Epidemiological and clinical study of sickle cell disease at tertiary care centre in Western India

Background: Sickle cell disorders are structural hemoglobinopathies, rendering red blood cells sickle shaped, less deformable and sticky leading to microvascular vaso-occlusion and premature red blood cells destruction which leads to varied clinical manifestations. It leads to lifelong morbidity thus affecting quality of life and contributes to early mortality thereby reducing the key national resources- the healthy workforce. This study was done to evaluate epidemiological and clinical profile of sickle cell disease attending the centre.Methods: This study was cross-sectional, observational study conducted at tertiary care hospital in Gujarat. After taking ethical clearance patients were enrolled as per inclusion and exclusion criteria and epidemiological and clinical profile of sickle cell disease patients was studied.Results: Mean age of sickle cell disease was 22.58 years. It was found in tribal communities of Gujarat like Rathwa, Baria, Tadvi etc. Commonest symptom was musculoskeletal pain (86.84%), followed by jaundice (71.05%), fever, dyspnoea, abdominal pain and chest pain. Most common systemic manifestation was pain crises (60.66%), followed by hemolytic anemia (31.15%), acute chest syndrome (30%), consolidation (11.67%), hepatopathy (10%) and avascular necrosis of hip. (6.56%).Conclusions: Sickle cell disease is seen in younger patients. In Gujarat mainly tribal communities are affected. Major systemic manifestations of sickle cell disease include pain crisis followed by hemolytic crisis, acute chest syndrome, hepatopathy and AVN of hip.

Childhood pneumonia: read the smear and clinch the diagnosis

Childhood pneumonia has a myriad of disease causing organisms. Identifying the etiology often helps us predict the natural course of the illness. We would like to share an interesting child with pneumonia by the hematological manifestation of the disease. Case report of this study is a 8 year old boy who presented with high grade fever for eleven days associated with cough. History of skin rashes which worsened following therapy with penicillin. On Examination child had maculopapular rashes predominantly over the trunk with decreased air entry in the left hemithorax. Chest x-ray done showed left lower lobe consolidation hence treated with cephalosporins and macrolide. Counts revealed falling trend in haemoglobin with high MCV count. Peripheral smear done showed agglutinated RBC'S and occasional nucleated RBC's. Direct Coombs test was positive. With these haematological manifestations child was diagnosed to have Mycoplasma pneumonia which was proven by positive antibodies against Mycoplasma. Child recovered completely and haematological manifestations became passive after four weeks. Cold agglutinin disease is poorly understood affecting 15% of patients with Autoimmune hemolytic anemia. Respiratory tract involvement and extrapulmonary complications manifest in 3-10% and 25% respectively. Antibodies (IgM) against the I antigen on human erythrocyte membranes appear during the course of M. pneumoniae infection and produce a cold agglutinin response. AIHA typically occurs during 2-3rd week after febrile illness with sudden onset of hemolysis which is self-remitting within 4-6 weeks. The conclusion of this study is extra-pulmonary manifestations in a child with pneumonia help in diagnosing the etiology. This in turn helps us like provide rationale management and Predict the natural course of the illness.

Assessment of the prevalence of G6PD deficiency in RBCs of live new-borns born at tertiary care hospital

Background: Glucose-6-phosphate dehydrogenase is one of many enzymes that help the body process carbohydrates and turn them into energy. The mechanism by which G6PD deficiency causes neonatal hyper bilirubin may be due to hemolysis, but other mechanisms like secondary impairment of bilirubin conjugation and clearance by the liver may play a role. Therefore, through this study authors attempt to study the need for a newborn screening program for G6PD deficiency because of high prevalence and high risk of incidence due to consanguineous marriages in India.Methods: This study was a prospective cross-sectional study conducted among 350 consecutively born live new-borns in maternity wards and NICU of Krishna Institute of Medical Sciences and Hospital and Research Centre, Karad, Maharashtra during October 2016 to October 2017.Results: The maximum numbers of newborns were in the age group of 0-10 hours (36.80%), followed by in 11-20 hours (21.80%). The mean age among newborns was 2.86'5.83 hours. Out of 350 cases females were 181 (51.71%) and males (48.29%) and female to male ratio was 1.07:1.Conclusions: G6PD deficiency is one of the major causes of neonatal jaundice within 24 hours of life in new-borns. Hence, neonatal screening for G6PD deficiency could be an alternative to the haemolytic crisis prevention strategy in order to optimize affected young child care and prevention of crisis occurrence by avoiding taking contraindicated foods and drugs.

Haemolytic anaemia complicating the concurrent use of allopurinol & azathioprine after kidney transplantation.

Gout is a common problem in renal transplant recipients but often difficult to treat. Allopurinol can be combined with azathioprine but clinicians should be aware of the need for dose reduction, the potential to measure azathioprine breakdown products and the possible side effects of this combination. Leucopenia is a known side effect but this case report shows that haemolytic anaemia can also occur.

Autoimmune haemolytic anemia in children: A review of 17 cases.

Seventeen children with autoimmune haemolytic anaemia (AIHA) seen at the Department of Paediatrics, Siriraj Hospital during 1977 to 1986 were analysed. The patients were devided into 2 groups, acute and chronic types, according to their response to treatment. In acute type (3 cases) the disease occured within the first year of life, with abrupt onset and severe clinical symptoms but dramatic response to prednisolone. In chronic type (14 cases), the disease appeared mostly at 9-11 years of age, associated with underlying diseases such as systemic lupus erythematosus, thalassaemia or Hodgkin’s disease and poorly responded to prednisolone. Two patients died, from septicaemia in one case and from the uncontrolled underlying disease in another case.