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Dialysis infant

Jason Misurac
Neonatal chronic kidney disease (CKD) occurs with an estimated incidence of 1 in 10,000 live births, whereas the incidence of neonatal end-stage renal disease (ESRD) is about 7.1 per million age-related population. The most frequent etiologies are renal hypoplasia/dysplasia, posterior urethral valves, and other congenital anomalies of the kidney and urinary tract. Other etiologies include polycystic kidney disease, cortical necrosis, and renal vascular thrombosis. Management of CKD focuses primarily on replacing renal functions such as erythropoietin, 1,25-hydroxylation of vitamin D, electrolyte homeostasis/excretion, and, in ESRD, waste product removal...
October 9, 2016: Seminars in Fetal & Neonatal Medicine
Shwetal Lalan, Hongying Dai, Bradley A Warady
BACKGROUND: Peritonitis is a severe complication of chronic peritoneal dialysis (CPD) in infants. Few studies have been conducted to evaluate the relationship between hypogammaglobulinemia and peritonitis risk, and the potential benefit of intravenous immunoglobulins (IVIG) therapy in infants receiving CPD. METHODS: Patients aged 0-12 months at initiation of CPD between 1985 and 2012 were eligible for inclusion in this retrospective study. Data collected from the start of CPD up to 2 years post-dialysis initiation included patient demographics, dialysis characteristics, serum immunoglobulin (IgG) levels, IVIG administration history, infectious complications and outcomes...
October 7, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Malcolm G Coulthard
Most babies with chronic renal failure are identified antenatally, and over half that are treated with peritoneal dialysis receive kidney transplants before school age. Most infants that develop acute renal failure have hypotension following cardiac surgery, or multiple organ failure. Sometimes the falls in glomerular filtration and urine output are physiological and reversible, and sometimes due to kidney injury, but (illogically) it is now common to define them all as having 'acute kidney injury'. Contrary to widespread opinion, careful interpretation of the plasma creatinine concentrations can provide sensitive evidence of early acute renal failure...
September 25, 2016: Early Human Development
Louise E Ross, Pauline A Swift, Sandra M Newbold, Kate Bramham, Anne Hurley, Hugh Gallagher
Pregnancy outcomes in patients with end-stage renal disease (ESRD) on dialysis are improving. Recent literature supports intensive hemodialysis (HD) as the modality of choice during pregnancy in ESRD. We report the successful delivery of a healthy infant at full term in a patient with ESRD by supplementing peritoneal dialysis (PD) with intermittent HD to achieve adequate dialysis intensity.
September 2016: Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis
P Danziger, D R Berman, K Luckritz, K Arbour, N Laventhal
OBJECTIVE: Decision-making for pregnancies complicated by severe congenital anomalies of the kidneys and urinary tract (CAKUT) are ethically challenging, partly because the outcomes are not well studied. STUDY DESIGN: Retrospective cohort study of severe cases of CAKUT over 14 years. RESULTS: Seventy-one of the 108 cases could be completely analyzed. Forty-six percent (n=33) infants were live-born; one-third (n=11) survived to 12 months. Twice as many non-surviving infants received a trial of therapy vs comfort care only...
July 28, 2016: Journal of Perinatology: Official Journal of the California Perinatal Association
Lyndsay A Harshman, Bobby G Ng, Hudson H Freeze, Pamela Trapane, Anna Dolezal, Patrick D Brophy, Jane E Brumbaugh
Congenital nephrotic syndrome (NS) in the newborn is most frequently related to mutations in genes specific for structural integrity of the glomerular basement membrane and associated filtration structures within the kidney, resulting in massive leakage of plasma proteins into the urine. Occurrence of congenital NS in a multi-system syndrome is less common. We describe the case of an infant with deteriorating neurological status, seizures, edema, and proteinuria who was found to have a mutation in gene ALG1 and a renal biopsy consistent with congenital NS...
August 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Y H Chan, A Lt Ma, P C Tong, W M Lai, N Kc Tse
OBJECTIVE: To review the outcome for Chinese infants and young children on chronic peritoneal dialysis. METHODS: The Paediatric Nephrology Centre of Princess Margaret Hospital is the designated site offering chronic dialysis to children in Hong Kong. Medical records of children who started chronic peritoneal dialysis before the age of 2 years, from 1 July 1995 to 31 December 2013, were retrieved and retrospectively reviewed. RESULTS: Nine Chinese patients (male-to-female ratio, 3:6) were identified...
August 2016: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
Nina Battelino, Karin Writzl, Nevenka Bratanič, Melita D Irving, Gregor Novljan
Hajdu-Cheney syndrome (HJCYS) is a rare, autosomal dominant, skeletal disorder caused by mutations in the NOTCH2 signaling pathway for which genetic testing has recently become available. Renal abnormalities are associated in at least 10% of cases. We present an 8-year-old Caucasian boy, born with multiple dysmorphic features consistent with HJCYS. Imaging of the urinary tract revealed bilateral cystic dysplastic kidneys with associated vesicoureteral reflux. Renal function has been impaired since birth and deteriorated progressively to end-stage renal disease (ESRD) by the age of two and a half years, when peritoneal dialysis was initiated and only recently renal transplantation was performed...
June 2016: Therapeutic Apheresis and Dialysis
Tomoyuki Sakai, Yoshitaka Murakami, Yusuke Okuda, Riku Hamada, Yuko Hamasaki, Kenji Ishikura, Hiroshi Hataya, Masataka Honda
BACKGROUND: Among comorbidities, pulmonary hypoplasia (PH) is known as a significant risk factor for mortality in infants with end-stage kidney disease (ESKD). However, the final outcomes of infants with both ESKD and PH are still not well defined, as the diagnosis modality, and definition of PH severity remain ambiguous. METHODS: Children initiating peritoneal dialysis during infancy from 1990 to 2015 were followed until death, date of last contact, or the end of 2015...
November 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Leyat Tal, Joseph R Angelo, Ayse Akcan-Arikan
Peritoneal dialysis (PD) is generally considered the preferred extracorporeal therapy for neonates with acute kidney injury (AKI). However, there are situations when PD is not suitable, such as in patients with previous abdominal surgery, hyperammonemia and significant ascites or anasarca. Additionally, with a need to start PD soon after catheter placement, there is increased risk of PD catheter leak and infection. Extracorporeal continuous renal replacement therapy (CRRT) is challenging in severely ill neonates as it requires obtaining adequately sized central venous access to accommodate adequate blood flow rates and also adaptation of a CRRT machine meant for older children and adults...
November 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Mattias Carlström, Carmen Cananau, Antonio Checa, Katarina Wide, Lisa Sartz, Anders Svensson, Craig E Wheelock, Susanne Westphal, Zivile Békássy, Peter Bárány, Jon O Lundberg, Sverker Hansson, Eddie Weitzberg, Rafael T Krmar
BACKGROUND & PURPOSE: Infants on chronic peritoneal dialysis (PD) have an increased risk of developing neurological morbidities; however, the underlying biological mechanisms are poorly understood. In this clinical study, we investigated whether PD-mediated impairment of nitric oxide (NO) bioavailability and signaling, in patients with persistently low systolic blood pressure (SBP), can explain the occurrence of cerebral ischemia. METHODS & RESULTS: Repeated blood pressure measurements, serial neuroimaging studies, and investigations of systemic nitrate and nitrite levels, as well as NO signaling, were performed in ten pediatric patients on PD...
August 31, 2016: Nitric Oxide: Biology and Chemistry
Jun Miyahara, Masaki Yamamoto, Kyoko Motoshige, Naoya Fujita, Shigeru Ohki
Recent advances in neonatal cardiorespiratory management and neonatal renal replacement therapy have led to occasional reports of favorable short-term and long-term outcomes for Potter sequence, once thought to be fatal. The present patient was a girl born at a gestational age of 34 weeks 4 days with a birthweight of 1398 g. She was diagnosed with Potter sequence complicated by pulmonary hypoplasia due to left renal agenesis and small right kidney. Hemodialysis was started because anuria persisted even after persistent pulmonary hypertension receded and cardiorespiratory status improved...
July 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Manel Jellouli, Mariem Ferjani, Kamel Abidi, Chokri Zarrouk, Ouns Naija, J Abdelmoula, Tahar Gargah
The infantile form of primary hyperoxaluria type-1 (PH-1) is characterized by a rapid progression to the end-stage renal disease (ESRD) due to both increased oxalate load and reduced glomerular filtration rate. In the literature, data on this form are limited. The purpose of this study is to analyze retrospectively the clinical, biological, and radiological features of children who were diagnosed with PH-1 during the 1(st) year of life. We reviewed the records of all children with PH-1 diagnosed and followed-up at our department between January 1995 and December 2013...
May 2016: Saudi Journal of Kidney Diseases and Transplantation
Marco Allinovi, Moin Saleem, Paola Romagnani, Peiman Nazerian, Wesley Hayes
BACKGROUND: Optimizing the target weight of infants and children on dialysis remains an important clinical challenge. The use of ultrasound to detect fluid overload in adult patients on dialysis is receiving growing attention. We hypothesized that fluid overload can be quantified in infants and children receiving dialysis using lung ultrasound. METHODS: In this prospective observational study, infants and children receiving dialysis for end-stage renal disease (ESRD) or acute kidney injury (AKI) in a regional paediatric nephrology centre were eligible...
April 6, 2016: Nephrology, Dialysis, Transplantation
Boutaina Zemrani, François Cachat, Olivier Bonny, Eric Giannoni, Jacques Durig, Florence Fellmann, Hassib Chehade
BACKGROUND: Pierson syndrome (PS) is a rare autosomal recessive disorder, caused by mutations in the laminin β2 (LAMB2) gene. It is characterized by congenital nephrotic syndrome, microcoria, and neurodevelopmental deficits. Several mutations with genotype-phenotype correlations have been reported, often with great clinical variability. We hereby report a novel homozygous nonsense mutation in the LAMB2 gene, associated with a severe phenotype presentation. CASE DIAGNOSIS: We describe a term male infant born from consanguineous parents...
2016: European Journal of Medical Research
Nathaniel R Sznycer-Taub, Ray Lowery, Sunkyung Yu, Sonal T Owens, Jennifer C Hirsch-Romano, Gabe E Owens
OBJECTIVES: Patients who require venoarterial extracorporeal membrane oxygenation because of cardiac failure frequently have supranormal blood oxygen tensions (hyperoxia). Recent studies have suggested worse outcomes in patients with hyperoxia after resuscitation from cardiac or respiratory arrests, presumably because of oxidative stress. There are limited data regarding the effect of hyperoxia on outcomes in pediatric patients on venoarterial extracorporeal membrane oxygenation. DESIGN: Retrospective chart review...
April 2016: Pediatric Critical Care Medicine
Aaron G Wightman, Michael A Freeman
Improvements in pediatric dialysis over the past 50 years have made the decision to proceed with dialysis straightforward for the majority of pediatric patients. For certain groups, however, such as children with multiple comorbid conditions, children and families with few social and economic resources, and neonates and infants, the decision of whether to proceed with dialysis remains much more controversial. In this review, we will examine the best available data regarding the outcomes of dialysis in these populations and analyze the important ethical considerations that should guide decisions regarding dialysis for these patients...
August 8, 2016: Clinical Journal of the American Society of Nephrology: CJASN
Rakan Odeh, Damien Noone, Paul R Bowlin, Luis H P Braga, Armando J Lorenzo
PURPOSE: There is paucity of validated objective early imaging markers to help predict future renal deterioration in infants with posterior urethral valves. We evaluated the prognostic value of total renal parenchymal area, renal echogenicity and corticomedullary differentiation regarding future development of chronic kidney disease. MATERIALS AND METHODS: We analyzed initial postnatal ultrasonographic images from serial posterior urethral valve cases seen at a single tertiary referral center using National Institutes of Health sponsored image processing software...
September 2016: Journal of Urology
Betti Schaefer, Maria Bartosova, Stephan Macher-Goeppinger, Akos Ujszaszi, Markus Wallwiener, Joanne Nyarangi-Dix, Peter Sallay, Dorothea Burkhardt, Uwe Querfeld, Viktoria Pfeifle, Bernd Lahrmann, Vedat Schwenger, Elke Wühl, Stefan Holland-Cunz, Franz Schaefer, Claus P Schmitt
The peritoneum plays an essential role in preventing abdominal frictions and adhesions and can be utilized as a dialysis membrane. Its physiological ultrastructure, however, has not yet been studied systematically. 106 standardized peritoneal and 69 omental specimens were obtained from 107 patients (0.1-60 years) undergoing surgery for disease not affecting the peritoneum for automated quantitative histomorphometry and immunohistochemistry. The mesothelial cell layer morphology and protein expression pattern is similar across all age groups...
2016: Scientific Reports
Najya A Attia, Yousef I Marzouk
Context. Pseudohypoaldosteronism type 1 (PHA1) is a life-threatening disease that causes severe hyperkalemia and cardiac arrest if not treated appropriately or if diagnosis is missed. Objective. To report a case of a newborn with vomiting and lethargy, ultimately diagnosed with pseudohypoaldosteronism. Patient. This case presented to the ED at an age of 14 days in hypovolemic shock. There was a family history of sudden infant death, her sister who was diagnosed with CAH and passed away at 3 months of age despite regular hormone replacement...
2016: Case Reports in Endocrinology
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