Glomerular filtration rate in homozygous sickle cell disease children in steady state and healthy Nigerian children: a comparative study in north-eastern Nigeria

Background: Homozygous sickle cell disease (HSCD) is the most common inherited blood disorder of public health importance worldwide, with Sub-Saharan Africa accounting for a third of the global burden. The effect of HbS on the kidneys results in sickle cell nephropathy, which contributes to increased mortality among HbSS patients beyond third decade of life. Glomerular filtration rate (GFR) is an important renal function test for evaluating progress of sickle cell nephropathy, however, this is seldom done to HbSS patients especially in the insurgency that devastated the North-eastern part of Nigeria, where displacement of people has led to increase in diarrhoeal diseases with its complications which also contributes to renal diseases, hence the need for this study. Objective: To determine the baseline glomerular filtration rate of homozygous SCD in steady state and compare same with normal controls. Methods: This is a prospective comparative study conducted at the University of Maiduguri Teaching Hospital (UMTH). The study population consisted of age and sex matched HbSS subjects in steady state and children with haemoglobin AA genotypeaged 3-14 years. The study was conducted over a period of 6 months. Anthropometry and serum creatinine of the subjects were determined and GFR calculated using Schwartz formula. Results: Two hundred and twenty children consisting 110 HbSS and 110 controls were enrolled. This consist of 106 males and 114 females with M:F ratio of 0.9:1. Mean ages of HbSS patients and HbAA subjects were 8.2years and 7.9 years respectively. The mean GFR (SD) was 125.9 (31.9) ml/min/1.73m2 and 93.0 (16.1) ml/min/1.73m2 for the HbSS and HbAA controls, the difference between the means was significant (P<0.001). The normal GFR range for the controls was 77 to 109 ml/min/1.73m2. Sixty-seven (61%) casesand 86 (78%) controls had GFRs within normal range. There was statistically significant difference for GFRs above and below the normal range (Z-score=6.2 & -2.9, p<0.001 & p<0.004). Conclusion: About a third of HbSS children in steady state have elevated GFR, this suggests the presence of moderate renal pathology. Regular monitoring of these children will lead to improvements in management of sickle cell nephropathy and their quality of life

Bone marrow invasion by aspergillus specie in a sickle cell trait patient with invasive aspergillosis: a fatal case in association with disseminated intravascular coagulation

Background: Invasive aspergillosis has been predominantly associated with pulmonary infection, particularly amongst immunocompromised individuals. Extrapulmonary infections with Aspergillus specie have been reported rarely irrespective of immune status. Risk factors for invasive aspergillosis include prolonged and severe neutropenia, haematopoietic stem cell and solid organ transplantation, advanced AIDS, and chronic granulomatous disease. The most frequently involved specie is Aspergillus fumigatus that constitutes over 90% of cases, followed by Aspergillus flavus, usually associated with a primary skin infection. Haematogenous spread to the bone causing osteomyelitis is the commonest form of disseminated aspergillosis and a surprisingly high proportion of these patients have no immunosuppression. We present a rare case of bone marrow invasion by Aspergillusspp. in a 3-year-old patient with sickle cell trait and chronic Aspergillosis. Case report: A 3-year-old patient with sickle cell trait was brought to the paediatric unit with recurrent diarrhoea, abdominal distention, weight loss and persistent cough. The child was severely wasted with generalised peripheral lymphadenopathy. She had marked respiratory distress and hepatosplenomegaly but no demonstrable ascites. Haematologic examination revealed leukaemoid reaction (leukocyte count of 44.0 x 109/L) with monocytosis (10%) and thrombocytopenia (platelet count of 97,000/mm3); no blast cells were seen on blood film. The bone marrow was hypercellular with a myeloid/erythroid ratio of 20:1, consistent with infection. Bone Marrow culture yielded Aspergillus spp. and other results of sepsis work up were negative. Conclusion: Cases of extrapulmonary invasive aspergillosis have been reported rarely in both immunocompetent and immunocompromised patients. Haematogenous spread to the bone is the commonest form of disseminated disease