Plasma L-Arginine in Sickle Cell Anaemia Patients in Crises and its Correlation with Markers of Severity of Disease.

INTRODUCTION/OBJECTIVES: Sickle Cell Anaemia (SCA) is a chronic haemolytic and inflammatory disorder characterized by repeated vaso-occlusive (VOC) and hyperhaemolytic crises (HC). These crises determine the quality of life of SCA patients. This study estimated the plasma L-arginine levels in SCApatients during crises (HC and VOC), and correlated these levels with the markers of inflammation in the patients. METHODS: A cross-sectional study comprising of 120 consenting adults (60 SCA patients and 60 HbAA controls). The SCA were grouped as SCA patients in VOC or SCA patients in HC and HbAA controls. All SCA patients presented at the emergency room or the daycare unit of Haematology Department, University College Hospital, Ibadan, Nigeria. Blood samples were collected and haematological parameters and plasma L-arginine levels were determined. RESULTS: There were 30 SCA patients in VOC, 30 SCA patients in HC, and 60 HbAA controls with age ranging from 18 to 58years. The controls had a higher haematocrit than the SCA patients (HbAA>SCA VOC> SCA HC, p<0.001) in each pair wise analysis. The VOC and the HC groups had higher mean White Blood Cell (WBC), platelet count, Absolute Neutrophil Count (ANC), Absolute Lymphocyte Count (ALC), Absolute Monocyte Count (AMC), and reticulocytes count than the HbAA controls. Similarly, the HC group had higher mean WBC, platelet count,ANC,ALC,AMC, and reticulocytes count, and a lower mean percentage irreversible sickle cell than the VOC group. The mean plasma L-arginine was significantly higher in the HbAA control group than those of SCA patients in crises (HbAA controls>SCAVOC>SCAHC, p < 0.001), and higher in the SCAVOC group than in the HC group. There was no significant correlation between plasma L-arginine levels, reticulocyte count, markers of VOC severity and markers of inflammations. CONCLUSION: The plasma L-arginine levels of SCA patients in VOC and HC were lower than those of the HbAA controls. The degree of reduction was however marked in patients in hyperhaemolytic crisis. There was no significant association between the plasma L-arginine levels and the markers of inflammation in the studied patients. The findings in this study buttress the need for consideration of L-arginine supplementation in patients with SCA, especially during acute hemolytic crises.

INTRODUCTION / OBJECTIFS: La SCA est un mal hémolytique et inflammatoire chronique caractérisé par des crises vaso-occlusives (COV) et hyperhémolytiques (HC) répétées, qui déterminent la qualité de vie des patients atteints de SCA. L'étude a estimé les taux plasmatiques de L-arginine chez les patients atteints de SCA pendant les crises (HC et COV), et a mis en corrélation ces taux avec les marqueurs de l'inflammation chez les patients. MÉTHODES: Une étude transversale comprenant 120 adultes consentants (60 patients SCA et 60 contrôles HbAA) regroupés en patients SCA en COV; Patients SCA à HC (tous les patients SCA présentés à la salle d'urgence ou à l'unité de garde du département d'hématologie, University College Hospital, Ibadan, Nigéria); et contrôles HbAA. Des échantillons de sang ont été prélevés et les paramètres hématologiques et les taux de L-arginine ont été déterminés. RÉSULTATS: Il y avait 30 patients SCA en COV, 30 patients SCA en HC et 60 contrôles HbAA avec un âge allant de 18 à 58 ans. Les témoins avaient un hématocrite plus élevé que les patients SCA (HbAA> SCA VOC> SCAHC, p <0,001) dans chaque analyse par paire. Les groupes VOC et HC avaient des taux moyens de globules blancs, de plaquettes, d'ANC, d'ALC, d'AMC et de réticulocytes plus élevés que les témoins HbAA. De même, le groupe HC avait un nombre moyen de globules blancs, de plaquettes, d'ANC, d'ALC, d'AMC et de réticulocytes plus élevés, et un pourcentage moyen de de drépanocytose irréversible plus faible que le groupe COV. La L-arginine plasmatique moyenne était significativement plus élevée dans le groupe témoin HbAA que chez les patients SCA en crise (contrôles HbAA> SCA VOC> SCA HC, p <0,001), et plus élevée dans le groupe SCA VOC que dans le groupe HC, p <0,001. Il n'y avait pas de corrélation significative entre les taux de L-arginine, le nombre de réticulocytes, les marqueurs de gravité des COV et les marqueurs d'inflammations. CONCLUSION: Les taux plasmatiques de L-arginine chez les patients SCAen COV et HC étaient inférieurs à ceux des contrôles HbAA, et le degré de réduction est cependant marqué chez les patients en crise hyperhémolytique. Il n'y avait pas d'association significative entre les taux plasmatiques de L-arginine et les marqueurs de l'inflammation chez les patients étudiés. Les résultats de cette étude renforcent la nécessité d'envisager une supplémentation en L-arginine chez les patients atteints de SCA, en particulier pendant les crises hémolytiques aiguës. MOTS CLÉS: anémie falciforme, L-arginine, crise vaso-oclussive, crise hyperhémolytique.

Haematology Consultations in a Tertiary Health Care Centre in Nigeria.

BACKGROUND AND OBJECTIVES: The ease with which investigations are done in this technological age increases the possibility of finding abnormal haematological values, which inevitably leads to haematologic consultations. The Department of Haematology at the University College Hospital (UCH), Ibadan provides a busy consultative service for in-patients and out-patients suspected of having a haematological disorder. This study reviewed the consultations received from different clinical departments to Consultant Haematologists at our tertiary hospital. METHODS: Analysis of consultation requests on in-patients received in the Haematology department, UCH between June 2015 and January 2016 was done. During this period, the department had six Haematologists and nine resident doctors. SPSS version 22 was employed for the statistical analysis. RESULTS: A total of 285 consultation requests were received during the study period, with an average of 36 consults per month. The highest number was received in July 2015. The patients reviewed had a mean age of 36.9±19 years, and requests on female patients exceeded those on males, with a M:F ratio 1:1.5. Consultation for a presumptive diagnosis of sickle cell disease (SCD) were the commonest indication (17%). The highest consultation was from Obstetrics and Gynaecology (O&G) (25%) while the least was from Radiation Oncology (0.4%). CONCLUSION: There is an increasing role for the Haematologist in sub-Saharan Africa as evidenced by the high frequency of consultations observed in this review. The management team of each teaching hospital should bear in mind the need to have adequate number of Haematologists so as to ensure optimal haematological services.

PATTERN OF ANAEMIA AND ITS CORRELATES IN NIGERIANS WITH HEART FAILURE.

BACKGROUND: Heart failure often coexists with many comorbidities, including anaemia. However, the pattern of anaemia in heart failure and its clinical and echocardiographic associations have not been adequately studied among Nigerians. OBJECTIVE: To describe the pattern of anaemia, its clinical characteristics, and its echocardiographic associations among heart failure subjects in Nigeria. METHODOS: One hundred and forty subjects with heart failure were recruited from the cardiology clinics of two teaching hospitals in southwest Nigeria: Ladoke Akintola University of Technology and Bowen University Teaching Hospitals, Ogbomoso. Complete blood analyses, among other tests, were done. Statistical analysis was done with Statistical Package for the Social Sciences (SPSS) 20.0. P <0.05 was taken as statistically significant. RESULTS: Anaemia, as defined by the World Health Organisation, occurred in 106 (75.7%) of the heart failure patients. The patterns of anaemia among participants include combined anaemia of chronic diseases (ACD) with iron deficiency in 64 (45.7%) patients, and ACD alone in 40 (28.6%). Anaemia was more significantly associated with previous diagnosis of diabetes mellitus, presence of pulmonary hypertension, and heart failure with reduced ejection fraction. Mean systolic and diastolic blood pressures, ejection fraction, and fractional shortening were significantly lower among heart failure subjects with anaemia, while serum creatinine, left atrial dimension, left ventricular end diastolic dimension, and left ventricular mass index were significantly higher among heart failure subjects with anaemia compared to those without anaemia. CONCLUSION: Anaemia occurs very frequently among heart failure patients in southwest Nigeria. It is associated with many poor prognostic factors, including diabetes mellitus, pulmonary hypertension, and kidney failure.

Circumcision Rates after the Release of Preputial Adhesions

Aim We aim to determine if the release of preputial adhesions (RPA) successfully managed symptoms related to physiological phimosis and prevented the need for circumcision. Methods We performed a retrospective review and analysed data on RPA procedures performed between January 2005 and December 2017. Results 534 RPA's were performed. Median age at RPA was 52.7 months (range: 3-197 months). Mean follow-up was 108 months (range: 4.7 to 152.4 months). 44 children and 1 child subsequently required a circumcision or preputioplasty respectively (8.4% incidence). There was no statistical difference in the circumcision rates in children who had RPA over 5 years vs those that had RPA under 5 years old (6.6% vs 9.8%; p = 0.21). The histology of the 44 excised foreskins showed BXO in 2 (4.5%) and chronic inflammation in 11 (25%). Conclusion RPA is an effective alternative to circumcision where either reassurance on the benign and self-limiting nature of physiologic phimosis or steroid treatment are unsuccessful in managing symptoms.

Hypereosinophilic atopic transverse myelitis.

Atopic transverse myelitis is a rare disorder that is defined as a localized myelitis of an unknown cause in patients with either high immunoglobulin E (IgE) level or mite-specific IgE or coexistent atopic disease. It is a cause of intramedullary cord lesions, but its diagnosis does not need tissue confirmation. We report a case of a patient who presented with bladder and anal incontinence, paresthesia, and lower limb weakness. Neither IgE level nor mite-specific IgE level could be determined due to lack of fund; however, magnetic resonance imaging (MRI) of the thoracolumbar region showed hypotense-isotense lesion within the spinal cord at T4 vertebral level, suggestive of transverse myelitis. Blood cell count showed hypereosinophilia. Therefore, a diagnosis of atopic transverse myelitis based on high eosinophil count and MRI was made. Patient was commenced on prednisolone and had good response to treatment. Complete blood count is a cheap simple diagnostic tool in resource-poor country to distinguish atopic transverse myelitis from other causes of intramedullary cord lesions.

Hypereosinophilic syndrome with severe hypokalaemia in a Nigerian woman: A case report.

Introduction: Hypereosinophilic syndrome (HES) is a rare disorder. It is defined as eosinophilia of greater than1.5x109 /L persisting for at least 6 months or death before 6 months without an identifiable cause and with eosinophil-mediated organ dysfunction. We present a rare case of hypereosinophilic syndrome with severe hypokalaemia in a Nigerian female patient. Case Presentation: A 43year old food vendor referred to the Haematology Department, University College Hospital, Ibadan on account of a 6-week history of cough productive of mucoid, brownish, foul smelling sputum with associated breathlessness, high grade intermittent fever, and intense pruritus. She had accompanying non-projectile,non-bloody vomiting of recently ingested meals. There was absolute eosinophilia of 83x109/L and bone marrow cytology revealed marked eosinophilia with blasts of less than 5%. She also had asymptomatic severe hypokalaemia (1.9mmol/l) likely due to vomiting and reduced dietary intake. The aetiology of the hypereosinophilia could not be ascertained.She was admitted and commenced on intranasal oxygen, Tabs Loratidine, intravenous hydration.The severe hypokalaemia was corrected with IV KCL over 48hours followed with the administration of slow K tablets 600mg tds. She also had tabs Hydroxyurea for cytoreduction and Allopurinol to prevent hyperuricaemia. She improved with the above line of management. Conclusion: This appears to be the first reported case of HES with asymptomatic severe hypokalaemia in the literature. Being a rare disorder it could easily have been missed without a review of the peripheral blood film and marrow aspirate. This finding suggests a possible relationship between hypereosinophilia and hypokalemia which needs to be explored.

A CASE OF PICA FOLLOWING A PARAMILITARY PRE-EMPLOYMENT ORIENTATION CAMP.

BACKGROUND: Pica is an unusual craving for and ingestion of either edible or inedible substances first observed in pregnant women but has been reported in all groups of people. Pica poses a significant health risk that often requires medical attention but the awareness of pica among physicians seems to be have reduced. METHOD: We report a case study of a young lady who presented with anaemia (pcv = 21%) and craving for sand. Laboratory investigations carried outwere in keepingwith iron deficiency anaemia. CONCLUSION: It is therefore postulated that pica may actually be a sign of iron deficiency anaemia.

Terminal ileum and total colonic duplication associated with a rectovestibular fistula in a child.

The presence of terminal ileum and complete colonic duplication associated with a rectovestibular fistula, caecal diverticulum and multiple appendixes in a child presents an extremely rare diagnostic and management conundrum. We report our surgical approach to successfully correcting this anomaly.

Trans-cervical, trans-tracheal approach to proximal tracheo-oesophageal fistula: a novel technique.

Esophageal atresia and tracheo-esophageal fistula (TEF) occur in 1/2400-4500 births. Whilst the diagnosis of esophageal atresia is readily made shortly after birth, patients with an isolated H type TEF can present with varying degrees of symptomatology which can pose a diagnostic challenge. A combination of contrast esophagogram and endoscopic evaluastion is the most commonly employed localization strategy. Despite accurate pre-operative localization, intra-operative identification of the TEF can prove substantially more challenging. The authors of this report describe a novel approach in the management of a proximal TEF, which allows direct visualization and cannulation via a trans-cervical, trans-tracheal approach.

An unusual cause of acute scrotum in a child.

Acute non-traumatic scrotal pain in children, commonly due to a torted testicular appendage (hydatid of Morgagni) or torted epididymal appendage is well described. These vestigial embryonal duct remnants are of Müllerian and Wolffian duct origin respectively. Very rarely, the other infrequently encountered Wolffian duct remnants known as the paradidymis or organ of Giraldés and the superior and inferior aberrant ducts known as the organs of Haller can become torted. We describe the presentation, management and diagnosis of a torted embryonal remnant arising from the distal spermatic cord.

Delayed diagnosis of anorectal malformation--a persistent problem.

Delayed diagnosis of anorectal malformation (ARM) is an avoidable event associated with significant complications and morbidity. Previous studies have suggested higher than expected rates of delayed diagnosis, especially when a threshold of 24 hours of life is used to define delayed diagnosis. The aim of this study is to highlight the prevalence of delayed diagnosis of ARM in Ireland and to determine if any improvement in rates of delayed diagnosis of ARM has occurred since we previously examined this problem over a 10 year period in 2010. We compared trends in the incidence of delayed diagnosis of ARM between two cohorts, A (1999-2009) and B (2010-2012). Delayed diagnosis was defined as one occurring after 48 hours of life. Delayed diagnosis occurred in 29 cases (21.3%) in total, with no difference in the incidence of delayed diagnosis between cohort A (21 patients [21.2%]) and cohort B (8 patients [21.6%) being recorded. The rate of bowel perforation in patients with delayed diagnosis was 10.3% (3 cases). Our findings highlight the importance of a careful, comprehensive clinical examination in diagnosing ARM and suggest this is still sub-optimal. We strongly support the use of a nationally devised algorithm to aid diagnosis of ARM in order to avoid life-threatening complications.

Biosorption equilibrium, kinetic and thermodynamic modelling of naphthalene removal from aqueous solution onto modified spent tea leaves.

The object of this study was to investigate the feasibility of using modified spent tea leaves to remove naphthalene from its aqueous solution under batch mode. The effects on the removal process of physical factors, such as initial naphthalene concentration, contact time, biosorbent dosage, pH and temperature, have been evaluated. The equilibrium biosorption data were analyzed by the Langmuir, Freundlich, Temkin and Dubinin-Radushkevich (D-R) adsorption isotherm models. These models provided a good fit to the experimental data, but the Langmuir isotherm model provided the best correlation (R2 = 0.993) to the experimental data. The biosorption kinetic data of naphthalene were analyzed by pseudo-first-order, pseudo-second-order and intra-particle diffusion and surface mass transfer kinetic models. These four kinetic models fitted the biosorption kinetic data well, but the pseudo-first-order kinetic model gave the best fit. The activation energy (E(a)) was found to be 15.89 kJ per mole and the thermodynamic properties of the biosorption process, such as the Gibbs free energy, enthalpy and the entropic change of biosorption, were also evaluated. It was established that the biosorption process was spontaneous, feasible and endothermic in nature.