Echocardiographic Left Ventricular Hypertrophy and Geometric Patterns in Patients with Sickle Cell Anaemia.

BACKGROUND: Left ventricular hypertrophy (LVH) is a common complication in patients with sickle cell anaemia (SCA), and it has been associated with systolic and diastolic dysfunction, and sudden death. There is a wide variation in the reported prevalence of LVH in patients with SCA, partly due to the varying criteria applied, and the impact of small weight and body surface area (BSA) in SCA patients. We used four different criteria to determine echocardiographic LVH and geometric patterns in patients with steady-state SCA. Left ventricular hypertrophy was defined by LVM, LVM indexed to BSA, LVM indexed to height and LVM indexed to height2.7 using gender-specific reference values. Left ventricular geometry was determined using LVH and relative wall thickness. RESULTS: Eighty-two patients with steady-state SCA, aged 18years and above were studied from January 2018 to April 2018. The median [IQR] age of the patients was 23 [10] years. Forty-seven (57.3%) were females. The prevalence of LVH was highest when LVM was indexed to BSA (80.5%), followed by LVM indexed to height (73.2%). Comparable prevalences of 68.3% and 69.5% were observed using LVM and LVM indexed to height2.7, respectively. The prevalence of LVH was similar in males and females for all the criteria. CONCLUSION: The prevalence of LVH is high among patients with steady-state SCA irrespective of the criteria applied. The most prevalent geometric pattern was eccentric LVH. Indexing to BSA might result in over-estimation of LVH given the relatively small BSA in patients with SCA. Indexing to height 2.7 might give a more accurate estimate of LVH.

CONTEXTE: L'hypertrophie ventriculaire gauche (HVG) est une complication fréquente chez les patients atteints d'anémie falciforme (ACS), et elle a été associée à un dysfonctionnement systolique et diastolique, ainsi qu'à une mort subite. La prévalence de l'HVG chez les patients atteints d'anémie falciforme varie considérablement, en partie à cause des différents critères appliqués et de l'impact du petit poids et de la surface corporelle (BSA) des patients atteints d'anémie falciforme. Nous avons utilisé quatre critères différents pour déterminer l'HVG échocardiographique et les schémas géométriques chez les patients atteints d'ACS à l'état stable. L'hypertrophie ventriculaire gauche a été définie par la MVL, la MVL indexée sur la surface corporelle, la MVL indexée sur la taille et la MVL indexée sur la taille2,7 en utilisant des valeurs de référence spécifiques au sexe. La géométrie du ventricule gauche a été déterminée en utilisant l'HVG et l'épaisseur relative de la paroi. RÉSULTATS: Quatre-vingts deux patients atteints d'ACS à l'état stable, âgés de 18 ans et plus ont été étudiés de janvier 2018 à avril 2018. L'âge médian [IQR] des patients était de 23 [10] ans. Quarantesept (57,3 %) étaient des femmes. La prévalence de l'HVG était la plus élevée lorsque la MVL était indexée sur la BSA (80,5 %), suivie de la MVL indexée sur la taille (73,2 %). Une prévalence comparable de 68,3 % et 69,5 % a été observée en utilisant la MVL et la MVL indexée sur la taille2,7, respectivement. La prévalence de l'HVG est similaire chez les hommes et les femmes pour tous les critères. CONCLUSION: La prévalence de l'HVG est élevée chez les patients atteints d'ACS à l'état stable, quel que soit le critère appliqué. Le modèle géométrique le plus répandu est l'HVG excentrique. L'indexation à la BSA pourrait entraîner une surestimation de l'HVG étant donné la BSA relativement faible chez les patients atteints d'ACS. L'indexation à la taille 2,7 pourrait donner une estimation plus précise de l'HVG. Mots clés: Hypertrophie ventriculaire gauche; Géométrie ventriculaire gauche; Drépanocytose ; Échocardiographie.

Chronic Kidney Disease amongst Sickle Cell Anaemia Patients at the University of Maiduguri Teaching Hospital, Northeastern Nigeria: A Study of Prevalence and Risk Factors.

BACKGROUND: Involvement of the kidneys in patients with sickle cell anaemia is a well recognised chronic complication. This study seeks to determine the prevalence of chronic kidney disease in patients with homozygous sickle cell disease (HbSS) and to identify risk factors associated with its development. METHODOLOGY: The subjects consisted of adolescents and adults with HbSS recruited sequentially from the adult haematology outpatient clinic and Daycare ward of the unit. Clinical variables including age at diagnosis of SCA, the frequency of vaso-occlusive crisis and transfusion therapy, as well as laboratory data including haematological profile and renal function tests were obtained. The glomerular filtration rate was estimated (eGFR) using the 'modification of diet in renal disease' (MDRD) formula. RESULTS: Two hundred and eighty-four HbSS patients were recruited. The prevalence of CKD amongst them was 38.9%. Further stratification of the patients based on eGFR showed that sixty-nine (26.8%) had hyperfiltration; 35 (13.6%) stage 1 CKD; 53 (20.6%) stage 2 CKD; 33 (12.8%) stage 3a CKD; 28 (10.9%) stage 3b CKD; 30 (11.7%) stage 4 CKD and 9 (3.5%) had end stage renal disease. There was significant association between eGFR and clinical parameters such as age (r -0.353, p=0.000), SBP (r -0.148, p= 0.021), DBP (r -0.213, p=0.001) and total number of blood received (r -0.276, p=0.000); and laboratory parameters such as PCV (r 0.371, p=0.000); urea ( r 0.527, p=000); creatinine (r 0.625, p=0.000) and uric acid ( r -0.419, p=0.000). CONCLUSIONS: The present study has revealed a high prevalence of CKD amongst patients with SCA in our region. Clinical and laboratory predictors of CKD using eGFR were identified to include age, SBP, number of units of blood transfusion, PCV, urea, creatinine and uric acid levels.

Frequency of hepatomegaly and splenomegaly in Nigerian patients with sickle cell disease.

BACKGROUND: Hepatobiliary and splenic complications (amongst others) are common in sickle cell disease (SCD) as a consequence of the progressive injury resulting from repeated sickling of HbS red blood cells. OBJECTIVE: To determine the degree and the frequency of persistent hepatomegaly and splenomegaly in relation to determinants of clinical severity in patients with SCD. METHODS: Two hundred and twenty SCD patients in clinical steady state were assessed for the presence and the size of palpably enlarged spleen and liver. Patients with hepatomegaly or splenomegaly were re-assessed after three months. Average size was recorded as well as the electrophoresis pattern, age at asessment, sex and the haematocrit. These were compiled over one year RESULTS: The mean age of the SCD patients was 24.7 (8.7) years. HbS and female subjects predominated, constituting 89% and 57% respectively. Females had statistically significantly higher mean age of years than males, (p < 0.001). Splenomegaly was present in 46 (21%) of SCD patients, of which 39 (84.8%) were HbSS, with a mean splenic size of 8.7 (7) cm. Out of the 220 SCD patients, hepatomegaly was present in 59%, with a mean size of 7.6 (6.5) cm. However, higher frequency and a significantly bigger size of hepatomegaly were found in patients with HbS than in those with HbSC. The mean age of 27.2 (10.9) years and the mean of PCV of 24.1 (4.9) in the 82 (37%) patients without hepatomegaly and splenomegaly were significantly higher (p = 0.001) than the general population of SCD patients studied. CONCLUSION: Hepatomegaly of varying sizes occurs commonly in patients with HbS and probably connotes a severe clinical course. The aversions that there is predominance of HbS, survival advantage of females and the fact that HbSC fair better than HbS remain true.

Benefit of modified plasmapheresis in the management of myasthenia gravis: a case report.

BACKGROUND: Myasthenia gravis is an autoimmune disease condition caused by the generation of antibodies against the acetylcholine receptor sites at the neuromuscular junction. The treatment modalities include anticholinesterase drugs, corticosteroids, immunotherapy, thymectomy and plasmapheresis. However, because of the poor financial state of our patients and the dearth of appropriate equipment in our centres modifications are made to standard treatment modalities including plasmapharesis. METHOD: We report a case of myasthenia gravis who was on various occasions on neostigmine, pyridostigmine and prednisolone. After about 18 months of treatment, he developed myasthenic crises on two occasions. He was admitted in the ICU for respiratory support where he also had modified plasmapheresis. RESULTS: The patient had remarkable improvement following the modified plasmapheresis with reversal of symptoms of the myasthenic crises. CONCLUSION: In the absence of facilities for standard plasmapheresis in this environment, the use of modified plasmapheresis is hereby recommended.

Levels of malaria specific immunoglobulin G in Nigerian sickle cell disease patients with and without splenomegaly.

Fifty patients with sickle cell disease(SCD) reporting for routine clinical evaluation and fifty normal individuals at the University College Hospital (UCH) community were recruited into this study. They were matched for age and sex. Patients were aged 15 to 54 years. Forty-four (88.0%) of the patients were haemoglobin S while 6 (12.0%) were haemoglobin S + C. Blood samples were collected and examined for malaria parasite. Malaria specific Immunoglobulin G (pf-IgG) absorbance reading was estimated by Enzyme linked immunosorbent assay in all the patients. Of the forty-four patients with sickle cell anaemia, twenty-two had palpable spleen while the remainder (twenty-two) did not have palpable spleen, and four of the six with Haemoglobulin S + C had palpable enlarged spleens. Spleens were graded using the Hacketts grading system. Five (10%) had grade I splenic size, seventeen (34.0%) had splenic size grade II, one (2%) had grade III, two (4%) had grade IV splenic size and one (2%) had grade V splenic size. Malarial specific. Immunoglobulin G (pf-IgG) was estimated by the Enzyme linked immunosorbent assay. Thick blood films stained with Giemsa were examined microscopically for malaria parasite. The result revealed the mean plasmodium falciparum specific pf-Ig G absorbance reading in patients was 1.081 +/- 0.66 while that of the control was 1.037 +/- 0.38. The difference was not statistically significant (p > 0.05). The high mean pf-IgG indicated intensive malarial exposure and higher impact of malaria infection in patients with sickle cell disease in this environment.

Quality assessment of platelet concentrate prepared at a tertiary centre in Nigeria.

The need for platelet transfusion has increased at the University College Hospital (U.C.H.) Ibadan, Nigeria because of improved patient care and use of intensive therapeutic regimen associated with bone marrow aplasia. Therefore there is a need to assess the quality of the platelet concentrate prepared in our environment with a view to improving the quality of services available, consequently this study was carried out. Fifty platelet concentrates were assessed for pH, swirling index, volume, platelet count, WBC count and red cell count. All the concentrate preparations were within acceptable pH value of 7.25. The average volume was 18.52 mls/bag. The average platelet count per concentrate was 41.7+/-39.5 x 10(9)/L. Thirty-five percent (35%) of the platelet concentrates had a value > 55 x 10(9)/L. White blood cell count (WBC) < 12 x 10(9)/L was seen 49% of the platelet concentrates prepared. Forty percent (40%) of the platelet concentrate had a red blood cell count (RBC count) > 12 x 10(9)/L with 30% not having red cell contamination. Swirling test was positive in 72% of the platelet concentrate units. The results from this study point to the need to improve the quality of the platelet concentrates being prepared in our blood bank in order to get maximum therapeutic values. There is also a need for regular quality control of the platelet concentrate being prepared in our blood bank.

Waldenstrom's macrogloblinaemia: modified plasmapheresis as treatment option in a Nigeria setting.

We describe a case of Waldenströms macroglobulinaemia in a 56 year old Negroid male, to whom modified plasmapheresis/plasma exchange was offered with good control of symptomatic hyperviscosity Amelioration of the clinical status and objective assessment of response was evident by dramatic reduction in monoclonal immunoglobulins M (IgM) from 100g/L to 14.7 g/L and a fall in erythrocyte sedimentation rate from 130 mm/hr to 80 mm/hr with this therapy. This highlights the need to adopt a modification of manual plasmapheresis in the treatment of the hyperviscosity syndrome complicating this B-cell disease in this environment.