The roles of homocysteinemia and methylmalonic acidemia in kidney injury in atypical hemolytic uremic syndrome caused by cobalamin C deficiency

4 days 2 hours ago
CONCLUSION: We question the roles of homocysteine and MMA as causative of aHUS/TMA in cblC deficiency as they remained elevated during follow-up but did not result in aHUS/TMA or AKI. Hyperhomocysteinemia and/or MMA caused by other metabolic diseases do not result in aHUS/TMA or AKI. This suggests that other nephrotoxic factors may trigger aHUS/TMA in cblC patients.
William D Wood

MRI monitoring of transplanted neonatal porcine islets labeled with polyvinylpyrrolidone-coated superparamagnetic iron oxide nanoparticles in a mouse model

5 days 2 hours ago
Islet transplantation is a potential treatment option for certain patients with type 1 diabetes; however, it still faces barriers to widespread use, including the lack of tools to monitor islet grafts post-transplantation. This study investigates whether labeling neonatal porcine islets (NPI) with polyvinylpyrrolidone-coated superparamagnetic iron oxide nanoparticles (PVP-SPIO) affects their function, and whether this nanoparticle can be utilized to monitor NPI xenografts with magnetic resonance...
Kieran Purich

Oxidative Stress in Preterm Newborns

1 week 2 days ago
Preterm babies are highly susceptible to oxidative stress (OS) due to an imbalance between the oxidant and antioxidant systems. The generation of free radicals (FR) induces oxidative damage to multiple body organs and systems. OS is the main factor responsible for the development of typical premature infant diseases, such as bronchopulmonary dysplasia, retinopathy of prematurity, necrotizing enterocolitis, intraventricular hemorrhage, periventricular leukomalacia, kidney damage, eryptosis, and...
Chiara Lembo

Maternal Perinatal Characteristics in Patients with Severe Preeclampsia: A Case-Control Nested Cohort Study

1 week 2 days ago
Preeclampsia is one of the most worrisome complications during pregnancy, affecting approximately 1 out of 20 women worldwide. Preeclampsia is mainly characterized by a sustained hypertension, proteinuria, also involving a significant organ dysfunction. Moreover, 25% of the cases could be classified as severe preeclampsia (SP), a serious condition that could be life-threatening for both the mother and fetus. Although there are many studies focusing on preeclampsia, less efforts have been made in...
Irene Aracil Moreno

Effect of cell density on the biological titer and yield of 146S fraction of foot-and-mouth disease virus O in cell suspension

1 week 3 days ago
Foot-and-mouth disease (FMD) is a highly infectious disease of cattle and other cloven-hoofed animals, causing huge economic losses annually worldwide. This disease is endemic in Pakistan where the serotypes of the foot-and-mouth disease virus (FMDV) are A, O and ASIA-1. At present, trivalent FMDV vaccines are being used to prevent FMD but the current production process is laborious and is unable to fulfill the needs of the meat and dairy industries. To meet the vaccine needs of Pakistan, the...
Azka Rizvi

Quality of Maternal Death Documentation in Afghanistan: A Retrospective Health Facility Record Review

1 week 5 days ago
Objectives: To assess the quality of health facility documentation related to maternal deaths at health facilities in Afghanistan. Methods: Analysis of a subset of findings from the 2016 National Maternal and Newborn Health Quality of Care Assessment in Afghanistan. At each facility, maternity registers were reviewed to obtain data related to maternity caseload, and number and causes of maternal deaths in the year preceding the survey. Detailed chart reviews were conducted for up to three...
Farzana Maruf

Structural and functional changes in the kidney caused by adverse fetal and neonatal environments

1 week 5 days ago
Health and disease risk in the adulthood are known to be affected by the early developmental environment. Kidney diseases are one of these diseases, and kidneys are altered both structurally and functionally by adverse pre- and perinatal events. The most known structural change is low nephron number seen in subjects born low birth weight and/or preterm. In various animal models of intrauterine growth restriction (IUGR), one of the causes of low birth weight, the mechanism of low nephron number...
Midori Awazu

A knock-in rat model unravels acute and chronic renal toxicity in glutaric aciduria type I

2 weeks 2 days ago
Glutaric aciduria type I (GA-I, OMIM # 231670) is an autosomal recessive inborn error of metabolism caused by deficiency of the mitochondrial enzyme glutaryl-CoA dehydrogenase (GCDH). The principal clinical manifestation in GA-I patients is striatal injury most often triggered by catabolic stress. Early diagnosis by newborn screening programs improved survival and reduced striatal damage in GA-I patients. However, the clinical phenotype is still evolving in the aging patient population....
Mary Gonzalez Melo

Extending the phenotype of DeSanto-Shinawi syndrome: A case report and literature review

2 weeks 3 days ago
DeSanto-Shinawi syndrome (DESSH, OMIM #616708) is a rare autosomal dominant neurodevelopmental disorder caused by loss-of-function variants in the WAC gene. Affected individuals are characterized by neonatal hypotonia, developmental delay, intellectual disability, behavioral problems, and dysmorphism. Epilepsy is present in some of the patients with DESSH. By far, less than 30 affected individuals have been reported worldwide. Herein, we report a 9-year-old Chinese girl with molecularly...
Stephanie Ho

Rare Clinical Case of Cryopyrin-Associated Periodic Syndrome Presented with Ankylosing Spondylitis: A Case Report

2 weeks 5 days ago
CONCLUSION: From the analysis, we can conclude that patient M. from early childhood suffers from a severe Neonatal-onset Multisystem Inflammatory Disease of a genetic nature. For a long time, the patient was diagnosed with ankylosing spondylitis, and appropriate treatment was carried out without significant success. The correct diagnosis of CAPS was made only in 2018. This patient has conditions of both CAPS and AS together, which is a very rare association in rheumatological practice. The only...
Anna A Zayaeva

Maternal, Decidual, and Neonatal Lymphocyte Composition Is Affected in Pregnant Kidney Transplant Recipients

3 weeks ago
Pregnancy after renal transplantation is associated with an increased risk of complications. While a delicately balanced uterine immune system is essential for a successful pregnancy, little is known about the uterine immune environment of pregnant kidney transplant recipients. Moreover, children born to kidney transplant recipients are exposed in utero to immunosuppressive drugs, with possible consequences for neonatal outcomes. Here, we defined the effects of kidney transplantation on the...
Dorien Feyaerts

Ultrasound and alternative multimodality imaging of intra-abdominal and pelvic cystic masses in the newborn

3 weeks ago
Introduction: Ultrasound is used commonly to detect and diagnose intra-abdominal and pelvic cystic masses in the newborn as it is easily available, relatively low cost, and non-invasive. Discussion: The diagnosis can be made or narrowed down by determining the location, size, sonographic features, organ involvement, and internal vascularity. The differential diagnoses include hydronephrosis, multicystic dysplastic kidney, adrenal haemorrhage, cystic teratomas, ovarian cysts, enteric cysts,...
Weiyong Lee

Cardiac Fibroblast Growth Factor 23 Excess Does Not Induce Left Ventricular Hypertrophy in Healthy Mice

3 weeks ago
Fibroblast growth factor (FGF) 23 is elevated in chronic kidney disease (CKD) to maintain phosphate homeostasis. FGF23 is associated with left ventricular hypertrophy (LVH) in CKD and induces LVH via klotho-independent FGFR4-mediated activation of calcineurin/nuclear factor of activated T cells (NFAT) signaling in animal models, displaying systemic alterations possibly contributing to heart injury. Whether elevated FGF23 per se causes LVH in healthy animals is unknown. By generating a mouse...
Maren Leifheit-Nestler

Dissemination of Candida auris to deep organs in neonatal murine invasive candidiasis

3 weeks 1 day ago
Candida auris is an emerging multidrug resistant fungal pathogen, which represents a major challenge for newborns systemic infections worldwide. Management of C. auris infections is complicated due to its intrinsic antifungal resistance and the limited information available on its pathogenesis, particularly during neonatal period. In this study, we developed a murine model of C. auris neonatal invasive infection. C. auris dissemination was evaluated by fungal burden and histopathological...
Orlando Flores-Maldonado

Association of early dysnatremia with mortality in the neonatal intensive care unit: results from the AWAKEN study

3 weeks 1 day ago
CONCLUSION: Dysnatremia within the first postnatal week was associated with increased risk of mortality. Hypernatremia and combined hypo/hypernatremia remained significantly associated with mortality in neonates without AKI. This may reflect fluid strategies kidney injury independent of creatinine and urine-output defined AKI, and/or systemic inflammation.
Abby M Basalely

Recurrent lupus nephritis in renal allograft triggered by pregnancy

3 weeks 4 days ago
Herein we report the case of a 37-year-old woman with recurrence of lupus nephritis (LN) in a renal allograft during pregnancy. She had developed end-stage renal disease due to LN and was put on hemodialysis at the age of 26 years. She underwent kidney transplantation at the age 28 years. Maintenance immunosuppressants included methylprednisolone, tacrolimus, and mycophenolate mofetil, which were changed to azathioprine when she desired pregnancy. The renal allograft function remained stable and...
Takahiro Shinzato