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Allogenic hematopoietic cell transplant (HCT) is the best curative approach patients with severe aplastic anemia (SAA). Outcome of HCT from haploidentical family donor (HFD) has improved, making it a feasible option for patients lacking HLA-identical donor. However, data on HFD-HCT for younger patients with SAA is sparse. In multicentre retrospective study, we report outcome of 79 patients undergoing HFD-HCT for SAA. All were heavily pre-transfused, median time to HCT12 months and 67% had failed previous therapies. Conditioning was based on Flu-Cy-ATG/TBI with or without thiotepa/melphalan (TT/Mel). Post-transplantation cyclophosphamide (PTCy) and CNI/Sirolimus were employed as GvHD prophylaxis with or without abatacept. Primary graft failure (PGF) was 16.43%, less in those conditioned with TT/Mel. Incidence of acute and chronic GVHD were 26.4% and 18.9%. At a median follow-up of 48 months, the overall survival (OS) and event free survival (EFS) were 61.6% and 58.1% respectively. Both OS/EFS were better in TT/Mel group and with abatacept as GVHD prophylaxis. On multivariate analysis, use of Abatacept was found to favourably impact the outcome variables including GVHD and EFS. Our study suggests that PTCy-based HFD-HCT is a reasonable option for young patients with high-risk SAA, where optimisation of conditioning and GVHD prophylaxis might improve outcomes further.

This paper presents the optics design of a dual channel miniaturized eye-safe laser range finder (ELRF). There are many design approaches for designing a LRF. All approaches have their positive and negative aspects. Dual channel (in which transmitter and receiver are in separate channels) approach is the simplest approach. It provides more freedom to the designer to make the LRF more compact and light weight. In this approach alignment and maintenance becomes easy as compared to single channel LRF. The paper presents the design aspects of a dual channel LRF. The beam expander, having 10X angular magnification, is used to expand the laser beam, so that by reducing its divergence it can illuminate the object to be ranged. The reflected/scattered part of the laser beam is received by the receiver using a separate optics. The received light is focused on to the detector by the focusing lens module. An interference filter is used in the receiver channel to block unwanted background radiation. Performance of the optical design has been analyzed through different evaluation methods like Rim Ray curve, Spot Diagram etc., and the design is found to meet the desired specifications/performance.

In this paper, the Optics Design of Three discrete FOV Optics for Day sight using HDTV sensor and near IR using LLLTV sensor are presented. The System has Wide Field of View (WFOV) for quick search mode to detect target of interest, and Narrow field of view (NFOV) for engaging the target and a Mid Field of view (MFOV), in-between WFOV and NFOV. There will be a front window for protection and a stabilized prism having scanning capability from +60° to −10° in the line of sight elevation for air search, or depression to look close in. The f#8 optics is designed for a 1920 × 1080 array with 5.5 μm pitch HDTV detector having spectral response from 0.45 to 0.65 μm and f#3.6 optics is designed for 2/3″ LLLTV detector with 6.45 μm pitch and having spectral response from 0.55 to 0.85 μm.

Driving in night without switching on headlights to avoid the detection is crucial for night fighting capability. Uncooled Thermal Imager provides good imagery but it is unable to detect beacon light of front moving vehicle. For better viewing experience, it requires electronic image fusion of output of day light camera with low light level imaging capability and Thermal imager, so that it can detect beacon light and thermal image simultaneously during night. This paper will cover the Optical design of wide angle, compact and fast optics for day light camera along with performance analysis.

Outcomes of high-risk and relapsed pediatric acute leukemias continue to be suboptimal. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative modality. However

BackgroundHematopoietic stem cell transplantation (HSCT) is the curative option for many primary immune deficiency disorders (PID). In the last 5 years, increased awareness, availability of diagnostics based on flow cytometry, genetic testing, improved supportive care, use of reduced toxicity conditioning, and success of haploidentical donor HSCT have improved access to HSCT for children with PID in India. We present results on children with PID who underwent HSCT across India and the factors that influenced outcome.Patients and MethodsWe collected retrospective data on the outcome of HSCT for PID from seven centers. We analyzed the impact of the type of PID, conditioning regimen, time period of HSCT- before or after January 2016, graft versus host disease prophylaxis, cause of mortality and overall survival.ResultsA total of 228 children underwent HSCT for PID at a median age of 12 months (range, 1 to 220 months) with a median follow up of 14.4 months. Infants accounted for 51.3% of the cohort and the male female ratio was 3:1. SCID (25%) and HLH (25%) were the more frequent diagnoses. Matched family donor was available in 36.4% and 44.3% children had a haploidentical HSCT. Reduced and myeloablative conditioning regimens were used with 64% children receiving a treosulfan based conditioning regimen. Peripheral blood stem cells were the predominant graft source at 69.3%. The survival in infants (60.2%) was inferior to children aged over 1 year (75.7% p value = 0.01). Children with Wiskott Aldrich syndrome (74.3%) and chronic granulomatous disease (82.6%) had the best outcomes. The survival was superior in children receiving HSCT from a matched sibling (78%) versus an alternate donor HSCT (61% p value = 0.04). In the cohort transplanted after January 2016 survival improved from 26.8% to 77.5% (p value = 0.00). Infection remains the main cause of mortality at in over 50% children. The 5-year overall survival rate was 68%.ConclusionSurvival of children with PID undergoing HSCT in India has improved dramatically in last 5 years. Alternate donor HSCT is now feasible and has made a therapeutic option accessible to all children with PID.

Chediak-Higashi syndrome is a rare immunodeficiency disorder for which hematopoietic stem cell transplant (HSCT) is the only curative treatment option. HSCT only corrects the hematological and immunologic manifestations of the disease but neurological complications may still progress after transplant. Haploidentical HSCT (haplo-HSCT) has evolved as a feasible alternative for patients with primary immunodeficiency. More recently, there has been use of haplo-HSCT with post-transplant cyclophosphamide. However, only 4 cases of Chediak-Higashi syndrome have been reported using this approach. Here, the authors describe a case of a 17-month-old boy who was successfully treated by haplo-HSCT with reduced-toxicity conditioning (fludarabine/treosulfan/melphalan) and post-transplant cyclophosphamide.

Hexokinase (HK) is one of the key regulatory enzymes for glycolysis. HK catalyses the phosphorylation of glucose to glucose – 6 – phosphate resulting in irreversible transfer of high-energy phosphate from ATP to glucose. HK deficiency is a rare red cell enzyme defect. There are approximately 30 cases and 10 mutations of HK deficiency reported till date. Many patients remain undiagnosed due to mild manifestations of the disease. Here we report a case of a 3-year-old boy who presented with haemolytic anaemia and was found to have HK deficiency with a novel mutation at exon 1 of HK1 gene.

Between 2014 and 2020, 31 patients with severe aplastic anemia (SAA) underwent full match allogeneic hematopoietic stem cell transplantation at our center. Of the 31 patients with SAA, 19 had acquired aplastic anemia, 2 had Diamond Blackfan anemia and 10 had Fanconi anemia. Donors were either matched sibling (n=29), related donors (n=2), or unrelated donors (n=3). Peripheral blood stem cells were the graft source in all the cases except 1. Fludarabine-based reduced intensity conditioning was used in all except for patients with a diagnosis of Diamond Blackfan anemia. All patients except 1 achieved hematologic recovery in the form of neutrophil engraftment at 13 days (range, 9 to 17), whereas platelet engraftment occurred at 14 days (range, 10 to 18). Graft versus host disease (GvHD) prophylaxis consisted of cyclosporine and methotrexate ±antithymocyte globulin (horse/rabbit). Acute GvHD developed in 12.9% patients, whereas no patients developed chronic GvHD till the time of last follow-up. The 2-year overall survival for the entire cohort was 93.21±4.6%. In patients with SAA, allogeneic stem cell transplant using fludarabine-based conditioning regimens are very well tolerated and have excellent outcomes in a full match setting.

Potato is a temperate crop and higher day temperatures cause some areas to less suitable for potato production due to lower tuber yields and its quality. Tuber growth and yield can be severely reduced by temperature fluctuations outside 5-30 °C. The rate of warming in last 50 years is double than that for the last century. Increase in temperature and atmospheric CO2 are interlinked occurring simultaneously under future climate change and global warming scenarios. If CO2 is elevated to 550 ppm the temperature rise is likely to be 3 ºC with decline in potato production by 13.72% in the year 2050. The changing climate will affect the potato production adversely due to drought, salinity, frost, flooding, erratic unseasonal rains etc. It may reduce seed tuber production, impact storage facility and potato processing industries. Therefore, the quantification of regional vulnerability and impact assessment is very important for the development of early warning on disease forecasting systems, breeding of short duration and heat, drought, salinity tolerant and disease resistant cultivars.

Practitioners and people need information about the therapeutic potential of umbilical cord blood stem cells and pros and cons of storing cord blood in public versus private banks.Indian Academy of Pediatrics conducted a consultative meeting on umbilical cord blood banking on 25th June 2016 in Pune, attended by experts in the field of hematopoietic stem cell transplantation working across India. Review of scientific literature was also performed. All expert committee members reviewed the final manuscript.To bring out consensus guidelines for umbilical cord banking in India.Umbilical cord blood stem cell transplantation has been used to cure many malignant disorders, hematological conditions, immune deficiency disorders and inherited metabolic disorders, even when it's partially HLA mismatched. Collection procedure is safe for mother and baby in an otherwise uncomplicated delivery. Public cord blood banking should be promoted over private banking. Private cord blood banking is highly recommended when an existing family member (sibling or biological parent) is suffering from diseases approved to be cured by allogenic stem cell transplantation. Otherwise, private cord blood banking is not a 'biological insurance', and should be discouraged. At present, autologous cord stem cells cannot be used for treating diseases of genetic origin, metabolic disorders and hematological cancers. Advertisements for private banking are often misleading. Legislative measures are required to regularize the marketing strategies of cord blood banking.

Introduction: Autologous Stem Cell Transplant (ASCT) provides long periods of progression-free-survival in multiple sclerosis (MS). This is an observational study to demonstrate the safety of ASCT in MS patients at a transplant center in North India using a lymphoablative regimen. Materials and Methods: MS patients > 18 years referred by a neurologist or who came of their own volition were evaluated. Kurtzke Expanded Disability Status Scale (EDSS) score was calculated and those with a score of >7 were excluded. Informed written consent was taken. Mobilization was done with G-CSF with prednisolone to prevent disease flare-up. A minimum of 2 × 106 CD34 cells/kg was collected. Conditioning regimen consisted of rabbit ATG and cyclophosphamide. Rituximab 375 mg/m2 was given to prevent EBV reactivation and disease relapse. Antibiotic prophylaxis was given with levofloxacin, fluconazole, and valacyclovir. Any persistent change in EDSS scores ≥0.5 was considered significant. Results: Twenty patients were included. Seven patients had positive urine cultures prior to transplant and were treated before starting any chemotherapy. Majority patients were women (13/20). All patients developed febrile neutropenia, which was managed as per department policy. There was no mortality. Subjective symptoms improved in all patients. EDSS score improved in 6/19 patients (5/6 with RRMS) with no disease progression in any patient at a median follow-up duration of 242 days. Conclusion: ASCT can be done safely for patients with relatively high EDSS scores with additional precautions for screening for infections. RRMS patients with the active disease show most improvement. SPMS patients may not show significant improvement in the short term.

The survival of children with cancer in India is inferior to that of children in high-income countries. The Indian Pediatric Hematology Oncology Group (IPHOG) held a series of online meetings via www.Cure4kids.org to identify barriers to cure and develop strategies to improve outcomes. Five major hurdles were identified: delayed diagnosis, abandonment, sepsis, lack of co-operative groups, and relapse. Development of regional networks like IPHOG has allowed rapid identification of local causes of treatment failure for children with cancer in India and identification of strategies likely to improve care and outcomes in the participating centers. Next steps will include interventions to raise community awareness of childhood cancer, promote early diagnosis and referral, and reduce abandonment and toxic death at each center. Starting of fellowship programs in pediatric hemato-oncology, short training programs for pediatricians, publishing outcome data, formation of parent and patient support groups, choosing the right and effective treatment protocol, and setting up of bone marrow transplant services are some of the effective steps taken in the last decade, which needs to be supported further.

We present a 15 year old boy who developed severe rhabdomyolysis and acute renal failure following influenza B infection. His renal function was restored after appropriate therapy for rhabdomyolysis. Although rapidly progressive pneumonia, respiratory failure, and acute respiratory distress syndrome are the most common severe complications of influenza B infection, clinicians should be aware that influenza B may be complicated with rhabdomyolysis and acute renal failure in children.

Increasing global food production to feed rapidly growing populations where cultivable land area is limited is a serious challenge. Moreover, increasing production costs, with high fertilizer input costs, particularly using nitrogen (N), and degrading soil health are the major concerns when enhancing the sustainable agricultural food production. Potato, being the major non-cereal food crop globally, is a heavy N fertilizer feeder crop. In the past several agricultural best management practices have been discussed regarding N management in potato crop production through the intervention of agronomy and soil science. However, unlike the advances in other model plants and cereals, the application of molecular genomics tools is lacking in potato, hence it is not possible to enhance plant genetic potential with better nitrogen use efficiency (NUE). Better N-use efficient plants can be grown with less fertilizer input and also on poor soil. This chapter highlights the application of novel genomics tools to improve NUE in potato through the discovery of novel genes and markers for applications in molecular breeding methods and gene manipulation (transgenic) techniques.