In CF patients diagnosed through neonatal screening, P aeruginosa pulmonary infections occurred 6 to 12 months before the organism was isolated from respiratory secretions. The longitudinal monitoring of P aeruginosa antibody titers, in concert with WCXR score, should facilitate diagnosis and treatment of P aeruginosa pulmonary infections in young children with CF.
Transfusion-dependent anaemia and portal hypertension are recognised complications of hereditary haemorrhagic telangiectasia (HHT). The anaemia is a result of chronic bleeding from gastrointestinal telangiectasias, which are usually multiple and located throughout the gastrointestinal tract. As a result, treatment with argon plasma coagulation via gastroscopy and or colonoscopy is often insuffi cient to prevent ongoing blood loss. Portal hypertension in HHT occurs as a result of blood shunting between the hepatic artery and the portal vein within the liver. The somatostatin analogue octreotide has been used as a treatment for bleeding angiodysplasia; however, its possible role as a treatment for diuretic intractable ascites secondary to portal hypertension has not been previously established. The authors report a case that apparently illustrates a dual benefi t of long-acting octreotide in the management of both occult bleeding and refractory ascites in a patient with HHT. Case reportAn 86-year-old man presented in March 2009 with breathlessness and a haemoglobin (Hb) count of 6.4 g/dl (mean cell volume 91 fl). There was no history of haematemesis or melaena. He was diagnosed with hereditary haemorrhagic telangiectasia (HHT) 40 years ago and also had hypertension and type II diabetes mellitus. On admission, he was taking amlodipine, metformin and ramipril but no antiplatelet agent. He did not drink alcohol to excess. Physical examination was unremarkable apart from buccal telangiectasia. His liver function tests were mildly deranged (alanine transaminase 18 IU/litre, alkaline phosphatase 519 IU/litre, bilirubin 15 mmol/litre); in addition, he had mildly impaired clotting (prothrombin time 15.9 seconds) and a low albumin of 23 g/litre. An upper gastrointestinal endoscopy showed multiple actively bleeding telangiectasias in the gastric antrum, which were injected with epinephrine. He then had repeat endoscopies at monthly intervals over the following 5 months. On each occasion, he received argon plasma coagulation treatment directed at the gastric telangiectasias, which became progressively smaller in size and number; by the sixth endoscopy, there was just a single, small, nonbleeding lesion (figure 1), but on this occasion, grade 1 oesophageal varices without any stigmata of recent haemorrhage were also noted.Despite eradication of the gastric telangiectasia and continuous oral iron therapy, he still required two units of blood transfusion every 3-4 weeks in order to maintain a Hb count of 9 g/dl. We did not pursue colonoscopy or capsule endoscopy because we felt the patient was unfit for further invasive investigation. A CT pneumocolon was performed (figure 2), which excluded large bowel malignancy but did show evidence of portal hypertension with a small cirrhotic liver containing multiple telangiectasias. Three months following the initial presentation, he developed ascites and bilateral leg oedema. A diagnostic ascitic tap showed a transudate sample with normal cell counts and negative cytology. Blood...
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