3-Hydroxydicarboxylic aciduria due to long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency associated with sudden neonatal death: protective effect of medium-chain triglyceride treatment.

abstract：UNLABELLED:The severity and duration of immunosuppression caused by corticosteroids (CSs) usage have not been extensively studied. We aimed to investigate the effects of CSs on the various compartments of immune system in relation to timing of initiation and persistence of therapy. Pediatric patients with idiopathic ne...

journal_title：European journal of pediatrics

authors： Baris HE,Baris S,Karakoc-Aydiner E,Gokce I,Yildiz N,Cicekkoku D,Ogulur I,Ozen A,Alpay H,Barlan I

更新日期：2016-05-01 00:00:00

abstract：:A girl, now three years old, is reported, in whom at the age of 5 months the diagnosis of 3-HMG-CoA lyase deficiency was established. The characteristic excretion pattern consisted of 3-HMG, 3-CH3-glutaconic acid, 3-CH3-glutaric acid and 3-HIVA. Activity of 3-HMG-CoA lyase in leucocytes was very low. She had compensat...

journal_title：European journal of pediatrics

authors： Leupold D,Bojasch M,Jakobs C

更新日期：1982-02-01 00:00:00

abstract：UNLABELLED:The aim of this study was to assess the physical performance in long-term survivors of acute leukaemia in childhood and to evaluate the effects of anthracycline therapy. Electrocardiography, echocardiography and spiroergometry were carried out on 56 patients aged 9-28 years, of whom 44 patients had been trea...

journal_title：European journal of pediatrics

authors： Black P,Gutjahr P,Stopfkuchen H

更新日期：1998-06-01 00:00:00

abstract：:The New Vaccinations of Infants in Practice online survey in seven countries evaluated vaccination-related attitudes and concerns of parents of infants and health care providers (HCPs) who provide pediatric medical care. The survey showed that HCPs and parents were open to adding new vaccines to the immunization sched...

journal_title：European journal of pediatrics

authors： Bakhache P,Rodrigo C,Davie S,Ahuja A,Sudovar B,Crudup T,Rose M

更新日期：2013-04-01 00:00:00

abstract：:The results of 24 h oesophageal pH monitoring, performed in 129 infants aged 6-10 weeks, were compared to those in the same patients after shorter periods (3, 6, 9 and 12 h). In the investigated population there was no significant difference between the reflux index (percentage of time with a pH < 4.0) after 12 or 24 ...

journal_title：European journal of pediatrics

authors： Vandenplas Y,Casteels A,Naert M,Derde MP,Blecker U

更新日期：1994-02-01 00:00:00

abstract：:The correlation between the appearance of functionally-atypical factor VII and perinatal complications was investigated in 66 newborn infants. The presence of an abnormal clotting factor was assumed if the ratio between clotting activity and antigen-related factor VII material exceeded the normal range for adult plasm...

journal_title：European journal of pediatrics

authors： Maak B,Frenzel J

更新日期：1977-08-12 00:00:00

abstract：UNLABELLED:Hypotension is a commonly diagnosed and treated complication of extremely low gestational age newborns (ELGAN), but enormous variation in diagnosis, management and clinical practice has been documented. We sought to evaluate practice regarding the management of hypotension in ELGANs and developed a web-based...

journal_title：European journal of pediatrics

authors： Stranak Z,Semberova J,Barrington K,O'Donnell C,Marlow N,Naulaers G,Dempsey E,HIP consortium.

更新日期：2014-06-01 00:00:00

abstract：:Histochemical and immunohistochemical studies performed in only a few cases of sinus histiocytosis with massive lymphoadenopathy (SHML) indicated that SHML cells belong to the macrophage--histiocyte system, though their exact origin is still uncertain. We analyzed the morphological, antigenic and enzymatic characteris...

journal_title：European journal of pediatrics

authors： Paulli M,Locatelli F,Kindl S,Boveri E,Facchetti F,Porta F,Rosso R,Nespoli L,Magrini U

更新日期：1992-09-01 00:00:00

abstract：UNLABELLED:Disseminated atypical mycobacterium infection is essentially reported in cellular immunodeficient children. Cell-mediated immunity including cytokines like tumour necrosis factor alpha (TNF) and gamma interferon (IFN) is the most important factor allowing control of the dissemination of Mycobacterium. We rep...

journal_title：European journal of pediatrics

authors： Tuerlinckx D,Vermylen C,Brichard B,Ninane J,Cornu G

更新日期：1997-03-01 00:00:00

abstract：:In a German pediatric oncology unit, the attending physicians diagnosed 27 cases of Clostridium difficile-associated disease (CDI) from January 01, 2010 to October 31, 2013. This refers to a CDI incidence density of 2.0/1000 inpatient days. According to the hospital hygiene standard, symptomatic patients with CDI were...

journal_title：European journal of pediatrics

authors： Simon A,Mock M,Graf N,von Müller L

更新日期：2018-03-01 00:00:00

abstract：:Prolonged emergency department (ED) length of stay (LOS) is used as a proxy for ED overcrowding and is associated with adverse outcomes of patients requiring therapy and reduced patient satisfaction. Our aim was to identify and quantify variables which affect ED-LOS. Patients admitted to the pediatric ED of a large re...

journal_title：European journal of pediatrics

authors： Hofer KD,Saurenmann RK

更新日期：2017-05-01 00:00:00

abstract：UNLABELLED:A 6-year-old male with partial ornithine transcarbamylase (OTC) deficiency had acute and rapidly progressive symmetrical swelling of the head of the caudate nuclei and putamina. Clinical presentation was ataxia and dysarthria progressing to seizures and coma; these symptoms gradually resolved with supportive...

journal_title：European journal of pediatrics

authors： Keegan CE,Martin DM,Quint DJ,Gorski JL

更新日期：2003-04-01 00:00:00

abstract：:Twenty patients with rheumatoid arthritis or Still's disease associated with growth failure were treated with human growth hormone, 7.5 to 17 U/m2 body surface per week. Five patients did not respond with better growth. In the remainder the mean growth rate increased from 1.9 cm/year (range: 0 to 3.3) to 6.2 cm/year (...

journal_title：European journal of pediatrics

authors： Butenandt O

更新日期：1979-01-18 00:00:00

abstract：:In order to determine the hepatotoxicity of rifampicin in children treated for tuberculosis, a survey was performed of 18 children receiving this medicine in combination with isoniazid. Fifteen of the 18 children (83%) showed a rise in ASAT values and 11 (61%) in ALAT values exceeding 29U/L. Seven children with maxima...

journal_title：European journal of pediatrics

authors： Linna O,Uhari M

更新日期：1980-09-01 00:00:00

abstract：:An autosomal dominant hereditary bone dysplasia was observed in three members of a Sardinian family living in Germany--the father and two sons. The guiding clinical symptom is thickening of the wrist proximal to the styloid process of the ulna. The radiologically recognizable changes--non-calcified cartilaginous islan...

journal_title：European journal of pediatrics

authors： Rosenberg E,Löhr H

更新日期：1986-04-01 00:00:00

abstract：UNLABELLED:Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterised by periodic attacks of fever and serositis. Recent genetic and epidemiological research have highlighted the importance of this disease. FMF is the most frequent periodic fever syndrome and is transmitted in an autosomal recessi...

journal_title：European journal of pediatrics

authors： Ozen S

更新日期：2003-07-01 00:00:00

abstract：UNLABELLED:Over the last few years, the importance of paediatric stroke has become more and more evident; however, there is still little known about long-term neurological and especially neuropsychological outcome of these children. By retrospective chart review, questionnaire and clinical examination with structured i...

journal_title：European journal of pediatrics

authors： Steinlin M,Roellin K,Schroth G

更新日期：2004-04-01 00:00:00

abstract：:The case history of a 10-year-old boy with chronic granulomatous disease (CGD) and gastric obstruction is presented. First abdominal symptoms occurred at 4 years of age when antral narrowing was detected. Due to unresponsiveness to antibiotic and steroid treatment, salazosulfapyridine therapy was initiated. Objective ...

journal_title：European journal of pediatrics

authors： Stopyrowa J,Fyderek K,Sikorska B,Kowalczyk D,Zembala M

更新日期：1989-10-01 00:00:00

abstract：:We report a patient with the characteristic features of the brittle cornea syndrome, a rare, autosomal recessively inherited disorder, namely brittle corneae, blue sclerae, and red hair. The patient also showed joint hyperextensibility, a soft skin, and dysplastic auricles with unusually soft cartilage. Phenotypically...

journal_title：European journal of pediatrics

authors： Royce PM,Steinmann B,Vogel A,Steinhorst U,Kohlschuetter A

更新日期：1990-04-01 00:00:00

abstract：UNLABELLED:A suprasellar arachnoid cyst may cause disorders of growth, puberty and hypothalamic-pituitary function, due to the proximity of the cyst to the hypothalamic-pituitary area. A total of 30 patients (17 boys) with cyst diagnosed at 4.3 +/- 1 years were routinely evaluated at 5.4 +/- 1 years; 24 of them had one...

journal_title：European journal of pediatrics

authors： Adan L,Bussières L,Dinand V,Zerah M,Pierre-Kahn A,Brauner R

更新日期：2000-05-01 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

authors： Ballardini E,Sisti M,Basaglia N,Benedetto M,Baldan A,Borgna-Pignatti C,Garani G

更新日期：2018-10-01 00:00:00

abstract：UNLABELLED:Neurological complications of immunizations are rare. We report the case of relapsing acute encephalitis in a boy after two subsequent diphtheria-tetanus-poliomyelitis vaccinations. First the clinical signs were those of acute disseminated encephalitis. During the second episode, the boy experienced optic ne...

journal_title：European journal of pediatrics

authors： Mancini J,Chabrol B,Moulene E,Pinsard N

更新日期：1996-02-01 00:00:00

abstract：:The purpose of this study was to determine the percentiles of interpopliteal distance to diagnose bowleg in 0-6 year-old children. Measurement of interpopliteal distance is a simple but valuable indicator in clinical examination to diagnose bowleg. We analyzed data from the anthropometry of Turkish children aged 0-6 y...

journal_title：European journal of pediatrics

authors： Kurtoglu S,Mazicioglu MM,Ozturk A,Hatipoglu N,Balci E,Ustunbas HB

更新日期：2011-09-01 00:00:00

abstract：:We compared epinephrine and dopamine as a first-line vasoactive drug in 40 neonates (enrolled in two gestational age strata ≤ 306/7 and ≥ 310/7 weeks) with fluid-refractory septic shock. Epinephrine or dopamine was initiated at 0.2 or 10 μg/kg/min, respectively. If shock persisted after 15 min, epinephrine or dopamine...

journal_title：European journal of pediatrics

authors： Baske K,Saini SS,Dutta S,Sundaram V

更新日期：2018-09-01 00:00:00

abstract：:Diagnostic tools for the management of acute osteomyelitis (OM) and septic arthritis (SA) have improved over the last decade. To investigate the influence and availability of magnetic resonance imaging (MRI) and nucleic acid testing (NAT), a retrospective cohort study was done. Patients admitted with acute OM or SA be...

journal_title：European journal of pediatrics

authors： Manz N,Krieg AH,Heininger U,Ritz N

更新日期：2018-07-01 00:00:00

abstract：:The renal function of 12 patients with non vitamin B12 responsive methylmalonic acidaemia has been investigated. Eight patients had reduced glomerular filtration rates, but the plasma creatinine concentration was only raised in those with values of less than 40 ml/min per 1.73 m2 surface area. The reduction in glomeru...

journal_title：European journal of pediatrics

authors： Walter JH,Michalski A,Wilson WM,Leonard JV,Barratt TM,Dillon MJ

更新日期：1989-01-01 00:00:00

abstract：UNLABELLED:Nemaline myopathy, one of the most benign myopathies in older children and adults, may be rarely associated with early death in the neonate. The authors report two cases, describing the ante- and postnatal clinical courses. Family history may help in establishing the diagnosis, but this was not the case in t...

journal_title：European journal of pediatrics

authors： Vendittelli F,Manciet-Labarchède C,Gilbert-Dussardier B

更新日期：1996-06-01 00:00:00

abstract：:Soon after birth a 36-week-gestational age, appropriate for dates, newborn infant developed hyperinsulinaemic hypoglycaemia which responded to diazoxide and glucose infusion. While normoglycaemic, he became increasingly ill with high lactate, pyruvate, and alanine levels. Provocation tests suggested a defect in lactat...

journal_title：European journal of pediatrics

authors： Aynsley-Green A,Weindling AM,Soltész G,Jenkins PA

更新日期：1983-12-01 00:00:00

abstract：UNLABELLED:The first live-attenuated Oka strain varicella vaccines needed to be stored at -20 degrees C. Reformulation of this vaccine by SmithKline Beecham Biologicals has provided a vaccine shelf life of up to 2 years when stored at +4 degrees C to +8 degrees C. In this study the immunogenicity and reactogenicity of ...

journal_title：European journal of pediatrics

authors： Tan AY,Connett CJ,Connett GJ,Quek SC,Yap HK,Meurice F,Lee BW

更新日期：1996-08-01 00:00:00

abstract：UNLABELLED:The true burden of influenza in children is difficult to assess and is probably underestimated as clinical signs are usually nonspecific, and formal viral identification is rarely searched. In this study, we compare the clinical features of infections related to the new H1N1/09 influenza virus with infection...

journal_title：European journal of pediatrics

authors： Crisinel PA,Barazzone C,Kaiser L,L'Huillier AG,Taguebue J,Wagner N,Delcò C,Siegrist CA,Posfay-Barbe KM,H1N1 Pediatric Epidemiology Study Group.

更新日期：2012-01-01 00:00:00