1/23. Chronic sinusitis in patients with cystic fibrosis.cystic fibrosis is a lethal genetic disorder whose victims currently have a median life span of approximately 30 years. With this increased life span, new aspects of the disease are identified in patients with cystic fibrosis, including chronic sinusitis. sinusitis severely affects the quality of life of patients with sinusitis. This article discusses several aspects of sinusitis in patients with cystic fibrosis, including clinical presentation, radiologic findings, and treatment options. By recognizing and treating sinusitis in patients with cystic fibrosis, physicians may improve the quality of life of these patients.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/23. Allergic bronchopulmonary aspergillosis in two patients with cystic fibrosis.Allergic bronchopulmonary aspergillosis (ABPA) is hypersensitivity to aspergillus fumigatus which manifests as episodic wheezing, usually refractory to bronchodilator therapy, with fixed and transient pulmonary infiltrates, central bronchiectasis, blood eosinophilia, elevated serum IgE level, immediate skin reactivity to an A. fumigatus antigen and precipitating antibodies to A. fumigatus. It is an unusual complication of asthma and cystic fibrosis (CF). We present two cystic fibrosis patients with ABPA treated successfully with prednisone and, in Case 1 also with itraconazole. The physician should be alert to the possibility of ABPA whenever CF patients present with the new infiltrates, high serum total IgE and other positive parameters of A. fumigatus sensitization. Treatment with systemic steroids should be started in order to prevent irreversible lung damage.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/23. 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.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/23. 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 = 1keywords = physician (Clic here for more details about this article) |
5/23. Religious/spiritual coping in childhood cystic fibrosis: a qualitative study.OBJECTIVE: To understand the role of religiousness/spirituality in coping in children with cystic fibrosis (CF). methods: Participants were a convenience sample of 23 patients with CF, ages 5 to 12 years, and their parent(s) in an ambulatory CF clinic. The design was a focused ethnography including in-depth interviews with children and parent(s), children's drawings, and self-administered written parental questionnaires. Analysis used grounded theory. RESULTS: Main outcome measures were participants' views on religion/spirituality in coping with illness. Data included 632 quotes organized into 257 codes categorized into 11 themes. One overarching domain emerged from analysis of the 11 themes: Religious/Spiritual Coping, composed of 11 religious/spiritual coping strategies. CONCLUSIONS: Children with CF reported a variety of religious/spiritual coping strategies they nearly always associated with adaptive health outcomes. A preliminary conceptual framework for religious/spiritual coping in children with CF is presented. More study is needed to assess how variability in age, disease type, disease severity, religious/spiritual preference, and religious/spiritual intensity affect religious/spiritual coping in children with chronic illness. Future studies should also investigate whether physician attention to religious/spiritual coping could assist patients in coping with CF and strengthen the doctor-patient relationship.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
6/23. Asymptomatic cystic fibrosis diagnosed in an adult evaluated for hematuria.We report a case of cystic fibrosis in an asymptomatic man evaluated for hematuria with infertility. The ever-broadening spectrum of atypical adult presentations of cystic fibrosis should prompt physicians to have a lower threshold for ordering genetic screening for cystic fibrosis transmembrane regulator (CFTR) gene mutations.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
7/23. cystic fibrosis and hemolytic uremic syndrome coexisting during pregnancy.BACKGROUND: As more women with cystic fibrosis are living into their reproductive years, this disease can complicate pregnancy and coexist with other entities. We report a case of cystic fibrosis with hemolytic uremic syndrome. CASE: An 18-year-old primigravida with cystic fibrosis was admitted at term with pulmonary symptoms, hypertension, and thrombocytopenia. She was delivered with the admitting diagnosis of severe preeclampsia. Postpartum, thrombocytopenia, and microangiopathic hemolytic anemia worsened. She developed renal failure and acute respiratory distress syndrome, requiring plasmapheresis, mechanical ventilation, and hemodialysis. Renal biopsy was consistent with the diagnosis of hemolytic uremic syndrome. CONCLUSION: cystic fibrosis, a disease once managed predominantly by pediatric subspecialists, will be seen increasingly by physicians caring for adults, including obstetrician-gynecologists. We may also expect to see it coexisting with other disorders. The management of such patients may prove challenging.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
8/23. A "story" of a woman with cystic fibrosis.munchausen syndrome is a pattern of abnormal behavior in which patients repeatedly present with symptoms that have no organic basis and are intentionally simulated or produced in order to assume the sick role and gain admission to the hospital. A 25-year-old white woman presented to our adult cystic fibrosis (CF) program and gave a detailed history consistent with a diagnosis of CF, a complicated disease with multiorgan involvement that is difficult to feign. However, discrepancies in her history were noted and objective measures of disease were inconsistent, including evidence of a fabricated sweat chloride result. We describe the classic characteristics of munchausen syndrome that our patient exhibited, and report this unusual presentation of munchausen syndrome to be consistent with the recommendation of reporting such cases to other physicians. Such knowledge is likely to result in less harm to the patient through unnecessary testing and treatment.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
9/23. The pulmonary physician in critical care. Illustrative case 1: cystic fibrosis.The case history of a patient with CF admitted to an ICU is presented and the appropriateness of intensive care management for patients with CF is discussed. Issues relevant to the ICU care of patients with CF are highlighted.- - - - - - - - - - ranking = 4keywords = physician (Clic here for more details about this article) |
10/23. The clinical spectrum of chronic appendiceal abscess in cystic fibrosis.OBJECTIVE--To describe the varied characteristics seen in patients with cystic fibrosis who develop chronic abscess formation secondary to unrecognized appendicitis. DESIGN--Patient series. SETTING--cystic fibrosis Care Centers in Columbus, ohio, and Tucson, Ariz. PARTICIPANTS--Five patients with cystic fibrosis who developed chronic abdominal abscesses secondary to occult appendicitis are described. Two patients developed fistula formation with purulent fluid drainage before diagnosis. One patient developed an extensive psoas abscess. Another presented with prolonged fever of unknown origin. These patients were identified by retrospective review of the past 20-year experience at two cystic fibrosis Care Centers. CONCLUSIONS--Development of chronic abdominal abscess related to unrecognized appendicitis is a rare but important complication in patients with cystic fibrosis. Prompt diagnosis depends on physician familiarity with the varied presentations of this entity. Diagnostic abdominal computed tomography and/or ultrasonography should particularly be considered when patients with cystic fibrosis present with pain, mass, or drainage from the right flank; prolonged fever; a limp; or failure of suspected meconium ileus equivalent syndrome to respond promptly to cathartic measures.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
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