Cases reported "Cystic Fibrosis"

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1/11. Influenza pneumonia in a paediatric lung transplant recipient.

    Although a common cause of morbidity and mortality in the general population, influenza infections are uncommon in lung transplant recipients and, to date, have only been associated with transient declines in pulmonary function and a relatively benign clinical course. This paper describes severe influenza pneumonia in a 13-year-old paediatric lung transplant recipient (5 months after double lung transplantation). Influenza pneumonia was diagnosed by direct fluorescent antibody testing and viral culture of bronchoalveolar lavage fluid. The patient required mechanical ventilation for 2 days due to respiratory failure and fatigue. Since his recovery from this pneumonia, he has developed obliterative bronchiolitis and currently awaits re-transplantation.
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ranking = 1
keywords = bronchiolitis
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2/11. bronchiolitis obliterans organizing pneumonia: a distinct pulmonary complication in cystic fibrosis.

    Organizing pneumonia in cystic fibrosis has hitherto been considered a nonspecific reparative process. We report on an adult patient with cystic fibrosis and histologically proven bronchiolitis obliterans organizing pneumonia, who experienced sustained clinical improvement under corticosteroid therapy. This case suggests that bronchiolitis obliterans organizing pneumonia may be a distinct pulmonary complication in cystic fibrosis and improve with specific therapy.
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ranking = 2.695779105531
keywords = bronchiolitis, bronchiolitis obliterans, obliterans
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3/11. cystic fibrosis and Down's syndrome: not always a poor prognosis.

    A child developed a bronchiolitis-like illness and was found to have mosaic Down's syndrome (diagnosed on karyotype) and also cystic fibrosis, diagnosed on the basis of a high sweat osmolality (247 mosmoles/kg sweat; normal, 62-137) and a homozygous delta F508 genotype. Despite two potentially life-threatening conditions, the child is doing well at the age of 7 years, despite pancreatic insufficiency.
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ranking = 1
keywords = bronchiolitis
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4/11. Inflammatory endobronchial polyposis with bronchiectasis in cystic fibrosis.

    An unusual case of endobronchial polyposis associated with extensive bronchiectasis in the context of cystic fibrosis (CF) has been described. A 15-yr-old female patient with CF underwent partial pneumonectomy for extensive bronchiectasis and frequent infective pulmonary exacerbations. Cylindrical bronchiectasis with associated purulent bronchitis and bronchiolitis, together with inflammatory polyposis, was noted in the resected lung. To the best of the authors' knowledge, this is the first report of multiple endobronchial polyposis and may represent a rare complication of bronchiectasis in a patient with cystic fibrosis. On-going infection and the cellular composition of the polyps are discussed in relation to their possible aetiological relevance and relationship to upper respiratory tract polyps.
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ranking = 1
keywords = bronchiolitis
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5/11. Coexistence of acute cellular rejection and lymphoproliferative disorder in a lung transplant patient.

    We report the case of a 37-year-old man who underwent bilateral lung transplantation for end-stage cystic fibrosis. Two months after his operation, a computed tomographic scan showed multifocal nodules throughout both lungs. Endobronchial biopsies revealed an Epstein-Barr virus-associated B-cell lymphoproliferation. Transbronchial biopsies revealed perivascular lymphoid infiltrates composed of predominantly small T lymphocytes. These perivascular infiltrates were retrospectively considered to be an acute cellular rejection rather than the periphery of the lymphoproliferative disorder. This opinion was based on several arguments: (a) a decrease in dosage of maintenance immunosuppression led to total regression of the lymphoproliferation but did not affect the perivascular lymphoid infiltrates; (b) the treatment of the acute cellular rejection temporarily induced the disappearance of the perivascular infiltrates; (c) the expression of Epstein-Barr virus was not detected in the perivascular infiltrates; and (d) on autopsy, performed 1 year later, severe obliterative bronchiolitis lesions were discovered, for which acute cellular rejection is the main risk factor. These observations point to the possibility that acute cellular rejection and an Epstein-Barr virus-associated lymphoproliferative disorder may coexist.
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ranking = 1
keywords = bronchiolitis
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6/11. pregnancy in cystic fibrosis lung transplant recipients: case series and review.

    A single centre experience of four pregnancies in four cystic fibrosis (CF) lung transplant recipients is reported. Six more cases were identified from the literature review and combined data analysis on 10 pregnancies in 10 CF lung transplant recipients was performed to determine maternal, foetal and graft outcome. There were nine live births and one therapeutic abortion. Three required caesarean sections. Five babies were premature but all nine children were well at follow-up. Five recipients who had a long, stable interval (i.e. at least three years) between transplant and pregnancy had a favourable outcome. Three recipients developed rejection during the pregnancy and one already had obliterative bronchiolitis before pregnancy. All showed progressive decline in lung function and subsequently died of chronic rejection within 38 months of delivery. pregnancy in CF lung transplant recipients is feasible but should still be regarded as a risky undertaking.
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ranking = 1
keywords = bronchiolitis
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7/11. advance care planning after lung transplantation: a case of missed opportunities.

    After lung transplantation, recipients are regularly evaluated by the transplant team and often require multiple hospitalizations. The primary focus of care during this time is on detecting and treating complications and may not necessarily include advance care planning discussions. This focus may leave clinicians unaware of the recipient's treatment preferences and place a burden on families trying to decide whether to undergo or forgo life-sustaining treatment when the recipient's medical condition deteriorates. We report the case of a woman with bronchiolitis obliterans who was admitted to the transplant center 37 times and died in the intensive care unit. Although progressive deterioration of her medical condition was well documented, her medical records revealed no evidence of advance care planning discussions with the patient and family. Incorporating timely advance care planning and integrating palliative care in the ongoing posttransplant clinical management may benefit patients, families, and clinicians as recipients approach the final stage of their illness.
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ranking = 1.297930975942
keywords = bronchiolitis, bronchiolitis obliterans, obliterans
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8/11. cystic fibrosis--a case presented with recurrent bronchiolitis in infancy in a Korean male infant.

    The aim of this case report is to draw the attention to the occurrence of cystic fibrosis (C.F.) in a Korean infant and thus increase the awareness for the diagnosis. The male infant was presented with a history of recurrent bronchiolitis manifested by severe cough, wheeze and dyspnea from three weeks of age, in whom the diagnosis of C.F. was clinically suspected and was confirmed by demonstration of two elevated sweat chloride levels (97 mEq/L and 99 mEq/L) in the patient. The diagnosis was delayed because the main manifestations of C.F. were the same as the main symptoms of common diseases such as cough, diarrhea and failure to thrive. C.F. is probably underdiagnosed in Korean population both because the diagnosis is not considered since the disease is thought to be uncommon or even not to occur and because diagnostic facilities including the quantitative iontophoresis sweat test are lacking.
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ranking = 5
keywords = bronchiolitis
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9/11. cystic fibrosis of the pancreas: report of a Japanese autopsy case.

    cystic fibrosis of the pancreas is considered to be rare in the Japanese population. Forty-five cases from the Japanese literature (1951-1983) are reviewed and one autopsy case added. The patient was a 17-year-old Japanese male with recurrent episodes of pulmonary infection. His sweat test was positive (sodium 332 mg/l, chloride 316 mg/l). No particular hereditary trait was identified. autopsy findings showed cystic dilatation of pancreatic ducts and ductules with mucous hypersecretion. Acini of the pancreas were replaced by fibrosis, while islands of Langerhans remained. These findings were compatible with cystic fibrosis of the pancreas (mucoviscidosis). The lungs showed chronic hypertrophic bronchitis and acute obliterating bronchiolitis. Cystic dilatation and hypersecretion were seen in the esophageal glands, Brenner's glands, and enteric glands. Goblet-cell hyperplasia was seen in the enteric glands. Central fatty metamorphosis and focal bile duct proliferation were seen in the liver. No obvious intralobular cholestasis was present. The epithelium of the gall bladder was highly columnar.
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ranking = 1
keywords = bronchiolitis
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10/11. Saprophytic fungal infections and lung transplantation--revisited.

    BACKGROUND: Fungal infections cause serious morbidity and death in lung transplant recipients. Some centers exclude and others will prophylactically treat patients with evidence of aspergillus colonization. methods: Of 126 patients undergoing lung transplantation at the University of north carolina since January 1990, five patients have died because of invasive aspergillus and other saprophytic fungal infections. Those cases are reviewed looking for common predisposing factors, including any evidence of prior colonization. In addition, all preoperative and postoperative culture data on all transplant recipients were retrospectively examined to define the prevalence of preoperative and postoperative aspergillus colonization in 121 patients with and without cystic fibrosis, none of whom had development of significant fungal disease. RESULTS: Fifty-two percent of 65 patients with cystic fibrosis were colonized with aspergillus before operation, and 40% after operation at some time. None had development of significant aspergillus infections, and none received prophylactic antifungal therapy. Most of the deaths from deep-seated fungal infections have been in patients without cystic fibrosis with no evidence of preoperative colonization. These patients had evidence of severe obliterative bronchiolitis, bacterial infections, persisting cytomegalovirus disease, or other major organ failure. CONCLUSION: The rationale for excluding patients or for giving amphotericin in the perioperative period in those patients who are colonized before surgery is questioned.
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ranking = 1
keywords = bronchiolitis
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