Haemophilia patients aged 0 - 18 years in the Western Cape

Objectives. To record the number of haemophiliacs aged 0- 18 years in the Western Cape (WC), what event led to the diagnosis, the level of clotting factor, treatment, functional status of their joints and impact of the disease on the family.Design. A prospective study of patients registered with the South African National Haemophilia Registry and new patients, utilising the patients' paediatricians, hospital records, patient and guardian interviews, physicalexamination and provincial nurse haemophilia co-ordinators.Setting. Haemophilia care centres at the three WC academic hospitals, regional hospitals and homes of patients. Two elective medical students, MHH and JJH, collected the information.Subjects. All boys with confirmed haemophilia A or B in the WC.Outcome measures. Events that led to diagnosis, degree of haemophilia, use of clotting factor, functional status, and effect on family.                                                                                                                                       Results. Of 78 patients (59 haemophilia A, 19 haemophilia B) identified, 49 could be studied. Forty-three per cent had severe, 29% moderate and 22% mild disease (6% unknown). Family history was present in 49%, but led to diagnosis in only 12%. The most common first symptoms were subcutaneous and mucosal bleeding. Delay in diagnosis varied from 0 to 9 months. Twenty-nine per cent of guardians were suspected of child abuse. RSA produced clotting factor was used 'on demand' in 73% of patients, for periodic prophylaxis in 20% and as continuous prophylaxis in 7%. Joints were functionally restricted in 43% of patients. The majority of guardians (59%) said the disease had a major impact on the family.Conclusions. The diagnosis of haemophilia in children with a positive family history was often delayed. Haemophilia causes significant morbidity in our patients and their families

Persistent groin pain following a trans-obturator sling procedure for stress urinary incontinence: a diagnostic and therapeutic challenge

Groin pain after a tension-free vaginal tape-obturator (TVT-O) procedure can occur but mostly disappears within 4 weeks. Persistent groin pain is extremely rare and there is a paucity of literature on how to diagnose and manage this adverse event. We present two cases with severe persistent groin pain after uncomplicated TVT-O, in which magnetic resonance imaging and electromyography did not reveal the cause. We concluded that the tape entrapped or cut through peripheral branches of the obturator nerve. We removed as much of the tape as possible in both cases. Removal partially relieved the pain although sensory loss of the obturator nerve persisted 1 year after surgery. In case of abnormal post-operative groin pain, soon removal of the tape enhances the chance that damage to the obturator nerve is reversible, although it is important to counsel patients with similar pathology that recovery can take long and may be only partia