Papers by Indira Jayakumar
Pediatric Critical Care Medicine, Jul 1, 2005
Pediatric Critical Care Medicine is proud to present translations of selected abstracts into Chin... more Pediatric Critical Care Medicine is proud to present translations of selected abstracts into Chinese, French, Italian, Japanese, Portuguese, and Spanish. We sincerely thank the following for their important work on these translations:

Journal of pediatric critical care, 2017
Trauma to the spine can cause injuries involving spinal cord, vertebra or both. Although less fre... more Trauma to the spine can cause injuries involving spinal cord, vertebra or both. Although less frequent compared to adults, Traumatic Spinal Cord Injury (TSCI) in the pediatric population is not rare and has high morbidity and mortality with significant physiological and psychological consequences. Pediatric patients with TSCI have different mechanisms of injury but better neurological recovery potential when compared to adults. TSCI should be strongly suspected in the presence of neck pain, abnormal neurological exam, high-risk mechanism ofinjury or a distracting injury, even in the absence ofradiological abnormality A high index of suspicion should be maintained in trauma victims until a spinal injury is identified or ruled out. As in Traumatic Brain Injury (TBI), hypoxia and hypotension can worsen secondary injury to the spinal cord and adversely affect neurologic outcome.

INDIAN JOURNAL OF APPLIED RESEARCH, Dec 1, 2022
An inordinate high demand for home visit (HV) physician medical team in third world and developin... more An inordinate high demand for home visit (HV) physician medical team in third world and developing countries. Aim : The study is to deliver medical treatment at home in emergency and immovable situations. We assessed, Introduction : managed and analyzed types of disorders, treatment outcome, and instructions to home caregivers about monitoring and intimating clinical response after HV and tele-consultation. Strict adherence to protocol based res Discussion : ults proved 99.5% convincing management with 0.5% death in terminally ill. Mean average age of 75 yrs for males and 71 yrs for females. General medical illness is 70.7%, Acute emergency 6.1%, Catheterisation 12.2%, Tracheostomy care 2.2%, Wound dressing 6.6%, Sutures 0.6%, Parenteral infusion 1.1%. We noticed HV is Conclusion : essential in third world countries and to include in policy making by healthcare. Limitation and recommendation : Can be overcome by increasing trained manpower.
Pediatric Hematology Oncology Journal, Dec 1, 2021
Jaypee Brothers Medical Publishers (P) Ltd. eBooks, 2008
Pediatric Transplantation, Dec 10, 2019
X-linked agammaglobulinemia (XLA) is a primary antibody disorder due to a mutation in the Bruton ... more X-linked agammaglobulinemia (XLA) is a primary antibody disorder due to a mutation in the Bruton tyrosine kinase gene that requires lifelong immunoglobulin replacement resulting in a significant economic burden and treatment abandonment. Hematopoietic stem cell transplantation (HSCT) offers an alternative option for complete cure. In our series, two children with XLA underwent successful HSCT using a myeloablative conditioning with thiotepa, treosulfan, and fludarabine from How to cite this article: Vellaichamy Swaminathan V, Uppuluri R, Patel S, et al. Treosulfan-based reduced toxicity hematopoietic stem cell transplantation in X-linked agammaglobulinemia: A cost-effective alternative to long-term immunoglobulin replacement in developing countries.
Pediatric Critical Care Medicine, Jul 1, 2005
Pediatric Critical Care Medicine is proud to present translations of selected abstracts into Chin... more Pediatric Critical Care Medicine is proud to present translations of selected abstracts into Chinese, French, Italian, Japanese, Portuguese, and Spanish. We sincerely thank the following for their important work on these translations:
Journal of Pediatric Hematology Oncology, Feb 18, 2021
We present our experience on the use of fludarabine, cytarabine, granulocyte colony-stimulating f... more We present our experience on the use of fludarabine, cytarabine, granulocyte colony-stimulating factor in combination with Bortezomib. In total, 13 children with relapsed/refractory leukemia (acute lymphoblastic leukemia = 9 and acute myeloid leukemia = 4) were included from January 2018 to May 2019. Culture-positive sepsis and intensive care unit admission rates were 38% and 30%, respectively, with no postchemotherapy mortality in this cohort. Morphologic remission was documented in 92% and negative minimal residual disease was achieved in 61%, with 100% remission in those with acute myeloid leukemia. These results bear significant relevance in developing countries where multidrug-resistant sepsis is on the rise.
PubMed, Mar 1, 2004
A 5-month-old infant with Shaken Baby Syndrome is reported. This form of physical child abuse is ... more A 5-month-old infant with Shaken Baby Syndrome is reported. This form of physical child abuse is often overlooked. It should be suspected in infant who present with drowsiness, coma, seizures or apnea.

Pediatric Blood & Cancer, Nov 25, 2019
To the Editor: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive hyperinflammatory disord... more To the Editor: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive hyperinflammatory disorder driven by the uncontrolled activation and proliferation of CD8+ T lymphocytes and macrophages.1 Hematopoietic stem cell transplantation (HSCT) is recommended in children with a primary or familial HLH as the only curative option available.2 Eighty percent of patients have at least a partial response to front-line therapy with dexamethasone and etoposide, while patients who lack a satisfactory responsemay require second-line agents.3 Ruxolitinib, a Janus kinase (JAK) 1/2 inhibitor, has shown promise in mouse models of primary HLH. Ruxolinitib when administered empirically to perforin-deficientmice inhibits interferon γ (IFN-γ), interleukin (IL)-6, and IL-12 production with reduction in cytokine production and tissue damage in active HLH.4,5 A 3-year-old female child born of third-degree consanguineous marriage presented to our center in April 2018 with fever of 2 weeks duration and sudden onset cervical lymphadenopathy. She had received a short course of steroids (oral prednisolone) and oral amoxycillin elsewhere. Cervical lymph node biopsy was performed, which was reported to have hyperplasia with no atypical features and was positive for Epstein Barr virus (EBV) on FISH. On evaluation, she was found to have elevated levels of serum ferritin, low fibrinogen, and normal triglycerides. Clinical exome sequencing was performed, which revealed homozygous deficiency of UNC13D suggestive of familial HLH type III. She was started on HLH 2004 protocol in June 2018 including dexamethasone, etoposide, cyclosporine, and intrathecal methotrexate. She completed induction in August 2018. Due to social reasons, further treatment was abandoned by the family. She presented to our center yet again in February 2019with a similar clinical profile. Investigations revealed recurrence of HLH disease and she was restarted on induction chemotherapy as per HLH 2004 protocol. The reinductionwas complicated by prolonged admission for febrile neutropenia. She developed features of right-sided preseptal cellulitis. Computed tomography scan of paranasal sinuses was performed to look for an active focus for infection and was reported to reveal rightmaxillary sinusitis. Right antral fluid lavagewas performed, which did not reveal any specific etiology. EBV copies per milliliter were found to be 3049 and she was given one dose of rituximab at 375 mg/m2. Subsequently she developed progressively increasinghepatosplenomegaly, cervical lymphadenopathy, and persistent fever. Positron emission tomography-computed tomography was performed that revealed FDG uptake in multiple cervical, mediastinal, supraclavicular, axillary, abdominal, retroperitoneal and pelvic nodes, hypermetabolic pulmonary nodules, and diffuse fluorodeoxyglucose uptake in spleen. A repeat biopsy of the left cervical lymph node revealed lymphohistiocytic infiltrate and immunohistochemistry was negative for malignancy. In view of refractory HLH and possibility of associated fungal infection, oral ruxolitinib with hydrocortisone was commenced for the HLH therapy. Ruxolitinib was started at a dose of 2.5 mg twice a day and hydrocortisone at a dose of 1 mg/kg/dose three times a day. Amphotericin B at 3 mg/sq.m was started concurrently for the fungal infection. She improved with stabilization of all clinical and laboratory parameters. She recovered from pancytopenia with improvement in total white cell counts from 1200 to 3500 μL–1, platelet counts from 23 000 to 160 000 μL–1, serum ferritin from 7600 to 1200 ng/mL. Clinically, her fever subsided complete and regression was noted in the cervical lymphadenopathy and hepatosplenomegaly. She was continued on the above medications and was taken up for HSCT after 4 weeks. Both parents were found to be haplo-matched and there were no compatible matched donors in unrelated registries worldwide. She was taken up for a haploidentical stem cell transplant from her father with negative donor specific antibodies. Conditioning chemotherapy included rabbit anti-thymocyte globulin, fludarabine, treosulphan, and one dose of total body irradiation of 200 centigray. Posttransplant cyclophosphamide was given on Day +3 and Day +4 at 50 mg/kg/day. She achieved neutrophil engraftment by day 16 to 20 postinfusion. She is currently 100-day post HSCT with no significant graft-versus-host disease or post-HSCT complications with 100% donor chimerism documented on two occasions. The JAK1/2 pathway is activated by cytokines, specifically IFNgamma, IL-2 and IL-6, which are critical contributors to inflammation in HLH. Ruxolitinib inhibits the activation of JAK1/2 and its downstream signaling pathways.6 The U.S. Food and Drug Administration approved JAK 1/2 inhibitors, such as ruxolitinib for the treatment of polycythemia vera andmyelofibrosis in adults. Recent data support the efficacy of ruxolitinib in reducing the symptoms of proinflammatory diseases and…

Journal of Clinical Immunology, Feb 1, 2019
Haploidentical stem cell transplantation (haplo SCT) has emerged as an acceptable alternative to ... more Haploidentical stem cell transplantation (haplo SCT) has emerged as an acceptable alternative to matched family donor transplantation for children diagnosed to have primary immune deficiency disorders (PIDs). We present data over 4 years on the challenges and efficacy of unmanipulated T cell replete haplo SCTs with post-transplant cyclophosphamide (PTCy) in children diagnosed to have PIDs. We performed a retrospective study in the pediatric blood and marrow transplantation unit where all children less than 18 years of age diagnosed to have PIDs and who underwent haplo SCT with PTCy from January 2014 to February 2018 were included in the study. Of the 16 transplants included in the study, 5 children were diagnosed to have Wiskott-Aldrich syndrome, 3 with congenital hemophagocytic lymphohistiocytosis, 2 each with Griscelli syndrome and Mendelian susceptibility to mycobacterial diseases, and one each with Chediak-Higashi syndrome, ORAI 1 mutation immune deficiency, severe combined immune deficiency, and Hyper IgM syndrome. The source of stem cells was PBSC in 62.5% and bone marrow in 32.5%. Engraftment by day 16-21 post hematopoietic stem cell transplantation was achieved in 75% transplants with 91% of these remaining in sustained complete chimerism. Acute skin and gut graft versus host disease of grade 2-3 were noted in 50% transplants and cytomegalovirus (CMV) reactivation in 43.7% transplants. One child with congenital HLH succumbed to refractory CMV, adenovirus, and BK virus infection. Cytokine release syndrome (CRS) was noted in 75% transplants with 2 children succumbing to the illness. Tocilizumab was successfully used early in one child. Overall mortality was found to be 37.5% with overall survival of 62.5% with a median follow-up of 23.3 months. In resource limited settings, PTCy has the potential to provide a cost-effective advantage in terms of accessibility of this curative procedure among children with PIDs.
Pediatric Hematology Oncology Journal, Mar 1, 2023

Indian Journal of Hematology and Blood Transfusion, Jan 28, 2019
We present our experience in haploidentical stem cell transplantation (haplo SCT) in children wit... more We present our experience in haploidentical stem cell transplantation (haplo SCT) in children with benign disorders. We performed a retrospective study where children aged up to 18 years diagnosed to have benign disorders and underwent haplo SCT from 2002 to September 2017 were included. Of the 54 children, the most common indications were Fanconi anaemia 12 (22%), severe aplastic anaemia 8 (14%) and primary immune deficiency disorders (PID) 25 (46%). Post-transplant cyclophosphamide (PTCy) was used in 41 (75.9%) and ex vivo T depletion in 13 (24.1%). Engraftment rates were 70% with acute graft versus host disease in 36% and cytomegalovirus reactivation in 55% children. There was a statistically significant difference found between survival with siblings as donors as compared to parents (p value 0.018). Overall survival was 60% which is the 1-year survival, with 68% survival among those with PIDs. Cytokine release syndrome was noted in 12/41 (29%) of children who received T replete graft and PTCy. In children over 6 months of age, PTCy at a cost of INR 1200 provides cost effective T cell depletion comparable with TCR a/b depletion priced at INR 1200,000. Haplo SCT is feasible option for cure in children with benign disorder.

Biology of Blood and Marrow Transplantation, Dec 1, 2020
INTRODUCTION Fanconi anemia is the most common inherited bone marrow failure syndrome, and hemato... more INTRODUCTION Fanconi anemia is the most common inherited bone marrow failure syndrome, and hematopoietic stem cell transplantation (HSCT) is the only curative option. Post-transplant cyclophosphamide (PTCy) is challenging in this group of children, given their increased sensitivity to chemotherapy. PATIENTS AND METHODS We performed a retrospective analysis of the data on children diagnosed with Fanconi anemia who underwent a haploidentical HSCT with PTCy from January 2014 to December 2019. RESULTS Nineteen children (M: F- 0.75:1) underwent 21 haplo-HSCTs with PTCy. Fludarabine, low dose cyclophosphamide, 200 centi-gray total body irradiation were included in the conditioning regimen with 25 milligram/kilogram of PTCy on days +3, +4. Haplo-graft was from a sibling in 38%, father in 57% transplants. The source of stem cells was peripheral blood stem cells in 81%, bone marrow in 19% transplants, median CD34 dose being 5.0 × 106/kilogram. We documented engraftment in 84% and primary graft failure in 10% transplants. N-acetylcysteine (NAC) was infused concomitantly during cyclophosphamide in 13 children. Mucositis of grade 2 and 3 were lower among those who received NAC as compared to those who did not (30% and 15% vs. 33% and 50%); while transaminitis was higher among those who did not receive the infusion. The incidence of acute graft versus host disease (GVHD) was 68%, and 81% of these were steroid responsive (grade I/II). We documented chronic GVHD in 25% children, predominantly involving the skin and mouth, which responded to low dose steroids and ruxolitinib. Serum ferritin was monitored twice weekly as a surrogate marker for cytokine release syndrome due to non-availability of IL6 levels. A one- or two-log increase in the titers of ferritin associated with clinical features guided the early addition of steroids in the peri-engraftment period. The mean survival was found to be less among those with high serum ferritin (>10000ng/dl) in the peri-engraftment period as compared to those with ferritin <10000ng/dl (mean survival of 25 ± 10 months vs. 50 ± 6 months respectively). The overall survival in our cohort was 68.4% with a mean survival time of 41.5 months (95% CI 29.3 months to 53.8 months), with a statistically significant correlation between inferior outcome and having received over 15 transfusions before HSCT (P-value 0.01). CONCLUSION PTCy can be considered a viable option in children with Fanconi anemia, particularly in resource-limited settings given the high costs of HSCTs. Focused interventions in this subset of children help improve survival outcomes. Early identification of cytokine release syndrome and risk-adapted steroid therapy during engraftment helps prevent mortality. The concomitant use of N-acetyl cysteine during cyclophosphamide infusion helps reduce oxygen free radical related tissue damage and reduce regimen-related toxicity.
Indian Journal of Hematology and Blood Transfusion, Apr 17, 2023
Pediatric Hematology Oncology Journal, Dec 1, 2021
Pediatric Critical Care Medicine, Jul 1, 2005
Pediatric Critical Care Medicine is proud to present translations of selected abstracts into Chin... more Pediatric Critical Care Medicine is proud to present translations of selected abstracts into Chinese, French, Italian, Japanese, Portuguese, and Spanish. We sincerely thank the following for their important work on these translations:
Journal of pediatric critical care, 2023
Indian Pediatrics, Jun 27, 2022
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Papers by Indira Jayakumar