✦ LIBER ✦

Sinonasal disease in cystic fibrosis: Clinical characteristics, diagnosis, and management

✍ Scribed by C. Gysin; G.A. Alothman; B.C. Papsin

Publisher: John Wiley and Sons
Year: 2000
Tongue: English
Weight: 201 KB
Volume: 30
Category: Article
ISSN: 8755-6863
DOI: 10.1002/1099-0496(200012)30:6<481::aid-ppul8>3.0.co;2-n

No coin nor oath required. For personal study only.

✦ Synopsis

Cystic fibrosis is an autosomal recessive genetic disorder that causes dysfunction of exocrine glands, and has several clinical manifestations. Among those, sinonasal involvement is almost universal, with or without chronic sinusitis and/or nasal polyposis. This review will detail the pathophysiologic changes of the sinonasal mucosa, and the clinical manifestations, diagnosis, and treatment. Developmental anatomic abnormalities, which are identified radiologically, will also be demonstrated. Medical management is the first treatment for patients with cystic fibrosis, but effective treatment of sinonasal disease in cystic fibrosis relies heavily on surgery. In the past, nasal polyposis was the main indication for surgery, and consisted mostly of polypectomy alone. This procedure was associated with a high recurrence rate. The development of functional endoscopic sinus surgery has contributed to decreasing the morbidity of sinonasal surgery and the recurrence of nasal polyposis in cystic fibrosis. The evolution of the surgical techniques will be discussed and a review of the literature will be provided.

📜 SIMILAR VOLUMES

Sweat electrolyte and cystic fibrosis mu

Sweat electrolyte and cystic fibrosis mutation analysis allows early diagnosis in Brazilian children with clinical signs compatible with cystic fibrosis

✍ Rabbi-Bortolini, Eliete; Bernardino, Andr�a L.F.; Lopes, Adoris L.; Ferri, Adria 📂 Article 📅 1998 🏛 John Wiley and Sons 🌐 English ⚖ 24 KB 👁 2 views

A total of 540 individuals with clinical signs suggestive of cystic fibrosis (CF) was studied. The sweat chloride was measured and the DF508, G542X, R553X, and W1282X mutations of the CF gene were screened by polymerase chain reaction (PCR). With this approach the diagnosis of CF was confirmed in 12

Phytanic acid storage disease (Refsum's

Phytanic acid storage disease (Refsum's disease): Clinical characteristics, pathophysiology and the role of therapeutic apheresis in its management

✍ Robert Weinstein 📂 Article 📅 1999 🏛 John Wiley and Sons 🌐 English ⚖ 26 KB 👁 2 views

Phytanic acid storage disease (known also as Refsum's Disease) is caused by inherited defects in the metabolic pathway for phytanic acid, a dietary branched-chain fatty acid. Poorly metabolized phytanic acid accumulates in fatty tissues, including myelin sheaths, and in organs including the liver an

Aerosolized dornase alfa in cystic fibro

Aerosolized dornase alfa in cystic fibrosis: Is there a role in the management of patients with early obstructive lung disease?

✍ David E. Geller 📂 Article 📅 1997 🏛 John Wiley and Sons 🌐 English ⚖ 75 KB 👁 1 views

Airway inflammation and infection are present in patients with mild lung disease in cystic fibrosis (CF), suggesting the need for early treatment. In a previously reported large multicenter trial, dornase alfa improved pulmonary function and decreased the need for hospitalization in patients with CF

A cystic fibrosis patient with two novel

A cystic fibrosis patient with two novel mutations in mitochondrial DNA: Mild disease led to delayed diagnosis of both disorders

✍ Wong, Lee-Jun C. ;Liang, Min-Hui ;Kwon, Haeyoung ;Bai, Ren-Kui ;Alper, �zg�l ;Gr 📂 Article 📅 2002 🏛 John Wiley and Sons 🌐 English ⚖ 242 KB 👁 2 views

Assessment of the reliability of single

Assessment of the reliability of single blastomere analysis for preimplantation diagnosis of the ΔF508 deletion causing cystic fibrosis in clinical practice

✍ Pierre F. Ray; Asangla Ao; Deborah M. Taylor; Robert M. L. Winston; Alan H. Hand 📂 Article 📅 1998 🏛 John Wiley and Sons 🌐 English ⚖ 197 KB 👁 2 views

Following the birth of a baby girl confirmed to be homozygous normal for the delta F508 deletion causing cystic fibrosis (CF), many single-gene defects have been diagnosed by polymerase chain reaction (PCR) for preimplantation genetic diagnosis (PGD). A few misdiagnoses have been reported but no lar