Design and synthesis of novel quercetin metal complexes as IL-6 inhibitors for anti-inflammatory effect in SARS-CoV-2

One of the most common causes of mortality in COVID-19 patients is cytokine release syndrome (CRS). Though several cytokines are involved in CRS, the role of Interleukin 6 is significant. Considering the importance of IL-6 inhibition and the drawbacks of the existing monoclonal antibodies, the present study develops new flavonoid metal complexes as immune boosters targeting IL-6 for SARS-CoV-2 treatment. To identify the potential flavonoids from 152 secondary plant metabolites, PyRx 0.9 tool has been used. The top scorer quercetin was converted into quercetin-oxime. Seven metal complexes (QM-1 to QM-7) were made from quercetin-oxime by utilizing divalent metals such as zinc, copper, magnesium, cobalt, barium, and cadmium. It was assumed that all compounds were moderately soluble and would not penetrate the BBB through in silico ADME studies. However, the in vitro heamolytic research revealed a modest heamolytic effect in all seven complexes. To know the IL-6 inhibitory potential preliminary level, the complexes were screened for cytotoxicity in cell lines MCF-7 which predominantly expresses the IL-6 level. The cytotoxic effects of all complexes were considerable relative to the marketable Nutridac formulation. The complexes quercetin-Zinc (QM1) and quercetin-Zinc-Ascorbic acid (QM7) showed significant cytotoxicity on MCF-7 compared to Nutridac and no cytotoxic toward the normal cell lines.

The Correlation Analysis between the Titer of IgG Anti-A/B Erythrocyte Antibody In Vivo of the Neonate and the Severity of Hemolytic Disease of Newborn / 中国实验血液学杂志

OBJECTIVE@#To investigate the titer of IgG anti-A/B erythrocyte antibody in vivo of the neonate with hemolytic disease of newborn(HDN), and explore its clinical valua in evaluating the severity of HDN.@*METHODS@#300 neonates with HDN, 50 neonates with neonatal hyperbilirubinemiain and 50 healthy neonates were selected as research object and Microtubes Gel Test was used to detect the titer of IgG anti-A/B erythrocyte antibody in vivo. Their clinical data and their mothers' prenatal examination data were retrospectively analyzed. Three hemolysis tests (direct antiglobulin test, free antibody test and release test), irregular antibody screening, and the titer of IgG anti-A/B blood group antibody was determined by serological method. Red blood cells(RBC), hemoglobin(Hb), reticulocytes(Ret) and nucleated red cells were detected by hematology analyzer. Indirect bilirubin and albumin(Alb) were detected by biochemical analyzer. The relationship between the titer of IgG anti-A/B erythrocyte antibody in vivo and the severity of HDN was analyzed.@*RESULTS@#There were six serological diagnosis modes in the HDN group,the difference between modes was statistically significant (P<0.05). The antibody titer relationship between HDN neonates and pregnant women was positive correlation(r=0.8302). The highest antibody titer of release test and free antibody test were 1∶32 and 1∶2, and the difference was statistically significant（P<0.05）. RBC, Hb and Alb in HDN patients were lower than those in neonatal hyperbilirubinemia patients and healthy neonates (P<0.05), and were negatively relevant with antibody titer in vivo (r=-0.8016). Bilirubin content in HDN patients were higher than those in neonatal hyperbiliru binemia patients and healthy neonates group(P<0.05), and was positively relevant with antibody titer in vivo (r=0.8731). The hospital day in HDN patients was significantly relevant with the antibody titer in vivo (r=0.8547), but not with the age, sex, weight and ABO blood types (P>0.05).@*CONCLUSION@#The detection of antibody titer in HDN patients can be used to evaluate the antibody concentration in vivo, predict the ability of antibody to induce erythrocyte hemolysis, and help to judge the serenrity and prognosis of HDN.

Autoimmune Haemolytic Anaemia: A cross sectional study in a Tertiary Haematological Centre (Anemia Hemolitik Autoimun: Kajian Keratan Lintang di Pusat Hemotologi Tertier) / Jurnal Sains Kesihatan Malaysia

@#Autoimmune haemolytic anaemia (AIHA) is a group of disorders wherein autoantibody causes decompensated acquired haemolysis. There has been no epidemiological study of autoimmune haemolytic anaemia (AIHA) in Malaysia. This study retrospectively analysed the epidemiology of AIHA including Evan’s Syndrome in a Tertiary Haematology Centre in Malaysia. Patients diagnosed with AIHA and Evan’s Syndrome at 18 years old and above between 1 January 1994 to 1 October 2020 at the out-patient Haematology Clinic of Hospital Raja Permaisuri Bainun, Ipoh were selected. Patients’ information was retrieved from the outpatient clinic records. A total of 71 patients were included of which predominantly female. The mean age for both genders were comparable. Ethnic stratification revealed AIHA was higher in Malays followed by Chinese and Indian. Warm AIHA was most prevalent at 40.8%, compared to cold AIHA and Evan’s Syndrome (both 23.9%), and mixed AIHA (11.3%). Primary was more common than secondary AIHA followed by Evan’s Syndrome. Approximately half of the secondary AIHA and secondary Evan’s Syndrome were due to SLE. Overall, 67.6% of patients received corticosteroid only and 28.2% combined with immunosuppressant. Individuals at higher age and females have higher risk of developing AIHA and Evan’s Syndrome. The highest prevalence was seen among the Malay ethnic. Primary warm AIHA is the most common type and majority of Evan’s syndrome are secondary to autoimmune diseases.

Reference range for ADAMTS13 antigen, activity and anti-ADAMTS13 antibody in the healthy adult Singapore population

INTRODUCTION@#ADAMTS13 (a disintegrin-like and metalloproteinase with a thrombospondin Type 1 motif, member 13) plays a fundamental role in the regulation of haemostasis and thrombosis. Its deficiency leads to an accumulation of ultra-large von Willebrand multimers, inducing spontaneous platelet aggregation, thrombosis in the microvasculature, and thrombotic thrombocytopenic purpura (TTP), a condition with 90% mortality when left untreated. Prompt quantification of ADAMTS13 antigen, activity and autoantibody plays a crucial role in the diagnosis and management of TTP and can help differentiate it from other thrombotic microangiopathies (TMAs). Reference ranges for ADAMTS13 are generally derived from Caucasian patients. Given that polymorphism in the ADAMTS13 gene can be associated with variable ADAMTS13 levels, we aimed to establish the first reference range in Singapore and provide a crucial laboratory test for institutions here and elsewhere.@*METHODS@#150 healthy voluntary donors (75 men, 75 women) aged 21-60 years, with an ethnic mix mirroring Singapore's population profile, were recruited. ADAMTS13 antigen, activity and autoantibody levels were measured using the fluorescence resonance energy transfer-vWF73 and enzyme-linked immunosorbent assay methodologies.@*RESULTS@#Levels (activity 0.65-1.79 IU/mL, antigen 0.36-1.17 IU/mL, autoantibody 1.4-12.5 U/mL) were not statistically different between the genders and various age groups.@*CONCLUSION@#TTP and TMAs are encountered in a wide range of specialties. The availability of new assays in Singapore will aid clinicians in the timely management of these conditions. Standardising reference ranges established for Singapore against World Health Organization standards allows harmonisation of measurements between laboratories and for future research collaborations.

Adverse reactions to blood transfusion: A retrospective analysis of 814 cases in Qingdao area from 2020 to 2021 / 中国输血杂志

【Objective】 To investigate the incidence and composition of adverse reactions to blood transfusion(ARBT) in Qingdao hospitals in recent years. 【Methods】 The "Statistical Table of Adverse Reactions to Blood Transfusion in Medical Institutions" issued by Qingdao Quality Control Center of Blood Transfusion throughout 2020 to 2021, involving 96 hospitals in the region, were collected and analyzed, including the number, proportion and types of ARBT, as well as the types of blood components transfused. 【Results】 From 2020 to 2021, 296 676 cases of blood transfusion in 96 hospitals occurred, and the incidence of ARBT was 0.27% (814/296 676), of which the incidence of ARBT involving plasma transfusion was 0.17% [accounting for 39.07% (318/814) of all transfusion reactions], involving platelet transfusion was 0.68% [31.08% (259/814)], involving erythrocyte transfusion was 0.11% [27.64% (225/814)] and cryoprecipitation transfusion 0.03% [1.47% (12/814)]. The types of ARBT were anaphylaxis 77.64% (632/814), fever 19.78% (161/814), transfusion-related dyspnea 1.47% (12/814), transfusion-related circulatory overload 0.37% (3/814), purpura 0.25% (2/814) and transfusion-related hypotension 0.25% (2/814), delayed hemolysis 0.12%(1/814) and acute hemolysis 0.12%(1/814), respectively. 【Conclusion】 In recent years, the incidence of ARBT in local medical institutions is lower than that of domestic general level, and the main reactions are anaphylaxis and fever following the transfusion of plasma or (and) platelets.The monitoring and control of ARBT should be strengthened in each hospital with accurate and timely report, and active preventive measures should be taken to control or reduce the incidence of ARBT effectively.

Impact of Transfusion Practice on the Occurrence of Acute Transfusion Reactions in a Malaysian Hospital: A Single-centre Retrospective Study

@#Introduction: Acute transfusion reactions (ATR) are commonly reported in clinical transfusion practice, which may result in significant morbidity and mortality. This study aims to explore the impact of transfusion practice on the prevalence, characteristics, and associated factors of ATR at Hospital Sultanah Bahiyah, Kedah, Malaysia. Methods: This was a retrospective study using records from haemovigilance forms of reported transfusion reactions, E-Delphyn (blood bank system), and E-His (hospital information system). A total of 118 cases of ATR from January 1, 2015 until December 31, 2017 were selected and analysed. For each case, a control was selected from a patient who did not develop ATR. Results: A total of 122,215 units of whole blood and blood component transfusions have been performed and 415 ATR were reported. The prevalence of ATR was 0.34% or one in 294 units transfused. There was a significant association between ATR and red cell concentrate (RCC) stored for more than 14 days (adj OR = 65.29, 95% CI 9.29-458.85). The most common ATR were allergic reactions with 63 cases (53.4%), followed by febrile non-haemolytic transfusion reactions (FNHTR) with 45 cases (38.1%). Allergic reactions were significantly associated with female patients (p = 0.038) and the paediatrics age group (p = 0.038). Multivariate analysis revealed a significant association between FNHTR and RCC stored more than 14 days (p = 0.002). Conclusion: The prevalence of ATR in this hospital was low and associated with RCC stored for more than 14 days. Implementation of pre-storage leucoreduction is recommended.

Markedly raised haemolysis index with upper limit of normal serum potassium levels

@#Introduction: Haemolytic specimens are a frequent occurrence in clinical laboratories, and they interfere with the analysis of many tests. Case report: We describe here an unusual case of leptospirosis complicated by haemolytic anaemia in a 70-year-old man with established kidney failure. He presented with an abrupt onset of shortness of breath, flushing and erythematous rash after completing haemodialysis. The patient’s biochemistry test samples were however rejected twice as they were grossly haemolysed. The integrated auto-verification alert system implemented in the hospital’s laboratory information system alerted the staff of the possibility of in vivo haemolysis. Discussion: The auto-verification alert system effectively distinguishes between in vitro and in vivo haemolysis and as such can be utilised as a diagnostic aid in patients with suspected intravascular haemolysis. Keywords:

Rare presentation of right Adrenal Mass: Extramedullary haematopoiesis in a patient with Thalassaemia Intermedia / Journal of the ASEAN Federation of Endocrine Societies

@#Extramedullary hematopoiesis (EMH) is a rare cause of adrenal mass. We present a 44-year-old woman who has thalassaemia intermedia, referred to Endocrinology clinic for huge adrenal mass. Along with a paraspinal lesion discovered in this patient, the leading diagnosis was EMH. The patient was treated with hypertransfusion and hydroxyurea, which led to a reduction in the size of the right adrenal mass and paraspinal mass. This case highlights the challenges in managing this rare condition. Although EMH is a rare cause of adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder, to avoid unnecessary surgical procedures.

Safety of RhD alloimmunization / 中国输血杂志

【Objective】 To explore the safety of RhD-positive red blood cells (RBCs) immunization schedules in RhD-negative volunteers, so as to facilitate the development of domestic anti-D immunoglobulin. 【Methods】 From January 2018 to April 2020, 23 RhD negative volunteers with informed consent were enrolled and divided into initial immunization group and booster immunization group. The initial immunization included first immunization, second immunization and third immunization. Four groups, i. e. 3 cases of 20 mL, 8 of 30 mL, 6 of 40 mL, and 6 of 50 mL, were involved in initial immunization. After the initial immunization response, booster immunizations were performed every 3 months. According to the anti-D titer before each immunization, the booster immunization doses were set to 0.5, 1 and 2 mL. Whole blood samples of 5mL/ person (time) were collected 24 h and 1 week after each infusion, and the blood routine, liver, kidney and blood coagulation function and anti-D titer were detected. The differences of detection (index) values at 24 h and 1 week after the first immunization and booster immunization in each (dose) group were compared. 【Results】 No statistically significant differences were observed in hemolysis index values (all within the range of medical reference values) 24 h or 1 week after initial immunization among RhD positive RBCs of 20, 30, 40 and 50mL(P>0.05). The differences between the hemolysis index values and the basic values before the immune response (all within the range of medical reference values) after 0.5 or 1 mL booster immunizations were also not statistically different (P>0.05). However, the differences (μmol/L)between total bilirubin levels and the basic values before the immune response (1.55±1.87, 6.29±2.66) were significantly different after 2 mL booster immunization (P<0.05). 【Conclusion】 No risks affecting the safety of RhD negative volunteers was found in the immunization schedule proposed in this study.

Eculizumab como opción terapéutica en paciente con crisis renal esclerodérmica asociada a síndrome hemolítico urémico / Eculizumab as a therapeutic option in patients with scleroderma renal crisis associated with haemolytic uraemic syndrome

RESUMEN La crisis renal esclerodérmica (CRS) es una manifestación rara de la esclerosis sistémica (ES). Se presenta como hipertensión arterial de nuevo inicio, empeoramiento o aceleración de la hipertensión arterial crónica o rápido deterioro de la función renal, frecuentemente acompañada de signos de hemolisis microangiopática. Su relación con el síndrome hemo lítico urémico es infrecuente, existiendo tan solo un caso similar reportado en la literatura. Presentamos el caso de una mujer de 36 arios en tratamiento para ES con cefalea global, pulsátil e intensa, convulsión tónico-clónica, cifras de presión arterial altas, falla renal aguda y hemólisis no autoinmune persistente. La evaluación de ADAMTS13 mostró un 60,6% de actividad. El estudio genético para búsqueda de mutaciones predisponentes para sín drome hemolítico urémico atípico (SHUa) reveló variante homocigota en el gen ADAMTS13, c.3287G>A (p.Arg1096His). Se inició tratamiento con eculizumab, observándose en poco tiempo mejoría de la hemólisis, función renal y estado clínico, con algunos efectos benéficos notorios e inesperados sobre la ES.

ABSTRACT Scleroderma renal crisis (SRC) is a rare manifestation of systemic sclerosis (SSc), presented as hypertension of new onset, worsening and / or acceleration of chronic hypertension, or rapid deterioration of renal function, often accompanied by signs of microangiopathic haemolysis. It is rarely associated with haemolytic uraemic syndrome, and there is only one similar case reported in the literature. The case is presented here of a 36-year-old woman on treatment for SSc with global, pulsatile and intense headache, clonic tonic convulsions, high blood pressure levels, acute renal failure, and persistent non-autoimmune haemoly sis. The evaluation of ADAMTS13 showed 60.6% of activity. The genetic study to search for mutations predisposing to atypical haemolytic uraemic syndrome (aHUS) revealed a homozygous variant in ADAMTS13 gene, c.3287G>A (p.Arg1096His). Eculizumab was star ted, with an improvement being observed in a short time in the haemolysis, renal function, and clinical status, with some notable and unexpected beneficial effects on SSc.

The Bombay Blood Group: How Rare is It? A Case Report and a Review of the Literature

The Bombay blood group is a rare type of blood group which is very distinct from the ABO system and was first discovered in India about five decades ago. This blood group describes individuals who lack the H antigen and thus present with the anti H antibodies in addition to anti A and anti B antibodies. Bombay blood group is sometimes mistaken for blood group O. Thesignificance of this blood group is in its ability to potentially cause fatal blood transfusionreaction and haemolytic disease of the foetus and the neonate. Aim:This case report aims to elucidate the rare occurrence of the Bombay blood group. Methods:We describe a case report of the pregnancy, labour and delivery of a multiparous Indian woman with the Bombay blood groupin her second pregnancy. Results: The index case had an uneventful pregnancy, labour and delivery without the need for blood transfusion. The neonate was Rhesus D positive and did not have any complications. Conclusion:Bombay blood group is a rare blood group which can lead to blood transfusion reactions and haemolytic disease of the foetus and newborn. Pregnancy, labour and delivery should be managed in a unit with the availability of anti H blood to avoid foetal and maternal complications.

Streptococcus pseudoporcinus: a rare cause of bacteraemia in an immune-compromised patient

Streptococcus pseudoporcinus, a ß-haemolytic Streptococcus is known to cause genital infections. Author report a rare case of Streptococcus pseudoporcinus bacteremia in an immune-compromised male patient diagnosed with acute myeloid leukemia eight months back. The organism was identified as a beta hemolytic bacterium which was catalase negative, oxidase positive and bacitracin resistant. Automated methods (VITEK-2) confirmed the organism to be Streptococcus pseudoporcinus.

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.

Postpartum atypical haemolytic uremic syndrome: a rare clinical entity

P-aHUS has incidence of 1 in 25000 pregnancies. It’s characterized by microangiopathic haemolytic anemia, thrombocytopenia and renal failure. Mrs X, 26 year old lady, G2 P1L1 with 39 weeks POG came to emergency of a tertiary care hospital. She underwent LSCS in view of previous caesarean section not willing for trial of labour. Antenatal, intrapartum and immediate post operative period were uneventful. However, she became anuric 36 hours post operatively. Laboratory investigations suggested hemolysis. Complement system evaluation showed decreased complement levels. Diagnosis of p-aHUS was made by taking multidisciplinary approach and renal biopsy. Patient received 4 sessions of plasmapheresis and symptomatic treatment. Gradually her urine output increased and she was discharged with the baby on post operative day 19. Diagnosis of p-aHUS is tricky owing to similar clinical features with many other pregnancy associated conditions. Timely management and diagnosis are imperative to save the mother’s life.

Maternal and perinatal outcome of Evan’s syndrome: a 5 years study in a tertiary care centre

Background: Evans syndrome is a rare autoimmune disorder characterized by simultaneous or sequential presence of a positive antiglobulin test, autoimmune haemolytic anemia (AIHA), and immune thrombocytopenia (ITP). It is characterised by frequent exacerbations and remissions within a chronic course. It was first described by Robert Evans in 1951.  Incidence of AIHA is 1 per 75 - 80,000 and ITP is 5.5 /100000 per general adult population.  Incidence of Evans syndrome is 1.8% to 10% of patients with ITP. Objective was to study the maternal and perinatal outcome of women with Evans syndrome (E).Methods: About 4 antenatal mothers were identified with Evans syndrome at St. Johns medical college and hospital, Bengaluru during the study period of 5 years from July 2013-July 2017. They were followed up during their antenatal, intra natal and postnatal period and outcomes were studied. All patients included in the study fulfilled the criteria for Evans syndrome.Results: There were 4 cases of Evans syndrome, with a total number of deliveries of 11859, during this 5 year study. Incidence was 0.09 per 1000 births. All patients presented with bleeding manifestations ranging from mucosal haemorrhage to subarachnoid haemorrhage (SAH) at the time of diagnosis. All patients were on treatment with either 1st or 2nd line of management with corticosteroids/ azathioprine. None had bleeding during pregnancy after the initiation of treatment. Patients had antenatal complications like preeclampsia 25%, IUGR 25%, oligohydraminos 50%, IUD 25%. 2 patients received platelet transfusions intrapartum. None had intrapartum or postpartum haemorrhage. There were no maternal and neonatal mortality.Conclusions: Evans syndrome in pregnancy is a rare condition and requires multi disciplinary approach involving specialists from obstetrics, neonatology, and hematology. Close maternal and fetal surveillance and management during pregnancy is essential to increase the possibility of a favourable pregnancy outcome in these women.

Auto Immune Mixed Haemolytic anaemia: A rare presentation of SLE

Auto Immune Mixed Haemolytic Anaemia (AIHA) is defined as presence of both warm and cold antibodies against patient’s own red blood cells which is diagnosed by monospecific Direct anti-globulin test. Hereby we report a middle-aged women old women who was a known case of hypothyroidism on regular medication, presented with history of fatigability, exercise intolerance and exertional breathlessness of 1 month duration. The patient was subjected for preliminary investigations, which revealed severe anaemia with hemoglobin of 3.6 g% and an increased reticulocyte count of 12% with normal total leukocyte and platelet counts. Peripheral smear revealed anisopoikilocytosis, nucleated RBCs and schistocytes. Biochemical tests for haemolysis was evaluated which showed and elevated LDH levels (780U/L), and a reduced serum haptoglobulin levels. Liver Function test revealed a total bilirubin of 6.8mg/dl with indirect bilirubin of 5.4 mg/dl with normal liver enzymes. Baseline evaluation of Auto immune haemolytic anaemia with coombs test turned out to be positive. Patient was subjected for Mono specific DAT, Indirect Anti-globulin test (IAT) and antibody screening. Mono specific DAT showed both Anti IgG and anti C3 antibodies. IAT test was positive at 4⁰C and negative at 22⁰C and 39⁰C which confirmed that the AIHA was of mixed warm and cold type. On evaluation for connective tissue diseases, patient serum was reactive for ANA and ds-DNA and found to have Systemic Lupus Erythematosus which is a rarity and was responded to corticosteroids.

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.

A study of clinicopathological profile and treatment outcomes in patients with autoimmune hemolytic anaemia

Background: Autoimmune haemolytic anaemia (AIHA) is relatively uncommon condition with grave consequences, if not diagnosed and treated early. The literature on the clinical outcome and response to treatment is relatively scarce. Aim was to study the clinic-pathological profile and the treatment outcomes in patients with AIHA.Methods: Around 25 patients with AIHA attending a tertiary care hospital over a period of one year were included in the study. The patients were divided based on severity of anaemia and etiology. All the patients data were analysed for the demographic data, clinico -pathological findings and the response to treatment. All the patients data were analysed using SPSS software (version 22).Results: Out of 25 patients, 76% were females and 24% were males. Based on severity of anaemia, 60%, 28% and 8% had severe, moderate and mild anaemia. Around 48% of the patients had thrombocytopenia along with anaemia. 8 (32%) and 17 (68%) patients have primary and secondary AIHAs respectively. In our study the commonest cause for the secondary AIHA was Systemic Lupus Erythematosus (SLE) followed by haematological malignancy, primary Sjogrens, Anti-phospholipid antibody (APLA) syndrome, carcinoma colon and Wilsons disease. Hepatosplenomegaly and lymphadenopathy were present in 36% and 4% respectively. Out of 22 (88%) patients on corticosteroid therapy, 15 (60%) patients responded to corticosteroids alone and 6(24%) patients required corticosteroid plus immunosuppressive therapy.Conclusions: AIHA has to be ruled out in all anaemia patients with indirect hyperbilirubinemia and abnormal peripheral smear. Secondary AIHA is more common than primary. Corticosteroids and immunosuppressive agents are the mainstay of treatment of AIHA.

Beta Thalassemia Major: Overview of Molecular Etiology, Pathophysiology, Current and Novel Therapeutic Approaches

Major beta thalassemia is a severe form of thalassemia caused by the alteration of two beta globin genes resulting in a defective synthesis of hemoglobin. It is characterized by chronic severe anemia, ineffective erythropoiesis (IE) and iron overload. However although the thransfusion and chelation assosciated constitute the basis of the traitement curently recommended, they do not allow always to control the iron overload induced by pathology and repeated transfusions. Hematopoietic stem cell transplantation (HSCT) has proven to be a definitive treatment for beta thalassemia. However, this procedure is confronted to immunological complications and the small nomber of histocompatible donors. In the face of these therapeutic blocks, much research has been undertaken in recent years leading to the development of a number of promising therapeutic strategies in order to reduce the constraints linked to current chronic treatments, and to move towards an access to healing for all patients. Among other three approaches are envisaged and are in the experimental phase: Gene therapy to restore globin chain imbalance, Improve ineffective erythropoiesis and Improve iron dysregulation. In this article we give a view on the pathophysiology, clinical manifestations, genetic origin of beta-thalassaemia major. The second part presents the therapeutic arsenal currently used, and its limits leading to therapeutic impasse. The last part explores the scientific tracks that present a real therapeutic potential in ?-Thalassemia.