There is no dietary strategy that has yet been specifically advocated for haemophilia. Therefore, we sought to assess the effect of the Dietary Approaches to Stop Hypertension (DASH) diet in adolescents with haemophilia. In this parallel trial, forty male adolescents with haemophilia were dichotomised into the DASH group or control group for 10 weeks. The serum high sensitivity C-reactive protein, IL-6, complete blood count (CBC), serum glutamic oxaloacetic transaminase, serum glutamic pyruvic transaminase, partial thromboplastin time (PTT), waist circumference (WC), percentage of body fat, fat-free mass and liver steatosis were measured at the beginning and end of the study. Serum vitamin C was measured as a biomarker of compliance with the DASH diet. The DASH diet was designed to include high amounts of whole grains, fruits, vegetables and low-fat dairy products, as well as low amounts of saturated fats, cholesterol, refined grains, sweets and red meat. Serum vitamin C in the DASH group was significantly increased compared with the control (P = 0·001). There was a significant reduction in WC (P = 0·005), fat mass (P = 0·006), hepatic fibrosis (P = 0·02) and PTT (P = 0·008) in the DASH group, compared with the control. However, there were no significant differences regarding other selected outcomes between groups. Patients in the DASH group had significantly greater increase in the levels of erythrocyte, Hb and haematocrit, as compared with the control. Adherence to the DASH diet in children with haemophilia yielded significant beneficial effects on body composition, CBC, inflammation and liver function.

The mainstay of gene transfer studies is the use of wild-type cDNAs to effect phenotypic correction of diseases. However, this strategy is not feasible for genetic diseases caused either by mutations of large genes or by dominant-negative mutations, or where the regulation of the gene is critical. In this review, we will discuss a novel RNA reprogramming strategy – spliceosome-mediated RNA trans-splicing – where the pre-messenger RNA is modified by the splicing of two independent RNA species. The use of trans-splicing to effect phenotypic change in the hereditary bleeding disorder haemophilia A will be discussed.

Most cases of epistaxis are due to simple causes and are easily treated on an out-patient basis. However, there are some cases where the origin of bleeding is not obvious or arises from an unusual pathological source. The authors describe a case of epistaxis due to a mass in the maxillary antrum that when biopsied showed the histological appearances of a haemophilic pseudotumour. The patient was anticoagulated on warfarin for a cardiac valve replacement and this was thought to be the cause of the ongoing haemorrhage necessary for development of the pseudotumour. Even in haemophiliacs, pseudotumours are rare and we believe this case is unique in that the patient is a non-haemophiliac. The epistaxis was eventually controlled by external beam radiotherapy to the pseudotumour. The management of this case is outlined as well as a review of the literature on haemophilic pseudotumour.

Haemophilic pseudotumour or haemophilic cyst is a rare complication of haemophilia, occurring in one to two per cent of individuals with a severe factor VIII or IX deficiency. We report a case of a haemophilic pseudotumour in a hitherto unreported site, the bony nasal pyramid, and believe this case is also unique on account of it having occurred in a patient with mild haemophilia. The diagnosis and treatment of this rare condition is also reviewed.

Inherited bleeding disorders are frequently considered an absolute contraindication to tonsillectomy and other ENT procedures. Over a 15-year period we have performed ten elective tonsillectomies and five bilateral myringotomies on children with inherited bleeding disorders. All procedures were carried out with the close co-operation of the Haematology Department in the hospital. All patients underwent uneventful surgery. One patient returned after tonsillectomy with a secondary haemorrhage which did not require surgical intervention. We present our team approach to the management of these children and demonstrate that necessary surgical intervention can be undertaken safely in this select group of patients.

We report an unusual case of unilateral vocal fold palsy due to intracranial haemorrhage in a neonate with undiagnosed haemophilia A. Bleeding disorders in neonates are briefly discussed and the importance of a systemic investigation of stridor in children is emphasized.

Haemophilia A is a sex-linked inherited disease in which those affected are usually males, and females are usually asymptomatic carriers. This paper presents a haemophilia A carrier who has a low factor VIII level first, to remind readers that females can have low factor VIII levels with consequent increased tendency to bleed; second, to stress the importance of routine questioning regarding a bleeding tendency in the patient or the patient's family and third, to illustrate the importance of pre-operative diagnosis to allow adequate correction of the defect so that surgery such as adenoidectomy can be undertaken with safety in such a patient.