INTRODUCTION: Antisynthetase Syndrome is associated with interstitial lung disease in adult patients, but this has not been described in children. MATERIALS AND METHODS: A 13-year-old with interstitial lung disease due to Antisynthetase Syndrome and pulmonary arterial hypertension underwent emergent bilateral lung transplantation after a rapid clinical decline. CONCLUSION: We present ...

Bilateral lung transplantation was performed in a 52-year old man with end-stage Kartagener's syndrome. A postimplantation right lower lobectomy was required for volume reduction and dextrocardia. A bronchial fistula developed with an intractable colonized residual pleural cavity. Closure was obtained successfully with multiple-stage procedures including decortication, muscle flap and an ...

STUDY QUESTION: What are the anatomic variants (and their frequencies) of double uterus, obstructed hemivagina and ipsilateral renal agenesis? SUMMARY ANSWER: Most cases examined (72.4%) were of the classic anatomic variant of the Herlyn-Werner-Wunderlich syndrome (with didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis) but the 27.6% of cases are ...

Meconium aspiration syndrome (MAS) is a major cause of respiratory insufficiency in the term and post-term newborns. There are several pathomechanisms participating in this disorder, particularly the airway obstruction, surfactant dysfunction, inflammation, lung edema, pulmonary vasoconstriction and bronchoconstriction. Inflammatory changes resulting from meconium aspiration cause severe impairment of lung parenchyma and surfactant, ...

Asymmetric obstructed uterus didelphys (Herlyn-Werner-Wunderlich syndrome), also known as obstructed hemivagina with ipsilateral renal agenesis syndrome, is a rare congenital müllerian duct anomaly. Herein we present a case report of incomplete Herlyn-Werner-Wunderlich syndrome, with absence of the hemivaginal septum, diagnosed in a 12-year-old girl. Treatment of the severe pain using ...

A forty-nine-year-old female patient with pulmonary tuberculosis developed syndrome of inadequate antidiuretic hormone secretion. Consequent restriction of fluid intake as a therapeutic measure was just as ineffective as a medication with tolvaptan which was performed later on. A probable explanation for the inefficacy of the aquaretic drug is an interaction ...

Patients with antiphospholipid syndrome may develop various lung manifestations. The lung complications that have been described so far are pulmonary thromboembolic disease, pulmonary hypertension, acute respiratory distress syndrome, primary thrombosis of large and small lung vessels, diffuse alveolar haemorrhage, fibrosing alveolitis and postpartum syndrome. Clinicians should be aware of these ...

Dysautonomia such as POTS syndrome presenting with respiratory symptoms can often be misdiagnosed for other common pulmonary conditions. It can be diagnosed with a comprehensive history and orthostatic vital measurement. Simple diagnostic test such as diffusing capacity in supine and standing position can emerge as a noninvasive tool to guide ...

Abstract Type 2 congenital pulmonary airway malformation (CPAM) has been reported in association with many other congenital anomalies. To the best of our knowledge, however, an association of type 2 CPAM with congenital nephrotic syndrome has not been heretofore reported. We present the first report of such an association in ...

Bronchiolitis obliterans syndrome is characterized by fibrotic obliteration of small airways which severely impairs graft function and survival after lung transplantation. Bronchial epithelial cells from the transplanted lung can undergo epithelial to mesenchymal transition and this can be accentuated by activated macrophages. Macrophages demonstrate significant plasticity and change phenotype in ...

Postpneumonectomy syndrome is a rare condition that is characterized by dyspnea resulting from an extreme mediastinal shift and bronchial compression of the residual lung following surgical pneumonectomy. It is even rarer for this syndrome to present in patients without a history of prior lung surgery but induced by autopneumonectomy due ...

THIS REVIEW PROPOSES A CRITICAL REASSESSMENT (BASED ENTIRELY ON PUBLISHED EVIDENCE) OF THE FOLLOWING SEVEN COMMON BELIEFS ABOUT CHRONIC OBSTRUCTIVE PULMONARY DISEASE (COPD): (1) COPD is one disease. (2) There is a valid definition for COPD. (The current definition includes cases of irreversible asthma and bronchiectasis, and occasionally, other obstructive ...

Introduction: Thrombotic microangiopathy (TMA) is characterized by aggregation of platelets in the renal and/or systemic circulation, thrombocytopenia and intravascular hemolysis. The syndrome classically spares the lung. The term pulmonary-renal syndrome describes a number of diseases in which pulmonary hemorrhages and glomerulopathy coexist. Case Report: We report a 44-year-old man admitted ...

Objectives: Acute compartment syndrome is an important condition with potentially serious consequences if not diagnosed and treated promptly. This report highlights a case of acute compartment syndrome of the forearm after radial artery blood gas analysis in a patient who had been thrombolyzed for a pulmonary embolus. Methods/Case Report: We ...

Background. Obesity and asthma are associated. There is a relationship between lung function impairment and the metabolic syndrome. Whether this relationship also exists in the morbidly obese patients is still unknown. Hypothesis. Low-grade systemic inflammation associated with the metabolic syndrome causes inflammation in the lungs and, hence, lung function impairment. ...

Cystic lung diseases are characterised by multiple intrapulmonary cysts. They can be mimicked by other more common causes of focal parenchymal lucencies such as emphysema, bronchiectasis and honeycombing. The various conditions have differing managements and prognoses, thus correct evaluation is essential. Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant disorder, that ...

A 49-year-old man with trisomy 21 and Eisenmenger's syndrome presented for hip arthroplasty. Eisenmenger's syndrome is defined by the presence of obstructive pulmonary vascular disease secondary to long-standing left-to-right shunt causing pulmonary hypertension, eventually leading to shunt reversal in to right-to-left direction. Patients with Eisenmenger's syndrome pose a significant perioperative ...

Paraneoplastic syndromes are indirect manifestations of cancer due to functional peptides/hormones produced by a tumour, or due to cross reactivity between tumour and host antigens. Here the case of a 58-year-old woman presenting with ataxia, paraesthesia and subacute and progressive loss of vision is reported. The patient exhibited strong serum ...

Mycobacterium tuberculosis infection is a common infection in developing countries, including India. It can induce several cutaneous reactions such as erythema nodosum, and erythema induratum; however, association of tuberculosis with Sweet's syndrome (also known as acute febrile neutrophilic dermatosis) is extremely rare. Here we present an interesting case of sputum-positive ...

Abstract Untreated chronic obstructive pulmonary disease (COPD) co-existing with obstructive sleep apnea (OSA), also known as overlap syndrome, has higher cardiovascular mortality than COPD alone but its underlying mechanism remains unclear. We hypothesize that the presence of overlap syndrome is associated with more extensive right ventricular (RV) remodeling compared to ...

Congenital high airway obstruction syndrome (CHAOS) is one indication for the ex utero intrapartum treatment (EXIT), which is used to secure the fetal airway, while fetal oxygenation is maintained by uteroplacental circulation. We report a successful EXIT procedure in a twin gestation in which one child had CHAOS while the ...

Posterior reversible encephalopathy syndrome (PRES) has been associated with many conditions - particularly inflammatory, neoplastic and following organ failure. We submit that Chronic Obstructive Pulmonary Disease (COPD) is a significant predisposing factor for a number of these cases. Increased levels of circulating TNF-alpha, IL-1 and endothelin-1 (ET-1) are herein proposed ...

Despite being a fairly recent discovery, DOK7 congenital myasthenic syndrome (CMS) is the third most common form of CMS in the United Kingdom. DOK7 is a postsynaptic protein associated with the AChR clustering pathway. In contrast to AChR deficiency due to epsilon subunit mutations, onset of DOK7 CMS tends to ...

We report the unusual case of a child referred for persistent pneumonia and possible pulmonary nodules who was found to have a cardiac fibroma and diagnosed with Gorlin syndrome. Pulmonologists should be aware of the potential for vascular anomalies associated with this syndrome as well as the risk for pulmonary ...

Chronic lung allograft dysfunction is a major challenge in long-term management of lung transplant recipients. Both alloimmune-dependent factors (rejection) and alloimmune-independent factors contribute to the development of chronic lung allograft dysfunction. Thus, use of the term "chronic rejection" tends to be intentionally avoided among specialists in the field, although "chronic ...

BACKGROUND: The autoimmune lymphoproliferative syndrome (ALPS) is a rare, multi-systemic disease, caused by an inherited defect in the Fas apoptotic pathway, characterized by a chronic non-malignant lymphoid accumulation, and autoimmune manifestations. Lung, kidney, liver, and gut infiltration is described in severe, multi-systemic cases; so far there is no description of ...

To the Editor: In their description of a patient with the hantavirus pulmonary syndrome, Rhee et al. (Aug. 2 issue)(1) note that hypoxemia developed after the patient received intravenous fluids. A lack of hemodynamic improvement in response to fluids is a hallmark of the hantavirus pulmonary syndrome,(2) and fluid restriction ...

To the Editor: In their description of a patient with the hantavirus pulmonary syndrome, Rhee et al. (Aug. 2 issue)(1) note that hypoxemia developed after the patient received intravenous fluids. A lack of hemodynamic improvement in response to fluids is a hallmark of the hantavirus pulmonary syndrome,(2) and fluid restriction ...

Superior Vena Cava Syndrome (SVCS) refers to signs and symptoms caused by obstruction of the superior vena cava. Superior mediastinal syndrome (SMS) is the term used when SVCS coexists with obstruction of trachea. In children, a mediastinal pathology causing SVCS generally results in SMS as well, due to the limited ...

An 8-year-old male presented with a cystic lung lesion in the left lower lobe, which was initially detected during surgery for a spontaneous rupture of the sigmoid colon at the age of 6 years. Tissue fragility and a tendency to bleed easily were noted during the surgery, which strongly suggested vascular ...

This review starts with discussions of several infectious causes of eosinophilic pneumonia, which are almost exclusively parasitic in nature. Pulmonary infections due specifically to Ascaris, hookworms, Strongyloides, Paragonimus, filariasis, and Toxocara are considered in detail. The discussion then moves to noninfectious causes of eosinophilic pulmonary infiltration, including allergic sensitization to ...

Scimitar syndrome is one of the large congenital pulmonary venolobar syndromes and is defined as hypogenetic lung associated with partial anomalous pulmonary venous return. We report the case of a 25-year-old man with complex and exceptional variant scimitar syndrome. A chest CT scan with three-dimensional (3-D) reconstruction led us to ...

We previously described restrictive allograft syndrome as a form of chronic lung allograft dysfunction, demonstrating restrictive pulmonary function decline. However, the histopathological correlates of restrictive allograft syndrome have yet to be satisfactorily described. We hypothesized that pulmonary pleuroparenchymal fibroelastosis, as has recently been described in bone marrow transplant recipients, may ...

Diffuse alveolar hemorrhage is a clinical syndrome that can be a manifestation of multiple different causes. Identification of the underlying etiology is of utmost importance and dictates treatment. Pulmonary vasculitis including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a common cause of diffuse alveolar hemorrhage. For AAV, treatment includes induction ...

This article describes the clinical presentation of pneumonia, acute respiratory distress syndrome, lung abscess, and empyema: life-threatening infections of the pulmonary system. The etiology and risk factors for each of these conditions are described, diagnostic approaches are discussed, and evidence-based management options are reviewed.

There has been mounting evidence of a causal role for telomere dysfunction in a number of degenerative disorders. Their manifestations encompass common disease states such as idiopathic pulmonary fibrosis and bone marrow failure. Although these disorders seem to be clinically diverse, collectively they comprise a single syndrome spectrum defined by ...

Introduction: Chronic obstructive pulmonary disease (COPD) has extrapulmonary effects that seems to be related with systemic inflammation. The relationship between metabolic syndrome which is an important determinant of systemic inflammation in general population and COPD is still not clear. The aim of the current study was to investigate the frequency ...

Acute life-threatening airway obstruction with pseudomembrane formation after percutaneous dilational tracheostomye.

Alagille syndrome (ALGS) is an autosomal dominant disorder associated with cholestatic liver disease, pulmonary valvar stenosis or atresia, vasculopathy, and renal disease. Although the liver and cardiac manifestations contribute to overall morbidity and mortality during their life span, these patients also carry a burden of important but often underappreciated vascular ...

We report the case of a 31-day-old girl presenting with severe respiratory distress and cutaneous lesions from Sweet syndrome. Pulmonary symptoms unresponsive to antibiotics in patients with Sweet syndrome should raise suspicion for neutrophilic infiltration.

Patients with immunodeficiency disorders have an increased incidence of lymphoproliferative disorders; however, only 4 such patients with DiGeorge/chromosome 22q11.2 deletion syndrome have been reported. We report a case of a pulmonary Epstein-Barr virus-negative extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a child with this syndrome.

Patients with Sjögren's syndrome frequently have pulmonary involvement, but the involvement of nodular pulmonary lesions, including pulmonary amyloidosis, is rare. Most cases of pulmonary amyloidosis involve multiple nodules; solitary pulmonary nodular amyloidosis, as an associated condition of Sjögren's syndrome, is very rare.In our report, we present the case of an ...

We report a 34 year old man who developed bilateral ptosis and predominantly respiratory, truncal and bulbar weakness, and a high titer of anti acetylcholine receptor antibodies along with a diagnosis of Philadelphia chromosome positive Chronic Myeloid Leukemia (CML). The temporal relationship suggests a possible association.

Since the discovery of miRNAs, a number of miRNAs have been identified as p53's transcriptional targets. Most of them are involved in regulation of the known p53 functions, such as cell cycle, apoptosis and senescence. Our recent study revealed miR-1246 as a novel target of p53 and its analogs p63 ...

Mounier-Kuhn syndrome is a rare disorder of unknown cause that is characterized by atrophy of the elastic and smooth muscle of the tracheobronchial tree leading to tracheobronchomegaly and bronchiectasis. The syndrome is likely underdiagnosed, because the patients usually present with common respiratory symptoms such as productive cough and usually labeled ...

Horseshoe lung is a congenital pulmonary malformation that is usually associated with scimitar syndrome. This malformation consists of fusion of both pulmonary lobes from the posterobasal segments. The fusion appears in the retrocardiac area, in front of the esophagus and thoracic aorta. Pleural separation of pulmonary lobes distinguishes pseudohorseshoe appearance ...

We report the case of a 38-year-old woman diagnosed with Gitelman syndrome. A kidney biopsy showed abundant floating cells in the Bowman's space of the mildly cystic glomeruli, moderate tubulointerstitial changes and apparent intimal thickening of small arteries. These floating cells were immunohistologically identified as podocytes, by the expression of ...

We describe the case of a 45-year-old female who developed pulmonary infiltrates and mild eosinophilia following an overdose of ibuprofen. We believe this was a case of pulmonary infiltrates with eosinophilia (PIE) syndrome and discuss the relevant literature. Although rare, PIE syndrome should be considered in those taking nonsteroidal anti-inflammatories ...

To present a patient with overlapping choriocapillaritis syndromes who first presented as a typical case of multiple evanescent white dot syndrome (MEWDS) and later with characteristic findings compatible with multifocal choroiditis (MFC). A 40-year-old myopic woman presented with a paracentral scotoma OS. Fundus examination revealed pale discolored areas around the ...

Paraneoplastic syndrome (PNS) related to lung cancer is very common. However, the syndrome of inappropriate anti-diuretic hormone secretion (SIADH) is rare in non-small cell lung cancer (NSCLC). We are reporting the case of a 58-year-old female presenting with dyspnea, cough, weight loss, digital clubbing, and one week of haemoptysis. CT ...