Abstract：
UNLABELLED:Atypical hemolytic uremic syndrome (HUS) refers to the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury in the absence of Shiga toxin-producing Escherichia coli exposure or Streptococcus pneumoniae infection. Currently, approximately 50 % of the atypical cases have demonstrable mutations in complement regulatory proteins. Historically, the diagnosis of atypical HUS portends a poor prognosis with a high rate of disease recurrence, progression to end-stage renal disease, and death. However, it is now evident that atypical HUS actually encompasses a heterogeneous group of disorders, and there are reports suggesting that some cases of atypical HUS have a favorable prognosis, similar to that of diarrhea-associated disease. We present three patients with the atypical HUS phenotype who had complete renal recovery and no disease recurrence. We believe it is important to distinguish those cases of atypical HUS associated with disorders of complement regulatory proteins from other idiopathic causes of nondiarrheal HUS given the implications for prognosis and treatment. CONCLUSION:Given the heterogeneous nature and variable prognosis of atypical HUS, treatment should be carefully considered prior to the use of long-term plasma therapy and/or eculizumab.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Ruebner RL,Kaplan BS,Copelovitch Ldoi
10.1007/s00431-012-1763-zsubject
Has Abstractpub_date
2012-10-01 00:00:00pages
1519-25issue
10eissn
0340-6199issn
1432-1076journal_volume
171pub_type
杂志文章abstract：:The main ethical imperative of all paediatric actions is the demand to do everything "in the best interests of children". Relevant guidelines can be derived from the UN Declaration on the Rights of Children, whereupon every child has the fundamental right to life and dignity, and is entitled to optimal medical care. P...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-005-0002-2
更新日期：2006-02-01 00:00:00
abstract：UNLABELLED:Self-medication is a common event. To use drugs correctly, a basic knowledge about drugs is required. Poor data are available about adults' drug knowledge. Furthermore, adolescents' basic drug knowledge has not been investigated. This study was designed in order to explore adolescents' drug use and knowledge...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s004310000503
更新日期：2000-08-01 00:00:00
abstract：:The use of supra-physiological, exogenous corticosteroids in pregnancy may lead to neonatal adrenal suppression. We report on a single-center, case series study carried out between 2006 and 2014, which included all newborns (n = 16) of mothers using prednisolone ≥10 mg/day during pregnancy. Newborns were routinely ass...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-017-2949-1
更新日期：2017-08-01 00:00:00
abstract：:Using a randomized double blind study design, the bronchodilating effect of 200 micrograms inhaled oxitropium bromide (OB) was compared with 200 micrograms inhaled fenoterol (F) after an interval of 20 min, in 20 asthmatic children aged 12.7 years (range: 4.9-15.1 years), suffering from mild bronchoconstriction (mean ...
journal_title：European journal of pediatrics
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1007/BF02190670
更新日期：1994-08-01 00:00:00
abstract：:The objective of this study was to investigate the clinical applicability of pulse oximetry to measure haemoglobin oxygen saturation and heart rate in the first 20 min of life and to analyse the effect of pre- or post-ductal (hand, respectively, foot) fixation of sensors on oxygen saturation. Measurements were carried...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF02072072
更新日期：1990-09-01 00:00:00
abstract：:The performance of two triggering systems was compared during patient triggered ventilation (PTV) of infants ventilator-dependent beyond 10 days of age. Ten infants were studied who had a median gestational age of 26.5 weeks and a postnatal age of 15.5 days. PTV was administered via the SLE ventilator and the two trig...
journal_title：European journal of pediatrics
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1007/BF01957937
更新日期：1992-11-01 00:00:00
abstract：:The urofacial syndrome is a rare condition that occurs in both genders and characterized by uropathy and facial abnormalities. Early diagnosis is crucial for the management and prognosis of urinary problems. Paradoxical inversion of facial musculature when smiling, giving an appearance of crying associated with severe...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-013-2172-7
更新日期：2014-05-01 00:00:00
abstract：:There are three forms of high frequency ventilation, high frequency jet ventilation (HFJV, up to 400/min), high frequency oscillation (HFO, up to 40 Hz), and high frequency positive pressure ventilation (HFPPV, rates between 60 and 150/min). The first two forms of ventilation are still experimental and have been used ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00441591
更新日期：1987-09-01 00:00:00
abstract：:Positional plagiocephaly (PP) denotes flattening of the skull that occurs frequently in healthy infants. Aim of this study was to estimate the prevalence of positional plagiocephaly and to identify the risk factors in a cohort of healthy infants in order to help prevention of PP. In a prospective design, all healthy f...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-018-3212-0
更新日期：2018-10-01 00:00:00
abstract：:Most reviews on weight status have focused on obesity, and little information on underweight children is available. This review aimed to examine the prevalence and trends of underweight status among Asian children and adolescent populations in the last two decades. A systematic review of publications between the years...
journal_title：European journal of pediatrics
pub_type： 杂志文章,评审
doi：10.1007/s00431-012-1685-9
更新日期：2012-05-01 00:00:00
abstract：UNLABELLED:The pattern and distribution of subcutaneous fat in term and preterm newborns has been assessed by skinfold thicknesses (ST), describing gender and gestational age variations. Weight, length and ST (triceps, biceps, subscapular and suprailiac) were measured in 4634 neonates (2445 males and 2189 females) aged...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-004-1468-z
更新日期：2004-08-01 00:00:00
abstract：UNLABELLED:Although a range of antiretroviral drugs are available for use in children, the appropriate paediatric regimen remains unclear. In a survey to investigate policies and practices relating to the therapeutic management of children infected by the human immunodeficiency virus (HIV), a postal questionnaire was s...
journal_title：European journal of pediatrics
pub_type： 杂志文章,多中心研究
doi：10.1007/s004310050045
更新日期：2000-03-01 00:00:00
abstract：UNLABELLED:Click-evoked oto-acoustic emissions (CEOAE) are acoustic responses produced by the inner ear, reflecting functional auditory integrity. We studied both the success rate of the CEOAE method and the CEOAE presence in preterm infants during their stay at the Neonatal Intensive Care Unit (NICU), by analysis of t...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s004310050986
更新日期：1998-12-01 00:00:00
abstract：:So far, a recognizable pattern of clinical symptoms for child sexual abuse (CSA), especially in young male children, is lacking. To improve early recognition of CSA, we reviewed physical complaints, physical examination, and tests on sexually transmitted infections (STIs) in confirmed victims (predominantly preschool ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-017-2996-7
更新日期：2017-10-01 00:00:00
abstract：UNLABELLED:The pathogenesis of sporadic hemiplegic migraine remains unclear, and perfusion-weighted magnetic resonance imaging (PWI) has been used for characterization of hemodynamic changes in migraine aura. We report a case of sporadic hemiplegic migraine in which magnetic resonance perfusion imaging showed left cere...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-015-2609-2
更新日期：2016-02-01 00:00:00
abstract：:The concentration of mRNA of insulin-like growth factor-II is (IGF-II) much elevated in some embryonic tumours such as Wilms tumour (nephroblastoma). In order to prove whether or not IGF-II is produced by the tumour tissue, IGF-II was extracted from freshly frozen tissue of Wilms tumour and hepatoblastoma. Normal adja...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF02072483
更新日期：1993-02-01 00:00:00
abstract：UNLABELLED:Aminoglycoside prescriptions were rarely evaluated in children care facilities. Because of risk of toxicity, these narrow spectrum antibiotics are commonly misused. In this study, we evaluate aminoglycoside prescription and assess the impact of an information campaign on modalities of prescription and monito...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-016-2691-0
更新日期：2016-05-01 00:00:00
abstract：:Of all newborn infants, 5% require some degree of basic life support at birth. Newborn resuscitation therefore is one of the most frequent procedures carried out in medicine. It is therefore important that the routines in use are evidence based. Newborn resuscitation can be divided into ten steps: (1) initial stabilis...
journal_title：European journal of pediatrics
pub_type： 杂志文章,评审
doi：10.1007/pl00014284
更新日期：1998-01-01 00:00:00
abstract：:Current practice favors serotesting adolescents with a negative history of chickenpox rather than offering presumptive vaccination. Recent epidemiologic data from Greece indicate that a high proportion of adolescents (21.5%) are susceptible to chickenpox. We assessed the reliability of negative varicella history in re...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-008-0701-6
更新日期：2009-01-01 00:00:00
abstract：:A 10-year-old boy presented with priapism of 10 h duration which after unsuccessful conservative measures, was relieved by a saphenocorporeal shunt. A 4-year history of intermittent vague aching of fingers and toes accompanied by low-grade fever was reported. Fabry disease was confirmed by the lack alpha-galactosidase...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF01959404
更新日期：1990-04-01 00:00:00
abstract：UNLABELLED:Medication prescribing errors (MPE) can result in serious consequences for patients. In order to reduce errors, we need to know more about the frequency, the type and the severity of such errors. We therefore performed a prospective observational study to determine the number and type of medication prescribi...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-015-2542-4
更新日期：2015-10-01 00:00:00
abstract：:Less invasive surfactant administration (LISA) is a method to deliver surfactant to spontaneously breathing premature infants via a thin catheter. Here we report the two-year outcome from the AMV (avoid mechanical ventilation) study, the first randomized controlled trial on this mode of surfactant delivery. No statist...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-020-03572-0
更新日期：2020-08-01 00:00:00
abstract：:Despite rapidly evolving knowledge about COVID 19 infection, routes of perinatal COVID 19 transmission and viral load in mother neonate dyad remain uncertain. Data were analysed to describe the clinicodemographic profile and viral load in neonates born to COVID 19 positive mothers. Of 2947 deliveries, 69 mothers were ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-020-03800-7
更新日期：2021-02-01 00:00:00
abstract：:The light- and electron-microscopic structure of articular and costal cartilage in a case of achondrogenesis type I has been described. The most characteristic ultrastructural change in the chondrocytes was conspicuous dilatation of the rough endoplasmatic reticulum (RER) which contained amorphous electronopaque mater...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00442406
更新日期：1980-06-01 00:00:00
abstract：:We updated our 2010 systematic review on the efficacy of probiotics in the treatment of constipation in children. The MEDLINE, EMBASE, and Cochrane Library databases; clinical trial registries; and reference lists of included studies were searched to February 2017 for randomized controlled trials (RCTs) performed in c...
journal_title：European journal of pediatrics
pub_type： 杂志文章,评审
doi：10.1007/s00431-017-2972-2
更新日期：2017-09-01 00:00:00
abstract：:Two girls aged 6 and 10 years treated with anticonvulsants developed nystagmus and ataxia. The peak plasma concentrations of phenytoin were 42.6 and 46.6 microgram/ml, respectively, compatible with phenytoin toxicity. The decline in plasma phenytoin levels did not fit first order kinetics, but followed Michaelis-Mente...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/BF00445896
更新日期：1980-10-01 00:00:00
abstract：UNLABELLED:Our aim was to evaluate effectiveness of pandemic influenza A/H1N1 vaccine in preventing acute otitis media (AOM) and/or otitis media with effusion (OME), in a randomized, prospective and single-blind study conducted in the children aged of 6-60 months. This study was done between December 1, 2009 and April ...
journal_title：European journal of pediatrics
pub_type： 杂志文章,随机对照试验
doi：10.1007/s00431-012-1797-2
更新日期：2012-11-01 00:00:00
abstract：UNLABELLED:A retrospective analysis was performed of the records of 133 patients with extrahepatic biliary atresia (EHBA) who had undergone a Kasai portoenterostomy. The patients were divided into a non-transplantation group who survived but did not receive liver transplantation after the procedure and a failure group ...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-003-1268-x
更新日期：2003-09-01 00:00:00
abstract：:Hypoglycaemia in children can be a life-threatening situation that needs to be assessed rigorously in order to treat efficiently and avoid relapse that can be responsible for cerebral damage. The diagnosis of impairment in glucose homeostasis requires the knowledge of the mechanisms regulating blood glucose concentrat...
journal_title：European journal of pediatrics
pub_type： 杂志文章,评审
doi：10.1007/s00431-007-0600-2
更新日期：2008-03-01 00:00:00
abstract：:This study is a retrospective analysis of seven patients with hypoechogenic lesions in the liver and/or spleen due to Bartonella henselae, who were followed from 1998 through to 2005 by the Department of Pediatrics, Turin University. In addition to showing constitutional symptoms, four children had skin lesions sugges...
journal_title：European journal of pediatrics
pub_type： 杂志文章
doi：10.1007/s00431-007-0500-5
更新日期：2008-04-01 00:00:00
