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    Assessing Dehydration Employing End-Tidal Carbon Dioxide in Children With Vomiting and Diarrhea.
    Pediatr Emerg Care 2017 May 23. Epub 2017 May 23.
    From the *Section of Pediatric Emergency Medicine and Gastroenterology, Department of Pediatrics, †Section of Pediatric Emergency Medicine, Departments of Pediatrics and Physiology and Pharmacology, ‡Department of Pediatrics, Alberta Children's Hospital, Alberta Children's Hospital Research Institute, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada; and §Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
    Objective: Serum bicarbonate reflects dehydration severity in children with gastroenteritis. Previous work in children receiving intravenous rehydration has correlated end-tidal carbon dioxide (EtCO2) with serum bicarbonate. We evaluated whether EtCO2 predicts weight change in children with vomiting and/or diarrhea. Read More

    Characterization of astrovirus-associated gastroenteritis in hospitalized children under five years of age.
    Infect Genet Evol 2017 May 20. Epub 2017 May 20.
    Center for Infectious Diseases Research, Faculty of Medicine, American University of Beirut, P.O. Box 11-0236, Riad El Solh, 1107-2020, Beirut, Lebanon; Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, American University of Beirut Medical Center, P.O. Box 11-0236, Riad El Solh, 1107-2020 Beirut, Lebanon; Department of Biochemistry and Molecular Genetics, Faculty of Medicine, American University of Beirut, P.O. Box 11-0236, Riad El Solh, 1107-2020 Beirut, Lebanon. Electronic address:
    Purpose: The aim of this study was to determine the incidence and genetic diversity of astrovirus (AstV) detected in children hospitalized for gastroenteritis (GE).

    Methods: A multi-center, hospital-based surveillance study was conducted across Lebanon to investigate the incidence of AstV among diarrheal hospitalizations. Viral RNA was extracted from stool samples collected between 2011 and 2013 from children, below the age of 5years, hospitalized for GE at six medical centers across Lebanon. Read More

    Transient Fanconi syndrome in two preterm infants with hydronephrosis and urinary tract infection.
    CEN Case Rep 2017 May 16;6(1):88-90. Epub 2017 Feb 16.
    Department of Pediatrics, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
    Type IV renal tubular acidosis is known to occur in obstructive uropathy with urinary tract infection. Fanconi syndrome, however, has not been described in these settings. We report two preterm infants who developed Fanconi syndrome associated with hydronephrosis and urinary tract infection. Read More

    Systemic Pseudohypoaldosteronism Type I: A Case Report and Review of the Literature.
    Case Rep Pediatr 2017 18;2017:7939854. Epub 2017 Apr 18.
    Division of Pediatric Endocrinology, Rhode Island Hospital and The Warren Alpert Medical School of Brown University, Providence, RI, USA.
    Systemic pseudohypoaldosteronism (PHA) type I is a rare genetic disorder resulting from mutations in the subunits of the epithelial sodium channel that manifests as severe salt wasting, hyperkalemia, and metabolic acidosis in infancy. In this article we report a patient with systemic PHA type I presenting with severe dehydration due to salt wasting at 6 days of life. She was found to have a known mutation in the SCNN1A gene and subsequently required treatment with sodium supplementation. Read More

    Severe Hypernatremic Dehydration and Lower Limb Gangrene in an Infant Exposed to Lamotrigine, Aripiprazole, and Sertraline in Breast Milk.
    Breastfeed Med 2017 May 8. Epub 2017 May 8.
    2 Department of Pediatrics, CHU Sainte-Justine, University of Montreal , Montreal, Canada .
    Background: Hypernatremic dehydration is well described in exclusively breastfed neonates, although life-threatening complications are rarely reported.

    Materials And Methods: The present article describes a case of severe hypernatremic dehydration in a previously healthy term neonate. Other published cases of severe complications of hypernatremic dehydration are discussed. Read More

    Biallelic mutations in CYP24A1 or SLC34A1 as a cause of infantile idiopathic hypercalcemia (IIH) with vitamin D hypersensitivity: molecular study of 11 historical IIH cases.
    J Appl Genet 2017 May 3. Epub 2017 May 3.
    Department of Nephrology, The Children's Memorial Health Institute, Warsaw, Poland.
    Idiopathic infantile hypercalcemia (IIH) is a mineral metabolism disorder characterized by severe hypercalcemia, failure to thrive, vomiting, dehydration, and nephrocalcinosis. The periodical increase in incidence of IIH, which occurred in the twentieth century in the United Kingdom, Poland, and West Germany, turned out to be a side effect of rickets over-prophylaxis. It was recently discovered that the condition is linked to two genes, CYP24A1 and SLC34A1. Read More

    Clinical Impact of Rapid Intravenous Rehydration With Dextrose Serum in Children With Acute Gastroenteritis.
    Pediatr Emerg Care 2017 May 1. Epub 2017 May 1.
    From the *Department of Pediatrics, Hospital General Universitario Gregorio Marañón; †Department of Emergency Pediatrics, Hospital Universitario Niño Jesús; and ‡Department of Emergency Pediatrics Hospital General Universitario Gregorio Marañón, Madrid, Spain.
    Objectives: We designed a study to compare rapid intravenous rehydration based on 0.9% normal saline (NS) or on NS + glucose 2.5% serum (SGS 2. Read More

    Determinants of severe dehydration from diarrheal disease at hospital presentation: Evidence from 22 years of admissions in Bangladesh.
    PLoS Negl Trop Dis 2017 Apr 27;11(4):e0005512. Epub 2017 Apr 27.
    Emerging Pathogens Institute and Child Health Research Institute, Department of Pediatrics, College of Medicine, University of Florida, Gainesville, FL, United States of America.
    Background: To take advantage of emerging opportunities to reduce morbidity and mortality from diarrheal disease, we need to better understand the determinants of life-threatening severe dehydration (SD) in resource-poor settings.

    Methodology/findings: We analyzed records of patients admitted with acute diarrheal disease over twenty-two years at the International Centre for Diarrhoeal Disease Research, Bangladesh (1993-2014). Patients presenting with and without SD were compared by multivariable logistic regression models, which included socio-demographic factors and pathogens isolated. Read More

    Randomized Double-Blind Trial of Ringer's Lactate versus Normal Saline in Pediatric Acute Severe Diarrheal Dehydration.
    J Pediatr Gastroenterol Nutr 2017 Apr 18. Epub 2017 Apr 18.
    *Junior Resident, Division of Pediatric Critical Care, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India †Assistant Professor, Division of Pediatric Critical Care, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India ‡Professor, Division of Pediatric Critical Care, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.
    Objective: To compare the effectiveness of Ringer Lactate (RL) versus normal saline (NS) in the correction of pediatric acute severe diarrheal dehydration, as measured by improvement in clinical status and pH (≥7.35).

    Methods: 68 children aged 1-month to 12-year with acute severe diarrheal dehydration (World Health Organization (WHO classification) were randomized into RL (n = 34) and NS-group (n = 34) and received 100 ml/kg of the assigned intravenous fluid according to WHO PLAN-C for the management of diarrheal dehydration. Read More

    Recruitment, Methods, and Descriptive Results of a Physiologic Assessment of Latino Farmworkers: The California Heat Illness Prevention Study.
    J Occup Environ Med 2017 Apr 14. Epub 2017 Apr 14.
    Department of Public Health Sciences (Dr Mitchell, Ms Armitage, Ms Vega-Arroyo, Dr Bennett, Dr Schenker); Center for Health and the Environment (Mr Castro); Betty Irene Moore School of Nursing, and Samuel Merritt School of Nursing (Dr Moyce); Department of Pediatrics and Center for Healthcare Policy and Research (Dr Tancredi), School of Medicine, Sacramento; Department of Surgical and Radiological Sciences, School of Veterinary Medicine (Dr Jones), University of California, Davis, California; Health and Environmental International Trust, Mapua, Nelson, New Zealand, and University College London, London, United Kingdom (Dr Kjellstrom).
    Objective: The California heat illness prevention study (CHIPS) devised methodology and collected physiological data to assess heat related illness (HRI) risk in Latino farmworkers.

    Methods: Bilingual researchers monitored HRI across a workshift, recording core temperature, work rate (metabolic equivalents [METs]), and heart rate at minute intervals. Hydration status was assessed by changes in weight and blood osmolality. Read More

    Do mothers with high sodium levels in their breast milk have high depression and anxiety scores?
    J Int Med Res 2017 Apr 29;45(2):843-848. Epub 2017 Mar 29.
    5 MD, Department of Paediatrics, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey.
    Objective This study aimed to assess the possible association of high breast milk sodium levels with postpartum depression and anxiety. Methods A total of 150 mothers and their healthy, exclusively breastfed newborns aged 8 to 15 days were recruited. Mothers were asked to complete scales for evaluation of postnatal depression and anxiety following an interview for consent and sociodemographic data collection. Read More

    Assessing process of paediatric care in a resource-limited setting: a cross-sectional audit of district hospitals in Rwanda.
    Paediatr Int Child Health 2017 Mar 27:1-9. Epub 2017 Mar 27.
    b Faculty of Pharmaceutical Sciences, Collaboration for Outcomes Research and Evaluation , University of British Columbia , Vancouver , Canada.
    Background: Routine assessment of quality of care helps identify deficiencies which need to be improved. While gaps in the emergency care of children have been documented across sub-Saharan Africa, data from Rwanda are lacking.

    Objective: To assess the care of sick infants and children admitted to Rwandan district hospitals and the extent to which it follows currently recommended clinical practice guidelines in Rwanda. Read More

    Poor phenotype-genotype association in a large series of patients with Type III Bartter syndrome.
    PLoS One 2017 13;12(3):e0173581. Epub 2017 Mar 13.
    Pediatric Nephrology, Vall d'Hebron University Hospital, Universitat Autonoma, Barcelona, Spain.
    Introduction: Type III Bartter syndrome (BS) is an autosomal recessive renal tubule disorder caused by loss-of-function mutations in the CLCNKB gene, which encodes the chloride channel protein ClC-Kb. In this study, we carried out a complete clinical and genetic characterization in a cohort of 30 patients, one of the largest series described. By comparing with other published populations, and considering that 80% of our patients presented the p. Read More



    Pacing as a Treatment for Reflex-Mediated (Vasovagal, Situational, or Carotid Sinus Hypersensitivity) Syncope: A Systematic Review for the 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients with Syncope: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.
    Heart Rhythm 2017 Mar 9. Epub 2017 Mar 9.
    Objective: To determine, using systematic review of the biomedical literature, whether pacing reduces risk of recurrent syncope and relevant clinical outcomes among adult patients with reflex-mediated syncope.

    Methods: MEDLINE (through PubMed), EMBASE, and the Cochrane Central Register of Controlled Trials (through October 7, 2015) were searched for randomized trials and observational studies examining pacing and syncope, and the bibliographies of known systematic reviews were also examined. Studies were rejected for poor-quality study methods and for the lack of the population, intervention, comparator, or outcome(s) of interest. Read More

    The Use of a Triage-Based Protocol for Oral Rehydration in a Pediatric Emergency Department.
    Pediatr Emerg Care 2017 Mar 9. Epub 2017 Mar 9.
    From the *Division of Pediatric Emergency Medicine, Department of Pediatrics, University of Minnesota Medical School, and †University of Minnesota Masonic Children's Hospital, Minneapolis, MN; ‡Biostatistics and Data Management Core, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI; §Department of Mathematics and Statistics, Auburn University, Auburn, AL; and ∥Department of Pediatric Emergency Medicine, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN.
    Background: Guidelines recommend oral rehydration therapy (ORT) and avoidance of laboratory tests and intravenous fluids for mild to moderate dehydration in children with gastroenteritis; oral ondansetron has been shown to be an effective adjunct.

    Objectives: The aim of this study was to determine if a triage-based, nurse-initiated protocol for early provision of ondansetron and ORT could safely improve the care of pediatric emergency department (ED) patients with symptoms of gastroenteritis.

    Methods: This study evaluated a protocol prompting triage nurses to assess dehydration in gastroenteritis patients and initiate ondansetron and ORT if indicated. Read More

    Pediatric emergency care capacity in a low-resource setting: An assessment of district hospitals in Rwanda.
    PLoS One 2017 3;12(3):e0173233. Epub 2017 Mar 3.
    Collaboration for Outcomes Research and Evaluation, Faculty of Pharmaceutical Sciences, University of British Columbia, Vancouver, British Columbia, Canada.
    Background: Health system strengthening is crucial to improving infant and child health outcomes in low-resource countries. While the knowledge related to improving newborn and child survival has advanced remarkably over the past few decades, many healthcare systems in such settings remain unable to effectively deliver pediatric advance life support management. With the introduction of the Emergency Triage, Assessment and Treatment plus Admission care (ETAT+)-a locally adapted pediatric advanced life support management program-in Rwandan district hospitals, we undertook this study to assess the extent to which these hospitals are prepared to provide this pediatric advanced life support management. Read More

    Clinical Manifestation and Molecular Analysis of Three Korean Patients with the Renal Form of Pseudohypoaldosteronism Type 1.
    Ann Clin Lab Sci 2017 Jan;47(1):83-87
    Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea
    Pseudohypoaldosteronism (PHA) type 1 is a rare, heterogeneous disease characterized by hyponatremia and hyperkalemia due to mineralocorticoid resistance. The clinical features of PHA are usually failure to thrive, vomiting, and dehydration in the neonatal period. Heterozygous mutations in the Nuclear receptor subfamily 3, group C, member 2 (NR3C2) gene result in the dominant renal form of PHA type 1. Read More

    How to Predict Oral Rehydration Failure in Children With Gastroenteritis.
    J Pediatr Gastroenterol Nutr 2017 Feb 28. Epub 2017 Feb 28.
    *Department of Paediatrics, Erasmus MC - Sophia Children's Hospital, Rotterdam, The Netherlands †Department of Public Health, Erasmus MC, Rotterdam, The Netherlands.
    Objectives: Oral rehydration is the standard in most current guidelines for young children with acute gastroenteritis (AGE). Failure of oral rehydration can complicate the disease course, leading to morbidity due to severe dehydration. We aimed to identify prognostic factors of oral rehydration failure in children with AGE. Read More

    Diminished Protein-Bound ω-Hydroxylated Ceramides In The Skin Of Ichthyosis Patients With 12r-Lox Or Elox-3 Deficiency.
    Br J Dermatol 2017 Feb 24. Epub 2017 Feb 24.
    Department of Pediatrics, University of Erlangen-Nürnberg, D-91054, Erlangen, Germany.
    Autosomal recessive congenital ichthyoses (ARCI) are a group of hereditary skin disorders. The disease phenotype is associated with an impaired epidermal barrier leading to increased transepidermal water loss, temperature instability and hypernatremic dehydration in infancy. ARCI are known to be caused by mutations in at least nine different genes which encode proteins involved in the formation of the epidermal barrier. Read More

    Food Protein-Induced Enterocolitis Syndrome.
    J Investig Allergol Clin Immunol 2017;27(1):1-18
    Unidade de Alergia e Imunologia do Instituto da Criança, HC Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
    Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-, cell-mediated food allergy of unknown prevalence and pathophysiology. Onset is typically during the first year of life; seafood-induced FPIES may start in adulthood. Acute FPIES manifests within 1-4 hours after ingestion with repetitive emesis, pallor, and lethargy progressing to dehydration and hypovolemic shock in 15% of cases. Read More

    Hypernatremia in the Neonate: Neonatal Hypernatremia and Hypernatremic Dehydration in Neonates Receiving Exclusive Breastfeeding.
    Indian J Crit Care Med 2017 Jan;21(1):30-33
    Department of Paediatrics, Lata Mangeshkar Hospital, Hingna, Nagpur, Maharashtra, India.
    Aims And Objectives: Evaluation of neonatal hypernatremia and hypernatremic dehydration in neonates receiving exclusive breastfeeding.

    Introduction: Neonatal hypernatremia is a serious condition in the newborn period. We present infants with hypernatremic dehydration due to breast milk (BM) hypernatremia. Read More

    Risk factors for hospitalization of children with congenital adrenal hyperplasia.
    Clin Endocrinol (Oxf) 2017 May 9;86(5):669-673. Epub 2017 Mar 9.
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, USA.
    Background And Objectives: Patients with congenital adrenal hyperplasia (CAH) owing to 21-hydroxylase deficiency are prone to potentially life-threatening adrenal crises. We tried to identify risk factors for postdiagnosis hospitalization for children with the salt-wasting form of CAH.

    Methods: We reviewed medical records of all children who presented to Children's Medical Center, Dallas, from 1999 to 2013 with CAH (ICD-9 code 255·2). Read More

    Renal Morphology, Clinical Findings, and Progression Rate in Mesoamerican Nephropathy.
    Am J Kidney Dis 2017 May 23;69(5):626-636. Epub 2017 Jan 23.
    Division of Renal Medicine, Department of Clinical Sciences, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden; Clinical Pathology and Cytology, Karolinska University Hospital, Stockholm, Sweden. Electronic address:
    Background: Mesoamerican nephropathy (MeN) is a chronic kidney disease affecting rural inhabitants in Central America. We have previously described the renal morphology in 8 patients from El Salvador. To confirm the renal pathology, we have studied kidney biopsies from patients with MeN in Nicaragua. Read More

    Hemoglobin C trait accentuates erythrocyte dehydration in hereditary xerocytosis.
    Pediatr Blood Cancer 2017 Jan 25. Epub 2017 Jan 25.
    Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut.
    A 17-year-old male presented with acute hemolysis with stomatocytosis, elevated mean corpuscular hemoglobin concentration (MCHC), and osmotic gradient ektacytometry consistent with marked erythrocyte dehydration. Erythrocytes from both parents also demonstrated evidence of dehydration with elevated MCHC and abnormal ektacytometry, but neither to the degree of the patient. Genetic studies revealed the patient had hereditary xerocytosis (HX) due to a novel PIEZO1 mutation inherited from his mother and hemoglobin C (HbC) trait inherited from his father. Read More

    Airway mucus, inflammation and remodeling: emerging links in the pathogenesis of chronic lung diseases.
    Cell Tissue Res 2017 Mar 20;367(3):537-550. Epub 2017 Jan 20.
    Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), University of Heidelberg, Im Neuenheimer Feld 156, 69120 Heidelberg, Germany.
    Airway mucus obstruction is a hallmark of many chronic lung diseases including rare genetic disorders such as cystic fibrosis (CF) and primary ciliary dyskinesia, as well as common lung diseases such as asthma and chronic obstructive pulmonary disease (COPD), which have emerged as a leading cause of morbidity and mortality worldwide. However, the role of excess airway mucus in the in vivo pathogenesis of these diseases remains poorly understood. The generation of mice with airway-specific overexpression of epithelial Na(+) channels (ENaC), exhibiting airway surface dehydration (mucus hyperconcentration), impaired mucociliary clearance (MCC) and mucus plugging, led to a model of muco-obstructive lung disease that shares key features of CF and COPD. Read More

    YouTube: are parent-uploaded videos of their unwell children a useful source of medical information for other parents?
    Arch Dis Child 2017 Jan 19. Epub 2017 Jan 19.
    Sophia Children's Hospital, Rotterdam, The Netherlands.
    Aims: YouTube is a vast source of freely accessible user-uploaded medical information. To our knowledge no study has analysed the quality of parent-uploaded videos which depict illness in their children. We aimed to investigate the quality and quantity of videos representing two common conditions, croup and dehydration. Read More

    Evaluation of a Smartphone Decision-Support Tool for Diarrheal Disease Management in a Resource-Limited Setting.
    PLoS Negl Trop Dis 2017 Jan 19;11(1):e0005290. Epub 2017 Jan 19.
    Department of Pediatrics, Stanford University School of Medicine, Stanford, California, United States of America.
    The emergence of mobile technology offers new opportunities to improve clinical guideline adherence in resource-limited settings. We conducted a clinical pilot study in rural Bangladesh to evaluate the impact of a smartphone adaptation of the World Health Organization (WHO) diarrheal disease management guidelines, including a modality for age-based weight estimation. Software development was guided by end-user input and evaluated in a resource-limited district and sub-district hospital during the fall 2015 cholera season; both hospitals lacked scales which necessitated weight estimation. Read More

    Secondary haemophagocytic lymphohistiocytosis triggered by postnatally acquired cytomegalovirus infection in a late preterm infant.
    Infection 2017 Jan 16. Epub 2017 Jan 16.
    Children's Hospital, University Hospital Wuerzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Germany.
    Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with impairment of cytotoxic T-cells and natural killer cells. Causes in infants are mostly hereditary immune defects as well as various infectious triggering factors, amongst these cytomegalovirus (CMV). Vertical CMV transmission may occur in utero, during birth, and by breast feeding. Read More

    Characteristics and Outcomes of Pediatric Patients With Ebola Virus Disease Admitted to Treatment Units in Liberia and Sierra Leone: A Retrospective Cohort Study.
    Clin Infect Dis 2017 Feb 25;64(3):243-249. Epub 2016 Oct 25.
    Warren Alpert Medical School, and
    Background: The clinical and virologic characteristics of Ebola virus disease (EVD) in children have not been thoroughly documented.

    Methods: Consecutive children aged <18 years with real-time polymerase chain reaction (RT-PCR)-confirmed EVD were enrolled retrospectively in 5 Ebola treatment units in Liberia and Sierra Leone in 2014/2015. Data collection and medical management were based on standardized International Medical Corps protocols. Read More

    Improved Management of Harlequin Ichthyosis With Advances in Neonatal Intensive Care.
    Pediatrics 2017 Jan 20;139(1). Epub 2016 Dec 20.
    Department of Dermatology, State University of New York Downstate Medical Center, Brooklyn, New York;
    Harlequin ichthyosis (HI) is the most severe phenotype of the autosomal recessive congenital ichthyoses. HI is caused by mutations in the lipid transporter adenosine triphosphate binding cassette A 12 (ABCA12). Neonates are born with a distinct clinical appearance, encased in a dense, platelike keratotic scale separated by deep erythematous fissures. Read More

    Hypernatremic Dehydration in Breastfed Term Infants: Retrospective Evaluation of 159 Cases.
    Breastfeed Med 2017 Jan/Feb;12:5-11. Epub 2016 Dec 19.
    1 Department of Pediatrics, Cumhuriyet University Faculty of Medicine , Sivas, Turkey .
    Objectives: The aim of this study was to reveal the frequency, presenting complaints, risk factors, complications, and ways for prevention of hypernatremic dehydration (HD) among term breastfed infants.

    Methods: The files of 159 breastfed term infants hospitalized because of HD between the years 2009 and 2014 were examined retrospectively in the Neonatal Intensive Care Unit of Sivas State Hospital, Turkey. The patients were classified according to serum sodium (Na) levels, group 1 (Na: 146-149 mEq/L, n = 68) and group 2 (Na ≥150 mEq/L, n = 91). Read More

    Knowledge insufficient: the management of haemoglobin SC disease.
    Br J Haematol 2017 Feb 16;176(4):515-526. Epub 2016 Dec 16.
    Cancer and Blood Diseases Institute, Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
    Although haemoglobin SC (HbSC) accounts for 30% of sickle cell disease (SCD) in the United States and United Kingdom, evidence-based guidelines for genotype specific management are lacking. The unique pathology of HbSC disease is complex, characterized by erythrocyte dehydration, intracellular sickling and increased blood viscosity. The evaluation and treatment of patients with HbSC is largely inferred from studies of SCD consisting mostly of haemoglobin SS (HbSS) patients. Read More

    Polymer-based oral rehydration solution for treating acute watery diarrhoea.
    Cochrane Database Syst Rev 2016 12 13;12:CD006519. Epub 2016 Dec 13.
    Department of Pediatrics, University of the Philippines Manila College of Medicine-Philippine General Hospital, Taft Avenue, Manila, National Capital Region, Philippines, 1000.
    Background: Acute diarrhoea is one of the main causes of morbidity and mortality among children in low-income countries. Glucose-based oral rehydration solution (ORS) helps replace fluid and prevent further dehydration from acute diarrhoea. Since 2004, the World Health Organization (WHO) has recommended the osmolarity of less than 270 mOsm/L (ORS ≤ 270) versus greater than 310 mOsm/L formulation (ORS ≥ 310). Read More

    Implementation of clinical decision support in young children with acute gastroenteritis: a randomized controlled trial at the emergency department.
    Eur J Pediatr 2017 Feb 8;176(2):173-181. Epub 2016 Dec 8.
    Department of Pediatrics, Erasmus MC - Sophia children's hospital, Wytemaweg 80, Rotterdam, CN, 3015, The Netherlands.
    Acute gastroenteritis (AGE) is one of the most frequent reasons for young children to visit emergency departments (EDs). We aimed to evaluate (1) feasibility of a nurse-guided clinical decision support system for rehydration treatment in children with AGE and (2) the impact on diagnostics, treatment, and costs compared with usual care by attending physician. A randomized controlled trial was performed in 222 children, aged 1 month to 5 years at the ED of the Erasmus MC-Sophia Children's hospital in The Netherlands ( 2010-2012). Read More

    Pediatric surgical readmissions: Are they truly preventable?
    J Pediatr Surg 2017 Jan 28;52(1):161-165. Epub 2016 Oct 28.
    University of California, Davis Health System, Sacramento, CA, USA.
    Background/purpose: Reimbursement penalties for excess hospital readmissions have begun for the pediatric population. Therefore, research determining incidence and predictors is critical.

    Methods: A retrospective review of University HealthSystem Consortium database (N=258 hospitals; 2,723,621 patients) for pediatric patients (age 0-17years) hospitalized from 9/2011 to 3/2015 was performed. Read More

    Associations Between Hydration Status, Intravenous Fluid Administration, and Outcomes of Patients Infected With Shiga Toxin-Producing Escherichia coli: A Systematic Review and Meta-analysis.
    JAMA Pediatr 2017 Jan;171(1):68-76
    Alberta Children's Hospital Research Institute, Section of Gastroenterology, Alberta Children's Hospital, University of Calgary, Calgary, Alberta, Canada7Alberta Children's Hospital Research Institute, Section of Pediatric Emergency Medicine, Alberta Children's Hospital, University of Calgary, Calgary, Alberta, Canada.
    Importance: The associations between hydration status, intravenous fluid administration, and outcomes of patients infected with Shiga toxin-producing Escherichia coli (STEC) remain unclear.

    Objective: To determine the relationship between hydration status, the development and severity of hemolytic uremic syndrome (HUS), and adverse outcomes in STEC-infected individuals.

    Data Sources: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials via the OvidSP platform, PubMed via the National Library of Medicine, CINAHL Plus with full text, Scopus, Web of Science, ClinicalTrials. Read More

    Human milk oligosaccharide effects on intestinal function and inflammation after preterm birth in pigs.
    J Nutr Biochem 2017 Feb 4;40:141-154. Epub 2016 Nov 4.
    Comparative Pediatrics and Nutrition, Faculty of Health and Medical Sciences, University of Copenhagen, Dyrlægevej 68, 1870 Frederiksberg C, Denmark; Department of Nutrition, Exercise, and Sports, Faculty of Science, University of Copenhagen, Rolighedsvej 30, 1958 Frederiksberg C, Denmark. Electronic address:
    Human milk oligosaccharides (HMOs) may mediate prebiotic and anti-inflammatory effects in newborns. This is particularly important for preterm infants who are highly susceptible to intestinal dysfunction and necrotizing enterocolitis (NEC). We hypothesized that HMO supplementation of infant formula (IF) improves intestinal function, bacterial colonization and NEC resistance immediately after preterm birth, as tested in a preterm pig model. Read More

    Oral Ondansetron versus Domperidone for Acute Gastroenteritis in Pediatric Emergency Departments: Multicenter Double Blind Randomized Controlled Trial.
    PLoS One 2016 23;11(11):e0165441. Epub 2016 Nov 23.
    Clinical Epidemiology and Public Health Research Unit, Institute for Maternal and Child Health - IRCCS "Burlo Garofolo", Trieste, Italy.
    The use of antiemetics for vomiting in acute gastroenteritis in children is still a matter of debate. We conducted a double-blind randomized trial to evaluate whether a single oral dose of ondansetron vs domperidone or placebo improves outcomes in children with gastroenteritis. After failure of initial oral rehydration administration, children aged 1-6 years admitted for gastroenteritis to the pediatric emergency departments of 15 hospitals in Italy were randomized to receive one oral dose of ondansetron (0. Read More

    Validation of a Simple Stool Diary used by Caregivers to Document Diarrhea Among Young Children in a Low-Income Country.
    J Pediatr Gastroenterol Nutr 2016 Nov 14. Epub 2016 Nov 14.
    *Department of Nutrition, Exercise and Sports, University of Copenhagen, Denmark†Mwanamugimu Nutrition Unit, Department of Paediatrics and Child Health, Mulago National Referral Hospital, Kampala, Uganda‡Faculty of Health and Medical Sciences, University of Copenhagen, Denmark§Pharma Evidence, Farum, Denmark||Nopia Research Group, Department of Urology, Ghent University Hospital, Ghent, Belgium.
    Objectives: Development and validation of a simple stool diary for caretakers collecting data on stool frequency and consistency among young children in a low-income country.

    Methods: Focus group studies evaluated how diarrhea was understood by caregivers (content validity). The sensitivity, reliability, and correlations between dehydration and diary scores (construct validity) were tested in a clinical trial. Read More

    Hyponatremia in children with tuberculous meningitis: A hospital-based cohort study.
    J Pediatr Neurosci 2016 Jul-Sep;11(3):182-187
    Department of Pediatrics, ESI-PGIMSR, MGM Hospital, Mumbai, Maharashtra, India.
    Background: Hyponatremia has long been recognized as a potentially serious metabolic consequence of tuberculous meningitis (TBM) occurring in 35-65% of children with the disease. The syndrome of inappropriate antidiuretic hormone (SIADH) secretion has for long been believed to be responsible for the majority of cases of hyponatremia in TBM. Cerebral salt wasting syndrome (CSWS) is being increasingly reported as a cause of hyponatremia in some of these children. Read More

    Endemic bladder calculi in children.
    Pediatr Nephrol 2016 Nov 15. Epub 2016 Nov 15.
    Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan.
    Urinary calculi are being recognized more frequently in children and the urinary bladder is the most common site for stone formation in the lower urinary tract. Bladder calculi are grouped into three basic categories: primary idiopathic/endemic, secondary, and migrant. The incidence of vesical calculi has declined significantly in the last 70 years in developed nations owing to improvements in nutrition and socioeconomic conditions, but it is still high in developing nations. Read More

    Prevalence, aetiology, treatment and outcomes of shock in children admitted to Kenyan hospitals.
    BMC Med 2016 Nov 16;14(1):184. Epub 2016 Nov 16.
    Kenya Medical Research Institute/Wellcome Trust Research Programme, P.O. Box 43640-00100, Nairobi, Kenya.
    Background: Shock may complicate several acute childhood illnesses in hospitals within low-income countries and has a high case fatality. Hypovolemic shock secondary to diarrhoea/dehydration and septic shock are thought to be common, but there are few reliable data on prevalence or treatment that differ for the two major forms of shock. Examining prevalence and treatment practices has become important since reports suggest high risks from liberal use of fluid boluses in African children. Read More

    Options for improving effectiveness of rotavirus vaccines in developing countries.
    Hum Vaccin Immunother 2017 Apr 11;13(4):921-927. Epub 2016 Nov 11.
    e Department of Paediatrics & The Ritchie Centre, Hudson Institute of Medical Research , Monash University, Melbourne, Australia; Infection and Immunity, Monash Children's Hospital, Monash Health, Melbourne, Australia; SAEFVIC, Murdoch Childrens Research Institute , Melbourne , Australia.
    Rotavirus gastroenteritis is a leading global cause of mortality and morbidity in young children due to diarrhea and dehydration. Over 85% of deaths occur in developing countries. In industrialised countries, 2 live oral rotavirus vaccines licensed in 2006 quickly demonstrated high effectiveness, dramatically reducing severe rotavirus gastroenteritis admissions in many settings by more than 90%. Read More

    Hypercalcemia in Patients with Williams-Beuren Syndrome.
    J Pediatr 2016 Nov 26;178:254-260.e4. Epub 2016 Aug 26.
    Department of Pediatrics, Washington University School of Medicine, St. Louis, MO; National Institutes of Health, National Heart, Lung, and Blood Institute, Bethesda, MD. Electronic address:
    Objective: To evaluate the timing, trajectory, and implications of hypercalcemia in Williams-Beuren syndrome (WBS) through a multicenter retrospective study.

    Study Design: Data on plasma calcium levels from 232 subjects with WBS aged 0-67.1 years were compared with that in controls and also with available normative data. Read More

    Methylmalonyl-coA epimerase deficiency: A new case, with an acute metabolic presentation and an intronic splicing mutation in the MCEE gene.
    Mol Genet Metab Rep 2016 Dec 24;9:19-24. Epub 2016 Sep 24.
    Medical Genetics Service, Department of Pediatrics, Centre hospitalier universitaire de Sherbrooke (CHUS) and University of Sherbrooke, Sherbrooke, Quebec, Canada.
    Methylmalonyl-coA epimerase (MCE) follows propionyl-coA carboxylase and precedes methylmalonyl-coA mutase in the pathway converting propionyl-coA to succinyl-coA. MCE deficiency has previously been described in six patients, one presenting with metabolic acidosis, the others with nonspecific neurological symptoms or asymptomatic. The clinical significance and biochemical characteristics of this rare condition have been incompletely defined. Read More

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