Impact of ABO blood group antigens on residual factor VIII levels and risk of inhibitor development in hemophilia A

Background@#The clinical phenotype of hemophilia A (HA) does not always correlate with severity.Similarly, the presence of inhibitors does not necessarily increase the risk of bleeding.This paradox between clinical and laboratory findings may be partially attributed to non-modifiable factors, such as blood group, which is known to influence FVIII levels in healthy individuals. Our aim was to assess the effect of ABO blood group antigens on FVIII levels across the severity spectrum of HA and risk of inhibitor development. @*Methods@#Data of consecutive patients with HA who visited the coagulation unit of a northern Indian tertiary care hospital between 2010‒2021 were reviewed. Patients with missing blood group data, transfusion histories, or baseline FVIII levels were excluded. @*Results@#Mild, moderate, and severe HA was present in 41 (6.9%), 72 (12.2%), and 479 (80.9%) patients, respectively. There were no differences in the FVIII levels among the various blood groups across the HA severity spectrum. Inhibitors were administered to 35 patients (5.9%). In the multivariate analysis, blood group A was an independent risk factor for the development of inhibitors (adjusted odds ratio 2.70, P =0.04) after adjusting for age at onset of bleeding, FVIII transfusion, age at first FVIII transfusion, and severity of HA. @*Conclusion@#Unlike what is observed in healthy individuals, blood group did not influence residual FVIII levels across the severity spectrum of HA. Patients in group A had a higher risk of developing inhibitors.

Perioperative Dexmedetomidine Reduces Delirium after Coronary Artery Bypass Graft Surgery: A Prospective, Single?blind, Observational Study

Background:Delirium is a commonly seen complication of cardiac surgery. Dexmedetomidine, by its anti?inflammatory properties and other effects, can attenuate postoperative delirium. Aims: The aim of this work was to study the incidence of delirium after coronary artery bypass graft surgery, and to compare the effects of dexmedetomidine and propofol on the incidence of postoperative delirium in coronary artery bypass graft surgery patients. Materials and Methods: A prospective, observational study was conducted on 180 consecutive patients undergoing off?pump or on?pump coronary artery bypass graft surgery. The patients were administered either intravenous dexmedetomidine (n = 90) or propofol (n = 90) after hemostasis was achieved, till they were ready for weaning from the ventilator. The Confusion Assessment Method was used to assess the incidence of postoperative delirium. Measurements and Main Results: A total of 25 (13.8%) patients developed delirium after coronary artery bypass graft surgery. Sedation with dexmedetomidine was associated with a significantly reduced incidence of postoperative delirium (8.9% v 18.9% propofol, P = 0.049). Subgroup analyses showed reduced incidence of postoperative delirium in off?pump patients compared to on?pump coronary artery bypass graft patients (3.3% vs. 20%, P = 0.009 dexmedetomidine group and 11.6% vs. 33.3%, P = 0.047 propofol group respectively). The mean age of the patients who had delirium was significantly more (64.9 ± 8.1 years vs. 52.5 ± 5.8 years, P = 0.046) compared to those who did not have delirium. Conclusion: Administration of dexmedetomidine?based sedation resulted in the reduced incidence of postoperative delirium compared to propofol?based sedation in patients after coronary artery bypass graft surgery

Diagnosis, Treatment and Prevention of Nutritional Anemia in Children: Recommendations of the Joint Committee of Pediatric HematologyOncology Chapter and Pediatric and Adolescent Nutrition Society of the Indian Academy of Pediatrics

Justification: Anemia in children is a significant public health problem in our country. Comprehensive National Nutrition Survey 2016-18 provides evidence that more than 50% of childhood anemia is due to an underlying nutritional deficiency. The National Family Health Survey-5 has reported an increase in the prevalence of anemia in the under-five age group from 59% to 67.1% over the last 5 years. Clearly, the existing public health programs to decrease the prevalence of anemia have not shown the desired results. Hence, there is a need to develop nationally acceptable guidelines for the diagnosis, treatment and prevention of nutritional anemia. Objective: To review the available literature and collate evidence-based observations to formulate guidelines for diagnosis, treatment and prevention of nutritional anemia in children. Process: These guidelines have been developed by the experts from the Pediatric Hematology-Oncology Chapter and the Pediatric and Adolescent Nutrition (PAN) Society of the Indian Academy of Pediatrics (IAP). Key areas were identified as: epidemiology, nomenclature and definitions, etiology and diagnosis of iron deficiency anemia (IDA), treatment of IDA, etiology and diagnosis of vitamin B12 and/or folic acid deficiency, treatment of vitamin B12 and/or folic acid deficiency anemia and prevention of nutritional anemia. Each of these key areas were reviewed by at least 2 to 3 experts. Four virtual meetings were held in November, 2021 and all the key issues were deliberated upon. Based on review and inputs received during meetings, draft recommendations were prepared. After this, a writing group was constituted which prepared the draft guidelines. The draft was circulated and approved by all the expert group members. Recommendations: We recommend use of World Health Organization (WHO) cut-off hemoglobin levels to define anemia in children and adolescents. Most cases suspected to have IDA can be started on treatment based on a compatible history, physical examination and hemogram report. Serum ferritin assay is recommended for the confirmation of the diagnosis of IDA. Most cases of IDA can be managed with oral iron therapy using 2-3 mg/kg elemental iron daily. The presence of macro-ovalocytes and hypersegmented neutrophils, along with an elevated mean corpuscular volume (MCV), should raise the suspicion of underlying vitamin B12 (cobalamin) or folic acid deficiency. Estimation of serum vitamin B12 and folate level are advisable in children with macrocytic anemia prior to starting treatment. When serum vitamin B12 and folate levels are unavailable, patients should be treated using both drugs. Vitamin B12 should preferably be started 10-14 days ahead of oral folic acid to avoid precipitating neurological symptoms. Children with macrocytic anemia in whom a quick response to treatment is required, such as those with pancytopenia, severe anemia, developmental delay and infantile tremor syndrome, should be managed using parenteral vitamin B12. Children with vitamin B12 deficiency having mild or moderate anemia may be managed using oral vitamin B12 preparations. After completing therapy for nutritional anemia, all infants and children should be advised to continue prophylactic iron-folic acid (IFA) supplementation as prescribed under Anemia Mukt Bharat guidelines. For prevention of anemia, in addition to age-appropriate IFA prophylaxis, routine screening of infants for anemia at 9 months during immunization visit is recommended.

Seroprevalence of Covid-19 infection among healthcare workers: A study from a tertiary care hospital in the National Capital Region of India

BACKGROUND Seroprevalence studies on severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection can provide information on the target populations for vaccination. We aimed to evaluate the seroprevalence among healthcare workers (HCWs) at our tertiary care institution and to identify parameters that may affect it. METHOD We assessed seroprevalence of SARS-CoV-2 by the chemiluminescence immunoassay test among 3258 HCW in our hospital and evaluated as per gender, age, their previous Covid-19 diagnosis, role in hospital and type/risk of exposure. RESULTS Of 3258 participants, 46.2% (95% CI 44.4%– 47.9%) were positive for SARS-CoV-2 IgG antibodies (i.e. IgG ?15 AU/ml). Higher seroprevalence was seen in non-clinical HCWs (50.2%) than in clinical HCWs (41.4%, p=0.0001). Furthermore, people with a history of Covid-19 were found to have significantly higher antibody levels (p=0.0001). Among the HCWs, doctors and nurses had lower relative risk (RR) of acquiring Covid-19 infection (RR=0.82; 95% CI 0.76–0.89) compared to non-clinical HCWs. CONCLUSION Seroprevalence in HCWs at our hospital was 46.2%. Clinical HCWs had lower seroprevalence compared to non-clinical HCWs. Previous history of Covid-19 almost doubled the seropositivity, particularly in those with current infection.

Safety, immunogenicity & effectiveness of the COVID-19 vaccine among healthcare workers in a tertiary care hospital

Background & objectives: The COVID-19 pandemic has caused significant global morbidity and mortality. As the vaccination was rolled out with prioritization on healthcare workers (HCWs), it was desirable to generate evidence on effectiveness of vaccine in prevailing real-life situation for policy planning. The objective of the study was to evaluate the safety, effectiveness and immunogenicity of COVID-19 vaccination among HCWs in a tertiary care hospital. Methods: This prospective observational study was undertaken on the safety, immunogenicity and effectiveness of the ChAdOx1 nCoV- 19 coronavirus vaccine (Recombinant) during the national vaccine roll out in January-March 2021, in a tertiary care hospital, New Delhi, India. Results: The vaccine was found to be safe, with local pain, fever and headache as the most common adverse events of milder nature which generally lasted for two days. The adverse events following vaccination were lower in the second dose as compared to the first dose. The vaccine was immunogenic, with seropositivity, which was 51 per cent before vaccination, increasing to 77 per cent after single dose and 98 per cent after two doses. Subgroup analysis indicated that those with the past history of COVID-19 attained seropositivity of 98 per cent even with single dose. The incidence of reverse transcription (RT)-PCR positive COVID-19 was significantly lower among vaccinated (11.7%) as compared to unvaccinated (22.2%). Seven cases of moderate COVID-19 needing hospitalization were seen in the unvaccinated and only one such in the vaccinated group. The difference was significant between the fully vaccinated (10.8%) and the partially vaccinated (12.7%). The hazard of COVID-19 infection was higher among male, age >50 yr and clinical role in the hospital. After adjustment for these factors, the hazard of COVID-19 infection among unvaccinated was 2.09 as compared to fully vaccinated. Vaccine effectiveness was 52.2 per cent in HCWs. Interpretation & conclusions: ChAdOx1 nCoV-19 coronavirus vaccine (Recombinant) was safe, immunogenic as well as showed effectiveness against the COVID-19 disease (CTRI/2021/01/030582).

Association of level of IL-6, IL-10, IL-18, tumour necrosis factor-?with rheumatic mitral stenosis and subsequent pulmonary hypertension

Background:Objective of the study was to provide insight on the immune response in patients of rheumatic heart disease, mitral stenosis and evaluation of various cytokines in pulmonary hypertension secondary to rheumatic heart disease.Methods:Total 163 subjects, more than 18 year of age, were enrolled in this study. 84 subjects with rheumatic mitral stenosis (group A) diagnosed on two-dimensional echocardiography (2D echo)and 79 normal healthy volunteers(group B). Patients with mitral stenosis were further divided into subgroups based on severity of mitral stenosis [mitral valve area (MVA >1 cm2 and MVA <1 cm2)(subgroup Aa and Ab)]and presence or absence of pulmonary hypertension [pulmonary arterial systolic pressure (PASP >36 mm Hg)(subgroup Ac and Ad)]. Interleukins IL-6, IL-10, IL-18, tumour necrosis factor alpha (TNF-?)andhigh-sensitivity C-reactive protein (hs-CRP)levels were assessed in both groups.Results:Mean IL-6, IL-10, IL-18, TNF-? and hs-CRP in group A and group B was 6.57�53 and 2.73�p?0.001), 8.185�8 and 3.51�86(p?0.001), 136.31�.0 and 47.96�76(p?0.001), 21.26�.59 and 5.36�57(p?0.001), 4.69�3 and 2.63�22(p?0.008) respectively. On subgroup analysis mean TNF-? in subgroup Aa was 20.71�.84, while in subgroup Ab was 7.56�93 (p?0.001). Mean IL-10 in subgroup Ac and Ad was 8.74�29 and 7.47�82, respectively. Differences in levels of other cytokines in these subgroups were not found statistically significant.Conclusions:This study finds increased IL-6, IL-10, IL-18, TNF-? and hs-CRP levels in subjects with rheumatic mitral stenosis. Subjects with severe mitral stenosis had increased TNF-? levels. Subjects of mitral stenosis having pulmonary hypertension had increased IL-10 levels.

Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology

ABSTRACT Background Fibrous hamartoma of infancy (FHI) is a rare soft tissue lesion arising as a subcutaneous mass involving the axilla, trunk, and upper arm in infants and children <2yrs. Sarcomatous transformation in FHI is described in anecdotal cases in the literature. Case Report We describe one such example arising as a mass in the lower back in a 3-month-old infant. On histology, the tumor contained classic triphasic morphology; however, brisk mitotic activity noted at multiple foci was diagnostically challenging to categorize. The tumor was evaluated for ETV6-NTRK3 fusion to exclude other common differentials. Conclusion While FHI may be frequently encountered in infants, rare sarcomatous transformation are known to occur and merits special attention as it can be misdiagnosed. Also, a close follow-up is warranted as the lesion is known to recur locally.

Primary Wilms tumor of the urinary bladder

ABSTRACT Wilms tumor (WT) can occur at various extrarenal sites; however, the urinary bladder as the primary site is occasional. A 4-year-old-female child presented with difficulty in micturition for the past month. The contrast-enhanced magnetic resonance imaging with magnetic resonance (MR) urography revealed a polypoidal, heterogeneous mass in the urinary bladder with no abnormality in the kidneys. Cystoscopy-guided biopsy was reported as an extrarenal Wilms tumor (ERWT) with triphasic components. Post-chemotherapy, a computed tomography scan revealed a residual tumor for which she underwent partial cystectomy. The diagnosis of ERWT was confirmed. She received adjuvant chemotherapy and remained well at the 9th month post completion of chemotherapy. The primary bladder WT must be considered in the differential of a small blue round cell tumor at an extrarenal site in the pediatric age group. The diagnosis is especially challenging in small biopsy material, although it has immense significance in management and prognosis.

Diagnostic and therapeutic endonasal rhinologic procedures generating aerosol during COVID-19 pandemic: a systematized review / Procedimentos endonasais de rinologia diagnósticos e terapêuticos geradores de aerossóis durante a pandemia de Covid-19: uma revisão sistematizada,

Abstract Introduction Most rhinologic procedures, particularly endoscopic sinonasal procedures, are liable to produce aerosols. The severe acute respiratory syndrome coronavirus 2 (SARS-Co V-2) transmits via respiratory droplets, but the degree of its spread through airborne routes by aerosol is unclear. Objective The aim of this article is to counsel rhinologists on how to modify their conventional practice during the COVID-19 pandemic by prioritising the need of procedures, identifying aerosol- generating procedures and using precise personal protection equipment for various endonasal procedures. Methods We did a review of articles indexed for MEDLINE on PubMed, ENT Cochrane, DOAJ and Web of Science databases using the keywords nasal endoscopy, SARS-CoV-2, COVID-19, aerosol generating medical procedures and rhinology to formulate guidelines for the safety of healthcare workers. Results The review included evidence from 28 articles from the otorhinolaryngology, surgery, infectious disease, head and neck surgery and cancer biology literature. We have provided recommendations and relevant information for rhinologists during the COVID-19 pandemic, based on the available studies and data, to warrant high-quality patient care and requisite levels of infection prevention during rhinology procedures. Conclusion In rhinology, marked care is advised during nasal packing, electrocauterisation and use of high-speed rotating devices in potentially infected tissue as they are considerable aerosol- producing procedures. The choice of personal protective equipment is based on the risk of exposure and possible modes of aerosol generation.

Resumo Introdução A maioria dos procedimentos rinológicos, principalmente os procedimentos endoscópicos nasossinusais, é suscetível à produção de aerossóis. A síndrome respiratória aguda grave coronavírus-2 (SARS-CoV-2) é transmitida através de gotículas respiratórias, mas o grau de sua disseminação através de aerossóis por via aérea não é claro. Objetivos Orientar os rinologistas sobre como modificar sua prática convencional durante a pandemia de Covid-19, priorizar a necessidade de procedimentos, identificar procedimentos geradores de aerossol e usar equipamento de proteção individual de precisão para vários procedimentos endonasais. Método Fizemos uma revisão de artigos indexados na Medline por meio das bases de dados PubMed, ENT Cochrane, DOAJ e Web of Science, com as palavras-chave nasal endoscopy, SARS-CoV-2, Covid-19, aerosol-generating medical procedures e rhinology para formular diretrizes para a segurança dos profissionais de saúde. Resultados A revisão incluiu evidências de 28 artigos de otorrinolaringologia, cirurgia, doenças infecciosas, cirurgia de cabeça e pescoço e biologia do câncer. Fornecemos recomendações e informações relevantes para rinologistas durante a pandemia de Covid-19, com base nos estudos e dados disponíveis, para garantir atendimento de alta qualidade ao paciente e níveis necessários de prevenção da infecção durante procedimentos de rinologia. Conclusões Em rinologia, recomenda-se cuidado acentuado durante o tamponamento nasal, eletrocauterização e uso de dispositivos rotativos de alta velocidade em tecidos potencialmente infectados, pois esses procedimentos são consideráveis produtores de aerossóis. A escolha do equipamento de proteção individual é baseada no risco de exposição e nos possíveis modos de geração de aerossol.

Demographics in trauma: a prospective observational study in a tertiary care zonal hospital

Background: Trauma represents a major challenge worldwide and is the leading cause of morbidity and mortality in young population. There is significant change in trauma related injuries due to urbanization, motorization, industrialization and the socioeconomic changes in India. Road traffic accidents (RTA) pose the largest threat to human lives and have become the number one public hazard all across the world causing morbidity and mortality. The aim of this study is to find out the demographics and to determine the injury pattern of surgical trauma cases presenting to this tertiary care zonal hospital.Methods: A prospective observational study was conducted in a tertiary care zonal hospital from June 2018 to December 2018. Data was collected for 105 patients presented with trauma in surgical emergency centre of this hospital were included in the study which excluded only orthopaedic trauma cases. The data collected was analysed.Results: Out of 105, 76 (72.38%) were male and 29 (27.61%) were female with 21 (20%) in the 31-40 age group. The average age of cases was 40.2 years.  RTA was the major cause of trauma i.e. (77.14%) followed by accidental fall and slips (18.09%) and assaults (4.76%). Contusion was the common injury sustained (45.71%) followed by laceration (38.09%), abrasion (35.23%) and fracture (17.14%).Conclusions: There is a need for trauma registries which can help to formulate strategies towards decreasing the burden of trauma and improved outcomes at hospital as well as in public health.

Primary Ewing sarcoma/primitive neuroectodermal tumor of the lung

We present the autopsy findings and differential diagnosis in a 42year old male who presented with fever and rapidly progressive respiratory symptoms like breathlessness, nonproductive cough and right sided chest pain. Initial imaging workup done at our hospital revealed a large unilateral tumor with tracheal shift. While being evaluated patient developed facial puffiness, tachypnea suggestive of superior vena cava obstruction. Antemortem biopsy of lung mass was attempted twice and that suggested malignant lesion. Unfortunately, the individual had a rapid downhill course following admission. Post mortem examination was conducted that on opening the thoracic cavity revealed total replacement of right lung tissue by a necrotic growth which was deeply adherent to the rib cage. The contralateral lung as well as all other visceral organs were unremarkable grossly. Histopathology confirmed primary Ewing sarcoma of the lung. We hereby, report a rare case of primary lung Ewing sarcoma diagnosed at autopsy.

Clinical profile and one-year survival of patients with heart failure with reduced ejection fraction: The largest report from India

Background India has one of the largest population of heart failure (HF) patients in the world; yet only limited information is available about HF in India. Methods This observational study was performed at Medanta- The Medicity, a large, tertiary-care institute in the National Capital Region of India. Records of HF patients with reduced left ventricular ejection fraction (LVEF) registered at Medanta HF clinic during the period early 2014 to mid-2017 were reviewed. Disease characteristics and one-year mortality details were collected. Results Mean age of the subjects (n = 5590) was 59.1 ± 11.8 years with 83.0% males. Mean LVEF was 30.0 ± 6.6%. Coronary artery disease (CAD) was the dominant cause of HF, accounting for 77.8% of the total population. Most patients received guideline-directed medical therapy with a beta blocker being prescribed to 81.8% subjects. The one-year all-cause mortality was 17.6%. On multivariate analysis, age, usage of loop diuretics and ivabradine, and serum creatinine were independently associated with one-year mortality, whereas rheumatic etiology had an inverse association. Conclusions This represents the largest single-center data of HF patients reported so far and the largest study describing clinical outcomes from HF patients in India. Our patients were younger, had high proportion of CAD, and there was higher usage of beta-blockers. Despite this, the one-year mortality was substantial. Given the enormous magnitude of HF burden in India and the paucity of information on this subject, these findings should be of help in identifying key problem areas and potential solutions for management of HF in India.

Comparison of hemodynamic response and postoperative pain score between general anaesthesia with intravenous analgesia versus general anesthesia with caudal analgesia in pediatric patients undergoing open-heart surgery

Context: Regional anesthesia may attenuate adverse physiological stress responses associated with cardiothoracic surgery. In this study, hemodynamic stress response at the different time of surgical stimuli was compared between patients receiving general anesthesia (GA) along with caudal epidural analgesia with GA with intravenous analgesia in pediatric population undergoing open-heart surgery. Aims: This study aims to compare the hemodynamic response at the different time of surgical stimuli and postoperative pain score, in pediatric patients undergoing open-heart procedures. Settings and Design: We designed a prospective randomized controlled trial to study hemodynamic effects between Group I and Group II. Fifty patients were randomly allocated equally into Group I (GA + caudal epidural) and Group II (GA + intravenous analgesia) by sealed envelope technique. Subjects and Methods: After obtaining approval from Institutional Ethical Committee, this prospective study was conducted in 50 American Society of Anesthesiologist Classes II and III pediatric patients aged between 1 and 12 years posted for cardiac surgery in our institution. Statistical Analysis: ANOVA, two-way ANOVA, and Student's test. Results: The heart rate, systolic blood pressure, diastolic blood pressure and mean blood pressure variations were compared between Groups I and II at different time intervals. The variations were found to be significantly higher at the time of skin incision and 2 min after skin incision in Group II as compared to Group I. Pain score was compared between the groups and was found to be significantly lower with Group I (2.5 ± 1.2) as compared to Group II (4.6 ± 1.7), P = (0.004). Conclusions: Caudal analgesia with GA (Group I) was found to have better hemodynamic control and significantly better postoperative pain relief in the first 24 h after awakening.

Effect of socio-economic status & proximity of patient residence to hospital on survival in childhood acute lymphoblastic leukaemia

Background & objectives: Survival in paediatric acute lymphoblastic leukaemia (ALL) in lower/middle income countries continues to lag behind outcomes seen in high-income countries. Socio-economic factors and distance of their residence from the hospital may contribute to this disparity. This study was aimed at identifying the impact of these factors on outcome in childhood ALL. Methods: In this retrospective study, file review of children with ALL was performed. Patients were treated with the modified United Kingdom (UK) ALL-2003 protocol. Details of socio-economic/demographic factors were noted from a web-based patients' database. Modified Kuppuswamy scale was used to classify socio-economic status. Results: A total of 308 patients with a median age of five years (range: 1-13 yr) were studied. Patients belonging to upper, middle and lower SE strata numbered 85 (28%), 68 (22%) and 155 (50%). Nearly one-third of the patients were underweight. There was no treatment abandonment among children whose mothers were graduates. Neutropenic deaths during maintenance therapy were lower in mothers who had passed high school. In patients who survived induction therapy, the five year event-free survival (EFS) of upper SE stratum was significantly better 78.7�9 vs. 59�2 and 58.1�6 per cent in middle and lower strata (P =0.026). Five year overall survival was higher in the higher SE group; being 91.2�5, 78.3�6 and 78.8�9 per cent (P =0.055) in the three strata. Survival was unaffected by a distance of residence from treating centre or rural/urban residence. High-risk and undernourished children had a greater hazard of mortality [1.80 (P =0.015); 1.98 (P =0.027)]. Interpretation & conclusions: Our findings showed that higher socio-economic status contributed to superior EFS in children with ALL who achieved remission. Undernutrition increased the risk of mortality.

Transradial access for coronary diagnostic and interventional procedures: Consensus statement and recommendations for India: Advancing Complex CoronariES Sciences through TransRADIAL intervention in India – ACCESS RADIAL™: Clinical consensus recommendations in collaboration with Cardiological Society of India (CSI)

Radial access for cardiac catheterization and intervention in India has been growing steadily over the last decade with favorable clinical outcomes. However, its usage by interventional cardiologists varies greatly among Indian operators and hospitals due to large geographic disparities in health care delivery systems and practice patterns. It also remains unclear whether the advantages, as well as limitations of transradial (TR) intervention (as reported in the western literature), are applicable to developing countries like India or not. An evidence-based review involving various facets of radial procedure for cardiac catheterization, including practical, patient-related and technical issues was conducted by an expert committee that formed a part of Advancing Complex CoronariES Sciences through TransRADIAL intervention (ACCESS RADIAL™) Advisory Board. Emerging challenges in redefining TR management based on evidence supporting practices were discussed to formulate these final recommendations through consensus.

Risk Factors for Microbiologically-documented Infections, Mortality and Prolonged Hospital Stay in Children with Febrile Neutropenia

Objective: To analyze the risk factors for microbiologicallydocumented infection, mortality and hospital stay more than 5days in children with febrile neutropenia.Design: Cross-sectional study (July 2013-September 2014).Setting: Government-run, tertiary-care, university hospital inChandigarh, Northern India.Participants: 414 episodes in 264 children aged <12 years, notundergoing stem-cell transplantation.Outcome measures: Predictors for ‘high-risk’ febrileneutropenia.Results: Microbiologically-documented infections wereobserved in 82 children (19.8%); bacterial 14.2%, fungal 4.3%,polymicrobial 9.7%. Complications were documented in 109(26%) children. 43 (10.3%) died: 8 due to fungal and 35 due tobacterial sepsis. Children admitted within 7 days of the lastchemotherapy (P<0.01) and having a non-upper respiratory focusof infection (P<0.02) were at risk of developing microbiologically-documented infections and death. Platelet count <20000/uL(P=0.03) was an additional predictor for microbiologically-documented infections, while albumin <2.5 g/dL (P=0.04) and C-reactive protein >90 mg/L (P=0.02) were risk factors predictingmortality. The median (IQR) duration of hospital stay was 5 (3,8)days. Hospital stay >5 days was seen in 144 (35%) children.Children with acute myeloid leukaemia (P<0.01) and admittedwithin 7 days of chemotherapy (P=0.02) were likely to have aprolonged hospital stay >5 days.Conclusions: Febrile neutropenicchildren admitted within 7days of completion of chemotherapy, those with a non-upperrespiratory focus of infection, CRP >90 mg/dL, platelet <20000/uLand albumin <2.5 g/dL need to be considered as ‘high risk’ forcomplications and mortality.

Levels of Serum Vitamin B6 and GABA In Clinically Depressed Patients: A Study

Background:Depression is the most common type of mental illness. As per the WHO criterion of prevalence of depression, this disease has a prevalence of 4.4% all over the world and 4.5% in India. Some water-soluble vitamins like vitamin B2, B6, B9, B12; play an important role in mental health. Of these vitamins pyridoxal phosphate, the active form of vitamin B6 plays a pivotal role in a number of transamination reactions, decarboxylation reactions and in the control of plasma homocysteine concentration. PLP is vital for the biosynthesis of GABA, which is being used as the neurotransmitter in at least 40% of the inhibitory synaptic processing in the brain. Therefore, the present study was planned to assay the blood levels of vitamin B6 and GABA in patients with depression. The objective of the study was to help psychiatrists to take appropriate and informed decision about supplementation of vitamin B6 and use of specific antidepressant medication in the treatment of depression cases. Methods: Current study was an experimental case control study conducted at Santosh Medical College and Hospitals. A total of 204 (102 controls and 102 patients) both males and females, in the age group between 18-65 years were included in the study. Hamilton Depression Rating Scale (HDRS) 17 items was used to rate the level of depression. Results: In the present study, a total of 204 subjects including 97 females and 107 males participated, out of which 102 were clinically diagnosed cases of depression attending psychiatry OPD and included 55 females and 47 males. 47.1% of the cases were of mild depression, 23.5% were of moderate depression, 11.8% of severe depression and the remaining 17.6% belonged to very severe depression category. The mean value of vitamin B6 was found lowest in severe to very severe depressed patients (5.25) than in mild to moderate depressed patients (8.83) and the mean value of GABA was found to be highest in severe to very severe category (5571.73) than in mild to moderate depressed patients (5184.73). Conclusion: This study showed that majority of the patients with clinical depression had vitamin B6 deficiency and increase in GABA levels.

A case of left atrial dissection after mitral valve replacement

Left atrial dissection (LatD) is a rare complication of cardiac surgery due to creation of a false chamber through a tear in the mitral valve annulus that extends into the left atrium wall. It is primarily associated with mitral valve surgery although other etiologies have also been defined. Perioperative transesophageal echocardiography (TEE) is a key to the diagnosis. This is a case report of management of LatD after mitral valve replacement.

Hepatic and Cardiac Iron-load in Children on Long-term Chelation with Deferiprone for Thalassemia Major

Objective: To evaluate the efficacy of prolonged deferiprone monotherapy in patients with ?-thalassemia major. Methods: This cross-sectional study included 40 patients (age range 9 to38 years) with thalassemia major receiving deferiprone for ?5 years. Serum ferritin, andmyocardial iron concentration (MIC) and liver iron concentration (LIC) assessed by T2*MRIwere recorded. Results: The patients were receiving deferiprone for a mean (SD) duration of12.1 (4.7) years. The median (IQR) dose of deferiprone was 85 (74.3, 95) mg/kg/day. TheMIC was normal or had a mild, moderate or severe elevation in 29 (72.5%), 3 (7.5%), 3(7.5%), and 5 (12.5%) patients. The LIC was normal or had a mild, moderate or severeelevation in 2 (5%), 4 (10%), 11 (27.5%) and 23 (57.5%) patients. Conclusions: The majorityof patients receiving deferiprone had a moderate/severe hepatic but normal cardiac iron load.Prolonged deferiprone monotherapy was suboptimal for hepatic iron load in the majority.

Is a diagnostic lumbar puncture indicated in intraocular retinoblastoma?

Background: Lumbar puncture (LP) is frequently performed in patients with advanced intraocular retinoblastoma. However, this may not be necessary in a significant proportion of patients. Materials and Methods: A file review of patients who were diagnosed with retinoblastoma over a 13-year-period was performed. Patients who underwent LP as part of staging were included in the study. Results: The study included 223 patients. One-third had bilateral retinoblastoma. The grouping was C, D, and E in 4 (2.9%), 41 (29.9%), and 92 (67.2%) patients, respectively. The stage was 0, I, II, III, and IV in 14 (6.3), 123 (55.2%), 13 (5.8%), 70 (31.4%), and 3 (1.3%) patients, respectively. Eight (3.6%) patients had a positive cerebrospinal fluid (CSF) cytology. None of the patients with intraocular disease and 7 (10%) patients with extraocular disease had a positive CSF. Conclusions: A diagnostic CSF is not indicated in patients with intraocular retinoblastoma.