Hereditary haemorrhagic telangiectasia in a black adult male: case report

Hereditary haemorrhagic telangiectasia, (HHT) or Rendu-Osler-Weber disease is a genetic autosomal dominant disorder that is characterised by telangiectasias, (small vascular malformations), in mucocutaneous tissues and arterial venous malformations, (AVMs), in various internal organs. Although HHT is relatively common in whites, the disorder has been reported to be rare in people of black African descent. Majority of HHT patients present with recurrent epistaxis, which in a significant proportion of patients is severe, warranting repeated blood transfusions and iron supplementation. Telangiectasias are most frequent on the tongue, hands, nose, lips and the gastrointestinal tract (GIT). AVMs occur in internal organs, particularly the lungs, brain, and the liver. Early and correct diagnosis of HHT is crucial as patients derive benefit from certain specific treatment modalities. Besides, AVMs which occur in various organs pose serious complications that may lead to death and therefore require early detection. We report a 55 year old black African male with HHT who presented with severe recurrent epistaxis and haematochezia leading to severe anaemia requiring repeated blood transfusions. His son, daughter and a maternal uncle experience milder recurrent epistaxis. The management of this patient and a brief review of the clinical features and management of HHT is presented. Our aim is to raise awareness of the occurrence of HHT in Kenya, in order to enhance early diagnosis and appropriate management.East African Medical Journal Vol. 85 (8) 2008: pp. 412-41

Maternal and neonatal outcomes after caesarean delivery in the African Surgical Outcomes Study: a 7-day prospective observational cohort study.

BACKGROUND: Maternal and neonatal mortality is high in Africa, but few large, prospective studies have been done to investigate the risk factors associated with these poor maternal and neonatal outcomes. METHODS: A 7-day, international, prospective, observational cohort study was done in patients having caesarean delivery in 183 hospitals across 22 countries in Africa. The inclusion criteria were all consecutive patients (aged ≥18 years) admitted to participating centres having elective and non-elective caesarean delivery during the 7-day study cohort period. To ensure a representative sample, each hospital had to provide data for 90% of the eligible patients during the recruitment week. The primary outcome was in-hospital maternal mortality and complications, which were assessed by local investigators. The study was registered on the South African National Health Research Database, number KZ_2015RP7_22, and on ClinicalTrials.gov, number NCT03044899. FINDINGS: Between February, 2016, and May, 2016, 3792 patients were recruited from hospitals across Africa. 3685 were included in the postoperative complications analysis (107 missing data) and 3684 were included in the maternal mortality analysis (108 missing data). These hospitals had a combined number of specialist surgeons, obstetricians, and anaesthetists totalling 0·7 per 100 000 population (IQR 0·2-2·0). Maternal mortality was 20 (0·5%) of 3684 patients (95% CI 0·3-0·8). Complications occurred in 633 (17·4%) of 3636 mothers (16·2-18·6), which were predominantly severe intraoperative and postoperative bleeding (136 [3·8%] of 3612 mothers). Maternal mortality was independently associated with a preoperative presentation of placenta praevia, placental abruption, ruptured uterus, antepartum haemorrhage (odds ratio 4·47 [95% CI 1·46-13·65]), and perioperative severe obstetric haemorrhage (5·87 [1·99-17·34]) or anaesthesia complications (11·47 (1·20-109·20]). Neonatal mortality was 153 (4·4%) of 3506 infants (95% CI 3·7-5·0). INTERPRETATION: Maternal mortality after caesarean delivery in Africa is 50 times higher than that of high-income countries and is driven by peripartum haemorrhage and anaesthesia complications. Neonatal mortality is double the global average. Early identification and appropriate management of mothers at risk of peripartum haemorrhage might improve maternal and neonatal outcomes in Africa. FUNDING: Medical Research Council of South Africa.Medical Research Council of South Africa