bluuebunny/crossref_metadata_2025 huggingface dataset:
Neuromuscular Involvement in Glycogen Storage Disease Type III
Authors: MOSES S. W. , GADOTH N. , BASHAN N. , BEN‐DAVID E. , SLONIM A. , WANDERMAN K. L.
Published: January 2008

ABSTRACT. Sixteen patients with glycogen storage disease type III (GSD III) aged 3 to 22 years underwent a detailed neuromuscular evaluation. A minimal impairment of skeletal muscle function was presented in eight patients, slight impairment in four and severe impairment in one patient. Serum creatinine phosphokinase (CPK) was elevated in all patients studied. In the nine patients, in whom electromyography (EMG) was performed; six exhibited a myopathic pattern while a “mixed” (neurogenic‐myopathic) pattern was present in three. Muscle biopsies performed in 12 patients, revealed in all cases amylo‐1,6,‐glucosidase deficiency and biochemical as well as morphological evidence of glycogen accumulation. Two brothers suffered from late onset myopathy, which in the older sibling was associated with clinical, EMG and EM findings of a peripheral neuropathy. Fifteen patients had either electrocardiographic and or echographic evidence of cardiomyopathy. Observations based on this patient material suggest a widespread myopathy in GSD III patients with heterogeneous expression, while peripheral nerve involvement is rarely encountered.
Severe Pancreatitis and Fatty Liver Progressing to Cirrhosis Associated with Coxsackie B4 Infection in a Three Year Old with Alpha‐1‐Antitrypsin Deficiency
Authors: KENNEDY J. D. , TALBOT I. C. , TANNER M. S.
Published: January 2008

ABSTRACT. A 3‐year‐old girl in whom severe acute pancreatitis was associated with evidence of Coxsackie B4 virus infection was alpha‐1‐antitrypsin deficient. Lack of this modulator of proteolysis may have been responsible for her severe course. Fatty liver at presentation progressed to cirrhosis in the ensuing 18 months.
Congenital Toxoplasmosis after Maternal Infection before or Slightly after Conception
Authors: HAENTJENS M. , SACRÉ L. , DEMEUTER F.
Published: January 2008

ABSTRACT. We report a newborn with typical lesions of congenital toxoplasmosis. Retrospectively, the time of infection could be located to or even before conception. A diagnostic work‐up should be performed in every newborn whose mother presents persistent high antibody titers against toxoplasma during pregnancy.
A Comparative Study of Size and Function of the Remnant Kidney in Patients Nephrectomized in Childhood for Wilms’Tumor and Hydronephrosis
Authors: WIKSTAD I. , PETTERSSON B. A. , ELINDER G. , SOKUCU S. , APERIA A.
Published: January 2008

ABSTRACT. To obtain more information about the natural history of compensatory renal hypertrophy beginning in childhood we traced those who were nephrectomized in childhood for Wilm's tumor (W) and hydronephrosis (Hn) between 1950 and 1978 at one department of surgery in Stockholm. AH W patients had received treatment that suppresses cellular division. None of the patients were in renal failure or treated with antihypertensive drugs. All the patients in the follow‐up study (22 W, 15 Hn) had a normal contralateral kidney at nephrectomy. Five healthy adults served as controls. The kidney was enlarged in both Hn (142 %) and W (125 %), but significantly larger in Hn than in W. Renal compensatory growth in W was retarded during the first two years after nephrectomy. The glomerular filtration rate (GFR) was 92 % of control in Hn and 82 % of control in W. The GFR did not seem to decline with a longer follow‐up time in any of the groups. PAH clearance was the same in Hn and W. Albumin excretion was significantly higher in Hn than in W, but not significantly higher in W than in controls. The highest albumin excretion rates were found among the Hn patients with long follow‐up time. The results suggest that the large increases in size and function that follow childhood nephrectomy can be blunted by antimitotic agents.
Alterations of Urinary Carbon Dioxide Tension, Electrolyte Handling and Low Molecular Weight Protein Excretion in Acute Pyelonephritis
Authors: TULASSAY T. , MILTÉNYI M. , DOBOS M.
Published: January 2008

ABSTRACT. Renal tubular function tests were performed in 45 children suffering from upper and lower urinary tract infections. Determinations were made of the urinary carbon dioxide tension in maximally alkaline urine as an index of distal tubular H+ ‐ion secretion, of urinary protein excretion, and of urinary sodium and phosphate handling. Urinary Pco2 was low (2.7±13.9 mmHg) in acute pyelonephritis compared to values in healthy children (52 ±32 mmHg) or those with cystitis (48±34 mmHg). At the onset of pyelonephritis an elevated fractional excretion of sodium (1.38±0.38 vs. 0.50±0.20%) and decreased phosphate reabsorption (69.2±7.1 vs. 90.4±4.9%) were also observed. Significantly elevated urinary low molecular weight protein excretion was also found in pyelonephritis. These data indicate the existence of proximal and distal tubular dysfunction at the onset of acute bacterial pyelonephritis.
Periventricular Leukomalacia:
Authors: CALVERT S. A. , HOSKINS E. M. , FONG K. W. , FORSYTH S. C.
Published: January 2008

ABSTRACT. The ultrasonographic findings and neurological development of 15 preterm infants, born between August 1981 and July 1984, who developed periventricular leukomalacia (P.V.L.) are described. The incidence of P.V.L. in infants with a birth weight less than 1500 g was 2.3 %. Nine of the 15 infants demonstrated areas of increased echogenicity in the periventricular regions prior to the development of cystic changes. The mean age for the appearance of cysts was 26 days. At follow‐up 14 infants showed evidence of abnormal motor development and 8 infants had visual defects. All infants appear to have normal hearing, but 3 infants show signs of delayed speech. To date, only 2 infants demonstrate signs of mental retardation, but many of the infants are too young to assess intelligence accurately.
Diagnosis of Cystic Fibrosis, by Assay of Immuno‐Reactive Trypsin in a Newborn Infant with Jejuno‐lleal Atresias
Authors: RAFFLES A. K. M. , SINGH P. , MIALL‐ALLEN V.
Published: January 2008

ABSTRACT. A newborn infant with ante‐natally ultrasonically diagnosed small bowel obstruction was found to have two isolated jejunal atresias at laparotomy. Assay of immuno‐reactive trypsin enabled an early diagnosis of cystic fibrosis to be made, later confirmed by an elevated sweat sodium.
Echocardiographic Measurements in Normal Preterm and Term Neonates
Authors: WALTHER F. J. , SIASSI B. , KING J. , WU P. Y. K.
Published: January 2008

ABSTRACT. To the purpose of better defining normative data on intracardiac dimensions and systolic time intervals in very low birth weight infants, we collected M‐mode echocardiograms from 210 healthy preterm and term neonates with birth weights between 780 and 5350 g and gestational ages ranging from 26 to 43 weeks. Fifty‐nine neonates were less than 24 h, 62 were 25‐48 h, and 89 were 48‐144 h of age. Diastolic and systolic left ventricular dimensions increased gradually with advancing birth weight (r=+0.84 and 0.78). Left atrial and aortic root dimensions tended to show a parabolic relationship with birth weight, increments were reduced at higher birth weights (r=+0.92 and 0.85). The shortening fraction of the left ventricle (mean + SD 33.8 ± 4.9%) and the left atrial/aortic ratio (1.16 ± 0.10) were constant throughout all weight subgroups. Pre‐ejection periods and ejection times of both ventricles were reduced in preterm infants due to their higher heart rates, but left and right ventricular PEP/ ET ratios in preterm and term infants were comparable. Septal thickness in diastole and in systole tended to increase slowly with advancing birth weight, but correlation coefficients were low. This information is currently used as data base for a computer program to interpret M‐mode echocardiograms performed in our nursery.
Vaccination of Children during Treatment for Leukemia
Authors: RAUTONEN J. , SIIMES M. A. , LUNDSTRÖM U. , PETTAY O. , LANNING M. , SALMI T. T. , PENTTINEN K. , KÄYHTY H. , LEINONEN M. , MÄKELÄ P. H.
Published: January 2008

ABSTRACT. Forty‐one children receiving maintenance treatment for leukemia were vaccinated with inactivated mumps virus, meningococcal polysaccharide groups A and C and polyvalent pneumococcal polysaccharide vaccines. Antibodies against pneumococcal types 3,18C, 19F and 23F and against meningococcal groups A and C were measured by radioimmunoassay. Mumps antibodies were determined by complement fixation, hemolysis‐in‐gel and enzyme‐linked immunosorbent assays. The antibody responses tended to be lower than those of healthy age‐matched controls. The individual responses were unpredictable; the magnitude of the response correlated neither with the age nor sex of the patient, duration of the chemotherapy nor with the prevaccination antibody concentration. Nevertheless, most patients achieved and even exceeded the geometric mean of the antibody concentrations of the healthy children, considered protective against infection.
A Prospective Comparative Study of the Aetiology of Cerebral Palsy and Epilepsy in a One‐Year Birth Cohort from Northern Finland
Authors: RANTAKALLIO P. , WENDT L. VON
Published: January 2008

ABSTRACT. The one‐year birth cohort from Northern Finland comprises 12058 children, representing 96% of all live births in 1966 in the two northernmost provinces of Finland, Oulu and Lapland. Only 14 children, 1.2 per thousand, were lost during the follow‐up to 14 years of age, in which data were collected prospectively on development, mortality and morbidity using questionnaires and registers. When all 47 children with cerebral palsy fulfilling the criteria of the Little Club of London (1959) were included, a prenatal risk factor was detected in 13 infants (27.7%) and a perinatal one in 25 children (53.2%), whereas 9 children (19.1%) represented an untraceable form of this disorder. There were 208 children with epilepsy, among which 18 (8.7%) had a prenatal risk factor, 38 (18.2%) a perinatal one, 33 (15.9%) a postnatal one, and 119 (57.2%) no identifiable risk factor. Among the 11320 healthy children in the cohort alive at 14 years of age, there were 992 (8.8%) with risk factors for cerebral palsy and epilepsy. The relative risk attached to these aetiological factors was lower for epilepsy than for cerebral palsy.
Precocious Puberty Associated with Oral‐Facial‐Digital Syndrome Type I
Authors: SOMER M. , LINDAHL E. , PERHEENTUPA J.
Published: January 2008

ABSTRACT. A girl with the oral‐facial‐digital syndrome type I (OFD I) developed precocious puberty at early infancy. This is presumed to be due to a hamartoma in the tuber cinereum region. Hypothalamic hamartomas have not been described in OFD I earlier, wheras lingual hamartomas are a common feature in this condition.
Anti‐inflammatory Properties of Human Milk
Authors: GOLDMAN A. S. , THORPE L. W. , GOLDBLUM R. M. , HANSON L. A.
Published: January 2008

ABSTRACT.An hypothesis was developed which predicts that human milk protects against infections of the alimentary tract of the breast‐fed infant by non‐inflammatory mechanisms. Human milk is poor in the initiators and mediators of inflammation and rich in anti‐inflammatory agents. Furthermore, many of the anti‐inflammatory agents are comparatively resistant to digestive enzymes and therefore might be expected to remain active in the gastrointestinal tract of the recipient. Further studies of these factors in in vivo models will be required to validate the hypothesis.
Neutralizing Activity in Human Milk Fractions against Respiratory Syncytial Virus
Authors: LÆGREID A. , OTNÆSS A.‐B. KOLSTø , ØRSTAVIK I. , CARLSEN K. H.
Published: January 2008

ABSTRACT. Neutralizing activity against respiratory syncytial virus (RSY) was measured in milk samples from 17 healthy women whose infants had an acute infection with respiratory syncytial virus (RSV) and from 27 women with healthy infants. All milk samples were obtained 2‐8 months post partum. Neutralizing activity was detected in 36 samples. No major difference in neutralizing titers was observed between the two groups, and the titers were low. RSV‐specific IgA was found in two samples, and RSV‐specific IgG in one sample. RSV‐specific IgM was not detected. In gel filtration studies, the neutralizing activity was eluted with an apparent molecular weight above 400 000. The neutralizing activity remained after removal of IgA by affinity chromatography. These findings suggest that both immunoglobulin and non‐im‐munoglobulin components in human milk can neutralize RSV.
Observations Questioning a Protective Role for Breast‐feeding in Severe Rotavirus Diarrhea
Authors: GLASS ROGER I. , STOLL BARBARA J. , WYATT RICHARD G. , HOSHINO YASUTAKA , BANU HASINA , KAPIKIAN ALBERT Z.
Published: January 2008

ABSTRACT. To investigate whether breast‐feeding protects children against rotavirus diarrhea (RVD), we compared rates of breast‐feeding by age and enteric pathogens among 2 276 children with diarrhea 0‐4 years of age who attended a diarrhea hospital in Bangladesh. Infants 0‐5 months were less likely to be breast‐fed than children 6‐11 months of age suggesting that some protection against diarrhea with all agents was associated with early breast‐feeding. In every age group studied, breast‐feeding was more common among children with RVD than among children with non‐RYD whereas it was less common among children with cholera and shigellosis. Twenty percent of breast milks consumed by infants less than 1 year of age had high levels of neutralizing activity (>320) to the Wa strain of rotavirus but this activity did not appear to be protective since the 30 infants with RVD consumed milk which had titers that did not differ significantly from those consumed by 44 infants with diarrhea of other cause. Despite the prolonged breast‐feeding which is common in Bangladesh, the mean age of hospitalization with RYD is approximately the same as in countries where the duration of breastfeeding is quite short. None of these 3 independent observations support a protective role for breast‐feeding against rotavirus diarrhea after the first months of life.
Growth Response to Human Growth Hormone Treatment in Children with Partial and Total Growth Hormone Deficiency
Authors: WIT J. M. , FABER J. A. J. , BRANDE J. L. VAN DEN
Published: January 2008

ABSTRACT. The growth response during the first and second years of human growth hormone (hGH) treatment was studied in 14 prepubertal children with so‐called “partial” GH deficiency (peak GH between 8 and 15 mU/1) and compared to 28 prepubertal children with “total” GH deficiency (peak GH less than 8 mU/1). There was no difference in growth acceleration between children with partial and total GH deficiency, when initial covariables were taken into account. In a stepwise multiple regression analysis initial stature, pre‐treatment growth velocity and skinfold thickness were shown to be most related to growth response, but after exclusion of 3 children with a genetic form of total GH deficiency and partial TSH deficiency this relationship was lost. GH levels during provocation tests and auxological criteria have a poor predictive value for growth response to hGH therapy.
Proportionality Changes in the First Year of Life; the Influence of Weight for Gestational Age at Birth
Authors: OUNSTED M. , MOAR V. A.
Published: January 2008

ABSTRACT. Data on 55 small‐for‐gestational age (SGA), 56 average‐for‐gestational age (AGA) and 55 large‐for‐gestational age (LGA) infants whose growth had been monitored regularly from birth to 12 months are presented. SGA head‐chest ratios were high at birth; but by 6 months they were very similar to AGA ratios, with values just below unity. Head‐length ratios showed a steady decrement; SGA infants having the highest and LGA infants the lowest values at all ages. Chest‐length ratios showed little change up to 6 months, with a steady decrease thereafter. Stem‐stature ratios showed a similar pattern; and were confined within a narrow margin from 6 months onwards. These changes seem to be target orientated. We suggest the goal is that proportionality which enables the infant to attain the upright stature; and thus to sit, stand and walk at the appropriate time.
Long‐term Follow‐up of Ventilator Treated Low Birth weight Infants
Authors: LINDROTH M. , MORTENSSON W.
Published: January 2008

ABSTRACT. Chest X‐ray, pulmonary mechanics, clinical lung disease and growth were studied in 48 low birthweight infants surviving after ventilator treatment in the neonatal period. Bronchopulmonary dysplasia (BPD) was present in 14 infants shortly after weaning off ventilator. At 4 to 6 years of age most patients had normal chest radiographs but 13 still showed signs of pulmonary fibrosis and hyperinflation. Most patients had low dynamic compliance and high pulmonary resistance shortly after ventilator treatment. All but 8, however, had normal findings at 1 to 11/2 years of age. Pneumonias and bronchitis were common during the first two years but thereafter declined in frequency. Weight and length development were retarded for BPD patients during the first two years and for non‐BPD patients for the first year. Both groups had a complete catch‐up.
Epidemiological Analysis of the Increased Risk of Disturbed Neonatal Respiratory Adaptation after Caesarean Section
Authors: KRANTZ M. E. , WENNERGREN M. , BENGTSON L. G. W. , HJALMARSON O. , KARLSSON K. , SELLGREN U.
Published: January 2008

ABSTRACT. In a prospective, unselected study of all 4659 infants born in Göteborg, Sweden, risk factors for all kinds of neonatal respiratory disturbances (RD) after Caesarean section (CS) were analyzed. After CS, a significantly increased incidence rate of RD was found compared to vaginal delivery (24.6% vs. 5.5%). The increased overall risk affected full term infants only but IRDS was more common after CS in preterm infants. Rupture of membranes or uterine contractions prior to CS significantly reduced the incidence rate of RD in full term infants. Acute maternal complications did not affect the incidence. The elevated RD rate could partly be related to an increased incidence of low Apgar score after CS, and to absence of labour and rupture of membranes before the CS. But full term infants with Apgar score of 7 or more, delivered surgically after rupture of membranes and start of contractions, still had almost three times higher incidence of RD.
C‐reactive Protein–an Indicator of Generalized Bacterial Infection in Children with Nephrotic Syndrome
Authors: ESKOLA J. , HOLMBERG C. , KOSKIMIES O.
Published: January 2008

ABSTRACT. The usefulness of C‐reactive protein for detecting bacterial infections in children with nephrotic syndrome was studied in 12 children who had a total of 29 episodes of infection. Four episodes were septic, with CRP values of 127‐270 mg/1 and 25 superficial or focal with all CRP values below 47 mg/1; 13 were below 10 mg/1. CRP thus discriminated the invasive bacterial diseases from other infections. It was not affected by the nephrotic syndrome activity.
Cerebrospinal Fluid Glutamine in Intracranial Hemorrhage in the Newborn
Authors: ANDERSEN ERIK ARTHUR , BUCHER DITLEF
Published: January 2008

ABSTRACT. Cerebrospinal fluid concentration of glutamine was measured in 43 premature and mature babies suffering from intracranial hemorrhage. Compared with a control group of 38 infants we found elevated levels in all infants with intraparenchymal and subarachnoid hemorrhages. Two out of ten children with subependymal hemorrhages and approximately half of the children with intraventricular hemorrhages also had elevated levels. Elevated levels of cerebrospinal fluid glutamine persisted for up to three weeks and maximum levels were reached two days to two weeks after birth. The analysis of cerebrospinal fluid glutamine concentration might be useful in the early diagnosis of intraparenchymal and subarachnoid hemorrhage in the newborn infant.
Continuous Wave Ultrasound Measure of Neonatal Cerebral Blood Flow
Authors: ELLISON P. , HORN J. , BROWN P. , DENNY J.
Published: January 2008

ABSTRACT. We report cerebral blood flow (CBF) values for healthy neonates in five gestational age categories for 9 single and 7 combination measures of continuous wave Doppler ultrasound. Combinations of measures, particularly those from the anterior cerebral artery with a 5.0 MgH probe, increased the reliability and validity. The most reliable and valid single measure was mean flow velocity from the anterior cerebral artery (5.0 MgH probe). CBF values were lower for neonates with respirator support than for neonates without respirator support. No significant differences were found in CBF measures between intubated neonates who developed IVH and intubated neonates who did not develop IVH. Neonates who were intubated and developed intraventricular hemorrhage (IVH) were treated more vigorously than those who were intubated and did not develop IVH. By day 6, neonates with IVH had a significantly higher mean pH, mean serum bicarbonate, and mean positive base balance.
Free Urinary Cortisol Immunoreactive Levels in Premature and Full Term Infants
Authors: SIAMOPOULOU‐MAVRIDOU A. , MAVRIDIS A. K. , VIZANDIADIS A. , HARSOULIS P.
Published: January 2008

ABSTRACT. Urinary free immunoreactive Cortisol excretion was measured in 20 full term, 20 premature and 20 premature newborns of mothers who had been treated with 12 mg betamethasone 48 hours before delivery. In 10 full term newborns delivered normally, values were 40 ±20 nmol/mmol creatinine on the first, 23±8 on the second and 21±6 on the third day of life. In 10 full term newborns with stressful delivery, the corresponding values were 63±39, 44±33 and 32±17 nmol/mmol creatinine in the first three days of life. The levels of urinary free immunoreactive Cortisol of 10 premature newborns delivered without stress were 170±116, 91±75 and 70±61 nmol/mmol creatinine respectively, on days one, two and three of life. Ten premature infants with respiratory distress syndrome had values of 471 ± 187, 526±465 and 636±906 nmol/mmol creatinine, respectively. The 10 premature newborns whose mothers had received betamethasone, had urinary free immunoreactive Cortisol levels of 109±120, 55±42 and 66±84 nmol/mmol creatinine, lower than the other premature infants. This difference, however, was not statistically significant. We conclude that premature infants regardless of stress or normal labor have high urinary free immunoreactive Cortisol excretion, suggesting that prematurity per se is a potent stress.
Rickets in Very Low Birthweight Infants
Authors: LINDROTH M. , WESTGREN U. , LAURIN S.
Published: January 2008

ABSTRACT. The influence of extra phosphorus (P) and calcium (Ca) on the incidence of rickets was studied in 40 infants with a birthweight below 1.5 kg. All were fed breastmilk and all received vitamin D 1200 IU/day. Half of the infants were supplemented with P 20 mg/kg/day and Ca 30 mg/kg/day. Rickets, diagnosed with X‐ray at 5 to 7 weeks of age, developed in 12 infants, 11 of whom weighed below 1.0 kg. In infants below 1.0 kg rickets was significantly more common in the group not receiving extra P and Ca. After diagnosis all were substituted and radiographic healing occurred in all. Serum Ca concentrations were normal in both groups whereas serum P was significantly lower in non‐supplemented patients. Serum alkaline phosphatases (ALP) were normal in all patients at the time of diagnosis demonstrating the risk of using ALP as a diagnostic test for rickets in very low birthweight.
Epidemiology of Nocturnal Enuresis in a Part of Khartoum, Sudan
Authors: CEDERBLAD M. , RAHIM S. I. A.
Published: January 2008

ABSTRACT. An intensive study of enuresis nocturna was made on 245 children selected through stratified sampling from a population of 8462 children. Subjects were 3‐15 years and living in a suburban part of Khartoum, Sudan. The prevalence of enuresis noctuma had been found to be high up to the age of ten in the previous extensive study. Few somatic, developmental or behavioural factors, and no family socio‐economic or emotional states or child rearing methods including bladder‐training, co‐varied with enuresis. Climatic conditions and their interaction with possible developmental lags are discussed.
Clinical Experience with Somatrem in Japan
Authors: TAKANO KAZUE , SHIZUME KAZUO
Published: January 2008

ABSTRACT. A total of 62 patients with pituitary dwarfism were treated with three different preparations of human growth hormone (hGH) produced by recombinant DNA technology (somatrem). They were given somatrem, 0.5 IU/kg body weight/week for 3–14 months. Their height velocity increased from 3.5 ± 0.9 to 8.2 ± 1.7 cm/year (mean ± SEM) during treatment. There were no significant changes in physical, blood and urine examinations. Anti‐hGH antibody was observed in 39 of 62 patients (62.9%) at the end of 3 months of treatment with three different preparations of somatrem and in 16 of 21 patients (76.2%) at the end of 12 months of treatment with highly purified Somatonorm. The presence of antibody to hGH did not affect the growth rate in 48 of 49 hGH deficient children who had measurable antibody.
Clinical Experience of Somatrem: UK Preliminary Report
Authors: MILNER R.D.G.
Published: January 2008

ABSTRACT. A total of 63 patients with hGH deficiency were recruited, of whom 59 are evaluable. Somatrem (Somatonorm), 4 IU three times/week, was given either subcutaneously or intramuscularly. Height velocity increased from a mean of 4.7 ± 2.1 cm/year before therapy to 8.2 ± 2.6 cm/year in the 15 patients who have been followed for 1 year. In patients with isolated hGH deficiency height velocity increased similarly to the whole group, but in those with multiple pituitary hormone deficiencies the pretreatment and on‐treatment growth velocities were lower. Of four children who had previously received spinal irradiation, three experienced only a small increase in height velocity on Somatonorm treatment; the fourth showed a considerable increase in height velocity. Anti‐hGH antibodies were present in about 80% of the children tested after 12 months of treatment, but the titres and binding capacities were low. Anti‐ECP antibodies were also detected, but again the titres were low, and 39% of the children tested had anti‐ECP antibodies before treatment commenced.
Clinical Experience with Somatonorm
Authors: GIRARD F. , GOURMELEN M.
Published: January 2008

ABSTRACT. A total of 21 patients with hGH deficiency was included in a double‐blind, randomized, comparative multicentre trial of somatrem (Somatonorm) and pituitary hGH. Owing to the withdrawal of the latter product, only 6 months follow‐up can be evaluated. Growth velocities on both preparations were similar, and patients with complete hGH deficiency responded better than those with only partial deficiency. No specific side‐effects were noted, but antibodies to hGH were noted in two patients on Somatonorm. Antibodies to Escherichia coli polypeptide (ECP) were found in both groups. In an open study of 14 children treated with Somatonorm, growth velocity increased from a mean of 3.26 ± 0.42 cm/year to 7.1 ± 0.47 cm/year during the 1 year of follow‐up; in this time, bone age increased by 10 months. No clinical or biological abnormalities were detected, though 50% of the children developed anti‐hGH antibodies and 13 out of 14 developed Z scores greater than 0.1 for ECP antibodies, compared with 5 out of 14 before treatment.
Experiences of Somatonorm in Sweden
Authors: WESTPHAL OTTO
Published: January 2008

ABSTRACT. About 180 patients in Sweden are currently receiving treatment with somatrem; a majority of them have previously received pituitary hGH. A high incidence of antibody formation was noted in patients without prior pituitary hGH treatment, but growth velocity was not decreased in these patients. Antibodies developed in about 15% of patients previously treated with pituitary hGH. Adverse effects occurred in four patients within 3 weeks of commencing therapy, but were temporary and treatment could be continued.
Growth Hormone Treatment in Turner's Syndrome
Authors: TAKANO KAZUE , HIZUKA NAOMI , SHIZUME KAZUO
Published: January 2008

ABSTRACT. A total of 21 patients with Turner's syndrome were treated with pituitary hGH and/or somatrem for 1–2 years. Plasma non‐esterified fatty acid increased significantly from 0.52 ± 0.06 to 1.30 ± 0.09 mEq/litre at 4 hours after injection of hGH, 4 IU (mean ± SEM, p < 0.001). Basal plasma IGF‐1 levels were within the normal range; however, they increased significantly at 24 hours after the first three daily injections of hGH, 4 IU (basal, 0.92 ± 0.14 units/ml; 24 hours, 1.39 ± 0.16 units/ml; 48 hours, 1.68 ± 0.19 units/ml; 72 hours, 1.91 ± 0.22 units/ml; p < 0.001). For long‐term treatment, patients were given hGH, 4–16 IU for 1–2 years. Their height velocity increased to 5.5 ± 1.2 cm/year and 5.1 ± 0.6 cm/year in the first and second year of the treatment, respectively. These values were greater than the pretreatment value of 3.6 ± 0.8 cm/year (p < 0.001). Antibody against hGH was observed in 60% of the patients at the end of 12 months of somatrem treatment. Otherwise there were no significant changes in physical, blood or urine examinations. These results indicate that hGH treatment is useful for the acceleration of growth velocity in patients with Turner's syndrome.
Clinical Experience with GRF
Authors: B.RANKE MICHAEL
Published: January 2008

ABSTRACT. Growth hormone releasing factor (GRF) is one of two main releasing hormones which control pituitary hGH production and release. This activity is retained even when the length of the GRF polypeptide is reduced to the 29 N‐terminal residues. This 29‐residue polypeptide has been chemically synthesized, and testing in healthy volunteers elicited secretion of hGH to a maximum level of about 40 ng/ml. In a trial of children with various disorders, the maximum hGH response was reached within 60 minutes; those children with hGH deficiency showed much lower responses than those with other disorders not related to hGH deficiency. The frequency of false‐positive tests was low, and it is proposed that GRF testing could provide a useful additional tool in the diagnosis of hGH deficiency.
Diagnosis and Assessment of Valve Stenosis and Regurgitation and Coarctation of the Aorta with Doppler Ultrasound
Authors: HATLE L.
Published: January 2008

ABSTRACT. With the use of Doppler ultrasound localized increases in blood flow velocities can be recorded and used to diagnose obstructions to blood flow. From the increase in maximal velocities the pressure drop across an obstruction can be calculated, both the peak instantaneous and the mean pressure drop. Regurgitations are diagnosed by recording reversal of blood flow across the valve. Semi‐quantitative evaluation of the degree of regurgitation can be made by using both jet width, extension and intensity, as well as increase in forward flow velocity, reversal of flow in great vessels and influence on pressures. In coarctation of the aorta localized increase in velocity in the descending aorta can be shown and the pressure drop can be calculated. In some, more than one level of obstruction can be shown. In neonates the presence of a patent ductus arteriosus may mask the obstruction and a significant pressure drop may become apparent only when narrowing or closure of the duct occurs.
Pulsed Doppler Echocardiography in Patients with Aorto‐pulmonary Connection
Authors: DANIËLS O. , HOPMAN J. , KNECHT S. DE , OORT A. VAN , BUSCH H.
Published: January 2008

ABSTRACT. The pulsed Doppler principle enables us to study the bloodflow velocity at specific points in the heart and great vessels. In such aorto‐pulmonary connections as patent dnctus arteriosus, aorto‐pulmonary window and truncus arteriosus the bloodflow velocity patterns are typical of the malformation. Based on knowledge of these abnormal flow patterns the diagnoses of these malformations can be made.
Doppler Evaluation of Surgically Repaired Congenital Heart Disease
Authors: STEVENSON J. GEOFFREY
Published: January 2008

ABSTRACT. Surgical repair of a wide variety of congenital heart defects has become commonplace. Evaluation of the adequacy of the surgical repair, and the effects of growth and time upon these repairs has traditionally been through clinical evaluation or invasive study; each method has limitations. Doppler echocardiography provides an accurate and fully noninvasive means for initial evaluation at the time of surgery, and for serial follow‐up, without the risk or cost of invasive study. Applications and experience with Doppler evaluation of a variety of surgically repaired defects are discussed. For most lesions, Doppler echocardiography is the procedure of choice for post‐operative evaluation.
Doppler Pressure Gradient Estimation in Children Accuracy, Effect of Activity and Exercise, and the Need for Sedation during Examination
Authors: STEVENSON J. GEOFFREY , KAWABORI ISAMU , FRENCH JAMES W.
Published: January 2008

ABSTRACT. Noninvasive estimation of pressure drop has been shown to be an accurate and useful application of Doppler echocardiography. Most accuracy series have used patient‐sedated catherization gradient measurements as the standard of reference. Doppler gradient estimates, however, are commonly made without sedation of the patient when hemodynamics may differ from those present at catheterization. We questioned whether (1) “unsedated” Doppler gradient estimates would correlate in a useful way with “sedated” catheter measurements, (2) Doppler gradient estimates would vary with patient activity, exercise or crying, and (3) Doppler gradient estimates made using chloral hydrate sedation would prove useful in predicting sedated catheter measurements. Twenty‐five infants and children were examined under conditions 1 and 2 above, and 20 under condition 3. Sedated Doppler estimates, performed at the time of catheterization corresponded closely with catheter measurements (r=0.97, SEE=4.8), confirming the accuracy of the Doppler method. Gradients estimated by Doppler without sedation, or with activity, correlated poorly with sedated catheter measurements (SEE=16.2, SEE=34.9, respectively). Use of chloral hydrate sedation for Doppler estimates resulted in good correlation with subsequent sedated catheter measurements. The results demonstrate marked increases in noninvasive Doppler pressure gradient estimates under conditions other than sedation. Clinicians tend to think in terms of sedated catheter‐gradients as the standard of reference for evaluation of severity and need for surgery. Ultrasonic data can only be used if Doppler estimates are performed under similar physiologic conditions; in children this requires sedation.
Cuff Pressures and Doppler Gradients after Coarctectomy A Long‐term Follow‐up
Authors: MICHAELSSON M. , SUNNEGARDH J. , HALLBERG M. , ÅBERG T.
Published: January 2008

ABSTRACT. Fourty‐six adult patients operated on as chidren with resection and end to end anastomosis because of coarctation of the aorta were studied. The age at operation was 7 to 13 years (mean age 10 years) and at follow‐up 18 to 28 years (mean age 21 years). Arm and thigh cuff pressure was measured at rest, during and immediately after submaximal exercise. The systolic gradients were also estimated using continuous wave Doppler. Systolic blood pressure at rest was 150 mmHg or more in 12 patients (26%). The cuff pressure at rest correlated well with arm‐leg gradients at rest and at work, and also with the Doppler gradients at rest. Doppler gradients slightly underestimated invasively measured gradients. The results imply that postoperative hypertension was explained by residual mechanical obstruction.
Intrathecal Immunoglobulin Production and Minor Motor Seizures
Authors: BENSON M. , BLENNOW G. , ROSÉN I.
Published: January 2008

ABSTRACT. Five infants with idiopathic infantile minor motor seizures had an elevated immunoglobulin G (IgG) index ((cerebrospinal fluid IgG/serum IgG): (cerebrospinal fluid albumin/serum albumin)). The infants had prolonged prodromal symptoms, and bad prognosis. It is suggested that immunological mechanisms may contribute to the pathophysiology in certain cases of infantile spasms.
Late Onset Coeliac Disease in the Monozygotic Twin of a Coeliac Child
Authors: DE SOUSA J. SALAZAR , DE ALMEIDA J. M. RAMOS , MONTEIRO M. V. , RAMALHO P. MAGALHÃES
Published: January 2008

ABSTRACT. A case of late onset coeliac disease (confirmed by serial intestinal biopsies) is described in the monozygotic twin of a coeliac patient. This is the second case reported in literature. The permanent discordance for coeliac disease in monozygotic twins is questioned.
Bile Salt‐Stimulated Lipase Activity in Human Colostrum from Mothers of Infants of Different Gestational Age and Birthweight
Authors: PAMBLANCO M. , TEN A. , COMÍN J.
Published: January 2008

ABSTRACT. The bile salt‐stimulated lipase (BSSL) activity was measured in milk collected at 3–4 days postpartum (colostrum) from 36 mothers divided into three groups according to gestational age and birthweight of their infants. BSSL activity changed with the length of gestation. Preterm colostrum presented a mean activity significantly higher than the term groups (small‐for‐gestational age and appropriate‐for‐gestational age) which had similar values. The ratio of BSSL activity to the estimated fat content was 6.33 in colostrum of mothers who delivered preterm and 4.20 in colostrum of both groups of term mothers. These data suggest that preterm colostrum has a higher fat digesting potential than term colostrum and that it is the gestational age rather than the adequacy or non‐adequacy of birthweight to gestational age that may influence the BSSL activity.
Arterial Blood Pressure in the Very Low Birthweight Neonate
Authors: SONESSON S.‐E. , BROBERGER U.
Published: January 2008

ABSTRACT. The noninvasive oscillometric technique of arterial blood pressure determination was evaluated in fifteen very low birth weight neonates (<1400 g). Measurement of invasive umbilical artery catheter pressure was used as a reference standard. By using a cuff width to arm circumference ratio of 0.33–0.42, mean blood pressure was overestimated by 6.2 (7.2) mmHg (mean and 1 SD), p<0.001. More accurate mean blood pressure measurements were recorded with a cuff width to arm circumference ratio of 0.44–0.55. Measurements of systolic and diastolic blood pressure showed similar results.
Corticosteroid Treatment of Maternal ITP and Risk of Neonatal Thrombocytopenia
Authors: DONNÉR M. , ARONSSON S. , HOLMBERG L. , OLOFSSON P.
Published: January 2008

ABSTRACT. Maternal ITP is associated with a high risk of thrombocytopenia in the fetus and newborn infant. Methods of predicting this risk include determinations of maternal platelet count and platelet‐associated IgG prior to delivery. In this report we show that prednisolone treatment normalized maternal platelet count and platelet‐associated IgG without eliminating circulating platelet antibodies, which thus crossed the placenta and rendered the infant severely thrombocytopenia. Determination of maternal circulating platelet antibodies may be the best way of predicting infant thrombocytopenia, especially if the mother has received corticosteroids.
Neonatal Group B Streptococcal Septicaemia in a Developing Country
Authors: MACFARLANE D. E.
Published: January 2008

ABSTRACT. During a 3‐year study at the neonatal intensive care unit, University Hospital of the West Indies, the incidence of group B streptococcal (GBS) septicaemia was 13.6/1000 admissions and 1.4/1000 live births. GBS accounted for 35% of blood culture isolates and was the most frequent cause of septicaemia. Underlying disease or other possible predisposing factors were identified in 16 of 25 neonates with GBS septicaemia. Comparison of early onset and late onset disease indicated an increased incidence of prematurity, prolonged rupture of membranes, and respiratory distress in the former and a predominance of meningitis associated cases in the latter. Mortality was 50% for early onset cases, 29% for late onset cases and 36% overall. Antimicrobial therapy and preventive measures which may be appropriate for a developing country are discussed.
Iron Balances in Infant Nutrition
Authors: SCHULZ‐LELL G. , BUSS R. , OLDIGS H.‐D. , DÖRNER K. , SCHAUB J.
Published: January 2008

ABSTRACT. Iron balance studies were performed in 17 full term male infants from their 3rd until their 17th week of life. The balance studies were made in the infant's home and comprised 5 periods with an interval of 3–4 weeks, each consisting of three 24‐hour collections. Ten infants were breast‐fed, 3 received an adapted infant formula (P1, iron content 1.1 mg/l) and 4 were given the same formula enriched with iron, copper and zinc (P2, iron content 10.35 mg/l). From the 3rd to the 17th week of life the breast‐fed infants got a mean iron intake of 0.2 mg/kg body weight ×3 days and they retained 0.09 mg/kg b.w. ×3 days. The P1 group received 0.48 and 0.47 mg/kg b.w. ×3 days and retained ‐0.01 and ‐0.5 mg iron/kg b.w. ×3 days, while the P2 group had an intake from 5.04 to 6.38 mg b.w. ×3 days and retained between 1.13 and 3.66 mg iron/kg b.w. ×3 days. Comparing the 3 groups it can be concluded that the P1 group retained definitely less iron than the breast‐fed group, whereas the P2 group retained 12 to 40 times more iron than the breast‐fed babies.
Acute illnesses in Young Children and Family Response
Authors: HÖJER B. , STERKY G. , WIDLUND G.
Published: January 2008

ABSTRACT. Swedish speaking parents of 446 children, all around one year of age and living in the suburbs of Stockholm, participated in a three‐week diary study. The aim was to describe the pattern of illnesses in children and the measures taken in the family and to correlate the findings with the socio‐economic conditions of the family. A health complaint, most frequently running or blocked nose or a cough, was recorded for about 1/3 of the days of observation. Some form of family action was recorded on 70% of these days. Medicines were given on 39% of the complaint days and relatives, friends or a health professional were contacted on less than 10% of the days. In case of acute respiratory infection medicines were given or a health professional contacted more often if the symptom was recorded as severe. Most frequently the Child Health Centre nurse was consulted. The measures taken by the parents were judged to be rational. However, one quarter of the parents expressed uncertainty about the care of the acute infectious disorders of the child. Correlations between family response and socioeconomic factors were weak.
Eventration of the Diaphragm in Infants and Children
Authors: OBARA H. , HOSHINA H. , IWAI S. , ITO H. , HISANO K.
Published: January 2008

ABSTRACT. The study reviews 18 infants and children with eventration of the diaphragm who were treated over a period of eight years. The affected diaphragm and pulmonary tissue were examined by light and electron microscopy. The 18 patients, ranging in age from 10 days to 6 years, were divided according to Thomas’classification into a group with the congenital (10 patients) and a group with the acquired type (8 patients). Fifteen of these patients underwent surgery with diaphragmatic plication. On microscopic examination, biopsies of the lung showed atelectasis and pneumonia. These pathological changes became increasingly diffuse and severe with age. The diaphragm in patients with the congenital type of eventration was occupied by diffuse fibroelastic tissue. In patients with the acquired type, the cross‐striated muscles of the diaphragm showed degenerative changes such as fragmentation, and interstitial fibrosis of the diaphragm became prominent with age. The results of this clinical study sugget that, in order to reduce the pathological changes in the lung, early surgical plication should be performed even in patients with the acquired type, if respiratory and digestive symptoms are noted.
Recent Advances in the Inherited Methylmalonic Acidemias
Authors: MAHONEY M. J. , BICK D.
Published: January 2008

Methylmalonic acidemia results from decreased activity of methylalonyl‐CoA mutase, an enzyme required for the catabolism of four amino acids. A cobalamin (vitamin B12) compound is required as coenzyme. Several inherited mutations of the mutase apoenzyme or of cobalamin coenzyme synthesis have been identified. Clinical disease is most commonly recognized as a severe protein intolerance state although a wide range of symptoms has been appreciated. Current therapy emphasizes strict dietary management and use of vitamin B12 in cobalamin responsive patients.
Effect of a Modified Fluid Therapy on Renal Function during Indomethacin Therapy for Persistent Ductus Arteriosus
Authors: LEITITIS J. U. , BURGHARD R. , GORDJANI N. , WILDBERG A. , SEYBERTH H. W. , BRANDIS M.
Published: January 2008

A rehydration with 7 ml/kg/h for six hours prior to indomethacin administration prevented the adverse effects of this drug on renal function in prematures with persistent ductus arteriosus. During the 36 hour observation period after indomethacin administration, no significant changes in serum creatinine, sodium, and potassium concentrations, or urinary flow, creatinine clearance, or filtered sodium could be detected. The only significant finding was a reduction in fractional sodium excretion. One can assume that this beneficial effect of the fluid load is due to a suppression of some parts of the vasoconstrictor mechanisms, which are responsible for the deterioration of renal function in newborns during indomethacin therapy. Using this modified fluid regimen, no cardiovascular side effects were noticed, a closure of the duct was achieved in 7 of 10 treatment courses.
The Prevalence of Microalbuminuria in Diabetic Children and Adolescents and Its Relation to Puberty
Authors: DAHLQUIST G. , RUDBERG S.
Published: January 2008

The albumin excretion rate (AER) was studied in two groups of diabetic children and adolescents. Twenty‐four‐hour AER was studied in 75 children with diabetes for 5 years, in 49 children with diabetes for 10 years, in 55 children with diabetes for 10–20 years and in 21 age matched healthy controls. Overnight AER was studied in 129 diabetic children and adolescents with a duration of diabetes varying from 1–14 years. Diabetics exhibited a wide range of AER‐values and when expressed per body surface area, diabetic children had significantly higher AER compared to controls. Log transformed AER‐values were significantly correlated to age and body surface area in diabetics but not in controls. In the diabetics, log AER was also correlated to systolic and diastolic blood pressure but not to HbA1c. 20% of the 0 diabetics had AER values exceeding the upper value for healthy controls which was 18.5 μg/min. 31/35 of them were older than 12 years. In both groups of diabetics, 5% had AER‐values exceeding those reported to be predictive for later development of overt nephropathy, the youngest being 16 years old. When comparing diabetic children 0–12 years (i.e. before the maximal growth spurt of puberty) to those older than 12 years, at the same duration of diabetes, the latter group had significantly higher AER‐values. No sex difference was found in either age group. It is concluded that after puberty diabetic patients also show evidence of incipient diabetic nephropathy. Thus, routine screening for microalbuminuria is recommended also in pediatric diabetes care after 12 years of age.
Growth in Head Circumference from Birth to Fifteen Years of Age in Japan
Authors: ISHIKAWA T. , FURUYAMA M. , ISHIKAWA M. , OGAWA J. , WADA Y.
Published: January 2008

Growth charts for head circumference in Japanese children from birth to fifteen years of age are presented. The charts were compiled from the pooled data obtained in a prospective longitudinal study on development and growth in Nagoya city. An adolescent growth spurt in head circumference was found and this spurt occurred earlier in girls than in boys. Heads were smaller up to the age of 10 years and larger beyond that age than those in Nellhaus' composite charts. The heads of children of both sexes in our study are definitely smaller than those of children born in the United Kingdom in the same period, but they are considerably larger than those of children born in Japan more than 50 years ago. Therefore, the standards for head circumference will differ from country to country and from generation to generation.