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Homozygous WNT9B variants in two families with bilateral renal agenesis/hypoplasia/dysplasia

3 days 9 hours ago
WNT9B plays a key role in the development of the mammalian urogenital system. It is essential for the induction of mesonephric and metanephric tubules, the regulation of renal tubule morphogenesis, and the regulation of renal progenitor cell expansion and differentiation. To our knowledge, WNT9B has not been associated with renal defects in humans; however, WNT9B^(-/-) mice have renal agenesis/hypoplasia and reproductive tract abnormalities. We report four individuals from two unrelated...
Gabrielle Lemire

Fluid management, electrolytes imbalance and renal management in neonates with neonatal encephalopathy treated with hypothermia

4 days 9 hours ago
Kidney dysfunction and acute kidney injury (AKI) frequently accompanies neonatal encephalopathy and contributes to neonatal morbidity and mortality. While there are currently no proven therapies for the treatment of AKI, understanding the pathophysiology along with early recognition and treatment of alterations in fluid, electrolyte and metabolic homeostasis that accompany AKI offer opportunity to reduce associated morbidity. Promising new tests and technologies, including urine and serum...
Jeffrey L Segar

Neonatal Renal Failure in the Setting of Anorectal Malformation: A Case Report and Literature Review

1 week 1 day ago
Anorectal malformations (ARMs) can occur in isolation or in association with other anomalies, most commonly those of the genitourinary systems. Morbidity and mortality are highest among patients who develop end-stage renal disease (ESRD) either from severe congenital anomalies (dysplastic kidneys) or from repeated infections in those who have vesicoureteral reflux or persistent recto-urinary fistulas. We describe our management strategy for a patient born with an ARM and bilateral dysplastic...
Wendy Jo Svetanoff

Amnion epithelial cells are an effective source of factor H and prevent kidney complement deposition in factor H-deficient mice

1 week 4 days ago
Complement factor H (FH) is the main plasma regulator of the alternative pathway of complement. Genetic and acquired abnormalities in FH cause uncontrolled complement activation amplifying, with the consequent accumulation of complement components on the renal glomeruli. This leads to conditions such as C3 glomerulopathy (C3G) and atypical hemolytic uremic syndrome (aHUS). There is no effective therapy for these diseases. Half of the patients progress to end-stage renal disease and the condition...
Federica Casiraghi

Case Report: Neonatal Diabetes Mellitus Caused by a Novel GLIS3 Mutation in Twins

2 weeks 1 day ago
CONCLUSIONS: This report highlights the importance of early molecular diagnosis for appropriate management of NDM. We describe a novel nonsense mutation of GLIS3 causing NDM, extend the phenotype, and discuss the challenges in clinical management. Our findings provide new areas for further investigation into the roles of GLIS3 in the pathophysiology of diabetes mellitus.
Shira London

Spontaneous intraperitoneal renal rupture with urinoma formation in the fetus

2 weeks 1 day ago
Spontaneous intraperitoneal renal rupture with urinoma formation in fetuses is an unusual condition that is caused by upper or lower urinary tract obstruction. We report the case of a neonatal male infant who presented with a spontaneous intraperitoneal right renal rupture accompanying ipsilateral ureterovesical junction obstruction (UVJO). Fetuses with UVJO accompanying contralateral multicystic dysplastic kidney should be observed carefully because of the risk of spontaneous renal rupture.
Junki Harada

Adherent and suspension baby hamster kidney cells have a different cytoskeleton and surface receptor repertoire

2 weeks 4 days ago
Animal cell culture, with single cells growing in suspension, ideally in a chemically defined environment, is a mainstay of biopharmaceutical production. The synthetic environment lacks exogenous growth factors and usually requires a time-consuming adaptation process to select cell clones that proliferate in suspension to high cell numbers. The molecular mechanisms that facilitate the adaptation and that take place inside the cell are largely unknown. Especially for cell lines that are used for...
Veronika Dill

BHK-21 Cell Clones Differ in Chikungunya Virus Infection and MXRA8 Receptor Expression

2 weeks 6 days ago
Baby hamster kidney-21 (BHK-21) cells are widely used to propagate and study many animal viruses using infection and transfection techniques. Among various BHK-21 cell clones, the fibroblast-like BHK-21/C-13 line and the epithelial-like BHK-21/WI-2 line are commonly used cell clones for alphavirus research. Here we report that BHK-21/WI-2 cells were significantly less susceptible to primary infection by the alphavirus chikungunya virus (CHIKV) than were BHK-21/C-13 cells. The electroporation...
Peiqi Yin

Subcutaneous Fat Necrosis and Hypercalcemia with Nephrocalcinosis in Infancy: Case Report and Review of the Literature

2 weeks 6 days ago
Subcutaneous fat necrosis is an uncommon benign panniculitis affecting more commonly full-term newborns. It has been associated with birth asphyxia and meconium aspiration, as well as therapeutic hypothermia. Although the prognosis is generally favorable, complications such as hypercalcemia, thrombocytopenia, hypoglycemia and hypertriglyceridemia may complicate its course. The most serious complication is hypercalcemia that may reach life threatening levels and can be associated with...
Katerina Chrysaidou

Production of a Foot-and-Mouth Disease Vaccine Antigen Using Suspension-Adapted BHK-21 Cells in a Bioreactor

2 weeks 6 days ago
The baby hamster kidney-21 (BHK-21) cell line is a continuous cell line used to propagate foot-and-mouth disease (FMD) virus for vaccine manufacturing. BHK-21 cells are anchorage-dependent, although suspension cultures would enable rapid growth in bioreactors, large-scale virus propagation, and cost-effective vaccine production with serum-free medium. Here, we report the successful adaptation of adherent BHK-21 cells to growth in suspension to a viable cell density of 7.65 × 10⁶ cells/mL on day...
Soonyong Park

Sexual Dimorphism of Corticosteroid Signaling during Kidney Development

2 weeks 6 days ago
Sexual dimorphism involves differences between biological sexes that go beyond sexual characteristics. In mammals, differences between sexes have been demonstrated regarding various biological processes, including blood pressure and predisposition to develop hypertension early in adulthood, which may rely on early events during development and in the neonatal period. Recent studies suggest that corticosteroid signaling pathways (comprising glucocorticoid and mineralocorticoid signaling pathways)...
Margaux Laulhé

Renal Handling of Albumin-From Early Findings to Current Concepts

2 weeks 6 days ago
Albumin is the main protein of blood plasma, lymph, cerebrospinal and interstitial fluid. The protein participates in a variety of important biological functions, such as maintenance of proper colloidal osmotic pressure, transport of important metabolites and antioxidant action. Synthesis of albumin takes place mainly in the liver, and its catabolism occurs mostly in vascular endothelium of muscle, skin and liver, as well as in the kidney tubular epithelium. Long-lasting investigation in this...
Jakub Gburek

Comparative Proteomic Profiling: Cellular Metabolisms Are Mainly Affected in <em>Senecavirus</em> A-Inoculated Cells at an Early Stage of Infection

2 weeks 6 days ago
Senecavirus A (SVA), also known as Seneca Valley virus, belongs to the genus Senecavirus in the family Picornaviridae. SVA can cause vesicular disease and epidemic transient neonatal losses in pigs. This virus efficiently propagates in some non-pig-derived cells, like the baby hamster kidney (BHK) cell line and its derivate (BSR-T7/5). Conventionally, a few proteins or only one protein is selected for exploiting a given mechanism concerning cellular regulation after SVA infection in vitro....
Fuxiao Liu

Nutrition Considerations in Neonatal Extracorporeal Life Support

2 weeks 6 days ago
Extracorporeal life support (ECLS) is a life-saving therapy, but neonates who require ECLS have unique nutritional needs and require aggressive, early nutritional support. These critically ill neonates are at increased risk for long-term feeding difficulties, malnutrition, and growth failure with associated increased morbidity and mortality. Unfortunately, few studies specific to this population exist. Clinical guidelines published by the American Society for Parenteral and Enteral Nutrition are...
Heidi J Murphy

Long-term follow-up of premature infants with urinary tract infection

3 weeks 3 days ago
Urinary tract infection (UTI) is common in preterm infants and may have long-term sequela, such as recurrent infections and renal scarring in older children. We assessed long-term outcomes of preterm infants with UTI, born during 1996-2008 in Schneider Children's Medical Center's neonatal intensive care unit (NICU), and incidence of UTI recurrence. Of 89 preterm infants, seven were excluded due to prenatal diagnosis of congenital anomalies of the kidney and urinary tract (CAKUT), 41 interviewed...
Lotem Goldberg