The Nose

1959 Lurie MH. Cystic fibrosis of the pancreas and the nasal mucosa. Ann Otol Rhinol Laryngol 1959; 68:478.[PubMed]
An early description of nasal polyps which are remarkably common in both children and adults with CF (6-48% in various series). In this paper three illustrative cases were described at an ENT meeting. (also Shwachman et al. 1962 below; Complete filling of the nasal sinuses was described by Pennington CL. AMA Arch Otolaryng 1956; 63:576; Bodian describes changes in the sinuses, (1952 above). Nasal polyposis may be the presenting feature of CF, the first evidence of CF being nasal polyps so large they are appearing at the external nares as occured in one of our Leeds children.

1961 Shwachman H, Kulczycki LL, Mueller HL. Nasal polyposis in patients with cystic fibrosis. Am J Dis Child 1961; 102:768-769.
An early description of nasal polyps (also Lurie MH, 1959 above). Of 650 patients with CF seen over the previous 4 years, 43 had nasal polyposis - 10% of those over 3 years. The effect on facial appearance, increasing the width of the nasal bridge, and the tendency to recur after removal were stressed. There was nothing characteristic about the histological appearance of the polyps. A sweat test was recommended in any child with nasal polyposis. (also Shwachman H. Kulczycki LL, Mueller HL, Flake CG. Nasal polyposis in patients with cystic fibrosis. Pediatrics 1962; 30: 389-401. Nasal polyposis occurring in 50 (6.7%) of 742 patients with CF in those with allergies and those without).
Later more accurate assessment of the incidence of polyps was possible with fibreoptic instruments indicating 37% of patients were affected. Inhaled steroids were used with some success as local treatment – eventually many years later confirmed in a controlled trial (Hadfield et al, 2000 below). Also as an incidental finding during a trial of oral ibuprofen for the chest infection, the nasal polyps were observed to shrink (Lindstrom et al, 2007 below).

1958 Barbero GJ, Chernick W. Function of the salivary gland in cystic fibrosis of the pancreas. Pediatrics 1958; 22: 945-952.[PubMed]
Children with CF had greater unstimulated salivary flow and the concentration of sodium and chloride was elevated compared with controls as had been shown by others. Response to mecholyl injection greater but potassium did not differ between CF and controls. This suggested an increased parasympathomimetic sensitivity in cystic fibrosis.

1959 Lurie MH. Cystic fibrosis of the pancreas and the nasal mucosa. Ann Otol Rhinol Laryngol 1959; 68:478.[PubMed]
An early description of nasal polyps (figure 21) which are remarkably common in both children and adults with CF (6-48% in various series). In this paper three illustrative cases were described at an ENT meeting. (also Shwachman et al. 1962 below; Complete filling of the nasal sinuses was described by Pennington CL. AMA Arch Otolaryng 1956; 63:576; Bodian describes changes in the sinuses, (1952 above). Nasal polyposis may be the presenting feature of CF, the first evidence of CF being nasal polyps so large they are appearing at the external nares as occured in one of our Leeds children.

1961 Shwachman H, Kulczycki LL, Mueller HL. Nasal polyposis in patients with cystic fibrosis. Am J Dis Child 1961; 102:768-769.
An early description of nasal polyps in cystic fibrosis (also Lurie MH, 1959 above). Of 650 patients with CF seen over the previous 4 years, 43 (7%) had nasal polyposis - 10% of those over 3 years. The effect on facial appearance, increasing the width of the nasal bridge, and the tendency to recur after removal were stressed. There was nothing characteristic about the histological appearance of the polyps. A sweat test was recommended in any child with nasal polyposis.
Also Shwachman H. Kulczycki LL, Mueller HL, Flake CG. Nasal polyposis in patients with cystic fibrosis. Pediatrics 1962; 30: 389-401.[PubMed] Nasal polyposis occurred in 50 (6.7%) of 742 patients with CF in those with allergies and those without.
Later more accurate assessment of the incidence of polyps was possible with fibreoptic instruments indicating that 37% of patients were affected. Inhaled steroids were used with some success as local treatment – eventually many years later confirmed in a controlled trial (Hadfield et al, 2000 below). Also as an incidental finding during a trial of oral ibuprofen for the chest infection, the nasal polyps were observed to shrink (Lindstrom et al, 2007 below).

1962 Barbero GJ, Sibinga MS. Enlargement of the submaxillary glands in cystic fibrosis. Pediatrics NY 1962; 29:788-793. [PubMed]
This is the first record of enlarged submandibular glands in CF – a feature which is sometimes quite marked (figure 11). During their studies on secretory activity of the salivary glands the authors noted many patients had enlarged submandibular glands. The submandibular glands of 300 normal children, 106 with CF and 20 with chronic pulmonary disease were examined. Enlarged submandibular glands were palpated in 92% of children with CF and only 2% of healthy controls and those with chronic pulmonary disorders. The presence of enlargement can be helpful when considering a diagnosis of CF.

2000 Hadfield PJ, Rowe-Jones JM, Mackay IS. The prevalence of nasal polyps in adults with cystic fibrosis. Clin Otolaryngol Allied Sci 2000; 25:19-22.[PubMed]
The prevalence of nasal polyps in this endoscopic study of 211 adults with CF was 37%. The commonest nasal symptoms were discharge, headache and obstruction. Intranasal endoscopy usually demonstrated mucosal oedema and thin, clear discharge. The prevalence of allergy, as diagnosed by skin prick testing, was almost twice that of the general population. Tympanometry showed that middle ear effusion was uncommon in these patients.

Surprisingly, previous studies have also shown a low incidence of middle ear infection in people with CF (also Hadfield et al. 2000. below). Endoscopic studies are required to give an accurate estimate of the incidence of nasal polyps that may have been under-estimated in previous studies.

2000 Hadfield PJ, Rowe-Jones JM, Mackay IS. A prospective treatment trial of nasal polyps in adults with cystic fibrosis. Rhinology 2000; 38:63-65. [PubMed]
The first prospective, randomised, double-blind trial of the treatment of nasal polyps in cystic fibrosis. Use of betamethasone nasal drops showed a statistically significant reduction in polyp size in comparison to placebo treatment.

This is the first trial of topical steroid treatment of nasal polyps, a complication that can prove a troublesome and recurring problem for the patient. Inhaled steroids appeared to have some effect but they had never been the subject of a clinical trial. The recurrence rate after surgery is very high.
In a more recent study of the non-steroidal anti-inflammatory ibuprofen for CF lung disease, the drug had a favourable and quite unexpected effect on the nasal polyps which was noted as an incidental finding (Lindstrom D R, et al. J Otolaryngol 2007; 36:309-314 [PubMed] below).

2003 Bateman ND, Fahy C, Woolford TJ. Nasal polyps: still more questions than answers. J Laryngol Otol 2003; 117:1-9.[PubMed].
Nasal polyps are common, affecting one to four per cent of the population. Their cause, however, remains unknown and it is possible that it is not the same in all patients. They have a clear association with asthma, aspirin sensitivity and cystic fibrosis. Histologically they demonstrate large quantities of extracellular fluid, mast cell degranulation and an infiltrate of inflammatory cells, usually eosinophils. The authors believe that an endoscopic approach using a microdebrider facilitates accurate removal of polyps with preservation of normal anatomy.

This is a detailed review of polyps in general with no new information.

2007 Lindstrom DR, Conley SF, Splaingard ML, Gershan WM. Ibuprofen therapy and nasal polyposis in cystic fibrosis patients. J Otolaryngol 2007; 36:309-314.[PubMed]
Twelve of 22 patients with CF were treated with high-dose ibuprofen therapy to benefit their pulmonary function. Twelve had nasal polyposis and all 12 patients had observed absence of nasal polyps at some point during their ibuprofen course; nasal polyps were present in five patients during ibuprofen therapy, and all resolved with increased ibuprofen doses. Polyps occurred in six of eight patients after ibuprofen therapy ceased. Five of the 12 patients required endoscopic sinus surgery for polyposis.

This is an interesting incidental finding that oral ibuprofen improves nasal polyposis. This could prove very helpful in patients with severe and recurring nasal polyposis which can be a very difficult problem.

2009 Buyukozturk S. Gelincik A. Aslan I. Aydin S. Colakoglu B. Dal M. Methotrexate: can it be a choice for nasal polyposis in aspirin exacerbated respiratory disease?. J Asthma 2009; 46:1037-1041. [PubMed].
A report of two patients with asthma whose NP dramatically reduced in size after a course of MTX therapy administered as an additional treatment for their steroid- dependent asthma.

Although these patients had asthma it is interesting that they responded to methotrexate - it is possible that the minority of people with severe recurring nasal polyps may also respond.