005 and 2009. The aim was to look at their management in the past and subsequent management by us. We also looked at whether early referral of progressive and recurrent BXO patients to reconstructive surgery could have prevented unnecessary delay in resolving symptoms at an earlier stage. Materials and Methods: Data was collected retrospectively and information regarding the exact anatomical location affected, the extent of the disease, the referring specialty and any previous surgical interventions was obtained. Alterations in urinary and sexual function and relief of symptoms following reconstructive surgery were analysed. Results: Of the 23 patients in the study, 43% had previous surgery and 60% of those had undergone two or more procedures. Twenty-one percent of patients had a history of BXO for over five years. Forty-seven percent of patients had alteration in their urinary function and 48% alteration in their sexual function due to the disease, prior to referral. Early results showed remarkable improvement in urinary and sexual function following reconstructive surgery in this group. Conclusions: Steroid creams have been shown to limit the progression of the disease but do not offer a cure in the majority of cases. Circumcision can be a curative procedure in early disease. Although there is conflicting evidence for

  1. Production of interferon in respiratory syncytial virus bronchiolitis.

    OpenAIRE

    Isaacs, D

    1989-01-01

    Production of interferon alfa in vitro was significantly reduced during acute respiratory syncytial virus bronchiolitis but subsequently returned to normal. Nasopharyngeal and endotracheal interferon alfa were detected intermittently and in low concentrations. The degree of impairment of in vitro production and poor in vivo production of interferon alfa suggest the need for a therapeutic trial of nebulised or systemic interferon in acute bronchiolitis.

  2. Circulating Angiogenic Factors in Patients with Thromboangiitis Obliterans

    OpenAIRE

    Hewing, Bernd; Stangl, Verena; Stangl, Karl; Enke-Melzer, Kathrin; Baumann, Gert; Ludwig, Antje

    2012-01-01

    Background Thromboangiitis obliterans (TAO, also known as Buerger's disease) is a non-atherosclerotic inflammatory vascular disease that primarily affects arteries in the extremities of young adult smokers. Since the etiology of TAO is still unknown, therapeutic options are limited. Recent attempts in therapeutic angiogenesis have been promising. Therefore, the aim of our study was to evaluate angiogenic processes and factors including circulating progenitor cells in TAO. Methodology/Principa...

  3. The clinical study of interventional therapy in thrombo angitis obliterans

    International Nuclear Information System (INIS)

    Objective: To evaluate the clinical feasibility and validity of interventional therapy in thrombo angitis obliterans. Methods: 13 patients with thrombo angitis obliterans were treated with percutaneous left subclavian artery port-catheter system implantation. The catheter external iliac artery with perfusion of prostaglandin E1 (PGE1) 100 μg, urokinase (UK) 200, 000U and mailuoning 20 ml every day. Each treatment lasted for 7-10 days. Results: 13 patients with angiographic demonstration showed occlusions of 1 case in the middle segment of superficial femoral artery, 9 cases of complete occlusion in popliteal artery and same for 3 cases in anterior tibial artery, the end of the peroneal artery and posterior tibial artery. After treatment, 3 patients had to undergo amputation due to gangrene in foot and 10 patients with no gangrene showed skin temperature rising up in the lower limb. Intermittent claudication and rest pain were relieved obviously accompanied by increasing step distance over 500 m with the Fontain grade improvement of twice at least. Follow up angiography one year later showed large quantity of vasculogenesis in the lower limb. Conclusion: The treatment of trans-port-catheter system is excellent and practical for improving the clinical status of patients with thrombo angitis obliterans. (authors)

  4. High incidence of balanitis xerotica obliterans in boys with phimosis: prospective 10-year study.

    Science.gov (United States)

    Kiss, András; Király, László; Kutasy, Balázs; Merksz, Miklós

    2005-01-01

    This prospective study was designed to address the incidence and clinical and histologic characteristics of balanitis xerotica obliterans in a large random pediatric population with phimosis. We investigated 1178 boys who presented consecutively with phimosis between 1991 and 2001. All patients who underwent complete circumcision and surgical specimens were typed histologically as early, intermediate, or late forms of this disorder or as nonspecific chronic inflammation. Patients with balanitis xerotica obliterans were controlled at 1, 6, and 12 months postoperatively, then yearly. Balanitis xerotica obliterans was found in 471 of the 1178 patients (40%), with the highest incidence in boys aged 9 to 11 years (76%). Secondary phimosis occurred in 93% of boys with balanitis xerotica obliterans and in 32% of those without the disorder. In six instances of balanitis xerotica obliterans, meatotomy and in one meatoplasty was performed, as well as circumcision. On histologic evaluation, we found 19% had early, 60% intermediate, and 21% late form of balanitis xerotica obliterans. Glanular lesions disappeared completely within 6 months in 229 out of 231 patients. Our data strongly suggest that the true incidence of childhood balanitis xerotica obliterans is higher than previously assumed. Its incidence peaks in the 9 to 11 years age group, in whom secondary phimosis was almost exclusively caused by balanitis xerotica obliterans. PMID:16060864

  5. Help Desk Answers: Do corticosteroids reduce bronchiolitis hospitalizations?

    Science.gov (United States)

    Jendi, Mohammed R; Scott, Quincy O; Smaga, Sharon A

    2016-05-01

    Corticosteroids alone don't decrease hospital admissions or length of stay among children with bronchiolitis. Combining oral dexamethasone and inhaled epinephrine appears to prevent one hospital admission for every 11 patients treated. PMID:27275940

  6. Prevention and nursing care of the complications occurred in interventional therapy for arteriosclerosis obliterans of lower extremity

    International Nuclear Information System (INIS)

    Objective: To discuss the prevention and nursing care of the perioperative complications occurred in interventional therapy for arteriosclerosis obliterans of lower extremity. Methods: During the period of July 2006 to June 2009, interventional treatment for the arteriosclerosis obliterans of lower extremity was performed in 380 cases. The clinical data and complications were reviewed and analyzed, and the prevention and nursing care of the complications were summarized. Results: Complications occurred in 41 cases. During the surgery, vascular rupture or arterial dissection occurred in 5 cases, hypoglycemia reaction in 3 cases and elevation of blood pressure in 2 cases. The complications,which occurred after the treatment,included acute arterial thrombosis (n=3), deep vein thrombosis (n=2), bleeding of different tissues or organs (n=17), acute myocardial infarction (n=2), pseudoaneurysm (n=2), excessive lower limb perfusion syndrome (n=4) and compression sores (n=1). Conclusion: Detailed information of medical history, careful observation of clinical condition, intensive care of patient, adequate preparation of medical materials, seriously handing over the duty to the next shift and taking one's turn on duty, etc. are all the effective measures to prevent and to reduce the occurrence of complications. (authors)

  7. CPAP and High-Flow Nasal Cannula Oxygen in Bronchiolitis.

    Science.gov (United States)

    Sinha, Ian P; McBride, Antonia K S; Smith, Rachel; Fernandes, Ricardo M

    2015-09-01

    Severe respiratory failure develops in some infants with bronchiolitis because of a complex pathophysiologic process involving increased airways resistance, alveolar atelectasis, muscle fatigue, and hypoxemia due to mismatch between ventilation and perfusion. Nasal CPAP and high-flow nasal cannula (HFNC) oxygen may improve the work of breathing and oxygenation. Although the mechanisms behind these noninvasive modalities of respiratory support are not well understood, they may help infants by way of distending pressure and delivery of high concentrations of warmed and humidified oxygen. Observational studies of varying quality have suggested that CPAP and HFNC may confer direct physiologic benefits to infants with bronchiolitis and that their use has reduced the need for intubation. No trials to our knowledge, however, have compared CPAP with HFNC in bronchiolitis. Two randomized trials compared CPAP with oxygen delivered by low-flow nasal cannula or face mask and found some improvements in blood gas results and some physiologic parameters, but these trials were unable to demonstrate a reduction in the need for intubation. Two trials evaluated HFNC in bronchiolitis (one comparing it with headbox oxygen, the other with nebulized hypertonic saline), with the results not seeming to suggest important clinical or physiologic benefits. In this article, we review the pathophysiology of respiratory failure in bronchiolitis, discuss these trials in detail, and consider how future research studies may be designed to best evaluate CPAP and HFNC in bronchiolitis. PMID:25836649

  8. Recidiva en bronquiolitis obliterante con neumonía en organización: BOOP RELAPSE IN BRONCHOLITIS OBLITERANS ORGANIZING PNEUMONIA: BOOP

    Directory of Open Access Journals (Sweden)

    JUAN I. VARGAS RT.

    2003-01-01

    Full Text Available Las recaídas son frecuentes en la bronquiolitis obliterante con neumonía en organización (BOOP, sin embargo, existe poca información con respecto a su causa. Se presenta el caso de una mujer de 63 años con un cuadro clínico radiológico compatible con BOOP. No se identificó una causa subyacente por lo que se planteó el diagnóstico de neumonía en organización criptogénica o COP. Se realizaron biopsias transbronquiales que demostraron una neumonía crónica organizada y bronquiolitis proliferativa. Se trató con prednisona con buena respuesta. Al octavo mes de tratamiento, mientras se disminuía la dosis de esteroides, comenzó con disnea y tos y aparición de nuevas opacidades pulmonares radiológicos, las que regresaron rápidamente al aumentar la dosis de esteroides. Discutimos las causas posibles de la recidiva de esta patología y su relación con la disminución de la dosis de esteroidesAlthough relapses are frequent in bronchiolitis obliterans organizing pneumonia (BOOP, there is scant information regarding the causes underlying its occurrence. We report a 63 year old woman with clinical and radiological features compatible with BOOP. No underlying cause was identified so she was thought to have cryptogenic BOOP or cryptogenic organizing pneumonia (COP. A transbronchial lung biopsy demonstrated chronic organizing pneumonia and features of proliferative bronchiolitis. She was successfully treated with prednisone. On the eighth month of steroid therapy, while tapering the dose, she begun with cough and dyspnea and developed new lung infiltrates on the chest x-ray film. The infiltrates cleared rapidly after increasing the dose of steroids. We discuss the possible causes of relapse in BOOP and its relation to steroid therapy

  9. Causal direction between respiratory syncytial virus bronchiolitis and asthma studied in monozygotic twins

    DEFF Research Database (Denmark)

    Poorisrisak, Porntiva; Halkjaer, Liselotte Brydensholt; Thomsen, Simon Francis;

    2010-01-01

    Respiratory syncytial virus (RSV) bronchiolitis has been associated with later development of asthma, wheezing, abnormal pulmonary function, and sensitization. Our aim was to determine the differential effect within monozygotic (MZ) twin pairs discordant for severe RSV bronchiolitis in infancy on...

  10. Obliterative bronchiolitis due to Mycoplasma pneumoniae infection in a child

    International Nuclear Information System (INIS)

    A six-year-old girl presented with Mycoplasma pneumoniae involving the right upper and lower lobes. She made a slow but complete recovery with resolution of the radiological changes. She represented 5 years later with a productive cough, recurrent wheezing and physical and radiological signs suggestive of obliterative bronchiolitis. This diagnosis was confirmed by ventilation - perfusion (dV/dt/dQ/dt) lung scan, and bronchography. The case highlights the value of dV/dt/dQ/dt scanning in the diagnosis of obliterative bronchiolitis and confirms the previous reports that mycoplasma infections are not always benign. (orig.)

  11. A prospective randomised trial comparing nasogastric with intravenous hydration in children with bronchiolitis (protocol) The comparative rehydration in bronchiolitis study (CRIB)

    OpenAIRE

    Borland Meredith; Acworth Jason; Babl Franz E; Oakley Ed; Kreiser David; Neutze Jocelyn; Theophilos Theane; Donath Susan; South Mike; Davidson Andrew

    2010-01-01

    Abstract Background Bronchiolitis is the most common reason for admission of infants to hospital in developed countries. Fluid replacement therapy is required in about 30% of children admitted with bronchiolitis. There are currently two techniques of fluid replacement therapy that are used with the same frequency-intravenous (IV) or nasogastric (NG). The evidence to determine the optimum route of hydration therapy for infants with bronchiolitis is inadequate. This randomised trial will be the...

  12. Mechanical ventilation drives inflammation in severe viral bronchiolitis.

    Directory of Open Access Journals (Sweden)

    Marije P Hennus

    Full Text Available INTRODUCTION: Respiratory insufficiency due to severe respiratory syncytial virus (RSV infection is the most frequent cause of paediatric intensive care unit admission in infants during the winter season. Previous studies have shown increased levels of inflammatory mediators in airways of mechanically ventilated children compared to spontaneous breathing children with viral bronchiolitis. In this prospective observational multi-center study we aimed to investigate whether this increase was related to disease severity or caused by mechanical ventilation. MATERIALS AND METHODS: Nasopharyngeal aspirates were collected <1 hour before intubation and 24 hours later in RSV bronchiolitis patients with respiratory failure (n = 18 and non-ventilated RSV bronchiolitis controls (n = 18. Concentrations of the following cytokines were measured: interleukin (IL-1α, IL-1β, IL-6, monocyte chemotactic protein (MCP-1 and macrophage inflammatory protein (MIP-1α. RESULTS: Baseline cytokine levels were comparable between ventilated and non-ventilated infants. After 24 hours of mechanical ventilation mean cytokine levels, except for MIP-1α, were elevated compared to non-ventilated infected controls: IL-1α (159 versus 4 pg/ml, p<0.01, IL-1β (1068 versus 99 pg/ml, p<0.01, IL-6 (2343 versus 958 pg/ml, p<0.05 and MCP-1 (174 versus 26 pg/ml, p<0.05. CONCLUSIONS: Using pre- and post-intubation observations, this study suggests that endotracheal intubation and subsequent mechanical ventilation cause a robust pulmonary inflammation in infants with RSV bronchiolitis.

  13. Acute viral bronchiolitis in South Africa: Diagnostic flow.

    Science.gov (United States)

    White, D A; Zar, H J; Madhi, S A; Jeena, P; Morrow, B; Masekela, R; Risenga, S; Green, R

    2016-04-01

    Bronchiolitis may be diagnosed on the basis of clinical signs and symptoms. In a young child, the diagnosis can be made on the clinical pattern of wheezing and hyperinflation. Clinical symptoms and signs typically start with an upper respiratory prodrome, including rhinorrhoea, low-grade fever, cough and poor feeding, followed 1 - 2 days later by tachypnoea, hyperinflation and wheeze as a consequence of airway inflammation and air trapping.The illness is generally self limiting, but may become more severe and include signs such as grunting, nasal flaring, subcostal chest wall retractions and hypoxaemia. The most reliable clinical feature of bronchiolitis is hyperinflation of the chest, evident by loss of cardiacdullness on percussion, an upper border of the liver pushed down to below the 6th intercostal space, and the presence of a Hoover sign(subcostal recession, which occurs when a flattened diaphragm pulls laterally against the lower chest wall).Measurement of peripheral arterial oxygen saturation is useful to indicate the need for supplemental oxygen. A saturation of <92% at sea level and 90% inland indicates that the child has to be admitted to hospital for supplemental oxygen. Chest radiographs are generally unhelpful and not required in children with a clear clinical diagnosis of bronchiolitis.Blood tests are not needed routinely. Complete blood count tests have not been shown to be useful in diagnosing bronchiolitis or guiding its therapy. Routine measurement of C-reactive protein does not aid in management and nasopharyngeal aspirates are not usually done.Viral testing adds little to routine management. Risk factors in patients with severe bronchiolitis that require hospitalisation and may even cause death, include prematurity, congenital heart disease and congenital lung malformations. PMID:27303779

  14. Targeting novel antigens in the arterial wall in thromboangiitis obliterans.

    Directory of Open Access Journals (Sweden)

    Murat Akkus

    2010-06-01

    Full Text Available Thromboangiitis obliterans is an inflammatory disease possibly resulting from cigarette smoking as a primary etiologic factor, perhaps as a delayed type of hypersensitivity or toxic angiitis. As little is known about the pathogenesis of the disease, we aimed to determine novel antigens that might be responsible from the local inflammatory reactions and structural changes observed in this disease. An indirect immunoperoxidase technique is used to examine the tissue samples obtained from the dorsalis pedis artery of affected individuals with twenty monoclonal antibodies. Among these several antigens which are not previously reported in TAO like CD34, CD44 and CD90 were determined in the tissue samples examined. On the other hand, many other antigens like cytokine/chemokine receptors, several enzymes and leukocyte/lymphocyte antigens were lacking giving some clues about the local pathological reactions. We briefly discussed our findings for several critical antigens those first described in the present work, possibly having roles in the development of the disease. Expression of the CD90/CD11c receptor/ligand pair seems to play an important role in mononuclear cell recruitment to the damage site. Vascular invasion of not only tunica intima but also the tunica media in affected vessels is clearly demonstrated using endothelial cell specific antigens.

  15. Thromboangiitis Obliterans (Buerger’s Disease—Current Practices

    Directory of Open Access Journals (Sweden)

    Abhishek Vijayakumar

    2013-01-01

    Full Text Available Thromboangiitis obliterans (TAO is a nonatherosclerotic, segmental inflammatory disease that most commonly affects the small and medium-sized arteries and veins in the upper and lower extremities. Cigarette smoking has been implicated as the main etiology of the disease. In eastern parts of the world TAO forms 40–60% of peripheral vascular diseases. Clinical features and angiographic finding are the basis of early diagnosis of TAO. Abstinence from smoking is the only definitive treatment to prevent disease progression. Medical management in form of aspirin, pentoxyfylline, cilostazol, and verapamil increase pain-free walking distance in intermittent claudication, but long term usage fails to prevent disease progression in patients who continue to smoke. Surgical treatment in form of revascularization, lumbar sympathectomy, omentopexy, and Ilizarov techniques help reduce pain and promote healing of trophic changes. Newer treatment modalities like spinal cord stimulation, prostacyclin, bosentan, VEGF, and stem cell therapy have shown promising results. Latest treatment options include peripheral mononuclear stem cell, and adipose tissue derived mononuclear stem cells have been shown to be effective in preventing disease progression, decrease major amputation rates, and improving quality of life.

  16. Mechanisms underlying epithelium-dependent relaxation in rat bronchioles

    DEFF Research Database (Denmark)

    Kroigaard, Christel; Dalsgaard, Thomas; Simonsen, Ulf

    2010-01-01

    (SK(Ca)) and intermediate (IK(Ca))-conductance calcium-activated potassium channels, NS309 (6,7-dichloro-1H-indole-2,3-dione 3-oxime) was used to induce EpDHF-type relaxation. IK(Ca) and SK(Ca)3 positive immunoreactions were observed mainly in the epithelium and endothelium of bronchioles and arteries......, respectively. In 5-hydroxytryptamine (1 microM)-contracted bronchioles (828 +/- 20 microm, n = 84) and U46619 (0.03 microM)-contracted arteries (720 +/- 24 microm, n = 68), NS309 (0.001-10 microM) induced concentration-dependent relaxations that were reduced by epithelium/endothelium removal and by blocking IK......(Ca) channels with charybdotoxin and in bronchioles also by blocking SK(Ca) channels with apamin. Inhibition of cyclooxygenase, nitric oxide synthase, and cytochrome 2C isoenzymes, or blockade of large (BK(Ca))-conductance calcium-activated potassium channels with iberiotoxin, failed to reduce NS309 relaxation...

  17. Bronchiolitis caused by occupational and ambient atmospheric particles.

    Science.gov (United States)

    Churg, Andrew; Wright, Joanne L

    2003-10-01

    Occupationally encountered mineral dusts such as asbestos, silica, silicates (talc, mica), and metals can produce a distinctive pattern of fibrosis and distortion of the small airways, particularly the distal membranous bronchioles (MB) and the respiratory bronchioles (RB). Recent reports show that the same types of changes, accompanied by considerable muscle hyperplasia, are found in individuals living in regions with high levels of particulate air pollutants (PM). Models and actual measurements suggest that these changes occur because the small airways are sites of high particle deposition, and inhaled and deposited particulates, including PM, enter the airway walls. Studies from our laboratory using a tracheal explant model have shown that, for many types of particles, entry into the airway wall causes expression of mediators that lead to airway wall fibrosis and airway wall smooth muscle hyperplasia, probably through oxidant mechanisms. These reactions are intrinsic properties of the particles and do not require exogenous inflammatory cells. There is considerable evidence that individuals with occupational exposure to a wide variety of mineral dusts, as well as individuals with chronic exposure to high levels of PM, develop chronic airflow obstruction. The type of small airway remodeling seen in particle-induced bronchiolitis appears to be one cause of chronic airflow obstruction in this setting. PMID:16088574

  18. Asthma, atopy and lung function at 11 years of age after bronchiolitis in infancy

    OpenAIRE

    Mikalsen, Ingvild Bruun

    2014-01-01

    Background: Bronchiolitis is a frequently occurring respiratory disorder in young children, and also an established risk factor for subsequent recurrent wheeze and asthma. The association between bronchiolitis and later asthma is complex and not fully understood, and probably related to interactions between viral aetiology, various host factors and environmental mechanisms. Few long-term follow-up studies have included children hospitalized for bronchiolitis below 12 months ...

  19. Effectiveness of Chest Physiotherapy in Infants Hospitalized with Acute Bronchiolitis: A Multicenter, Randomized, Controlled Trial

    OpenAIRE

    Gajdos, Vincent; Katsahian, Sandrine; Beydon, Nicole; Abadie, Véronique; de Pontual, Loïc; Larrar, Sophie; Epaud, Ralph; Chevallier, Bertrand; Bailleux, Sylvain; Mollet-Boudjemline, Alix; Bouyer, Jean; Chevret, Sylvie; Labrune, Philippe

    2010-01-01

    Editors' Summary Background Bronchiolitis, which is usually caused by the respiratory syncytial virus (RSV), is the commonest infection of the lower respiratory tract (the lungs and the passages through which air enters the lungs) in infants. A third of all children have bronchiolitis during their first year of life. The illness begins with stuffiness, a runny nose, a mild cough, and mild fever. Then, as the smallest airways in the lung (the bronchioles) become inflamed (swell) and blocked wi...

  20. Progress of research on follicular bronchiolitis%滤泡性细支气管炎研究进展

    Institute of Scientific and Technical Information of China (English)

    陈石; 张德平

    2009-01-01

    Follicular bronchiolitis is a histopathological diagnosis,and is characterized with follicular hyperplasia in lymphoid germinal center and bronchiole wall.It is often associated with chronic infections and inflammatory airway diseases such as cystic fibrosis,bronchiectasis,chronic aspiration,connective tissue diseases,including immunodeficiency syndrome like AIDS.Chest imaging mainly manifests small nodules or reticular shadow,accompanied with intrathoracic lymph nodes,nodules and ground-glass shadow diffuse distribution in both lungs,located at the bronchial perivascular tissues.Clinical manifestations and imaging can be used to make the diagnosis of follicular bronchiolitis.It is usually lack of treatment means but have a good prognosis.%滤泡性细支气管炎是一个组织病理学诊断,以细支气管壁伴有生发中心的淋巴样滤泡增生为特征.常与慢性感染和炎症性气道疾病,例如囊性纤维化、支气管扩张、慢性误吸、结缔组织疾病、包括获得性免疫缺陷综合征在内的免疫缺陷综合征有一定的相关性.在胸部影像学主要表现为两肺小结节或网状阴影,伴有胸腔内淋巴结肿大,结节和毛玻璃样影可以两肺弥漫性分布,主要位于支气管血管周围.结合临床表现和影像学表现可以用于滤泡性细支气管炎的诊断.治疗缺乏特效治疗手段,一般预后较好.

  1. Circulating angiogenic factors in patients with thromboangiitis obliterans.

    Directory of Open Access Journals (Sweden)

    Bernd Hewing

    Full Text Available BACKGROUND: Thromboangiitis obliterans (TAO, also known as Buerger's disease is a non-atherosclerotic inflammatory vascular disease that primarily affects arteries in the extremities of young adult smokers. Since the etiology of TAO is still unknown, therapeutic options are limited. Recent attempts in therapeutic angiogenesis have been promising. Therefore, the aim of our study was to evaluate angiogenic processes and factors including circulating progenitor cells in TAO. METHODOLOGY/PRINCIPAL FINDINGS: TAO patients with critical limb ischemia and age- and gender-matched nonsmokers and smokers without cardiovascular disease (n = 12 in each group were enrolled in the study. Flow cytometric analysis of peripheral blood showed significantly decreased levels of circulating CD45(dimCD34(+ progenitor cells in TAO patients and in smokers compared to nonsmokers. In contrast to both control groups, the proportion of CD45(dimCD34(+ progenitor cells co-expressing VEGF receptor-2 (VEGFR2 was significantly elevated in TAO patients. Enzyme-linked immunosorbent assay (ELISA of common angiogenic factors (such as VEGF did not clearly point to pro- or antiangiogenic conditions in serum or plasma of TAO patients. Serum of TAO patients and controls was evaluated in proliferation, migration (scratch assay and spheroid sprouting assays using human umbilical vein endothelial cells (HUVECs. Serum of TAO patients exhibited a diminished sprouting capacity of HUVECs compared to both control groups. Proliferation and migration of endothelial cells were impaired after treatment with serum of TAO patients. CONCLUSION: Levels of circulating progenitor cells were altered in TAO patients compared to healthy nonsmokers and smokers. Furthermore, serum of TAO patients exhibited an antiangiogenic activity (impaired endothelial cell sprouting, migration and proliferation on endothelial cells, which may contribute to vascular pathology in this patient population.

  2. Case of a congenital urethral duplication being unmasked following circumcision for balanitis xerotica obliterans.

    Science.gov (United States)

    Boyd, Matthew; Woodward, Mark; Lambert, Anthony

    2010-07-01

    We present the case of an 11-year-old boy diagnosed with an Effmann Type II A1 urethral duplication after routine circumcision for balanitis xerotica obliterans (BXO). We discuss the pathophysiology, investigation and management both of BXO and urethral duplication. PMID:20529516

  3. Extensive balanitis xerotica obliterans of urethrocutaneous fistula presenting as mass in scrotum.

    Science.gov (United States)

    Kumar, Santosh; Nagappa, Bhubnesh; Ganesamoniv, Raguram

    2010-08-01

    A rare case of extensive lichen sclerosis (balanitis xerotica obliterans) involving the anterior urethra, urethrocutaneous fistula, and scrotum is described in a middle-aged man who presented with a history of obstructive voiding symptoms. He was managed by excision of mass along with the fistulae and staged buccal mucosal urethroplasty. PMID:19913886

  4. Multiple complications due to osteoradionecrosis in a patient with thromboangiitis obliterans.

    NARCIS (Netherlands)

    Barkhuysen, R.; Janssens, G.O.; Wilde, P.C.M. de; Merkx, M.A.W.

    2007-01-01

    A case is presented of a patient with thromboangiitis obliterans (TAO) who developed severe necrosis of the intraoral soft tissues and maxillary and mandibular bone after radiotherapy for a cT2N0M0 squamous cell carcinoma of the soft palate. Multiple surgical procedures including partial resection o

  5. Investigation of the Etiology of Anemia in Thromboangiitis Obliterans.

    Science.gov (United States)

    Akbarin, Mohammad Mehdi; Ravari, Hassan; Rajabnejad, Ataollah; Valizadeh, Narges; Fazeli, Bahare

    2016-09-01

    During a review of patients admitted with thromboangiitis obliterans (TAO), there was evidence of normochromic normocytic anemia and abrupt changes in hemoglobin (Hgb) levels in patients with several hospital admissions. Therefore, the evidence of hemolytic anemia was evaluated based on 37 banked plasma samples taken from Caucasian male TAO patients during disease exacerbation between 2012 and 2014. The patients' hospital records, including clinical manifestations and complete blood count, were evaluated. The following tests were performed on all samples: indirect antiglobulin test (IAT), C-reactive protein (CRP), high-sensitivity CRP (hsCRP), lactate dehydrogenase (LDH), haptoglobin, indirect bilirubin, d-aspartate aminotransferase (AST), and d-alanine aminotransferase (ALT). The mean age of the patients was 40 ± 7 years. Two patients underwent below-knee amputation. The mean hospital-documented Hgb of the patients was 12.9 ± 2.6 g/dL. CRP and IAT were positive in 75.6 and 70.2% of the samples, respectively. The tests and corresponding results were as follows: hsCRP, 14.07 ± 2.37 µg/mL; LDH, 2,552 ± 315 u/L; haptoglobin, 2.27 ± 1.1 g/L; indirect bilirubin, 0.09 ± 0.04 mg/dL; AST, 67 ± 7 u/L; and ALT, 26 ± 3 u/L. There was a significant inverse correlation between hsCRP and hospital-documented Hgb level (p = 0.03). Anemia with the positive IAT in most of the samples, high LDH and AST, and normal ALT are suggestive of hemolytic anemia. Normal indirect bilirubin is consistent with intravascular hemolysis. The positive CRP and elevated haptoglobin levels could be due to systemic inflammation in TAO. However, it is not known if an autoantigen or an infectious antigen is responsible for TAO systemic inflammation and induction hemolytic anemia. As such, the underlying mechanism of anemia in TAO could be part of the footprint of its main etiology. PMID:27574381

  6. Extensive balanitis xerotica obliterans (BXO) involving the anterior urethra and scrotum.

    Science.gov (United States)

    Singh, Iqbal; Ansari, M S

    2006-01-01

    We describe an unusual case of balanitis xerotica obliterans (BXO) involving the entire anterior urethra and the scrotum that had presented as a palpable nodular scrotal mass with obstructive voiding symptoms in middle aged man. He was managed by a staged urethroplasty. We report the first such case of BXO involving the scrotum resulting in a nodular mass that has not been described and reported till date. PMID:17180441

  7. Detection of human papillomavirus types in balanitis xerotica obliterans and other penile conditions.

    OpenAIRE

    Lau, P W; Cook, N.; Andrews, H.; Bracka, A; Myint, S H

    1995-01-01

    OBJECTIVES--To determine the prevalence of human papillomavirus (HPV) types 6, 11, 16 and 18 in foreskin biopsies from patients with balanitis xerotica obliterans (BXO) and other penile conditions. MATERIALS AND METHODS--Foreskin biopsy specimens from 24 patients with penile lesions and 5 control patients were analysed by type-specific polymerase chain reaction (PCR). RESULTS--HPV6 or HPV16 were not detected in patients with BXO. HPV6 was detected in 2 controls. CONCLUSIONS--Genital papilloma...

  8. Types of Childhood Interstitial Lung Disease

    Science.gov (United States)

    ... or postinfectious disease ( bronchiolitis obliterans) Eosinophilic pneumonia Pulmonary alveolar proteinosis Pulmonary infiltrates with eosinophilia Pulmonary lymphatic disorders (lymphangiomatosis, ...

  9. Exhaled nitric oxide is related to atopy, but not asthma in adolescents with bronchiolitis in infancy

    OpenAIRE

    Mikalsen, Ingvild Bruun; Halvorsen, Thomas; Øymar, Knut

    2013-01-01

    Background: The fraction of exhaled nitric oxide (FeNO) has been suggested as a non-invasive marker of eosinophilic inflammation in asthma, but lately rather as a biomarker of atopy than of asthma itself. Asthma after bronchiolitis is common up to early adolescence, but the inflammation and pathophysiology may differ from other phenotypes of childhood asthma. We aimed to assess if FeNO was different in children with former hospitalization for bronchiolitis and a control group, and...

  10. A Case–Crossover Study of Wintertime Ambient Air Pollution and Infant Bronchiolitis

    OpenAIRE

    Karr, Catherine; Lumley, Thomas; Shepherd, Kristen; Davis, Robert; Larson, Timothy; Ritz, Beate; Kaufman, Joel

    2005-01-01

    We examined the association of infant bronchiolitis with acute exposure to ambient air pollutants. Design We employed a time-stratified case–crossover method and based the exposure windows on a priori, biologically based hypotheses. Participants We evaluated effects in 19,901 infants in the South Coast Air Basin of California in 1995–2000 with a hospital discharge record for bronchiolitis in the first year of life (International Classification of Diseases, 9th Revision, CM466.1). Evaluations/...

  11. Evaluation of physiological parameters before and after respiratory physiotherapy in newborns with acute viral bronchiolitis

    OpenAIRE

    S Gonçalves, Rodrigo A; Feitosa, Sérgio; de Castro Selestrin, Cláudia; Vitor E. Valenti; de Sousa, Fernando H; F Siqueira, Arnaldo A; Petenusso, Márcio; de Abreu, Luiz Carlos

    2014-01-01

    Background Acute viral bronchiolitis is a respiratory disease with high morbidity that affects newborn in the first two years of life. Its treatment with physiotherapy has been highlighted as an important tool, however, there is no consensus regarding its effects on patients improvement. We aimed to evaluate the physiological parameters before and after the procedure respiratory therapy in newborn with acute viral bronchiolitis. Method This was a cross sectional observational study in 30 newb...

  12. Altered cardiac rhythm in infants with bronchiolitis and respiratory syncytial virus infection

    OpenAIRE

    Galeone Carlotta; Barbier Paolo; Gualtieri Laura; Tagliabue Claudia; Tremolati Elena; Ghiglia Silvia; Bosis Samantha; Salice Patrizia; Esposito Susanna; Marchisio Paola; Principi Nicola

    2010-01-01

    Abstract Background Although the most frequent extra-pulmonary manifestations of respiratory syncytial virus (RSV) infection involve the cardiovascular system, no data regarding heart function in infants with bronchiolitis associated with RSV infection have yet been systematically collected. The aim of this study was to verify the real frequency of heart involvement in patients with bronchiolitis associated with RSV infection, and whether infants with mild or moderate disease also risk heart ...

  13. Costs associated with infant bronchiolitis in the Baffin region of Nunavut

    OpenAIRE

    Creery, David; Iyer, Priya; Samson, Lindy; Coyle, Doug; Osborne, Geraldine; MacDonald, Alexander

    2005-01-01

    Objective. Although infants living in the north of Canada have been reported to have one of the highest rates of hospital admission for bronchiolitis in the world, the economic effects of this condition have not been reported. Passive immunization against the Respiratory Syncytial Virus, the most common causative agent of infant bronchiolitis, is available. Methods. We tabulated transportation, in-hospital care and family accommodation costs for infants of less than 12 months of age residing ...

  14. Lack of effect of zinc sulfate on wheezing after bronchiolitis in infants less than two years

    Directory of Open Access Journals (Sweden)

    R Abasi

    2014-10-01

    Full Text Available Backgrounds & aim: Wheezing after bronchiolitis is known as a risk factor for asthma. No cure or effective vaccine has been identified for such disease. The purpose of this study was to determine the effects of zinc sulfate on persistent wheezing after bronchiolitis in infants less than two years. Methods: The present randomized clinical trial study was conducted on 90 infants under two years of age suffering from the first attack with a clinical diagnosis of bronchiolitis wheezing hospitalized in Imam Sajjad Hospital, Yasuj. All infants were equally placed in test groups and control group. In addition to bronchiolitis usual treatment, test group received 20 mg of elemental zinc for seven days. Patient information including risk factors associated with wheezing and persistent wheezing were compared 48 hours and two weeks after treatment. Data were analyzed using chi-square and t tests. Results: In all 90 infants diagnosed with bronchiolitis, wheezing was observed. After 48 hours and two weeks wheezing was continued in 67 patients (74.4% and 54 patients (60% respectively Conclusion: The results showed Zinc sulfate had no effect on the improvement of wheezing after bronchiolitis.

  15. [Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)].

    Science.gov (United States)

    Goeckenjan, G

    2003-05-01

    Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) designates interstitial lung changes in smokers, characterized histologically by bronchiolocentric accumulation of pigmented alveolar macrophages and fibrotic or cellular inflammatory changes of pulmonary interstitium. The definition is nearly identical to that of condensate pneumopathy, smoker's pneumopathy or smoker's lung, defined by accumulation of pigmented alveolar macrophages with bland alveoloseptal or peribronchial fibrosis and cellular inflammation of the bronchial wall. In addition to respiratory bronchiolitis, which is found in nearly all smokers, RB-ILD comprises a broad spectrum of varying degrees of the interstitial reaction to the exogenous injury of inhalation smoking with gradual transition to desquamative interstitial pneumonia (DIP). In most cases RB-ILD manifestations are subclinical and detected coincidentally. Radiographic features are reticulonodular and ground glass opacities of the lung. The high resolution computed tomography reveals centrilobular nodules, ground glass opacities, thickening of bronchial walls, and in some cases a reticular pattern. Mild emphysema is frequent. Lung function analysis reveals only minor restrictive or obstructive defects in most cases, often combined with hyperinflation. CO diffusing capacity is slightly to moderately impaired. Pronounced interstitial lung diseases with serious restrictive defects and arterial hypoxemia have been reported infrequently. In differential diagnosis smoking related interstitial lung diseases (DIP, Langerhans cell histiocytosis, idiopathic pulmonary fibrosis) and other interstitial lung diseases have to be excluded. In most cases diagnosis can be achieved by bronchoalveolar lavage and transbronchial lung biopsy. In cases of pronounced interstitial lung disease or assumption of an additional interstitial lung disease besides RB-ILD a thoracoscopic or open lung biopsy can be necessary. RB-ILD has a favourable

  16. [Correction of dyslipidemia in patients with arteriosclerosis obliterans as a method for improving the results of endovascular angioplasty].

    Science.gov (United States)

    Petukhov, V A; Seitmykhamedov, M D; Dibirov, A D; Dubovik, S G; Sergeeva, N A; Prokubovskiĭ, V I

    1992-01-01

    The authors analyse the results of endovascular angioplasty of the pelvic and lower limb arteries in 147 patients of various age suffering from arteriosclerosis obliterans. It was found that the results of endovascular angioplasty depended on the state of the lipid balance. The necessity for correcting the shifts in the lipid metabolism is shown. The article evaluates the results of dyslipidemia correction by dietotherapy, pharmacotherapy, and operation for partial ilio-shunting in patients with arteriosclerosis obliterans of the pelvic and lower limb arteries. The efficacy of the listed methods of treatment are analysed according to the severity of dyslipidemia. PMID:1457133

  17. A prospective randomised trial comparing nasogastric with intravenous hydration in children with bronchiolitis (protocol The comparative rehydration in bronchiolitis study (CRIB

    Directory of Open Access Journals (Sweden)

    Borland Meredith

    2010-06-01

    Full Text Available Abstract Background Bronchiolitis is the most common reason for admission of infants to hospital in developed countries. Fluid replacement therapy is required in about 30% of children admitted with bronchiolitis. There are currently two techniques of fluid replacement therapy that are used with the same frequency-intravenous (IV or nasogastric (NG. The evidence to determine the optimum route of hydration therapy for infants with bronchiolitis is inadequate. This randomised trial will be the first to provide good quality evidence of whether nasogastric rehydration (NGR offers benefits over intravenous rehydration (IVR using the clinically relevant continuous outcome measure of duration of hospital admission. Methods/Design A prospective randomised multi-centre trial in Australia and New Zealand where children between 2 and 12 months of age with bronchiolitis, needing non oral fluid replacement, are randomised to receive either intravenous (IV or nasogastric (NG rehydration. 750 patients admitted to participating hospitals will be recruited, and will be followed daily during the admission and by telephone 1 week after discharge. Patients with chronic respiratory, cardiac, or neurological disease; choanal atresia; needing IV fluid resuscitation; needing an IV for other reasons, and those requiring CPAP or ventilation are excluded. The primary endpoint is duration of hospital admission. Secondary outcomes are complications, need for ICU admission, parental satisfaction, and an economic evaluation. Results will be analysed using t-test for continuous data, and chi squared for categorical data. Non parametric data will be log transformed. Discussion This trial will define the role of NGR and IVR in bronchiolitis Trail registration The trial is registered with the Australian and New Zealand Clinical Trials Registry - ACTRN12605000033640

  18. Clinical profile of bronchiolitis in infants younger than 90 days in Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Syed Amir Ahmad

    2014-01-01

    Full Text Available Background: Bronchiolitis is a self-limiting disease of children caused by viral infections of the small airways with a wide spectrum of illness severity. Search of the literature reveals a need for refinement of criteria for testing for concomitant severe bacterial infections as well as appropriate therapeutic interventions for patients <90-day-old diagnosed with clinical bronchiolitis. We believe that a better understanding of the disease spectrum will help optimize health-care delivery to these patients. Aims: The aim of this study was to determine the clinical profile at presentation, disease course and outcome of bronchiolitis in <3-month-old infants who presented to our Pediatric Emergency Department (PED during one disease season. Settings: Retrospective chart review during one bronchiolitis season, from November 1, 2011 to April 20, 2012. Subjects: All <90-day-old infants presenting with clinical bronchiolitis presenting to Urban PED of a tertiary care university hospital during one bronchiolitis season. Materials and Methods: A retrospective chart review based on computer records of all emergency department visits of infants less than 90 days with a clinical diagnosis of bronchiolitis, covering the period between November 1, 2011 and April 30, 2012. Results: Out of the total of 1895 infants <90 days of age, 141 had a clinical diagnosis of bronchiolitis and 35 needed admission to hospital. Blood for culture was obtained from 47 infants, urine for culture was obtained from 46 infants and cerebrospinal fluid for culture was obtained from eight infants. One case of bacteremia was documented, but this was found to be a contaminant. No cases of meningitis occurred among these infants. However, one infant had a positive urine culture consistent with infection (Escherichia coli . Conclusion: Based on the results, it can be conclude that the risk of bacteremia or meningitis among infants <90 days of age with fever and bronchiolitis is low. The

  19. Use of flow cytometry to investigate the cytokine response pattern in infants with respiratory syncytial virus infection and bronchiolitis

    Institute of Scientific and Technical Information of China (English)

    陈志敏; 杜立中; 毛建华; 汤永民

    2002-01-01

    Objective: To investigate the cytokine response pattern (IL-4/IFN-γ) in infants with RSV infections and bronchiolitis during the acute phase. Methods: Four-color flow cytometry was used to measure intracellular IL-4 and IFN-γ expressions in peripheral blood CD3+ and CD8+ lymphocytes from RSV-infected and bronchiolitis infants. Serum IL-4 and IFN-γ levels were also determined. Results: RSV-infected and bronchiolitis infants showed no statistical differences from not-RSV-infected or pneumonia infants and control in the frequency of IL-4 and IFN-γ expressions in CD3+CD8- lymphocytes, showed no obvious Th1/Th2 imbalance, while IFN-γ was expressed much more frequently in CD3+CD8+ lymphocytes. Systematically, RSV-infected and bronchiolitis infants showed much lower levels of serum IL-4 and IL-4/IFN-γ ratios and much higher serum IFN-γ levels than control. However, there were no statistical differences in the above three indices between RSV-infected and not-RSV infected infants or between bronchiolitis and pneumonia infants, except that bronchiolitis infants had a higher level of serum IFN-γ than pneumonia infants statistically. Conclusions: There is no type-2 cytokine response predominance in the acute phase of RSV infection and bronchiolitis. IL-4 production is suppressed and IFN-γ production upregulated, the latter being most prominent in bronchiolitis infants.

  20. Dynamics of nasopharyngeal pneumococcal carriage during the course of viral bronchiolitis.

    Science.gov (United States)

    Faber, Tina E; Schuurs, Theo A; Veeger, Nic J G M; Hennus, Marije P; Bont, Louis J

    2016-08-01

    The effect of viral infection on nasopharyngeal carriage of Streptococcus pneumoniae during childhood is not well known. We studied dynamics of pneumococcal colonization by quantitative PCR during the natural course of viral bronchiolitis. At time of admission, 47 (47%) of 100 patients with bronchiolitis carried pneumococci. In patients with viral bronchiolitis who did not receive antibiotics, pneumococcal load decreased from time of admission to discharge (n = 35, cycle threshold 23 vs. 25, P = 0.0017) and from discharge to follow-up (n = 22, cycle threshold 25 vs. 40, P = 0.003). We conclude that viral respiratory infection is negatively associated with pneumococcal colonization of the upper airways. Pediatr Pulmonol. 2016;51:863-867. © 2016 Wiley Periodicals, Inc. PMID:26859410

  1. Preliminary clinical application of SilverHawk directional atherectomy device in arteriosclerosis obliterans of lower extremity

    International Nuclear Information System (INIS)

    Objective: To evaluate the clinical efficiency and security of SilverHawk directional atherectomy device in treating arteriosclerosis obliterans of lower extremity. Methods: Seven patients with lower extremity ischemia were treated with SilverHawk directional atherectomy. The lesions length varied from 1.8 to 14.5 cm. Clinical symptoms (Fontaine classification) included 4 cases of grade Ⅱ b, I case of grade Ⅲ, 2 cases of grade Ⅳ. The ABI varied from 0.28 to 0.65. Patency was evaluated with color duplex sonography or CTA besides clinical examination during follow-up. Results: Seven occlusive lesions were recanalizated successfully. The technical success (residual stenosis < 30%) rate was 100%. Clinical symptoms were obviously improved or disappeared. The ABI varied from 0.82 to 1.18 (P=0.002), and Fontaine classification included 6 cases of Ⅰ; 1 case of Ⅱ a. Patency rate was 100% and the ABI varied from 0.75 to 1.14 (P=0.938). Fontaine classifications keep consistent 1-6 months after operation. Conclusion: SilverHawk directional atherectomy was a safe and effective way for treatment of lower arteriosclerosis obliteran. (authors)

  2. Infant swimming in chlorinated pools and the risks of bronchiolitis, asthma and allergy.

    Science.gov (United States)

    Voisin, C; Sardella, A; Marcucci, F; Bernard, A

    2010-07-01

    Recent studies suggest that swimming in chlorinated pools during infancy may increase the risks of lower respiratory tract infection. The aim of the present study was to assess the influence of swimming in chlorinated pools on the risks of bronchiolitis and its late consequences. A total of 430 children (47% female; mean age 5.7 yrs) in 30 kindergartens were examined. Parents completed a questionnaire regarding the child's health history, swimming practice and potential confounders. Attendance at indoor or outdoor chlorinated pools ever before the age of 2 yrs was associated with an increased risk of bronchiolitis (OR 1.68; 95% CI 1.08-2.68; p = 0.03), which was exposure-dependent for both types of pool (p-value for trend 20 h spent in chlorinated pools during infancy. Infant swimmers who developed bronchiolitis also showed higher risks of asthma and respiratory allergies later in childhood. Swimming pool attendance during infancy is associated with a higher risk of bronchiolitis, with ensuing increased risks of asthma and allergic sensitisation. PMID:20075053

  3. Placebo-controlled trial of nebulization with adrenaline in acute bronchiolitis: a quasi-experimental study

    International Nuclear Information System (INIS)

    Background: Bronchiolitis is an acute inflammatory obstruction. of small In children that occurs In first two years of life and is by fever, rhinitis, cough, tachypnoea, expiratory wheeze and increased respiratory effort To study efficacy of nebulized adrenaline compared with placebo in acute bronchiolitis. Quasi-experimental study carried out at Department of aediatrics, King Edward Medical University/ Mayo Hospital, Lahore from October 2006 through March 2007. After consent from parents, sixty children of age between 2 months to 2 years with the first episode consistent with clinical case definition of bronchiolitis were included by using convenient sampling. clinical scoring system was used to grade the severity of disease as well as to monitor the efficacy of intervention. Those having score = 8 were randomly allocated to the two study groups. The information was recorded at 0 minute and effect of each method of treatment was followed for 90 minutes. Results: Our study population was 60 children. The mean age was 11:1:6 Months. Male to female ratio was 1.2: 1. Mean weight of the Children was 9:1:3 kg. Improvement in clinical score, oxygen saturation, and length of hospital at 0 and 90 minutes was noted in both groups but when compared with placebo, there was no Statistically significant difference. Conclusion: There is no difference in the efficacy of nebulization with adrenaline compared with placebo in the management of acute bronchiolitis. (author)

  4. Microdialysis as a tool for drug quantification in the bronchioles of anaesthetized pigs

    DEFF Research Database (Denmark)

    Rottbøll, Lisa Amanda Holm; Friis, Christian

    2014-01-01

    guided through a tracheostomy into the distal bronchioles using an insertion tube. Intravenously administered inulin served as a marker of extracellular fluid contamination of PELF. Concentrations of free drug in MD fractions were compared to total and non-protein-bound drug concentrations in plasma...

  5. Treatment of urethral strictures in balanitis xerotica obliterans (BXO) using circular buccal mucosal meatoplasy: Experience of 15 cases

    OpenAIRE

    Abdulmuttalip Simsek; Sinasi Yavuz Onol; Omer Kurt

    2014-01-01

    Objectives: Balanitis xerotica obliterans (BXO) related strictures involving the external urethral meatus. We reviewed our result with the use of circular mucosal graft in the reconstruction of strictures. Methods: Between March 1997 and January 2012, 15 patients underwent circular buccal mucosal urethroplasy for BXO related anterior urethral strictures. Urethral catheter was removed within 2 weeks. Follow-up included patient symptoms assessment, cosmetic outcome and uroflowmetry. Results: Me...

  6. Thromboangiitis obliterans

    Science.gov (United States)

    ... hands and feet get cold or during emotional stress. Pain in the legs , ankles, or feet when walking (intermittent claudication). The pain is often located in the arch of the foot. Skin changes or small painful ulcers on the fingers or toes.

  7. Use of flow cytometry to investigate the cytokine response pattern in infants with respiratory syncytial virus infection and bronchiolitis

    Institute of Scientific and Technical Information of China (English)

    2002-01-01

    Objective:To investigate the cytokine response pattern(IL-4/IFN-γ)in infants with RSV infections and bronchiolitis during the acute phase,Methods:Four-color flow cytometry was used to measure intracellular IL-4 and IFN-γ expressions in peripheral blood CD3+ and CD*+ lymphocytes from RSV-infected and bronchiolitis infants.Serum IL-4 and INF-γ levels were also determined.Results:RSV-infected and bronchiolitis infants showed no statistical differences from not-RSV-infected or pneumonia infants and control in the frequency of IL-4 and INF-γ levels were also determined.Results;RSV-infected and bronchiolitis infants showed no statistical differences from not-RSV-infected or pneumonia infants and control in the frequency of IL-4 and IFN-γ expressions in CD3+CD8- lymphocytes,showed no obvious Th1/Th2 imbalance,while IFN-γ was expressed much more frequently in CD3+CD*+ lymphocytes.Systematically,RSV-infected and bronchiolitis infants showed moch lower levels of serum IL-4 and IL-4/INF-γ ratios and much higher serum IFN-γ levels than control.Howerver,there were no statistical differences in the above three indices between RSV-infected and not-RSV infected infants or between bronchiolitis and pneumonia infants,except that bronchiolitis infants had a higher level of serum INF-γ than pneumonia infants statistically .Conclusions:There is no type-2 cytokine response predominance in the acute thase of RSV infection and bronchilitis.IL-4 productioin is suppressed and IFN-γ production upregulated,the latter being most prominent in bronchiolitis infants.

  8. The use of C-reactive protein in predicting bacterial co-Infection in children with bronchiolitis

    OpenAIRE

    Mohamad Fares; Sawsan Mourad; Mariam Rajab; Nahida Rifai

    2011-01-01

    Background: Bronchiolitis is a potentially life-threatening respiratory illness commonly affecting children who are less than two years of age. Patients with viral lower respiratory tract infection are at risk for co-bacterial infection. Aim: The aim of our study was to evaluate the use of C-reactive protein (CRP) in predicting bacterial co-infection in patients hospitalized for bronchiolitis and to correlate the results with the use of antibiotics. Patients and Methods: This is a prospective...

  9. Diagnostic role of magnetic resonance angiography in Swyer James syndrome: Case series of two cases

    OpenAIRE

    Parashari Umesh; Singh Ragini; Parihar Anit; Aga Pallavi; Yadav Rajesh

    2010-01-01

    Swyer James syndrome is a rare syndrome which occurs due to viral illness in early childhood. The post infective obliterative bronchiolitis results in arrest of lung growth and alveolarization with reduced vascularity resulting in classical radiological features. We describe two cases of patients fulfilling all the criteria of the syndrome - 1) Unilateral hyperlucent small lung in chest radiograph with air trapping on expiration, small ipsilateral hila and pulmonary artery. 2) Diffuse decreas...

  10. Not all infantile respiratory distress in winter is bronchiolitis: congenital lobar emphysema

    OpenAIRE

    Taqvi, Laura; Griksaitis, Michael; Eastham, Katherine

    2011-01-01

    The authors report the case of a 4-week-old male infant presented during the winter period with respiratory distress. He had a 3 day history of cough and coryza, and a 2 day history of breathlessness and reduced feeding. He had evidence of tachypnoea, subcostal recession and hypoxia on examination. An initial diagnosis of bronchiolitis was made. The authors explore how the correct diagnosis of congenital lobar emphysema (CLE) was reached, highlighting key clinical signs and investigations. He...

  11. Diffuse Neuroendocrine Hyperplasia with Obliterative Bronchiolitis and Usual Interstitial Pneumonia: An Unusual "Headcheese Pattern" with Nodules.

    Science.gov (United States)

    Pietrangeli, V; Piciucchi, S; Tomassetti, S; Ravaglia, C; Gurioli, C; Gurioli, Ch; Cavazza, A; Dubini, A; Poletti, V

    2015-12-01

    A 74-year-old non-smoker female presented to our attention with a history of dyspnea and cough. CT scan revealed multiple areas of patchy ground glass attenuation associated to a diffuse mosaic oligoemia. Scattered bilateral subcentimetric pulmonary nodules were also present. Patient underwent a surgical lung biopsy. Specimens showed features of diffuse neuroendocrine hyperplasia, microhoneycombing, fibroblast foci. A final diagnosis of diffuse neuroendocrine hyperplasia with obliterative bronchiolitis and UIP was rendered. PMID:26446675

  12. Helium-oxygen mixture does not improve gas exchange in mechanically ventilated children with bronchiolitis

    OpenAIRE

    Gross, Matthew F; Spear, Robert M.; Peterson, Bradley M

    2000-01-01

    Introduction: Heliox has been found to reduce both the arterial carbon dioxide tension (PaCO2) and work of breathing in children and adults with status asthmaticus. We hypothesized that, in mechanically ventilated children with bronchiolitis, increasing the ratio of helium:oxygen concentrations would improve both ventilation and oxygenation. Objective: To examine the effect of varying concentrations of heliox mixtures on ventilation and oxygenation in mechanically ventilated children with bro...

  13. Matrilysin-1 Mediates Bronchiolization of Alveoli, a Potential Premalignant Change in Lung Cancer

    OpenAIRE

    Wang, Xiao-Yang; Demelash, Abeba; Kim, Heungnam; Jensen-Taubman, Sandra; Dakir, El Habib; Ozbun, Laurent; Birrer, Michael J.; Linnoila, R. Ilona

    2009-01-01

    Matrilysin-1 (also called matrix metalloproteinase-7) is expressed in injured lung and in cancer but not in normal epithelia. Bronchiolization of the alveoli (BOA), a potential precursor of lung cancer, is a histologically distinct type of metaplasia that is composed of cells resembling airway epithelium in the alveolar compartment. We demonstrate that there is increased expression of matrilysin-1 in human lesions and BOA in the CC10-human achaete-scute homolog-1 transgenic mouse model. Force...

  14. Oxygen saturation targets in infants with bronchiolitis (BIDS): a double-blind, randomised, equivalence trial

    OpenAIRE

    Cunningham, Steve; Rodriguez Carbonell, Aryelly; Adams, Tim; Boyd, Kathleen A; Butcher, Isabella; Enderby, Beth; MacLean, Morag; McCormick, Jonathan; Paton, James Y; Wee, Fiona; Thomas, Huw; Riding, Kay; Turner, Steve W.; Williams, Chris; McIntosh, Emma

    2015-01-01

    BACKGROUND: The American Academy of Pediatrics recommends a permissive hypoxaemic target for an oxygen saturation of 90% for children with bronchiolitis, which is consistent with the WHO recommendations for targets in children with lower respiratory tract infections. No evidence exists to support this threshold. We aimed to assess whether the 90% or higher target for management of oxygen supplementation was equivalent to a normoxic 94% or higher target for infants admitted to hospital with vi...

  15. Etiological diagnosis reduces the use of antibiotics in infants with bronchiolitis

    Directory of Open Access Journals (Sweden)

    Ângela Esposito Ferronato

    2012-09-01

    Full Text Available OBJECTIVE: Acute bronchiolitis is a leading cause of infant hospitalization and is most commonly caused by respiratory syncytial virus. Etiological tests are not required for its diagnosis, but the influence of viral screening on the therapeutic approach for acute bronchiolitis remains unclear. METHODS: A historical cohort was performed to assess the impact of viral screening on drug prescriptions. The study included infants up to one year of age who were hospitalized for bronchiolitis. Virus screening was performed using immunofluorescence assays in nasopharyngeal aspirates. The clinical data were obtained from the patients' medical records. Therapeutic changes were considered to be associated with viral screening when made within 24 hours of the release of the results. RESULTS: The frequency of prescriptions for beta agonists, corticosteroids and antibiotics was high at the time of admission and was similar among the 230 patients. The diagnosis of pneumonia and otitis was associated with the introduction of antibiotics but did not influence antibiotics maintenance after the results of the virus screening were obtained. Changes in the prescriptions were more frequent for the respiratory syncytial virus patients compared to patients who had negative viral screening results (p =0.004, especially the discontinuation of antibiotics (p<0.001. The identification of respiratory syncytial virus was associated with the suspension of antibiotics (p= 0.003, even after adjusting for confounding variables (p = 0.004; however, it did not influence the suspension of beta-agonists or corticosteroids. CONCLUSION: The identification of respiratory syncytial virus in infants with bronchiolitis was independently associated with the discontinuation of antibiotics during hospitalization

  16. CT densitovolumetry in children with obliterative bronchiolitis: correlation with clinical scores and pulmonary function test results* , **

    OpenAIRE

    Mocelin, Helena; Bueno, Gilberto; Irion, Klaus; Marchiori, Edson; Sarria, Edgar; Watte, Guilherme; Hochhegger, Bruno

    2013-01-01

    OBJECTIVE: To determine whether air trapping (expressed as the percentage of air trapping relative to total lung volume [AT%]) correlates with clinical and functional parameters in children with obliterative bronchiolitis (OB). METHODS: CT scans of 19 children with OB were post-processed for AT% quantification with the use of a fixed threshold of −950 HU (AT%950) and of thresholds selected with the aid of density masks (AT%DM). Patients were divided into three groups by AT% severity. We exami...

  17. Efficacy comparison of nebulizator or spacer usage for acute bronchiolitis treatment in emergency department

    OpenAIRE

    SAZ, Eylem Ulaş; MİDYAT, Levent; Duyu, Muhterem; OZANANAR, Yeliz; KARAPINAR, Bülent; ÖZÇETİN, Mustafa

    2009-01-01

    Acute bronchiolitis is one of the most common causes of lower respiratory tract infections in children. Nebulization of inhaled beta-2 agonists in emergency departments is time-consuming. In this study, effects of inhalation of appropriate dose of inhaled salbutamol by nebulization or by spacer are compared. Thirty-five patients between ages of 1-24 months old who were admitted in daytime to Ege University Faculty of Medicine Child Emergency Department for cough and/or wheezing and diagnosed ...

  18. Trends in Respiratory Syncytial Virus and Bronchiolitis Hospitalization Rates in High-Risk Infants in a United States Nationally Representative Database, 1997–2012

    Science.gov (United States)

    Doucette, Abigail; Jiang, Xiaohui; Fryzek, Jon; Coalson, Jenna; McLaurin, Kimmie; Ambrose, Christopher S.

    2016-01-01

    Background Respiratory syncytial virus (RSV) causes significant pediatric morbidity and is the most common cause of bronchiolitis. Bronchiolitis hospitalizations declined among US infants from 2000‒2009; however, rates in infants at high risk for RSV have not been described. This study examined RSV and unspecified bronchiolitis (UB) hospitalization rates from 1997‒2012 among US high-risk infants. Methods The Kids’ Inpatient Database (KID) infant annual RSV (ICD-9 079.6, 466.11, 480.1) and UB (ICD-9 466.19, 466.1) hospitalization rates were estimated using weighted counts. Denominators were based on birth hospitalizations with conditions associated with high-risk for RSV: chronic perinatal respiratory disease (chronic lung disease [CLD]); congenital airway anomalies (CAA); congenital heart disease (CHD); Down syndrome (DS); and other genetic, metabolic, musculoskeletal, and immunodeficiency conditions. Preterm infants could not be identified. Hospitalizations were characterized by mechanical ventilation, inpatient mortality, length of stay, and total cost (2015$). Poisson and linear regression were used to test statistical significance of trends. Results RSV and UB hospitalization rates were substantially elevated for infants with higher-risk CHD, CLD, CAA and DS without CHD compared with all infants. RSV rates declined by 47.0% in CLD and 49.7% in higher-risk CHD infants; no other declines in high-risk groups were observed. UB rates increased in all high-risk groups except for a 22.5% decrease among higher-risk CHD. Among high-risk infants, mechanical ventilation increased through 2012 to 20.4% and 13.5% of RSV and UB hospitalizations; geometric mean cost increased to $31,742 and $25,962, respectively, and RSV mortality declined to 0.9%. Conclusions Among high-risk infants between 1997 and 2012, RSV hospitalization rates declined among CLD and higher-risk CHD infants, coincident with widespread RSV immunoprophylaxis use in these populations. UB

  19. Clinical significance of changes of plasma CGRP and VIP levels in infants with bronchiolitis

    International Nuclear Information System (INIS)

    Objective: To explore the clinical significance of changes of calcitonin gene-related peptide (CGRP) and vasoactive intestinal peptide (VIP) levels in infants (2-24months) with bronchiolitis. Methods: Plasma levels of CGRP and VIP were determined with RIA in 31 infants with bronchiolitis both during acute infection and convalescence as well as in 35 controls. Results: Plasma CGRP levels in patients during acute infection were significantly higher than those in patients during convalescence and in controls (P<0.05). Levels of CGRP dropped during convalescence, but still remained significantly higher than those in controls (P<0.05). The reverse was true for the plasma VIP levels. The plasma VIP levels in patients during acute infection were significantly lower than those in patients during convalescence and in controls (P<0.05). During convalescence, the plasma VIP levels rose but remained significantly lower than those in controls (P<0.05). Conclusion: There were dynamic changes of plasma CGRP and VIP levels in the course of infant bronchiolitis and the two peptides played opposite roles. (authors)

  20. Rib fractures after chest physiotherapy for bronchiolitis or pneumonia in infants

    Energy Technology Data Exchange (ETDEWEB)

    Chalumeau, Martin [Service de Pneumologie et d' Allergologie Pediatriques, Hopital Necker-Enfants Malades, Paris (France); Service de Pediatrie, Hopital Saint-Vincent-de-Paul, Paris (France); Foix-l' Helias, Laurence; Scheinmann, Pierre; Zuani, Pierre [Service de Pneumologie et d' Allergologie Pediatriques, Hopital Necker-Enfants Malades, Paris (France); Gendrel, Dominique [Service de Pediatrie, Hopital Saint-Vincent-de-Paul, Paris (France); Ducou-le-Pointe, Hubert [Service de Radiologie, Hopital d' Enfants Armand-Trousseau, Avenue du Docteur Arnold Netter, 75571 Paris Cedex 12 (France)

    2002-09-01

    Heading AbstractBackground. The reported causes of rib fractures in infants are: child abuse, accidental injury, cardiopulmonary resuscitation, bone fragility, birth trauma and severe cough.Objective. To report chest physiotherapy (CPT) as a new cause of rib fractures in five infants.Materials and methods. We retrospectively identified all infants with rib fractures after CPT for bronchiolitis or pneumonia over a 4-year period in two paediatric and one paediatric radiology units in three university hospitals in Paris.Results. Five boys were identified. Their median age was 3 months. None had any other potential cause of rib fractures. The indication for CPT was bronchiolitis in four cases and pneumonia in one. The median number of rib fractures was four (range 1-5). Fractures were located between the 3rd and 8th ribs; they were lateral in four patients and posterior in one; they were unilateral in four patients and bilateral in one. Evolution was favourable in all cases. The prevalence of rib fractures after CPT during the study period was estimated at 1 in 1,000 infants hospitalised for bronchiolitis or pneumonia.Conclusions. CPT should be considered a potential, but very rare cause of rib fractures in infants. It can be of clinical relevance when rib fractures are the only feature suggestive of child abuse. (orig.)

  1. Rib fractures after chest physiotherapy for bronchiolitis or pneumonia in infants

    International Nuclear Information System (INIS)

    Heading AbstractBackground. The reported causes of rib fractures in infants are: child abuse, accidental injury, cardiopulmonary resuscitation, bone fragility, birth trauma and severe cough.Objective. To report chest physiotherapy (CPT) as a new cause of rib fractures in five infants.Materials and methods. We retrospectively identified all infants with rib fractures after CPT for bronchiolitis or pneumonia over a 4-year period in two paediatric and one paediatric radiology units in three university hospitals in Paris.Results. Five boys were identified. Their median age was 3 months. None had any other potential cause of rib fractures. The indication for CPT was bronchiolitis in four cases and pneumonia in one. The median number of rib fractures was four (range 1-5). Fractures were located between the 3rd and 8th ribs; they were lateral in four patients and posterior in one; they were unilateral in four patients and bilateral in one. Evolution was favourable in all cases. The prevalence of rib fractures after CPT during the study period was estimated at 1 in 1,000 infants hospitalised for bronchiolitis or pneumonia.Conclusions. CPT should be considered a potential, but very rare cause of rib fractures in infants. It can be of clinical relevance when rib fractures are the only feature suggestive of child abuse. (orig.)

  2. Predicting the severity of acute bronchiolitis in infants: should we use a clinical score or a biomarker?

    Science.gov (United States)

    Amat, Flore; Henquell, Cécile; Verdan, Matthieu; Roszyk, Laurence; Mulliez, Aurélien; Labbé, André

    2014-11-01

    Krebs von den Lungen 6 antigen (KL-6) has been shown to be a useful biomarker of the severity of Respiratory syncytial virus bronchiolitis. To assess the correlation between the clinical severity of acute bronchiolitis, serum KL-6, and the causative viruses, 222 infants with acute bronchiolitis presenting at the Pediatric Emergency Department of Estaing University Hospital, Clermont-Ferrand, France, were prospectively enrolled from October 2011 to May 2012. Disease severity was assessed with a score calculated from oxygen saturation, respiratory rate, and respiratory effort. A nasopharyngeal aspirate was collected to screen for a panel of 20 respiratory viruses. Serum was assessed and compared with a control group of 38 bronchiolitis-free infants. No significant difference in KL-6 levels was found between the children with bronchiolitis (mean 231 IU/mL ± 106) and those without (230 IU/mL ± 102), or between children who were hospitalized or not, or between the types of virus. No correlation was found between serum KL-6 levels and the disease severity score. The absence of Human Rhinovirus was a predictive factor for hospitalization (OR 3.4 [1.4-7.9]; P = 0.006). Older age and a higher oxygen saturation were protective factors (OR 0.65[0.55-0.77]; P < 0.0001 and OR 0.67 [0.54-0.85] P < 0.001, respectively). These results suggest that in infants presenting with bronchiolitis for the first time, clinical outcome depends more on the adaptive capacities of the host than on epithelial dysfunction intensity. Many of the features of bronchiolitis are affected by underlying disease and by treatment. PMID:24374757

  3. Pooled Sequencing of Candidate Genes Implicates Rare Variants in the Development of Asthma Following Severe RSV Bronchiolitis in Infancy.

    Directory of Open Access Journals (Sweden)

    Dara G Torgerson

    Full Text Available Severe infection with respiratory syncytial virus (RSV during infancy is strongly associated with the development of asthma. To identify genetic variation that contributes to asthma following severe RSV bronchiolitis during infancy, we sequenced the coding exons of 131 asthma candidate genes in 182 European and African American children with severe RSV bronchiolitis in infancy using anonymous pools for variant discovery, and then directly genotyped a set of 190 nonsynonymous variants. Association testing was performed for physician-diagnosed asthma before the 7th birthday (asthma using genotypes from 6,500 individuals from the Exome Sequencing Project (ESP as controls to gain statistical power. In addition, among patients with severe RSV bronchiolitis during infancy, we examined genetic associations with asthma, active asthma, persistent wheeze, and bronchial hyperreactivity (methacholine PC20 at age 6 years. We identified four rare nonsynonymous variants that were significantly associated with asthma following severe RSV bronchiolitis, including single variants in ADRB2, FLG and NCAM1 in European Americans (p = 4.6x10-4, 1.9x10-13 and 5.0x10-5, respectively, and NOS1 in African Americans (p = 2.3x10-11. One of the variants was a highly functional nonsynonymous variant in ADRB2 (rs1800888, which was also nominally associated with asthma (p = 0.027 and active asthma (p = 0.013 among European Americans with severe RSV bronchiolitis without including the ESP. Our results suggest that rare nonsynonymous variants contribute to the development of asthma following severe RSV bronchiolitis in infancy, notably in ADRB2. Additional studies are required to explore the role of rare variants in the etiology of asthma and asthma-related traits following severe RSV bronchiolitis.

  4. [Topical therapy of balanitis xerotica obliterans in childhood. Long-term clinical results and an overview].

    Science.gov (United States)

    Ebert, A-K; Vogt, T; Rösch, W H

    2007-12-01

    Balanitis xerotica obliterans (BXO) is a chronic and progressive dermatitis of unknown aetiology and incidence. Its management in childhood is controversial. Although in most cases only the prepuce is affected, meatal and urethral involvement may lead to major surgical reconstruction. Therefore complete surgical excision of the affected skin is considered to be mandatory. In case of involvement, incidental histological evidence or a relapse, or when complete removal of the affected skin is not possible, a topical therapy should be implemented. In a retrospective analysis of our study population (13 children) with BXO, relapse rate was lower after topical therapy with tacrolimus (Protopic), a highly selective immune modulator, than after the standard anti-inflammatory therapy with betamethasone. The use of tacrolimus ointment is a safe therapy with no severe side effects. Due to the fact that there are no predictive factors for progression or relapse of BXO, we consider a topical anti-inflammatory therapy is always indicated after any type of surgery for BXO. Follow-up monitoring should be close, so that any relapse can be detected and treated as early as possible. PMID:17994212

  5. Long standing balanitis xerotica obliterans resulting in renal impairment in a child.

    Science.gov (United States)

    Sandler, Gideon; Patrick, Emily; Cass, Danny

    2008-08-01

    Balanitis xerotica obliterans (BXO) is the most common cause of pathological phimosis in boys. Presented here is the case of a previously well 13-year-old boy who developed obstructive renal impairment (serum creatinine = 190 micromol/l) at least in part from phimosis due to BXO. A circumcision and, 2.5 months later, meatal dilatation were done. Nine months after his initial presentation, his serum creatinine returned to a permanently elevated nadir of 119 mumol/l. Presentation with the complications of phimosis can be delayed in teenage boys because they may feel embarrassed to come forward. Circumcision remains the definitive treatment of BXO induced phimosis though if the penile meatus is involved, more complex surgery is sometimes required. Topical steroids are useful for residual disease. Follow-up is very important due to the frequent involvement of the skin of the glans. In the very long term there is an increased chance of penile malignancy, which can occur even after circumcision. PMID:18587589

  6. [Balanitis xerotica obliterans with phimosis in elderly patients presenting with difficulty in urination].

    Science.gov (United States)

    Nemoto, Kaoru; Ishidate, Takuzo

    2013-06-01

    Eight elderly patients (average age 76.1±4.3 years) with balanitis xerotica obliterans (BXO) accompanied by phimosis presented with difficulty in urination. Preoperative average international prostate symptom score, average maximum urinary flow rate, and average volume of residual urine were 20.7±6.3 points (n=8), 5.1±3.6 ml/s (n=5), and 85.4±77.3 ml (n=8), respectively. Some of the patient's complaints, such as severe dribbling of urine, urinary stream division, and ballooning of the foreskin, were not included in the items of the major questionnaire on urination. Dorsal incision and circumcision was performed in all patients, and all were pathologically diagnosed with BXO. Meatoplasty was performed in one patient with a meatal stenosis. No coexistence of penile cancer was observed. Statistically significant improvements were observed in subjective and objective findings after treatment. In conclusion, BXO with phimosis in elderly patients should be considered as a cause of lower urinary tract symptoms. PMID:23827865

  7. Bronquiolite obliterante pós-infecciosa: aspectos clínicos e exames complementares de 48 crianças Post-infectious bronchiolitis obliterans: clinical aspects and complementary testing of 48 children

    OpenAIRE

    Rosaly Vieira dos Santos; Rosário, Nelson A.; Carlos Antônio Ried

    2004-01-01

    INTRODUÇÃO: A evolução clínica da bronquiolite obliterante pós infecciosa é variável. OBJETIVO: Verificar as características clínicas, a evolução e os exames complementares de 48 pacientes com bronquiolite obliterante (BO) pós-infecciosa. MÉTODO: Estudo observacional e retrospecitvo. O diagnóstico de bronquiolite obliterante foi baseado em critérios clínicos, tomográficos e pela exclusão de outras doenças. Avaliou-se a história prévia ao diagnóstico e exames complementares. A saturação arteri...

  8. Associação de bronquiolite obliterante pós-infecciosa e hemossiderose pulmonar na infância Postinfectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in childhood

    OpenAIRE

    Leonardo Araújo Pinto; Anick Oliveira; Sintia Collaziol; Paulo Márcio Pitrez; Marcus Jones; João Carlos Prolla; Marisa Dolhnikoff; Renato T Stein

    2006-01-01

    Descreve-se uma apresentação rara de bronquiolite obliterante associada a hemossiderose pulmonar, em paciente de nove anos com sintomas respiratórios persistentes iniciados após episódio de bronquiolite aguda grave aos sete meses. Após o episódio agudo, apresentou sintomas respiratórios persistentes, piorando significativamente aos sete anos, quando começou a apresentar dificuldade respiratória em pequenos esforços. A tomografia computadorizada de tórax demonstrou achados compatíveis com bron...

  9. Função pulmonar de crianças e adolescentes com bronquiolite obliterante pós-infecciosa Pulmonary function in children and adolescents with postinfectious bronchiolitis obliterans

    OpenAIRE

    Rita Mattiello; Javier Mallol; Gilberto Bueno Fischer; Helena Teresinha Mocelin; Belkys Rueda; Edgar Enrique Sarria

    2010-01-01

    OBJETIVO: Descrever a função pulmonar de crianças e adolescentes com bronquiolite obliterante pós-infecciosa (BOPI) e avaliar potenciais fatores de risco para pior função pulmonar. MÉTODOS: A função pulmonar de 77 participantes, com idades de 8-18 anos, foi avaliada por meio de espirometria e pletismografia. Os seguintes parâmetros foram analisados: CVF, VEF1, FEF25-75%, VEF1/CVF, VR, CPT, VR/CPT, volume de gás intratorácico e specific airway resistance (sRaw, resistência específica das vias ...

  10. Test de marcha de seis minutos en niños con bronquiolitis obliterante postviral: Correlación con espirometría Six minute walk test in children with post-infectious obliterans bronchiolitis: Its relation with spirometry

    OpenAIRE

    DANIEL ZENTENO A; HOMERO PUPPO G; RAMIRO GONZÁLEZ V; DOLORES PAVÓN T; ROBERTO VERA U; RODRIGO TORRES C; CHUNG KUO K; MARÍA ANGÉLICA PÉREZ H; GUIDO GIRARDI B

    2008-01-01

    la Bronquiolitis Obliterante (BO) postviral produce alteraciones en la función pulmonar que pueden repercutir en la capacidad para realizar ejercicio, la cual se puede evaluar con el test de marcha de 6 minutos (TM6). Objetivo: Estudiar con esta prueba a niños con BO postviral y determinar la relación entre variables espirométricas y los resultados del TM6. Veintisiete pacientes, se estudiaron con espirometría basal y TM6. Se relacionaron variables espirométricas con resultados del TM6: dista...

  11. Función pulmonar y calidad de vida en niños y adolescentes con bronquiolitis obliterante por adenovirus Pulmonary function and quality of life in children and adolescents with bronchiolitis obliterans post-adenoviral infection

    OpenAIRE

    Stefhanie Nayar E; Mónica Saavedra B; Ana María Escobar C; Alberto Vidal G

    2011-01-01

    Introducción: La infección por adenovirus es una causa importante de neumonía en niños chilenos. La bronquiolitis obliterante (BOPI) es la complicación más importante. Existen pocos estudios donde evalúen la función pulmonar y la calidad de vida de los pacientes con BOPI. Objetivo: Evaluar la función pulmonar y la calidad de vida de los pacientes con BOPI y la correlación entre ambas variables. Métodos: Se incluyeron 14 niños con BOPI en elpoliclínico de la unidad de broncopulmonar de un hosp...

  12. [Bronchiolitis among infants under 1 year of age in France: epidemiology and factors associated with mortality].

    Science.gov (United States)

    Che, D; Nicolau, J; Bergounioux, J; Perez, T; Bitar, D

    2012-07-01

    Little information is available on the characteristics of infants hospitalized for acute bronchiolitis in France. An analysis of hospital records (PMSI) was conducted at the national level to describe the cases of bronchiolitis that require hospitalization among infants under 1 year of age and the factors associated with death. The analysis of all admissions that occurred during 2009, for which the diagnosis of acute bronchiolitis was recorded in the PMSI database for infants aged less than 1 year, was performed. Cases were described according to age, sex, underlying conditions (including bronchopulmonary dysplasia, cystic fibrosis, and congenital heart disease), length of hospital stay, recurrent admissions, admission to an intensive care unit (ICU), and use of assisted ventilation. Factors associated with death during hospitalization were studied by logistic regression. The hospitalization rate was 35.8 per 1000 infants under 1 year in 2009 in France. Approximately 10% of hospitalized infants required ICU admission. Twenty-two infants died. The estimated case-fatality rate was 0.08% among hospitalized infants and 0.56% for those hospitalized in the ICU. Mortality among all infants under 1 year was 2.6/10(5) in France. Factors associated with death were bronchopulmonary dysplasia (OR=6.7, 95% CI [1.5-29.8]), hospitalization in an ICU (OR=6.46, 95% CI [2.4-17.4]), and the use of assisted ventilation (OR=6.2, 95% CI [2.2-17.1]). This study has enabled the quantification of the rate of hospitalization and mortality, and a better description of infants who need hospitalization. The results are consistent with international literature, but further prospective analysis will be needed to better describe the cases at higher risk, aiming to improve their management. PMID:22652519

  13. Physical therapy for airway clearance improves cardiac autonomic modulation in children with acute bronchiolitis

    Directory of Open Access Journals (Sweden)

    Cynthia P. Jacinto

    2013-12-01

    Full Text Available BACKGROUND: The effects of physical therapy on heart rate variability (HRV, especially in children, are still inconclusive. OBJECTIVE: We investigated the effects of conventional physical therapy (CPT for airway clearance and nasotracheal suction on the HRV of pediatric patients with acute bronchiolitis. METHOD: 24 children were divided into two groups: control group (CG, n=12 without respiratory diseases and acute bronchiolitis group (BG, n=12. The heart rate was recorded in the BG at four different moments: basal recording (30 minutes, 5 minutes after the CPT (10 minutes, 5 minutes after nasotracheal suction (10 minutes, and 40 minutes after nasotracheal suction (30 minutes. The CG was subjected to the same protocol, except for nasotracheal suction. To assess the HRV, we used spectrum analysis, which decomposes the heart rate oscillations into frequency bands: low frequency (LF=0.04-0.15Hz, which corresponds mainly to sympathetic modulation; and high frequency (HF=0.15-1.2Hz, corresponding to vagal modulation. RESULTS: Under baseline conditions, the BG showed higher values in LF oscillations, lower values in HF oscillations, and increased LF/HF ratio when compared to the CG. After CPT, the values for HRV in the BG were similar to those observed in the CG during basal recording. Five minutes after nasotracheal suction, the BG showed a decrease in LF and HF oscillations; however, after 40 minutes, the values were similar to those observed after application of CPT. CONCLUSIONS: The CPT and nasotracheal suction, both used for airway clearance, promote improvement in autonomic modulation of HRV in children with acute bronchiolitis.

  14. Lymphocytic Bronchiolitis as Presenting Disorder in an Undiagnosed Adult Patient with Chronic Granulomatous Disease

    Directory of Open Access Journals (Sweden)

    Payam Tabarsi

    2007-12-01

    Full Text Available Chronic Granulomatous Disease (CGD is a rare primary immunodeficiency disease. Although the most affected patients are diagnosed in childhood, there are several reports of the disease presenting in adult patients. Here we present a 40 years old man who was admitted in hospital due to respiratory symptoms and ground glass pattern in high resolution computed tomography of lung. Open lung biopsy revealed lymphocytic bronchiolitis. Because of past medical history of granulomatous lesion in lung and recurrent abscesses of skin and soft tissue, NBT test was conducted which its result revealed that the disorder was compatible with CGD and then it was confirmed by fluorescent cytometry.

  15. Validation of a pediatric caregiver diary to measure symptoms of postacute respiratory syncytial virus bronchiolitis

    DEFF Research Database (Denmark)

    Santanello, Nancy C; Norquist, Josephine M; Nelsen, Linda M;

    2005-01-01

    responsiveness of the bronchiolitis caregiver diary (BCD) of symptoms and healthcare utilization associated with postacute RSV. The BCD measures four symptoms (daytime cough, wheeze, trouble breathing, and nighttime cough), healthcare utilization, and rescue medication for worsening of lung symptoms. Data from......-groups analyses), and responsiveness. The primary outcome of this study was the percentage of symptom-free days (SFD). The secondary outcome was a composite symptom score (CSS; average of daytime cough, wheezing, and trouble breathing). Cronbach's alpha of 0.85 indicated that the four symptoms were internally...

  16. Follicular Bronchiolitis in a Nigerian Female Child: A Case Report and Review of the Literature

    Science.gov (United States)

    Umerah, Godwin O.; Ugwu, Christopher Emeka; Olusoji, Olugbenga; Adeyomoye, Adekunle

    2016-01-01

    Small airways diseases are not uncommon in childhood. They account for about 28.4% of hospital admissions for lower respiratory tract infections in South West Nigeria, most of which are due to respiratory syncytial virus (RSV) infection. Noninfectious causes of small airways diseases, on the other hand, are poorly recognized and rarely feature in the differential diagnoses of chronic/recurrent lower respiratory tract disease in our environment. We present a case of follicular bronchiolitis in a 2.5-year-old Nigerian female who had left upper lobectomy on account of recurrent cough and progressive shortness of breath. PMID:27313935

  17. Clinical study of the relationship between arteriosclerosis obliterans (ASO) and cerebral infarction

    International Nuclear Information System (INIS)

    To clarify the relationship between arteriosclerosis obliterans (ASO) and cerebral infarction (CI), brain CT was performed and the risk factors for atherosclerosis were assessed. Thirty-five male and 5 female patients with intermittent claudication and/or leg ulceration were angiographically diagnosed as having ASO. According to CT findings, these patients were divided into three groups [no low-density areas (NLDA), hemorrhage, and infarction (CI)]. CI was subdivided as lacunar, cortical, and watershed infarction. Thirteen patients were in the NLDA group and 26 in the CI group (17 lacunar, 3 cortical and 6 mixed infarcts), indicating a CI incidence of 65%. Comparing the risk factors of the CI group with those of the NLDA group, hypertension (53.8%), diabetes (34.6%), and cigarette smoking (69.2%) was often seen in the CI group, although hypercholesterolemia (53.8%) and ischemic heart disease (42.3%) was the same in both groups. Multivariate analysis revealed that smoking had the strongest effect on the occurrence of CI in ASO patients. Furthermore, the number of combined risk factors (hypertension, diabetes, smoking, hypercholesterolemia) had a significant positive correlation with cortical infarction. As to the chronological relationship between the onset of ASO and CI, CI was present in 14 of 27 ASO patients on CT when the ischemic leg symptoms appeared, while symptomatic cortical infarction preceded ASO in 5 patients. CI patients increased gradually over a decade to 26 out of 40, among whom 16 patients with lacunae had silent infarcts. These findings suggested that ASO is frequently associated with CI, not only due to atherosclerosis of the main trunks of the cerebral vessels, but also due to arteriolosclerosis of the perforating arteries. (author)

  18. Activation of monocytes and cytokine production in patients with peripheral atherosclerosis obliterans

    Directory of Open Access Journals (Sweden)

    Lastória Sidney

    2011-08-01

    Full Text Available Abstract Background Arterial peripheral disease is a condition caused by the blocked blood flow resulting from arterial cholesterol deposits within the arms, legs and aorta. Studies have shown that macrophages in atherosclerotic plaque are highly activated, which makes these cells important antigen-presenting cells that develop a specific immune response, in which LDLox is the inducing antigen. As functional changes of cells which participate in the atherogenesis process may occur in the peripheral blood, the objectives of the present study were to evaluate plasma levels of anti-inflammatory and inflammatory cytokines including TNF-α, IFN-γ, interleukin-6 (IL-6, IL-10 and TGF-β in patients with peripheral arteriosclerosis obliterans, to assess the monocyte activation level in peripheral blood through the ability of these cells to release hydrogen peroxide (H2O2 and to develop fungicidal activity against Candida albicans (C. albicans in vitro. Methods TNF-α, IFN-γ, IL-6, IL-10 and TGF-β from plasma of patients were detected by ELISA. Monocyte cultures activated in vitro with TNF-alpha and IFN-gamma were evaluated by fungicidal activity against C. albicans by culture plating and Colony Forming Unit (CFU recovery, and by H2O2 production. Results Plasma levels of all cytokines were significantly higher in patients compared to those detected in control subjects. Control group monocytes did not release substantial levels of H2O2 in vitro, but these levels were significantly increased after activation with IFN-γ and TNF-α. Monocytes of patients, before and after activation, responded less than those of control subjects. Similar results were found when fungicidal activity was evaluated. The results seen in patients were always significantly smaller than among control subjects. Conclusions: The results revealed an unresponsiveness of patient monocytes in vitro probably due to the high activation process occurring in vivo as corroborated by high

  19. Skin phototype and local trauma in the onset of balanitis xerotica obliterans (BXO) in circumcised patients.

    Science.gov (United States)

    Villa, Massimo; Dragonetti, Emanuele; Grande, Michele; Bove, Pierluigi; Sansalone, Salvatore; Rulli, Francesco; Tambucci, Roberto; Tucci, Gianfranco; Baldi, Alfonso

    2012-01-01

    The association between balanitis xerotica obliterans (BXO) and skin disorders is long established, however, the role of skin phototype and local trauma in its onset has never been investigated in detail. Medical records of all Caucasian children circumcised over a 6-year period were reviewed. The excised skin underwent histological examination for BXO. Children with histological diagnosis of BXO were classified as group A, whereas children without histological diagnosis of BXO were classified as group B. The Fitzpatrick phototype (FT) was obtained in all children performing a personal or family interview with regards to their sunburn and suntan experience. According to their FT, both group A and B patients were divided into two subgroups: FT 1-2, with a higher tendency to sunburn due to their low skin melanin content; and FT 3-4 with a higher tendency to tan due to their higher skin melanin content. Maneuvers of mechanical reduction of the foreskin (MRF) performed at least 5-10 times per month during the year preceding circumcision was also considered. Statistical analysis was performed using univariate and multivariate analysis. A total of 297 patients met the inclusion criteria of our study: 78 patients were classified as group A and 219 as group B. The risk of developing BXO was significantly greater in FT 1-2 patients (n=76) (odd ratio=0.232, 95% confidence interval=0.124-0.435, p<0.0001). Furthermore, those undergoing MRF (n=131) had a significantly higher risk of developing BXO (odds ratio= 5.344, 95% confidence interval=2.860-9.987, p<0.0001). Although the foreskin is not directly exposed to sunlight, this study emphasizes the role of skin phototype on the onset of BXO in circumcised individuals. Moreover, the data produced suggest should the advantages of repeated MRF be weighed against the increased risk of developing BXO, which in turn may increase complication rate of circumcision surgery. PMID:22210729

  20. Antibody-Mediated Lung Transplant Rejection

    OpenAIRE

    Hachem, Ramsey

    2012-01-01

    Antibody-mediated rejection after lung transplantation remains enigmatic. However, emerging evidence over the past several years suggests that humoral immunity plays an important role in allograft rejection. Indeed, the development of donor-specific antibodies after transplantation has been identified as an independent risk factor for acute cellular rejection and bronchiolitis obliterans syndrome. Furthermore, cases of acute antibody-mediated rejection resulting in severe allograft dysfunctio...

  1. Changes in the Lung Microbiome following Lung Transplantation Include the Emergence of Two Distinct Pseudomonas Species with Distinct Clinical Associations

    OpenAIRE

    Dickson, Robert P; Erb-Downward, John R.; Freeman, Christine M.; Walker, Natalie; Scales, Brittan S.; Beck, James M.; Martinez, Fernando J; Curtis, Jeffrey L.; Lama, Vibha N.; Huffnagle, Gary B.

    2014-01-01

    Background Multiple independent culture-based studies have identified the presence of Pseudomonas aeruginosa in respiratory samples as a positive risk factor for bronchiolitis obliterans syndrome (BOS). Yet, culture-independent microbiological techniques have identified a negative association between Pseudomonas species and BOS. Our objective was to investigate whether there may be a unifying explanation for these apparently dichotomous results. Methods We performed bronchoscopies with bronch...

  2. Noninvasive Investigations for the Early Detection of Chronic Airways Dysfunction Following Lung Transplantation

    OpenAIRE

    Cook, Richard C.; Guy Fradet; Nestor L Muller; Daniel F Worsley; David Ostrow; Levy, Robert D

    2003-01-01

    BACKGROUND: The diagnosis of chronic rejection after lung transplantation is limited by the lack of a reliable test to detect airways disease early.OBJECTIVES: To determine whether maximum midexpiratory flow (MMEF), or changes on high resolution computed tomography (HRCT) or ventilation/perfusion lung (V/Q) scans are sensitive and specific for early detection of bronchiolitis obliterans syndrome (BOS; forced expiratory volume in 1 s [FEV1] less than 80% post-transplant baseline) by evaluating...

  3. Mechanisms of relaxations of bovine isolated bronchioles by the nitric oxide donor, GEA 3175

    Science.gov (United States)

    Hernández, Medardo; Elmedal, Britt; Mulvany, Michael J; Simonsen, Ulf

    1998-01-01

    The present study was designed to investigate the effects and mechanisms of relaxation induced by the nitric oxide (NO) donor, GEA 3175 (a 3-aryl-substituted oxatriazole derivative) on bovine bronchioles (effective lumen diameter 200–800 μm) suspended in microvascular myographs for isometric tension recording. In segments of bovine bronchioles contracted to 5-hydroxytryptamine, GEA 3175 (10−8–10−4 M) induced concentration-dependent reproducible relaxations. These relaxations were slow in onset compared to other NO-donors such as 3-morpholinosydonimine-hydrochloride (SIN-1) and S-nitroso-N-acetylpenicillamine (SNAP). In 5-hydroxytryptamine-contracted preparations the order of relaxant potency (pD2) was: salbutamol (7.80)>GEA 3175 (6.18)>SIN-1 (4.90)>SNAP (3.55). In segments contracted to acetylcholine, the relaxant responses were reduced and GEA 3175 relaxed the bronchioles with pD2=4.41±0.12 and relaxations of 66±10% (n=4), while SNAP and salbutamol caused relaxations of 19±6% (n=4) and 27±6% (n=8) at the highest concentration used, respectively. Oxyhaemoglobin (10−5 M), the scavenger of nitric oxide, caused rightward shifts of the concentration-relaxation curves to GEA 3175 and NO. 1H-[1,2,4]oxadiazolo[4,3,-a]quinoxalin-1-one (ODQ, 3×10−6 M) and LY 83583 (10−6 M), the inhibitors of soluble guanylate cyclase, also reduced the relaxations induced by GEA 3175 and nitric oxide. However, ODQ did not affect salbutamol-evoked relaxation in the bovine small bronchioles. GEA 3175-induced relaxations were reduced in potassium-rich (60 mmol l−1 K+) solution. Glibenclamide (10−6 M) markedly inhibited the relaxations induced by the opener of ATP-sensitive K+ channels, levcromakalim (3×10−8–10−5 M), but it did not modify the relaxations induced by GEA 3175 or salbutamol. Apamin (5×10−7 M), a blocker of the small Ca2+-activated K+-channels did not affect the relaxations to GEA 3175. In contrast, blockers of large Ca2

  4. The use of C-reactive protein in predicting bacterial co-Infection in children with bronchiolitis

    Directory of Open Access Journals (Sweden)

    Mohamad Fares

    2011-03-01

    Full Text Available Background: Bronchiolitis is a potentially life-threatening respiratory illness commonly affecting children who are less than two years of age. Patients with viral lower respiratory tract infection are at risk for co-bacterial infection. Aim: The aim of our study was to evaluate the use of C-reactive protein (CRP in predicting bacterial co-infection in patients hospitalized for bronchiolitis and to correlate the results with the use of antibiotics. Patients and Methods: This is a prospective study that included patients diagnosed with bronchiolitis admitted to Makassed General Hospital in Beirut from October 2008 to April 2009. A tracheal aspirate culture was taken from all patients with bronchiolitis on admission to the hospital. Blood was drawn to test C-reactive protein level, white cell count, transaminases level, and blood sugar level. Results: Forty-nine patients were enrolled in the study and were divided into two groups. Group 1 included patients with positive tracheal aspirate culture and Group 2 included those with negative culture. All patients with a CRP level ≥2 mg/dL have had bacterial co-infection. White cell count, transaminases and blood sugar levels were not predictive for bacterial co-infection. The presence of bacterial co-infection increased the length of hospital stay in the first group by 2 days compared to those in the second group. Conclusion: Bacterial co-infection is frequent in infants with moderate to severe bronchiolitis and requires admission. Our data showed that a CRP level greater than 1.1 mg/dL raised suspicion for bacterial co-infection. Thus, a tracheal aspirate should be investigated microbiologically in all hospitalized patients in order to avoid unnecessary antimicrobial therapy and to shorten the duration of the hospital stay.

  5. Effectiveness of chest physiotherapy in infants hospitalized with acute bronchiolitis: a multicenter, randomized, controlled trial.

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    Vincent Gajdos

    2010-09-01

    Full Text Available Acute bronchiolitis treatment in children and infants is largely supportive, but chest physiotherapy is routinely performed in some countries. In France, national guidelines recommend a specific type of physiotherapy combining the increased exhalation technique (IET and assisted cough (AC. Our objective was to evaluate the efficacy of chest physiotherapy (IET + AC in previously healthy infants hospitalized for a first episode of acute bronchiolitis.We conducted a multicenter, randomized, outcome assessor-blind and parent-blind trial in seven French pediatric departments. We recruited 496 infants hospitalized for first-episode acute bronchiolitis between October 2004 and January 2008. Patients were randomly allocated to receive from physiotherapists three times a day, either IET + AC (intervention group, n=246 or nasal suction (NS, control group, n=250. Only physiotherapists were aware of the allocation group of the infant. The primary outcome was time to recovery, defined as 8 hours without oxygen supplementation associated with minimal or no chest recession, and ingesting more than two-thirds of daily food requirements. Secondary outcomes were intensive care unit admissions, artificial ventilation, antibiotic treatment, description of side effects during procedures, and parental perception of comfort. Statistical analysis was performed on an intent-to-treat basis. Median time to recovery was 2.31 days, (95% confidence interval [CI] 1.97-2.73 for the control group and 2.02 days (95% CI 1.96-2.34 for the intervention group, indicating no significant effect of physiotherapy (hazard ratio [HR]=1.09, 95% CI 0.91-1.31, p=0.33. No treatment by age interaction was found (p=0.97. Frequency of vomiting and transient respiratory destabilization was higher in the IET + AC group during the procedure (relative risk [RR]=10.2, 95% CI 1.3-78.8, p=0.005 and RR=5.4, 95% CI 1.6-18.4, p=0.002, respectively. No difference between groups in bradycardia with or

  6. The Therapeutic Effect of Gamma Interferon in Chronic Bronchiolitis Due to Mustard Gas

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    Yunes Panahi

    2005-06-01

    In the two groups, FEV1 did not have statistical differences at base line (49.3±2.9 and 48.7±4.1, respectively, p = 0.6, whereas after treatment the data for FEV1 showed a significant increase in the case group (66.3±5.4 when compared with those in the control group (57.3±8.6, p=0.001. The findings of this study indicate that a 6-month treatment with interferon gamma -1b plus a low-dose of prednisolone is associated with the improvement of the lung function in mustard gas exposed patients with bronchiolitis

  7. Complicated bronchiolitis: another use for your bedside ultrasound in the emergency department.

    Science.gov (United States)

    Soto, Fernando; Soltero, Roxana; Rovira, Helen

    2009-01-01

    Diaphragmatic eventration or diaphragmatic herniations are congenital defect that involve abnormal development of the diaphragm and stretching of the muscular fibers leading to protrusion of abdominal organs into the thoracic cavity. Left sided defects will lead to stomach contents into the chest cavity while, right sided defects will have bowel or liver in the thoracic cavity. Infants with Congenital diaphragmatic hernia often present with respiratory distress that can be life-threatening unless treated appropriately. Bedside ultrasound in the Emergency Department, performed by the Emergency Physician can be a very useful tool in diagnosing conditions such as this one. We present a case of diaphragmatic eventration in a two month old male sent to Emergency Department (ED) by his primary care physician due to a severe case of bronchiolitis. PMID:20120987

  8. Treatment of urethral strictures in balanitis xerotica obliterans (BXO using circular buccal mucosal meatoplasy: Experience of 15 cases

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    Abdulmuttalip Simsek

    2014-03-01

    Full Text Available Objectives: Balanitis xerotica obliterans (BXO related strictures involving the external urethral meatus. We reviewed our result with the use of circular mucosal graft in the reconstruction of strictures. Methods: Between March 1997 and January 2012, 15 patients underwent circular buccal mucosal urethroplasy for BXO related anterior urethral strictures. Urethral catheter was removed within 2 weeks. Follow-up included patient symptoms assessment, cosmetic outcome and uroflowmetry. Results: Median follow-up was 20.5 months (range 4 to 96. Mean postoperative peak urinary flow rate obtained 1 month after catheter removal was 22.4 ml per second. All patients had a normal meatus and none had recurrent stricture, chordee or erectile dysfunction. A functional and cosmetic outcome was achieved in 100% of the patients. Conclusions: Circular mucosal graft technique for treatment of meatal strictures is an efficient method for the restoration of a functional and cosmetic penis.

  9. Impact of hospitalizations for bronchiolitis in preterm infants on long-term health care costs in Italy: a retrospective case-control study

    OpenAIRE

    Roggeri, Alessandro; Roggeri,Daniela Paola; Rossi, Elisa; Cataudella,Salvatore; Martini, Nello

    2016-01-01

    Daniela Paola Roggeri,1 Alessandro Roggeri,1 Elisa Rossi,2 Salvatore Cataudella,2 Nello Martini,3 1ProCure Solutions, Nembro, Bergamo, 2CINECA Interuniversity Consortium, Bologna, 3Accademia Nazionale di Medicina, Rome, Italy Purpose: Bronchiolitis is an acute inflammatory injury of the bronchioles, and is the most frequent cause of hospitalization for lower respiratory tract infections in preterm infants. This was a retrospective, observational, case-control study conducted in Italy...

  10. Study of montelukast for the treatment of respiratory symptoms of post-respiratory syncytial virus bronchiolitis in children

    DEFF Research Database (Denmark)

    Bisgaard, H.; Flores-Nunez, A.; Goh, A.; Azimi, P.; Halkas, A.; Malice, M.P.; Marchal, J.L.; Dass, S.B.; Reiss, T.F.; Knorr, B.A.

    2008-01-01

    RATIONALE: A pilot study (Bisgaard H; Study Group on Montelukast and Respiratory Syncytial Virus. A randomized trial of montelukast in respiratory syncytial virus postbronchiolitis. Am J Respir Crit Care Med 2003;167:379-383) reported the efficacy of montelukast in post-respiratory syncytial virus...... (RSV) bronchiolitic respiratory symptoms. OBJECTIVES: To evaluate the efficacy and safety of montelukast, 4 and 8 mg, in treating recurrent respiratory symptoms of post-RSV bronchiolitis in children in a large, multicenter study. METHODS: This was a double-blind study of 3- to 24-month-old children who...... had been hospitalized for a first or second episode of physician-diagnosed RSV bronchiolitis and who tested positive for RSV. Patients (n = 979) were randomized to placebo or to montelukast at 4 or 8 mg/day for 4 weeks (period I) and 20 weeks (period II). The primary end point was percentage symptom...

  11. Histopathologic features in a case of hyperimmunoglobulinemia D syndrome.

    Science.gov (United States)

    Pace, Sarah; Bingham, Jonathan; Royer, Michael

    2015-12-01

    We describe a case of Mevalonate Kinase Deficiency (MKD) also known as Hyperimmunoglobulinemia D Syndrome (HIDS) presenting as a Sweet-like syndrome in a 5-week-old with multiple erythematous plaques, fever, aseptic meningitis, and bronchiolitis. The locations of the predominant plaques were periumbilical and periocular, which originally prompted concern for omphalitis and preseptal cellulitis. Histopathology demonstrated a neutrophilic and histiocytic dermatitis with prominent squamous syringometaplasia and leukocytoclasis in the absence of a vasculitis. This case is reported here due to the unique findings of a prominent histiocytic component in addition to the typically described neutrophilic infiltrate. PMID:26904447

  12. Histopathologic features in a case of hyperimmunoglobulinemia D syndrome

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    Sarah Pace

    2015-01-01

    Full Text Available We describe a case of Mevalonate Kinase Deficiency (MKD also known as Hyperimmunoglobulinemia D Syndrome (HIDS presenting as a Sweet-like syndrome in a 5-week-old with multiple erythematous plaques, fever, aseptic meningitis, and bronchiolitis. The locations of the predominant plaques were periumbilical and periocular, which originally prompted concern for omphalitis and preseptal cellulitis. Histopathology demonstrated a neutrophilic and histiocytic dermatitis with prominent squamous syringometaplasia and leukocytoclasis in the absence of a vasculitis. This case is reported here due to the unique findings of a prominent histiocytic component in addition to the typically described neutrophilic infiltrate.

  13. Prevention of bronchiolitis from the hospital to home: enviromental and pharmacological strategies

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    Andrea Dotta

    2015-10-01

    Full Text Available Bronchiolitis is one of the major cause of mortality and morbidity during infancy, with high hospitalization rate during epidemic season for high risk infants during the first year of life. There is no specific therapy with proven efficacy apart hydration and hypertonic saline. Mild forms can be treated in outpatient setting avoiding a useless hospitalization. Enviromental prevention is crucial in hospital setting, in outpatient ambulatory and at home: for this purpose hand hygiene, tobacco exposure, breastfeeding must be emphasized with families and caregivers. Pharmacological prophylaxis uses a humanized monoclonal antibody (palivizumab shown effective in reducing hospitalization rate in preterm infants < 35 weeks gestational age, infants with bronchopulmonary dysplasia and infants with congenital heart disease. During the last 2 years different recommandations and guidelines confirmed the importance of prophylaxis with palivizumab with some differences between Groups and Societies based on different healthcare systems. In any case it is important a good contact between hospital and all caregivers of these patients to reduce the viral exposure and increase the defense capability of high risk patients. Proceedings of the 11th International Workshop on Neonatology and Satellite Meetings · Cagliari (Italy · October 26th-31st, 2015 · From the womb to the adultGuest Editors: Vassilios Fanos (Cagliari, Italy, Michele Mussap (Genoa, Italy, Antonio Del Vecchio (Bari, Italy, Bo Sun (Shanghai, China, Dorret I. Boomsma (Amsterdam, the Netherlands, Gavino Faa (Cagliari, Italy, Antonio Giordano (Philadelphia, USA

  14. Activation of endothelial and epithelial KCa2.3 calcium-activated potassium channels by NS309 relaxes human small pulmonary arteries and bronchioles

    Science.gov (United States)

    Kroigaard, Christel; Dalsgaard, Thomas; Nielsen, Gorm; Laursen, Britt E; Pilegaard, Hans; Köhler, Ralf; Simonsen, Ulf

    2012-01-01

    BACKGROUND AND PURPOSE Small (KCa2) and intermediate (KCa3.1) conductance calcium-activated potassium channels (KCa) may contribute to both epithelium- and endothelium-dependent relaxations, but this has not been established in human pulmonary arteries and bronchioles. Therefore, we investigated the expression of KCa2.3 and KCa3.1 channels, and hypothesized that activation of these channels would produce relaxation of human bronchioles and pulmonary arteries. EXPERIMENTAL APPROACH Channel expression and functional studies were conducted in human isolated small pulmonary arteries and bronchioles. KCa2 and KCa3.1 currents were examined in human small airways epithelial (HSAEpi) cells by whole-cell patch clamp techniques. RESULTS While KCa2.3 expression was similar, KCa3.1 protein was more highly expressed in pulmonary arteries than bronchioles. Immunoreactive KCa2.3 and KCa3.1 proteins were found in both endothelium and epithelium. KCa currents were present in HSAEpi cells and sensitive to the KCa2.3 blocker UCL1684 and the KCa3.1 blocker TRAM-34. In pulmonary arteries contracted by U46619 and in bronchioles contracted by histamine, the KCa2.3/ KCa3.1 activator, NS309, induced concentration-dependent relaxations. NS309 was equally potent in relaxing pulmonary arteries, but less potent in bronchioles, than salbutamol. NS309 relaxations were blocked by the KCa2 channel blocker apamin, while the KCa3.1 channel blocker, charybdotoxin failed to reduce relaxation to NS309 (0.01–1 µM). CONCLUSIONS AND IMPLICATIONS KCa2.3 and KCa3.1 channels are expressed in the endothelium of human pulmonary arteries and epithelium of bronchioles. KCa2.3 channels contributed to endo- and epithelium-dependent relaxations suggesting that these channels are potential targets for treatment of pulmonary hypertension and chronic obstructive pulmonary disease. PMID:22506557

  15. Noninvasive Transcutaneous Monitoring in Long-Term Follow-Up of Patients With Thromboangiitis Obliterans Treated With Intravenous Iloprost.

    Science.gov (United States)

    Melillo, Elio; Grigoratos, Chrysanthos; Sanctis, Francesco De; Spontoni, Paolo; Nuti, Marco; Dell'Omodarme, Matteo; Ferrari, Mauro; Balbarini, Alberto

    2015-07-01

    We evaluated the effectiveness of intravenous iloprost (IVI) in outpatients with thromboangiitis obliterans (TAO) and lower limb noninvasive transcutaneous monitoring (TCM) at follow-up (FU). Ten consecutive patients with TAO underwent IVI therapy. Transcutaneous oxygen (TcPo 2) and carbon dioxide (TcPco 2) determination and laser Doppler flowmetry (LDF) were performed before and after IVI at 3, 6, and 12 months of FU. Clinical response was positive in 7 patients, whereas 3 nonresponders underwent a second IVI cycle with 1 showing a late positive clinical response. After 12 months of FU, all patients were alive without amputations. Supine and dependent TcP2 levels significantly improved (P < .005). Hallux LDF values showed significant change with the maximal hyperemic test at 44°C (P < .005). Forefoot maximal hyperemic test at 44°C LDF (P < .005) and improved venous arterial reflex (P < .05) showed statistically significant time evolution. We demonstrated some degree of IVI effectiveness and evaluated TCM in patients with TAO. PMID:25005765

  16. Incidence and risk factors of hospitalization for bronchiolitis in preterm children: a retrospective longitudinal study in Italy

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    Mucchino Eleonora

    2009-09-01

    Full Text Available Abstract Background Bronchiolitis is a distressing, potentially life-threatening respiratory condition that affects infants. We evaluated the incidence and risk factors of hospitalization for broncholitis in preterm infants (i.e., a gestational age of Methods Retrospective cohort study that linked data from four health administrative databases in the Lazio region (a region of central Italy: the birth register, the hospital discharge register, and two ad-hoc databases that record the doses of Palivizumab administered at two local health units. Results Among 2407 preterm infants, 137 had at least one hospitalization for bronchiolitis in the first 18 months of life, an overall incidence rate of 4.70 per 100 person-years (95%CI: 3.98-5.56; similar incidence rates were observed by calendar year. A multiple Poisson model showed that the following characteristics were associated with higher incidence: younger age of the infant, the period between October-April, male gender, low Apgar score at birth, low birth weight, and low maternal educational level. At least one dose of Palivizumab was administered to 324 (13.5% children; a dramatic increase from 2000 (2.8% to 2006 (19.1% (p Conclusion In Italy the incidence of hospitalization for bronchiolitis, and its associated risk factors, are similar to that found in other countries. Although Palivizumab use is associated with the most important characteristics of severe prematurity, other aspects of its non-use in children with congenital heart disease, the age and the birth country of the mother suggest the need for public health measures that can reduce these health disparities. Finally, the estimated effectiveness of Palivizumab in routine practice, although not significant, confirms the results of previous clinical trials, but its impact on modifying the temporal trend in this population is still negligible.

  17. Hospitalisations for respiratory syncytial virus bronchiolitis in Akershus, Norway, 1993–2000: a population-based retrospective study

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    Bratlid Dag

    2004-12-01

    Full Text Available Abstract Background RSV is recognized as the most important cause of serious lower respiratory tract illness in infants and young children worldwide leading to hospitalisation in a great number of cases, especially in certain high-risk groups. The aims of the present study were to identify risk groups, outcome and incidences of hospitalisation for RSV bronchiolitis in Norwegian children under two years of age and to compare the results with other studies. Methods We performed a population-based retrospective survey for the period 1993–2000 in children under two years of age hospitalised for RSV bronchiolitis. Results 822 admissions from 764 patients were identified, 93% had one hospitalisation, while 7% had two or more hospitalisations. Mean annual hospitalisation incidences were 21.7 per 1.000 children under one year of age, 6.8 per 1.000 children at 1–2 years of age and 14.1 per 1.000 children under two years of age. 77 children (85 admissions belonged to one or more high-risk groups such as preterm birth, trisomy 21 and congenital heart disease. For preterm children under one year of age, at 1–2 years of age and under two years of age hospitalisation incidences per 1.000 children were 23.5, 8.7 and 16.2 respectively. The incidence for children under two years of age with trisomy 21 was 153.8 per 1.000 children. Conclusion While the overall hospitalisation incidences and outcome of RSV bronchiolitis were in agreement with other studies, hospitalisation incidences for preterm children were lower than in many other studies. Age on admission for preterm children, when corrected for prematurity, was comparable to low-risk children. Length of hospitalisation and morbidity was high in both preterm children, children with a congenital heart disease and in children with trisomy 21, the last group being at particular high risk for severe disease.

  18. Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren Syndrome.

    Science.gov (United States)

    Gupta, Nishant; Wikenheiser-Brokamp, Kathryn A; Fischer, Aryeh; McCormack, Francis X

    2016-03-01

    Interstitial lung diseases, especially lymphoproliferative disorders such as follicular bronchiolitis and lymphoid interstitial pneumonia, are commonly seen in association with Sjögren syndrome. Although the predominant computed tomographic (CT) findings in patients with lymphoid interstitial pneumonia/follicular bronchiolitis include poorly defined centrilobular nodules and ground-glass attenuation, cystic changes can be seen in approximately two-thirds of these patients. The objective of this study was to define the clinical, radiological, and histopathological features of cyst-predominant lymphoid interstitial pneumonia/follicular bronchiolitis in patients with Sjögren syndrome. We present four patients who were referred to our institution with diffuse cystic changes on chest CT imaging. All four had a presumptive diagnosis of lymphangioleiomyomatosis but were subsequently found to have Sjögren syndrome. The diagnosis was established based on the clinical symptoms of xerostomia and xerophthalmia along with serologic detection of antinuclear antibodies, rheumatoid factor, anti-Sjögren's syndrome-related antigen A (SSA)/Ro antibodies, and anti-Sjögren's syndrome-related antigen B (SSB)/La antibodies. The cystic pattern associated with Sjögren syndrome had a characteristic appearance on chest CT images. Typical features included a wide variation in cyst size, internal structure within cysts, geographic simplification of parenchymal architecture producing a "dissolving lung appearance," perivascular and often basilar-predominant distribution, and frequent association with ground-glass opacities and nodules. In a compatible clinical context, we submit that these findings can be sufficiently distinctive to obviate the need for lung biopsy, even in the absence of confirmatory serological studies or lip biopsy. Clinicians should consider occult Sjögren syndrome in the differential diagnosis of patients presenting with idiopathic diffuse cystic lung disease. PMID

  19. Perioperative changes in coagulative and fibrinolytic function during surgical treatment of abdominal aortic aneurysm and arteriosclerosis obliterans.

    Science.gov (United States)

    Aramoto, H; Shigematsu, H; Muto, T

    1994-12-01

    To determine the factors which influence perioperative coagulative and fibrinolytic function, we studied 41 patients who underwent surgical repair of unruptured abdominal aortic aneurysm (AAA) and 30 patients who underwent arterial reconstruction for arteriosclerosis obliterans (ASO). In patients with AAA, the levels of fibrin/fibrinogen degradation products (FDP) (11.4 +/- 20.1 micrograms/ml), thrombin-antithrombin III complex (TAT) (22.0 +/- 21.8 micrograms/l), plasmin-alpha 2 plasmin inhibitor complex (PIC) (2.6 +/- 2.9 micrograms/ml) and d-dimer of cross-linked fibrin degradation products (D-D) (8.4 +/- 10.8 micrograms/ml) were elevated, particularly when the AAAs had a large mural thrombus surface area or were accompanied by aneurysm of the iliac or femoral artery. In arterial aneurysms, blood coagulability and secondary fibrinolytic activity were believed to be enhanced. In patients with ASO, the level of TAT (17.2 +/- 24.8 micrograms/l) was so elevated that they were considered to show chronic hypercoagulability. Among the ASO patients with aorto-iliac lesions, those with concomitant graft occlusion or anastomotic aneurysm had significantly elevated levels of TAT. Proximal arterial occlusion or accompanying aneurysm in the ASO patients was associated with increased levels of PIC and D-D. Postoperative fluctuations in conventional hematological variables did not differ significantly among the surgical procedures. Conventional markers showed a transient decrease due to consumption during surgery, and a subsequent recovery or an actual increase within several days after surgery.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:7737753

  20. Acute respiratory bronchiolitis: an ultrastructural and autoradiographic study of epithelial cell injury and renewal in Rhesus monkeys exposed to ozone

    International Nuclear Information System (INIS)

    The pathogenesis of acute respiratory bronchiolitis was examined in Rhesus monkeys exposed to 0.8 ppM ozone for 4 to 50 hours. Epithelial injury and renewal were qualitatively and quantitatively characterized by correlated techniques of scanning and transmission electron microscopy as well as by light-microscopic autoradiography following labeling with tritiated thymidine. Extensive degeneration and necrosis of Type 1 epithelial cells occurred on the respiratory bronchiolar wall during the initial 4 to 12 hours of exposure. Increased numbers of labeled epithelial cells were present in this region after 18 hours of exposure, and the highest labeling index (18%) was measured after 50 hours of exposure. Most (67 to 80%) of the labeled cells and all the mitotic epithelial cells (22) observed ultrastructurally were cuboidal bronchiolar epithelial cells. Of the labeled epithelial cells, 20 to 33% were Type 2 epithelial cells. After 50 hours of exposure the respiratory bronchiolar epithelium was hyperplastic. The predominant inflammatory cell in respiratory bronchiolar exudate was the alveolar macrophage. Monkeys that were exposed for 50 hours and allowed to recover in unozonized air for 7 days had incomplete resolution of respiratory bronchiolar epithelial hyperplasia. The results indicate that Type 1 epithelial cells lining respiratory bronchioles are the cell types most sensitive to injury and that both cuboidal bronchiolar epithelial cells and Type 2 epithelial cells function as stem cells in epithelial renewal

  1. Broad respiratory virus detection in infants hospitalized for bronchiolitis by use of a multiplex RT-PCR DNA microarray system.

    Science.gov (United States)

    Huguenin, Antoine; Moutte, Lauryane; Renois, Fanny; Leveque, Nicolas; Talmud, Deborah; Abely, Michel; Nguyen, Yohan; Carrat, Fabrice; Andreoletti, Laurent

    2012-06-01

    Newly available molecular tools allow a sensitive detection of a broad panel of viruses in respiratory tract specimens. In the present study, the application of a multiplex RT-PCR DNA microarray in diagnosis and epidemiological survey of viral infections in infants hospitalized for bronchiolitis was assessed. One hundred and thirty-eight nasopharyngeal aspirates collected from October 2007 to September 2008 were tested by direct immunofluorescence and viral culture, a combination of referenced RT-PCRs and the DNA microarray. One or more viruses were detected in 96, 126 and 126 of the specimens by direct immunofluorescence and viral culture, RT-PCRs and DNA microarray, respectively (70 vs. 91 vs. 91%, P 0.1). In conclusion, the use of this DNA microarray in clinical virology practice allows rapid and accurate identification of common and uncommon viral respiratory pathogens in infants hospitalized for bronchiolitis. It should improve the clinical management, the epidemiological survey, and the prevention of the nosocomial transmission of respiratory viruses in pediatric wards. PMID:22499022

  2. Bronquiolite aguda, uma revisão atualizada Acute bronchiolitis, an updated review

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    Werther Brunow de Carvalho

    2007-04-01

    Full Text Available A bronquiolite aguda (BA é um diagnóstico freqüente de internação hospitalar em pediatria, ocasionada principalmente pelo vírus sincicial respiratório (VSR. Ocorre epidemicamente nos meses de outono e inverno. Algumas populações de crianças (recém-nascidos pré-termo, cardiopatia congênita, doença pulmonar crônica, imunocomprometidos, desnutridos, entre outros apresentam maior risco de morbidade e mortalidade. Os vírus multiplicam-se nas células epiteliais ciliadas, e a inflamação e os debris celulares ocasionam obstrução da via aérea, hiperinsuflação, atelectasia localizada, chiado e alterações das trocas gasosas. Não existem evidências definitivas em relação aos tratamentos utilizados para esta doença. O tratamento inclui a utilização de oxigênio, hidratação, beta-2 agonistas por via inalatória, epinefrina racêmica, DNase recombinante, fisioterapia respiratória, entre outros. Medidas profiláticas: administração de anticorpos monoclonais (palivizumab. A maioria das crianças com BA, independentemente da gravidade da doença, recuperam-se sem seqüelas. O curso natural desta doença, habitualmente, varia entre sete a dez dias, mas algumas crianças permanecem doentes por semanas.Acute bronchiolitis (AB is a frequent cause of hospitalization among children and its main etiological agent is respiratory syncytial virus (RSV. It occurs epidemically during autumn and winter. Some populations of children such as premature newborns, infants with congenital heart disease and those with chronic lung disease, immunocompromised, undernourished, among others, present increased morbidity and mortality risk. The virus multiplies in epithelial ciliated cells while inflammation and cellular debris cause obstruction of the airways, hyperinflation, atelectasis, and wheezing and gas exchange imbalance. Definitive evidence still does not exist about treatment of this disease, Treatment includes oxygen therapy, hydration

  3. T-2 toxin impairs murine immune response to respiratory reovirus and exacerbates viral bronchiolitis

    International Nuclear Information System (INIS)

    Exposure to immunosuppressive environmental contaminants is a possible contributing factor to increased occurrence of viral respiratory diseases. The objective of this study was to test the hypothesis that the trichothecene mycotoxin T-2 toxin (T-2), a frequent food contaminant, alters host resistance to lung infection by reovirus, a model respiratory virus. Balb/c mice (4 week old) were treated intraperitoneally with T-2 toxin (1.75 mg/kg bw) or saline vehicle and then intranasally instilled 2 h later with 107 plaque forming unit (PFU) of reovirus, strain Lang (T1/L) or saline vehicle. At 10 days post-instillation (PI), both virus plaque-forming responses and reovirus L2 gene expression were 10-fold higher in lungs of T-2-treated mice compared to controls. No-effect and lowest-effect levels for T-2-induced suppression of reovirus clearance were 20 and 200 μg/kg bw, respectively. Respiratory reovirus infection resulted in a mild bronchiolitis with minimal alveolitis, which was markedly exacerbated by T-2 pretreatment. Reovirus exposure induced marked increases in total cells, neutrophils and lymphocytes at 3 and 7 days PI in bronchial alveolar lavage fluid (BALF) whereas macrophages were increased only at 7 days PI. Although prior T-2 exposure attenuated total cell and macrophage counts in BALF of control and infected mice at 3 days PI, the toxin potentiated total cell, macrophage, neutrophil and lymphocyte counts in infected mice at 7 days PI. At 3 days PI, T-2 suppressed reovirus-induced IFN-γ elevation in BALF, but enhanced production of IL-6 and MCP-1. T-2 pretreatment also suppressed reovirus-specific mucosal IgA responses in lung and enteric tract, but potentiated serum IgA and IgG responses. Taken together, T-2 increased lung viral burden, bronchopneumonia and pulmonary cellular infiltration in reovirus-infected mice. These effects might be attributable to reduced alveolar macrophage levels as well as modulated cytokine and mucosal Ig responses

  4. Study of montelukast for the treatment of respiratory symptoms of post-respiratory syncytial virus bronchiolitis in children

    DEFF Research Database (Denmark)

    Bisgaard, Hans; Flores-Nunez, Alejandro; Goh, Anne;

    2008-01-01

    (RSV) bronchiolitic respiratory symptoms. OBJECTIVES: To evaluate the efficacy and safety of montelukast, 4 and 8 mg, in treating recurrent respiratory symptoms of post-RSV bronchiolitis in children in a large, multicenter study. METHODS: This was a double-blind study of 3- to 24-month-old children who......RATIONALE: A pilot study (Bisgaard H; Study Group on Montelukast and Respiratory Syncytial Virus. A randomized trial of montelukast in respiratory syncytial virus postbronchiolitis. Am J Respir Crit Care Med 2003;167:379-383) reported the efficacy of montelukast in post-respiratory syncytial virus...... were generally well tolerated. During the first two treatment weeks, average %SFD was approximately 29%. In post hoc analyses of patients (n = 523) with persistent symptoms (%SFD < or = 30% over Weeks 1-2), differences in %SFD were seen between montelukast and placebo over Weeks 3-24: difference were 5...

  5. Effectiveness of 3% hypertonic saline nebulization in acute bronchiolitis among Indian children: A quasi-experimental study

    Directory of Open Access Journals (Sweden)

    Harsh V Gupta

    2016-01-01

    Full Text Available Objective: To compare the effects of 3% hypertonic saline (HS and 0.9% normal saline with nebulized 0.9% normal saline with salbutamol in patients of acute viral bronchiolitis. Materials and Methods: Participants were divided into three groups, that is, 3% HS group, 0.9% normal saline group and 0.9% saline with salbutamol group. Four doses at interval of 6 h were given daily until discharge. Average CS score and length of hospital stay were compared. One-way analysis of variance paired t-test and Chi-square test were utilized for statistical analysis. Results: The mean ages of the patients in three groups were 6.03 ± 3.71, 5.69 ± 3.34 and 5.48 ± 3.35 respectively. The 3rd day CS scores for all the groups were 1.0 ± 1.1, 1.9 ± 1.1 and 3.3 ± 0.5 respectively (P = 0.000. The average length of hospital stay was 3.4 ± 1.7, 3.7 ± 1.9 and 4.9 ± 1.4 days respectively (P = 0.001. Conclusion: The present study concludes that 3% HS nebulization (without additional bronchodilators is an effective and safe treatment for nonasthmatic, moderately ill patients of acute bronchiolitis. The economic benefit of this comparably priced modality of treatment can be enormous in terms of hospital costs with parents returning to work sooner.

  6. Effectiveness of 3% hypertonic saline nebulization in acute bronchiolitis among Indian children: A quasi-experimental study

    Science.gov (United States)

    Gupta, Harsh V.; Gupta, Vivek V.; Kaur, Gurmeet; Baidwan, Amitoz S.; George, Pardeep P.; Shah, Jay C.; Shinde, Kushal; Malik, Ruku; Chitkara, Neha; Bajaj, Krushnan V.

    2016-01-01

    Objective: To compare the effects of 3% hypertonic saline (HS) and 0.9% normal saline with nebulized 0.9% normal saline with salbutamol in patients of acute viral bronchiolitis. Materials and Methods: Participants were divided into three groups, that is, 3% HS group, 0.9% normal saline group and 0.9% saline with salbutamol group. Four doses at interval of 6 h were given daily until discharge. Average CS score and length of hospital stay were compared. One-way analysis of variance paired t-test and Chi-square test were utilized for statistical analysis. Results: The mean ages of the patients in three groups were 6.03 ± 3.71, 5.69 ± 3.34 and 5.48 ± 3.35 respectively. The 3rd day CS scores for all the groups were 1.0 ± 1.1, 1.9 ± 1.1 and 3.3 ± 0.5 respectively (P = 0.000). The average length of hospital stay was 3.4 ± 1.7, 3.7 ± 1.9 and 4.9 ± 1.4 days respectively (P = 0.001). Conclusion: The present study concludes that 3% HS nebulization (without additional bronchodilators) is an effective and safe treatment for nonasthmatic, moderately ill patients of acute bronchiolitis. The economic benefit of this comparably priced modality of treatment can be enormous in terms of hospital costs with parents returning to work sooner.

  7. Beals Syndrome

    Science.gov (United States)

    ... Boards & Staff Annual Report & Financials Contact Us Donate Marfan & Related Disorders What is Marfan Syndrome? What are ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of ...

  8. Diagnosis of bronchdyskinetic syndrome of patients with dust-induced respiratory system diseases

    Energy Technology Data Exchange (ETDEWEB)

    Levitskaya, V.L.

    1987-12-01

    Bronchospastic syndrome aggravates non-specific, chronic diseases of lungs. Methods of pneumotachography and registration of parameters of flux/content loop enhances diagnosis of ventilatory insufficiency of patients with dust-related diseases. The flux/content loop presents a graphic record of speed of flow of inhaled and exhaled air depending on volume of lungs. Seventy Donbass miners with pneumoconiosis and chronic bronchitis were investigated. Measurements were made of following parameters: speed of inhaled and exhaled air, vital capacity and speed of air at 75%, 50% and 25% of vital capacity. Miners were then treated with salbutamol, an antispasmodic, and same parameters measured. Results are presented in a table showing change in parameters of pneumotachography of exhalation during salbutamol tests. Following salbutamol course, patients with pneumconiosis had a positive reaction in 67% of cases, negative in 24%, none in 9%; those with chronic bronchitis had a positive reaction in 79% and negative in 21% of cases. Respiratory disturbances of lung diseases are due to dyskinesia of bronchioles. Failure to respond to salbutamol, a sympathomimetic, may be due to heightened tone of bronchioles due to parasympathetic innervation requiring a cholinolytic to open bronchiole pathways. Method of pneumotachography with registration of parameter of flow/content loop is combined with pharmacologic tests with antispasmodic preparations to reveal location and mechanism of disturbance of bronchial tone and to determine choice of adequate treatment. 8 refs.

  9. The Role of Human Coronaviruses in Children Hospitalized for Acute Bronchiolitis, Acute Gastroenteritis, and Febrile Seizures: A 2-Year Prospective Study

    OpenAIRE

    Monika Jevšnik; Andrej Steyer; Marko Pokorn; Tatjana Mrvič; Štefan Grosek; Franc Strle; Lara Lusa; Miroslav Petrovec

    2016-01-01

    Human coronaviruses (HCoVs) are associated with a variety of clinical presentations in children, but their role in disease remains uncertain. The objective of our prospective study was to investigate HCoVs associations with various clinical presentations in hospitalized children up to 6 years of age. Children hospitalized with acute bronchiolitis (AB), acute gastroenteritis (AGE), or febrile seizures (FS), and children admitted for elective surgical procedures (healthy controls) were included...

  10. Expiratory CT scan in patients with normal inspiratory CT scan: a finding of obliterative bronchiolitis and other causes of bronchiolar obstruction

    OpenAIRE

    Gaeta, Michele; Minutoli, Fabio; Girbino, Giuseppe; Murabito, Alessandra; Benedetto, Caterina; Contiguglia, Rosario; Ruggeri, Paolo; Privitera, Salvatore

    2013-01-01

    Expiratory CT scan is usually obtained as supplement to normal inspiratory CT scan to recognize air-trapping, which is expression of small airways obstruction. In some patients the air-trapping may be the only sign of an early-stage small airways disease in an otherwise normal lung. The purpose of this article is to illustrate pathologic conditions, namely obliterative bronchiolitis, in which expiratory CT scan can be abnormal despite normal inspiratory CT examination, and to highlight indica...

  11. Study on Effectiveness of Low Dose Theophylline as Add-on to Inhaled Corticosteroid for Patients with Sulfur Mustard Induced Bronchiolitis

    OpenAIRE

    Yunes Panahi; Zohreh Poursaleh; Ali Amini-Harandi; Amin Saburi; Majid Shohrati; Mostafa Ghanei

    2013-01-01

    Background: Theophylline may reverse steroid resistance and decrease inflammation in patients with chronic pulmonary disease and sulfur mustard (SM) induced bronchiolitis. This study was designed to assess the effects of low-dose theophylline on improvement of pulmonary function tests (PFTs) of SM exposed patients.Methods: In this comparative observational study, a group of SM-exposed victims during the Iraq-Iran war who were treated with oral slow releasing (SR) theophylline, salmetrol, flux...

  12. Activation of endothelial and epithelial KCa2.3 calcium-activated potassium channels by NS309 relaxes human small pulmonary arteries and bronchioles

    DEFF Research Database (Denmark)

    Kroigaard, Christel; Dalsgaard, Thomas; Nielsen, Gorm; Laursen, Britt E; Pilegaard, Hans; Köhler, Ralf; Simonsen, Ulf

    2012-01-01

    (Ca) 2.3 and K(Ca) 3.1 proteins were found in both endothelium and epithelium. K(Ca) currents were present in HSAEpi cells and sensitive to the K(Ca) 2.3 blocker UCL1684 and the K(Ca) 3.1 blocker TRAM-34. In pulmonary arteries contracted by U46619 and in bronchioles contracted by histamine, the K(Ca) 2...

  13. Antiphospholipid Syndrome

    Science.gov (United States)

    ... Awards Enhancing Diversity Find People About NINDS NINDS Antiphospholipid Syndrome Information Page Synonym(s): Hughes Syndrome Table of Contents ( ... research is being done? Clinical Trials What is Antiphospholipid Syndrome? Antiphospholipid syndrome (APS) is an autoimmune disorder caused ...

  14. Lung structure-respiratory function relationships in experimentally-induced bronchiolitis, bronchopneumonia and interstitial pneumonia in rats

    International Nuclear Information System (INIS)

    Histopathology and respiratory function of rats with three different types and distributions of lower lung inflammation were compared to better understand lung structure-function relationships. Rats were exposed 21 h/day for 7 days to 0.8 ppm ozone (O3), sham-exposed as controls, or given 5 mg/kg bacterial endotoxin either intratracheally (ITE) or intraperitoneally (IPE). Respiratory function was measured 24 h after the end of treatment, than the rats were sacrificed and the distribution of inflammation was evaluated morphometrically. Chronic centriacinar inflammation with formation of new respiratory bronchioles caused an obstructive functional impairment in the O3 rats, which was clearly distinguished from the restrictive impairments resulting from acute inflammation in ITE and IPE rats. Only the magnitudes of changes related to the distribution of inflammation differentiated the ITE and IPE groups. Flow parameters previously thought sensitive to large airway resistance were changed in the O3 rats. Alveolar luminal inflammatory exudate affected quasistatic compliance more than septal inflammation in ITE and IPE rats. Quasistatic chord compliance was the most sensitive of three indices of pressure-volume relationships. The findings in this study improve the basis for interpreting respiratory function changes of rats. (author)

  15. Chronic rejection of a lung transplant is characterized by a profile of specific autoantibodies

    DEFF Research Database (Denmark)

    Hagedorn, Peter; Burton, Christopher Malcolm; Carlsen, Jørn;

    2010-01-01

    SUMMARY: Obliterative bronchiolitis (OB) continues to be the major limitation to long-term survival after lung transplantation. The specific aetiology and pathogenesis of OB are not well understood. To explore the role of autoreactivity in OB, we spotted 751 different self molecules onto glass...... slides, and used these antigen microarrays to profile 48 human serum samples for immunoglobulin G (IgG) and IgM autoantibodies; 27 patients showed no or mild bronchiolitis obliterans syndrome (BOS; a clinical correlate of OB) and 15 patients showed medium to severe BOS. We now report that these BOS...... independent test patients. Analysis of the functions of the 28 informative self antigens showed that eight of them are connected in an interaction network involved in apoptosis and protein metabolism. Thus, a profile of autoantibodies may reflect pathological processes in the lung allograft, suggesting a role...

  16. Chronic rejection of a lung transplant is characterized by a profile of specific autoantibodies

    DEFF Research Database (Denmark)

    Hagedorn, Peter; Burton, Christopher M.; Carlsen, Jørn;

    2010-01-01

    Obliterative bronchiolitis (OB) continues to be the major limitation to long-term survival after lung transplantation. The specific aetiology and pathogenesis of OB are not well understood. To explore the role of autoreactivity in OB, we spotted 751 different self molecules onto glass slides, and...... used these antigen microarrays to profile 48 human serum samples for immunoglobulin G (IgG) and IgM autoantibodies; 27 patients showed no or mild bronchiolitis obliterans syndrome (BOS; a clinical correlate of OB) and 15 patients showed medium to severe BOS. We now report that these BOS grades could be....... Analysis of the functions of the 28 informative self antigens showed that eight of them are connected in an interaction network involved in apoptosis and protein metabolism. Thus, a profile of autoantibodies may reflect pathological processes in the lung allograft, suggesting a role for autoimmunity in...

  17. A randomized, open-label, multicentre study to evaluate plasma atherosclerotic biomarkers in patients with type 2 diabetes mellitus and arteriosclerosis obliterans when treated with Probucol and Cilostazol

    Institute of Scientific and Technical Information of China (English)

    Xiao-Wei Ma; Xiao-Hui Guo; Xin-Hua Xiao; Li-Xin Guo; Xiao-Feng Lv; Quan-Min Li; Yan Gao

    2012-01-01

    Objectives To evaluate the plasma atherosclerotic biomarkers in patients with type 2 diabetes mellitus (T2DM) and arteriosclerosis obliteran (ASO) when treated with Probucol plus Cilostazol in combination and individually. Methods In this open-label study, patients aged 40-75 years were randomized to receive conventional therapy alone, or with Cilostazol 100 mg bid, or with Probucol 250 mg bid, or with both in combination. Endpoints included changes in plasma biomarker and safety at 12 weeks. Results Of the 200 randomized patients, 165 for per-protocol and 160 for the safety (QTc intervals) were set, respectively. Probucol significantly reduced total cholesterol (P < 0.001), low-density lipoprotein cholesterol (LDL-C), (P = 0.01), and high-density lipoprotein cholesterol (HDL-C) (P < 0.001) compared with conventional therapy. Cilostazol was effective in increasing HDL-C (P = 0.002) and reducing triglycerides levels (P < 0.01) compared with conventional therapy. A trend towards significance was observed for the difference between conventional therapy alone and Probucol plus Cilostazol group for the change in oxidized low-density lipoprotein (Ox-LDL, P = 0.065). No significant effects on the majority of the remaining biomarkers were found across the treatment groups. Conclusions We have confirmed that Ox-LDL could be a possible plasma atherosclerotic biomarker among the evaluated biomarkers, which reflected the synergetic effect of Cilostazol plus Probucol in patients with T2DM and ASO shown previously in preclinical studies.

  18. Usefulness of the {sup 201}TlCl exercise leg perfusion scintigraphy in arteriosclerosis obliterans (ASO). With evaluation of leg perfusion comparing before and after PTA

    Energy Technology Data Exchange (ETDEWEB)

    Kijima, Tetsuji; Kumita, Shin-ichiro; Mizumura, Sunao; Kumazaki, Tatsuo [Nippon Medical School, Tokyo (Japan)

    1995-09-01

    Twenty-eight patients of arteriosclerosis obliterans (ASO) complaining of intermittent claudication or pain at rest underwent symptom limited exercise leg perfusion scintigraphy using {sup 201}TlCl (Tl). Regions of interest (ROI) were drawn around each buttocks, thighs, calves and feet in whole body image, and we calculated Lesion/Normal Index (LNI) which was the devided value of the average count per pixel of each ROI of the affected side by that of the normal side. The average LNI of the foot was 0.81 and was smaller than other regions (p<0.05). Other region except foot showed Tl high uptake in affected side in some cases. Fifteen patients were compared after percutaneous transluminal angioplasty (PTA) with before PTA, and LNI of the foot statistically improved after PTA (p<0.005). The period suffering from disease of the group of Tl hight uptake in the affected leg was statistically shorter than that of the group of Tl non-high uptake (p<0.05). We supposed that the Tl uptake of the foot reflects ischemia of the leg sensitively, and high uptake of Tl in affected leg is concerned with compensatory change of microcirculation of ischemic leg in subacute period. This scintigraphy was thought to be useful to detect the ASO and to evaluate the effect of PTA, and was able to avail diagnosis and observation of the course of ASO patient. (author).

  19. Cerebrovascular diseases in patients with arteriosclerotic aortic aneurysm and arteriosclerosis obliterans studied by carotid ultrasonography and by computed tomography of the brain

    Energy Technology Data Exchange (ETDEWEB)

    Iwamoto, Toshihiko; Sugiyama, Tsuyoshi; Oyama, Tetsuo; Takasaki, Masaru [Tokyo Medical Coll. (Japan)

    1998-09-01

    To estimate the incidence of cerebrovascular diseases in arteriosclerotic aortic aneurysm (AA) and arteriosclerosis obliterans (ASO) and their characteristics, 92 patients with AA and 102 patients with ASO were studied with carotid ultrasonography and brain computed tomography (CT), and were compared with 49 patients with hypertension. The mean ages of the patients were 70 to 72 years old and all were male. Hypertension was common in the AA group; diabetes and cigarette smoking were common in the ASO group. Carotid plaque lesions seen on ultrasonography were significantly more common in the AA (66%) and ASO (85%) groups than in the patients with hypertension (39%). The mean carotid diameter was significantly greater in the AA group than in the other two groups. The mean wall thicknesses in the AA and ASO groups were greater than in the patients with hypertension. Computed tomography showed that low-density areas were also common in the AA group (56%) and ASO (53%) groups than in the patients with hypertension (24%). Most of the low-density areas were thought to be lacunae or dilated perivascular spaces in the subinsula and putamen. Moderately and highly severe periventricular lucencies were also common in the AA group. These findings indicate that carotid changes, seen in AA and ASO, reflected the characteristics of arterial lesions, and that arteriosclerotic lesions were common in patients with AA and ASO. Therefore, cerebrovascular diseases should be taken into account in the management of patients with AA and ASO. (author)

  20. Usefulness of preoperative coronary angiography and brain computed tomography in cases of coronary artery disease and cerebrovascular disease undergoing revascularization for arteriosclerosis obliterans

    International Nuclear Information System (INIS)

    Coronary angiography and brain computed tomography were preoperatively performed to evaluate the clinical condition of coronary artery disease and cerebrovascular disease in 101 patients (mean age, 68.4 years) with revascularization for arteriosclerosis obliterans. Eighty patients had hypertension, 12 had diabetes, and 26 had hyperlipidemia. Seventy-one patients (70.3%) had coronary stenosis. Significant stenoses in major coronary artery branches were confirmed in 35 patients, including 13 patients with old myocardial infarction. Coronary artery bypass grafting and percutaneous coronary angioplasty were performed in 2 and 7 patients with critical stenosis, respectively. Of 57 patients, who underwent brain computed tomography, abnormalities were found in 52 patients (91.2%), including cortical infarction in 9, lacunar infarction in 35, and leukoaraiosis in 27 patients. During the follow-up period 13 patients died (including 3 cases of myocardial infarction and 3 cases of stroke). Actuarial survival rate at 5 years was 80.4%. The influence of ischemic heart disease and cerebrovascular disease on early and late mortality after surgical reconstruction for peripheral occlusive vascular disease is significant. Using visual diagnostic techniques, such as coronary angiography and brain computed tomography, long term survivor should be closely observed for multiple arteriosclerotic vascular diseases. (author)

  1. Usefulness of preoperative coronary angiography and brain computed tomography in cases of coronary artery disease and cerebrovascular disease undergoing revascularization for arteriosclerosis obliterans

    Energy Technology Data Exchange (ETDEWEB)

    Sakurada, Tall; Shibata, Yoshiki [Southern Tohoku Fukushima Hospital (Japan)

    2003-05-01

    Coronary angiography and brain computed tomography were preoperatively performed to evaluate the clinical condition of coronary artery disease and cerebrovascular disease in 101 patients (mean age, 68.4 years) with revascularization for arteriosclerosis obliterans. Eighty patients had hypertension, 12 had diabetes, and 26 had hyperlipidemia. Seventy-one patients (70.3%) had coronary stenosis. Significant stenoses in major coronary artery branches were confirmed in 35 patients, including 13 patients with old myocardial infarction. Coronary artery bypass grafting and percutaneous coronary angioplasty were performed in 2 and 7 patients with critical stenosis, respectively. Of 57 patients, who underwent brain computed tomography, abnormalities were found in 52 patients (91.2%), including cortical infarction in 9, lacunar infarction in 35, and leukoaraiosis in 27 patients. During the follow-up period 13 patients died (including 3 cases of myocardial infarction and 3 cases of stroke). Actuarial survival rate at 5 years was 80.4%. The influence of ischemic heart disease and cerebrovascular disease on early and late mortality after surgical reconstruction for peripheral occlusive vascular disease is significant. Using visual diagnostic techniques, such as coronary angiography and brain computed tomography, long term survivor should be closely observed for multiple arteriosclerotic vascular diseases. (author)

  2. Cerebrovascular diseases in patients with arteriosclerotic aortic aneurysm and arteriosclerosis obliterans studied by carotid ultrasonography and by computed tomography of the brain

    International Nuclear Information System (INIS)

    To estimate the incidence of cerebrovascular diseases in arteriosclerotic aortic aneurysm (AA) and arteriosclerosis obliterans (ASO) and their characteristics, 92 patients with AA and 102 patients with ASO were studied with carotid ultrasonography and brain computed tomography (CT), and were compared with 49 patients with hypertension. The mean ages of the patients were 70 to 72 years old and all were male. Hypertension was common in the AA group; diabetes and cigarette smoking were common in the ASO group. Carotid plaque lesions seen on ultrasonography were significantly more common in the AA (66%) and ASO (85%) groups than in the patients with hypertension (39%). The mean carotid diameter was significantly greater in the AA group than in the other two groups. The mean wall thicknesses in the AA and ASO groups were greater than in the patients with hypertension. Computed tomography showed that low-density areas were also common in the AA group (56%) and ASO (53%) groups than in the patients with hypertension (24%). Most of the low-density areas were thought to be lacunae or dilated perivascular spaces in the subinsula and putamen. Moderately and highly severe periventricular lucencies were also common in the AA group. These findings indicate that carotid changes, seen in AA and ASO, reflected the characteristics of arterial lesions, and that arteriosclerotic lesions were common in patients with AA and ASO. Therefore, cerebrovascular diseases should be taken into account in the management of patients with AA and ASO. (author)

  3. Diagnostic role of magnetic resonance angiography in Swyer James syndrome: Case series of two cases

    Directory of Open Access Journals (Sweden)

    Parashari Umesh

    2010-01-01

    Full Text Available Swyer James syndrome is a rare syndrome which occurs due to viral illness in early childhood. The post infective obliterative bronchiolitis results in arrest of lung growth and alveolarization with reduced vascularity resulting in classical radiological features. We describe two cases of patients fulfilling all the criteria of the syndrome - 1 Unilateral hyperlucent small lung in chest radiograph with air trapping on expiration, small ipsilateral hila and pulmonary artery. 2 Diffuse decrease in attenuation of lung parenchyma with bronchiectasis and reduction in vascularity. 3 Unilateral pruned tree appearance on angiography (MRA. The clinical presentation was recurrent chest infection in a child and infrequent bouts of hemoptysis in a middle aged female. The study demonstrates the role of magnetic resonance angiography in diagnosing the condition.

  4. Guía clínica para el diagnóstico y cuidado de niños/adolescentes con bronquiolitis obliterante post-infecciosa, 2009 Clinical guide for diagnosis and care of children and adolescents with post-infectious bronchiolitis obliterans, 2009

    OpenAIRE

    Luis E Vega-Briceño; DANIEL ZENTENO A

    2009-01-01

    La bronquiolitis obliterante (BO) es un síndrome clínico poco frecuente en niños, caracterizado por la obstrucción crónica al flujo de aire asociado a cambios inflamatorios y distintos grados de fibrosis en la vía aérea pequeña. Si bien existen muchas etiologías, la causa más frecuente se asocia a infecciones respiratorias virales, principalmente adenovirus. No existe un consenso para establecer su diagnóstico; sin embargo, se considera un espectro de síntomas persistentes asociados a un patr...

  5. Parental and household smoking and the increased risk of bronchitis, bronchiolitis and other lower respiratory infections in infancy: systematic review and meta-analysis

    Directory of Open Access Journals (Sweden)

    Britton John

    2011-01-01

    Full Text Available Abstract Background Passive smoke exposure increases the risk of lower respiratory infection (LRI in infants, but the extensive literature on this association has not been systematically reviewed for nearly ten years. The aim of this paper is to provide an updated systematic review and meta-analysis of studies of the association between passive smoking and LRI, and with diagnostic subcategories including bronchiolitis, in infants aged two years and under. Methods We searched MEDLINE and EMBASE (to November 2010, reference lists from publications and abstracts from major conference proceedings to identify all relevant publications. Random effect pooled odds ratios (OR with 95% confidence intervals (CI were estimated. Results We identified 60 studies suitable for inclusion in the meta-analysis. Smoking by either parent or other household members significantly increased the risk of LRI; odds ratios (OR were 1.22 (95% CI 1.10 to 1.35 for paternal smoking, 1.62 (95% CI 1.38 to 1.89 if both parents smoked, and 1.54 (95% CI 1.40 to 1.69 for any household member smoking. Pre-natal maternal smoking (OR 1.24, 95% CI 1.11 to 1.38 had a weaker effect than post-natal smoking (OR 1.58, 95% CI 1.45 to 1.73. The strongest effect was on bronchiolitis, where the risk of any household smoking was increased by an OR of 2.51 (95% CI 1.96 to 3.21. Conclusions Passive smoking in the family home is a major influence on the risk of LRI in infants, and especially on bronchiolitis. Risk is particularly strong in relation to post-natal maternal smoking. Strategies to prevent passive smoke exposure in young children are an urgent public and child health priority.

  6. Paediatric intensive care admissions for respiratory syncytial virus bronchiolitis in France: results of a retrospective survey and evaluation of the validity of a medical information system programme.

    Science.gov (United States)

    Soilly, A-L; Ferdynus, C; Desplanches, O; Grimaldi, M; Gouyon, J B

    2012-04-01

    The purpose of this study was to describe the characteristics of patients with bronchiolitis admitted to a paediatric intensive care unit (PICU), and to evaluate a national registry of hospitalizations (Programme de Médicalisation des Systèmes d'Information; PMSI) as a potential source of epidemiological data. Of the 49 French PICUs invited to take part in a retrospective survey of children aged PMSI data was good only for mechanical ventilation (0·63) and the death rate (0·86). PMID:21733254

  7. Joubert Syndrome

    Science.gov (United States)

    ... Awards Enhancing Diversity Find People About NINDS NINDS Joubert Syndrome Information Page Table of Contents (click to ... Organizations Related NINDS Publications and Information What is Joubert Syndrome? Joubert syndrome is a rare brain malformation ...

  8. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, ...

  9. Rett syndrome: An autoimmune disease?

    Science.gov (United States)

    De Felice, Claudio; Leoncini, Silvia; Signorini, Cinzia; Cortelazzo, Alessio; Rovero, Paolo; Durand, Thierry; Ciccoli, Lucia; Papini, Anna Maria; Hayek, Joussef

    2016-04-01

    Rett syndrome (RTT) is a devastating neurodevelopmental disease, previously included into the autistic spectrum disorders, affecting almost exclusively females (frequency 1:10,000). RTT leads to intellective deficit, purposeful hands use loss and late major motor impairment besides featuring breathing disorders, epilepsy and increased risk of sudden death. The condition is caused in up to 95% of the cases by mutations in the X-linked methyl-CpG binding protein 2 (MECP2) gene. Our group has shown a number of previously unrecognized features, such as systemic redox imbalance, chronic inflammatory status, respiratory bronchiolitis-associated interstitial lung disease-like lung disease, and erythrocyte morphology changes. While evidence on an intimate involvement of MeCP2 in the immune response is cumulating, we have recently shown a cytokine dysregulation in RTT. Increasing evidence on the relationship between MeCP2 and an immune dysfunction is reported, with, apparently, a link between MECP2 gene polymorphisms and autoimmune diseases, including primary Sjögren's syndrome, systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis. Antineuronal (i.e., brain proteins) antibodies have been shown in RTT. Recently, high levels of anti-N-glucosylation (N-Glc) IgM serum autoantibodies [i.e., anti-CSF114(N-Glc) IgMs] have been detected by our group in a statistically significant number of RTT patients. In the current review, the Authors explore the current evidence, either in favor or against, the presence of an autoimmune component in RTT. PMID:26807990

  10. Bronchiole alveolar carcinoma: biological profile, clinicopathologic presentation, evaluation and treatment. Literature review and national series of 26 cases

    International Nuclear Information System (INIS)

    B. Objective: To study the biological profile, clinic pathologic presentation of BAC, characterized by a peculiar evolutionary behavior, study and treatment. P Methodology: Literature review national and international communication based on 26 cases clinical studied and treated in our midst. Results: The BAC behaves as a well-differentiated adenocarcinoma of origin peripheral, typically linked to the Airborne spread to bilateral intrapulmonary cell its three forms: a clear cells and mucous secreting alveolar type II. Present day not considered characteristic of BAC nodal dissemination or hematologic level in the absence of type IV collagenase secretion by tumor cells. It is of no destroying, in one or more cell layers, the bronchiole alveolar septa. Its origin is discussed single or multicenter, while some authors establish etiological factors from benign diseases that can evolve into this neoplasm. It may or may not be linked smoking, presenting clinically in three forms: uni nodular, multi nodular and sclerosing, is studied by CT, FBC generally negative cytological aspiration, puncture or biopsy. It is treated by curative treatment with surgery alone, Rt, Qt and this study the use of tyrosine kinase inhibitors with apparent antitumor activity in BAC Conclusions Some authors present it as a different adenocarcinoma to other CBPNPC, especially in its advanced form multi nodular. Biological tests set for it that his understanding is confirmed. In its evolution can indiferenciarse to infiltrantres forms characteristic of other adenocarcinomas, particularly in the form sclerosing. this fact biological can explain the emergence of mixed tumors with extensive BAC component together another adenocarcinoma. We believe that there is insufficient evidence at present to say that this is a different from the rest of the CBPNCP adenocarcinoma, although studies biological-genetic of this point to gather evidence for this difference. characteristically FBC is usually negative

  11. Kindler syndrome

    Directory of Open Access Journals (Sweden)

    Kaviarasan P

    2005-01-01

    Full Text Available Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma. The differential diagnosis of Kindler syndrome includes diseases like Bloom syndrome, Cockayne syndrome, dyskeratosis congenita, epidermolysis bullosa, Rothmund-Thomson syndrome and xeroderma pigmentosum. Our patient had classical cutaneous features of Kindler syndrome with phimosis as a complication.

  12. Steroids and/or Cytotoxic Agents Should Be Used Early in the Management of Patients with IPF -- The Pro Argument

    OpenAIRE

    Sat Sharma

    2004-01-01

    Idiopathic interstitial pneumonias comprise a heterogenous group of acute and chronic respiratory conditions. Katzenstein and Myers (1) classified these disorders according to several pathologically distinct categories: usual interstitial pneumonitis (UIP), desquamative interstitial pneumonitis ? respiratory bronchiolitis interstitial lung disease, acute interstitial pneumonitis, nonspecific interstitial pneumonitis and cryptogenic organizing pneumonitis or bronchiolitis obliterans organizing...

  13. Bronchiolitis (For Parents)

    Science.gov (United States)

    ... Influenza (Flu) Activity: The Lungs Movie: Lungs & Respiratory System Your Lungs & Respiratory System Flu Chilling Out With Colds Coping With Colds Pneumonia Flu Facts Lungs and Respiratory System Contact Us Print Resources Send to a friend ...

  14. A COMPARATIVE STUDY TO ASSESS THE EFFE CTS OF NEBULISED 3% HYPERTONIC SALINE, 0.9% NORMAL SALINE AND SALBUTALMOL IN MANAGEMENT OF ACUTE BRONCHIOLITIS AMONG INDIAN CHILDREN

    Directory of Open Access Journals (Sweden)

    Gaurav

    2015-03-01

    Full Text Available AIMS AND OBJECTIVES: To compare the effects of nebulised 3% hypertonic saline , 0.9% saline and salbutamol in patients of acute bronchiolitis . DESIGN: R andomised controlled trial . SETTING: tertiary care teaching hospital . MATERIAL AND METHODS: 100 Children with age 1 to 24 months admitted in hospital with clinical diagnosis of acute bronchiolitis for 2 consecutive years were included in the study. Participants were divided into 3 groups – 3% hypertonic saline (HS , 0.9% norm al saline and salbutalmol. 4 doses of nebulisation at an interval of 6 hours were given daily in each group till discharge. RESULTS: The mean age of the patients in the study population was 5.7 ± 3.4 months. Maximum number of the patients i.e. 65.7% belong ed to the age group of 0 - 6 months. There was male preponderance in all 3 groups. Baseline Clinical Severity (CS scores in 3%HS , 0.9% Normal Saline and Salbutamol groups were 5.9±1.5 , 5.5±1.0 and 5.1±2.3 respectively (p=0.146. After treatment , the CS scor es dropped to 1.0±1.1 , 3.3±0.5 and 1.9±1.1 in 3%HS , 0.9% Normal Saline and Salbutamol groups respectively on the 3 rd day of treatment (p<0.01. Length of hospital stay in 3% HS , 0.9% Normal Saline and Salbutamol groups was 3.4±1.7 , 4.9±1.4 and 3.7±1.9 days respectively , which was found to be statistically significant (p= 0.001. CONCLUSION: 3% Hypertonic Saline nebulization (without additional bronchodilators is an effective and safe treatment in patients of acute bronchiolitis. It significantly reduces th e CS scores and length of hospital stay as compared to 0.9% Normal Saline and Salbutamol nebulizations.

  15. Study on Effectiveness of Low Dose Theophylline as Add-on to Inhaled Corticosteroid for Patients with Sulfur Mustard Induced Bronchiolitis

    Directory of Open Access Journals (Sweden)

    Yunes Panahi

    2013-12-01

    Full Text Available Background: Theophylline may reverse steroid resistance and decrease inflammation in patients with chronic pulmonary disease and sulfur mustard (SM induced bronchiolitis. This study was designed to assess the effects of low-dose theophylline on improvement of pulmonary function tests (PFTs of SM exposed patients.Methods: In this comparative observational study, a group of SM-exposed victims during the Iraq-Iran war who were treated with oral slow releasing (SR theophylline, salmetrol, fluxitide, omeprazole and NAC (study group were compared to a group of age and gender matched SM-exposed patients who received same medications except oral SR theophylline (historical control group. PFTs were measured at the beginning of the study and after 8 weeks of the treatment.Results: In total, 33 subjects in the study group and 27 subjects in the control group were studied. Mean (SD age of all subjects was 51 (14.1 years. In the study group, on the 8th week post-treatment, PFTs decreased, though the differences of tests between before and after treatment were not significant. In the control group, all the tests decreased in the same period and these reductions were not also significant. However, the changes in PFTs were significantly different between the two groups. The results of most PFTs in the controls decreased in greater extents compared to theophylline treated patients. This shows that despite theophylline was unable to improve the patients; it was partially able to decelerate the reductions in PFTs.Conclusion: Theophylline may not improve PFTs of SM exposed patients but it may decelerate the progress of the underlying respiratory disease. Further studies in this setting with higher doses of theophylline and longer term of evaluation are needed to better understand the pathophysiological mechanism of SM induced bronchiolitis and the effectiveness of the treatment with theophylline.   How to cite this article: Panahi Y, Poursaleh Z, Amini-Harandi A

  16. Cushing's Syndrome

    Science.gov (United States)

    ... Cushing's syndrome, also called hypercortisolism , is a rare endocrine disorder caused by chronic exposure of the body's tissues ... removing the tumor while minimizing the chance of endocrine deficiency or long-term ... for Cushing's Syndrome Clinical Trials ...

  17. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  18. Metabolic Syndrome

    Science.gov (United States)

    Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These ... doctors agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

  19. Asperger syndrome

    Science.gov (United States)

    Asperger syndrome is often considered a high functioning form of autism. It can lead to difficulty interacting socially, repeat behaviors, and clumsiness. Asperger syndrome is a part of the larger developmental disorder ...

  20. Pseudoaminopterin syndrome.

    Science.gov (United States)

    Kraoua, Lilia; Capri, Yline; Perrin, Laurence; Benmansour, Abdelmajjid; Verloes, Alain

    2012-09-01

    Pseudoaminopterin syndrome or aminopterin syndrome-like sine aminopterin (ASSA syndrome--OMIM 600325] is a rare autosomal recessive syndrome defined by characteristic dysmorphic features, skeletal defects, limb anomalies, cryptorchidism, and growth retardation. The syndrome owes its name to the fact that patients resemble the children exposed to aminopterin or to methotrexate, two dihydrofolate reductase inhibitors used for chemotherapy, or as an abortificient in early pregnancy. Ten patients have been described with pseudoaminopterin syndrome. Their phenotype is variable, and differs from the phenotype resulting from folic acid deprivation, leading to the notion that the pathogenesis may be more complex than simple vitamin deficiency. We report on an Algerian patient with pseudoaminopterin syndrome, review the previously reported cases and confirm that pseudoaminopterin syndrome does not result from a detectable contiguous gene imbalance as high resolution CGH array was normal in this child. PMID:22811276

  1. Usher Syndrome

    Science.gov (United States)

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder ... hearing and vision. There are three types of Usher syndrome: People with type I are deaf from ...

  2. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of ...

  3. Proteus Syndrome

    Science.gov (United States)

    ... Gift Stock Gift Sunshine Society Contact Privacy Policy Proteus Syndrome Definition Common Signs Diagnostic Criteria (I have ... NIH to go with this criteria) Glossary Videos Proteus Syndrome is a condition which involves atypical growth ...

  4. Learning about Marfan Syndrome

    Science.gov (United States)

    ... genetic terms used on this page Learning About Marfan Syndrome What is Marfan syndrome? What are the ... Syndrome Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most ...

  5. Evolução e característica de lactantes com bronquiolite viral aguda submetidos à ventilação mecânica em uma unidade de terapia intensiva pediátrica brasileira Outcome and characteristics of infants with acute viral bronchiolitis submitted to mechanical ventilation in a Brazilian pediatric intensive care

    Directory of Open Access Journals (Sweden)

    Fernanda Umpierre Bueno

    2009-06-01

    bronquiolite viral aguda.OBJECTIVE: To describe the characteristics and the outcome of infants with acute viral bronchiolitis submitted to mechanical ventilation. METHODS: We performed a retrospective study enrolling all infants (less than 12 months old admitted with the diagnosis of acute viral bronchiolitis and submitted to mechanical ventilation in an university affiliated Brazilian pediatric intensive care unit between March, 2004 and September, 2006 (3 consecutives winters. The mechanical ventilation parameters' employed on 1st, 2nd, 3rd, 7th day and before extubation were evaluated as well as the evolution (mortality rate, presence of acute respiratory distress syndrome and the prevalence of complications. The groups were compared using the Student t test, the Mann-Whitney U test and the Chi-square test. RESULTS: Fifty-nine infants were included (3.8 ± 2.7 months old, 59% male, with 9.0 ± 9.4 days on mechanical ventilation. Prior mechanical ventilation, non invasive ventilation was instituted in 71% of children. Anemia was observed in 78% of the sample. In 51 infants (86.5% the lower airway obstructive pattern was maintained up to tracheal extubation with a nil mortality and low prevalence of pneumothorax (7.8%. Acute respiratory distress syndrome occurred in 8 infants (13.5%, with higher mortality and a higher prevalence of pneumothorax (62.5%. CONCLUSIONS: The declining mortality in acute viral bronchiolitis is observed even in non developed regions, involving children with high rates of anemia and premature labor. The low mortality is associated with the maintenance of the lower airway obstructive pattern during the period on mechanical ventilation. The development of acute respiratory distress syndrome is associated with increased mortality and higher prevalence of complications, representing the actual challenge in the management of children with severe acute viral bronchiolitis.

  6. Alagille syndrome.

    OpenAIRE

    Krantz, I D; Piccoli, D A; Spinner, N B

    1997-01-01

    Alagille syndrome (OMIM 118450) is an autosomal dominant disorder associated with abnormalities of the liver, heart, eye, skeleton, and a characteristic facial appearance. Also referred to as the Alagille-Watson syndrome, syndromic bile duct paucity, and arteriohepatic dysplasia, it is a significant cause of neonatal jaundice and cholestasis in older children. In the fully expressed syndrome, affected subjects have intrahepatic bile duct paucity and cholestasis, in conjunction with cardiac ma...

  7. Cushing Syndrome

    Science.gov (United States)

    ... links Share this: Page Content What is Cushing’s syndrome? Cushing’s syndrome is a condition that occurs when the body’s ... medication or as a result of a tumor, Cushing’s syndrome can develop. Many factors influence whether this happens, ...

  8. Dumping Syndrome

    Science.gov (United States)

    ... Disease Organizations​​ (PDF, 341 KB)​​​​​ Alternate Language URL Dumping Syndrome Page Content On this page: What is ... Nutrition Points to Remember Clinical Trials What is dumping syndrome? Dumping syndrome occurs when food, especially sugar, ...

  9. Association of Obstructive Sleep Apnea Syndrome and Buerger's Disease: a Pilot Study

    Directory of Open Access Journals (Sweden)

    Gholam Hosein Kazemzadeh

    2015-10-01

    Full Text Available In this study we evaluated the incidence and severity of obstructive sleep apnea and Obstructive sleep apnea syndrome in patients with thromboangiitis obliterans for reduction of crisis. In 40 patients with Buerger's disease daily sleepiness and risk of Obstructive sleep apnea were evaluated using the Epworth sleeping scale (ESS and the Stop-Bang score. An Apnea-link device was used for evaluation of chest motion, peripheral oxygenation, and nasal airflow during night-time sleep. The apnea/hypopnea index (AHI and respiratory tdisurbance index were used for Obstructive sleep apnea syndrome diagnosis. All subjects were cigarette smokers and 80% were opium addicted. The prevalence of Obstructive sleep apnea (AHI>5 was 80%, but incidence of Obstructive sleep apnea syndrome (AHI>5 + ESS≥10 was 5% (2/40. There was no association between duration or frequency of hospitalization and Obstructive sleep apnea syndrome (P=0.74 and 0.86, respectively. In addition, no correlation between ESS and Stop-Bang scores and AHI was observed (P=0.58 and 0.41, respectively. There was an inverse correlation between smoking rate and AHI (P=0.032, r = −0.48. We did not find an association between Buerger's disease and Obstructive sleep apnea syndrome. Although the AHI was high (80% and daily sleepiness was low. The negative correlation of smoking with AHI and on the other hand daily napping in addiction may be caused by the absence of a clear relationship between Obstructive sleep apnea syndrome and Buerger's disease.

  10. Urofacial syndrome

    Directory of Open Access Journals (Sweden)

    Kamal F Akl

    2012-01-01

    Full Text Available The urofacial syndrome is characterized by functional obstructive uropathy asso-ciated with an inverted smile. The importance of the subject is that it sheds light, not only on the muscles of facial expression, but also on the inheritance of voiding disorders and lower urinary tract malformations. We report a 10-year-old-male patient who had the urofacial syndrome. Early diagnosis of the urofacial syndrome is important to avoid upper urinary tract damage and renal failure.

  11. Waardenburg syndrome.

    OpenAIRE

    Read, A P; Newton, V E

    1997-01-01

    Auditory-pigmentary syndromes are caused by physical absence of melanocytes from the skin, hair, eyes, or the stria vascularis of the cochlea. Dominantly inherited examples with patchy depigmentation are usually labelled Waardenburg syndrome (WS). Type I WS, characterised by dystopia canthorum, is caused by loss of function mutations in the PAX3 gene. Type III WS (Klein-Waardenburg syndrome, with abnormalities of the arms) is an extreme presentation of type I; some but not all patients are ho...

  12. Sweet Syndrome

    OpenAIRE

    Kasapçopur, Özgür; Sever, Lale; Çalışkan, Salim; Kodakoğlu, Ramazan; Mat, Cem; Kaner, Gültekin; Arısoy, Nil

    1996-01-01

    Sweet syndrome is a vasculitis characterized with fever leucocytosis neutrophilia and dermal neutrophilic infiltration In children Sweet syndrome usually occurs with secondary to infection and in adults to malignancy We report a Sweet syndrome in a five years old girl with respiratory infections otitis dactylitis long lasting fever and cutaneous rash A neutrophilic dermal infiltration is noted in cutaneous biopsy These signs have disappeared with corticosteroid treatment In conclusion Sweet s...

  13. Revesz syndrome

    Directory of Open Access Journals (Sweden)

    Dayane Cristine Issaho

    2015-04-01

    Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

  14. Metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Gogia Atul

    2006-02-01

    Full Text Available The Metabolic syndrome is a widely prevalent and multi-factorial disorder. The syndrome has been given several names, including- the metabolic syndrome, the insulin resistance syndrome, the plurimetabolic syndrome, and the deadly quartet. With the formulation of NCEP/ATP III guidelines, some uniformity and standardization has occurred in the definition of metabolic syndrome and has been very useful for epidemiological purposes. The mechanisms underlying the metabolic syndrome are not fully known; however resistance to insulin stimulated glucose uptake seems to modify biochemical responses in a way that predisposes to metabolic risk factors. The clinical relevance of the metabolic syndrome is related to its role in the development of cardiovascular disease. Management of the metabolic syndrome involves patient-education and intervention at various levels. Weight reduction is one of the main stays of treatment. In this article we comprehensively discuss this syndrome- the epidemiology, pathogenesis, clinical relevance and management. The need to do a comprehensive review of this particular syndrome has arisen in view of the ever increasing incidence of this entitiy. Soon, metabolic syndrome will overtake cigarette smoking as the number one risk factor for heart disease among the US population. Hardly any issue of any primary care medical journal can be opened without encountering an article on type 2 diabetes, dyslipidemia or hypertension. It is rare to see type 2 diabetes, dyslipidemia, obesity or hypertension in isolation. Insulin resistance and resulting hyperinsulinemia have been implicated in the development of glucose intolerance (and progression to type 2 diabetes, hypertriglyceridemia, hypertension, polycystic ovary yndrome, hypercoagulability and vascular inflammation, as well as the eventual development of atherosclerotic cardiovascular disease manifested as myocardial infarction, stroke and myriad end organ diseases. Conversely

  15. Brugada syndrome

    Directory of Open Access Journals (Sweden)

    Bockeria O.L.

    2015-03-01

    Full Text Available Brugada syndrome is characterized by sudden death associated with one of several ECG patterns including incomplete right bundle-branch block and ST-segment elevation in the anterior precordial leads. According to the ECG patterns there are three types of Brugada syndrome. Brugada syndrome is genetically determined and has an autosomal dominant pattern of transmission in about 50% of familial cases. Nowadays implantation of cardioverter-defibrillator is the only proven method of sudden cardiac death prevention.

  16. Velocardiofacial syndrome.

    OpenAIRE

    Pike, A. C.; Super, M.

    1997-01-01

    Velocardiofacial syndrome is a syndrome of multiple anomalies that include cleft palate, cardiac defects, learning difficulties, speech disorder and characteristic facial features. It has an estimated incidence of 1 in 5000. The majority of cases have a microdeletion of chromosome 22q11.2. The phenotype of this condition shows considerable variation, not all the principal features are present in each case. Identification of the syndrome can be difficult as many of the anomalies are minor and ...

  17. Sheehan syndrome

    Science.gov (United States)

    Postpartum hypopituitarism; Postpartum pituitary insufficiency; Hypopituitarism Syndrome ... Malee MP. Pituitary and adrenal disorders in pregnancy. In: Gabbe ... Problem Pregnancies . 6th ed. Philadelphia, PA: Elsevier Mosby; ...

  18. A call to arms: a critical need for interventions to limit pulmonary toxicity in the stem cell transplantation patient population.

    Science.gov (United States)

    Radhakrishnan, Sabarinath Venniyil; Hildebrandt, Gerhard C

    2015-03-01

    Noninfectious pulmonary toxicity after allogeneic hematopoietic stem cell transplantation (allo-HSCT) causes significant morbidity and mortality. Main presentations are idiopathic pneumonia syndrome (IPS) in the acute setting and bronchiolitis obliterans syndrome (BOS) and cryptogenic organizing pneumonia (COP) at later time point. While COP responds well to corticosteroids, IPS and BOS often are treatment refractory. IPS, in most cases, is rapidly fatal, whereas BOS progresses over time, resulting in chronic respiratory failure, impaired quality of life, and eventually, death. Standard second-line treatments are currently lacking, and current approaches, such as augmented T cell-directed immunosuppression, B cell depletion, TNF blockade, extracorporeal photopheresis, and tyroskine kinase inhibitor therapy, are unsatisfactory with responses in only a subset of patients. Better understanding of underlying pathophysiology hopefully results in the identification of future targets for preventive and therapeutic strategies along with an emphasis on currently underutilized rehabilitative and supportive measures. PMID:25662904

  19. What Is Down Syndrome?

    Science.gov (United States)

    ... NDSS Home » Down Syndrome » What Is Down Syndrome? What Is Down Syndrome? In every cell in the ... chromosome 21 causes the characteristics of Down syndrome. What Causes Down Syndrome? Regardless of the type of ...

  20. Marfan Syndrome (For Teens)

    Science.gov (United States)

    ... How Can I Help a Friend Who Cuts? Marfan Syndrome KidsHealth > For Teens > Marfan Syndrome Print A ... a genetic disorder called Marfan syndrome. What Is Marfan Syndrome? Marfan syndrome is named after Antoine Marfan, ...

  1. Down Syndrome: Eye Problems

    Science.gov (United States)

    ... En Español Read in Chinese What causes Down syndrome? Down syndrome is caused by a duplication of all ... in persons with Down syndrome. How common is Down syndrome? The frequency of Down syndrome is approximately 1 ...

  2. Proteus Syndrome Foundation

    Science.gov (United States)

    ... Gift Stock Gift Sunshine Society Contact Privacy Policy Proteus Syndrome Foundation The Proteus Syndrome Foundation , a 501c3 ... 1 Trial with ARQ 092 in Proteus Syndrome Proteus Syndrome Patient Registry The Proteus Syndrome Foundation Contact ...

  3. Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Ravinder K. Gupta, Ritu Gupta, Sunil Dutt Sharma

    2006-10-01

    Full Text Available Turner Syndrome is one of the important chromosomal disorders characterised by loss (total or part ofsex chromosome. The manifestations being peripheral edema, short stature, extra skin fold, webbing ofneck, renal and cardiovascular anomalies, sexual infantilism, learning disability etc. We present here aone month female baby who had classical features of Turner Syndrome. The karyotape analysis wasconsistent with the diagnosis.

  4. Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Akcan AB.

    2013-06-01

    Full Text Available Turner syndrome is an important cause of short stature in girls and primer amenorrhea in young women that is usually caused by loss of part or all of an X chromosome. This topic will review the clinical manifestations, diagnosis and management of Turner syndrome.

  5. Tourette Syndrome.

    Science.gov (United States)

    Look, Kathy

    Tourette Syndrome has a history of being misdiagnosed or undiagnosed due to its unusual and complex symptoms. This paper describes: the symptoms of Tourette Syndrome; its etiology; age of onset; therapeutic methods, such as drug therapy, psychotherapy, diet control, and hypnosis; educational implications; and employment prospects. Several…

  6. Antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Cervera, Ricard; Piette, Jean-Charles; Font, Josep;

    2002-01-01

    To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

  7. Proteus syndrome

    Directory of Open Access Journals (Sweden)

    George Renu

    1993-01-01

    Full Text Available A case of proteus syndrome in a 20 year old male is repoted. Hemihypertrophy, asymmetric megalodactyly, linear epidermal naevus, naevus flammeus, angiokeratoma, lymphangioma circumscriptum, thickening of the palms and soles, scoliosis and varicose veins were present. There are only few reports of these cases in adults. The syndrome has not been reported from India.

  8. Burnout Syndrome

    OpenAIRE

    Panova, Gordana; Panov, Nenad; Stojanov, H; Sumanov, Gorgi; Panova, Blagica; Stojanovski, Angel; Nikolovska, Lence; Jovevska, Svetlana; Trajanovski, D; Asanova, D

    2013-01-01

    Introduction: Increasing work responsibilities, allocation of duties, loss of energy and motivation in everyday activities, emotional exhaustion, lack of time for themselves, insuffi cient time for rest and recreation, dissatisfaction in private life. All these symptoms can be cause of Burnout Syndrome. Aim: To see the importance of this syndrome, the consequences of job dissatisfaction, the environment, family and expression in drastic chan...

  9. Poland syndrome

    Directory of Open Access Journals (Sweden)

    Chandra Madhur Sharma

    2014-01-01

    Full Text Available Poland′s syndrome is a rare congenital condition, characterized by the absence of the sternal or breastbone portion of the pectoralis major muscle, which may be associated with the absence of nearby musculoskeletal structures. We hereby report an 8-year-old boy with typical features of Poland syndrome, the first documented case from Uttar Pradesh, India.

  10. Noonan Syndrome

    Directory of Open Access Journals (Sweden)

    Sanjeev K. Digra, Deep Aman Singh, Vikram Gupta, Ghanshyam Saini

    2004-10-01

    Full Text Available We report a 11 year old boy and his father both Noonan’s. Noonan syndrome occurs in 1 out of 2000live births. Short stature, webbing of neck, pectus carinatum or pectus excavatum, hypertelorismcubitus valgus, epicanthus, downward slanted palpebral fissures, ptosis, microganthia and earabnormalities are the common features of Noonan syndrome.

  11. Bloom's Syndrome

    Science.gov (United States)

    ... Niemann-Pick Disease, Type A Spinal Muscular Atrophy Tay-Sachs Disease Usher Syndrome, Type 1F and Type III ... Niemann-Pick Disease, Type A Spinal Muscular Atrophy Tay-Sachs Disease Usher Syndrome, Type 1F and Type III ...

  12. Fatores de risco associados à hospitalização por bronquiolite aguda no período pós-neonatal Risk factors associated with hospitalization for bronchiolitis in the post-neonatal period

    Directory of Open Access Journals (Sweden)

    Elaine P Albernaz

    2003-08-01

    Full Text Available OBJETIVO: Investigar a relação entre fatores socioeconômicos, características maternas, aleitamento e hospitalização por bronquiolite no período pós-neonatal. MÉTODOS: Estudo caso-controle, aninhado a estudo de coorte de 5.304 crianças nascidas na cidade de Pelotas, RS. O estudo de coorte constitui-se de quatro subestudos, cada um com métodos e logística específicos. As mães foram entrevistadas por meio de questionário padronizado, em visitas hospitalares e domiciliares. Foram definidos como casos as crianças com idade entre 28 e 364 dias, que haviam sido hospitalizadas por bronquiolite. RESULTADOS: De 5.304 crianças da coorte, 113 (2,1% foram hospitalizadas por bronquiolite. A análise multivariada hierarquizada, realizada pela regressão logística, mostrou os seguintes resultados: renda familiar, assim como idade gestacional, estiveram inversamente relacionadas ao risco de hospitalização por bronquiolite. O aleitamento materno desempenhou um fator protetor; crianças com tempo de aleitamento materno inferior a um mês tiveram risco 7 vezes maior de serem hospitalizadas por bronquiolite aguda nos primeiros três meses de vida. O risco de hospitalização por bronquiolite foi 57% maior naquelas expostas ao fumo materno. CONCLUSÕES: A hospitalização por bronquiolite está inversamente relacionada com renda familiar, idade gestacional e tempo de aleitamento materno, e diretamente relacionada com exposição a fumo materno. Não foi evidenciada associação com paridade ou história materna de asma brônquica.OBJECTIVE: To investigate the relationship between socioeconomic factors, maternal characteristics, breastfeeding, and hospitalization for bronchiolitis in the post-neonatal period. METHODS: A nested case-control study with a cohort of 5,304 children born in the city of Pelotas, Brazil, was conducted. The cohort study consisted of four sub-studies with their own methods and logistics. Mothers were interviewed using a

  13. Emergency department syndromic surveillance providing early warning of seasonal respiratory activity in England.

    Science.gov (United States)

    Hughes, H E; Morbey, R; Hughes, T C; Locker, T E; Pebody, R; Green, H K; Ellis, J; Smith, G E; Elliot, A J

    2016-04-01

    Seasonal respiratory infections place an increased burden on health services annually. We used a sentinel emergency department syndromic surveillance system to understand the factors driving respiratory attendances at emergency departments (EDs) in England. Trends in different respiratory indicators were observed to peak at different points during winter, with further variation observed in the distribution of attendances by age. Multiple linear regression analysis revealed acute respiratory infection and bronchitis/bronchiolitis ED attendances in patients aged 1-4 years were particularly sensitive indicators for increasing respiratory syncytial virus activity. Using near real-time surveillance of respiratory ED attendances may provide early warning of increased winter pressures in EDs, particularly driven by seasonal pathogens. This surveillance may provide additional intelligence about different categories of attendance, highlighting pressures in particular age groups, thereby aiding planning and preparation to respond to acute changes in EDs, and thus the health service in general. PMID:26415918

  14. Kounis syndrome.

    Science.gov (United States)

    Ntuli, P M; Makambwa, E

    2015-10-01

    Kounis syndrome is characterised by a group of symptoms that manifest as unstable vasospastic or non-vasospastic angina secondary to a hypersensitivity reaction. It was first described by Kounis and Zavras in 1991 as the concurrence of an allergic response with an anaphylactoid or anaphylactic reaction and coronary artery spasm or even myocardial infarction. Since then, this condition has evolved to include a number of mast cell activation disorders associated with acute coronary syndrome. There are many triggering factors, including reactions to multiple medications, exposure to radiological contrast media, poison ivy, bee stings, shellfish and coronary stents. In addition to coronary arterial involvement, Kounis syndrome comprises other arterial systems with similar physiologies, such as mesenteric and cerebral circulation resulting in ischaemia/infarction of the vital organs. The incidence of this condition is difficult to establish owing to the number of potential instigating factors and its relatively infrequent documentation in the literature.We report the case of an HIV-negative 39-year-old man with no coronary risk factors or family history of premature coronary artery disease, who developed Kounis syndrome after the administration of fluoroquinolone for dysuria. However, to the best of our knowledge,no data on the incidence and prevalence of Kounis syndrome in South Africa have ever been reported in the literature. The recent understanding of Kounis syndrome has led to the condition being classified into three syndrome variants. PMID:26636160

  15. HYDROLETHALUS SYNDROME

    Directory of Open Access Journals (Sweden)

    Aradhana

    2013-06-01

    Full Text Available INTRODUCTION: Hydrolethalus Syndrome (HLS is a rare lethal genetic syndrome, recognized as a consequence of a study on Meckle syndrome in Finland .1 HLS is characterized by multiple developmental defects of fetus which include fetal hydrocephalus, agenesis of corpus callosum, absent midline structures of brain, Cleft lip and cleft palate, defective lobulation of lungs, micrognathia and very characteristic abnormality of polydactyly. About 80% of patients have polydactyly, in hands it is postaxial and preaxial in feet with duplicated big toe. A highly characteristic hallux duplex is seen in almost no other situation .2 Club feet is also common.

  16. Neuroacanthocytosis Syndromes

    Directory of Open Access Journals (Sweden)

    Walker Ruth H

    2011-10-01

    Full Text Available Abstract Neuroacanthocytosis (NA syndromes are a group of genetically defined diseases characterized by the association of red blood cell acanthocytosis and progressive degeneration of the basal ganglia. NA syndromes are exceptionally rare with an estimated prevalence of less than 1 to 5 per 1'000'000 inhabitants for each disorder. The core NA syndromes include autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome which have a Huntington´s disease-like phenotype consisting of a choreatic movement disorder, psychiatric manifestations and cognitive decline, and additional multi-system features including myopathy and axonal neuropathy. In addition, cardiomyopathy may occur in McLeod syndrome. Acanthocytes are also found in a proportion of patients with autosomal dominant Huntington's disease-like 2, autosomal recessive pantothenate kinase-associated neurodegeneration and several inherited disorders of lipoprotein metabolism, namely abetalipoproteinemia (Bassen-Kornzweig syndrome and hypobetalipoproteinemia leading to vitamin E malabsorption. The latter disorders are characterized by a peripheral neuropathy and sensory ataxia due to dorsal column degeneration, but movement disorders and cognitive impairment are not present. NA syndromes are caused by disease-specific genetic mutations. The mechanism by which these mutations cause neurodegeneration is not known. The association of the acanthocytic membrane abnormality with selective degeneration of the basal ganglia, however, suggests a common pathogenetic pathway. Laboratory tests include blood smears to detect acanthocytosis and determination of serum creatine kinase. Cerebral magnetic resonance imaging may demonstrate striatal atrophy. Kell and Kx blood group antigens are reduced or absent in McLeod syndrome. Western blot for chorein demonstrates absence of this protein in red blood cells of chorea-acanthocytosis patients. Specific genetic testing is possible in all NA syndromes

  17. Piriformis syndrome

    Science.gov (United States)

    ... Wallet sciatica; Hip socket neuropathy; Pelvic outlet syndrome; Low back pain - piriformis ... medical help immediately if: You have sudden severe pain in your lower back or legs, along with muscle weakness or numbness ...

  18. Rett syndrome

    Science.gov (United States)

    An infant with Rett syndrome usually has normal development for the first 6 to 18 months. Symptoms range from ... of social engagement Ongoing, severe constipation and gastroesophageal reflux (GERD ) Poor circulation that can lead to cold ...

  19. Gardner Syndrome

    Science.gov (United States)

    ... syndromes. For more information, talk with an assisted reproduction specialist at a fertility clinic. How common is ... detected X-ray or computed tomography (CT or CAT) scan of the small bowel if adenomas are ...

  20. Piriformis Syndrome

    Science.gov (United States)

    ... syndrome occurs when this muscle presses on your sciatic nerve (the nerve that goes from your spinal cord ... cause the piriformis muscle to press against the sciatic nerve, such as sitting, walking up stairs or running. ...

  1. Marfan Syndrome

    Science.gov (United States)

    ... caved-in look. He also wore glasses for myopia (say: my-OH-pee-uh), or nearsightedness, which ... syndrome, this "glue" is weaker than normal. This causes changes in many systems of the body, but ...

  2. Aase syndrome

    Science.gov (United States)

    ... a provider who has experience treating anemias. A bone marrow transplant may be necessary if other treatment fails. ... counseling is recommended if you have a family history of this syndrome and wish to become pregnant.

  3. Hunter syndrome

    Science.gov (United States)

    ... to your health care provider for more information. Bone marrow transplant has been tried for the early-onset form, ... to have children and who have a family history of Hunter syndrome. Prenatal testing is available. Carrier ...

  4. Hurler syndrome

    Science.gov (United States)

    ... to your health care provider for more information. Bone marrow transplant has been used in several people with this ... Call your provider if: You have a family history of Hurler syndrome and are considering having children ...

  5. [Heptopulmonary syndrome].

    Science.gov (United States)

    Cuadrado, Antonio; Díaz, Ainhoa; Iruzubieta, Paula; Salcines, José Ramón; Crespo, Javier

    2015-01-01

    Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients' prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome. PMID:25840463

  6. Turcot Syndrome

    Science.gov (United States)

    ... procedure done in conjunction with in-vitro fertilization (IVF). It allows people who carry a specific known ... screening? If you are concerned about your family history and think your family may have Turcot syndrome, ...

  7. Levator Syndrome

    Science.gov (United States)

    ... 2 Diabetes, Heart Disease a Dangerous Combo Are 'Workaholics' Prone to OCD, Anxiety? ALL NEWS > Resources First ... are variations of levator syndrome. The muscle spasm causes pain that typically is not related to defecation. ...

  8. Pendred Syndrome

    Science.gov (United States)

    ... Health & Human Services National Institutes of Health Search Search form Search A–Z Index Español Menu Home ... children, the thyroid is important for normal growth and development. Children with Pendred syndrome, however, rarely have problems ...

  9. Goodpasture syndrome

    Science.gov (United States)

    ... glomerulonephritis with pulmonary hemorrhage; Pulmonary renal syndrome; Glomerulonephritis - pulmonary hemorrhage ... when urinating Nausea and vomiting Pale skin Swelling (edema) in any area of the body, especially in the legs

  10. Tourette Syndrome

    Science.gov (United States)

    ... methylphenidate and clonidine in children with ADHD and tics. Developing New Treatments for Tourette Syndrome: Clinical and Basic Science Dialogue Publicaciones en Español Síndrome de Tourette Prepared ...

  11. Alport Syndrome

    Science.gov (United States)

    ... syndrome diagnosed? Your healthcare provider will have to watch your signs, symptoms, and look at your family ... 05/2016 - 10:00am Philadelphia, PA Kidney Camp Sun, 07/17/2016 - 6:00pm Ingleside, IL Register ...

  12. Barth Syndrome

    DEFF Research Database (Denmark)

    Saric, Ana; Andreau, Karine; Armand, Anne-Sophie;

    2016-01-01

    Mutations in the gene encoding the enzyme tafazzin, TAZ, cause Barth syndrome (BTHS). Individuals with this X-linked multisystem disorder present cardiomyopathy (CM) (often dilated), skeletal muscle weakness, neutropenia, growth retardation, and 3-methylglutaconic aciduria. Biopsies of the heart...

  13. [DIDMOAD syndrome].

    Science.gov (United States)

    Alicanoğlu, R; Canbakan, B; Yildiz, N; Arikan, E; Kundur, H; Bahtiyar, K; Sayali, E

    1994-01-01

    The DIDMOAD or so called Wolfram syndrome is a hereditary disease with autosomal-recessive transmission showing 4 main features: diabetes mellitus, diabetes insipidus, nervus opticus atrophia and deafness. Beside this it shows multiple organ involvement. Our 38-year old male patient, showing all above mentioned features except deafness had urinary tract involvement and neurological symptoms. EEG, cerebral MRI, tests with evoked potentials and HLA-typing were performed to discuss the aetiopathogenetic background in our patient. Almost all symptoms of the Wolfram syndrome can be mixed up with complications of diabetes mellitus, which is usually the first symptom of the Wolfram syndrome. Because of this, wrong diagnosis is not rare. Hence in differential diagnosis in any diabetes mellitus type I patient, the possibility of the Wolfram syndrome should be discussed. PMID:8023526

  14. Heyde's syndrome

    Directory of Open Access Journals (Sweden)

    Perišić Nenad

    2006-01-01

    Full Text Available Background: Heyde's syndrome implies an association of calcified aortic stenosis with the high gradient of pressure and angiodysplasic bleeding from the digestive tract. It has been proven that in patients with this syndrome, acquired form of von Willebrand type II A develops. Replacing of aortic valves by artificial ones brings about the spontaneous retreat of coagulation disorder, and the stoppage of the digestive tract bleeding. Case report. We reported two patients with the Heyde's syndrome. In one of the patients the aortic valves were replaced by biologic valves, after which the digestive tract bleeding stopped, while the second patient was treated conservatively due to a high operation risk. Conclusion. Patients with Heyde's syndrome are a complex multidisciplinary problem, thus their adequate treatment requires a team work in order to provide the most rational type of therapy for each patient separately.

  15. Reifenstein syndrome

    Science.gov (United States)

    ... male sex hormones (androgens). Testosterone is a male sex hormone. This disorder is a type of androgen insufficiency syndrome. ... Donohoue PA. Disorders of sex development. In: Kliegman RM, Stanton ... J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics . ...

  16. HELLP syndrome

    Science.gov (United States)

    ... out of 1,000 pregnancies. In women with preeclampsia or eclampsia , the condition develops in 10 to ... have high blood pressure and are diagnosed with preeclampsia before they develop HELLP syndrome. In some cases, ...

  17. Kindler syndrome

    OpenAIRE

    Kaviarasan P; Prasad P; Shradda; Viswanathan P

    2005-01-01

    Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderm...

  18. Turner Syndrome

    OpenAIRE

    Ramachandran Sudarshan; G Sree Vijayabala; KS Prem Kumar

    2012-01-01

    Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate th...

  19. Pendred's syndrome

    International Nuclear Information System (INIS)

    This report describes Pendred's syndrome in three siblings of a consanguineous marriage, belonging to Rahimyar Khan. The children presented with deafmutism and goiters. The investigations included scintigram, perchlorate discharge test and audiometery. The perchlorate discharge was positive in index case. Bilateral sensorineural hearing defect was detected on Pure Tone Average (PTA) audiometry. Meticulous clinical and laboratory evaluation is mandatory for the detection of rare disorders like Pendred's syndrome. (author)

  20. Turner Syndrome: Other FAQs

    Science.gov (United States)

    ... NICHD Research Information Clinical Trials Resources and Publications Turner Syndrome: Other FAQs Skip sharing on social media links ... been diagnosed with Turner syndrome. Now what? Is Turner syndrome inherited? Turner syndrome is usually not inherited, but ...

  1. Learning about Down Syndrome

    Science.gov (United States)

    ... for the genetic terms used on this page Learning About Down Syndrome What is Down syndrome? What ... Down syndrome? People who have Down syndrome have learning difficulties, mental retardation, a characteristic facial appearance, and ...

  2. Burning Mouth Syndrome

    Science.gov (United States)

    ... OralHealth > Topics > Burning Mouth Syndrome > Burning Mouth Syndrome Burning Mouth Syndrome Main Content Key Points Symptoms Diagnosis Primary and Secondary BMS Treatment Helpful Tips Key Points Burning mouth syndrome is burning pain in the mouth that may ...

  3. Pfeiffer syndrome

    Directory of Open Access Journals (Sweden)

    Fryns Jean-Pierre

    2006-06-01

    Full Text Available Abstract Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.

  4. Antiphospholipid syndrome.

    Science.gov (United States)

    Ruiz-Irastorza, Guillermo; Crowther, Mark; Branch, Ware; Khamashta, Munther A

    2010-10-30

    The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs. PMID:20822807

  5. Antibody-Mediated Lung Transplant Rejection

    Science.gov (United States)

    Hachem, Ramsey

    2012-01-01

    Antibody-mediated rejection after lung transplantation remains enigmatic. However, emerging evidence over the past several years suggests that humoral immunity plays an important role in allograft rejection. Indeed, the development of donor-specific antibodies after transplantation has been identified as an independent risk factor for acute cellular rejection and bronchiolitis obliterans syndrome. Furthermore, cases of acute antibody-mediated rejection resulting in severe allograft dysfunction have been reported, and these demonstrate that antibodies can directly injure the allograft. However, the incidence and toll of antibody-mediated rejection are unknown because there is no widely accepted definition and some cases may be unrecognized. Clearly, humoral immunity has become an important area for research and clinical investigation. PMID:23002428

  6. An international ISHLT/ATS/ERS clinical practice guideline:

    DEFF Research Database (Denmark)

    Meyer, Keith C; Raghu, Ganesh; Verleden, Geert M;

    2014-01-01

    describe and/or provide recommendations for 1) the definition of BOS, 2) the risk factors for developing BOS, 3) the diagnosis of BOS, and 4) the management and prevention of BOS. A pragmatic evidence synthesis was performed to identify all unique citations related to BOS published from 1980 through to...... March, 2013. The expert committee discussed the available research evidence upon which the updated definition of BOS, identified risk factors and recommendations are based. The committee followed the GRADE (Grading of Recommendation, Assessment, Development and Evaluation) approach to develop specific......Bronchiolitis obliterans syndrome (BOS) is a major complication of lung transplantation that is associated with poor survival. The International Society for Heart and Lung Transplantation, American Thoracic Society, and European Respiratory Society convened a committee of international experts to...

  7. Computed tomography findings of postoperative complications in lung transplantation; Achados tomograficos nas complicacoes pos-operatorias do transplante pulmonar

    Energy Technology Data Exchange (ETDEWEB)

    Hochhegger, Bruno; Irion, Klaus Loureiro; Marchiori, Edson; Bello, Rodrigo; Moreira, Jose; Camargo, Jose Jesus [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Postgraduate Program in Respiratory Sciences; Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Postgraduate Program in Radiological Sciences], e-mail: brunorgs@mail.ufsm.br

    2009-03-15

    Due to the increasing number and improved survival of lung transplant recipients, radiologists should be aware of the imaging features of the postoperative complications that can occur in such patients. The early treatment of complications is important for the long-term survival of lung transplant recipients. Frequently, HRCT plays a central role in the investigation of such complications. Early recognition of the signs of complications allows treatment to be initiated earlier, which improves survival. The aim of this pictorial review was to demonstrate the CT scan appearance of pulmonary complications such as reperfusion edema, acute rejection, infection, pulmonary thromboembolism, chronic rejection, bronchiolitis obliterans syndrome, cryptogenic organizing pneumonia, post transplant lymphoproliferative disorder, bronchial dehiscence and bronchial stenosis. (author)

  8. Computed tomography findings of postoperative complications in lung transplantation

    International Nuclear Information System (INIS)

    Due to the increasing number and improved survival of lung transplant recipients, radiologists should be aware of the imaging features of the postoperative complications that can occur in such patients. The early treatment of complications is important for the long-term survival of lung transplant recipients. Frequently, HRCT plays a central role in the investigation of such complications. Early recognition of the signs of complications allows treatment to be initiated earlier, which improves survival. The aim of this pictorial review was to demonstrate the CT scan appearance of pulmonary complications such as reperfusion edema, acute rejection, infection, pulmonary thromboembolism, chronic rejection, bronchiolitis obliterans syndrome, cryptogenic organizing pneumonia, post transplant lymphoproliferative disorder, bronchial dehiscence and bronchial stenosis. (author)

  9. Advantages and drawbacks of long-term macrolide use in the treatment of non-cystic fibrosis bronchiectasis

    Science.gov (United States)

    Fan, Li-Chao

    2014-01-01

    Non-cystic fibrosis (non-CF) bronchiectasis is a respiratory disease characterized by persistent airway inflammation and dilation of bronchial wall driven by various causes. Patients with bronchiectasis suffer from excessive sputum production, recurrent exacerbations, and progressive airway destruction. Major therapy for bronchiectasis is focused on breaking the “vicious cycle” of mucus stasis, infection, inflammation, and airway destruction. Growing evidences have been shown that macrolides possess immunoregulatory and anti-inflammatory functions beyond their antimicrobial effects. Macrolide antibiotics have been effectively used in the treatment of diffuse panbronchiolitis, CF and bronchiolitis obliterans syndrome. Currently a number of clinical trials were performed to assess macrolide treatment in the management of non-CF bronchiectasis. The purpose of this paper is to review the efficacy and potential risks of these recent studies on the use of macrolides in non-CF bronchiectasis. PMID:25093082

  10. Serotonin Syndrome

    Directory of Open Access Journals (Sweden)

    Harold Muñoz Cortés

    2004-08-01

    Full Text Available The serotonin syndrome is a clinical condition associated with serotonin agonists, prescribed to treat some psychiatric and non psychiatric diseases like affective, anxiety and pain disorders. Is due to an excessive stimulation of central and peripheral serotonin receptors that leads to mental, autonomic and neuromuscular changes. Usually the disorder resolves within the first 24 hours after the medications are discontinued, however some patients progress to a multiple organ failure and die. This paper is a theoretical review of the fundamental aspects of the serotonin syndrome, beginning with a brief review of the anatomic and physiologic features of serotonin system, to continue to examine the most relevant historic, diagnosis, clinical and treatment aspects of the syndrome.

  11. Postconcussional Syndrome

    Directory of Open Access Journals (Sweden)

    Necla Keskin

    2013-02-01

    Full Text Available Postconcussional syndrome is characterized by somatic, cognitive and psychiatric (emotional, behavioral symptoms that occurs after mild traumatic brain injury. It has been known that these symptoms recover fully within 3-6 months almost in 90% of patients. Although its etiology is still controversial, biological, psychological and social factors may account for the development and continuation of the symptoms. Diagnosis is based on the subjective complaints. To find out an objective method for definite diagnosis, trials searching for both neuroimaging and specific serum biomarkers stil continue. The treatment of the syndrome is mainly of palliative nature. Information, education, reassurance and multifaceted rehabilitation programmes can be beneficial. There are promising trials reporting the effectiveness of cognitive behavioral therapy in the treatment of postconcussional syndrome. [Archives Medical Review Journal 2013; 22(1.000: 96-109

  12. Nutcracker syndrome

    International Nuclear Information System (INIS)

    Purpose: The purpose of this case study is to highlight the symptoms of the Nutcracker Syndrome (NCS), the methods of clinical investigations and the importance of differential diagnosis. Introduction: The NCS refers to left renal vein entrapment caused by abnormal branching patterns of the superior mesenteric artery from the aorta.1,2 Clinical case presentation: A 27 years old female presented to the emergency department with complaints of abdominal discomfort, bloating, loose bowel motions and irregular micro-haematuria. The radiologist's report indicated the findings from computed tomography examination to be consistent with anterior NCS. Discussion: In most of the NCS cases the clinical symptoms are non-specific.3 The syndrome is caused by a vascular disorder, but its clinical manifestation can relate to a wide range of abdominal, urological, endovascular or gynaecological pathologies.4 Conclusion: Nutcracker Syndrome is a relatively rare disease and underdiagnosed may lead to left renal vein thrombosis

  13. Refeeding syndrome

    Directory of Open Access Journals (Sweden)

    Tripathy Swagata

    2008-01-01

    Full Text Available We report a case of a fifty-year-old male who was admitted with a three month history of increasing weakness, prostration, decreasing appetite and inability to swallow. The patient was a chronic alcoholic, unemployed, and of very poor socioeconomic background. The patient was initially investigated for upper GI malignancy, Addisons disease, bulbar palsy and other endocrinopathies. Concurrent management was started for severe electrolyte abnormalities and enteral nutritional supplementation was begun. By the fourth day of feeding patient developed severe hypophosphatemia and other life-threatening features suggesting refeeding syndrome. The patient was managed for the manifestations of refeeding syndrome. A final diagnosis of chronic alcoholic malnutrition with refeeding syndrome was made. Refeeding of previously starving patients may lead to a variety of complications including sudden death.

  14. Eagle's Syndrome

    Directory of Open Access Journals (Sweden)

    Pinheiro, Thaís Gonçalves

    2014-01-01

    Full Text Available Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical.

  15. Waardenburg syndrome

    Directory of Open Access Journals (Sweden)

    Tagra Sunita

    2006-01-01

    Full Text Available Waardenburg syndrome is a rare inherited and genetically heterogenous disorder of neural crest cell development. Four distinct subtypes showing marked interfamilial and intrafamilial variability have been described. We report a girl showing constellation of congenital hearing impairment with 110 dB and 105 dB loss in right and left ear respectively, hypoplastic blue iridis, white forelock, dystopia canthorum and broad nasal root. Other affected relatives of the family, with variable features of the syndrome, have been depicted in the pedigree.

  16. [Eisenmenger syndrome].

    Science.gov (United States)

    Jensen, Annette Schophuus; Iversen, Kasper; Vejlstrup, Niels G; Hansen, Peter Bo; Søndergaard, Lars

    2009-04-01

    Congenital heart disease with left-to-right shunt can induce proliferation, vasoconstriction and thrombosis in the pulmonary vascular bed. Eventually, the patient may develop Eisenmenger syndrome defined as pulmonary arterial hypertension caused by high pulmonary vascular resistance with right-to-left shunt and cyanosis. Patients with Eisenmenger syndrome suffer a high risk of complications in connection with acute medical conditions, extra-cardiac surgery and pregnancy. This article describes the precautions that should be taken to reduce morbidity and mortality in these patients. PMID:19416617

  17. Rapunzel syndrome

    International Nuclear Information System (INIS)

    An 18-year-old single female patient, presented with non specific gastrointestinal symptoms of anorexia, abdominal pain, and change in bowel habit. Clinically she was anemic, cachectic, and depressed. Abdominal examination revealed mobile epigastric mass. The scalp alopecia and endoscopy coupled by computed tomography scan, confirmed the diagnoses of trichobezoar, but it was not diagnosed as Rapunzel syndrome except after laparotomy, gastrotomy, and enterotomy. There are less than 16 cases of Rapunzel syndrome described worldwide, and this is the first case to be described in the middle east. (author)

  18. Joubert syndrome

    International Nuclear Information System (INIS)

    Joubert syndrome is a rare malformation of the posterior fossa, mainly affecting the cerebellar vermis, which generally appears as a dysplastic lesion. Other structures of the cervico medullary junction may be involved, with accompanying brainstem hypoplasia according to neuroimaging studies. The diagnosis is usually reached during, childhood, based on a constellation of changes in the child's neurological development that are supported by the results of imaging studied. Respiratory problems are the most common signs in newborns,leading to the suspicion of the presence of this syndrome. (Author) 11 refs

  19. Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Ramachandran Sudarshan

    2012-08-01

    Full Text Available Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate their possible management. [Archives Medical Review Journal 2012; 21(4.000: 246-252

  20. Eisenmengers syndrom

    DEFF Research Database (Denmark)

    Jensen, Annette Schophuus; Iversen, Kasper; Vejlstrup, Niels G;

    2009-01-01

    Congenital heart disease with left-to-right shunt can induce proliferation, vasoconstriction and thrombosis in the pulmonary vascular bed. Eventually, the patient may develop Eisenmenger syndrome defined as pulmonary arterial hypertension caused by high pulmonary vascular resistance with right......-to-left shunt and cyanosis. Patients with Eisenmenger syndrome suffer a high risk of complications in connection with acute medical conditions, extra-cardiac surgery and pregnancy. This article describes the precautions that should be taken to reduce morbidity and mortality in these patients. Udgivelsesdato...

  1. Olmsted syndrome

    Directory of Open Access Journals (Sweden)

    Kumar Pramod

    2008-01-01

    Full Text Available Olmsted syndrome is a rare disorder characterized by the combination of periorificial, keratotic plaques and bilateral palmoplantar keratoderma. New associated features are being reported. Olmsted syndrome is particularly rare in a female patient, and we report such a case in a six year-old Indian girl, who presented with keratoderma of her soles since birth and on her palms since the age of two years along with perioral and perinasal hyperkeratosis. She had sparse, light brown, thin hair. Although the psychomotor development of the child was normal until 18 months of age, the keratoderma plaques had restricted the child′s mobility after that stage.

  2. Eagle syndrome

    International Nuclear Information System (INIS)

    Eagle syndrome occurs due to elongation of the styloid process or calcification of the stylohyoid ligament, which then may produce a pain sensation due the pressure exerted on various structures in the head and neck. When suspected, imaging helps in identifying the abnormally elongated styloid process or the calcified ligament. In recent years, three-dimensional CT (3DCT) has proved to be valuable in these cases. We report the case of a 62-year-old man with this syndrome in whom imaging with 3DCT conclusively established the diagnosis

  3. Lemierre's syndrome.

    LENUS (Irish Health Repository)

    O'Dwyer, D N

    2012-02-01

    Lemierre\\'s syndrome is a rare disease that results in an oropharyngeal infection, which precipitates an internal jugular vein thrombosis and metastatic infection. Fusobacterium necrophorum is an anaerobic Gram-negative bacillus and has been identified as the causative agent. We describe the case of a young girl whose presentation and diagnosis were confounded by a history of valvular heart disease. Infection of heart valves can produce many of the signs and symptoms associated with Lemierre\\'s syndrome. We describe the diagnosis, investigation and optimal management of this rare disorder.

  4. Morbihan syndrome

    Directory of Open Access Journals (Sweden)

    Stefano Veraldi

    2013-01-01

    Full Text Available We report a case of severe Morbihan syndrome (chronic erythematous edema of the upper portion of the face in a 60-year-old man. The syndrome was characterized clinically by erythematous edema involving the forehead, glabella, and both eyelids, because of which the patient was not able to open completely his eyes. Furthermore, erythema and telangiectasiae were visible on the nose and cheeks. Laboratory and instrumental examinations were within normal ranges or negative. Histopathological examination showed dermal edema, perivascular and periadnexal lympho-histiocytic infiltrate, and sebaceous gland hyperplasia. Oral isotretinoin was ineffective despite the relatively long duration of the therapy (26 weeks.

  5. Burnout syndrome

    OpenAIRE

    Bábská, Simona

    2014-01-01

    This bachelor thesis deals with the so-called burnout syndrome, which, as I believe, is getting to be a serious problem in today´s busy world. This issue deserves a full attention especially from those concerned – workers in assisting professions. What usually precedes the burnout syndrome is a big enthusiasm and motivation for work in which a potential patient can help other people and get them out of their troubles, sometimes he /she feels even like having a mission. However, without kno...

  6. Marfan syndrome masked by Down syndrome?

    NARCIS (Netherlands)

    J.C. Vis; K. van Engelen; J. Timmermans; B.C. Hamel; B.J.M. Mulder

    2009-01-01

    Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare. We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly few manifestations of Marfan syndrome. Althou

  7. Metabolic Syndrome

    Science.gov (United States)

    ... If you already have metabolic syndrome, making these healthy lifestyle choices can help reduce your risk of heart disease and other health problems. If lifestyle changes alone can’t control your ... to help. Maintain a healthy weight Your doctor can measure your body mass ...

  8. Nephrotic Syndrome

    Science.gov (United States)

    ... use of certain legal and illegal drugs, or morbid obesity can lead to nephrotic syndrome. Symptoms Some kids ... KidsHealth® is for educational purposes only. For specific medical advice, diagnoses, and treatment, consult your doctor. © 1995- The Nemours Foundation. All ...

  9. Robinow Syndrome

    Directory of Open Access Journals (Sweden)

    Gökhan Gökalp

    2010-05-01

    Full Text Available Introduction: Robinow syndrome is characterized by dwarfism demonstrating short-limbed extremities, vertebral malsegmentation/malformation (hemivertebra, costal dysplasia, genital hypoplasia, and fetal facial appearance (wide and prominent forehead, hypertelorism, small and wide nose, molar hypoplasia, and retrognathia. It is a rare genetic disease which may present with either mild autosomal dominant form or severe recessive form. Vertebral and costal abnormalities are common diagnostic signs that may be severe. The disease presents with kyphoscoliosis and chest abnormalities along with thoracic vertebral fusion and hemivertebral appearance. Ribs may demonstrate fusion. Based on those involvements, the disease can be categorized as spondylothoracic, spondylocostal, ischiovertebral dysplasia, and cervicofaciothoracic syndrome.Diagnosis is established by the help of clinical characteristics. Radiography might contribute to the diagnosis by revealing changes in the skeletal system. Case Report: A three-year-old male patient presented with operated left undescendent testis and buried penis. On physical examination, he also had a dysmorphic face characterized by macrocephaly, hypertelorism, prominent eyes, a flattened nasal bridge, triangular-fish mouth, gingival hypertrophy and left hand clinodactyly. Radiographic examination documented mesomelic shortening of the radius-ulna, malsegmentation of the thoracal spine and the ribs fusion.Conclusion: Robinow syndrome is a rare syndrome which can be diagnosed by typical facial appearance and radiologic findings. (Journal of Current Pediatrics 2010; 8: 44-7

  10. Rett Syndrome.

    Science.gov (United States)

    Culbert, Linda A.

    This pamphlet reviews the historical process involved in initially recognizing Rett Syndrome as a specific disorder in girls. Its etiology is unknown, but studies have considered factors as hyperammonemia, a two-step mutation, a fragile X chromosome, metabolic disorder, environmental causation, dopamine deficiency, and an inactive X chromosome.…

  11. Nodding Syndrome

    Centers for Disease Control (CDC) Podcasts

    2013-12-19

    Dr. Scott Dowell, a CDC director, discusses the rare illness, nodding syndrome, in children in Africa.  Created: 12/19/2013 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 1/27/2014.

  12. Usher Syndrome

    Science.gov (United States)

    ... of their hearing within the first year of life. Progressive vision loss caused by retinitis pigmentosa becomes occurs in childhood. ... type III have progressive hearing loss and vision loss beginning in the first few decades of life. Unlike the other forms of Usher syndrome, infants ...

  13. [Refeeding syndrome].

    Science.gov (United States)

    Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

    2016-01-01

    Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it. PMID:27088791

  14. Noonan syndrome.

    NARCIS (Netherlands)

    Burgt, I. van der

    2007-01-01

    Noonan Syndrome (NS) is characterised by short stature, typical facial dysmorphology and congenital heart defects. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. The main facial features of NS are hypertelorism with down-slanting palpebral fissures, ptosis and low-set

  15. Metabolic syndrome

    Institute of Scientific and Technical Information of China (English)

    Charles Shaeffer

    2004-01-01

    @@ The emergence of cardiac disease as the number one world-wide cause of death justifies efforts to identify individuals at higher risk for preventive therapy. The metabolic syndrome, originally described by Reaven, 1 has been associated with higher cardiovascular disease risk. 2 Type Ⅱ diabetes is also a frequent sequela. 3

  16. Tourette Syndrome

    Science.gov (United States)

    ... writing, painting, or making music help focus the mind on other things. There's speculation that the composer Mozart had TS. Find support. The Tourette Syndrome Association sponsors support groups with others who understand the challenges of TS. Take control. People with TS can feel more in control ...

  17. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine; Bødtger, Uffe; Heltberg, Ole

    2014-01-01

    a variety of infectious complications. Rapid diagnosis and treatment is necessary to avoid severe complications or death. Close collaboration with local microbiologist is pivotal. Treatment consists of longterm treatment with penicillin and metronidazole. This is a case report of Lemierre's syndrome....

  18. Marfan syndrome masked by Down syndrome?

    OpenAIRE

    Mulder, B. J.; van Engelen, K.; Vis, J.C.; Timmermans, J.; Hamel, B C J

    2009-01-01

    Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare. We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly few manifestations of Marfan syndrome. Although variable expression is known to be present in Marfan syndrome, phenotypic expression of Marfan syndrome in our patient might be masked by the co-occurrence of Down syndrome. (Neth Heart J 2009;1...

  19. The Role of Human Coronaviruses in Children Hospitalized for Acute Bronchiolitis, Acute Gastroenteritis, and Febrile Seizures: A 2-Year Prospective Study.

    Directory of Open Access Journals (Sweden)

    Monika Jevšnik

    Full Text Available Human coronaviruses (HCoVs are associated with a variety of clinical presentations in children, but their role in disease remains uncertain. The objective of our prospective study was to investigate HCoVs associations with various clinical presentations in hospitalized children up to 6 years of age. Children hospitalized with acute bronchiolitis (AB, acute gastroenteritis (AGE, or febrile seizures (FS, and children admitted for elective surgical procedures (healthy controls were included in the study. In patients with AB, AGE, and FS, a nasopharyngeal (NP swab and blood sample were obtained upon admission and the follow-up visit 14 days later, whereas in children with AGE a stool sample was also acquired upon admission; in healthy controls a NP swab and stool sample were taken upon admission. Amplification of polymerase 1b gene was used to detect HCoVs in the specimens. HCoVs-positive specimens were also examined for the presence of several other viruses. HCoVs were most often detected in children with FS (19/192, 9.9%, 95% CI: 6-15%, followed by children with AGE (19/218, 8.7%, 95% CI: 5.3-13.3% and AB (20/308, 6.5%, 95% CI: 4.0-9.8%. The presence of other viruses was a common finding, most frequent in the group of children with AB (19/20, 95%, 95% CI: 75.1-99.8%, followed by FS (10/19, 52.6%, 95% CI: 28.9-75.6% and AGE (7/19, 36.8%, 95% CI: 16.3-61.6%. In healthy control children HCoVs were detected in 3/156 (1.9%, 95% CI: 0.4-5.5% NP swabs and 1/150 (0.7%, 95% CI: 0.02-3.3% stool samples. It seems that an etiological role of HCoVs is most likely in children with FS, considering that they had a higher proportion of positive HCoVs results than patients with AB and those with AGE, and had the highest viral load; however, the co-detection of other viruses was 52.6%.ClinicalTrials.gov NCT00987519.

  20. The Role of Human Coronaviruses in Children Hospitalized for Acute Bronchiolitis, Acute Gastroenteritis, and Febrile Seizures: A 2-Year Prospective Study

    Science.gov (United States)

    Jevšnik, Monika; Steyer, Andrej; Pokorn, Marko; Mrvič, Tatjana; Grosek, Štefan; Strle, Franc; Lusa, Lara; Petrovec, Miroslav

    2016-01-01

    Human coronaviruses (HCoVs) are associated with a variety of clinical presentations in children, but their role in disease remains uncertain. The objective of our prospective study was to investigate HCoVs associations with various clinical presentations in hospitalized children up to 6 years of age. Children hospitalized with acute bronchiolitis (AB), acute gastroenteritis (AGE), or febrile seizures (FS), and children admitted for elective surgical procedures (healthy controls) were included in the study. In patients with AB, AGE, and FS, a nasopharyngeal (NP) swab and blood sample were obtained upon admission and the follow-up visit 14 days later, whereas in children with AGE a stool sample was also acquired upon admission; in healthy controls a NP swab and stool sample were taken upon admission. Amplification of polymerase 1b gene was used to detect HCoVs in the specimens. HCoVs-positive specimens were also examined for the presence of several other viruses. HCoVs were most often detected in children with FS (19/192, 9.9%, 95% CI: 6–15%), followed by children with AGE (19/218, 8.7%, 95% CI: 5.3–13.3%) and AB (20/308, 6.5%, 95% CI: 4.0–9.8%). The presence of other viruses was a common finding, most frequent in the group of children with AB (19/20, 95%, 95% CI: 75.1–99.8%), followed by FS (10/19, 52.6%, 95% CI: 28.9–75.6%) and AGE (7/19, 36.8%, 95% CI: 16.3–61.6%). In healthy control children HCoVs were detected in 3/156 (1.9%, 95% CI: 0.4–5.5%) NP swabs and 1/150 (0.7%, 95% CI: 0.02–3.3%) stool samples. It seems that an etiological role of HCoVs is most likely in children with FS, considering that they had a higher proportion of positive HCoVs results than patients with AB and those with AGE, and had the highest viral load; however, the co-detection of other viruses was 52.6%. Trial Registration: ClinicalTrials.gov NCT00987519 PMID:27171141

  1. Acute respiratory distress syndrome (ARDS): HRCT findings in survivors

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Jung Im; Park, Seog Hee; Lee, Jae Mun; Song, Jeong Sup; Lee, Kyo Young [The Catholic Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-08-01

    The purpose of this report is to describe the high-resolution computed tomography (HRCT) findings of the lung in survivors of acute respiratory distress syndrome (ARDS). Among eleven patients who survived ARDS for one year, chest radiography and HRCT revealed pulmonary fibrosis in four. Causes of ARDS included pneumonia during pregnancy, near drowning, pneumonia during liver cirrhosis, and postoperative sepsis. Thoracoscopic biopsy and histopathologic correlation were available in one patient. HRCT showed diffuse interlobular septal thickening, ground glass opacity, parenchymal distortion, and traction bronchiectasis. Fuzzy centrilobular nodules were seen in two patients and one patient had multiple, large bullae in the left hemithorax. In all patients, lesions affected the upper and anterior zones of the lung more prominently. The distribution of pulmonary fibrosis was characteristic and reflected the pathogenesis of lung injury; fibrosis was largely due to hyperoxia caused by ventilator care. In one patient, histopathologic correlation showed that imaging findings were accounted for by thickening of the alveolar septum along with infiltration of chronic inflammatory cells and fibrosis. Fuzzy centrilobular nodules corresponded with bronchiolitis.

  2. Paraneoplastic syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    Paraneoplastic syndromes (PNS) comprise a diverse group of disorders that are associated with cancer but unrelated to the size, location, metastases, or physiologic activities of the mature tissue of origin. They are remote effects of tumors that may appear as signs, symptoms, or syndromes which can mimic other disease conditions encountered in veterinary medicine. Recognition of PNS is valuable for several reasons: the observed abnormalities may represent tumor cell markers and facilitate early diagnosis of the tumor; they may allow assessment of premalignant states; they may aid in the search metastases; they may help quantify and monitor response to therapy; and, they may provide insight into the study of malignant transformation and oncogene expression. This review will concentrate on the pathophysiology, diagnosis, and treatment of some of the common PNS encountered in veterinary medicine.

  3. Waardenburg syndrome

    OpenAIRE

    Mehta, Manish; Kavadu, Paresh; Chougule, Sachin

    2004-01-01

    We report a case of Waardenburg syndrome in a female child aged 2yrs. Petrus Johannes Waardenburg(1) , a Dutch Ophthalmologist in 1951 described individuals with retinal pigmentary differences who had varying degrees of hearing loss and dystopia canthorum (i.e., latral displacement of inner canthi of eyes). The disease runs in families with a dominant inheritance pattern with varying degree of clinical presentation. Patient usually present with heterochromic iris, pigmentary abnormalities of ...

  4. Waardenburg syndrome

    OpenAIRE

    Tagra Sunita; Talwar Amrita; Walia Rattan Lal; Sidhu Puneet

    2006-01-01

    Waardenburg syndrome is a rare inherited and genetically heterogenous disorder of neural crest cell development. Four distinct subtypes showing marked interfamilial and intrafamilial variability have been described. We report a girl showing constellation of congenital hearing impairment with 110 dB and 105 dB loss in right and left ear respectively, hypoplastic blue iridis, white forelock, dystopia canthorum and broad nasal root. Other affected relatives of the family, with variable features ...

  5. HABERLAND SYNDROME

    Directory of Open Access Journals (Sweden)

    Ratnakumari

    2014-08-01

    Full Text Available Encephalo cranio cutaneous lipomatosis (ECCL is a rare neuro-cutaneous syndrome. It is characterized by unilateral lipomas of the cranium, face, and neck, ipsilateral lipodermoids of the eye, ipsilateral brain anomalies. There are 53 cases mentioned so far in the literature. To our knowledge, only 3 cases were reported from India. We report a case of a baby girl who presented in our institution for neuro-radiological evaluation based on which diagnosis of ECCL was made.

  6. Turner Syndrome

    OpenAIRE

    Akcan AB.

    2007-01-01

    Turner syndrome (TS) is a neurogenetic disorder characterized by partial or complete monosomy-X. TS is associated with certain physical and medical features including estrogen deficiency, short stature and increased risk for several diseases with cardiac conditions being among the most serious. Girls with TS are typically treated with growth hormone and estrogen replacement therapies to address short stature and estrogen deficiency. The cognitive-behavioral phenotype associated with TS includ...

  7. Robinow Syndrome

    OpenAIRE

    Gökhan Gökalp; Erdal Eren; Zeynep Yazıcı; Halil Sağlam

    2010-01-01

    Introduction: Robinow syndrome is characterized by dwarfism demonstrating short-limbed extremities, vertebral malsegmentation/malformation (hemivertebra), costal dysplasia, genital hypoplasia, and fetal facial appearance (wide and prominent forehead, hypertelorism, small and wide nose, molar hypoplasia, and retrognathia). It is a rare genetic disease which may present with either mild autosomal dominant form or severe recessive form. Vertebral and costal abnormalities are common diagnostic si...

  8. Marfan syndrome.

    OpenAIRE

    Jain, Eesha; Pandey, Ramesh Kumar

    1997-01-01

    Marfan syndrome is a rare autosomal dominant disorder of the connective tissue, with skeletal, ligamentous, orooculofacial, pulmonary, abdominal, neurological and the most fatal, cardiovascular manifestations. It has no cure but early diagnosis, regular monitoring and preventive lifestyle regimen ensure a good prognosis. However, the diagnosis can be difficult as it is essentially a clinical one, relying on family history, meticulous physical examination and investigation of involved organ sy...

  9. Mermaid syndrome

    OpenAIRE

    Çelik, Yalçın; Turhan, Ali Haydar; Gülaşı, Selvi; Kara, Tuğba; Şenli, Hicran; Atıcı, Aytuğ

    2013-01-01

    Sirenomelia also known as the mermaid syndrome is a very rare congenital anomaly characterized by lower limb fusion and severe urogenital gastrointestinal cardiovasculer central nervous system malformations We report a case of sirenomelia who had a single umblical artery renal agenesis pulmoner hypoplasia esophageal atresia ventricular septal defect anal atresia intestinal atresia and who was lost at fifth hour of life Turk Arch Ped 2013; 48: 65 7

  10. Noonan Syndrome

    OpenAIRE

    Bhambhani, Vikas; Muenke, Maximilian

    2014-01-01

    Noonan syndrome is a common genetic disorder that causes multiple congenital abnormalities and a large number of potential health conditions. Most affected individuals have characteristic facial features that evolve with age; a broad, webbed neck; increased bleeding tendency; and a high incidence of congenital heart disease, failure to thrive, short stature, feeding difficulties, sternal deformity, renal malformation, pubertal delay, cryptorchidism, developmental or behavioral problems, visio...

  11. Apert's Syndrome

    OpenAIRE

    Kumar, Gudipaneni Ravi; Jyothsna, Mandapati; Ahmed, Syed Basheer; Sree Lakshmi, Ketham Reddy

    2014-01-01

    ABSTRACT Apert's syndrome (acrocephalosyndactyly) is a rare congenital disorder characterized by craniosynostosis, midfacial malforma­tion and symmetrical syndactyly of hands and feet. Craniofacial deformities include cone-shaped calvarium, fat forehead, prop-tosis, hypertelorism and short nose with a bulbous tip. Intraoral findings include high arched palate with pseudocleft, maxillary transverse and sagittal hypoplasia with concomitant dental crowding, skeletal and dental anterior open bite...

  12. Hepatorenal syndrome

    Institute of Scientific and Technical Information of China (English)

    Sharon Turban; Paul J Thuluvath; Mohamed G Atta

    2007-01-01

    Hepatorenal syndrome (HRS) is a "functional" and reversible form of renal failure that occurs in patients with advanced chronic liver disease. The distinctive hallmark feature of HRS is the intense renal vasoconstriction caused by interactions between systemic and portal hemodynamics. This results in activation of vasoconstrictors and suppression of vasodilators in the renal circulation. Epidemiology, pathophysiology, as well as current and emerging therapies of HRS are discussed in this review.

  13. Brachycephalic Syndrome.

    Science.gov (United States)

    Dupré, Gilles; Heidenreich, Dorothee

    2016-07-01

    Animals presenting with brachycephalic syndrome suffer from multilevel obstruction of the airways as well as secondary structural collapse. Stenotic nares, aberrant turbinates, nasopharyngeal collapse, soft palate elongation and hyperplasia, laryngeal collapse, and left bronchus collapse are being described as the most common associated anomalies. Rhinoplasty and palatoplasty as well as newer surgical techniques and postoperative care strategies have resulted in significant improvement of the prognosis even in middle-aged dogs. PMID:27012936

  14. Caroli's syndrome

    International Nuclear Information System (INIS)

    In 1958 Caroli described the syndrome of congenital, either segmental or involving the entire bile duct system, saccular extensions of the intrahepatic bile ducts. He differentiated between two types of this disease pattern. The first form concerns pure cystic dilatations of the intrahepatic bile ducts, whereas the second one is combined with hepatic fibrosis and portal hypertension. Both types are characterised by cystic dilatations in the kidneys and in the extrahepatic bile ducts, pancreas and spleen. (orig.)

  15. Griscelli syndrome

    Directory of Open Access Journals (Sweden)

    Kumar T

    2006-01-01

    Full Text Available Partial albinism with immunodeficiency is a rare and fatal immunologic disorder characterized by pigmentary dilution and variable cellular immunodeficiency. It was initially described in 1978. Primary abnormalities included silvery grayish sheen to the hair, large pigment agglomerations in hair shafts and an abundance of mature melanosomes in melanocytes, with reduced pigmentation of adjacent keratinocytes. We describe a child with Griscelli syndrome who presented with hepatitis, pancytopenia and silvery hair. The diagnosis was confirmed by microscopic skin and hair examination.

  16. Asperger syndrome

    OpenAIRE

    Woodbury-Smith, Marc R.; Volkmar, Fred R.

    2008-01-01

    Abstract Asperger syndrome (AS) is a chronic neurodevelopmental disorder of social interaction, communication, and a restricted range of behaviors or interests. Although not generally associated with intellectual disability, the severe social disability and, in many cases, associated mental health and other medical problems, result in disability throughout life. The diagnosis is often delayed, sometimes into adulthood, which is unfortunate because there are now a range...

  17. Brugada syndrome

    Directory of Open Access Journals (Sweden)

    Rachel Bastiaenen

    2011-12-01

    Full Text Available The Brugada syndrome demonstrates characteristic electrocardiogram features and is a significant cause of sudden death in young adults with overtly normal cardiac structure and function. The genetic basis has not yet been fully elucidated but our understanding of the causative mutations and modifiers of arrhythmic events is advancing rapidly alongside sequencing technologies. We expect that the future will include risk stratification according to genotype and management tailored to the genetic diagnosis.

  18. Burnout syndrome

    Czech Academy of Sciences Publication Activity Database

    Kebza, V.; Šolcová, Iva

    Praha: EFPA/UPA, 2007 - (Polišenská, V.; Šolc, M.; Kotrlová, J.). s. 31 ISBN 978-80-7064-017-3. [European Conress of Psychology /10./. 03.07.2007-06.07.2007, Praha] R&D Projects: GA ČR GA406/06/0747 Institutional research plan: CEZ:AV0Z70250504 Keywords : burnout syndrome * type D personality * physiological indicators Subject RIV: AN - Psychology

  19. CREST Syndrome

    Directory of Open Access Journals (Sweden)

    Tuğçe Köksüz

    2014-06-01

    Full Text Available We report a case of CREST syndrome (calsinosis cutis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia with all of the five major symptoms. A 46-year-old woman was admitted to our clinic with the complaint of erythema, rigidity and pain on the plantar surface of the feet. She had had Raynaud’s phenomenon for 20 years and oesophageal reflux for five years. Her face had become masklike and there was prominent telangiectasies on her face and hands. Sclerosis were confined to the fingers (sclerodactyly. Direct X-ray graphy demonstrated calcinosis cutis on the left hand and suprapatellar region. She was treated with nifedipine 30 mg/day, acetylsalicylic acid 100 mg/day for Raynaud’s phenomenon and famotidine 40 mg/day, metoclopramide HCL 30 mg/day for oesophageal dysmotility. Her complaints were partially relieved after the treatment. This case had all of the five major symptoms of CREST syndrome, and we aimed to emphasize the major symptoms and complications of CREST syndrome. (Turk J Dermatol 2012; 6: 48-50

  20. Noonan syndrome.

    Science.gov (United States)

    Bhambhani, Vikas; Muenke, Maximilian

    2014-01-01

    Noonan syndrome is a common genetic disorder that causes multiple congenital abnormalities and a large number of potential health conditions. Most affected individuals have characteristic facial features that evolve with age; a broad, webbed neck; increased bleeding tendency; and a high incidence of congenital heart disease, failure to thrive, short stature, feeding difficulties, sternal deformity, renal malformation, pubertal delay, cryptorchidism, developmental or behavioral problems, vision problems, hearing loss, and lymphedema. Familial recurrence is consistent with an autosomal dominant mode of inheritance, but most cases are due to de novo mutations. Diagnosis can be made on the basis of clinical features, but may be missed in mildly affected patients. Molecular genetic testing can confirm diagnosis in 70% of cases and has important implications for genetic counseling and management. Most patients with Noonan syndrome are intellectually normal as adults, but some may require multidisciplinary evaluation and regular follow-up care. Age-based Noonan syndrome-specific growth charts and treatment guidelines are available. PMID:24444506

  1. Antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Pavlović Dragan M.

    2010-01-01

    Full Text Available Antiphospholipid syndrome (APS is an autoimmune disease with recurrent thromboses and pregnancy complications (90% are female patients that can be primary and secondary (with concomitant autoimmune disease. Antiphospholipid antibodies are prothrombotic but also act directly with brain tissue. One clinical and one laboratory criterion is necessary for the diagnosis of APS. Positive serological tests have to be confirmed after at least 12 weeks. Clinical picture consists of thromboses in many organs and spontaneous miscarriages, sometimes thrombocytopaenia and haemolytic anaemia, but neurological cases are the most frequent: headaches, stroke, encephalopathy, seizures, visual disturbances, Sneddon syndrome, dementia, vertigo, chorea, balism, transitory global amnesia, psychosis, transversal myelopathy and Guillain-Barre syndrome. About 50% of strokes below 50 years of age are caused by APS. The first line of therapy in stroke is anticoagulation: intravenous heparin or low-weight heparins. In chronic treatment, oral anticoagulation and antiplatelet therapy are used, warfarin and aspirin, mostly for life. In resistant cases, corticosteroids, intravenous immunoglobulins and plasmapheresis are necessary. Prognosis is good in most patients but some are treatment-resistant with recurrent thrombotic events and eventually death.

  2. National Down Syndrome Society

    Science.gov (United States)

    ... with Down Syndrome Since 1979 National Down Syndrome Society 8 E 41st Street, 8th Floor New York ... Program! The mission of the National Down Syndrome Society is to be the national advocate for the ...

  3. Central Pain Syndrome

    Science.gov (United States)

    ... Enhancing Diversity Find People About NINDS NINDS Central Pain Syndrome Information Page Table of Contents (click to ... being done? Clinical Trials Organizations What is Central Pain Syndrome? Central pain syndrome is a neurological condition ...

  4. Fetal Alcohol Syndrome

    Science.gov (United States)

    ... Condiciones Chinese Conditions Fetal Alcohol Syndrome Read in Chinese What is Fetal Alcohol Syndrome (FAS)? Fetal Alcohol Syndrome (FAS) describes changes in a baby born to a mother whose pregnancy was complicated by alcohol consumption. A broader term ...

  5. Tourette Syndrome (For Parents)

    Science.gov (United States)

    ... their child cope with the condition. About Tourette Syndrome Tourette syndrome (TS) is named for French doctor Georges ... people with TS. previous continue Diagnosing and Treating Tourette Syndrome Pediatricians and family doctors may refer a child ...

  6. Barth Syndrome (BTHS)

    Science.gov (United States)

    ... Awards Enhancing Diversity Find People About NINDS NINDS Barth Syndrome Information Page Table of Contents (click to jump ... is being done? Clinical Trials Organizations What is Barth Syndrome? Barth syndrome (BTHS) is a rare, genetic disorder ...

  7. Sexuality and Down Syndrome

    Science.gov (United States)

    ... NDSS Home » Resources » Wellness » Sexuality » Sexuality & Down Syndrome Sexuality & Down Syndrome Human sexuality encompasses an individual's self- ... community standards for adult behavior. How Can Healthy Sexuality be Encouraged for Individuals with Down Syndrome? Creating ...

  8. Narcotic Bowel Syndrome

    Science.gov (United States)

    ... Intolerance Malabsorption Narcotic Bowel Syndrome Radiation Therapy Injury Short Bowel Syndrome Symptoms & Causes Treatments Nutrition and Diet Managing Secondary Effects Medications Surgery Daily Living with SBS Resources SMA Syndrome Volvulus ...

  9. Middle East Respiratory Syndrome Virus Pathogenesis.

    Science.gov (United States)

    Singh, Sunit K

    2016-08-01

    Coronaviruses (CoVs) are enveloped RNA viruses that infect birds, mammals, and humans. Infections caused by human coronaviruses (hCoVs) are mostly associated with the respiratory, enteric, and nervous systems. The hCoVs only occasionally induce lower respiratory tract disease, including bronchitis, bronchiolitis, and pneumonia. In 2002 to 2003, a global outbreak of severe acute respiratory syndrome (SARS) was the seminal detection of a novel CoV (SARS-CoV). A decade later (June 2012), another novel CoV was implicated as the cause of Middle East respiratory syndrome (MERS) in Saudi Arabia. Although bats might serve as a reservoir of MERS-CoV, it is unlikely that they are the direct source for most human cases. Severe lines of evidence suggest that dromedary camels have been the major cause of transmission to humans. The emergence of MERS-CoV has triggered serious concerns about the potential for a widespread outbreak. All MERS cases were linked directly or indirectly to the Middle East region including Saudi Arabia, Jordan, Qatar, Oman, Kuwait, and UAE. MERS cases have also been reported in the later phases in the United Kingdom, France, Germany, Italy, Spain, and Tunisia. Most of these MERS cases were linked with the Middle East. The high mortality rates in family-based and hospital-based outbreaks were reported among patients with comorbidities such as diabetes and renal failure. MERS-CoV causes an acute, highly lethal pneumonia and renal dysfunction. The major complications reported in fatal cases are hyperkalemia with associated ventricular tachycardia, disseminated intravascular coagulation, pericarditis, and multiorgan failure. The case-fatality rate seems to be higher for MERS-CoV (around 30%) than for SARS-CoV (9.6%). The combination regimen of type 1 interferon + lopinavir/ritonavir is considered as the first-line therapy for MERS. Antiviral treatment is generally recommended for 10 to 14 days in patients with MERS-CoV infection. Convalescent plasma

  10. 干扰素联合布地奈德雾化吸入佐治毛细支气管炎疗效观察%The Clinical Study of Interferon Combined with Budesonide for Bronchiolitis

    Institute of Scientific and Technical Information of China (English)

    段莉; 周安海

    2012-01-01

    Objective: To evaluate the clinical efficacy of interferon combined with budesonide for bronchiolitis. Methods: One hundred and four hospitalized children who had been diagnosed bronchiolitis were randomly divided into two groups. Two groups received anti-infectives, cough suppressants, expectorants, and oxygen therapy. The treatment group was treated with interferon and budesonide in addition of the routine therapy. The symptoms and signs were observed before and after the treatment. Results: In the treatment group the total effective rate was 98.0% , while in the control group, it was 84.6% (P<0.05). The symptoms and signs such as coughing, wheezing, shortness of breath, difficulty breathing and lung wheezing, wet rale disappearance time and the average length of hospitalization in the treatment group were better than those in the control group. There was a significant difference (P<0. 05). Conclusions: Interferon combined with budesonide is effective against bronchiolitis in children patients.%目的:观察干扰素联合布地奈德雾化吸入治疗毛细支气管炎的疗效.方法:选取凉山州第一人民医院2010年2月至2012年1月诊断为毛细支气管炎的住院患儿104例,随机分为对照组和治疗组各52例.两组均予抗感染、止咳化痰、吸氧等综合治疗,治疗组在此基础上加用干扰素α-2b和布地奈德雾化吸入治疗,观察两组治疗前后咳嗽、喘憋、肺部哮鸣音等症状体征的变化.结果:治疗组和对照组的总有效率分别为98.0%、84.6%,两组比较差异有统计学意义(P<0.05).治疗组在咳嗽、喘憋、气促、呼吸困难和肺部喘鸣音、湿啰音消失时间、平均住院天数等方面均优于对照组,差异有统计学意义(P<0.05).结论:干扰素联合布地奈德雾化吸入治疗毛细支气管炎具有较好的临床疗效.

  11. Hepatorenal syndrome

    Institute of Scientific and Technical Information of China (English)

    Jan Lata

    2012-01-01

    Hepatorenal syndrome (HRS) is defined as a functional renal failure in patients with liver disease with portal hypertension and it constitutes the climax of systemic circulatory changes associated with portal hypertension.This term refers to a precisely specified syndrome featuring in particular morphologically intact kidneys,where regulatory mechanisms have minimised glomerular filtration and maximised tubular resorption and urine concentration,which ultimately results in uraemia.The syndrome occurs almost exclusively in patients with ascites.Type 1 HRS develops as a consequence of a severe reduction of effective circulating volume due to both an extreme splanchnic arterial vasodilatation and a reduction of cardiac output.Type 2 HRS is characterised by a stable or slowly progressive renal failure so that its main clinical consequence is not acute renal failure,but refractory ascites,and its impact on prognosis is less negative.Liver transplantation is the most appropriate therapeutic method,nevertheless,only a few patients can receive it.The most suitable "bridge treatments" or treatment for patients ineligible for a liver transplant include terlipressin plus albumin.Terlipressin is at an initial dose of 0.5-1 mg every 4 h by intravenous bolus to 3 mg every 4 h in cases when there is no response.Renal function recovery can be achieved in less than 50% of patients and a considerable decrease in renal function may reoccur even in patients who have been responding to therapy over the short term.Transjugular intrahepatic portosystemic shunt plays only a marginal role in the treatment of HRS.

  12. Morvan Syndrome

    Science.gov (United States)

    Maskery, Mark; Chhetri, Suresh K.; Dayanandan, Rejith; Gall, Claire

    2016-01-01

    A 74-year-old gentleman was admitted to the regional neurosciences center with encephalopathy, myokymia, and dysautonomia. Chest imaging had previously identified an incidental mass in the anterior mediastinum, consistent with a primary thymic tumor. Antivoltage-gated potassium channel (anti-VGKC) antibodies were positive (titer 1273 pmol/L) and he was hypokalemic. Electromyogram and nerve conduction studies were in keeping with peripheral nerve hyperexcitability syndrome, and an electroencephalogram was consistent with encephalopathy. A diagnosis of Morvan syndrome was made, for which he was initially treated with high-dose steroids, followed by a 5-day course of intravenous immunoglobulin (IVIG) therapy. He also underwent thymectomy, followed by a postexcision flare of his symptoms requiring intensive care management. Further steroids, plasmapheresis, and IVIG achieved stabilization of his clinical condition, enabling transfer for inpatient neurorehabilitation. He was commenced on azathioprine and a prolonged oral steroid taper. A subsequent presumed incipient relapse responded well to further IVIG treatment. This case report documents a thymoma-associated presentation of anti-VGKC-positive Morvan syndrome supplemented by patient and carer narrative and video, both of which provide valuable further insights into this rare disorder. There are a limited number of publications surrounding this rare condition available in the English literature. This, combined with the heterogenous presentation, association with underlying malignancy, response to treatment, and prognosis, provides a diagnostic challenge. However, the association with anti-VGKC antibody-associated complexes and 2 recent case series have provided some scope for both accurate diagnosis and management. PMID:26740856

  13. Trichorhinophalangeal syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Tuzovic, S.; Fiebach, B.J.O.; Magnus, L.; Sauerbrei, H.U.

    1982-11-01

    This article reports on 14 cases of a trichorhinophalangeal syndrome in five successive generations. Besides the well-known characteristics of the TRPS the following symptoms observed in this family are new: Teething was considerably delayed, intelligence was reduced, and there were skin manifestations resembling eczema. Besides, struma colli and colitis ulcerosa were also observed. Subsequent observations have to clarify whether these symptoms are a facultative part of the TRPS pattern. The constant appearance of carriers of these characteristics during five generation points to dominant heredity.

  14. Olmsted Syndrome

    Directory of Open Access Journals (Sweden)

    Sirka C

    1999-01-01

    Full Text Available A 20-year-old Sikh man had palmoplantar keratoderma, flexion deformity of digits, universal alopecia, keratotic plaques at the angles of mouth, gluteal cleft, knees and dorsal aspects of the metacarpophalangeal joints of the hand; features of Olmsted syndrome. He had normal nails, teeth, oral mucosa and normal joint movements. Treatment with acitretin, 25mg/day for three and a half months, followed by 25mg once daily alternating with 50mg once daily for 3 months resulted in significant improvement.

  15. [Piriformis syndrome].

    Science.gov (United States)

    Erauso, Thomas; Pégorie, Anne; Gaveau, Yves-Marie; Tardy, Dominique

    2010-09-20

    Sciatic pain is often misleading and establishing the link with a local muscular cause can be difficult and lead to errors, especially when faced with a young sportsman, with typical discogenic pain. Simple, specific and reproducible tests enable a better identification and treatment of a muscular cause or canal syndrome. Physiotherapy, or local infiltrations are generally very efficient, and sufficient. Surgery may be considered only in a very limited number of cases, lack of response to the first line treatment and then only if it is the absolute diagnosis, diagnosis which must remain a diagnosis of exception, more so of exclusion. PMID:21033479

  16. CREST Syndrome

    OpenAIRE

    Tuğçe Köksüz; Zeynep Nurhan Saraçoğlu; Ayşe Esra Koku-Aksu; İlham Sabuncu; Cengiz Korkmaz

    2014-01-01

    We report a case of CREST syndrome (calsinosis cutis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia) with all of the five major symptoms. A 46-year-old woman was admitted to our clinic with the complaint of erythema, rigidity and pain on the plantar surface of the feet. She had had Raynaud’s phenomenon for 20 years and oesophageal reflux for five years. Her face had become masklike and there was prominent telangiectasies on her face and hands. Sclerosis were ...

  17. Rett Syndrome

    OpenAIRE

    Sitholey, Prabhat; Agarwal, Vivek; Srivastava, Rohit

    2012-01-01

    Rett syndrome is one of the most common causes of complex disability in girls. It is characterized by early neurological regression that severely affects motor, cognitive and communication skills, by autonomic dysfunction and often a seizure disorder. It is a monogenic X-linked dominant neurodevelopmental disorder related to mutation in MECP2, which encodes the methyl-CpG-binding protein MeCP2. There are several mouse models either based on conditional knocking out of the Mecp2 gene or on a t...

  18. [Ascher's syndrome].

    Science.gov (United States)

    Halling, F; Sandrock, D; Merten, H A; Hönig, J F

    1991-01-01

    Ascher's syndrome is composed of the triad blepharochalasis, double lip and goitre. In many of the cases reported in the literature this typical constellation of symptoms is not complete; particularly the struma is not mandatorily involved. A 58-year-old patient with this rare disease who exhibited blepharochalasis and double upper and lower lip is presented. Additionally, subclinical hypothyroidism and alopecia areata totalis were found. In differential diagnosis other causes of double lips or enlargement of the lips must be considered. PMID:1817784

  19. Mazabraud syndrome

    Science.gov (United States)

    John, Anulekha Mary; Behera, Kishore Kumar; Mathai, Thomas; Parmar, Harshad; Paul, Thomas V.

    2013-01-01

    A 25 year old lady presented with pain and swelling of left thigh. On examination she was found to have tenderness of left femur with a separate soft tissue swelling within the thigh muscle. Further evaluation revealed expansile bony lesion on X-ray of left tibia and multiple hot spots on bone scan suggestive of fibrous dysplasia. The soft tissue swelling on excision and histopathological examination was found to be intramuscular myxoma. The combination of the above two, called Mazabraud syndrome is being reported. PMID:23961498

  20. Mazabraud syndrome

    Directory of Open Access Journals (Sweden)

    Anulekha Mary John

    2013-01-01

    Full Text Available A 25 year old lady presented with pain and swelling of left thigh. On examination she was found to have tenderness of left femur with a separate soft tissue swelling within the thigh muscle. Further evaluation revealed expansile bony lesion on X-ray of left tibia and multiple hot spots on bone scan suggestive of fibrous dysplasia. The soft tissue swelling on excision and histopathological examination was found to be intramuscular myxoma. The combination of the above two, called Mazabraud syndrome is being reported.

  1. Griscelli syndrome.

    Science.gov (United States)

    Ariffin, H; Geikowski, A; Chin, T F; Chau, D; Arshad, A; Abu Bakar, K; Krishnan, S

    2014-08-01

    We report a case of Griscelli Syndrome (GS). Our patient initially presented with a diagnosis of haemophagocytic lymphistiocytosis (HLH). Subsequent microscopic analysis of the patient's hair follicle revealed abnormal distribution of melanosomes in the shaft, which is a hallmark for GS. Analysis of RAB27A gene in this patient revealed a homozygous mutation in exon 6, c.550C>T, p.R184X . This nonsense mutation causes premature truncation of the protein resulting in a dysfunctional RAB27A. Recognition of GS allows appropriate institution of therapy namely chemotherapy for HLH and curative haemotopoeitic stem cell transplantation. PMID:25500851

  2. HELLP syndrome

    Directory of Open Access Journals (Sweden)

    Dilek Acar

    2014-08-01

    Suggested treatment modality consists, stabilization of blood pressure and magnesium sulfate infusion. Then evaluation of fetal status and planning delivery method and time if maternal status remains unstable. If prognosis seems favorable without urgent delivery and fetus can benefit from it, a course of betamethasone can be given to fetuses between 24 and 34 weeks of gestational age. The only and definite treatment of HELLP syndrome is delivering the baby. Suggested benefits of steroid therapy and other experimental treatments are still to be proven effective by large randomized controlled trials. [Archives Medical Review Journal 2014; 23(4.000: 735-760

  3. OCULO-CEREBRO-RENAL SYNDROME (LOWE'S SYNDROME)

    Institute of Scientific and Technical Information of China (English)

    1991-01-01

    Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.

  4. KBG syndrome

    Directory of Open Access Journals (Sweden)

    Brancati Francesco

    2006-12-01

    Full Text Available Abstract KBG syndrome is a rare condition characterised by a typical facial dysmorphism, macrodontia of the upper central incisors, skeletal (mainly costovertebral anomalies and developmental delay. To date, KBG syndrome has been reported in 45 patients. Clinical features observed in more than half of patients that may support the diagnosis are short stature, electroencephalogram (EEG anomalies (with or without seizures and abnormal hair implantation. Cutaneous syndactyly, webbed short neck, cryptorchidism, hearing loss, palatal defects, strabismus and congenital heart defects are less common findings. Autosomal dominant transmission has been observed in some families, and it is predominantly the mother, often showing a milder clinical picture, that transmits the disease. The diagnosis is currently based solely on clinical findings as the aetiology is unknown. The final diagnosis is generally achieved after the eruption of upper permanent central incisors at 7–8 years of age when the management of possible congenital anomalies should have been already planned. A full developmental assessment should be done at diagnosis and, if delays are noted, an infant stimulation program should be initiated. Subsequent management and follow-up should include an EEG, complete orthodontic evaluation, skeletal investigation with particular regard to spine curvatures and limb asymmetry, hearing testing and ophthalmologic assessment.

  5. Myasthenic syndromes.

    Science.gov (United States)

    Farrugia, M E

    2011-03-01

    The neuromuscular junction is vulnerable to autoimmune attack both at the pre-synaptic nerve terminal and at the post-synaptic muscle membrane. Antibodies directed to the nicotinic acetylcholine receptor at the muscle surface are the cause of myasthenia gravis in the majority of cases. Myasthenia gravis is an acquired condition, characterised by weakness and fatigability of the skeletal muscles. The ocular muscles are commonly affected first, but the disease often generalises. Treatment includes symptom control and immunosuppression. The thymus gland plays an important role in the pathogenesis of myasthenia gravis and thymectomy is indicated in certain subgroups. Lambert-Eaton myasthenic syndrome is associated with antibodies directed to the voltage-gated calcium channel antibodies at the pre-synaptic nerve terminal. It is an acquired condition and, in some cases, may be paraneoplastic, often secondary to underlying small cell lung carcinoma. Clinical presentation is distinct from myasthenia gravis, with patients often first presenting with lower limb muscle fatigability and autonomic symptoms. Congenital myasthenic syndromes are inherited neuromuscular disorders due to mutations in proteins at the neuromuscular junction. Various phenotypes exist depending on the protein mutation. Treatment is directed towards symptom control and immunosuppression is not indicated. PMID:21365067

  6. Classical Imaging Triad in a Very Young Child with Swyer-James Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Damle, Nishikant A. [All India Institute of Medical Sciences, New Delhi (India); Mishra, Rohini; Wadhwa, Jasmeet Kaur [B.L Kapur Memorial Hospital, New Delhi (India)

    2012-06-15

    Swyer-James syndrome (SJS) or Swyer-James-MacLeod syndrome is a rare disorder characterized by roentgenographical hyperlucency of one lung, lobe, or part of a lobe. As originally described, it shows a unilateral small lung with hyperlucency and air trapping on radiographs. It is postinfectious obliterative bronchiolitis due to childhood respiratory infection. In SJS, the involved lung or portion of the lung does not grow normally. The characteristic radiologic appearance is that of pulmonary hyperlucency on chest X-ray/CT caused by over-distention of the alveoli in conjunction with diminished arterial flow detected on Tc-99m MAA pulmonary perfusion scan. Radionuclide pulmonary ventilation studies are considered difficult to perform un very young children as patient co-operation is crucial to achieve adequate tracer entry into the lungs. We present here the case of a female child aged 2 years, who presented with chronic productive cough and fever off and on for 1.5 years. She had classical features on imaging and markedly diminished ventilation on Tc-99m DTPA aerosol study. Our case highlights the typical findings the typical findings on ventilation-perfusion scan (V/Q scan) and suggests that it is possible to conduct a satisfactory ventilation study with Tc-99m DTPA aerosol even in very young children.

  7. Classical Imaging Triad in a Very Young Child with Swyer-James Syndrome

    International Nuclear Information System (INIS)

    Swyer-James syndrome (SJS) or Swyer-James-MacLeod syndrome is a rare disorder characterized by roentgenographical hyperlucency of one lung, lobe, or part of a lobe. As originally described, it shows a unilateral small lung with hyperlucency and air trapping on radiographs. It is postinfectious obliterative bronchiolitis due to childhood respiratory infection. In SJS, the involved lung or portion of the lung does not grow normally. The characteristic radiologic appearance is that of pulmonary hyperlucency on chest X-ray/CT caused by over-distention of the alveoli in conjunction with diminished arterial flow detected on Tc-99m MAA pulmonary perfusion scan. Radionuclide pulmonary ventilation studies are considered difficult to perform un very young children as patient co-operation is crucial to achieve adequate tracer entry into the lungs. We present here the case of a female child aged 2 years, who presented with chronic productive cough and fever off and on for 1.5 years. She had classical features on imaging and markedly diminished ventilation on Tc-99m DTPA aerosol study. Our case highlights the typical findings the typical findings on ventilation-perfusion scan (V/Q scan) and suggests that it is possible to conduct a satisfactory ventilation study with Tc-99m DTPA aerosol even in very young children

  8. Dangerous drug interactions leading to hemolytic uremic syndrome following lung transplantation

    Directory of Open Access Journals (Sweden)

    Parissis Haralabos

    2010-09-01

    Full Text Available Abstract Background To report our experience of a rather uncommon drug interaction, resulting in hemolytic uremic syndrome (HUS. Methods Two consecutive cases of hemolytic uremic syndrome were diagnosed in our service. In both patients the use of macrolides in patients taking Tacrolimus, resulted in high levels of Tacrolimus. Results The first patient was a 48 years old female with Bilateral emphysema. She underwent Single Sequential Lung Transplantation. She developed reperfusion injury requiring prolonged stay. Tacrolimus introduced (Day 51. The patient remained well up till 5 months later; Erythromycin commenced for chest infection. High Tacrolimus levels and a clinical diagnosis of HUS were made. She was treated with plasmapheresis successfully. The second case was a 57 years old female with Emphysema & A1 Antithrypsin deficiency. She underwent Right Single Lung Transplantation. A2 rejection with mild Obliterative Bronchiolitis diagnosed 1 year later and she switched to Tacrolimus. She was admitted to her local Hospital two and a half years later with right middle lobe consolidation. The patient commenced on amoxicillin and clarithromycin. Worsening renal indices, high Tacrolimus levels, hemolytic anemia & low Platelets were detected. HUS diagnosed & treated with plasmapheresis. Conclusions There are 21 cases of HUS following lung transplantation in the literature that may have been induced by high tacrolimus levels. Macrolides in patients taking Cyclosporin or Tacrolimus lead to high levels. Mechanism of action could be glomeruloconstrictor effect with reduced GFR increased production of Endothelin-1 and increased Platelet aggregation.

  9. Marfan Syndrome (For Parents)

    Science.gov (United States)

    ... Tropical Delight: Melon Smoothie Pregnant? Your Baby's Growth Marfan Syndrome KidsHealth > For Parents > Marfan Syndrome Print A ... the Doctor en español Síndrome de Marfan About Marfan Syndrome Marfan syndrome is a progressive genetic disorder ...

  10. Facts about Down Syndrome

    Science.gov (United States)

    ... Us Information For... Media Policy Makers Facts about Down Syndrome Language: English Español (Spanish) Recommend on Facebook Tweet ... with Down syndrome. View charts » What is Down Syndrome? Down syndrome is a condition in which a ...

  11. Hamartomatous polyposis syndromes

    DEFF Research Database (Denmark)

    Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus;

    2014-01-01

    -intestinal symptoms and types of cancers differs.Clinical awareness and early diagnosis of HPS is important, as affected patients and at-risk family members should be offered genetic counselling and surveillance. Surveillance in children with HPS might prevent or detect intestinal or extra-intestinal complications......Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as......-intestinal cancer. The syndromes are rare and inherited in an autosomal dominant manner.The diagnosis of HPS has traditionally been based on clinical criteria, but can sometimes be difficult as the severity of symptoms range considerably from only a few symptoms to very severe cases - even within the same family...

  12. Superior Mesenteric Artery Syndrome or Wilkie Syndrome

    International Nuclear Information System (INIS)

    We described three cases of superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, chronic duodenal ileus, or cast syndrome. This syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. The major risk factors for development of SMA syndrome are rapid weight loss and surgical correction of spinal deformities. The clinical presentation of SMA syndrome is variable and nonspecific, including nausea, vomiting, abdominal pain, and weight loss. The diagnosis is based on endoscopic, radiographic and tomographic findings of duodenal compression by the SMA. The treatment of SMA syndrome is aimed at the precipitating factor, which usually is related to weight loss. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to nutritional therapy.

  13. Leopard syndrome

    Directory of Open Access Journals (Sweden)

    Dallapiccola Bruno

    2008-05-01

    Full Text Available Abstract LEOPARD syndrome (LS, OMIM 151100 is a rare multiple congenital anomalies condition, mainly characterized by skin, facial and cardiac anomalies. LEOPARD is an acronym for the major features of this disorder, including multiple Lentigines, ECG conduction abnormalities, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth, and sensorineural Deafness. About 200 patients have been reported worldwide but the real incidence of LS has not been assessed. Facial dysmorphism includes ocular hypertelorism, palpebral ptosis and low-set ears. Stature is usually below the 25th centile. Cardiac defects, in particular hypertrophic cardiomyopathy mostly involving the left ventricle, and ECG anomalies are common. The lentigines may be congenital, although more frequently manifest by the age of 4–5 years and increase throughout puberty. Additional common features are café-au-lait spots (CLS, chest anomalies, cryptorchidism, delayed puberty, hypotonia, mild developmental delay, sensorineural deafness and learning difficulties. In about 85% of the cases, a heterozygous missense mutation is detected in exons 7, 12 or 13 of the PTPN11 gene. Recently, missense mutations in the RAF1 gene have been found in two out of six PTPN11-negative LS patients. Mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. LS is largely overlapping Noonan syndrome and, during childhood, Neurofibromatosis type 1-Noonan syndrome. Diagnostic clues of LS are multiple lentigines and CLS, hypertrophic cardiomyopathy and deafness. Mutation-based differential diagnosis in patients with borderline clinical manifestations is warranted. LS is an autosomal dominant condition, with full penetrance and variable expressivity. If one parent is affected, a 50% recurrence risk is appropriate. LS should be suspected in foetuses with severe cardiac hypertrophy and prenatal DNA test may be performed. Clinical management should

  14. Metabolic Syndrome: Polycystic Ovary Syndrome.

    Science.gov (United States)

    Mortada, Rami; Williams, Tracy

    2015-08-01

    Polycystic ovary syndrome (PCOS) is a heterogeneous condition characterized by androgen excess, ovulatory dysfunction, and polycystic ovaries. It is the most common endocrinopathy among women of reproductive age, affecting between 6.5% and 8% of women, and is the most common cause of infertility. Insulin resistance is almost always present in women with PCOS, regardless of weight, and they often develop diabetes and metabolic syndrome. The Rotterdam criteria are widely used for diagnosis. These criteria require that patients have at least two of the following conditions: hyperandrogenism, ovulatory dysfunction, and polycystic ovaries. The diagnosis of PCOS also requires exclusion of other potential etiologies of hyperandrogenism and ovulatory dysfunction. The approach to PCOS management differs according to the presenting symptoms and treatment goals, particularly the patient's desire for pregnancy. Weight loss through dietary modifications and exercise is recommended for patients with PCOS who are overweight. Oral contraceptives are the first-line treatment for regulating menstrual cycles and reducing manifestations of hyperandrogenism, such as acne and hirsutism. Clomiphene is the first-line drug for management of anovulatory infertility. Metformin is recommended for metabolic abnormalities such as prediabetes, and a statin should be prescribed for cardioprotection if the patient meets standard criteria for statin therapy. PMID:26280343

  15. [Hepatopulmonary syndrome].

    Science.gov (United States)

    Thévenot, Thierry; Weil, Delphine; Garioud, Armand; Lison, Hortensia; Cadranel, Jean-François; Degano, Bruno

    2016-05-01

    Hepatopulmonary syndrome (HPS) is defined by the association of portal hypertension, increased alveolar-arterial oxygen gradient and intrapulmonary vascular dilations. Pathophysiological mechanisms of hypoxemia are characterized by ventilation-perfusion mismatch, oxygen diffusion limitation between alveolus and the centre of the dilated capillary, and right-to-left shunting. An excess of vasodilator molecules (like nitric monoxide) and proangiogenic factors (like VEGF) play an important role in the occurrence of HPS. Symptoms of HPS are not specific and dominated by a progressive dyspnea in upright position. Pulse oximetry is a simple non-invasive screening test but only detect the most severe forms of HPS. Medical treatment is disappointing and only liver transplantation may lead to resolution of HPS. Survival following liver transplantation is promising when hypoxemia is not severely decreased. PMID:27021476

  16. Antiphospholipid syndrome.

    Science.gov (United States)

    George, Diane; Erkan, Doruk

    2009-01-01

    The antiphospholipid syndrome (APS) is an autoimmune systemic disease that is diagnosed when there is vascular thrombosis and/or pregnancy morbidity occurring with persistently positive antiphospholipid antibodies (aPL) (lupus anticoagulant test, anticardiolipin antibodies, and/or anti-beta(2)-glycoprotein I antibodies). Although International APS Classification Criteria have been formulated to provide a uniform approach to APS research, aPL may cause a spectrum of clinical manifestations, some of which are not included in these criteria. The main aPL-related cardiac manifestations include valve abnormalities (vegetations and/or thickening), myocardial infarction (MI), intracardiac thrombi, and myocardial microthrombosis. In this article, we will review the definition, etiopathogenesis, clinical manifestations, diagnosis, and treatment of aPL-related clinical events with emphasis on cardiac manifestations. PMID:19732604

  17. Noonan syndrome

    Directory of Open Access Journals (Sweden)

    van der Burgt Ineke

    2007-01-01

    Full Text Available Abstract Noonan Syndrome (NS is characterised by short stature, typical facial dysmorphology and congenital heart defects. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. The main facial features of NS are hypertelorism with down-slanting palpebral fissures, ptosis and low-set posteriorly rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency and lymphatic dysplasias. The syndrome is transmitted as an autosomal dominant trait. In approximately 50% of cases, the disease is caused by missense mutations in the PTPN11 gene on chromosome 12, resulting in a gain of function of the non-receptor protein tyrosine phosphatase SHP-2 protein. Recently, mutations in the KRAS gene have been identified in a small proportion of patients with NS. A DNA test for mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. NS should be considered in all foetuses with polyhydramnion, pleural effusions, oedema and increased nuchal fluid with a normal karyotype. With special care and counselling, the majority of children with NS will grow up and function normally in the adult world. Management should address feeding problems in early childhood, evaluation of cardiac function and assessment of growth and motor development. Physiotherapy and/or speech therapy should be offered if indicated. A complete eye examination and hearing evaluation should be performed during the first few years of schooling. Preoperative coagulation studies are indicated. Signs and symptoms lessen with age and most adults with NS do not require special medical care.

  18. Clinical spectrum of cryptogenic organising pneumonitis.

    OpenAIRE

    Bellomo, R; Finlay, M.; McLaughlin, P.; Tai, E.

    1991-01-01

    Cryptogenic organising pneumonitis (bronchiolitis obliterans organising pneumonia) is an uncommon condition that often responds to steroids. It is characterised clinically by constitutional symptoms, pathologically by intra-alveolar organising fibrosis, and radiologically by patchy pulmonary infiltrates. Its full clinical spectrum and course are only partially described and understood. Six patients are described, seen over three years, with considerably diverse clinical and radiological prese...

  19. Cryptogenic Organising Pneumonia As The Initial Presenting Manifestation of SLE

    OpenAIRE

    Neena Mampilly; Manoj, G.; Paul, Binoy J.

    2015-01-01

    Cryptogenic Organising Pneumonia (COP), also called idiopathic Bronchiolitis Obliterans Organising Pneumonia( BOOP), is a distinct entity among the idiopathic interstitial pneumonias defined histopathologically by intraalveolar buds of granulation tissue. The etiology includes idiopathic, infectious, drug induced radiation induced and connective tissue diseases. Organising pneumonia occurs particularly in patients with dermatomyositis-polymyositis where it may be the presenting manifestat...

  20. Pneumopathies organisées: à propos de 3 cas

    OpenAIRE

    Nepper, S.; FRUSCH, Nicolas; Louis, Renaud; Ghaye, Benoit; De Leval, Laurence; BOSQUEE, Léon

    2010-01-01

    Infiltrative lung lesions are not always linked to infectious processes or cancers. An interesting entity, the OP (Organizing Pneumonia) or COP (Cryptogenic Organizing Pneumonia)--formerly BOOP (Bronchiolitis Obliterans Organizing Pneumonia)--is discussed through observations repor. ted in this article. We provide some keys to allow the astute observer to target this often curable disease.

  1. Chronic rejection of a lung transplant is characterized by a profile of specific autoantibodies

    Science.gov (United States)

    Hagedorn, Peter H; Burton, Christopher M; Carlsen, Jørn; Steinbrüchel, Daniel; Andersen, Claus B; Sahar, Eli; Domany, Eytan; Cohen, Irun R; Flyvbjerg, Henrik; Iversen, Martin

    2010-01-01

    Obliterative bronchiolitis (OB) continues to be the major limitation to long-term survival after lung transplantation. The specific aetiology and pathogenesis of OB are not well understood. To explore the role of autoreactivity in OB, we spotted 751 different self molecules onto glass slides, and used these antigen microarrays to profile 48 human serum samples for immunoglobulin G (IgG) and IgM autoantibodies; 27 patients showed no or mild bronchiolitis obliterans syndrome (BOS; a clinical correlate of OB) and 15 patients showed medium to severe BOS. We now report that these BOS grades could be differentiated by a profile of autoantibodies binding to 28 proteins or their peptides. The informative autoantibody profile included down-regulation as well as up-regulation of both IgM and IgG specific reactivities. This profile was evaluated for robustness using a panel of six independent test patients. Analysis of the functions of the 28 informative self antigens showed that eight of them are connected in an interaction network involved in apoptosis and protein metabolism. Thus, a profile of autoantibodies may reflect pathological processes in the lung allograft, suggesting a role for autoimmunity in chronic rejection leading to OB. PMID:20201985

  2. Patterns of airway involvement in inflammatory bowel diseases

    Institute of Scientific and Technical Information of China (English)

    Ilias; Papanikolaou; Konstantinos; Kagouridis; Spyros; A; Papiris

    2014-01-01

    Extraintestinal manifestations occur commonly in inflammatory bowel diseases(IBD). Pulmonary manifestations(PM) of IBD may be divided in airway disorders, interstitial lung disorders, serositis, pulmonary vasculitis, necrobiotic nodules, drug-induced lung disease, thromboembolic lung disease and enteropulmonary fistulas. Pulmonary involvement may often be asymptomatic and detected solely on the basis of abnormal screening tests. The common embryonic origin of the intestine and the lungs from the primitive foregut, the co-existence of mucosa associated lymphoid tissue in both organs, autoimmunity, smoking and bacterial translocation from the colon to the lungs may all be involved in the pathogenesis of PM in IBD. PM are mainly detected by pulmonary function tests and highresolution computed tomography. This review will focus on the involvement of the airways in the context of IBD, especially stenoses of the large airways, tracheo-bronchitis, bronchiectasis, bronchitis, mucoid impaction, bronchial granulomas, bronchiolitis, bronchiolitis obliterans syndrome and the co-existence of IBD with asthma, chronic obstructive pulmonary disease, sarcoidosis and a1-antitrypsin deficiency.

  3. Prenatal Tests for Down Syndrome

    Science.gov (United States)

    PRENATAL TESTS FOR DOWN SYNDROME S HARE W ITH W OMEN PRENATAL TESTS FOR DOWN SYNDROME What Is Down Syndrome? ... suggests that you consult your health care provider. PRENATAL TESTS FOR DOWN SYNDROME 256 Volume 50, No. ...

  4. Genetics Home Reference: Rett syndrome

    Science.gov (United States)

    ... Help Me Understand Genetics Home Health Conditions Rett syndrome Rett syndrome Enable Javascript to view the expand/collapse ... autism-dementia-ataxia-loss of purposeful hand use syndrome Rett disorder Rett's disorder Rett's syndrome RTS RTT Related ...

  5. Gilles de la Tourette syndrome

    Science.gov (United States)

    ... cannot control. The condition is commonly called Tourette syndrome. ... Tourette syndrome ... fewer people have more severe forms of Tourette syndrome. Tourette syndrome is four times as likely to occur ...

  6. Nevoid Basal Cell Carcinoma Syndrome

    Science.gov (United States)

    ... Nevoid Basal Cell Carcinoma Syndrome Request Permissions Nevoid Basal Cell Carcinoma Syndrome Approved by the Cancer.Net Editorial Board , 04/2016 What is Nevoid Basal Cell Carcinoma Syndrome? Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is ...

  7. Burning Mouth Syndrome and "Burning Mouth Syndrome".

    Science.gov (United States)

    Rifkind, Jacob Bernard

    2016-03-01

    Burning mouth syndrome is distressing to both the patient and practitioner unable to determine the cause of the patient's symptoms. Burning mouth syndrome is a diagnosis of exclusion, which is used only after nutritional deficiencies, mucosal disease, fungal infections, hormonal disturbances and contact stomatitis have been ruled out. This article will explore the many causes and treatment of patients who present with a chief complaint of "my mouth burns," including symptomatic treatment for those with burning mouth syndrome. PMID:27209717

  8. Mobbing syndrome

    Directory of Open Access Journals (Sweden)

    Sakoula Z.

    2014-07-01

    Full Text Available Introduction: The term mobbing comes from the English word mob, meaning attack, Compass bother. Today is the systematic psychological attack and a strategic marginalization accepted at the workplace from their superiors or colleagues unwanted, for various reasons, employees. The term was used in 1800 by British biology, description of aggressive behavior in flight, certain species of migratory birds. In 1900, ethologist Konrad Lorenz uses it to interpret the hostility of the majority of the herd, compared to lean animals of the same breed. The German psychologist Heinz Leyman, is the first, which is in the 80s, attributes the condition in human society, describing all the negative health effects of mobbing in the workplace as a "syndrome mobbing». Purpose: To work is to illustrate the phenomenon mobbing, which can appear as a problem in the relationship of the perpetrator to the victim, but also implies the presence of such conditions to occur and flourish. Literature Review: searched the literature, internet, Keyword: Work or Employee Abuse, Mistreatment, Emotional Abuse, Bossing, Victimization, Intimidation, Psychological terrorization, Psychological violence. The mobbing syndrome is defined as "repeated abusive behavior, manifested through actions, words, intimidation, acts, gestures, ways of organizing work and have the character or purpose to offend the personality, dignity or physical or mental integrity of the worker in the performance of his work, to jeopardize the employment status or to create a hostile, intimidating, degrading, humiliating or offensive working environment. According to the French psychiatrist Marie France Hirigoyen, the "offender" is a personality that satisfied 'hurting' his fellows and develops self-esteem, conveying to others the "pain" that cannot feel, but also the internal contradictions that refuses edited. Conclusions: the mobbing is the reason for the development of mental and physical diseases as an

  9. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Pınar Zeyneloğlu

    2012-04-01

    Full Text Available Renal failure is a common major complication in patients with advanced cirrhosis and generally indicates a poor prognosis when combined with liver failure. Hepatorenal syndrome (HRS is characterised by a combination of disturbances in circulatory and kidney function. Arterial pressure is decreased in the systemic circulation due to reduced total systemic vascular resistance. Kidney dysfunction is caused by reduction in renal blood flow. The diagnosis of HRS is based on exclusion of other disorders that cause acute kidney injury in cirrhosis as there are no specific tests. There are two types of HRS with different characteristics and prognostics. Liver transplantation is the treatment of choice for all patients without contraindication. The best approach to the pharmacologic management is the administration vasoconstrictor drugs based on the pathogenesis. Many vasoconstrictors including vasopressin analogues (terlipressin, ornipressin and vasopressin, somatostatin analogues (octreotide and alpha-adrenergic analogues (midodrine and norepinephrine have been studied. In most of the studies intravenous albumin therapy was coadministered with vasoconstrictor drugs and suggested that albumin should be considered as the component of pharmacologic intervention in patients with HRS. Renal replacement therapy in the form of hemodialysis or continuous venovenous hemofiltration has been used in the management of HRS patients awaiting transplantation or in those with acute potentially reversible conditions. The artificial hepatic support systems require further investigation. (Journal of the Turkish Society Intensive Care 2012; 10: 37-44

  10. Tourette syndrome.

    Science.gov (United States)

    Cavanna, Andrea E; Termine, Cristiano

    2012-01-01

    Tourette syndrome (TS) is a neurodevelopmental disorder consisting of multiple motor and one or more vocal/phonic tics. TS is increasingly recognized as a common neuropsychiatric disorder usually diagnosed in early childhood and comorbid neuropsychiatric disorders occur in approximately 90% of patients, with attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD) being the most common ones. Moreover, a high prevalence of depression and personality disorders has been reported. Although the mainstream of tic management is represented by pharmacotherapy, different kinds of psychotherapy, along with neurosurgical interventions (especially deep brain stimulation, DBS) play a major role in the treatment of TS. The current diagnostic systems have dictated that TS is a unitary condition. However, recent studies have demonstrated that there may be more than one TS phenotype. In conclusion, it appears that TS probably should no longer be considered merely a motor disorder and, most importantly, that TS is no longer a unitary condition, as it was previously thought. PMID:22411257

  11. Leigh syndrome

    International Nuclear Information System (INIS)

    A male infant developed hypotonia at 5 months, vomiting, diarrhea, fever, generalized clonic convulsion, tonic spasm and periodical opisthotonus at 8 months, swallowing difficulty at 10 months, pes equinovarus and optic atrophy at 11 months, and then tachypnea, and died at 14 months of age. Parents were consanguinous. Laboratory studies revealed elevated serum LDH, CPK, lactate and Pyruvate. TPP-ATP phosphoryl transferase inhibitor was negative in urine. EEG showed irregular and diffuse slow waves and periodic diffuse spike and waves. CT scan at 9 months of age showed slightly low attenuation areas in the putamen bilaterally. At 11 months, a diffuse cerebral atrophy was found, and the low attenuation of the basal ganglia became more definite. No enhanced lesion was seen at 13 months of age. Thiamine tetra-hydrofurfuryl disulfide and lipoic acid were tried without success. The pathological findings of the brain were astrogliosis and proliferation of capillaries in putamen, thalamus, caudate neucleus, substantia nigra, pontine brachium and cerebral cortex, which were symmetrically involved. The symmetrical cavitation was found in putamen. Optic nerve and mamillary body were spared. CT scan findings corresponded well with the pathology of the necrotic lesions of the brain. It was concluded that these CT scan pictures described above may be diagnostic of Leigh syndrome. (author)

  12. Milk-alkali syndrome

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000332.htm Milk-alkali syndrome To use the sharing features on this page, please enable JavaScript. Milk-alkali syndrome is a condition in which there ...

  13. Androgen insensitivity syndrome

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001180.htm Androgen insensitivity syndrome To use the sharing features on this page, please enable JavaScript. Androgen insensitivity syndrome (AIS) is when a person who ...

  14. Diabetic hyperglycemic hyperosmolar syndrome

    Science.gov (United States)

    Diabetic hyperglycemic hyperosmolar syndrome (HHS) is a complication of type 2 diabetes . It involves extremely high blood ... Diabetic hyperglycemic hyperosmolar syndrome is a condition of: Extremely high blood sugar (glucose) level Extreme lack of ...

  15. Chinese restaurant syndrome

    Science.gov (United States)

    Chinese restaurant syndrome is a set of symptoms that some people have after eating Chinese food. A food additive ... Chinese restaurant syndrome is most often diagnosed based on the symptoms. The health care provider may ask the following ...

  16. International Rett Syndrome Foundation

    Science.gov (United States)

    ... Website What’s in Your State? For Families: Find Rett syndrome related resources in your state! State Resources Rettsyndrome.org is the world's leading Rett syndrome research funding organization We have invested $38 million ...

  17. What Causes Rett Syndrome?

    Science.gov (United States)

    ... Information Clinical Trials Resources and Publications What causes Rett syndrome? Skip sharing on social media links Share this: ... as bad for development as too little. Is Rett syndrome passed from one generation to the next? In ...

  18. What Is Marfan Syndrome?

    Science.gov (United States)

    ... 11:11 Size: 10.5 MB November 2014 What Is Marfan Syndrome? Fast Facts: An Easy-to- ... Being Done on Marfan Syndrome? For More Information What Is Connective Tissue? Connective tissue supports many parts ...

  19. Moebius Syndrome Foundation

    Science.gov (United States)

    ... FRAME video on Moebius syndrome The Moebius Syndrome Foundation is excited to announce the premiere of the FRAME video, produced by Rick Guidotti and his non-profit organization, Positive Exposure! FRAME is a web-based ...

  20. Diabetic hyperglycemic hyperosmolar syndrome

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000304.htm Diabetic hyperglycemic hyperosmolar syndrome To use the sharing features on this page, please enable JavaScript. Diabetic hyperglycemic hyperosmolar syndrome (HHS) is a complication of ...

  1. Green Nail Syndrome

    Science.gov (United States)

    ... Favorite Name: Category: Share: Yes No, Keep Private Green Nail Syndrome Share | Green nail syndrome (GNS) is an infection of the ... discoloration of nails, also known as chloronychia. The green discoloration varies from blue-green to dark green ...

  2. Sick sinus syndrome

    Science.gov (United States)

    ... chambers is a common cause of sick sinus syndrome. Coronary artery disease , high blood pressure, and aortic and ... pressure may be normal or low. Sick sinus syndrome may cause symptoms of heart failure to start or get worse. Sick sinus ...

  3. Carpal tunnel syndrome

    Science.gov (United States)

    Median nerve dysfunction; Median nerve entrapment ... Calandruccio JH. Carpal tunnel syndrome, ulnar tunnel syndrome, and stenosing tenosynovitis. In: Canale ST, Beaty JH, eds. Campbell's Operative Orthopaedics . 12th ed. Philadelphia, PA: Elsevier Mosby; 2013: ...

  4. Obesity hypoventilation syndrome (OHS)

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000085.htm Obesity hypoventilation syndrome (OHS) To use the sharing features on this page, please enable JavaScript. Obesity hypoventilation syndrome (OHS) is a condition in some ...

  5. Abdominal Pain Syndrome

    Science.gov (United States)

    ... inspection of a drop of urine), and urine culture for bacterial infection. Stools can be analyzed for ... Hepatitis C Inflammatory Bowel Disease Irritable Bowel Syndrome Obesity Digestive Health Topics Abdominal Pain Syndrome Belching, Bloating, ...

  6. Ovarian hyperstimulation syndrome

    Science.gov (United States)

    Ovarian hyperstimulation syndrome (OHSS) is a problem that is sometimes seen in women who take fertility medicines ... the belly and chest area. This is called ovarian hyperstimulation syndrome (OHSS). OHSS occurs only after the ...

  7. What is Down Syndrome?

    Science.gov (United States)

    ... Syndrome/Down-Syndrome-Facts/ [top] What are common symptoms? » ​​ Last Reviewed: 01/17/2014 Related A-Z Topics Autism Spectrum Disorder (ASD) Early Learning Intellectual and Developmental Disabilities (IDDs) All related ...

  8. Rubinstein-Taybi syndrome

    Science.gov (United States)

    Rubinstein syndrome, RTS ... Rubinstein-Taybi Parents Group USA: www.rubinstein-taybi.org ... Philadelphia, PA: Elsevier; 2016:chap 14. Stevens CA. Rubinstein-Taybi syndrome. Gene Reviews. 2014;8. PMID: 20301699 ...

  9. Munchausen syndrome by proxy

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/001555.htm Munchausen syndrome by proxy To use the sharing features on this page, please enable JavaScript. Munchausen syndrome by proxy is a mental illness and a form of ...

  10. Learning about Klinefelter Syndrome

    Science.gov (United States)

    ... for the genetic terms used on this page Learning About Klinefelter Syndrome What is Klinefelter syndrome? What ... they are referred to a doctor to evaluate learning disabilities. The diagnosis may also be considered in ...

  11. Antiphospholipid Antibody Syndrome

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Is Antiphospholipid Antibody Syndrome? Antiphospholipid (AN-te-fos-fo-LIP-id) antibody ... weeks or months. This condition is called catastrophic antiphospholipid syndrome (CAPS). People who have APS also are at ...

  12. Down Syndrome (For Kids)

    Science.gov (United States)

    ... Dictionary of Medical Words En Español What Other Kids Are Reading Movie: Digestive System Winter Sports: Sledding, ... people who have it. What's Life Like for Kids With Down Syndrome? Many kids with Down syndrome ...

  13. Down Syndrome: Education

    Science.gov (United States)

    ... Kit Financials Newsroom Shop NDSS Home » Resources » Education Education This section includes information about inclusion, elementary and ... and postsecondary options for students with Down syndrome. Education & Down Syndrome This section provides an overview and ...

  14. Hantavirus Pulmonary Syndrome (HPS)

    Science.gov (United States)

    ... this page: About CDC.gov . Hantavirus Share Compartir Hantavirus Pulmonary Syndrome (HPS) Severe HPS. Image courtesy D. ... the workers showed evidence of infection or illness. Hantavirus Pulmonary Syndrome (HPS) Topics Transmission Where HPS is ...

  15. Turner Syndrome (For Parents)

    Science.gov (United States)

    ... special blood test that looks at chromosomes — a karyotype — is used to diagnose Turner syndrome. Several physical ... and prompt him or her to order a karyotype. Results that indicate Turner syndrome show 45 chromosomes ...

  16. Metabolic Syndrome and Migraine

    OpenAIRE

    Sachdev, Amit; Marmura, Michael J.

    2012-01-01

    Migraine and metabolic syndrome are highly prevalent and costly conditions. The two conditions coexist, but it is unclear what relationship may exist between the two processes. Metabolic syndrome involves a number of findings, including insulin resistance, systemic hypertension, obesity, a proinflammatory state, and a prothrombotic state. Only one study addresses migraine in metabolic syndrome, finding significant differences in the presentation of metabolic syndrome in migraineurs. However, ...

  17. The wellness syndrome

    DEFF Research Database (Denmark)

    Mik-Meyer, Nanna

    2015-01-01

    Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.).......Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.)....

  18. PRES syndrome

    International Nuclear Information System (INIS)

    Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological entity characterized by headache, confusion, visual disturbances, seizures and posterior transient changes on neuroimaging. PRES has been described in several conditions including hypertensive encephalopathy, preeclampsia, eclampsia, infections, electrolyte imbalance, hypercalcaemia and use of several drugs. It occurs due to elevated blood pressure which exceeds the autoregulatory capacity of brain vasculature. The posterior circulation supplied by vertibro-basilar system has poor sympathetic innervation and, therefore, is frequently involved. The role of neuroimaging is to establish the initial diagnosis and to exclude other causes of neurological symptoms and signs. NCCT is sufficient to make the diagnosis in a proper clinical setting. MRI features are characteristic and has diagnostic and prognostic value. Diffusion weighted imaging (DWI) can differentiate this condition from ischemia/cytotoxic edema. Differential diagnosis of PRES includes PCA territory infarcts, venous thrombosis, demyelinating disorders, vasculitis and encephalitis. The diagnosis has important implications because the reversibility of the clinico-radiological abnormalities is contingent on the prompt control of blood pressure and/or withdrawing of the offending drug. We describe here a case of PRES in a 12 years old girl with acute lymphoblasts leukaemia, treated with cytostatics-vincristine, pharmorubycin and methotrexate. After 39 days from the beginning of the treatment there are good results in the myelogram and the flowcytometric examination, but the patient made two tonic-clonic seizures. CT and MRI were made and signs of leucoencephalopathy were diagnosed. Several control MRI examinations after cessation of the therapy and disappearance of the neurologic symptoms were made. The normal findings and the clinical course were the reasons for the PRES diagnosis

  19. Brugada syndrome

    Directory of Open Access Journals (Sweden)

    Priori Silvia G

    2006-09-01

    Full Text Available Abstract A novel clinical entity characterized by ST segment elevation in right precordial leads (V1 to V3, incomplete or complete right bundle branch block, and susceptibility to ventricular tachyarrhythmia and sudden cardiac death has been described by Brugada et al. in 1992. This disease is now frequently called "Brugada syndrome" (BrS. The prevalence of BrS in the general population is unknown. The suggested prevalence ranges from 5/1,000 (Caucasians to 14/1,000 (Japanese. Syncope, typically occurring at rest or during sleep (in individuals in their third or fourth decades of life is a common presentation of BrS. In some cases, tachycardia does not terminate spontaneously and it may degenerate into ventricular fibrillation and lead to sudden death. Both sporadic and familial cases have been reported and pedigree analysis suggests an autosomal dominant pattern of inheritance. In approximately 20% of the cases BrS is caused by mutations in the SCN5A gene on chromosome 3p21-23, encoding the cardiac sodium channel, a protein involved in the control of myocardial excitability. Since the use of the implantable cardioverter defibrillator (ICD is the only therapeutic option of proven efficacy for primary and secondary prophylaxis of cardiac arrest, the identification of high-risk subjects is one of the major goals in the clinical decision-making process. Quinidine may be regarded as an adjunctive therapy for patients at higher risk and may reduce the number of cases of ICD shock in patients with multiple recurrences.

  20. The acute radiation syndrome

    International Nuclear Information System (INIS)

    Symptoms and signs from medical aspects resulting from whole body exposure, or in the main part, to ionizing radiation are described. The dose-response relationship is studied and the exposure is divided in three parts: central nervous system syndrome, gastrointestinal syndrome and hematopoietic syndrome. Brief comments about the treatment are reported. (M.A.C.)

  1. CONSTIPATION IN RETT SYNDROME

    Science.gov (United States)

    Gastrointestinal problems occur frequently in girls with Rett syndrome. Constipation is a common problem in girls with Rett syndrome because of their neurological abnormalities. Research studies to better understand the abnormalities of large bowel function in our girls with Rett syndrome have not b...

  2. What Is Usher Syndrome?

    Science.gov (United States)

    ... into electrical impulses that transfer messages to the brain. How is Usher syndrome inherited? Usher syndrome is ... required for the child to be affected. A person with only one copy of the gene is a ... in deafness and deaf-blindness, but are not related to Usher syndrome. ...

  3. Stiff skin syndrome.

    Science.gov (United States)

    Geng, S; Lei, X; Toyohara, J P; Zhan, P; Wang, J; Tan, S

    2006-07-01

    Stiff skin syndrome is a rare disorder characterized by pronounced skin induration, mild hypertrichosis and limited joint mobility, predominantly on the buttocks and thighs. Many heterogeneous cases have been reported under the name of stiff skin syndrome. We present a case of stiff skin syndrome from China, the diagnosis based on the patient's typical clinical and histopathological features. PMID:16836505

  4. Munchausen syndrome by proxy

    Science.gov (United States)

    Munchausen syndrome by proxy is a mental illness and a form of child abuse . The caretaker of ... No one is sure what causes Munchausen syndrome by proxy. Sometimes, the person was abused as a child or has Munchausen syndrome (fake illness for themselves).

  5. Fragile X Syndrome Overview

    Science.gov (United States)

    ... NICHD Research Information Clinical Trials Resources and Publications Fragile X Syndrome: Overview Skip sharing on social media links Share ... menu on the left. ​ Common Name Fragile X syndrome or Fragile X Medical or Scientific Names Martin-Bell syndrome Last ...

  6. Interstitial inflammatory lesions of the pulmonary allograft: a retrospective analysis of 2697 transbronchial biopsies

    DEFF Research Database (Denmark)

    Burton, C.M.; Iversen, M.; Carlsen, J.;

    2008-01-01

    of 2697 biopsies were evaluated corresponding to a mean of 6+/-2 (median 8) completed schedules per patient. Diffuse alveolar damage (DAD) was the second most common histological finding within the first 2 weeks after transplantation. The peak prevalence of bronchiolitis obliterans organizing...... pneumonia (BOOP) and interstitial pneumonitis occurred at 4 to 6 weeks, and 6 to 12 weeks, respectively. There was a steady increase in the cumulative proportion of patients with fibrosis and bronchiolitis obliterans, at each successive scheduled surveillance time point beyond 3 months posttransplantation....... The strongest histological correlations were between ACR and lymphocytic bronchiolitis (OR 5.1, P<0.0001) or interstitial fibrosis (OR 3.2, P<0.0001). Patients with interstitial pneumonitis and pulmonary hemosiderosis were also more likely to demonstrate the finding of interstitial fibrosis (OR 3...

  7. CANDLE syndrome: a recently described autoinflammatory syndrome.

    Science.gov (United States)

    Tüfekçi, Özlem; Bengoa, ŞebnemYilmaz; Karapinar, Tuba Hilkay; Ataseven, Eda Büke; İrken, Gülersu; Ören, Hale

    2015-05-01

    CANDLE syndrome (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) is a recently described autoinflammatory syndrome characterized by early onset, recurrent fever, skin lesions, and multisystemic inflammatory manifestations. Most of the patients have been shown to have mutation in PSMB8 gene. Herein, we report a 2-year-old patient with young onset recurrent fever, atypical facies, widespread skin lesions, generalized lymphadenopathy, hepatosplenomegaly, joint contractures, hypertrglyceridemia, lipodystrophy, and autoimmune hemolytic anemia. Clinical features together with the skin biopsy findings were consistent with the CANDLE syndrome. The pathogenesis and treatment of this syndrome have not been fully understood. Increased awareness of this recently described syndrome may lead to recognition of new cases and better understanding of its pathogenesis which in turn may help for development of an effective treatment. PMID:25036278

  8. Barth syndrome

    Directory of Open Access Journals (Sweden)

    Clarke Sarah LN

    2013-02-01

    Full Text Available Abstract First described in 1983, Barth syndrome (BTHS is widely regarded as a rare X-linked genetic disease characterised by cardiomyopathy (CM, skeletal myopathy, growth delay, neutropenia and increased urinary excretion of 3-methylglutaconic acid (3-MGCA. Fewer than 200 living males are known worldwide, but evidence is accumulating that the disorder is substantially under-diagnosed. Clinical features include variable combinations of the following wide spectrum: dilated cardiomyopathy (DCM, hypertrophic cardiomyopathy (HCM, endocardial fibroelastosis (EFE, left ventricular non-compaction (LVNC, ventricular arrhythmia, sudden cardiac death, prolonged QTc interval, delayed motor milestones, proximal myopathy, lethargy and fatigue, neutropenia (absent to severe; persistent, intermittent or perfectly cyclical, compensatory monocytosis, recurrent bacterial infection, hypoglycaemia, lactic acidosis, growth and pubertal delay, feeding problems, failure to thrive, episodic diarrhoea, characteristic facies, and X-linked family history. Historically regarded as a cardiac disease, BTHS is now considered a multi-system disorder which may be first seen by many different specialists or generalists. Phenotypic breadth and variability present a major challenge to the diagnostician: some children with BTHS have never been neutropenic, whereas others lack increased 3-MGCA and a minority has occult or absent CM. Furthermore, BTHS was first described in 2010 as an unrecognised cause of fetal death. Disabling mutations or deletions of the tafazzin (TAZ gene, located at Xq28, cause the disorder by reducing remodeling of cardiolipin, a principal phospholipid of the inner mitochondrial membrane. A definitive biochemical test, based on detecting abnormal ratios of different cardiolipin species, was first described in 2008. Key areas of differential diagnosis include metabolic and viral cardiomyopathies, mitochondrial diseases, and many causes of neutropenia and

  9. Hypereosinophilic syndromes

    Directory of Open Access Journals (Sweden)

    Goldman Michel

    2007-09-01

    Full Text Available Abstract Hypereosinophilic syndromes (HES constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia (> 1.5 × 109/L for more than six consecutive months associated with evidence of eosinophil-induced organ damage, where other causes of hypereosinophilia such as allergic, parasitic, and malignant disorders have been excluded. Prevalence is unknown. HES occur most frequently in young to middle-aged patients, but may concern any age group. Male predominance (4–9:1 ratio has been reported in historic series but this is likely to reflect the quasi-exclusive male distribution of a sporadic hematopoietic stem cell mutation found in a recently characterized disease variant. Target-organ damage mediated by eosinophils is highly variable among patients, with involvement of skin, heart, lungs, and central and peripheral nervous systems in more than 50% of cases. Other frequently observed complications include hepato- and/or splenomegaly, eosinophilic gastroenteritis, and coagulation disorders. Recent advances in underlying pathogenesis have established that hypereosinophilia may be due either to primitive involvement of myeloid cells, essentially due to occurrence of an interstitial chromosomal deletion on 4q12 leading to creation of the FIP1L1-PDGFRA fusion gene (F/P+ variant, or to increased interleukin (IL-5 production by a clonally expanded T cell population (lymphocytic variant, most frequently characterized by a CD3-CD4+ phenotype. Diagnosis of HES relies on observation of persistent and marked hypereosinophilia responsible for target-organ damage, and exclusion of underlying causes of hypereosinophilia, including allergic and parasitic disorders, solid and hematological malignancies, Churg-Strauss disease, and HTLV infection. Once these criteria are fulfilled, further testing for eventual pathogenic classification is warranted using appropriate cytogenetic and functional approaches. Therapeutic

  10. Poland-Möbius syndrome.

    OpenAIRE

    Parker, D. L.; Mitchell, P. R.; Holmes, G. L.

    1981-01-01

    A patient with stigmata of both the Möbius syndrome and the Poland syndrome is presented. This is now the twelfth well-documented patient with a combination of the two syndromes. The association of the Poland syndrome and the Möbius syndrome occurs with sufficient frequency that the combination probably represents a formal genesis malformation syndrome of unknown aetiology that should be designated the Poland-Möbius syndrome.

  11. Analysis of correlation factors of the development from bronchiolitis to bronchial asthma%毛细支气管炎发展为支气管哮喘的相关因素分析

    Institute of Scientific and Technical Information of China (English)

    刘雄诚; 李琴丽

    2016-01-01

    目的:探讨毛细支气管炎发展为支气管哮喘(简称“哮喘”)的影响因素。方法对2012年12月至2015年6月,峨眉山市人民医院收治的150例毛细支气管炎,并随访2年的患儿的临床病历资料进行回顾性分析。对性别、饲养宠物、哮喘家族史、肥胖、湿疹史、母乳喂养时间、按时接种卡介苗(BCG)、父亲吸烟情况、过敏体质、疾病严重程度及应用免疫球蛋白(Ig)治疗11项因素影响下毛细支气管炎患儿的哮喘发生率差异进行比较;采用非条件多因素 logistic 回归分析,探讨毛细支气管炎发展为哮喘的影响因素。本研究遵循的程序符合峨眉山市人民医院人体试验委员会制定的伦理学标准,得到该委员会批准,征得受试对象监护人知情同意,并与监护人签署临床研究知情同意书。结果①不同性别及家中是否饲养宠物患儿的哮喘发生率比较,差异无统计学意义(P >0.05);有哮喘家族史、肥胖、有湿疹史、母乳喂养≤4个月、未按时接种 BCG、被动吸烟、过敏体质、重型毛细支气管炎和未应用 Ig 治疗患儿的哮喘发生率(45.2%、41.7%、42.6%、40.8%、37.5%、94.7%、57.9%、48.8%、37.3%)较无哮喘家族史、无肥胖、无湿疹史、母乳喂养>4个月、按时接种 BCG、未被动吸烟、无过敏体质、轻型毛细支气管炎和应用 Ig 治疗患儿(19.4%、21.9%、17.7%、19.8%、22.7%、3.6%、16.1%、18.3%、4.2%)高,且差异均有统计学意义(χ2=7.96、5.37、8.47、5.02、3.84、18.48、10.27、9.65、13.27,P 4个月(OR =0.472,95% CI :1.926~15.687,P 0.05).The incidence rates of asthma in children with family history of asthma,obesity,history of eczema,breast feeding ≤ 4 months,took BCG vaccination not on time, second-hand smoking,allergic constitution,severe type of bronchiolitis,and non-applied Ig treatment (45.2%,41.7%,42.6%,40.8%,37.5%,94.7%,57.9%,48.8%,37.3%)were higher than incidence rates of asthma

  12. [Tic syndrome].

    Science.gov (United States)

    Czapliński, Adam; Steck, Andreas J; Fuhr, Peter

    2002-01-01

    A tic is an involuntary, sudden, rapid, recurrent, nonrrhythmic, stereotyped, motor movement or vocalization. This paper reviews clinical, pathophysiological, epidemiological and treatment issues of tic disorders. The clinical presentation of tic disorders with simple and complex motor or vocal tics is reviewed in detail. The most common psychiatric comorbid conditions, such as personality disorder (PD), Obsessive-Compulsive Disorder (OCD), Self-Destructive Behavior (SDB) and Attention Deficit Hyperactivity Disorder (ADHD) are presented too. All forms of tics may be exacerbated by anger or stress, but they are usually markedly diminished during sleep. Premonitory feelings or "sensory experiences", which are distinct from the actual motor or phonic tics and precede the tics, occur in over 80% of tic-patients and in 95% of patients with Gilles de la Tourette Syndrome (GTS). The American Psychiatric Association recognizes three types of tic disorders on the basis of clinical criteria: Transient Tic Disorder, Chronic Motor or Vocal Tic Disorder and GTS. The diagnostic criteria for these types are described. According to epidemiological data, up to 10% of children have at least somewhere a transient tic disorder. The onset of tics, whether simple or multiple, occurs at approximately 7 years of age. The accepted prevalence figure for GTS is 0.05-3%. Although tics can appear as the result of brain injury, Huntington chorea or encephalitis, they are most commonly idiopathic. Genetic factors appear to be present in many but not in all cases of tic disorders. Autosomal dominant, sex-linked models or semirecessive-semidominant-oligogenic models have been considered. Based on the review of the literature we believe that tic disorders are related to altered neurotransmitter function within the CNS, especially that the functional abnormality is somehow related to dopaminergic mechanism. Several authors have recently investigated the possible role of autoimmune response to

  13. Nevoid basal cell carcinoma syndrome (Gorlin syndrome)

    OpenAIRE

    Lo Muzio Lorenzo

    2008-01-01

    Abstract Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The estimated prevalence varies from 1/57,000 to 1/256,000, with a male-to-female ratio of 1:1. Main clinical manifestations include multiple basal cell carcinomas (BCCs), odontogenic keratocysts of the jaws, hyperkeratosis of palms and soles, skeletal abnormalities, intracranial ectopic ...

  14. Metabolic syndrome and migraine

    Directory of Open Access Journals (Sweden)

    Amit eSachdev

    2012-11-01

    Full Text Available Migraine and metabolic syndrome are highly prevaleirnt and costly conditions.The two conditions coexist, but it is unclear what relationship may exist between the two processes. Metabolic syndrome involves a number of findings, including insulin resistance, systemic hypertension, obesity, a proinflammatory state, and a prothrombotic state. Only one study addresses migraine in metabolic syndrome, finding significant differences in the presentation of metabolic syndrome in migraineurs. However, controversy exists regarding the contribution of each individual risk factor to migraine pathogensis and prevalence. It is unclear what treatment implications, if any, exist as a result of the concomitant diagnosis of migraine and metabolic syndrome. The cornerstone of migraine and metabolic syndrome treatments is prevention, relying heavily on diet modification, sleep hygiene, medication use, and exercise.

  15. Dialysis disequilibrium syndrome

    OpenAIRE

    Harris, C. P.; Townsend, J J

    2012-01-01

    The dialysis disequilibrium syndrome is a rare but serious complication of hemodialysis. Despite the fact that maintenance hemodialysis has been a routine procedure for over 50 years, this syndrome remains poorly understood. The signs and symptoms vary widely from restlessness and headache to coma and death. While cerebral edema and increased intracranial pressure are the primary contributing factors to this syndrome and are the target of therapy, the precise mechanisms for their development ...

  16. Gambaran Radiografi Crouzon Syndrome

    OpenAIRE

    Chairani

    2010-01-01

    Crouzon’s syndrome merupakan penyakit autosomal dominan yang disebabkan oleh mutasi gen pertumbuhan FGFR 2 (Fibroblast Growth Factor Receptor 2) kromosom 10, kepala tidak berkembang dengan sempurna. Insiden crouzon syndrome berkisar antara 1: 25000 sampai 1: 60000 kelahiran. Secara klinis mempunyai kepala yang pendek dan lebar, atau sekitar 30% penderita crouzon’s syndrome mengalami hydrocephalus. Manifestasi penyakit ini di rongga mulut antara lain: protrusi mandibula, gigi berjejal pad...

  17. Rubinsten Taybi Syndrome

    OpenAIRE

    J. Jannati

    2008-01-01

    Rubinstein Taybi syndrome or Broad Thumb and Hallux syndrome is a genetic multisystem disorder with unknown mode of inheritance. "nThis syndrome characterized by Broad terminal phalange of the thumbs and /or hallucess broad terminal phalanges of other fingers, characteristic facies (small head, beaked nose, hypertelorism, antimongoloid slant of the palpebral fissures, strabismus, high arch palate, abnormalities of ears, mental and motor retardation."nRadiologic manifestations are sh...

  18. [Paraneoplastic syndromes: a review].

    Science.gov (United States)

    Berardi, R; Grilli, G; Romagnoli, E; Saladino, T; Freddari, F; Tamburrano, T; Galizia, E; Carbonari, G; Mariani, C; Braconi, C; Pierantoni, C; Battelli, N; Scartozzi, M; Cascinu, S

    2005-01-01

    Modern oncology often obtains good results against earlier neoplasms, whilst it's still in difficulties against the advanced ones. The knowledge of paraneoplastic syndromes is crucial both to cure patients and to do an earlier diagnosis. When we recognize a paraneoplastic syndrome that comes before the clinic beginning of a neoplasm, perhaps we save a life. This review discusses all the main paraneoplastic syndromes, focusing mainly on their clinical aspect and reminding the most commonly associated cancers. PMID:16463565

  19. Cannabinoid Hyperemesis Syndrome

    OpenAIRE

    Galli, Jonathan A.; Sawaya, Ronald Andari; Friedenberg, Frank K.

    2011-01-01

    Coinciding with the increasing rates of cannabis abuse has been the recognition of a new clinical condition known as Cannabinoid Hyperemesis Syndrome. Cannabinoid Hyperemesis Syndrome is characterized by chronic cannabis use, cyclic episodes of nausea and vomiting, and frequent hot bathing. Cannabinoid Hyperemesis Syndrome occurs by an unknown mechanism. Despite the well-established anti-emetic properties of marijuana, there is increasing evidence of its paradoxical effects on the gastrointes...

  20. Introduction: Williams Syndrome

    OpenAIRE

    Morris, Colleen A.

    2010-01-01

    In the nearly 50 years since the description of Williams syndrome by Williams et al. in 1961, the focus of scientific inquiry has shifted from identification, definition, and description of the syndrome in small series to genotype-phenotype correlation, pathophysiologic investigation in both humans and in animal models, and therapeutic outcomes in large cohorts. Study of this rare syndrome has provided insight into the structure and function of the extracellular matrix, has contributed to und...

  1. Understanding Brugada syndrome.

    Science.gov (United States)

    Gehshan, Janine Mary; Rizzolo, Denise

    2015-06-01

    Brugada syndrome is an established cause of sudden cardiac arrest in patients without structural cardiac abnormalities. Recognition and diagnosis of this syndrome has been slowly increasing. Syncope, ventricular dysrhythmia, or sudden cardiac arrest may be the presenting symptom, although detection of the characteristic right precordial ST-segment elevation on ECG can be a potentially lifesaving intervention. This article reviews the clinical presentation, pathophysiology, genetics, and current management of Brugada syndrome. PMID:25932713

  2. Coeliac artery compression syndrome

    OpenAIRE

    OKTAY, Özgür; MEMİŞ, Ahmet; Parildar, Mustafa; Oran, İsmail

    2003-01-01

    Celiac artery compression syndrome, also called median arcuate ligament compression syndrome, causes gastrointestinal ischemia secondary to compression of the proximal portion of the celiac artery just beyond its origin by the median arcuate ligament of the diaphragm. This syndrome is frequently demonstrated on aortography performed in patients without complaints of intestinal angina. Isolated stenosis or even occlusion of the celiac artery is always compensated for by collateral circul...

  3. Genetics Home Reference: Waardenburg syndrome

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions Waardenburg syndrome Waardenburg syndrome Enable Javascript to view the expand/collapse boxes. Download PDF Open All Close All Description Waardenburg syndrome is a group of genetic conditions that can ...

  4. Genetics Home Reference: Rotor syndrome

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions Rotor syndrome Rotor syndrome Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Rotor syndrome is a relatively mild condition characterized by ...

  5. Genetics Home Reference: Joubert syndrome

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions Joubert syndrome Joubert syndrome Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Joubert syndrome is a disorder that affects many parts ...

  6. Genetics Home Reference: Turner syndrome

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions Turner syndrome Turner syndrome Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Turner syndrome is a chromosomal condition that affects development in ...

  7. Hemolytic Uremic Syndrome in Children

    Science.gov (United States)

    ... KB)​​​​​ Alternate Language URL Hemolytic Uremic Syndrome in Children Page Content On this page: What is hemolytic ... spine. [ Top ] What causes hemolytic uremic syndrome in children? The most common cause of hemolytic uremic syndrome ...

  8. First Trimester Down Syndrome Screen

    Science.gov (United States)

    ... Home Visit Global Sites Search Help? First Trimester Down Syndrome Screen Share this page: Was this page helpful? ... is carrying has a chromosomal abnormality such as Down syndrome (trisomy 21) or Edwards syndrome (trisomy 18) . The ...

  9. Features of Fragile X Syndrome

    Science.gov (United States)

    ... Figuring Out CGG Repeats! Donate | Print Fragile X Syndrome Fragile X syndrome (FXS) is a genetic condition that causes ... health concerns associated with the condition. Features of Fragile X Syndrome in Males Read our Story The majority of ...

  10. Genetics Home Reference: Arts syndrome

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions Arts syndrome Arts syndrome Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Arts syndrome is a disorder that causes serious neurological ...

  11. Posterior Reversible Encephalopathy Syndrome

    OpenAIRE

    J Gordon Millichap

    2013-01-01

    Investigators at Children's Hospital of Montefiore, Albert Einstein College of Medicine, NY, determined the incidence of posterior reversible encephalopathy syndrome (PRES) in a pediatric critical care unit.

  12. Neonatal abstinence syndrome

    Science.gov (United States)

    ... JR, Isemann B, Ward LP, et al. Current management of neonatal abstinence syndrome secondary to ... MD, MSc, IBCLC, Associate Professor of Pediatrics, Division of Neonatology, Medical University of ...

  13. Short Bowel Syndrome

    Science.gov (United States)

    ... may include nutritional support medications surgery intestinal transplant Nutritional Support The main treatment for short bowel syndrome is nutritional support, which may include the following: Oral rehydration. Adults ...

  14. Organic brain syndrome

    Science.gov (United States)

    OBS; Organic mental disorder (OMS); Chronic organic brain syndrome ... Listed below are disorders associated with OBS. Brain injury caused by ... the brain ( subarachnoid hemorrhage ) Blood clot inside the ...

  15. Do you know this syndrome? *

    OpenAIRE

    Rosmaninho, A.; Pinto-Almeida, T.; Fernandes, I; Machado, S; Selores, M.

    2013-01-01

    Noonan Syndrome is one of the most common genetic syndromes and also an important differential diagnosis in children presenting with syndromic facies similar to Turner's syndrome phenotype. This syndrome is characterized by facial dysmorphism, congenital heart defects, short stature and also a wide phenotypic variation. This article discusses the case of a 10 year-old patient with Noonan syndrome that presented typical facies, cardiac defects (pulmonary dilatation and mitral regurgitation), d...

  16. Metabolic syndrome in acute coronary syndrome

    International Nuclear Information System (INIS)

    Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

  17. Accumulation and significance of MDSCs, IL-10 and IL-12 in the peripheral blood of children with asthma or bronchiolitis%哮喘、毛细支气管炎患儿外周血MDSCs、IL-10和IL-12水平及意义

    Institute of Scientific and Technical Information of China (English)

    张艳丽; 王秀芳; 雷瑞瑞; 栾斌; 刘莹; 宋丽

    2013-01-01

    目的 探讨哮喘、毛细支气管炎(简称毛支)患儿髓系抑制细胞(MDSCs)占外周单个核细胞比例、血清白介素-10(IL-10)及白介素-12( IL-12)的水平及意义.方法 以正常健康儿童及肺炎组为对照,应用流式细胞仪检测哮喘急性发作组、缓解组及毛支Ⅰ组(特应质组)、毛支Ⅱ组(非特应质组)外周血MDSCs占外周单个核细胞的比例(MDSCs%);用ELISA法检测各组儿童血清IL-10、IL-12水平.结果 ①哮喘发作组较哮喘缓解组、肺炎组及正常对照组外周血MDSCs%、血清IL-10升高,IL-12降低(P0.05);④相关性分析:哮喘发作组及毛支Ⅰ组MDSCs占外周血单核细胞的比例和血清IL-10水平存在正相关,与血清IL-12水平呈负相关.结论 MDSCs可能通过上调IL-10、下调IL-12水平在毛细支气管炎发展为哮喘的过程中起重要作用,有望成为毛支筛选为哮喘的指标.%Objective To investigate the accumulation of myeloid-derived suppressor cells (MDSCs), interleukin-10 (IL-10) and interleukin-12 (IL-12) in children with asthma or bronchiolitis and understand their possible significance. Methods We performed flow cytometry to analyze the accumulation of MDSCs in peripheral blood mononuclear cells of children with attack asthma, children with alleviated asthma, bronchiolitis group Ⅰ (atopic group), bronchiolitis group Ⅱ (non-atopic group), as well as children with pneumonia and normal healthy children who served as controls. The accumulation of IL-10 and IL-12 in serum of different groups was analyzed by ELISA. Results ① The accumulation of MDSCs and serum IL-10 in children with attack asthma was significantly higher than that in children with alleviated asthma, children with pneumonia and healthy control group (P0.05). ④Correlation analysis: The accumulation of MDSCs in children with attack asthma and bronchiolitis group Ⅰ was positively correlated with the accumulation of IL-10, but negatively correlated with the

  18. Clinical Observation of Montelukast Sodium Assistant Treatment for Syncytial Virus Bronchiolitis%孟鲁司特钠辅助治疗合胞病毒毛细支气管炎的临床观察

    Institute of Scientific and Technical Information of China (English)

    陈进庆

    2015-01-01

    OBJECTIVE:To observe therapeutic efficacy and safety of montelukast sodium assistant treatment for respiratory syncytial virus(RSV)bronchiolitis. METHODS:76 RSV bronchiolitis neonatus were included in this study and randomly divided into control group(37 cases)and observation group(39 cases). Control group was treated with regular treatment,oxygen inhala-tion,sedation,abatement of fever,cough suppression,eliminating phlegm,Budesonide suspension,aerosol inhalation of Albuter-ol solution;observation group was additionally given montelukast 4 mg,qd,on the basis of control group. The clinical efficacy, cough,suffocative and wheezing rale disappearance time,length of stay were compared between 2 groups. The serum level of Cys-teinyl leukotrienes (CysLTs) before and after treatment and ADR were also compared between 2 groups. RESULTS:After treat-ment,total clinical efficacy of observation group(94.87%)was significantly higher than that of control group(78.38%),with sta-tistical significance(P<0.05);the time of couth,suffocative,wheezing rale disappearance and length of stay in observation group were significantly shorter than those of control group,with statistical significance (P<0.05);after treatment,the serum level of CysLTs in 2 groups were both significantly lower than before,the observation group was lower than the control group,with statisti-cal significance(P<0.05). No obvious ADR was found in 2 group during treatment. CONCLUSIONS:Montelukast sodium assis-tant treatment for RSV bronchiolitis is effective with less ADR.%目的:观察孟鲁司特钠辅助治疗呼吸道合胞病毒(RSV)毛细支气管炎的疗效及安全性。方法:选取RSV毛细支气管炎患儿76例,按随机数字表法分为对照组(37例)与观察组(39例)。对照组患儿采用吸氧、镇静、退热、止咳祛痰及布地奈德混悬液、沙丁胺醇溶液雾化吸入等常规治疗,观察组患儿在对照组基础上加用孟鲁司特钠4 mg,qd。观察两

  19. Syndrome in question*

    Science.gov (United States)

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

  20. Syndrome in Question.

    Science.gov (United States)

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

  1. Syndrome in question*

    OpenAIRE

    Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

    2015-01-01

    Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness.

  2. Chediak-Higashi syndrome.

    Science.gov (United States)

    Kumar, P; Rao, K S; Shashikala, P; Chandrashekar, H R; Banapurmath, C R

    2000-08-01

    A case of Chediak-Higashi syndrome is reported in a four-year-old boy who presented with recurrent chest infection, partial albinism, hyperpigmentation of the extremities and presence of giant granules in leucocytes and melanocytes in the skin. Parental consanguinity was present. Though uncommon, hyperpigmentation of sun exposed areas may be the initial symptom in Chediak-Higashi syndrome. PMID:10985003

  3. Kleine Levin Syndrome

    Directory of Open Access Journals (Sweden)

    Abdul Wahid Khan, Zia Ud Din, Abdul Salam

    2002-07-01

    Full Text Available A case of Kleine Levin Syndrome is presented. Episodic course with spontaneous remission of eachepisode and characteristic features of hypersomnia, hyperphagia, disinhibited behavior. affective featureslike ilTitability and cognitive disturbance made the diagnosis of Kleine Levin syndrome 111 ourpatient.

  4. Poland's syndrome: radiologic findings

    International Nuclear Information System (INIS)

    Poland's syndrome is a rare non-inherited congenital anomaly. The authors describe the classic radiologic findings of Poland's syndrome by reporting the case of a male four-year old patient with asymmetry of hands and chest, illustrating the fundamental imaging criteria for a conclusive diagnosis. (author)

  5. Polycystic ovarian syndrome

    OpenAIRE

    Nina Madnani; Kaleem Khan; Phulrenu Chauhan; Girish Parmar

    2013-01-01

    Polycystic ovarian syndrome (PCOS) is a "multispeciality" disorder suspected in patients with irregular menses and clinical signs of hyperandrogenism such as acne, seborrhoea, hirsutism, irregular menses, infertility, and alopecia. Recently, PCOS has been associated with the metabolic syndrome. Patients may develop obesity, insulin resistance, acanthosis nigricans, Type 2 diabetes, dyslipidemias, hypertension, non-alcoholic liver disease, and obstructive sleep apnoea. Good clinical examinatio...

  6. Klippel-Feil Syndrome

    Science.gov (United States)

    ... such as Klippel-Feil Syndrome and open promising new avenues for treatment. NIH Patient Recruitment for Klippel-Feil Syndrome Clinical Trials At NIH Clinical Center Throughout the U.S. and Worldwide NINDS Clinical Trials Organizations Column1 Column2 March of Dimes 1275 Mamaroneck Avenue ...

  7. Apert Syndrome. Case Report

    Directory of Open Access Journals (Sweden)

    Ninecta Pérez Breña

    2010-08-01

    Full Text Available The case of a white female aged 7 is evaluated in the Primary Care Service of the Barrio Adentro medical mission in Nueva Esparta state, Republic of Venezuela. After a clinical and radiological evaluation she is diagnosed with a genetic syndrome known as Apert Syndrome.

  8. MECP2 Duplication Syndrome

    DEFF Research Database (Denmark)

    Signorini, Cinzia; De Felice, Claudio; Leoncini, Silvia;

    2016-01-01

    Rett syndrome (RTT) and MECP2 duplication syndrome (MDS) are neurodevelopmental disorders caused by alterations in the methyl-CpG binding protein 2 (MECP2) gene expression. A relationship between MECP2 loss-of-function mutations and oxidative stress has been previously documented in RTT patients...

  9. Middle East Respiratory Syndrome

    Centers for Disease Control (CDC) Podcasts

    2014-07-07

    This podcast discusses Middle East Respiratory Syndrome, or MERS, a viral respiratory illness caused by Middle East Respiratory Syndrome Coronavirus—MERS-CoV.  Created: 7/7/2014 by National Center for Immunization and Respiratory Diseases (NCIRD).   Date Released: 7/7/2014.

  10. Trigeminalt trofisk syndrom--

    DEFF Research Database (Denmark)

    Kjaerskov, Mette Wanscher; Bygum, Anette

    2009-01-01

    Trigeminal trophic syndrome (TTS) is a rare but well-described syndrome consisting of the triad: paraesthesia, anaesthesia and crescent-shaped ulceration of the ala nasi. We report a case of a 62-year-old woman presenting with TTS after operative excision of an acusticus neurinoma. She attended s...

  11. Yellow nail syndrome

    Directory of Open Access Journals (Sweden)

    Dixit Ramakant

    2007-01-01

    Full Text Available A case of yellow nail syndrome is described in a forty year old male patient who presented with classical triad of this syndrome i.e. deformed yellow nails, lymph-edema and chronic recurrent pleural effusion. The practical problems in the di-agnosis are also briefly discussed with emphasis on awareness of this rare clinical entity.

  12. Bronchiectasis and Marfan's syndrome.

    OpenAIRE

    Foster, M E; Foster, D R

    1980-01-01

    Marfan's syndrome is a rare hereditary disorder characterized by skeletal, cardiovascular and ocular abnormalities. Pulmonary abnormalities occur in approximately 10% of patients the commonest being spontaneous pneumothorax and emphysema. A patient is described who had Marfan's syndrome and bronchiectasis, an association only described on 2 previous occasions in the literature.

  13. Restless Legs Syndrome

    Science.gov (United States)

    ... Us FAQs Home » Health Information for the Public » Health Topics » Restless Legs Syndrome Explore Restless Legs Syndrome What Is... Causes Who Is at Risk Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics Insomnia Sleep Deprivation and Deficiency Sleep Studies Send a ...

  14. Metabolic syndrome and menopause

    Directory of Open Access Journals (Sweden)

    Jouyandeh Zahra

    2013-01-01

    Full Text Available Abstract Background The metabolic syndrome is defined as an assemblage of risk factors for cardiovascular diseases, and menopause is associated with an increase in metabolic syndrome prevalence. The aim of this study was to assess the prevalence of metabolic syndrome and its components among postmenopausal women in Tehran, Iran. Methods In this cross-sectional study in menopause clinic in Tehran, 118 postmenopausal women were investigated. We used the adult treatment panel 3 (ATP3 criteria to classify subjects as having metabolic syndrome. Results Total prevalence of metabolic syndrome among our subjects was 30.1%. Waist circumference, HDL-cholesterol, fasting blood glucose, diastolic blood pressure ,Systolic blood pressure, and triglyceride were significantly higher among women with metabolic syndrome (P-value Conclusions Our study shows that postmenopausal status is associated with an increased risk of metabolic syndrome. Therefore, to prevent cardiovascular disease there is a need to evaluate metabolic syndrome and its components from the time of the menopause.

  15. Rothmund - Thomson Syndrome

    Directory of Open Access Journals (Sweden)

    Sharma N. L

    2003-01-01

    Full Text Available Rothmund-Thomson syndrome is a rare geno-photodermatosis of children. Poikilodermatous cutaneous changes, growth retardation, juvenile cataract and high incidence of malignancy are its classical features. A Thomson type of Rothmund-Thomson syndrome with characteristic poikiloderma congenitale, growth retardation, absence of juvenile cataract and parental non-consanguinity is described in an 8 year old Indian girl.

  16. [Frey syndrome in childhood].

    Science.gov (United States)

    Clarós, P; González-Enseñat, M A; Arimany, J; Vincente, M A; Clarós, A

    1993-01-01

    Frey's syndrome is distinguished by the appearing of erythema, sensation of hotness, sometimes pain, and transpiration discharge in the preauricular and temporal area when ingestion stars. We present an eleven month old child with this pathology and we review the etiology and clinic manifestations of this syndrome. PMID:8129975

  17. Adult onset Leigh syndrome

    Directory of Open Access Journals (Sweden)

    Pandit Lekha

    2007-01-01

    Full Text Available Leigh syndrome is a rare progressive mitochondrial disorder of oxidative metabolism. Though it has been reported in infancy and childhood, it is rarely described in adults. The authors describe a patient who had clinical and magnetic resonance imaging features diagnostic of Leigh syndrome, with supportive biochemical and muscle histochemistry evidence.

  18. Kleine-Levin Syndrome

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2005-12-01

    Full Text Available A systematic review of all published cases of Kleine-Levin syndrome (KLS described in 195 articles since 1962 is presented from Stanford University Center for Narcolepsy, Palo Alto, CA; the Kleine-Levin Syndrome Foundation, Boston, MA; and Hopital Pitie-Salpetriere, Paris, France.

  19. Fragile X syndrome.

    OpenAIRE

    Wiebe, E.; A. Wiebe

    1994-01-01

    Fragile X syndrome is the most common form of inherited mental retardation. Only recently has it been possible to detect all carriers and transmitters. We review the syndrome and discuss the pedigree of a large fragile X family. Family doctors should identify cases in their practices so genetic counseling can be offered to the families.

  20. Lemierre's Syndrome Mimicking Leptospirosis

    OpenAIRE

    Lin, Dagmar; Suwantarat, Nuntra; Young, Royden S.

    2010-01-01

    Lemierre's syndrome is a suppurative thrombophlebitis involving the internal jugular vein, most commonly associated with Fusobacterium necrophorum, usually a complication of oropharyngeal infections. This syndrome is rare and is often overlooked. We present a case of sepsis mimicking initially severe leptospirosis (Weil's disease) due to acute febrile illness with multiorgan failure and hyperbilirubinemia. Finally, blood cultures revealed Fusobacterium necrophorum and computed tomography (CT)...