Congenital myotonic dystrophy requiring prolonged endotracheal and noninvasive assisted ventilation: not a uniformly fatal condition.

Abstract:

:In this report we present two infants with congenital myotonic dystrophy (CMD) who were successfully weaned from prolonged ventilatory support using nasal continuous positive airway pressure (N-CPAP). The first infant received 127 days of endotracheal mechanical ventilation as part of 141 days of total ventilatory support, including N-CPAP; the second infant received 27 days of endotracheal mechanical ventilation as part of 84 days of total ventilatory support. Noninvasive N-CPAP facilitated weaning these two infants from ventilatory support, thereby minimizing the morbidity associated with prolonged intubation. The developmental outcomes of our two infants were comparable to infants not requiring prolonged endotracheal mechanical ventilation. We suggest that this noninvasive modality of ventilatory support may be advantageous in the management and beneficial to the outcome of infants with CMD who are respirator-dependent >30 days.

journal_name

Pediatrics

journal_title

Pediatrics

authors

Keller C,Reynolds A,Lee B,Garcia-Prats J

doi

10.1542/peds.101.4.704

subject

Has Abstract

pub_date

1998-04-01 00:00:00

pages

704-6

issue

4 Pt 1

eissn

0031-4005

issn

1098-4275

journal_volume

101

pub_type

杂志文章
  • Recognition of a Kawasaki disease shock syndrome.

    abstract:OBJECTIVE:We sought to define the characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease. METHODS:We collected data prospectively for all patients with Kawasaki disease who were treated at a single institution during a 4-year period. We defined Kawasaki disease sh...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2008-1871

    authors: Kanegaye JT,Wilder MS,Molkara D,Frazer JR,Pancheri J,Tremoulet AH,Watson VE,Best BM,Burns JC

    更新日期:2009-05-01 00:00:00

  • Amniotic fluid microviscosity determined by fluorescence polarization: methodology and relation to gestational age.

    abstract::The fluorescence polarization of 116 amniotic fluid specimens obtained from 22 isoimmunized pregnant women was determined. The degree of fluorescence polarization of amniotic fluid provides an index of microvisocity in lipid aggregates that is dependent on the lecithin-to-sphingomyelin ratio and the degree of saturati...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Stark RI,Blumenfeld TA,George JD,Freda VJ,James LS

    更新日期:1979-02-01 00:00:00

  • Mainstream end-tidal carbon dioxide monitoring in the neonatal intensive care unit.

    abstract:BACKGROUND:Continuous noninvasive monitoring of arterial carbon dioxide (CO2) in neonatal intensive care unit (NICU) patients would help clinicians avoid complications of hypocarbia and hypercarbia. End-tidal CO2 monitoring has not been used in this population to date, but recent technical advances and the introduction...

    journal_title:Pediatrics

    pub_type: 临床试验,杂志文章

    doi:10.1542/peds.101.4.648

    authors: Rozycki HJ,Sysyn GD,Marshall MK,Malloy R,Wiswell TE

    更新日期:1998-04-01 00:00:00

  • Predicting time to hospital discharge for extremely preterm infants.

    abstract:BACKGROUND:As extremely preterm infant mortality rates have decreased, concerns regarding resource use have intensified. Accurate models for predicting time to hospital discharge could aid in resource planning, family counseling, and stimulate quality-improvement initiatives. OBJECTIVES:To develop, validate, and compa...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2009-0810

    authors: Hintz SR,Bann CM,Ambalavanan N,Cotten CM,Das A,Higgins RD,Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network.

    更新日期:2010-01-01 00:00:00

  • Milk intolerance in children with persistent sleeplessness: a prospective double-blind crossover evaluation.

    abstract::From July 1986 to July 1988, 146 children less than 5 years of age were referred by their physicians to our university sleep clinic for continual waking and crying during sleep hours. For 85 children (58.2%), the sleeplessness was attributed to inappropriate sleep habits. For 17 children (11.6%), no explanation was fo...

    journal_title:Pediatrics

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:

    authors: Kahn A,Mozin MJ,Rebuffat E,Sottiaux M,Muller MF

    更新日期:1989-10-01 00:00:00

  • Prevalence of Chlamydia trachomatis and Neisseria gonorrhoeae infection in pediatric private practice.

    abstract:BACKGROUND:Universal screening of sexually active adolescents for Chlamydia trachomatis (CT) and Neisseria gonorrhoeae (GC) has been recommended in settings in which prevalence is 2% or greater. Although believed to be above 2%, the prevalence of CT and GC infection in private practice settings has not been clearly est...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.108.6.e103

    authors: Best D,Ford CA,Miller WC

    更新日期:2001-12-01 00:00:00

  • Effect of dose formulation on isoniazid absorption in two young children.

    abstract::In an 8-month-old infant with tuberculous meningitis treatment with isoniazid was unsuccessful and was associated with lower than expected plasma concentrations of isoniazid (measured concentration 0.1 microgram/mL). The infant had received isoniazid as a crushed tablet admixed with apple sauce. Oral administration of...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Notterman DA,Nardi M,Saslow JG

    更新日期:1986-06-01 00:00:00

  • Promoting best-care practices in childhood asthma: quality improvement in community health centers.

    abstract:OBJECTIVE:Outpatient asthma management remains suboptimal. We previously reported significant improvements in asthma guideline adherence and outcomes in children by using quality-improvement processes and community health workers. We hypothesized that a larger project could achieve comparable outcome improvements with ...

    journal_title:Pediatrics

    pub_type: 杂志文章,多中心研究

    doi:10.1542/peds.2010-1962

    authors: Lob SH,Boer JH,Porter PG,Núñez D,Fox P

    更新日期:2011-07-01 00:00:00

  • Recognizing and treating toilet-seat contact dermatitis in children.

    abstract::Toilet-seat contact dermatitis is a common condition around the world and is reemerging in the United States. It can be easily recognized and treated. However, few practitioners consider this diagnosis, which results in a delay in treatment and often exacerbation of the skin eruption. In the past, exposure to wooden t...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2009-2430

    authors: Litvinov IV,Sugathan P,Cohen BA

    更新日期:2010-02-01 00:00:00

  • Adult outcomes of pediatric recurrent abdominal pain: do they just grow out of it?

    abstract:OBJECTIVE:To determine whether medically unexplained recurrent abdominal pain (RAP) in childhood predicts abdominal pain, irritable bowel syndrome (IBS), other somatic complaints, and psychiatric symptoms and disorders in young adulthood. METHODS:A sample of 28 young adults evaluated for RAP between the ages of 6 and ...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.108.1.e1

    authors: Campo JV,Di Lorenzo C,Chiappetta L,Bridge J,Colborn DK,Gartner JC Jr,Gaffney P,Kocoshis S,Brent D

    更新日期:2001-07-01 00:00:00

  • A practical clinical approach to diagnosis of fetal alcohol spectrum disorders: clarification of the 1996 institute of medicine criteria.

    abstract:BACKGROUND:The adverse effects of alcohol on the developing human represent a spectrum of structural anomalies and behavioral and neurocognitive disabilities, most accurately termed fetal alcohol spectrum disorders (FASD). The first descriptions in the modern medical literature of a distinctly recognizable pattern of m...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2004-0259

    authors: Hoyme HE,May PA,Kalberg WO,Kodituwakku P,Gossage JP,Trujillo PM,Buckley DG,Miller JH,Aragon AS,Khaole N,Viljoen DL,Jones KL,Robinson LK

    更新日期:2005-01-01 00:00:00

  • Early Intervention, Parent Talk, and Pragmatic Language in Children With Hearing Loss.

    abstract:BACKGROUND AND OBJECTIVES:Pragmatic language skills form the foundation for conversational competence, whereas deficits in this area are associated with behavioral problems and low literacy skills. Children who are deaf or hard of hearing demonstrate significant delays in this critical area of language. Our purpose wit...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2020-0242F

    authors: Yoshinaga-Itano C,Sedey AL,Mason CA,Wiggin M,Chung W

    更新日期:2020-11-01 00:00:00

  • Use of complementary and alternative medical therapies among children with special health care needs in southern Arizona.

    abstract:OBJECTIVE:The objective of this study was to assess the frequency and type of complementary and alternative medicine (CAM) therapies used by families of children with special health care needs in southern Arizona, as well as the correlates of their use. METHODS:Families of 376 children who were receiving services in a...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.111.3.584

    authors: Sanders H,Davis MF,Duncan B,Meaney FJ,Haynes J,Barton LL

    更新日期:2003-03-01 00:00:00

  • When is lack of supervision neglect?

    abstract::Occasionally, pediatricians become aware of children who are inadequately supervised. More frequently, pediatricians treat children for traumatic injuries or ingestions that they suspect could have been prevented with better supervision. This clinical report contains guidance for pediatricians considering a referral t...

    journal_title:Pediatrics

    pub_type: 指南,杂志文章

    doi:10.1542/peds.2006-1780

    authors: Hymel KP,Committee on Child Abuse and Neglect.

    更新日期:2006-09-01 00:00:00

  • Improving transition from pediatric to adult cystic fibrosis care: lessons from a national survey of current practices.

    abstract:OBJECTIVES:More than 500,000 adolescents with special health care needs age into adulthood each year in the United States, and there is growing recognition of the need for support of their transition to adult-oriented health care. Because of improved survival, cystic fibrosis has experienced this increasing transition ...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2007-2217

    authors: McLaughlin SE,Diener-West M,Indurkhya A,Rubin H,Heckmann R,Boyle MP

    更新日期:2008-05-01 00:00:00

  • Can developmental screening tests identify children who are developmentally at risk?

    abstract::Developmental screening tests are in widespread use, but few reliable and valid tests are available. The most frequently used screening instrument for detecting young children who are at risk for developmental delays is the Denver Developmental Screening Test (DDST). Although the DDST has excellent test specificity, o...

    journal_title:Pediatrics

    pub_type: 杂志文章,评审

    doi:

    authors: Meisels SJ

    更新日期:1989-04-01 00:00:00

  • Population-based studies of varicella complications.

    abstract::Population-based data on varicella complications are presented using information both from national sample surveys of hospitalizations and physician office visits and from reviews of medical records for all cases occurring within one community (Olmsted County, Minnesota) during a specified period. Acute cerebellar ata...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Guess HA,Broughton DD,Melton LJ 3rd,Kurland LT

    更新日期:1986-10-01 00:00:00

  • Does television viewing increase obesity and reduce physical activity? Cross-sectional and longitudinal analyses among adolescent girls.

    abstract::To examine the relationships between hours of television viewing and adiposity and physical activity among female adolescents, a cohort study with follow-up assessments 7, 14, and 24 months after baseline was conducted. All sixth- and seventh-grade girls (N = 971) attending four northern California middle schools were...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Robinson TN,Hammer LD,Killen JD,Kraemer HC,Wilson DM,Hayward C,Taylor CB

    更新日期:1993-02-01 00:00:00

  • Renal failure as a complication of acute antihypertensive therapy.

    abstract::Adult patients with long-standing hypertension have been reported to experience an impairment in renal function when treatment with potent vasodilating agents is initiated. To document that this sequence may occur in children as well, we report the case of a 4-year-old boy with renal disease in whom reduction of blood...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Green TP,Nevins TE,Houser MT,Sibley R,Fish AJ,Sinaiko AR

    更新日期:1981-06-01 00:00:00

  • Effect of exchange transfusion on bilirubin binding.

    abstract::The bilirubin titration point--the lowest bilirubin concentration at which loosely bound bilirubin could be demonstrated by Sephadex gel filtration--was determined in samples collected before, during, and on completion of 17 exchange transfusions as well as in the discarded and donor blood. The bilirubin titration poi...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Valaes T,Hyte M

    更新日期:1977-06-01 00:00:00

  • Identification and evaluation of children with autism spectrum disorders.

    abstract::Autism spectrum disorders are not rare; many primary care pediatricians care for several children with autism spectrum disorders. Pediatricians play an important role in early recognition of autism spectrum disorders, because they usually are the first point of contact for parents. Parents are now much more aware of t...

    journal_title:Pediatrics

    pub_type: 杂志文章,评审

    doi:10.1542/peds.2007-2361

    authors: Johnson CP,Myers SM,American Academy of Pediatrics Council on Children With Disabilities.

    更新日期:2007-11-01 00:00:00

  • Parent health and functioning 13 months after infant or child NICU/PICU death.

    abstract:BACKGROUND:After a child's death, parents may experience depression, posttraumatic stress disorder (PTSD), and increased risk for cancers, diabetes, psychiatric hospitalization, and suicide. Racial/ethnic differences are unknown. This longitudinal study investigated health and functioning of Hispanic, black, and white ...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2013-1194

    authors: Youngblut JM,Brooten D,Cantwell GP,del Moral T,Totapally B

    更新日期:2013-11-01 00:00:00

  • Perinatal human immunodeficiency virus-1 transmission and intrauterine growth: a cohort study in Butare, Rwanda.

    abstract:OBJECTIVE:To study the association of perinatal human immunodeficiency virus (HIV)-1 transmission with birth outcomes, including birth weight, gestational age, ponderal index, head circumference, and weight/head ratio. METHODS:Data from a prospective cohort study of 627 pregnant women and their infants in Butare, Rwan...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.102.2.e24

    authors: Weng S,Bulterys M,Chao A,Stidley CA,Dushimimana A,Mbarutso E,Saah A

    更新日期:1998-08-01 00:00:00

  • Respiratory failure and hypercoagulability in a toddler with Lemierre's syndrome.

    abstract::A 3.5-year-old healthy boy with 4 days of fever was referred to the emergency department for respiratory distress. The physical examination was remarkable for stupor, tachycardia, tachypnea, and dyspnea. Initial blood tests showed pancytopenia. He rapidly developed torticollis. Computerized tomography of the neck reve...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2004-2505

    authors: Schmid T,Miskin H,Schlesinger Y,Argaman Z,Kleid D

    更新日期:2005-05-01 00:00:00

  • Nurse Home Visiting and Maternal Mental Health: 3-Year Follow-Up of a Randomized Trial.

    abstract:BACKGROUND:Poor mental health is recognized as one of the greatest global burdens of disease. Maternal mental health is crucial for the optimal health of mothers and their children. We examined the effects of an Australian Nurse Home Visiting (NHV) program (right@home), offered to pregnant women experiencing adversity,...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2020-025361

    authors: Goldfeld S,Bryson H,Mensah F,Gold L,Orsini F,Perlen S,Price A,Hiscock H,Grobler A,Dakin P,Bruce T,Harris D,Kemp L

    更新日期:2021-02-01 00:00:00

  • The population cost-effectiveness of interventions designed to prevent childhood depression.

    abstract:BACKGROUND AND OBJECTIVES:Depression in childhood and adolescence is common and often persists into adulthood. This study assessed the population-level cost-effectiveness of a preventive intervention that screens children and adolescents for symptoms of depression in schools and the subsequent provision of a psychologi...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2011-1823

    authors: Mihalopoulos C,Vos T,Pirkis J,Carter R

    更新日期:2012-03-01 00:00:00

  • Recurrent pneumonia in children and its relationship to bronchial hyperreactivity.

    abstract::To determine what conditions are most likely to cause persistent or recurrent pneumonia (PRP) in children, the records of 81 children referred to James Whitcomb Riley Hospital for Children for evaluation of PRP were reviewed. Twenty patients had an apparent underlying cause that predisposed them to PRP. Of the 61 rema...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Eigen H,Laughlin JJ,Homrighausen J

    更新日期:1982-11-01 00:00:00

  • Systematic evidence review of newborn screening and treatment of severe combined immunodeficiency.

    abstract:CONTEXT:Severe combined immunodeficiency (SCID) is a group of disorders that leads to early childhood death as a result of severe infections. Recent research has addressed potential newborn screening for SCID. OBJECTIVE:To conduct a systematic review of the evidence for newborn screening for SCID, including test chara...

    journal_title:Pediatrics

    pub_type: 杂志文章,评审

    doi:10.1542/peds.2009-1567

    authors: Lipstein EA,Vorono S,Browning MF,Green NS,Kemper AR,Knapp AA,Prosser LA,Perrin JM

    更新日期:2010-05-01 00:00:00

  • A perimortem protocol for suspected genetic disease.

    abstract::A considerable portion of pediatric deaths represent disease with risk of recurrence in subsequent family members. Procedures to obtain samples of body fluids and tissues suitable for diagnosis of mendelian and chromosomal disorders are described. These procedures, the "perimortem protocol," are used in studying child...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:

    authors: Kronick JB,Scriver CR,Goodyer PR,Kaplan PB

    更新日期:1983-06-01 00:00:00

  • Timing of Complementary Feeding Introduction and Adiposity Throughout Childhood.

    abstract:OBJECTIVES:To examine associations of the timing of complementary feeding (CF) introduction with adiposity throughout childhood. METHODS:We studied 1013 children from Project Viva. Our exposure was CF introduction, categorized as <4 months (19%), 4 to <6 months (68%; reference group), and ≥6 months of age (14%). Our o...

    journal_title:Pediatrics

    pub_type: 杂志文章

    doi:10.1542/peds.2019-1320

    authors: Gingras V,Aris IM,Rifas-Shiman SL,Switkowski KM,Oken E,Hivert MF

    更新日期:2019-12-01 00:00:00