Background: Acute chest syndrome (ACS) is a leading cause of death from sickle cell disease worldwide accounting for about 25% of all deaths. The aim of this study was to determine the prevalence, clinical features and outcome in Port Harcourt, Nigeria. Materials and Methods: A retrospective cohort study during a five year period. Records of all patients with sickle cell anaemia (SCA) admitted into the Wards were examined. Those enrolled for the study satisfied two criteria: 1) lower respiratory tract symptoms and 2) new pulmonary infiltrates on the chest radiograph. Sociodemographics, genotype, clinical and laboratory features, treatment given and outcome were obtained. Data were analysed by descriptive statistics. Variables were compared by students' t-test. P value ≤ 0.05 was regarded as significant. Results: A total of 345 children with sickle cell anaemia were admitted during the 5 year period. Twelve of them had acute chest syndrome (3.5%). Majority 7 (58.3%) of them were under 5 years. There were more males 8 (66.7%) than female 4 (33.3%). The most common clinical features were fever 12 (100%), cough 10 (83.3%), chest pain 5 (41.7%), pulmonary consolidation 12 (100%), and respiratory distress 12 (100%). The admitting diagnosis were bronchopneumonia 6 (50%), severe malaria 3 (25%) and vaso-occlusive crises 3 (25%). There were very high levels of leukocyte. Received ceftriaxone or ampicillin + gentamicin ± oral erythromycin), paracetamol 12 (100%), ibuprofen 8 (66.7%), tramadol 3 (25.0%), pentazocine 8 (66.7%) and blood transfusion 9 (75%). The average length of stay was 7 days (range 4 -14 days). One patient died (8.3%). Conclusion: ACS is not uncommon in children with SCA in Port Harcourt. Education of parents on the need to recognize early symptoms of the disease is essential. Clinicians must be trained to correctly diagnose and manage it promptly and efficiently to avoid its related disastrous consequences.

Background: Priapism is a complication of sickle cell disease (SCD) that if left untreated results in irreversible fibrosis and impotency. The aim of this study was to determine the prevalence, pattern, steady state laboratory parameters of children with priapism and their treatment. Methods: A retrospective study of children with sickle cell disease who were diagnosed with priapism at the Sickle Cell Clinic and the Emergency Ward of the University of Port Harcourt Teaching Hospital (UPTH), Port Harcourt, Nigeria, from 1st August 2010 to 31st July 2015. Demographic as well as steady-state clinical data were extracted from the patients' medical records. Laboratory parameters were aged matched with children (HbSS) without priapism in steady state. Data analysed included age, sex, steady-state packed cell volume, leucocyte and platelet count; and treatment. Results: A total of 345 folders of children with SCD (342, HbSS; 3HbSC) were retrieved during the period under review. Five children were found with diagnosis of priapism. The mean age was 8.0±4.6 (range 3-15years). The mean packed cell volume (PCV), white blood cell count (WBC) and platelet counts were 22±2.7; 8±2.9 and 179.4±25.7 respectively. The mean PCV of children with priapism was significantly (P<0.05) higher than the control. Fever 4(80%), dehydration 2(40%), rigorous physical exercise 1(20%) and emotional disturbances 1(20%) were associated risks factors. Stuttering pattern was the commonest 3(60%). Most 4(80%) of them were managed conservatively with irrigation of saline and adrenaline. None of them had exchange blood transfusion. Two(40%) of them had shunt after intumescence of 5 and10 days respectively. Only 1(20%) had recurrence. None had impotency. Conclusion: The prevalence of priapism among patients with SCD is low (1.5%) in Port Harcourt. High steady state haematocrit values were significantly associated with priapism. Fever and dehydrated were the commonest pre-morbid conditions associated with priapism. The treatment options for all types of priapism were initially conservative but surgical therapy must be available when applicable to circumvent erectile dysfunction.