Measurable residual mutated IDH2 before allogeneic transplant for acute myeloid leukemia.

Routine genetic profiling of acute myeloid leukemia (AML) at initial diagnosis has allowed subgroup specific prognostication, drug development, and clinical management strategies. The optimal approach for treatment response assessment for AML subgroups has not yet however been determined. A nationwide cohort of 257 adult patients in first remission (CR1) from AML associated with an IDH2 mutation (IDH2m) undergoing allogeneic transplant during the period 2013-2019 in the United States had rates of relapse and survival three years after transplantation of 24% and 71%, respectively.

Transient Neonatal Hypocortisolism in Neonates with Hypoglycemia - Coexistence or Cause?

Introduction: Infants born preterm, with low birth weight (LBW), or with perinatal stress are at high risk for neonatal hypoglycemia. Low cortisol levels have also been demonstrated in this group of neonates, which is often transient. We report a series of neonates with transient hypocortisolism who had neonatal hypoglycemia.

mutation screening for patients at risk of myeloid malignancy.

There is increasing recognition of the risk of developing therapy-related myeloid malignancy, including after cellular therapy. While retrospective studies have implicated pre-existing mutated hematopoietic clones as a common causative mechanism, no prospective screening to identify those patients at greatest risk is currently possible. We demonstrate that ultradeep DNA-sequencing prior to therapy may be used for discovery of mutations that are subsequently associated with malignancy.

Measurable Residual before Allogeneic Transplant for Acute Myeloid Leukemia.

Measurable residual disease (MRD) in adults with acute myeloid leukemia (AML) in complete remission is an important prognostic marker, but detection methodology requires optimization. The persistence of mutated or -ITD in the blood of adult patients with AML in first complete remission (CR1) prior to allogeneic hematopoetic cell transplant (alloHCT) has been established as associated with increased relapse and death after transplant. The prognostic implications of persistence of other common AML-associated mutations, such as , at this treatment landmark however remains incompletely defined.

Single- versus Divided-Dose Prednisolone for the First Episode of Nephrotic Syndrome in Children: An Open-Label RCT.

Background: Early morning single-dose prednisolone has a hypothetical advantage of less hypothalamic-pituitary-adrenal (HPA) axis suppression, but lack of robust evidence has resulted in variation in practice, with divided-dose prednisolone still commonly used. We conducted this open-label randomized control trial to compare HPA axis suppression between single-dose or divided-dose prednisolone among children with first episode of nephrotic syndrome.

Methods: Sixty children with first episode of nephrotic syndrome were randomized (1:1) to receive prednisolone (2 mg/kg per day), either as single or two divided doses for 6 weeks, followed by single alternative daily dose of 1.

Challenges to manage primary chylopericardium in children.

Primary chylopericardium (PC) is a rare entity in the pediatric population with very few reported cases. Most cases of chylopericardium manifest after trauma or following cardiac surgery. The other etiologies which may lead to chylopericardium are malignancy, tuberculosis, or congenital lymphangiomatosis.

DNA Sequencing to Detect Residual Disease in Adults With Acute Myeloid Leukemia Prior to Hematopoietic Cell Transplant.

Importance: Preventing relapse for adults with acute myeloid leukemia (AML) in first remission is the most common indication for allogeneic hematopoietic cell transplant. The presence of AML measurable residual disease (MRD) has been associated with higher relapse rates, but testing is not standardized.

Objective: To determine whether DNA sequencing to identify residual variants in the blood of adults with AML in first remission before allogeneic hematopoietic cell transplant identifies patients at increased risk of relapse and poorer overall survival compared with those without these DNA variants.

Increased incidence of hematologic malignancies in SCD after HCT in adults with graft failure and mixed chimerism.

Lawal et al report on a 45-fold increase in secondary hematologic malignancy in 120 patients following hematopoietic stem cell transplantation (HSCT) for sickle cell disease (SCD), comparable to what has been reported following gene therapy. Notably, the cohort is enriched for older patients and for haploidentical transplant recipients with mixed chimerism following HSCT. These data further support the idea that pre-existing premalignant myeloid clones undergo clonal selection in the setting of nonmyeloablative HSCT and contribute to secondary malignancy.

Neonatal MIS-C: Managing the Cytokine Storm.

A term infant girl with uneventful antenatal history had an erythematous rash followed by fever from day 8. She was diagnosed with late-onset sepsis and was treated accordingly. She received immunoglobulin for persistent thrombocytopenia, after which there was transient improvement.

Clinico-Epidemiological Profile, Etiology, and Imaging in Neonatal Stroke: An Observational Study from Eastern India.

Aim: The aim of this study was to assess the clinico-epidemiological profile, etiology, and imaging findings in neonatal stroke (NS).

Materials And Methods: This was a retrospective, observational study on neonates presenting with stroke between August 2014 and July 2016 to a tertiary care hospital in eastern India.

Results: In all, 43 neonates were analyzed, with a male-to-female ratio of 2.

Lipid Profile in Children With Thalassemia: A Prospective Observational Study From Eastern India.

This was a prospective observational study to evaluate abnormalities in lipid profile in 50 children with transfusion dependent thalassemia. Dyslipidemia characterized by high triglycerides, low high density lipoprotein (HDL), and high total cholesterol: HDL ratio was noted. These pro atherogenic risk factors may be lead to significant cardiovascular morbidity in these patients.

X-Linked Myotubular Myopathy and Duchenne Muscular Dystrophy in a Preterm Infant: A Rare Combination.

Disorders of central and peripheral nervous system should be considered in floppy infants with ventilator dependence. Workup for neuromuscular disorders should be undertaken in infants with hypotonia, weakness, contractures, feeding difficulties, or failed attempts at extubation. We present the case of a preterm infant with hypotonia and ventilator dependence where despite a positive result, further investigations were undertaken because of lack of clinical correlation.

Selective Self-Assembly of 5-Fluorouracil through Nonlinear Solvent Response Modulates Membrane Dynamics.

Controllable self-assembly and understanding of the interaction between single metabolite fibrils and live-cell membranes have paramount importance in providing minimal treatment in several neurodegenerative disorders. Here, utilizing the nonlinear nature and peculiar hydrogen bonding behavior of the dimethyl sulfoxide (DMSO)-water mixture, the selective self-assembly of a single metabolite 5-fluorouracil (5-FU) is achieved. A direct correlation between water availability and selective self-assembly of 5-FU is ratified from the excited-state dynamics.

A portable spinning disc for complete blood count (CBC).

Complete Blood Count (CBC) is a collection of the most commonly required clinical tests to assess the manifestations of pathological conditions in blood. The existing clinical methods for this test are prohibitively expensive for the underprivileged global population due to the requirements of sophisticated instrumentation and trained personnel. To overcome these, we propose a unique low cost device as a blood cell counting platform.

Staphylococcal superantigen-like proteins interact with human MAP kinase signaling protein ERK2.

This study aimed to identify the intracellular binding partner of a unique class of staphylococcal secreted exotoxins called superantigen-like proteins (SSL) from human macrophage and keratinocyte cell lysates. Here, we report that SSL1 specifically binds to human extracellular signal-regulated kinase 2 (hERK2), an important stress-activated kinase in mitogen-activated protein kinase signaling pathways. Western blot and in vitro binding studies with recombinant hERK2 confirmed the binding interaction of SSL1, SSL7, and SSL10 with hERK2.

Dorsal dermal sinus presenting as quadriparesis.

Dorsal dermal sinus (DDS) represents the spectrum of spinal dysraphism. Children may present with features of meningitis. A 13-month male child presented with features of meningitis and quadriparesis.

Highly Luminescent Thermoresponsive Green Emitting Gold Nanoclusters for Intracellular Nanothermometry and Cellular Imaging: A Dual Function Optical Probe.

In view of many promising applications of gold nanoclusters (AuNCs), nanothermometry is an important field of research in biology and medicine. Here, we demonstrate the temperature dependent photophysical properties of highly luminescent green emitting 6-aza-2-thiothymine/l-arginine-stabilized Au nanosclusters (ATT/Arg Au NCs) by using steady state and time-resolved photoluminescence spectroscopy. Significantly, thermoresponsive properties of these highly photostable and biocompatible Au NCs are reversible, which endow the probe for further bioanalytical applications with great prospects.

Unusual presentation of late-onset disseminated staphylococcal sepsis in a preterm infant.

An ex-30-week gestation, preterm male baby was admitted to a tertiary neonatal unit and noted to have increased ventilator requirements and diagnosed with sepsis. The baby also developed an abscess over the left elbow and over the xiphisternum along with a decrease in movement of the left hand and the right leg. Panton-Valentine leukocidin (PVL)-producing (SA) was isolated from the blood culture.