Long-term subcutaneous protein C replacement in neonatal severe protein C deficiency.

Abstract:

:We describe here the case of a boy who presented 2 days after birth with purpura fulminans on his feet and scalp. Laboratory investigations revealed signs of disseminated intravascular coagulation. An underlying coagulation disorder was suspected, and therapy with recombinant tissue plasminogen activator, fresh-frozen plasma, and unfractionated heparin was started. On the basis of plasma protein C activity and antigen levels of 0.02 and 0.03 IU/mL, respectively, after administration of fresh-frozen plasma, a diagnosis of severe protein C deficiency was established, and therapy with intravenous protein C concentrate (Ceprotin [Baxter, Deerfield, IL]) was started. Because of difficulties with venous access, we switched to subcutaneous administration after 6 weeks. The precise dosing schedule for subcutaneously administered protein C concentrate is unknown. In the literature, a trough level of protein C activity at >0.25 IU/mL is recommended to prevent recurrent thrombosis. During 1 year of follow-up our patient frequently had protein C activity levels at <0.25 IU/mL. Clinically, however, there was no recurrent thrombosis, and we kept the dosage unchanged. This report highlights 2 important points: (1) subcutaneously administered protein C concentrate is effective in treating severe protein C deficiency; and (2) in accordance with previous studies, after the acute phase trough levels of protein C activity at >0.25 IU/mL may not be necessary to prevent recurrent thrombosis. However, further research on the dosing, efficacy, and safety of protein C concentrate for prophylaxis and treatment of severe protein C deficiency is needed.

journal_name

Pediatrics

journal_title

Pediatrics

authors

de Kort EH,Vrancken SL,van Heijst AF,Binkhorst M,Cuppen MP,Brons PP

doi

10.1542/peds.2009-2913

subject

Has Abstract

pub_date

2011-05-01 00:00:00

pages

e1338-42

issue

5

eissn

0031-4005

issn

1098-4275

pii

peds.2009-2913

journal_volume

127

pub_type

杂志文章,评审
  • Mycoplasma pneumoniae and atypical Stevens-Johnson syndrome: a case series.

    abstract::Mycoplasma pneumoniae is a common cause of community-acquired respiratory illness in the adolescent population. Stevens-Johnson syndrome is an extrapulmonary manifestation that has been associated with M. pneumoniae infections. Three adolescent males presented within a 1-month period with M. pneumoniae respiratory ill...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2006-2401

    authors: Ravin KA,Rappaport LD,Zuckerbraun NS,Wadowsky RM,Wald ER,Michaels MM

    更新日期:2007-04-01 00:00:00

  • Prevalence and correlates of high-quality basic pediatric preventive care.

    abstract:BACKGROUND:The list of recommended pediatric preventive services has grown considerably in the past decade, and clinician variability, clinician distribution, and other correlates of provision of these basic preventive services (BPS) are not known. OBJECTIVE:To describe the proportion of high-quality basic pediatric p...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2004-0635

    authors: Zuckerman B,Stevens GD,Inkelas M,Halfon N

    更新日期:2004-12-01 00:00:00

  • Benefits of Delayed Cord Clamping in Red Blood Cell Alloimmunization.

    abstract:BACKGROUND AND OBJECTIVE:Several studies have shown the benefits of delayed cord clamping (DCC) in preterm and in healthy newborns at short and long term. Our objective was to evaluate the potentials benefits and risks of DCC in red cell alloimmunization. METHODS:This was a comparative before/after study of all living...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2015-3236

    authors: Garabedian C,Rakza T,Drumez E,Poleszczuk M,Ghesquiere L,Wibaut B,Depoortere MH,Vaast P,Storme L,Houfflin-Debarge V

    更新日期:2016-03-01 00:00:00

  • Functional gastrointestinal disorders in children: an Italian prospective survey.

    abstract:OBJECTIVES:To assess the prevalence and natural history of functional gastrointestinal disorders (FGIDs) in children using the Rome Criteria. METHODS:Thirteen primary care pediatricians were randomly selected from the Campania region of the Italian National Health Service. Each pediatrician completed a detailed FGID q...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.114.1.73

    authors: Miele E,Simeone D,Marino A,Greco L,Auricchio R,Novek SJ,Staiano A

    更新日期:2004-07-01 00:00:00

  • Is moderate drinking during pregnancy associated with an increased risk for malformations?

    abstract::The relationship between heavy drinking during pregnancy and congenital malformations is well known; however, whether moderate or light drinking is teratogenic is controversial. This prospective birth defects study collected information from 32,870 women about alcohol consumption during the first trimester of pregnanc...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Mills JL,Graubard BI

    更新日期:1987-09-01 00:00:00

  • Outcomes of a universal shared reading intervention by 2 years of age: the Let's Read trial.

    abstract:BACKGROUND:Early shared reading and literacy promotion benefits have stimulated international interest in the development of early-years literacy-promotion programs despite limited evidence of effectiveness at a broader population level. OBJECTIVE:To determine whether a population-based primary care literacy promotion...

    journal_title:Pediatrics

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1542/peds.2009-3043

    authors: Goldfeld S,Napiza N,Quach J,Reilly S,Ukoumunne OC,Wake M

    更新日期:2011-03-01 00:00:00

  • Changing Neurodevelopment at 8 Years in Children Born Extremely Preterm Since the 1990s.

    abstract:BACKGROUND AND OBJECTIVE:Survival of extremely preterm (EP; <28 weeks' gestation) infants has increased over the last 2 decades. Equivalent reductions in developmental morbidity in early childhood have not been consistently reported. The aim of this study was to determine trends in neurodevelopmental outcomes at 8 year...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2016-4086

    authors: Cheong JLY,Anderson PJ,Burnett AC,Roberts G,Davis N,Hickey L,Carse E,Doyle LW,Victorian Infant Collaborative Study Group.

    更新日期:2017-06-01 00:00:00

  • Absorption of protein and protein fragments in the developing intestine: role in immunologic/allergic reactions.

    abstract::An important adaptation of the gastrointestinal tract to the extrauterine environment is its development of a mucosal barrier against the penetration of proteins and protein fragments. To combat the potential danger of invasion across the mucosal barrier the newborn infant must develop within the lumen and on the lumi...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Walker WA

    更新日期:1985-01-01 00:00:00

  • Early Postnatal Discharge for Infants: A Meta-analysis.

    abstract:CONTEXT:Postnatal length of hospital stay has reduced internationally but evidence-based policies to support earlier discharge are lacking. OBJECTIVE:To determine the effects of early postnatal discharge on infant outcomes. DATA SOURCES:CENTRAL (Cochrane Central Register of Controlled Trials), Medline, Embase, Cumula...

    journal_title:Pediatrics

    pub_type: 杂志文章,meta分析,评审

    doi:10.1542/peds.2019-3365

    authors: Jones E,Taylor B,MacArthur C,Bradshaw S,Hope L,Cummins C

    更新日期:2020-09-01 00:00:00

  • Physical education class injuries treated in emergency departments in the US in 1997-2007.

    abstract:OBJECTIVE:The goal was to describe the epidemiological features of physical education (PE)-related injuries treated in US emergency departments. METHODS:A retrospective analysis was conducted with data for children and adolescents (5-18 years of age) from the National Electronic Injury Surveillance Study of the US Con...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2008-3843

    authors: Nelson NG,Alhajj M,Yard E,Comstock D,McKenzie LB

    更新日期:2009-09-01 00:00:00

  • Promoter polymorphism of the CD14 endotoxin receptor gene is associated with biliary atresia and idiopathic neonatal cholestasis.

    abstract:OBJECTIVE:To investigate whether single-nucleotide polymorphisms in the promoter regions of endotoxin-responsive genes CD14 and tumor necrosis factor-alpha (TNF-alpha) are associated with biliary atresia (BA) and idiopathic neonatal cholestasis (INC). METHODS:We obtained genomic DNA from 90 patients with established d...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2004-1900

    authors: Shih HH,Lin TM,Chuang JH,Eng HL,Juo SH,Huang FC,Chen CL,Chen HL

    更新日期:2005-08-01 00:00:00

  • Incidence, prognosis, and risk factors for fatigue and chronic fatigue syndrome in adolescents: a prospective community study.

    abstract:OBJECTIVE:The objective of this study was to describe the incidence, prevalence, risk factors, and prognosis of fatigue, chronic fatigue, and chronic fatigue syndrome in 11- to 15-year-olds. METHODS:A random general population sample (n = 842) of British adolescents and their parents were assessed at baseline and 4 to...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2006-2231

    authors: Rimes KA,Goodman R,Hotopf M,Wessely S,Meltzer H,Chalder T

    更新日期:2007-03-01 00:00:00

  • Prolonged mechanical ventilation and intracranial hemorrhage: impact on developmental progress through 18 months in infants weighing 1,200 grams or less at birth.

    abstract::In this prospective, longitudinal study, the relative impact of intracranial hemorrhage and prolonged mechanical ventilation on developmental progress during the first 18 months of life of infants weighing 1,200 g or less at birth was examined. A total of 159 surviving infants were divided into two groups: infants wit...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Bozynski ME,Nelson MN,Matalon TA,O'Donnell KJ,Naughton PM,Vasan U,Meier WA,Ploughman L

    更新日期:1987-05-01 00:00:00

  • Coagulase-negative staphylococcal bacteremia among very low birth weight infants: relation to admission illness severity, resource use, and outcome.

    abstract:OBJECTIVE:To examine the impact of admission-day illness severity on nosocomial bacteremia risk after consideration of traditional risk determinants such as birth weight and length of stay. METHODS:The hospital courses for 302 consecutive very low birth weight (less than 1500 g) infants admitted to two neonatal intens...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Gray JE,Richardson DK,McCormick MC,Goldmann DA

    更新日期:1995-02-01 00:00:00

  • A pacifier increases the risk of recurrent acute otitis media in children in day care centers.

    abstract:OBJECTIVE:To follow up a previous retrospective analysis in which we found the use of a pacifier to be a risk factor for recurrent acute otitis media (AOM). METHOD:In the present prospective study, the occurrence of AOM and the use of a pacifier were recorded in 845 children attending day care centers during a 15-mont...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Niemelä M,Uhari M,Möttönen M

    更新日期:1995-11-01 00:00:00

  • Vitamin D₃supplementation and childhood diarrhea: a randomized controlled trial.

    abstract:OBJECTIVE:To investigate the effect of vitamin D3 supplementation on the incidence and risk for first and recurrent diarrheal illnesses among children in Kabul, Afghanistan. METHODS:This double-blind placebo-controlled trial randomized 3046 high-risk 1- to 11-month-old infants to receive 6 quarterly doses of oral vita...

    journal_title:Pediatrics

    pub_type: 杂志文章,随机对照试验

    doi:10.1542/peds.2012-3986

    authors: Aluisio AR,Maroof Z,Chandramohan D,Bruce J,Mughal MZ,Bhutta Z,Walraven G,Masher MI,Ensink JH,Manaseki-Holland S

    更新日期:2013-10-01 00:00:00

  • Daptomycin for Complicated Skin Infections: A Randomized Trial.

    abstract:BACKGROUND:Complicated skin and skin structure infections (cSSSI) are common in children. Due to safety and resistance issues with recommended agents, new treatment options would be advantageous. METHODS:Multicenter, evaluator-blinded clinical trial. Patients 1 to 17 years old with cSSSI caused by Gram-positive pathog...

    journal_title:Pediatrics

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1542/peds.2016-2477

    authors: Bradley J,Glasser C,Patino H,Arnold SR,Arrieta A,Congeni B,Daum RS,Kojaoghlanian T,Yoon M,Anastasiou D,Wolf DJ,Bokesch P

    更新日期:2017-03-01 00:00:00

  • Childhood overweight and maturational timing in the development of adult overweight and fatness: the Newton Girls Study and its follow-up.

    abstract:OBJECTIVE:Although several studies have suggested that early menarche is associated with the development of adult overweight, few have accounted for childhood overweight before menarche. STUDY DESIGN:A 30-year follow-up of the original participants in the Newton Girls Study, a prospective study of development in a coh...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2004-1604

    authors: Must A,Naumova EN,Phillips SM,Blum M,Dawson-Hughes B,Rand WM

    更新日期:2005-09-01 00:00:00

  • The National Children's Study: Recruitment Outcomes Using the Provider-Based Recruitment Approach.

    abstract:OBJECTIVE:In 2009, the National Children's Study (NCS) Vanguard Study tested the feasibility of household-based recruitment and participant enrollment using a birth-rate probability sample. In 2010, the NCS Program Office launched 3 additional recruitment approaches. We tested whether provider-based recruitment could i...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2015-4410E

    authors: Hale DE,Wyatt SB,Buka S,Cherry D,Cislo KK,Dudley DJ,McElfish PA,Norman GS,Reynolds SA,Siega-Riz AM,Wadlinger S,Walker CK,Robbins JM

    更新日期:2016-06-01 00:00:00

  • A prospective multicenter study of cervical spine injury in children.

    abstract:OBJECTIVE:Pediatric victims of blunt trauma have developmental and anatomic characteristics that can make it difficult to assess their risk of cervical spine injury (CSI). Previous reports, all retrospective in nature, have not identified any cases of CSI in either children or adults in the absence of neck pain, neurol...

    journal_title:Pediatrics

    pub_type: 杂志文章,多中心研究

    doi:10.1542/peds.108.2.e20

    authors: Viccellio P,Simon H,Pressman BD,Shah MN,Mower WR,Hoffman JR,NEXUS Group.

    更新日期:2001-08-01 00:00:00

  • Successful Chest Radiograph Reduction by Using Quality Improvement Methodology for Children With Asthma.

    abstract:OBJECTIVES:Implementation of an asthma clinical practice guideline did not achieve desired chest radiograph (CXR) usage goals. We attempt to use quality improvement methodology to decrease the percentage of CXRs obtained for pediatric patients with acute asthma exacerbations from 29.3% to <20% and to evaluate whether d...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2017-4003

    authors: Watnick CS,Arnold DH,Latuska R,O'Connor M,Johnson DP

    更新日期:2018-08-01 00:00:00

  • Ineffectiveness of historical data in predicting measles susceptibility.

    abstract::The Immunization Practices Advisory Committee ( ACIP ) has devised noninvasive, historical criteria for determining individuals who are susceptible to measles. These criteria, which involve proof of vaccination, are incorporated into school entrance regulations and are used to indicate people who require vaccination d...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Scott RM,Butler AB,Schydlower M,Rawlings P

    更新日期:1984-06-01 00:00:00

  • Brief approaches to developmental-behavioral promotion in primary care: updates on methods and technology.

    abstract::Well-child visits are a critical opportunity to promote learning and development, encourage positive parenting practices, help children acquire behavioral self-control, enhance the development and well-being of children and their families, identify problems not amenable to brief in-office counseling, and refer for ser...

    journal_title:Pediatrics

    pub_type: 杂志文章,评审

    doi:10.1542/peds.2013-1859

    authors: Glascoe FP,Trimm F

    更新日期:2014-05-01 00:00:00

  • Trends in Weight-for-Length Among Infants in WIC From 2000 to 2014.

    abstract:OBJECTIVES:To describe the prevalence and secular trends of high weight-for-length among infants (ages, 3-23 months) in the biennial US Department of Agriculture Women, Infants, and Children Program and Participants Characteristic (WIC-PC) Survey from 2000 through 2014 (n = 16 927 120). METHODS:Weight-for-length was c...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2016-2034

    authors: Freedman DS,Sharma AJ,Hamner HC,Pan L,Panzera A,Smith RB,Blanck HM

    更新日期:2017-01-01 00:00:00

  • A Core Outcome Set for Neonatal Opioid Withdrawal Syndrome.

    abstract:BACKGROUND:As rates of neonatal opioid withdrawal are increasing, the need for research to evaluate new treatments is growing. Large heterogeneity exists in health outcomes reported in current literature. Our objective is to develop an evidence-informed and consensus-based core outcome set in neonatal opioid withdrawal...

    journal_title:Pediatrics

    pub_type: 共识发展会议,杂志文章

    doi:10.1542/peds.2020-0018

    authors: Kelly LE,Shan F,MacVicar S,Czaplinksi E,Moulsdale W,Simpson S,Allegaert K,Jansson LM,Offringa M

    更新日期:2020-07-01 00:00:00

  • Improvement of short- and long-term outcomes for very low birth weight infants: Edmonton NIDCAP trial.

    abstract:OBJECTIVE:Our objective was to determine the impact of Newborn Individualized Developmental Care and Assessment Program (NIDCAP)-based care on length of stay of very low birth weight (VLBW) infants. Secondary outcome measures were days of ventilation, incidence of chronic lung disease, and 18-month neurodevelopmental o...

    journal_title:Pediatrics

    pub_type: 杂志文章,随机对照试验

    doi:10.1542/peds.2008-3808

    authors: Peters KL,Rosychuk RJ,Hendson L,Coté JJ,McPherson C,Tyebkhan JM

    更新日期:2009-10-01 00:00:00

  • Emergency Department and Hospital Use Among Adolescents With Justice System Involvement.

    abstract:OBJECTIVES:Adolescents with justice system involvement have high rates of physical and behavioral health disorders and are potentially high users of costly health care services. We examined emergency department (ED) and hospital use among a national sample of adolescents with various levels of justice involvement. MET...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2017-1144

    authors: Winkelman TNA,Genao I,Wildeman C,Wang EA

    更新日期:2017-11-01 00:00:00

  • The adverse effect of negative comments about weight and shape from family and siblings on women at high risk for eating disorders.

    abstract:OBJECTIVE:Our purpose with this work was to examine the relationship between negative comments about weight, shape, and eating and social adjustment, social support, self-esteem, and perceived childhood abuse and neglect. METHODS:A retrospective study was conducted with 455 college women with high weight and shape con...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2005-1806

    authors: Taylor CB,Bryson S,Celio Doyle AA,Luce KH,Cunning D,Abascal LB,Rockwell R,Field AE,Striegel-Moore R,Winzelberg AJ,Wilfley DE

    更新日期:2006-08-01 00:00:00

  • Antecedents of neonatal encephalopathy in the Vermont Oxford Network Encephalopathy Registry.

    abstract:BACKGROUND:Neonatal encephalopathy (NE) is a major predictor of death and long-term neurologic disability, but there are few studies of antecedents of NE. OBJECTIVES:To identify antecedents in a large registry of infants who had NE. METHODS:This was a maternal and infant record review of 4165 singleton neonates, gest...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2012-0714

    authors: Nelson KB,Bingham P,Edwards EM,Horbar JD,Kenny MJ,Inder T,Pfister RH,Raju T,Soll RF

    更新日期:2012-11-01 00:00:00

  • Iodine deficiency, pollutant chemicals, and the thyroid: new information on an old problem.

    abstract::Many women of reproductive age in the United States are marginally iodine deficient, perhaps because the salt in processed foods is not iodized. Iodine deficiency, per se, can interfere with normal brain development in their offspring; in addition, it increases vulnerability to the effects of certain environmental pol...

    journal_title:Pediatrics

    pub_type: 杂志文章,评审

    doi:10.1542/peds.2014-0900

    authors: Council on Environmental Health.,Rogan WJ,Paulson JA,Baum C,Brock-Utne AC,Brumberg HL,Campbell CC,Lanphear BP,Lowry JA,Osterhoudt KC,Sandel MT,Spanier A,Trasande L

    更新日期:2014-06-01 00:00:00