Framework for Kidney Health Follow-Up Among Neonates With Critical Cardiac Disease: A Report From the Neonatal Kidney Health Consensus Workshop.

Acute kidney injury is common among neonates with critical cardiac disease. Risk factors and associations with kidney-related outcomes are heterogeneous and distinct from other neonates. As survival of children with critical cardiac disease increases to adulthood, the burden of chronic kidney disease is increasing.

Kidney Health Monitoring in Neonatal Intensive Care Unit Graduates: A Modified Delphi Consensus Statement.

Importance: Kidney disease is common in infants admitted to the neonatal intensive care unit (NICU). Despite the risk of chronic kidney disease (CKD) in infants discharged from the NICU, neither evidence- nor expert-based recommendations exist to guide clinical care after discharge.

Objective: To develop recommendations for risk stratification and kidney health monitoring among infants after discharge from the NICU.

Critical Organ Dysfunction and Preoperative Mortality in Newborns with Hypoplastic Left Heart Syndrome.

Hypoplastic left heart syndrome (HLHS) is fatal without surgical intervention. An important subset of HLHS patients die prior to surgical intervention, but this population is underevaluated. The neonatal sequential organ failure assessment score (nSOFA) is an operational definition of organ dysfunction that can identify those with a high risk of mortality among neonatal intensive care unit (NICU) patients.

Integrative modeling of hemodynamic changes and perfusion impairment in coronary microvascular disease.

Coronary microvascular disease is one of the responsible factors for cardiac perfusion impairment. Due to diagnostic and treatment challenges, this pathology (characterized by alterations to microvasculature local hemodynamics) represents a significant yet unsolved clinical problem. Due to the poor understanding of the onset and progression of this disease, we propose a new and noninvasive strategy to quantify hemodynamic changes occurring in the microvasculature.

Perioperative Care Models for Neonates With Congenital Heart Disease: Evolving Role of Neonatology Within the Cardiac Intensive Care Unit.

A multidisciplinary team is needed to optimally care for infants with congenital heart disease (CHD). Different compositions of teams trained in cardiology, critical care, cardiothoracic surgery, anesthesia, and neonatology have been identified as being primarily responsible for perioperative care of this high-risk population in dedicated cardiac intensive care units (CICUs). Although the specific role of cardiac intensivists has become more well defined over the past two decades, the responsibilities of neonatologists remain highly variable in the CICU with neonatologists providing care along with a unique spectrum of primary, shared, or consultative care.

Collagen VI Is a Gi-Biased Ligand of the Adhesion GPCR GPR126/ADGRG6.

GPR126/ADGRG6, a member of the adhesion G-protein-coupled receptor family, balances cell differentiation and proliferation through fine-tuning of intracellular cAMP levels, which is achieved through coupling to Gs and Gi proteins. While GPR126-mediated cAMP increase has been proven to be essential for differentiation of Schwann cells, adipocytes and osteoblasts, Gi-signaling of the receptor was found to propagate breast cancer cell proliferation. Extracellular ligands or mechanical forces can modulate GPR126 activity but require an intact encrypted agonist sequence, coined the .

Factors associated with the need for inotropic support following pulmonary artery banding surgery for CHD.

Objective: We aimed to identify factors independently associated with the need for inotropic support for low cardiac output or haemodynamic instability after pulmonary artery banding surgery for CHD.

Methods: We performed a retrospective chart review of all neonates and infants who underwent pulmonary banding between January 2016 and June 2019 at our institution. Bivariate and multivariable analyses were performed to identify factors independently associated with the use of post-operative inotropic support, defined as the initiation of inotropic infusion(s) for depressed myocardial function, hypotension, or compromised perfusion within 24 hours of pulmonary artery banding.

Documentation of acute kidney injury at discharge from the neonatal intensive care unit and role of nephrology consultation.

Objective: To investigate whether NICU discharge summaries documented neonatal AKI and estimate if nephrology consultation mediated this association.

Study Design: Secondary analysis of AWAKEN multicenter retrospective cohort.

Exposures: AKI severity and diagnostic criteria.

Retrieval Of Balloon From Right Coronary Artery Surgically.

Angioplasty balloon entrapment remains an infrequent but dangerous obstacle that requires forbearance and pre-built management strategies in order to avoid morbidity or worse, mortality. Here, we discuss an un-expected hurdle of an undeflated stuck balloon with fractured shaft during angioplasty of proximal right coronary artery (RCA) in a 60-year-old male which was attempted percutaneously and redeemed surgically but massive infarction to RCA territory was inevitable.

Whole exome sequencing identifies a novel splice-site mutation in IMPG2 gene causing Stargardt-like juvenile macular dystrophy in a north Indian family.

We report on the genetic analysis of a north Indian family affected with Stargardt-like juvenile macular dystrophy. Considering an autosomal recessive inheritance of macular dystrophy in the recruited family, whole exome sequencing was employed in two affected siblings and their mother. We have identified a novel splice-site variant NC_000003.

Improving the identification of acute kidney injury in the neonatal ICU: three centers' experiences.

Objective: To describe three different standardized approaches to improving neonatal acute kidney injury (AKI) identification and the impact on AKI identification, incidence, and nephrology consultation and referral.

Study Design: A retrospective cohort study in three academic NICUs. We compared AKI identification, AKI incidence, nephrology consultation, and nephrology follow-up before and after implantation of local protocols to standardize neonatal AKI identification.

BRCA1 Promoter Methylation and Clinical Outcomes in Ovarian Cancer: An Individual Patient Data Meta-Analysis.

Background: BRCA1 methylation has been associated with homologous recombination deficiency, a biomarker of platinum sensitivity. Studies evaluating BRCA1-methylated tubal and ovarian cancer (OC) do not consistently support improved survival following platinum chemotherapy. We examine the characteristics of BRCA1-methylated OC in a meta-analysis of individual participant data.

Advances in Protein-Based Materials: From Origin to Novel Biomaterials.

Biomaterials play a very important role in biomedicine and tissue engineering where they directly affect the cellular activities and their microenvironment . Myriad of techniques have been employed to fabricate a vast number natural, artificial and recombinant polymer s in order to harness these biomaterials in tissue regene ration , drug delivery and various other applications. Despite of tremendous efforts made in this field during last few decades, advanced and new generation biomaterials are still lacking.

EPHB4 is a therapeutic target in AML and promotes leukemia cell survival via AKT.

EPHB4, an ephrin type B receptor, is implicated in the growth of several epithelial tumors and is a promising target in cancer therapy; however, little is known about its role in hematologic malignancies. In this article, we show that EPHB4 is highly expressed in ∼30% of acute myeloid leukemia (AML) samples. In an unbiased RNA interference screen of primary leukemia samples, we found that EPHB4 drives survival in a subset of AML cases.

Whole exome sequencing unveils a frameshift mutation in CNGB3 for cone dystrophy: A case report of an Indian family.

Rationale: Genetic elucidation of cone-dominated retinal dystrophies in Indian subcontinent is much needed to identify and catalog underlying genetic defects. In this context, the present study recruited a consanguineous Indian family affected with autosomal recessive cone dystrophy (CD). Considering the huge genetic heterogeneity and recessive inheritance of the disease, we chose to dissect out causal variant in this family by whole exome sequencing (WES).

GLI3 repressor determines Hedgehog pathway activation and is required for response to SMO antagonist glasdegib in AML.

The Hedgehog (Hh) signaling pathway is activated in many cancers and is a promising target for therapeutic development. Deletions in the receptor Patched (PTCH) or activating mutations in Smoothened (SMO) have been reported in basal cell carcinoma and medulloblastoma, but are largely absent in most tumor types. Therefore, the mechanism of pathway activation in most cancers, including hematological malignancies, remains unknown.

Isolated naratriptan-associated ischemic colitis.

We report a 41-year-old woman who developed histology- and colonoscopy-proven ischemic colitis with the use of naratriptan not exceeding the maximum 2 doses a day and 3 days per week and without a known medical or cardiovascular history. By exclusion of other causes of colonic ischemia, naratriptan was considered the sole causal agent. Discontinuation of naratriptan resulted in a complete clinical recovery.

Primary cilia are present on human blood and bone marrow cells and mediate Hedgehog signaling.

Primary cilia are nonmotile, microtubule-based organelles that are present on the cellular membrane of all eukaryotic cells. Functional cilia are required for the response to developmental signaling pathways such as Hedgehog (Hh) and Wnt/β-catenin. Although the Hh pathway has been shown to be active in leukemia and other blood cancers, there have been no reports describing the presence of primary cilia in human blood or leukemia cells.

Neuroimaging in Secondary Headache Disorders.

A secondary headache may develop de novo or in patients with a history of primary headaches, and a thorough history and neurological exam often helps to suspect a secondary etiology. The causes of secondary headaches include tumors, vascular etiologies, structural brain disorders, infection, inflammation, and alterations of cerebrospinal fluid pressure dynamics. Computed tomography (CT) is very sensitive for detecting acute hemorrhage but magnetic resonance imaging (MRI) is preferred over a head CT in subacute and non-emergent cases.

A new clinico-tomographic classification and management algorithm for Descemet's membrane detachment.

Objective: To propose a new clinico-tomographic classification of Descemet's detachment (DD).

Methods: Interventional case series of 35 eyes with DD were clinico-tomographically classified as: (1)Rhegmatogenous DD (RDD)-lax, free floating DM secondary to DM tear/hole/dialysis; ASOCT showing undulating linear signal with total length equalling overlying stromal arc length. (2) Tractional DD (TDD)-foreshortened, taut DM with tractional/fibrotic component; ASOCT showing detached DM chord length less than overlying stromal arc length.

A novel mutation in FRMD7 causes X-linked idiopathic congenital nystagmus in a North Indian family.

Idiopathic congenital nystagmus (ICN) is the most common form of oculomotor disorder characterized by involuntary bilateral ocular oscillations. Primarily the disease is an ocular anomaly but the pathophysiology is associated with neuronal cytoskeletal dynamics in the brain. In the current study, a three generation North Indian family affected with X-linked idiopathic congenital nystagmus (XLICN) was recruited.

Hiccups as a migraine aura.

Objective: To report a case series of hiccup as a migraine aura.

Background: Hiccup is a syndrome of inappropriate muscle contraction. To our knowledge, hiccup as a migraine aura has not been reported.

MTHFR C677T predisposes to POAG but not to PACG in a North Indian population: a case control study.

Hyperhomocysteinemia induced by the C677T genetic variant in MTHFR (methylenetetrahydrofolate reductase) has been implicated in neuronal cell death of retinal ganglion cells (RGC), which is a characteristic feature of glaucoma. However, association of MTHFR C677T with glaucoma has been controversial because of inconsistent results across association studies. Association between MTHFR C677T and glaucoma has not been reported in Indian population.