Cases reported "Cystic Fibrosis"

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1/21. Bronchoscopically administered recombinant human DNase for lobar atelectasis in cystic fibrosis.

    Lobar atelectasis is a common complication of cystic fibrosis. The majority of cases respond to intravenous antibiotics and chest physiotherapy. In a subgroup of patients, atelectasis is resistant to medical therapy, and its persistence in the pediatric population is associated with a poor prognosis. Bronchoscopic instillation of human recombinant DNase expanded atelectatic lobes in three children resistant to at least 2 weeks of medical therapy. This method of administration of DNase has been successful in resistant cases of lobar atelectasis in patients with chronic obstructive pulmonary disease, quadriplegia, and status asthmaticus. Purulent cystic fibrosis sputum has a very high dna content, and dna has been shown to become more pourable in vitro when treated with rhDNase. Bronchoscopic instillation of rhDNase should be considered in cases of persistent lobar atelectasis unresponsive to medical therapy.
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2/21. Pamidronate results in symptom control of hypertrophic pulmonary osteoarthropathy in cystic fibrosis.

    Hypertrophic pulmonary osteoarthropathy (HPOA) may complicate the advanced lung disease that is associated with cystic fibrosis, resulting in severe joint pain and early-morning stiffness. Symptoms are usually controlled with the administration of nonsteroidal anti-inflammatory drugs, physiotherapy, and, on occasions, oral corticosteroids. This report describes a case of refractory HPOA with complete remission following the administration of IV pamidronate, which is a potent inhibitor of osteoclastic bone resorption. Symptom relief resulted for up to 3 months, but repeated courses of pamidronate have been required to maintain symptom control.
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3/21. Successful meropenem desensitization in a patient with cystic fibrosis.

    OBJECTIVE: To report a case of successful meropenem desensitization in a patient with cystic fibrosis (CF) with documented hypersensitivity to multiple antibiotics including carbapenems. CASE SUMMARY: A 20-year-old white man with CF was admitted to the hospital for treatment of an acute pulmonary exacerbation caused by multidrug-resistant burkholderia cepacia complex and methicillin-resistant staphylococcus aureus (MRSA). Past treatments of his CF exacerbations were complicated by urticarial eruptions following administration of beta-lactams, including meropenem, and ototoxicity from aminoglycosides. Skin testing revealed hypersensitivity reactions to beta-lactam antibiotics including penicillin, piperacillin/tazobactam, ceftazidime, and imipenem. A literature review (medline, January 3, 2002) and communication with the manufacturer of meropenem revealed no specific information on desensitizing patients to this agent. Because of meropenem's activity against B. cepacia complex alone and in combination with other antimicrobials, a desensitization protocol was adapted and applied to meropenem in an effort to provide the most beneficial treatment available. A 12-dose escalation protocol was successfully employed without incident. DISCUSSION: Antimicrobial therapy is limited in CF patients by susceptibility profiles of common infecting organisms (e.g., Pseudomonas spp., B. cepacia complex, MRSA). Unfortunately, host responses may further reduce the utility of many effective antibiotic classes due to hypersensitivity and/or adverse reactions. Desensitization is a useful alternative that allows the administration of beneficial medications to patients with documented allergy histories. CONCLUSIONS: Meropenem is an important treatment option in the CF population, particularly due to its activity against B. cepacia complex. Successful desensitization using a dose-escalation protocol in patients with a documented carbapenem allergy will allow the most beneficial therapy to continue.
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4/21. Intraoperative administration of nitric oxide.

    Despite its therapeutic efficacy in various clinical scenarios in the intensive care unit setting, there are limited reports regarding the intraoperative applications of nitric oxide (NO). The authors present 2 pediatric patients to whom inhaled NO was administered intraoperatively. In one patient, NO was used to treat hypoxemia that developed after the institution of one-lung ventilation during thoracoscopic resection of a bronchiectatic section of lung. In the second patient, NO was used to alleviate pulmonary hypertension and cardiovascular dysfunction with pulmonary artery cross-clamping for placement of a modified Blalock-Taussig shunt. The potential intraoperative applications and techniques for the intraoperative delivery of NO are presented.
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5/21. Inhaled budesonide induced Cushing's syndrome in cystic fibrosis patients, due to drug inhibition of cytochrome P450.

    Two CF patients developed Cushing's syndrome during administration of inhaled budesonide (400 microg/d) with oral itraconazole in one and with clarithromycin in the other patient. Clinical features appeared respectively after 2 and 6 weeks of drug co-administration, with prolonged adrenal suppression, and a slow recovery after ceasing the drugs. Inhibitors of the cytochrome P450 interfere with the metabolism of corticosteroids. Combination of these drugs even with moderate doses of budesonide should be closely monitored.
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6/21. cyclosporine-related neurotoxicity in a patient after bilateral lung transplantation for cystic fibrosis.

    cyclosporine (CsA) is a widely used immunosuppressant following solid organ transplantation. CsA administration is associated with a number of systemic complications, including neurotoxicity. A 33-year-old man with cystic fibrosis, who underwent bilateral lung transplantation, presented with severe neurotoxic symptoms leading to coma in association with CsA administration combined with high doses of methylprednisolone for treatment of an acute rejection episode. After discontinuation of CsA, a quick resolution of his clinical status was observed, as well as of the pathological findings on magnetic resonance imaging (MRI). CsA was replaced with tacrolimus leading to an uneventful course.
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7/21. pseudomonas aeruginosa endogenous endophthalmitis with choroidal abscess in a patient with cystic fibrosis.

    PURPOSE: To describe the clinical presentation, management, and outcome of a case of endogenous intraocular infection due to pseudomonas aeruginosa in a patient with cystic fibrosis (CF). methods: The authors describe a case of an adult patient with CF who developed an intraocular infection by P. aeruginosa. RESULTS: diagnosis was confirmed by culture of purulent fluid obtained by transcleral incision and drainage. The infection persisted in spite of aggressive surgical intervention including extensive endoresection of the visibly affected retina and choroid and multiple administrations of appropriate intraocular, subconjunctival, and systemic antibiotics. Eventually, the eye became blind and painful and was enucleated. CONCLUSIONS: Endogenous intraocular infection by P. aeruginosa can occur in patients with CF who have not undergone lung transplantation. An infection of this type may be impossible to eradicate by aggressive surgical intervention and appropriate antibiotic therapy.
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8/21. breast-feeding in a woman with cystic fibrosis undergoing antibiotic intravenous treatment.

    We report the case of a 30-year-old woman with cystic fibrosis (CF) chronically infected with pseudomonas aeruginosa who delivered and breast-fed a healthy boy. While breast-feeding the woman had to undergo an i.v. antibiotic course with tobramycin, due to pulmonary exacerbation. tobramycin was not detected in her milk and lactation could be continued. This is the first time that the presence of tobramycin in the milk of a CF woman during i.v. administration has been investigated.
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9/21. Benign intracranial hypertension after ciprofloxacin administration.

    Many drugs, including antibiotics, have been implicated in the aetiology of benign intracranial hypertension. We report the development of benign intracranial hypertension after the use of ciprofloxacin in a teenager with cystic fibrosis.
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10/21. Acute desensitization of a patient with cystic fibrosis allergic to both beta-lactam and aminoglycoside antibiotics.

    A 15-year-old patient with cystic fibrosis developed urticarial reactions to tobramycin, gentamicin, and cephoperazone, and an anaphylactic reaction to ticarcillin during therapy for an extensive pulmonary infection with Pseudomonas aeruginosa and Staphylococcus aureus. Immediate wheal-and-flare skin tests were positive with tobramycin and with penicilloylpoly-L-lysine. Desensitization with tobramycin in gradually increasing intravenous doses was accomplished during 8 hours. The procedure was complicated by a macular rash that remitted within minutes without therapy, but no symptoms or signs of an allergic reaction to tobramycin were detected during full dose therapy. Skin test responses to tobramycin became negative by the end of the desensitization procedure, whereas the responses to penicilloylpoly-L-lysine and histamine remained positive. A worsening course led to an unsuccessful attempt to desensitize the patient to beta-lactam determinants. Wheezing appeared during the administration of oral doses. This case demonstrates the feasibility of acute, antigen-specific desensitization of an aminoglycoside-allergic patient and the failure to achieve a second, simultaneous desensitization. This patient experienced the first serious reaction to oral penicillin desensitization.
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