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Vitamin K deficient bleeding in cystic fibrosis
  1. T Verghese,
  2. D Beverley
  1. York District Hospital, Wigginton Road, York YO31 3HE, UK; David.W.Beverley{at}york.nhs.uk

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We would like to report a female infant (initially breast fed and subsequently formula fed) who had received two 1 mg doses of vitamin K orally, and presented at 9 weeks of age with large haematomas at the sites of her primary immunisations. Her weight had dropped from the 25th to 50th centile at birth to the 2nd centile.

Her haemoglobin was 720 g/l, white blood cell count 13×109/l, platelet count 523×109/l, prothrombin time >10 seconds (normal range 0.8–1.2), activated partial thromboplastin time 109.4 seconds (normal range 24.0–34.0), and fibrinogen 4.5 g/l (normal range 1.7–4.5). She received 1 mg of vitamin K intravenously and repeat coagulation screen was then normal. Sweat osmolality was 110 mmol/l (normal range 17–80) and 105 mmol/l on repeat testing. No chymotrypsin activity was found in the faeces. DNA analysis confirmed homozygosity for delta F 508.

Vitamin K deficiency can occur in undiagnosed cystic fibrosis (CF) infants due to malabsorption of fat soluble vitamins. It is uncommon, since vitamin K is given to all newborns in the UK. As universal screening for CF is not undertaken in the UK, asymptomatic CF patients can be missed and a bleeding diathesis may be the presenting symptom.

Torstenson and colleagues1 reported three cases of severe life threatening bleeding subsequently diagnosed as CF in infants less than 6 months of age, and Rashid and colleagues2 found that 78% of pancreatic insufficient patients had PIVKA-II concentrations >3 μg/l.

Deficiency of vitamin K in children with CF may be due to inadequate dietary intake, maldigestion, and malabsorption.3 Decreased intestinal synthesis of vitamin K2 following diarrhoeal disease or antibiotic administration can also be a contributing factor.

Our patient developed vitamin K deficient coagulopathy despite receiving oral supplementation and vitamin K from formula feed. The vitamin K deficiency can be attributed to malabsorption secondary to CF and emphasises the need to consider CF as a differential diagnosis in bleeding diathesis presenting in the first year of life. If a universal neonatal screening programme for diagnosing CF had been in place, a potentially life threatening complication may have been prevented.

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