Cases reported "Lung Diseases"

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1/81. Negative pressure pulmonary hemorrhage.

    Negative pressure pulmonary edema, a well-recognized phenomenon, is the formation of pulmonary edema following an acute upper airway obstruction (UAO). To our knowledge, diffuse alveolar hemorrhage has not been reported previously as a complication of an UAO. We describe a case of negative pressure pulmonary hemorrhage, and we propose that its etiology is stress failure, the mechanical disruption of the alveolar-capillary membrane.
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2/81. The decline and early fall of neonatal pulmonary hemorrhage.

    Seven years' experience (1965-1972) with neonatal pulmonary hemorrhage at the University of colorado was reviewed. Pulmonary hemorrhage still occurs frequently as a preterminal event in hyaline membrane disease. As an isolated "pure" entity, however, pulmonary hemorrhage has shown a marked decline. This phenomenon is probably related to advances in neonatal intensive care. The radiographic pattern of "pure" pulmonary hemorrhage in the newborn is most commonly a diffuse alveolar infiltrate. Since pure hemorrhage is now an uncommon entity, the radiologist can reasonably suggest this diagnosis on rare occasions only, in the clinical setting of profound hypoxia.
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3/81. Demonstration of communication between alveolus and interstitium in persistent interstitial pulmonary emphysema: case report.

    Persistent interstitial pulmonary emphysema (PIPE) is an uncommon complication of premature infants suffering from hyaline membrane disease who have been treated with mechanical ventilation. The presumed mechanism for the development of the disease is via a break in the bronchioalveolar system that allows air to escape into the interstitium. We report a case of a 9-week-old child who developed the localized form of the disease and underwent a lobectomy. Immunohistochemical stains helped to demonstrate the communication between the airway system and interstitium. This report strengthens the theory that the disease develops from airway rupture at the alveolar level.
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4/81. Immunohistochemical study of a patient with diffuse pulmonary corpora amylacea detected by open lung biopsy.

    The chest radiographs of an asymptomatic 58-year-old Japanese man with pulmonary corpora amylacea (PCA) revealed bilateral patchy and nodular infiltrates. Lung specimens obtained by open lung biopsy were histochemically and immunohistochemically analyzed. In all sections of dissected lung tissue, hematoxylin and eosin staining revealed homogeneous eosinophilic acellular round bodies (50-100 microm in diameter) containing granular black pigments in the alveolar spaces. Some round bodies were surrounded and phagocytized by alveolar macrophages. The laminated round bodies stained positively with PAS and congo red. In addition, many of the rounded bodies contained particles which stained positively with berlin blue. Immunohistochemical staining for epithelial membrane antigen (EMA) as well as PE-10 was distinctively positive. This is a very rare case of diffuse PCA found by open lung biopsy. Immunohistochemical examination suggested that PCA consisted of pulmonary surfactant protein and epithelial membrane antigen.
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5/81. Aortopulmonary collateral artery embolization during postoperative extracorporeal membrane oxygenation after arterial switch procedure.

    Aortopulmonary collateral arteries sometimes complicate cyanotic congenital heart defects. Combined with a relevant left-right shunt, this could result in massive airway bleeding during and after corrective surgery. A preoperatively diagnosed 1.2 mm small aortopulmonary collateral artery in a newborn suffering from transposition of the great arteries caused life-threatening airway bleeding during surgery. Postoperative extracorporeal membrane oxygenation (ECMO) was necessary, and coil embolization was performed on ECMO to terminate pulmonary bleeding.
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6/81. Lung symptoms in pseudohypoaldosteronism type 1 are associated with deficiency of the alpha-subunit of the epithelial sodium channel.

    OBJECTIVE: To study patients with autosomal recessive pseudohypoaldosteronism type 1 and to relate pulmonary disease to gene mutations of the epithelial sodium channel (ENaC). STUDY DESIGN: Clinical and laboratory data were collected from 4 Swedish patients with pseudohypoaldosteronism type 1. The genes for ENaC and cystic fibrosis transmembrane conductance regulator were analyzed for mutations with methods including dna sequencing. RESULTS: Three novel mutations were found in the alpha-gene of ENaC, 2 frameshifts (1449delC and 729delA) and 1 missense mutation resulting in the substitution of leucine for serine 562 in the alpha-chain (S562L). The 1449delC mutation was found in all patients in either homozygous or heterozygous form and seems to be the predominant cause of pseudohypoaldosteronism in sweden. The allele coding for S562L also contained a transition converting tryptophan 493 to arginine (W493R), which seems to be a rare polymorphism. All patients had pulmonary symptoms to various degrees. The bacterial findings resembled, to some extent, those in cystic fibrosis, but development of chronic lung disease and progressive decline in lung function were not observed. CONCLUSIONS: Genetic deficiencies of the alpha subunit of the ENaC are associated with prominent lung symptoms, which are, however, clearly different from those in cystic fibrosis.
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7/81. Sequential development of pulmonary hemorrhage with MPO-ANCA complicating anti-glomerular basement membrane antibody-mediated glomerulonephritis.

    We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (anti-GBM) antibodies that progressed to end-stage renal disease in a 67-year-old woman with diabetes. Intensive combined immunosuppressive therapy with methylprednisolone bolus, oral prednisone, and cyclophosphamide led to negativity of anti-GBM antibodies but was not able to restore renal function. After 28 months of hemodialysis, the patient suddenly presented with pulmonary hemorrhage. In this setting, high levels of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) and negative anti-GBM antibodies were found. Therapy with oral prednisone and cyclophosphamide led to resolution of pulmonary hemorrhage and negativity of MPO-ANCA.
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8/81. Reaching the diagnosis of cystic fibrosis--the limits of the spectrum.

    BACKGROUND: Cystic fibrosis is the most common life-limiting autosomal recessive genetic disorder in Caucasians. Typically it is a multisystem disease diagnosed by increased chloride levels on sweat testing, with mortality due mainly to progressive respiratory disease. The clinical spectrum of CF has recently been much expanded. genetic testing for mutant CF transmembrane regulator has revealed atypical cases where sweat test results are borderline or normal. In other patients, genetic mutations cannot be identified but abnormal CFTR function is shown using nasal potential difference measurement. OBJECTIVES: To highlight the diagnostic and therapeutic dilemmas in cases of atypical cystic fibrosis. methods: We reviewed patients with atypical CF and widely varying phenotype who are managed at Schneider Children's Medical Center of israel. RESULTS: Two patients had severe lung disease but little expression in other organs. Accurate diagnosis was essential to enable aggressive therapy in a specialized center. Four other patients are in excellent general health but have symptoms limited to male infertility, heat exhaustion, pancreatitis or transient liver dysfunction, while lung disease is minimal. For these patients, careful counseling is needed to avoid unnecessary upheaval, inappropriately aggressive management, and the psychosocial implications of a CF diagnosis. These dilemmas have increased considerably in our center, as in others worldwide. CONCLUSION: It is our obligation as clinicians--at the level of both primary physician and referral center--to maintain an ever higher index of suspicion for CF, tempered by a rational program of counseling and management appropriate to the individual.
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9/81. Idiopathic pulmonary hemosiderosis with cystic lesions: a rare presentation.

    This report describes a case of a 49-year-old man with cough, recurrent hemoptysis, and dyspnea during 18 months, presenting with radiological findings of alveolar infiltrate and cystic lesions in left upper lobe. Laboratory studies revealed normocytic hypochromic anemia and normal coagulation tests. c-reactive protein and mucoproteins were negative. serum protein electrophoresis and complement, urinalysis, serum creatinine, creatinine clearance, and 24-hour urine protein were normal. Tests for antineutrophil cytoplasmic antibodies and anti-glomerular-basement membrane antibodies were negative. Tests for connective tissue diseases were all negative. Histological findings were consistent with those of idiopathic pulmonary hemosiderosis. Radiological findings are discussed.
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10/81. Femoral venoarterial extracorporeal membrane oxygenation for severe reimplantation response after lung transplantation.

    Severe pulmonary reimplantation response after lung transplantation is not very common, although the mortality can be high. We present a patient who developed an extremely severe reperfusion injury after bilateral lung transplantation. Because of severe hypoxia and hemodynamic instability, despite aggressive ventilator settings, venoarterial extracorporeal membrane oxygenation (ECMO) was instituted using the femoral approach at the bedside. During ECMO, the patient developed a thoracic wall hematoma that was treated with transfusion alone. After 50 h of ECMO, his chest radiograph had dramatically improved, his oxygen need had been reduced to 50%, and he was successfully weaned from ECMO. Two years later, he is doing extremely well. Therefore, institution of ECMO using the femoral approach can be performed safely at the bedside in the ICU, and can be lifesaving in the context of a very severe reimplantation response after lung transplantation.
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