Abstract:
:Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare genetic disease that causes recurrent swelling attacks that may affect various body tissues. Angioedematous attacks can be fatal in the case of upper airway edema and are often preceded by prodromal symptoms like erythema marginatum. Initial symptoms usually occur in the first decade of life. We report on manifestation of profound and recurrent erythema marginatum in 2 newborns. In both cases, prodromal symptoms could help determine the diagnosis of C1-INH-HAE such that, at a later time, angioedematous attacks could be treated promptly and effectively. Awareness of C1-INH-HAE is low among physicians and even lower among the general public. This report aims at raising the level of awareness and shows that initial symptoms of the potentially life-threatening condition can manifest in newborns and that erythema marginatum can even be present at birth. Recognition of early symptoms and timely diagnosis of the disease along with adequate education of the pediatrician and parents are a prerequisite for prompt and effective treatment of attacks and the successful management of the disease.
journal_name
Pediatricsjournal_title
Pediatricsauthors
Martinez-Saguer I,Farkas Hdoi
10.1542/peds.2015-2411subject
Has Abstractpub_date
2016-02-01 00:00:00pages
e20152411issue
2eissn
0031-4005issn
1098-4275pii
peds.2015-2411journal_volume
137pub_type
杂志文章相关文献
PEDIATRICS文献大全abstract:OBJECTIVE:To determine the usefulness of measurements of the vaginal opening and amount of hymenal tissue present inferiorly and laterally in the diagnosis of vaginal penetration. METHODS:The transhymenal diameters and the amount of tissue present between the hymenal edge and vestibule inferiorly at 6 o'clock and late...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.109.2.228
更新日期:2002-02-01 00:00:00
abstract::Intestinal lymphangiectasia is a well-recognized complication of the Fontan procedure, occurring in up to 24% of patients. Because of the loss of chylous fluid into the gut lumen, protein-losing enteropathy results as well as lymphopenia and hypogammaglobulinaemia. In some cases, dilated lymphatics in the intestinal s...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.112.3.e242
更新日期:2003-09-01 00:00:00
abstract::In an attempt to determine whether iron-fortified infant formulas produced gastrointestinal symptoms in infants, we fed two similar formulas, one with and one without iron fortification, to a group of 93 term infants upon discharge from the nursery on the third day of life. Infants were fed the formulas for a period o...
journal_title:Pediatrics
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1980-08-01 00:00:00
abstract:OBJECTIVES:Patent ductus arteriosus (PDA) remains a challenging issue in very low birth weight (VLBW) infants, and its management varies widely. Our aim in this study was to document the natural course of ductus arteriosus in a cohort of VLBW infants who underwent conservative PDA management with no medical or surgical...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2016-4258
更新日期:2017-08-01 00:00:00
abstract::External auditory canal polyps are most commonly inflammatory in nature but may also manifest more severe disease. Prolonged conservative therapy may delay the correct diagnosis and appropriate intervention. A case is presented of a child with chronic otorrhea treated for 4 months with topical drops and antibiotics. O...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.113.4.e371
更新日期:2004-04-01 00:00:00
abstract:CONTEXT:Late-preterm infants born at 34 to 36 weeks' gestation have increased risks of various health problems. Health service utilization (HSU) of late-preterm infants has not been systematically summarized before. OBJECTIVES:To summarize the published literature on short- and long-term HSU by late-preterm infants ve...
journal_title:Pediatrics
pub_type: 杂志文章,meta分析,评审
doi:10.1542/peds.2017-0266
更新日期:2017-07-01 00:00:00
abstract::Hydrocarbon ingestions account for a substantial number of accidental poisonings; when aspirated, it can lead to severe pneumonitis. Treatment for severe pneumonitis is generally supportive, and outcomes are frequently poor. We report here the case of a 19-month-old girl who was treated successfully with early adminis...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2010-3229
更新日期:2011-06-01 00:00:00
abstract:OBJECTIVE:Vaccination does not cause sudden infant death syndrome (SIDS). However, SIDS peaks at 2 months of age, when vaccination encounters are frequent. There are no published estimates using population data on age of death and immunization coverage to indicate to practitioners how often coincident vaccination may o...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2004-2185
更新日期:2005-06-01 00:00:00
abstract::The interval from the beginning of the Q wave to the origin of the T wave (Q-oTc interval) and total and ionized serum calcium levels were measured in 27 full-term and 77 premature infants. The correlation between Q-oTc and total and ionized calcium levels was significant in both full-term and normal premature infants...
journal_title:Pediatrics
pub_type: 杂志文章
doi:
更新日期:1978-06-01 00:00:00
abstract:OBJECTIVE:There is no safe level of exposure to tobacco smoke. Thirdhand smoke is residual tobacco smoke contamination that remains after the cigarette is extinguished. Children are uniquely susceptible to thirdhand smoke exposure. The objective of this study was to assess health beliefs of adults regarding thirdhand s...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2008-2184
更新日期:2009-01-01 00:00:00
abstract::Heart rate and blood pressure changes following 0.5% tropicamide and 2.5% phenylephrine hydrochloride were evaluated in seven ill preterm infants (birth weight, 910 to 2,060 gm; gestational age, 26 to 36 weeks) during the first day of life. Each infant was monitored continuously for 30 minutes before and for 75 minute...
journal_title:Pediatrics
pub_type: 杂志文章
doi:
更新日期:1981-08-01 00:00:00
abstract:OBJECTIVES:We tested the hypotheses that apolipoprotein E genotype, in particular carriage of the epsilon4 allele, is more likely to be associated with cerebral palsy and that children with more severe neurologic impairment are more likely to carry this allele. METHODS:In this cross-sectional study, 209 children with ...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2006-1083
更新日期:2007-02-01 00:00:00
abstract:OBJECTIVE:Greater maternal prepregnancy adiposity has been associated with behavioral problems, such as attention-deficit/hyperactivity disorder, and lower intellectual function in offspring. However, few studies of humans have explored this, and it is unclear if intrauterine mechanisms or confounding factors drive the...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2010-0651
更新日期:2011-01-01 00:00:00
abstract::Servocontrol of skin temperature for the critically ill premature neonate nursed on a radiant warmer bed has been assumed to be analogous to skin temperature control for infants nursed in convection-warmed incubators. There are significant differences between these two warming techniques, and no definitive data exist ...
journal_title:Pediatrics
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1987-01-01 00:00:00
abstract:OBJECTIVE:To identify possible causes of suboptimal glycemic control (ascertained by hemoglobin A1c [HbA1c] level) in youths using insulin pump therapy. METHODS:Forty-eight youths who were receiving insulin pump therapy for > or =6 months, and who were using insulin pumps and blood glucose meters with data that could ...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.113.3.e221
更新日期:2004-03-01 00:00:00
abstract::Aerosolized racemic epinephrine, but not L-epinephrine, is commonly used in treating croup. The efficacy and adverse effects of nebulized racemic and L-epinephrine in the treatment of laryngotracheitis were compared. Children 6 months to 6 years of age with a croup score of 6 or above were assigned in a randomized dou...
journal_title:Pediatrics
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1992-02-01 00:00:00
abstract:OBJECTIVE:The clinical diagnosis of sepsis remains difficult, particularly in the young child, and would be improved by a rapid and reliable method for identification of bacteria in blood and other body fluids. Polymerase chain reaction (PCR) amplification of highly conserved DNA sequences found in all bacteria would p...
journal_title:Pediatrics
pub_type: 杂志文章
doi:
更新日期:1995-02-01 00:00:00
abstract:OBJECTIVES:Unintended variation in the care of patients with Crohn disease (CD) and ulcerative colitis (UC) may prevent achievement of optimal outcomes. We sought to improve chronic care delivery and outcomes for children with inflammatory bowel disease by using network-based quality improvement methods. METHODS:By us...
journal_title:Pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1542/peds.2011-1700
更新日期:2012-04-01 00:00:00
abstract:BACKGROUND:Blood transfusions in the neonatal patient population are common, but there are no established guidelines regarding transfusion thresholds. Little is known about postoperative outcomes in neonates who receive preoperative blood transfusions (PBTs). METHODS:Using the American College of Surgeons National Sur...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2019-3718
更新日期:2020-11-01 00:00:00
abstract::Age at diagnosis is a modifiable risk factor in outcomes after hepatoportoenterostomy in biliary atresia; however, distinguishing biliary atresia from other more common causes of prolonged neonatal jaundice can be difficult. To focus attention on diagnosis of biliary atresia, we analyzed secular trends in the age at d...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2007-2709
更新日期:2008-05-01 00:00:00
abstract::This publication contains the pediatric and neonatal sections of the 2005 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations (COSTR). The consensus process that produced this document was sponsored by the International Liaison Committee on ...
journal_title:Pediatrics
pub_type: 杂志文章,实务指引
doi:10.1542/peds.2006-0206
更新日期:2006-05-01 00:00:00
abstract::Each year, 5,000 Americans die and 300,000 are hospitalized as a result of 2.8 million residential fires. Almost all house fires allow time for safe exit if an early warning is given. Smoke detectors are an effective, reliable, and inexpensive method of providing such warning. After an upsurge of deaths related to fir...
journal_title:Pediatrics
pub_type: 杂志文章
doi:
更新日期:1985-01-01 00:00:00
abstract::Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs greater than or equal to 3.5%. Cross-sectional analysis...
journal_title:Pediatrics
pub_type: 杂志文章
doi:
更新日期:1985-09-01 00:00:00
abstract:BACKGROUND:Disparities in health service use have been described across a range of sociodemographic factors. Patterns of PICU use have not been thoroughly assessed. METHODS:This was a population-level, retrospective analysis of admissions to the Cincinnati Children's Hospital Medical Center PICU between 2011 and 2016....
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2019-0748
更新日期:2019-12-01 00:00:00
abstract:OBJECTIVE:We sought to document gender differences in lymphocyte subsets and plasma RNA levels in a pediatric cohort with presumed minimal hormonal differences (on the basis of age). METHODS:Blood samples from antiretroviral therapy-treated, HIV-infected children (n = 158) and HIV-uninfected children (n = 1801) who we...
journal_title:Pediatrics
pub_type: 杂志文章,多中心研究
doi:10.1542/peds.2005-0294
更新日期:2006-07-01 00:00:00
abstract:OBJECTIVES:The specific objectives of this study were (1) to assess the safety and efficacy of recombinant human erythropoietin (rhEPO) in reducing postnatal hemoglobin decline in premature infants of less than 33 weeks' gestation, and thus reducing the need for transfusion; and (2) to determine the optimal dosage of r...
journal_title:Pediatrics
pub_type: 临床试验,杂志文章
doi:
更新日期:1993-10-01 00:00:00
abstract::High-flow hepatic vascular anomalies with arteriovenous shunting commonly manifest during the neonatal period with signs and symptoms of congestive heart failure, but to our knowledge, they have never been described in patients with hereditary hemorrhagic telangiectasia (HHT). We report here our experience with 3 pati...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2010-2366
更新日期:2011-06-01 00:00:00
abstract::The clinical response of atypical mycobacterial cervical adenitis to standard antituberculous therapy has been disappointing. Surgical procedures in the anterior cervical triangle are difficult and often complete excision is impossible. In each of four children with atypical mycobacterial cervical adenitis in this stu...
journal_title:Pediatrics
pub_type: 杂志文章
doi:
更新日期:1975-01-01 00:00:00
abstract:OBJECTIVES:The current Australian epidemiology of Kawasaki disease (KD) is poorly defined. Previous enhanced surveillance (1993-1995) estimated an incidence of 3.7/100,000 <5 years. METHODS:We identified all patients hospitalized in Western Australia (current population ∼2.4 million) 1979 through 2009 with a discharge...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2013-2936
更新日期:2014-04-01 00:00:00
abstract:BACKGROUND:Pediatric functional abdominal pain disorders are common, costly, and disabling. Clinical anxiety is highly prevalent and is associated with increased pain and functional disability. Thus, a psychological screening process is recommended but is infrequently used in current practice. METHODS:A screening proc...
journal_title:Pediatrics
pub_type: 杂志文章
doi:10.1542/peds.2017-2876
更新日期:2018-08-01 00:00:00