Other complications of Cystic Fibrosis
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Despite theoretical risks from the use of sweetening agents in the syrup formulations of medications and possible increased sugar intake in high calorie diets, evidence suggests a decreased prevalence of dental caries, plaque and gingivitis in cystic fibrosis1, possibly due to the protective effects from long term antibiotic use.
Enamel opacities are thought to be more common in cystic fibrosis.
Although tetracycline use has declined significantly, it is occasionally used in some infections. The risk of harm to dentition must still be borne in mind when prescribing.
Routine dental care from toddlerhood is to be encouraged through the school dental programme. Use of oral calorie supplements can contribute to dental caries therefore advice on good dental hygiene should be given when starting supplements.
Vitamin A deficiency in cystic fibrosis has been reported to lead to xerophthalmia. Any patients with cystic fibrosis complaining of dry eyes or issues with night vision should be examined by ophthalmology as you need a good slit lamp examination to assess for xerophthalmia.
Pseudomonal endophthalmitis has been reported post lung transplant.
Although cystic fibrosis transmembrane regulator (CFTR) is found on neural cell membranes, there are no recognized clinical neurological problems arising as a direct result of CFTR dysfunction in neuronal tissue.
Neurological problems that do occur are more likely secondary to other sequelae of cystic fibrosis, such as:
- Headache (common) - possibly a reflection of sleep disordered breathing, or chronic hypoxia/hypercarbia.
- Seizures - can arise from metabolic and electrolytic disturbance, or occasionally drug reaction (e.g. ciprofloxacin).
Severe fat soluble vitamin deficiency may be associated with neurological disorders. Vitamin A deficiency has caused visual disturbances and raised intracranial pressure in cystic fibrosis and hypovitaminosis D is associated with several chronic neurological conditions including multiple sclerosis, dementia, and epilepsy. These have not been seen in cystic fibrosis as a direct result of vitamin deficiency from malabsorption. Vitamin E deficiency has been associated with ataxia and other neurological signs.
Aside from the risks arising from delayed clotting times, vitamin K deficiency is not thought to directly affect nervous tissue in cystic fibrosis.
Rarely, occult patent foramen ovale may predispose to cerebro-vascular accidents from totally implantable venous access devices (TIVAD).
Cerebral abscess has been reported in the context of severe lung disease.
Iron deficiency is common in children with cystic fibrosis. Anaemia from low haematinics is well recognised in cystic fibrosis, as is microcytosis without anaemia.
Thrombophilic tendency is thought to be more common in cystic fibrosis than in the general population. Balfour-Lynn2 reported higher than expected prevalence of antithrombin III (1%), protein S deficiency (5%), protein C deficiency (4%) and lupus anti-coagulant (9%). 5% of this cystic fibrosis population has protein C resistance, but this is the same as the general population. Unless there is a clinical concern (eg a problem for which thrombus may be the cause), routine testing for thrombophilia is not indicated.
For a child in whom a totally implantable venous access device (TIVAD) is considered, the occurrence of previous line-associated thrombus or relevant family history may justify further testing prior to line insertion, particularly if longer term thrombo-prophylaxis is considered. Testing should only occur after discussion with a paediatric haematologist. Management of a thrombophilic tendency, in the presence of TIVAD, should also be discussed with a clinician with expertise in this area.
Vitamin K deficiency is a recognised consequence of malabsorption of fat soluble vitamins. It should be considered in a child with cystic fibrosis who has clinical signs of a bleeding tendency, or prolonged clotting times on blood testing.
Cystic fibrosis associated arthropathy
This is an episodic, acute arthropathy which affects 2-8% of adults with cystic fibrosis. It is of unknown aetiology, although it is thought to relate to circulating immune complexes.
- acute onset mono-or poly-articular arthritis generally affecting large joints, but occasionally small joints
- episodes usually transient lasting 7-10 days
- can be relapsing/remitting.
- associated with fever, erythema nodosum, vasculitic rash.
- rheumatoid factor negative, almost invariably non-erosive on radiology
- symptomatic with NSAID, but may require intra-articular steroid injections and disease-modifying anti-rheumatic drugs
Hypertrophic Pulmonary Osteo-Arthropathy (HPOA)
HPAO rarely occurs before 10 years of age, and median age of onset is around 20 years. The aetiology remains unknown, but may relate to humoral or neurogenic responses to chronic infection/inflammation and/or hypoxia. Onset of HPOA is associated with increased risk of mortality.
- pain and/or swelling (effusions) of joints (knees ,wrists, ankles).
- Bone pain with tenderness of ends of long bones, with bony enlargement
- gynaecomastia/ mastalgia
- treat underlying condition: NSAID; bisphosphonates like pamidronate and zolendronate for bone pain; and manage arthritis as per JIA (i.e. consider steroid joint injections)
- Narang A et al. Arch Dis Child. 2003;88:702
- Balfour-Lynn, I.M., Malbon, K., Burman, J.F. and Davidson, S.J. (2005), Thrombophilia in children with cystic fibrosis. Pediatr. Pulmonol., 39: 306-310. doi:10.1002/ppul.20181
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