abstract：:A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral immunodeficiency consisted of subnormal concentration of serum IgG, in particular IgG2 and IgG4, and low concentration of serum IgM. Serum IgA and IgD were elevated, IgE was absent....

journal_title：European journal of pediatrics

authors： Haraldsson A,van der Burgt CJ,Weemaes CM,Otten B,Bakkeren JA,Stoelinga GB

更新日期：1993-06-01 00:00:00

abstract：:We describe a female infant with the Schinzel-Giedion syndrome. Features present in 11 patients include coarse face, midface retraction, urogenital anomalies, poor skull vault mineralisation and variable anomalies of the long bones. Outcome is poor and mental retardation is the rule among survivors. Prenatal diagnosis...

journal_title：European journal of pediatrics

authors： Verloes A,Moës D,Palumbo L,Elmer C,François A,Bricteux G

更新日期：1993-05-01 00:00:00

abstract：:DiGeorge syndrome is a rare congenital anomaly with a wide range of clinical manifestations. This syndrome is usually associated with hypocalcaemia resulting from primary hypoparathyroidism. We report here a case of an 8-year-old boy with partial DiGeorge syndrome who presented initially with neonatal hypocalcaemia, b...

journal_title：European journal of pediatrics

authors： Hasegawa T,Hasegawa Y,Yokoyama T,Koto S,Asamura S,Tsuchiya Y

更新日期：1993-04-01 00:00:00

abstract：:Two regimens of steroid treatment for the initial attack of idiopathic nephrotic syndrome (NS) in children were compared in a controlled prospective multi-centre study. Long prednisone therapy consisted of 60 mg/m2 per 24 h for 6 weeks, followed by alternate day 40 mg/m2 per 48 h for 6 weeks. The standard prednisone t...

journal_title：European journal of pediatrics

authors： Ehrich JH,Brodehl J

更新日期：1993-04-01 00:00:00

abstract：:Patients suffering from a mitochondrial (encephalo-)myopathy have a remarkable clinical heterogeneity. A reliable and extensive investigation must be performed in order to obtain a correct diagnosis, but many factors may influence the ultimate results of these investigations leading, under certain circumstances, to an...

journal_title：European journal of pediatrics

authors： Trijbels JM,Scholte HR,Ruitenbeek W,Sengers RC,Janssen AJ,Busch HF

更新日期：1993-03-01 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：: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

authors： Ren-Qiu Q,Ruelicke T,Hassam S,Haselbacher GK,Schoenle EJ

更新日期：1993-02-01 00:00:00

abstract：:Resting metabolic rate (RMR) and the thermic effect of a meal (TEM) were measured in a group of 26 prepubertal children divided into three groups: (1) children with both parents obese (n = 8, group OB2); (2) children with no obese parents and without familial history of obesity (n = 8, OB0); and (3) normal body weight...

journal_title：European journal of pediatrics

authors： Maffeis C,Schutz Y,Zoccante L,Pinelli L

更新日期：1993-02-01 00:00:00

abstract：:A case of painful bilateral hip effusions resolving spontaneously in the diuretic phase of steroid responsive nephrotic syndrome in a 6-year-old girl is reported. ...

journal_title：European journal of pediatrics

authors： Gupta S,Nirmal JM,Lennox CM

更新日期：1993-02-01 00:00:00

abstract：:The glycogen storage disorders (GSD)-I, -III, -VI and -VIII are associated with hypertriglyceridaemia or mixed hyperlipidaemia which poses the question whether these patients have an increased risk for atherosclerosis. The atherogenicity of triglycerides has remained controversial, while increased plasma cholesterol l...

journal_title：European journal of pediatrics

authors： Schmitz G,Hohage H,Ullrich K

更新日期：1993-01-01 00:00:00

abstract：:Chylothorax is defined as an effusion of lymph in the pleural cavity. In the neonate both congenital and traumatic (iatrogenic) forms exist. Birth asphyxia and respiratory insufficiency are major symptoms of congenital chylothorax, requiring resuscitation and artificial ventilation. Antenatal diagnosis by ultrasound a...

journal_title：European journal of pediatrics

authors： van Straaten HL,Gerards LJ,Krediet TG

更新日期：1993-01-01 00:00:00

abstract：:Staphylococcus saprophyticus commonly causes urinary tract infection (UTI) in young women; in males it is found mainly in the elderly. In this study S. saprophyticus UTI occurred in 59 children (45 girls and 14 boys) below 16 years of age, of whom 20 were less than 13 years of age. The common presentation was dysuria ...

journal_title：European journal of pediatrics

authors： Abrahamsson K,Hansson S,Jodal U,Lincoln K

更新日期：1993-01-01 00:00:00

abstract：:In 238 boys with cryptorchidism, between the ages of 4 and 48 months, luteinizing hormone releasing hormone (LHRH) was administered as nasal spray 1.2 mg/day for 4 weeks. The nonresponders received human chorionic gonadotropin (HCG) 500 I.U. i.m. three times a week for 3 weeks. With the combined treatment 37.8% of tes...

journal_title：European journal of pediatrics

authors： Lala R,Matarazzo P,Chiabotto P,de Sanctis C,Canavese F,Hadziselimovic F

更新日期：1993-01-01 00:00:00

abstract：:Male pseudohermaphroditism (MPH) is defined as incomplete masculinization in patients with normal male karyotype (XY) and testicular histology. MPH encompasses a spectrum of female to male phenotypes and presents both diagnostic and technical challenges to the surgeon. Once gender is assigned, based on phenotype, adeq...

journal_title：European journal of pediatrics

authors： Packer MG

更新日期：1993-01-01 00:00:00

abstract：:We report a prospective, non-blind, randomised, multicentre, parallel group, multinational investigation to compare ceftazidime to aminoglycoside based regimens as empirical treatment in 1316 cases of suspected sepsis in the newborn. In each of the 15 study centres either ceftazidime alone (CAZ) or ceftazidime + ampic...

journal_title：European journal of pediatrics

authors： de Louvois J,Dagan R,Tessin I

更新日期：1992-12-01 00:00:00

abstract：:Though children with Perlman and Wiedemann-Beckwith syndromes have a number of features in common, the two conditions are probably separate entities. The distinction may not always be easy, however, partly because of the extreme rarity of Perlman syndrome, only nine cases of which have been reported so far. We report ...

journal_title：European journal of pediatrics

authors： Grundy RG,Pritchard J,Baraitser M,Risdon A,Robards M

更新日期：1992-12-01 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

authors： Chan V,Greenough A

更新日期：1992-11-01 00:00:00

abstract：:The factors influencing the final height of central precocious puberty patients treated with gonadotrophin releasing hormone (GnRH) analogues remain a critical issue. This study compares the predicted final height before and after GnRH analogue therapy to identify predictive factors for final height. Fourteen girls wi...

journal_title：European journal of pediatrics

authors： Brauner R,Malandry F,Rappaport R

更新日期：1992-10-01 00:00:00

abstract：:Type I collagen is the major component of bone matrix; circulating carboxyterminal propeptide of type I procollagen (P-I-CP) levels reflect type I collagen synthesis in tissues and may be an useful index to investigate bone metabolism. We measured P-I-CP by a new radioimmunoassay in 300 healthy children and adolescent...

journal_title：European journal of pediatrics

authors： Saggese G,Bertelloni S,Baroncelli GI,Di Nero G

更新日期：1992-10-01 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：:We report two Chinese boys with Wiskott-Aldrich syndrome presenting with gastro-intestinal bleeding, eczema and recurrent infection. They had thrombocytopenia and the mean platelet volume was small. Serum IgG and IgA were elevated and lymphocyte proliferation in response to phytohaemagglutinin, concanavalin A and poke...

journal_title：European journal of pediatrics

authors： Lau YL,Jones BM,Low LC,Wong SN,Leung NK

更新日期：1992-09-01 00:00:00

abstract：:Antithyroid drugs, considered the treatment of choice for hyperthyroidism during pregnancy, may have an adverse effect on intellectual development of the offspring. We examined the intellectual capacity of 31 subjects aged 4-23 years, born to women with Graves disease who received antithyroid drugs throughout pregnanc...

journal_title：European journal of pediatrics

authors： Eisenstein Z,Weiss M,Katz Y,Bank H

更新日期：1992-08-01 00:00:00

abstract：:Surfactant-associated protein (SP-A) was measured in tracheal aspirates of ventilated infants with (n = 51) and without (n = 21) respiratory distress syndrome (RDS). SP-A concentrations in samples collected after birth were significantly lower in RDS than in infants ventilated for other reasons than RDS (median 0.03 v...

journal_title：European journal of pediatrics

authors： Stevens PA,Schadow B,Bartholain S,Segerer H,Obladen M

更新日期：1992-08-01 00:00:00

abstract：:Pseudohyperkalaemia was observed in 3 of 16 patients with Kawasaki disease showing remarkably increased platelet counts. Their plasma potassium concentration, which is not affected by in vitro coagulation, was in the normal range despite the increased serum level. A significant correlation was observed between the pla...

journal_title：European journal of pediatrics

authors： Shimizu T,Yamashiro Y,Yabuta K

更新日期：1992-07-01 00:00:00

abstract：:Since traction-associated hypertension seems to be a relatively unknown phenomenon, a survey was done of its incidence in children treated with skeletal traction for fractures and orthopaedic diseases. The correlation with hypercalcaemia, a possible aetiological factor, was also explored. Blood pressure was recorded t...

journal_title：European journal of pediatrics

authors： Heij HA,Ekkelkamp S,Vos A

更新日期：1992-07-01 00:00:00

abstract：:Protein C deficiency can lead to cerebrovascular occlusive disease. We describe a patient in whom heterozygous protein C deficiency (type 1) is suspected on the grounds of reduced protein C activity and who suffered from multiple thrombo-embolic events involving the brain and peripheral organs. The patient developed h...

journal_title：European journal of pediatrics

authors： Schmitt S,Auberger K,Fendel T,Kiess W

更新日期：1992-06-01 00:00:00

abstract：:Growth in height of 16 patients (5 boys and 11 girls) with hypophosphataemic rickets (HR) was studied in a longitudinal survey. The data shortly before and during puberty were analysed on the basis of Preece Baines curves, fitted to the original data; for the analysis at the age of 5 years, the original data were used...

journal_title：European journal of pediatrics

authors： Steendijk R,Hauspie RC

更新日期：1992-06-01 00:00:00

abstract：:In a prospective study elastase alpha 1-proteinase inhibitor (E alpha 1PI), polymorphonuclear (PMN) count, the immature to total neutrophil count ratio (I/T ratio), and C-reactive protein (CRP) were analysed in 74 patients (76 cases) with neonatal septicaemia at the time of initial clinical symptoms. At that early sta...

journal_title：European journal of pediatrics

authors： Tegtmeyer FK,Horn C,Richter A,van Wees J

更新日期：1992-05-01 00:00:00

abstract：:We studied the incidence of IgG subclass deficiency in children with recurrent bronchitis. Recurrent bronchitis was defined as three or more episodes a year during at least 2 consecutive years, of bronchopulmonary infection, productive cough with or without fever and/or diffuse râles by physical examination in the abs...

journal_title：European journal of pediatrics

authors： DeBaets F,Kint J,Pauwels R,Leroy J

更新日期：1992-04-01 00:00:00

abstract：:It is known that the 5%-10% of adults infected with hepatitis B virus (HBV) develop a chronic infection and that HBV infection acquired at birth by an hepatitis B surface antigen (HBsAg)/hepatitis B "e" antigen (HBeAg)-positive mother almost invariably leads to chronic infection. Little information is, however, availa...

journal_title：European journal of pediatrics

authors： Vegnente A,Iorio R,Guida S,Cimmino L

更新日期：1992-03-01 00:00:00

abstract：:We describe a patient with leucocyte adhesion deficiency (LAD). Clinically, the patient had delayed umbilical cord detachment, omphalitis, impaired wound healing and persistent leucocytosis. The patient had the severe form of LAD, with a total absence of leucocyte cell adhesion molecules (LeuCAMs) and undetectable mRN...

journal_title：European journal of pediatrics

authors： Weening RS,Bredius RG,Vomberg PP,van der Schoot CE,Hoogerwerf M,Roos D

更新日期：1992-02-01 00:00:00

abstract：:Four months after termination of successful chemotherapy for epipharyngeal B-non-Hodgkin lymphoma, an enlarging anterior mediastinal mass was discovered in a 15-year-old boy. There was no other suspicion of tumour recurrence. A simple thymic rebound was likely and a conservative management was chosen. Follow up for mo...

journal_title：European journal of pediatrics

authors： Leibundgut K,Willi U,Plüss HJ

更新日期：1992-02-01 00:00:00

abstract：:Recent data indicate that insulin-like growth factor II (IGF II) and lysosomal enzymes bind to a common receptor. We measured serum IGF I and II levels in 16 patients with various lysosomal storage disorders. The IGF serum concentrations were normal as long as no marked liver disease was present. Under these condition...

journal_title：European journal of pediatrics

authors： Jaeggi-Groisman SE,Kiess W,Christomanou H,Kessler U,Froesch ER

更新日期：1992-01-01 00:00:00

abstract：:A 10-year-old boy with chyluria due to a congenital fistulous communication between the lymphatic system and the bladder is described. Chyluria can be parasitic or non-parasitic. Many causes of non-parasitic chyluria have been reported. Lymphography is the preoperative imaging procedure of choice since it demonstrates...

journal_title：European journal of pediatrics

authors： Stalens JP,Falk M,Howmann-Giles R,Roy LP

更新日期：1992-01-01 00:00:00

abstract：:There is far-reaching consent in the literature that public and consensual agreements on the basics of the different aspects of medical ethics are inalienable before the instigation of any innovative transplant procedure. In the case of a certain method and/or of an individual patient, however, the ultimate ethical ev...

journal_title：European journal of pediatrics

authors： Wolff G

更新日期：1992-01-01 00:00:00

abstract：:Twelve preterm infants, median gestational age 31.5 weeks, were entered into a randomised, placebo-controlled trial of bronchodilator therapy. Their postnatal age was a median of 17.5 months and all suffered from recurrent respiratory symptoms. The infants received either inhaled placebo or 40 micrograms of ipratropiu...

journal_title：European journal of pediatrics

authors： Yuksel B,Greenough A

更新日期：1991-10-01 00:00:00

abstract：:A case-control study was conducted to examine the association of beta-haemolytic non-group A streptococci and pharyngitis in a paediatric population (n = 502). No association could be found between disease and traditional Lancefield groups B, C, F, or G. When the analysis included the clustering of genetically related...

journal_title：European journal of pediatrics

authors： Cimolai N,Morrison BJ,MacCulloch L,Smith DF,Hlady J

更新日期：1991-09-01 00:00:00

abstract：:Regional variations in the human leucocyte antigen (HLA) distribution patterns of coeliac disease (CD) have been reported. This study focuses on phenotype frequencies of a cohort of Austrian paediatric CD patients in comparison with those recorded in the literature. HLA class I and II typing was performed in 136 CD pa...

journal_title：European journal of pediatrics

authors： Winklhofer-Roob BM,Rossipal E,Lanzer G

更新日期：1991-08-01 00:00:00

abstract：:The peak flow velocities in the middle cerebral artery were continuously recorded by transcranial Doppler sonography in 29 children. Arterial blood pressure and heart rates were measured every minute. The values observed during orthostasis and physical exercise were compared to baseline values obtained in the supine p...

journal_title：European journal of pediatrics

authors： Bode H

更新日期：1991-08-01 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