Infectious complications in children with nephrotic syndrome: Can they be prevented?

Background: Sixty percent of children with nephrotic syndrome have frequently relapsing or steroid-dependent course. Serious infections like peritonitis, cellulitis, pneumonia etc. and anasarca with reduced urine output and complications there of including acute kidney injury and thromboembolism contribute significantly to morbidity and mortality in these children.

New-onset diabetic ketoacidosis with purpura fulminans in a child with COVID-19-related multisystem inflammatory syndrome.

Background: Coronavirus disease 2019 (COVID 19) usually causes a mild illness among children. However, in a minority of children, it may be associated with the life-threatening multisystem inflammatory syndrome (MIS-C), or thrombotic microangiopathy, or sequelae like type-1 diabetes mellitus (T1DM). We describe a previously healthy, 12-year-old boy with new-onset T1DM with diabetic ketoacidosis (DKA) in the setting of MIS-C, with a course complicated by thrombotic microangiopathy.

Impact of Cerebral Oxygen Saturation Monitoring on Short-term Neurodevelopmental Outcomes in Neonates with Encephalopathy - A Prospective Cohort Study.

Background: Near-infrared spectroscopy (NIRS) has been used for monitoring cerebral oxygen saturation (rSO2) in neonates. There is a lack of data from low-middle income countries (LMIC) setting of cerebral rSO2 in neonates with encephalopathy of diverse etiologies. This study aimed to monitor cerebral rSO2 using NIRS in encephalopathic neonates to maintain the rSO2 between 55 to 85 % in the first 72 hours of admission to improve short-term neurodevelopmental outcomes (NDO).

Upshaw-Schulman Syndrome with a Novel Deletion in Exon 17 of ADAMTS 13 Gene.

Upshaw-Schulman syndrome is a rare congenital form of thrombotic thrombocytopenic purpura (TTP) characterized by single or recurrent episodes of thrombocytopenia, microangiopathic hemolyticanemia (MAHA), and widespread microvascular thrombosis, leading to the ischemic damage of multiple organs (mainly kidney, heart and brain). A 6-mo-old female infant, second born of a third-degree consanguineous marriage, with a history of severe neonatal jaundice with thrombocytopenia secondary to hemolysis requiring exchange transfusion on day 2 of life, presented with high-grade fever without focus of 2-d duration. Initial workup revealed microangiopathic hemolyticanemia with thrombocytopenia.

Clinical and molecular spectrum associated with Polymerase-γ related disorders.

Background: pathogenic variants are the commonest single-gene cause of inherited mitochondrial disease. However, the data on clinicogenetic associations in -related disorders are sparse. This study maps the clinicogenetic spectrum of -related disorders in the pediatric population.

Correction of hypernatremic dehydration in neonates with supervised breast-feeding: A cross-sectional observational study.

Background: Hypernatremic dehydration is an uncommon but a serious cause of readmission in neonates especially in the ones on exclusive breast-feeding. The management of such neonates is challenging as serious complications can occur both because of hypernatremic dehydration and its rapid correction. The aim was to study the clinical profile of neonates with hypernatremic dehydration and determine the outcome of these neonates after appropriate management.

Novel RNASEH2C mutation in multiple members of a large family: insights into phenotypic spectrum of Aicardi-Goutières Syndrome.

Background: Aicardi-Goutières syndrome (AGS) is a genetic inflammatory disorder that presents with early infantile encephalopathy. We report the clinical and molecular details of multiple members of a family with AGS secondary to a novel RNASEH2C mutation, highlighting the evolution of phenotypic abnormalities in AGS.

Methods: Between February 2018 and June 2019, a pedigree tree was constructed for 141 members of a family.