Longitudinal study on early diagnosis and treatment of phenylketonuria in Poland.

abstract：UNLABELLED:To assess the role of dexamethasone treatment as a cause of systemic hypertension and associated complications, blood pressure was registered prospectively before, during and after a 4-week dexamethasone course in 22 neonates with chronic lung disease. In all patients systolic blood pressure rose significant...

journal_title：European journal of pediatrics

authors： Smets K,Vanhaesebrouck P

更新日期：1996-07-01 00:00:00

abstract：UNLABELLED:Patients diagnosed with intracranial teratoma are at risk for developing a recurrent malignant germ cell tumor. We describe a 14-year-old boy initially diagnosed with a mature teratoma in the pineal region that recurred as a metastatic beta-human chorionic gonadotropin (βHCG)-secreting germ cell tumor 3 year...

journal_title：European journal of pediatrics

authors： Cardellicchio S,Farina S,Buccoliero AM,Agresti B,Genitori L,de Martino M,Fangusaro J,Sardi I

更新日期：2014-08-01 00:00:00

abstract：:A male infant with transient citrullinemia is described. Initially, he was found to have hypertyrosinemia and hypermethioninemia upon routine neonatal screening for inborn errors of metabolism performed at 4 days of age and was revealed to have citrullinemia upon detailed examination of plasma amino acids. At 30 days ...

journal_title：European journal of pediatrics

authors： Ohtake A,Takayanagi M,Ogura N,Nakajima H

更新日期：1983-10-01 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：: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：:Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis. CONCLUSION. Portal hypertension is a complication of systemic mastocytosis that can lead to dea...

journal_title：European journal of pediatrics

authors： Fonga-Djimi HS,Gottrand F,Bonnevalle M,Farriaux JP

更新日期：1995-10-01 00:00:00

abstract：:To elucidate the basis for the recurrent infections in patients with glycogen storage disease (GSD) Ib we tested polymorphonuclear leukocyte (PMN) function in one patient. Bactericidal capacity and phagocytosis-induced O2 consumption were reduced. Also, phorbol myristate acetate-stimulated superoxide production and gl...

journal_title：European journal of pediatrics

authors： Gahr M,Heyne K

更新日期：1983-09-01 00:00:00

abstract：INTRODUCTION:Randomly estimated fasting hyperglycaemia in an asymptomatic individual may represent the first sign of pancreatic beta-cell dysfunction. OBJECTIVE:We aimed at specifying the genetic aetiology of asymptomatic hyperglycaemia in a cohort of children and adolescents. SUBJECTS AND METHODS:We analysed the aet...

journal_title：European journal of pediatrics

authors： Feigerlová E,Pruhová S,Dittertová L,Lebl J,Pinterová D,Kolostová K,Cerná M,Pedersen O,Hansen T

更新日期：2006-07-01 00:00:00

abstract：:A 16-year-old girl developed rapidly progressive glomerulonephritis and renal failure. The disease was associated with high titres of antiglomerular basement membrane antibodies in serum, linear deposits of immunoglobulin G and diffuse epithelial crescents on renal biopsy. Past history revealed heavy smoking and delib...

journal_title：European journal of pediatrics

authors： Bonzel KE,Müller-Wiefel DE,Ruder H,Wingen AM,Waldherr R,Weber M

更新日期：1987-05-01 00:00:00

abstract：:To nine cystic fibrosis patients with chronic bronchopulmonary infection of severely damaged lungs invaded by Pseudomonas aeruginosa, eleven courses of prolonged tobramycin treatment (5 mg/kg/day) for four to 16 weeks were administered. Pulmonary symptoms improved and a better quality of life was achieved in all but o...

journal_title：European journal of pediatrics

authors： Paporisz U,Posselt HG,Wönne R,Ristow W,Röser D,Knothe H,Bender SW

更新日期：1979-04-03 00:00:00

abstract：UNLABELLED:We describe a case of iatrogenic pseudo-Bartter syndrome caused by administration of prostaglandin E1 (PGE1 alprostadil). Although the use of i.v. PGE1 is a well-established pharmacological therapy in neonates with a ductus-dependent congenital cardiopathy to ensure ductus-dependent flow, we could only find ...

journal_title：European journal of pediatrics

authors： Vanhaesebrouck S,Allegaert K,Vanhole C,Devlieger H,Gewillig M,Proesmans W

更新日期：2003-09-01 00:00:00

abstract：:We discuss the unusual presentation of acquired toxoplasmosis in a girl with severe and transient hemidystonia as a unique symptom. Serum titres of anti-toxoplasma antibodies increased whereas no specific antibody response in the CSF was observed. While symptomatic drugs were inefficacious, specific anti-toxoplasmosis...

journal_title：European journal of pediatrics

authors： Micheli R,Perini A,Duse M

更新日期：1994-10-01 00:00:00

abstract：UNLABELLED:The need to reappraise sedation policy is dictated by newer noninvasive imaging modalities. In this study we evaluate the safety and efficacy of rectally-administered thiopental in children undergoing MRI. Eighty-three consecutive patients with congenital heart disease (mean age 2.9 years, SD 2.5) undergoing...

journal_title：European journal of pediatrics

authors： Beekman RP,Hoorntje TM,Beek FJ,Kuijten RH

更新日期：1996-09-01 00:00:00

abstract：:By the description of two cases of osteoarticular infections due to Kingella kingae in two young children we wish to draw the attention of clinicians to invasive infections due to this micro-organism. Since its biological characterization in 1976, K. kingae has been increasingly reported as a human pathogen. Most comm...

journal_title：European journal of pediatrics

authors： Lacour M,Duarte M,Beutler A,Auckenthaler R,Suter S

更新日期：1991-07-01 00:00:00

abstract：:Respiratory syncytial virus (RSV) is the leading cause of lower respiratory tract infections and hospitalizations in children aged < 2 years. The aim of this retrospective, single-centre study was to examine the characteristics of patients admitted to a paediatric intensive care unit (PICU) with RSV infection followin...

journal_title：European journal of pediatrics

authors： Butt ML,Symington A,Janes M,Elliott L,Steele S,Paes BA

更新日期：2011-07-01 00:00:00

abstract：:A 10-month-old infant is described who suffered from extensive atopic dermatitis, failure to thrive, hypoalbuminaemia and oedema. Large amounts of sticky exudate were lost through the skin and were shown to be rich in albumin. As renal and intestinal loss of protein was excluded, the patient's condition was ascribed t...

journal_title：European journal of pediatrics

authors： Abrahamov A,Schifmann R,Goldstein R,Tal Y,Freier S

更新日期：1986-08-01 00:00:00

abstract：:In 166 full term, small-for-gestational-age (FT-SGA), 53 preterm, appropriate-for-gestational-age (PT-AGA), 27 PT-SGA and 206 FT-AGA infants a neurological examination at the age of 6 years was carried out. Data were collected on behaviour and school achievement. Major and minor neurological abnormalities were more fr...

journal_title：European journal of pediatrics

authors： Hadders-Algra M,Huisjes HJ,Touwen BC

更新日期：1988-06-01 00:00:00

abstract：:Cerebral blood flow velocities (CBFV) were measured by the pulsed Doppler method in 41 infants of smoking mothers and in 59 apparently healthy control infants. Although gestational age, birth weight, and systolic blood pressure were lower in infants exposed to tobacco smoke prenatally, systolic (65 +/- 11 vs. 47 +/- 1...

journal_title：European journal of pediatrics

authors： Abdul-Khaliq H,Segerer H,Luck W,Obladen M

更新日期：1993-03-01 00:00:00

abstract：:Continuous negative extrathoracic pressure (CNEP) has been recently reintroduced as therapy for respiratory failure. To determine its effects on cardiac output a pilot study was performed in ten patients aged 2 months-3 years (median 4 months). All had chronic respiratory failure (seven with bronchopulmonary dysplasia...

journal_title：European journal of pediatrics

authors： Raine J,Redington AN,Benatar A,Samuels MP,Southall DP

更新日期：1993-07-01 00:00:00

abstract：:Rasmussen's syndrome (RS) is a rare acquired progressive inflammatory encephalopathy characterized by drug-resistant partial seizures and cognitive deterioration resulting from a gradual impairment and a subsequent atrophy of a single brain hemisphere. It was firstly described by Theodore Rasmussen in 1958. The origin...

journal_title：European journal of pediatrics

authors： Mastrangelo M,Mariani R,Menichella A

更新日期：2010-08-01 00:00:00

abstract：:The primary objective of this study was to evaluate whether pediatric respiratory rate-oxygenation index (p-ROXI) and variation in p-ROXI (p-ROXV) can serve as objective markers in children with high-flow nasal cannula (HFNC) failure. In this prospective, single-center observational study, all patients who received HF...

journal_title：European journal of pediatrics

authors： Yildizdas D,Yontem A,Iplik G,Horoz OO,Ekinci F

更新日期：2020-10-19 00:00:00

abstract：:It is rare that coloboma, heart anomalies, choanal atresia, retarded growth and development, and genital and ear anomalies (CHARGE) syndrome patients have DiGeorge sequence showing severe immunodeficiency due to the defect of the thymus. Although the only treatment to achieve immunological recovery for these patients ...

journal_title：European journal of pediatrics

authors： Inoue H,Takada H,Kusuda T,Goto T,Ochiai M,Kinjo T,Muneuchi J,Takahata Y,Takahashi N,Morio T,Kosaki K,Hara T

更新日期：2010-07-01 00:00:00

abstract：:A 4.5-year-old male patient is described with chorioretinopathy, minor facial anomalies, delayed closure of the fontanel, mental retardation, moderate hypotonia, epilepsy and hepatic fibrosis. Postural control, intentional vocalising and manual dexterity were superior to the performance of patients with classical Zell...

journal_title：European journal of pediatrics

authors： Barth PG,Schutgens RB,Bakkeren JA,Dingemans KP,Heymans HS,Douwes AC,van der Klei-van Moorsel JM

更新日期：1985-11-01 00:00:00

abstract：:In fourteen infants, aged 9--60 days, with generalized oedema seen during a one year period the common denominator was the administration of dipyrone one to two days prior to the development of oedema. None of the other causes of oedema in early life could be incriminated in any of these babies. Pediatricians should b...

journal_title：European journal of pediatrics

authors： Bajoghli M,Ajudani TS,Gharavi M

更新日期：1977-11-04 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：:Heart rate variability (HRV) is currently considered the most valuable non-invasive test to investigate the autonomic nervous system function, based on the fact that fast fluctuations might specifically reflect changes of sympathetic and vagal activity. An association between abnormal values of HRV and brain impairmen...

journal_title：European journal of pediatrics

authors： Bersani I,Piersigilli F,Gazzolo D,Campi F,Savarese I,Dotta A,Tamborrino PP,Auriti C,Di Mambro C

更新日期：2020-11-27 00:00:00

abstract：UNLABELLED:Our aim was to study the frequency and clinical correlates of two radiographic patterns of bronchopulmonary dysplasia (BPD), the cystic BPD (cBPD) and the leaky lung syndrome (LLS). Radiographic findings of BPD from sixth day of life until term in a cohort of 82 very low birth weight (VLBW) infants were eval...

journal_title：European journal of pediatrics

authors： Hyödynmaa E,Korhonen P,Ahonen S,Luukkaala T,Tammela O

更新日期：2012-01-01 00:00:00

abstract：:A girl suffering from marked muscular hypotonia, severe statomotor and mental retardation, bilateral optic atrophy with chorioretinal degeneration, convulsions and a moderate compensated metabolic acidosis is described. Screening for metabolic disorders revealed massive 3-methylglutaconic with 3-methylglutaric aciduri...

journal_title：European journal of pediatrics

authors： Lehnert W,Scharf J,Wendel U

更新日期：1985-03-01 00:00:00

abstract：UNLABELLED:Of the many complications associated with glycogen storage disease type I, hepatic tumours cause great concern because of their malignant potential and the current difficulties in monitoring them. Hepatic adenomas occur in 22%-75% of affected adults, according to the population studied, and from those report...

journal_title：European journal of pediatrics

authors： Lee PJ

更新日期：2002-10-01 00:00:00

abstract：UNLABELLED:A total of 22 Japanese patients with hypophosphatasia were included in a study analysing the relationship between mutations in the tissue-nonspecific alkaline phosphatase (TNSALP) gene and the severity of the phenotype in Japanese patients with hypophosphatasia. The enzymatic activity of some of the identifi...

journal_title：European journal of pediatrics

authors： Michigami T,Uchihashi T,Suzuki A,Tachikawa K,Nakajima S,Ozono K

更新日期：2005-05-01 00:00:00