1/76. Pseudomonal pericarditis complicating cystic fibrosis.patients with advanced cystic fibrosis typically have chronic bacterial infection of the upper and lower respiratory tracts, but rarely develop extrapulmonary sites of infection. We report a case of purulent pericarditis due to pseudomonas aeruginosa in a patient with cystic fibrosis and no other risk factors for pericarditis. This is a previously unreported complication in cystic fibrosis prior to lung transplantation.- - - - - - - - - - ranking = 1keywords = respiratory tract, tract, upper (Clic here for more details about this article) |
2/76. Pyloric channel stricture secondary to high-dose ibuprofen therapy in a patient with cystic fibrosis.OBJECTIVE: To describe a case of pyloric channel stricture secondary to high-dose ibuprofen therapy in a pediatric patient with cystic fibrosis. CASE SUMMARY: A 12-year-old white girl started taking high-dose ibuprofen to treat the pulmonary manifestations of cystic fibrosis. The peak plasma concentration at dose initiation was within the accepted therapeutic range. Approximately one month later, the patient developed emesis and intolerance of solid foods, which persisted for several months and resulted in a weight loss of seven kilograms. The patient was referred to a pediatric gastroenterologist, who performed an upper endoscopy and subsequently diagnosed a pyloric channel stricture. The patient's pyloric channel was successfully dilated with two balloons. It is felt that the pyloric stricture developed from healing antral/pyloric channel ulcers. ibuprofen was discontinued and omeprazole therapy was begun. Over the course of the following year, the patient was asymptomatic. Follow-up upper gastrointestinal barium swallows were normal. DISCUSSION: When used for analgesia and fever in the pediatric population, ibuprofen has been shown to be a relatively safe drug. While it is known that ibuprofen may cause gastrointestinal adverse effects, the pediatric population is at lower risk; however, large doses of ibuprofen increase the risk of gastrointestinal adverse effects. The use of large doses of ibuprofen in the treatment of cystic fibrosis is a relatively new therapy. Limited data thus far in cystic fibrosis patients do not suggest increased risk of gastrointestinal complications. CONCLUSIONS: Limited data to date indicate that ibuprofen, when used in large doses to treat the pulmonary manifestations of cystic fibrosis, is relatively safe. However, because of the potential risks to the gastrointestinal tract of high-dose ibuprofen therapy, clinicians should be aware of its possible complications.- - - - - - - - - - ranking = 0.0064477024118704keywords = tract, upper (Clic here for more details about this article) |
3/76. Chronic airway colonization by penicillium emersonii in a patient with cystic fibrosis.penicillium emersonii Stolk, the conidial state of talaromyces emersoniii Stolk, is a heat-resistant fungus usually isolated from soil. In this paper the authors report, to our knowledge, the first human case in which P. emersonii chronically colonized the respiratory tract and induced an immune response in a patient with cystic fibrosis.- - - - - - - - - - ranking = 0.99928384188142keywords = respiratory tract, tract (Clic here for more details about this article) |
4/76. pneumonectomy in cystic fibrosis.A 17-year-old boy and a 12-year-old girl with cystic fibrosis (forced expiratory volume in 1 sec, 36% and 14% of predicted values, respectively) developed severe right-sided lung infections with abscess formations and complete atelectases unresponsive to medical therapy. In both patients, unilateral emergency pneumonectomy resulted in rapid clinical improvement. Despite her severe underlying lung disease, the girl experienced a remarkable increase in quality of life; 2 years after surgery, she died from respiratory failure. The male patient has now survived for 4 years, and lung transplantation still remains a therapeutic option for him. We believe that pneumonectomy is a valuable rescue therapy for patients with cystic fibrosis and intractable unilateral lung infections who are at high risk of dying while waiting for lung transplantation.- - - - - - - - - - ranking = 0.0050153861747083keywords = tract (Clic here for more details about this article) |
5/76. Histopathologic features of burkholderia cepacia pneumonia in patients without cystic fibrosis.We present the histopathologic features of fatal burkholderia cepacia pneumonia in three adults (one man [age 44 years] and two women [aged 40 and 43 years]). In all patients, the pulmonary infiltrates initially were localized (right middle lobe, left upper lobe, and right middle lobe) but rapidly progressed. Two open-lung biopsies and one pneumonectomy specimen showed necrotizing granulomatous inflammation merging with areas of more conventional necrotizing bronchopneumonia In one patient, a mediastinal lymph node also showed stellate necrotizing granulomas. vasculitis was absent. B. cepacia was cultured from the open-lung biopsies and bronchial wash specimens in two patients and from postmortem cultures of lung, subcarinal lymph nodes, and blood in the third. The histopathology in these patients resembles that of melioidosis, which is caused by a related organism, burkholderia pseudomallei. B. cepacia needs to be considered in the differential diagnosis of necrotizing granulomatous inflammation. In addition, given the rarity with which B. cepacia is identified as a cause of pneumonia in the immunocompetent host, isolation of B. cepacia should trigger a workup for underlying immunodeficiency or lead to an investigation to exclude the possibility of a nosocomial infection.- - - - - - - - - - ranking = 0.00071615811858104keywords = upper (Clic here for more details about this article) |
6/76. Protracted neonatal hypertrypsinogenaemia, normal sweat chloride, and cystic fibrosis.The cystic fibrosis (CF) clinical spectrum has greatly expanded in the past few years, including atypical forms with low sweat chloride concentrations. Two cases are presented which suggest that children detected by neonatal CF screening whose trypsinogen concentrations are still raised by the second month of age could, despite a negative sweat test, be affected by an atypical CF with fully expressed pulmonary involvement.- - - - - - - - - - ranking = 0.020061544698833keywords = tract (Clic here for more details about this article) |
7/76. prenatal diagnosis of cystic fibrosis: a case of twin pregnancy diagnosis and a review of 5 years' experience.We performed prenatal diagnoses for cystic fibrosis in 32 high risk (1:4) couples (including a dizygotic pregnancy). chorionic villi sampling did not cause abortion or fetal malformation in any case. The preliminary analysis of 9 short tandem repeats always excluded maternal contamination of the dna extracted from chorionic villi and confirmed paternity. Twenty-two prenatal diagnoses were made by direct analysis of the mutations. In seven cases diagnosis was made by the analysis of intragenic polymorphisms; in three cases, we analyzed two extragenic polymorphisms. The prenatal diagnosis (including genetic counselling) was completed within 24 h from the sampling. Seven prenatal diagnoses revealed an affected fetus; all couples opted for therapeutic abortion. In 17 cases the fetus was heterozygote, and in seven cases it was non carrier of mutated alleles. In the twin pregnancy, mutations were DeltaF508/N1303K. Direct analysis of the dna extracted from the two independent samples of chorionic villi revealed one fetus non carrier of mutated alleles and the other a carrier of the N1303K mutation. Analysis of the HPRT locus predicted both the fetuses as males. Furthermore, the genotype of each fetus was defined after birth. The prenatal diagnosis with chorionic villi sampling plays a key role in the prevention of cystic fibrosis. The laboratories must be equipped for both the direct analysis of mutations and for the analysis of a large number of polymorphisms. The preliminary analysis of short tandem repeats is recommended both to exclude maternal contamination and to confirm parentage.- - - - - - - - - - ranking = 0.010030772349417keywords = tract (Clic here for more details about this article) |
8/76. travel-associated burkholderia pseudomallei infection (melioidosis) in a patient with cystic fibrosis: a case report.In September 1997, a 25-year-old Italian woman with cystic fibrosis (CF) spent 3 weeks in thailand. In August 1998, her pulmonary function rapidly declined, with productive cough and intermittent fever. Chest x-ray films revealed diffuse, small, patchy opacities in the upper lobes. burkholderia pseudomallei (BP) was isolated from specimens of the patient's sputum and was identified by means of 16S rDNA sequencing. The diagnosis of melioidosis was serologically confirmed. Continuous therapy with ceftazidime and co-trimoxazole and maintenance with co-trimoxazole, doxycycline, and chloramphenicol resulted in eradication of BP. We present the issue of whether patients with CF represent a population particularly at risk for melioidosis.- - - - - - - - - - ranking = 0.00071615811858104keywords = upper (Clic here for more details about this article) |
9/76. noninvasive ventilation in respiratory failure due to cystic fibrosis.BACKGROUND: Noninvasive positive-pressure ventilation (NIPPV) is increasingly used as an effective means of avoiding endotracheal intubation and mechanical ventilation in patients with respiratory insufficiency or failure. methods: We retrospectively reviewed our experience with NIPPV to treat respiratory failure in five patients with cystic fibrosis (CF). RESULTS: Despite chronic lung disease related to CF, none of our cases were end-stage. All patients had recent pulmonary function tests showing a forced expiratory volume in 1 second (FEV1) of more than 30% predicted for age. All patients had progressive atelectasis, hypoxemia, and impending respiratory failure related to an acute pulmonary exacerbation or upper abdominal surgical procedure (open gastrostomy tube placement). Respiratory rates decreased, oxygen saturation increased, fraction of inspired oxygen (FiO2) requirement decreased, transcutaneous CO2 decreased, and atelectasis resolved with NIPPV. CONCLUSIONS: Use of NIPPV provides effective respiratory support while avoiding the need for endotracheal intubation. The applications of NIPPV, reports of its use in patients with CF, and the equipment required are reviewed.- - - - - - - - - - ranking = 0.00071615811858104keywords = upper (Clic here for more details about this article) |
10/76. The child with persistent cough.Coughing is a healthy reflex. Causes of a cough can vary from minor upper respiratory illnesses to malignancy. When a child's cough continues for weeks, parents worry. Primary care providers must decide when reassessment is needed and if a vigorous workup and referral to a pulmonologist are required. The above discussion should assist these physicians.- - - - - - - - - - ranking = 0.00071615811858104keywords = upper (Clic here for more details about this article) |
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