bluuebunny/crossref_metadata_2025 huggingface dataset:
PHYSIKALISCHE GEWEBSVERÄNDERUNGEN BEI FIEBER‐ UND HYPERPYRETISCHEN TEMPERATUREN
Authors: FÅHRAEUS R.
Published: August 2010

Zusammenpassung

A) Bei Wärmebehandlung in vitro von Blut und seinen Hauptbestandteilen bei einer Reihe verschiedener Temperaturen treten diskontinuierliche Veränderungen auf in dem Sinne, dass Wärmegrade unterhalb der normalen Körpertemperatur wirkungslos sind, höher liegende Wärmegrade dagegen wirksam.

Die letztgenannten Wärmegrade, welche den Fieber‐ und hyperpyretischen Temperaturen entsprechen, verursachen

verminderte Aggregabilität der roten Blutkörperchen und der Thrombocyten.

verminderte bzw. aufgehobene Retraction des Blutgerinnsels, was auf eine Wärmeeinwirkung auf die Thrombocyten berunt.

verlängerte Koagulationszeit bzw. aufgehobene Koagulation. Bei den untersuchten Säugetieren tritt diese Veränderung etwa oberhalb 37°–38°, bei Vögeln erst oberhalb etwa 42°, bei Fischen schon oberhalb etwa 30° ein.

B) In der Nebennierenrinde, im Thymus und in der Hypophyse vorkommende sowie in die Blutgefässwand, in die Mere und in das Gehirn pathologisch auftretende Lipoide, welche unterhalb und bei der normalen Körpertemperatur doppelbrechend sind, verlieren bei den nächst höher liegenden Wärmegraden ihre Doppelbrechung.

Es wird versucht das Vorkommen und die Bedeutung physikalischer Zustandsänderungen lipoider Substanze bei Fieber und Hitzschlag zu diskutieren.
INVESTIGATIONS CONCERNING THE ORIGIN OF BLOOD‐PLATELETS
Authors: PETRI SVEND
Published: August 2010

Resumè

An extensive, isolated blood‐platelet bleeding on first day of experiment produces an unproportionately considerable blood‐platelet decrease, compensated in the course of 2–6 days.

Characteristic is the primary rise in haemoglobin percentage on 2nd day of experiment; likewise in one of the experiments (No. 3) the simultaneously occurring, but passing increase in the number of erythrocytes.

Experiment No. 3 would, a priori, appear to be especially suited to shew tissue changes by an accentuated formation of blood‐platelets. Histological changes that might be attributable to the formation of blood‐platelets were not, however, demonstrable.

Moreover, circumstances have been discovered that again refute Wright's theory.

Reinjection of plasma constituents seems to produce a increase in the number of the megakaryocytes, enclosing minute marrow cells. This effect is only noticeable during the first days after the operation.

In spite of the fact that the plasma removed by the bleeding is reinjected, a slight opalescence is at times observable in the specimens of plasma.
ALTÉRATIONS PATHOLOGIQUES DES NERFS DE LA PAROI GASTRIQUE DANS L'ULCÈRE CHRONIQUE DE L'ESTOMAC*
Authors: OKKELS HARALD
Published: August 2010

La présence fréquante de nerfs dans le voisinage immédiat de l'ulcère souligne d'une manière frappante la grande force de résistance du tissu nerveux.

Des cellules ganglionnaires sont assez souvent démontrées dans le tissu cicatriciel même du fond de l'ulcère.

Les altérations inflammatoires sont le plus souvent périnévritiques; l'origine en est une lymphangite ascendante.

La périnévrite se progage continûment.

Les altérations prolifératives des nerfs mêmes, se présentent sous forme de névromes cicatriciels centraux; ceux‐ci peuvent contenir des cellules nerveuses et sont, en ce cas, dérivés du plexus myogastricus (Auerbach); ou bien, ils peuvent se développer des nerfs périartériels, dans lequel cas ils ne contiennent pas de cellules nerveuses.

Aucune des altérations nerveuses n'est spécifique de l'ulcère chronique de l'estomac; il faut les considérer comme secondaires.

Les périnévrites et l'inclusion des nerfs en tissu conjonetif serré peuvent dégager les douleurs dans l'ulcère de l'estomac.
STUDIEN ÜBER DIE FRAGE DER KäLTEAGGLUTINATION DES BLUTES BEI MENSCHEN (VORLäUFIGE MITTEILUNG*
Authors: Kettel Karsten
Published: August 2010

Es wird gezeigt, dass man das bei Zimmertemperatur wirksame Kälteagglutinin am häufigsten nachzuweisen vermag, wenn das Gerinnen des Blutes und die Ausscheidung und Abpipettierung des Serums bei 37° C erfolgt sind.

Während die bei 0° aufbewahrten Sera ihr Kälteagglutinin in der Kegel mindestens 8 Monate (Dauer der Untersuchung) behalten, kann das Kälteagglutinin in wenigen Tagen vÖllig verschwinden, wenn die Aufbewahrung bei Zimmertemperatur stattfindet.

Durch halbstÜndige Inaktivierung des Serums bei 56° C wird das Kälteagglutinin nicht geschwächt.*)

Es ist nicht ratsam, die Erythrocyten zu waschen, da eine schwache Reaktion dadurch ganz wegfallen kann. Als Kontrolle ist eine Probe mitzunehmen, die gegen eine von dem zwischen den ungewaschenen Erytrocyten zurÜckgebliebenen SerumÜberrest verursachte Autoagglutination sichert.

In physiologischem Salzwasser aufgeschlemmte Erythrocyten bÜssen ihre Agglutinabilität schnell ein, eine in den ersten 4–5 Tagen aus dem Gerinnsel hergestellte Aufschlemmung hingegen ist oft fast ebenso empfindlich (wenn auch häufig nicht so empfindlich) wie eine frische Aufschlemmung.

Die Empfindlichkeit der Erythrocyten dem gleichen Serum gegenÜber ist individuell sehr verschieden; die Bedeutung dieses Verhaltens in bezug auf den Nachweis von Kälteagglutinin wird eingehend dargetan.

Die Verwendbarkeit von W.‐R.‐Blut zum Nachweis von Kälteagglutinin wird einer Kritik unterzogen. Zwischen 378 Fällen ist in etwa 95 Proz. ein Gehalt an Kälteagglutinin nachgewiesen worden, es wird aber vermutet, dass durch hinlänglich variierende Kombinationen von Serum und Blutproben in einem jeden Serum Kälteagglutinin nachgewiesen werden kann. Autoagglutination ist keine so seltene Erscheinung wie frÜher angenommen.

Es besteht keine Beziehung zwischen dem Titer des α‐ bezw.β‐Agglutinins in einem Serum und seinem Kälteagglutinintiter.

Die WärmeamplitÜde fÜr das Kälteagglutinin ist nicht als ein Ausdruck fÜr den Titer allein zu betrachten.

Es werden 10 Fälle besprochen, in denen die AmplitÜde des Kälteagglutinins die gewÖhnliche Zimmertemperatur Überstieg. Es wird gezeigt, dass es sich um ein absorbierbares Agglutinin handelt, da es bei niederer Temperatur fixiert und bei hÖherer Temperatur wieder freigemacht werden kann.

Dass die Wirkung des α‐ bezw.β‐Agglutinins bei 0° stärker ist als bei Zimmertemperatur, kann nicht auf einer Addition der genannten Agglutinine und des Kälteagglutinins beruhen, sondern muss auf die die Eeaktion fÖrdernde Wirkung der niedrigen Temperatur selbst zurÜckzufÜhren sein.
EIN FALL VON ATHEROSKLEROSIS PULMONALIS MIT ANEURYSMA ARTERIAE PULMONALIS BEI OFFEN‐ STEHEN DES FORAMEN OVALE
Authors: OKKELS HARALD , THERKELSEN FREDERIK
Published: August 2010

— Es wird ein Fall berichtet, wo em 31‐jähriger Mann seit dem späten Kindesalter an hochgradiger Dyspnöe und Zyanose gelitten hat. Sektionsresultat: Athero‐sklerosis pulmonalis bei Offenstehen des Foramen ovale. Aneurysma arteriae pulmonalis dicht über den Klappen. Durch Ausfüllung des Sackes mit Thrombenmaterial teilweise Erhaltung der freien Passage nach der rechten Lunge. Die linke Arteria pulmonalis war von Thrombenmassen völlig verschlossen. Kein Offenbleiben des Ductus Botalli, keine Stenose des Pulmonal‐ostiums, kein Defekt des Ventrikelseptums.

Die ungemein komplizierte Pathologie der Pulmonalarterie wird betont.
EIN FALL VON EXTRAUTERINGRAVIDITÄT BEI TUBENTUBERKULOSE
Authors: Therkelsen Frederik
Published: August 2010

Es wird ein Fall von Extrauteringravidität bei ausgebreiteter Tubentuberkulose berichtet. Aus der Literatur werden 8 früher veröffentlichte Fälle kurz referiert. Es wird betont, dass gerade bei der tuberkulösen oder gonorrhöischen Salpingitis frühzeitig ein Verschluss der Tuben eintritt, der Sterilität bedingt. Dadurch. erklärt sich die verhältnismässige Seltenheit der Extrauteringravidität.

Unser Fall erklärt sich mutmasslich durcb. die Tatsache (Hilbschmann), dass kanälchen‐ oder schlitzartige, eventuell von Schleimhautepithel ausgekleidete, innerhalb der Käsemassen befindliche Gebilde wohl für die Spermatozoen, nicht aber für das befruchtete Ei, passabel sind.
EXPERIMENTELLE UNTERSUCHUNGEN ÜBER GASTROGENE ANÄMIEN (BEI HUNDEN)
Authors: PETRI SVEND , OHLSEN A. SØEBORG
Published: August 2010

Es werden eine Reihe Avitaminoseveränderungen gesehildert, die nach elektiven Ventrikel‐Duodenum‐Resektionen namentlich bei heranwachsenden Hunden nach längerer Beobachtungszeit in Erscheinung traten. Der krankhafte Zustand, wovon anzunehmen ist, dass er hauptsächlich von B2‐Mangel verschuldet ist, gehört zur Pellagragruppe und hat nicht geringe Ähnlichkeit mit der beim Menschen auftretenden Form. Die Ursachen der Entwicklung des Avitaminosezustandes werden erörtert. Das Vorhandensein einer früher nicht beobachteten Eigenschaft des Magensaftes (bei Mensch und Hund), die auf die Ausnutsung der Vitamin B2‐Gruppe Einfluss hat, wird nachgewiesen.

Nachtrag: Nach Abschluss der vorstehenden Untersuchungen ist die beim Magensaft nachgewiesene Eigenschaft an ein paar Patienten der 2. Abteilung des Kommunehospitals (Prof. H. J. Bing) geprüft worden. Bei dem einen, einem Manne mit schwerer alkoholischer Polyneuritis, Stomatitis und Hautpigmentierung, erfolgte nach Eingabe von Magensaftpräparat schnell eine sehr erhebliche Besserung seines Zustandes. Das Untersuchungsmaterial wird später veröffentlicht werden (Petri & Wanscher).
CANCER GASTRIQUE (LINITE PLASTIQUE) SIMULANT UNE MALADIE DE SYSTÈME DE L'APPAREIL HÉMOPOÏÉTIQUE (RÉTICULOSE). Contribution au diagnostic différentiel
Authors: Jørgensen Johannes Vesterdal
Published: August 2010

REASUMÉ

On rapporte un cas non caractéristique au point de vue clinique de cancer gastrique (à cellules indépendantes) sans tumeur primitive démontrable et sans rétrécissement de l'estomac, ayant probablement son point de départ dans un ulcère cicatrisé de la petite courbure. Les métastases s'étaient systématisées dans les voies lymphatiques et dans le tissu osseux.

Les métastases au niveau des ganglions lymphatiques étaient caractérisées entre autres choses par l'occupation par les cellules tumorales du réticulum accompagnée de la formation de parties granulomateuses, ce qui a fait que temporairement le cas a été considéré comme une réticulose.

Pour le diagnostic la coloration par la réaction de mucus fut d'une importance décisive.
ÜBER DIE FÄHIGKEIT DER STAUBBAKTERIEN BEI SERA WR‐POSITIVE REAKTIONEN HERVORZURUFEN
Authors: Aminoff Maja
Published: August 2010

80 verschiedene Stämme grampositiver, sporenbildender, aerob wachsender Stäbchenbakterien wurden hinsichtlich ihrer Fähigkeit in kurzer Zeit ein ursprünglich WR‐negatives Serum in ein WR‐positives verwandeln zu können geprüft. Tabelle II zeigt die Ergebnisse. Die verschiedenen B. cereus‐und B. mycoidesstämme riefen meistens eine derartige Veränderung hervor, während die B. vulgatus‐ und B. mesentericusstämme dieses meistensnichttaten.
FIBRINOLYSIS IN NEWBORNS
Authors: EKELUND H. , HEDNER ULLA , NILSSON INGA MARIE
Published: August 2010

The fibrinolytic system was studied in 207 normal fullterm newborns during the first 5 days of life. A high, but rapidly decreasing plasminogen activator activity was found during the first 4 hours in umbilical vein blood in all of the children studied. The activity in the cord blood was lower and cannot be regarded as representative of the child. Fibrinolytic split products were demonstrated in large and rapidly decreasing amounts in serum samples without the addition of EACA but in at most small amounts in serum when EACA was added at sampling. These findings confirm the high plasminogen activator activity at birth which rapidly falls and furthermore show that there are no significant amounts of split products in vivo in the newborn. The plasminogen level was decreased in accordance with previous investigations. The high activator activity is counterbalanced by adequate inhibitory protection with an increase of the α2‐macroglobulin and inhibitors of plasminogen activation and the adult content of antiplasmin. The mean fibrinogen values in the first 48 hours of life are in the lower part of the normal range, but increase on the third day to a significantly higher level.
HEMOGLOBIN, HEMATOCRIT AND RED BLOOD CELL COUNT IN “CAPILLARY” (SKIN‐PRICK) BLOOD COMPARED TO VENOUS BLOOD IN CHILDREN
Authors: MOE PETER JOHAN
Published: August 2010

Hb, Htc and RBC in “capillary” (skin‐prick) and venous blood have been compared in 30 children and in 30 adults (15 males and 15 females). “Capillary” blood was obtained from a 3 mm deep puncture in the finger with a “Medipoint” blood lancet. Higher mean values were found in venous blood, particularly in the children.
HYPONATREMIA FOLLOWING ASPHYXIA NEONATORUM
Authors: FELDMAN WILLIAM , DRUMMOND KEITH N. , KLEIN MICHAEL
Published: August 2010

Three newborns with transient asphyxia developed hyponatremia and hypertonic urine. Inappropriate secretion of ADH is proposed as a reasonable explanation for these findings. Serum sodium and urine conzentration should be assessed in newborns with asphyxia or meningitis since this condition may be more common than realized.
GLUCOSE‐GALACTOSE MALABSORPTION
Authors: MEEUWISSE G. W. , LINDQUIST B.
Published: August 2010

In 2 children with glucose‐galactose malabsorption the intermediate metabolism of hexoses was studied. The removal rate of injected glucose was slow when the diet of the children contained limited amounts of well absorbed carbohydrate (fructose). When sufficient fructose was given in the diet, the half‐life of injected glucose was normal. A fast removal rate was seen when large amounts of carbohydrates had been administered prior to the intravenous GTT. The results of oral fructose tolerance tests varied with the pre‐test diet in a similar manner.

The fructosemia was small and independent of the diet, but the blood glucose concentration reacted in a way indicating decreased glucose tolerance if the diet contained little absorbable carbohydrate. This was evidently an unspecific effect of carbohydrate deprivation; in a control case, an infant with cystic fibrosis, initially malnourished, signs of decreased glucose tolerance were seen with an oral fructose tolerance test. In him, as well as in the patient with glucose‐galactose malabsorption, the supranorma1 rise of blood glucose was abolished by dietary measures ensuring an ample supply of calories and carbohydrates.

The intravenous galactose tolerance, tested in one of the patients, was probably normal at 13 months of age and certainly normal at 6 years of age.

It is concluded that the observed abnormalities of the metabolism of glucose in patients with glucose‐galactose malabsorption are unspecific for this disease and merely secondary to carbohydrate deprivation.
SINGLE INJECTION POLYFRUCTOSAN CLEARANCE IN NORMAL and ASPHYXIATED NEONATES
Authors: SVENNINGSEN N. W.
Published: August 2010

Abstract. Svenningsen, N. VV. (Department of Paediatrics, University Hospital, Lund, Sweden). Single injection polyfructosan clearance in normal and asphyxiated neonates. Acta Paediatr Scand, 64:87, 1975.–The postnatal development of the glomerular filtration rate in 36 neonates has been evaluated by the single injection polyfructosan clearance method (GFRpfr) by studies performed at 0–1, 2–4 and 5–7 weeks of postnatal age. During the first weeks of life there is a rapid postnatal increase of GFRpfr of almost the same magnitude in term and preterm healthy infants. Thus the postnatal development of glomerular filtration rate is more closely related to extra‐uterine excretory needs than to the maturatinal stage and gestational age at birth. In the asphyxiated neonates studied the GFRpfr was significantly lowered in the immediate postasphyxial stage while the infants were still hypoxemic. However the postnatal rise of GFRPfr in the time period following the postasphyxial stage was of the same order as in the non‐asphyxiated healthy neonates.
GLYCOSIDASES IN HUMAN SKIN FIBROBLAST CULTURES
Authors: HULTBERG B. , SJÖBLAD S. , ÖCKERMAN P. A.
Published: August 2010

Abstract. Hultberg, B., Sjöblad, S. and Ockerman, P. A. (Department of Clinical Chemistry, University Hospital, Lund, Sweden). Glycosidases in human skin fibroblast cultures. Acta Paediatr Scand, 64:123, 1975.–Five glycosidases, α‐fucosidase, α‐galactosidase, α‐glucosidase, β‐mannosidase and N‐acetyl‐α‐glucosaminidase were studied in human skin fibroblast cultures. The pH‐dependency, kinetic properties of the enzymes and results of isoelectric focusing and ion exchange chromatography are presented. Techniques suitable for diagnosing inborn lysosomal diseases in skin fibroblast cultures are defined.
WOLFF‐PARKINSON‐WHITE SYNDROME
Authors: FASTH A.
Published: August 2010

Abstract. Fasth, A. (Department of Paediatrics, the General Hospital, Helsingborg, Sweden). Wolff‐Parkinson‐White Syndrome. A fatal case in a girl with no other heart disease. Acta Paediatr Stand, 64:138, 1975.–A fatal case of the pre‐excitation syndrome in a 310/12‐ years old girl with no other heart disease is presented. Her death is thought to be the consequence of ventricular fibrillation. This observation is in contrast to the reported benign course in infants and children with the Wolff‐Parkinson‐White syndrome without other heart disease.
Oestrogen Therapy in Turner's Syndrome
Authors: KASTRUP K.W.
Published: August 2010

Oestradiol stimulates growth and development in Turner's syndrome. Previous results with low‐dose oestradiol on growth rate are reviewed. The effect of oestradiol in low concentrations on somatomedin generation, GH secretion and directly on osseous tissue, may explain the growth response. The observations presented here of 35 girls treated with 17‐β‐oestradiol demonstrated a definite increase in growth rate in the first year of therapy. Bone maturation was accelerated, but a reduction in final height was not found.
Magnetic Resonance Imaging in Growth Hormone Deficiency
Authors: MOMOI T. , KIKUCHI K. , FUJISAWA I. , YAMANAKA C. , KAJI M. , SUDO M. , MIKAWA H.
Published: August 2010

More than 20 patients with complete growth hormone (GH) deficiency have been examined by magnetic resonance imaging (MRI); transection of the pituitary stalk was found in 12 patients. Radiological (1) and endocrinological (2) studies in these patients will be published elsewhere. Another two patients with complete pituitary stalk transection have recently been identified, and together with one patient with secondary GH deficiency due to head trauma are presented in this report.
Synchronization of Growth Hormone Therapy
Authors: HERMANUSSEN M. , GEIGER‐BENOIT K. , BURMEISTER J. , SIPPELL W.G.
Published: August 2010

Based on the hypothesis of periodical refractoriness of the growing tissue to exogenous administration of GH, an experimental approach is presented in which the dosage of GH was adjusted to the natural occurrence of periodical changes of the lower leg growth velocity in five children with short stature. Two 6‐month periods were compared, during which the children received an identical cumulative dose of GH of 224 IU/m2. During the first period, the children received a constant GH dose of 14 IU/m2/week by daily subcutaneous injection, whereas a low/high dose alternation was administered at approximately 3‐week intervals synchronously with the occurrence of mini growth spurts during the second 6‐month period. In all the children, mean linear growth of the lower leg accelerated during the period of synchronization by 595% compared to the previous growth rate during constant GH administration.
Measurement of the Yellow Colour of the Skin as a Test of Hyperbilirubinemia in Mature Newborns
Authors: KNUDSEN AAGE
Published: August 2010

ABSTRACT. The relation between the yellow colour of the skin as measured by the Minolta Air Shields jaundice meter and the serum bilirubin concentration was investigated in 207 mature icteric, but otherwise healthy, newborns and the relationship between the changes in the meter readings and the changes in serum bilirubin concentration was studied in 99 of the infants. Good correlation was found both between the meter readings and the serum bilirubin concentration (p=0.81), and between the changes in meter readings and the changes in serum bilirubin concentration (p=0.69). The jaundice meter proved to be effective in predicting hyperbilirubinemia defined as serum bilirubin values 175 or 225 μmol/1, and was also effective in predicting increasing or decreasing serum bilirubin concentrations by sequential meter readings. The gestational age correlated slightly but significantly with the meter readings (p=–0.15) and with the serum bilirubin concentration (p=–0.15). However, these findings did not affect the action levels for use of the jaundice meter as a screening device for hyperbilirubinemia. The postnatal age and the birthweight did not significantly influence the meter readings.
Peripheral Facial Palsy in Childhood‐Lyme Borreliosis to be Suspected Unless Proven Otherwise
Authors: CHRISTEN H.‐J. , BARTLAU N. , HANEFELD F. , EIFFERT H. , THOMSSEN R.
Published: August 2010

ABSTRACT. 27 consecutive cases with acute peripheral facial palsy were studied for Lyme borreliosis. In 16 out of 27 children Lyme borreliosis could be diagnosed by detection of specific IgM antibodies in CSF. CSF findings allow a clear distinction according to etiology. All children with facial palsy due to Lyme borreliosis revealed lymphocytic CSF pleocytosis, whereas in cases of unknown etiology CSF was usually normal. Bilateral facial palsy occurred only in children with Lyme borreliosis. All cases with a positive history of tick bite and/or erythema migrans in the head‐neck region showed ipsilateral neurological affection suggesting a direct invasion via the affected nerve by Borrelia burgdorferi.
Periodic Variations in Skin Perfusion in Full‐Term and Preterm Neonates Using Laser Doppler Technique
Authors: PÖSCHL J. , WEISS T. , DIEHM C. , LINDERKAMP O.
Published: August 2010

ABSTRACT. In 37 full‐term and preterm infants periodic oscillations of skin blood flux were studied by means of laser Doppler technique during the first week of life. The development of rhythmic oscillations of skin blood flux was similar in all infants. On the first postnatal day rhythmic oscillations were present in the heel skin of all full‐term and preterm infants, but were rarely observed in the back and thigh skin. These flux motion patterns were not influenced by small changes in skin temperature. On day 4 rhythmic oscillations became predominant in all body regions. The oscillation frequencies of blood flux in the back, thigh and heel skin of full‐term neonates reached the lower range of adult values at the end of the first postnatal week, whereas the oscillation frequencies in the preterm infants were still below the range of full‐term neonates.
Neonatal Macrosomia and Hypoglycaemia in Children of Mothers with Insulin‐Treated Gestational Diabetes Mellitus
Authors: STENNINGER E. , SCHOLLIN J. , ÅMAN J.
Published: August 2010

ABSTRACT. All newborn children to mothers with gestational diabetes mellitus (GDM) in the county of Örebro were investigated during a one year prospective study. Neonatal macrosomia (birthweight > 3 SD) was observed in 27% of children of mothers with GDM and was significantly correlated to the cord C‐peptide concentration. Hypoglycaemia (B‐glucose <1.5 mmol/l) was observed in 38% of the children, most frequently two hours after delivery. Hypoglycaemia was not more common in macrosomic children and could not be predicted by the blood glucose concentration of the mother at delivery or by the cord C‐peptide level. It is concluded that mothers with GDM must be intensively treated in order to avoid the occurrence of macrosomia in their infants and that the newborn child must be carefully observed and treated in order to avoid neonatal hypoglycaemia.
Brain Abscess in Glycogen Storage Disease Type Ib
Authors: PARK HAE K. , KAHLER STEPHEN G. , CHEN YUAN‐TSONG
Published: August 2010

ABSTRACT. Neutropenia, neutrophil dysfunction, and recurrent infections are important manifestations of glycogen storage disease type Ib. We report here a child who has had adequate metabolic control, but developed brain abscess, an infection not previously described in this disease. This case provides further evidence that the neutrophil anomaly is related to the basic enzyme defect, not secondary to the metabolic derangement.
Pharmacokinetics of growth hormone‐releasing hormone(1–29)‐NH2 and stimulation of growth hormone secretion in healthy subjects after intravenous or intranasal administration
Authors: Wilton P , Chardet Y , Danielson K , Widlund L , Gunnarsson R
Published: August 2010

The growth hormone‐releasing hormone analogue GHRH(1–29)‐NH, was administered intravenously or intranasally to 30 healthy men aged 19–43 years. Intravenous injection of the lowest dose tested, 0.25 μg/kg body weight, elicited significant release of growth hormone (GH). Maximal release (mean GH peaks of about 90 mU/1) was obtained with a dose of 1–2 μg/kg. Although GHRH(1–29)‐NH2 was rapidly eliminated after intravenous injection, GH levels were elevated for about 3 hours. Absorption of GHRH(1–29)‐NH2 through the nasal mucosa was found to be low, and the bioavailability was only 3–5%. There was a dose‐dependent release of GH after intranasal administration of GHRH(1–29)‐NH2, with the maximal response obtained with about 50 μg/kg; this dose was approximately as potent as 1 μg/kg injected intravenously. The GH response after repeated intranasal administration of GHRH(1–29)‐NH2 was sustained; there was no suppression of GH secretion during the night following a day when GHRH(1–29)‐NH2 had been given three times intranasally. Based on these findings and the obvious convenience of intranasal administration compared with injections, it would be justified to test intranasal therapy for treatment of short stature in children with GH deficiency caused by hypothalamic damage.
Growth response to growth hormone‐releasing hormone(1–29)‐NH2 compared with growth hormone
Authors: Neyzi O , Yordam N , Oca1 G , Bundak R , Darendeliler F , AGikǧoz E , Berberoglu M , Günoz H , Saka N , Calikoglu AS
Published: August 2010

To assess the growth‐promoting effect of different doses of growth hormone‐releasing hormone(1–29)‐NH, (GHRH(1–29)‐NH2) in GH deficiency (GHD) of hypothalamic origin, 43 prepubertal children aged between 4.3 and 18.9 years (mean 10.4 k 2.9 years) were randomly assigned to three treatment regimens: low‐dose GHRH(1–29)‐NH2 (LD group; n = 15), high‐dose GHRH(1–29)‐NH, (HD group; n = 12) and GH (GH group; n = 16). The LD group received GHRH(1–29)‐NH, at 30 yglkglday S.C. in three daily doses, the HD group received 60 μg/kg/day S.C. in three daily doses and the GH group received GH, 0.1 IU/kg/day S.C. once daily. All children were treated for a period of 6 months. Evaluation included anthropometry, bone age, intravenous and subcutaneous GHRH(1–29)‐NHZ tests and determination of insulin‐like growth factor I (IGF‐I) levels. An increase in height velocity of 2 cm/year or more was observed in all except two children. Height velocity during treatment was lowest in the LD group, but comparable in the HD and GH groups. An increase in height SDS for bone age occurred only in the GH‐treated group. GH responses to intravenous GHRH(1–29)‐NH, showed a priming effect of the LD GHRH(1–29)‐NH, treatment, while a decrease in response occurred in the GH‐treated group. Following a subcutaneous test dose of one‐third of the daily dose of GHRH(1–29)‐NH, GH levels remained unchanged in both the LD and HD groups. There was accumulation of GHRH immunoreactivity over time in the HD group, but there was no correlation between measured GHRH and GH levels. IGF‐I levels increased in the HD and GH‐treated groups. Antibodies to GHRH were detected in 54% and 58% of children in the LD and HD groups, respectively. None of these variables correlated with changes in height velocity. The results indicate that 6 months of GH and high‐dose GHRH(1–29)‐NH, treatment have similar growth‐promoting effects.
Growth hormone (GH) profiles in response to continuous subcutaneous infusion of GH‐releasing hormone(1— 29)‐NH2 in children with GH deficiency
Authors: Tauber MT , Pienkowski C , Pigeon P , Cataldi M , Rochiccioli P
Published: August 2010

Six children presenting with partial growth hormone (GH) deficiency (mean GH peak in two different tests, 8.0 k1.3 μg/l) aged 8–10.3 years (mean, 2.7 ± 0.9 years) were treated for 6 months by continuous subcutaneous infusion of GH‐releasing hormone(1–29)‐NH, (GHRH(1–29)‐NH2); 24‐hour GH profiles and height velocity were measured. A biphasic effect of GHRH(1–29)‐NH2 infusion was observed. After an early substantial increase in the 24‐hour integrated concentration of GH, from 1.6 ± 0.1 to 3.5 ± 0.7 μg/l/minute, a subsequent consistent decrease occurred by 3 months, which was more pronounced after 6 months (mean 24‐hour integrated concentration of GH, 1.9± 0.9 μg/l/minute). This effect reflects modification of both pulse amplitude and frequency of GH secretion. At the end of the study, one child had complete suppression of GH secretion and two others showed only one peak above 5 μg/1 during a 24‐hour period. No correlation was found between these changes and height velocity. Three children did not grow significantly; the other three children who had a growth response to GHRH(1–29)‐NH2 were those with the lowest 24‐hour integrated GH concentration at the end of the study. The possible mechanisms involved in this biphasic effect, including GHRH antibodies, changes in somatostatin levels and/or desensitization of pituitary GHRH receptors, have been investigated.
A comparative study of growth hormone (GH) and GH‐releasing hormone(1–29)‐NH2 for stimulation of growth in children with GH deficiency
Authors: Chen R‐G , Shen Y‐N , Yei J , Wang C‐F , Xie D‐H , Wang X‐H , Zhou J‐D , Chen C‐Y , Wu Y‐L , Gunnarsson R , Danielson K , Chardet Y
Published: August 2010

In this study, 60 patients with proven growth hormone deficiency (GHD) of hypothalamic origin were randomized into three equal groups, and received growth hormone‐releasing hormone(1–29)‐NH, (GHRH(1–29)‐NH,), 30 or 60 μg/kg/day, or growth hormone (GH), 0.1 IU/kg/day, for 6 months. There were no significant differences in growth between the two groups given GHRH(1–29)‐NH, but growth in the GH group was significantly better than in the other two groups (p< 0.01). Mean height velocities at 6 months were 9.2, 9.3 and 14.6 cm/year for the three groups, respectively. Plasma GHRH concentrations increased steadily over the 6‐month treatment period, with higher levels in the group on the higher dose. During GHRH(1–29)‐NH2 treatment, serum concentrations of insulin‐like growth factor I rose initially, but then fell to values similar to those before treatment. No GH antibodies were detected, but all 20 patients on high‐dose GHRH(1–29)‐NH, and 19 of 20 patients on low‐dose GHRH(1–29)‐NH2 developed GHRH antibodies. These had almost disappeared by 9 months after stopping treatment. There was no correlation between antibody titres and increase in height. No serious side‐effects were seen, but three patients receiving GHRH(1–29)‐NH, reported mild irritation at the injection site. These results from the continuous infusion of GHRH(1–29)‐NH2 over 6 months suggest that this treatment, or the related use of a depot preparation, is unlikely to be as effective as GH for the promotion of growth in GHD.
Transient pyelonephritic changes on 99mTechnetium‐dimercaptosuccinic acid scan for at least five months after infection
Authors: Jakobsson B , Svensson L
Published: August 2010

Abstract A prospective study was performed on 185 children with symptomatic urinary tract infection (UTI), 130F and 55M, having a median age of 0.9 y (range 0.1–9.8) at the time of UTI. The aim of the study was to find out how the 99mTechnetium‐dimercaptosuccinic acid (DMS A) scan should be used to investigate UTI, and to follow the development of renal changes during pyelonephritis into subsequent permanent renal damage. All children were investigated with a DMSA scan within 5 days after admission and after 3.9–53.3 (median 9.2) weeks, and 159 were studied again after approximately 2 y (range 1.5‐3.9 y). They all underwent micturition cystourethrography at the time of the second study. At the time of infection, the DMSA scan was abnormal in 85% of the children, in 58% at the first follow‐up and in 36% at the second follow‐up. An abnormal DMSA scan performed within 20 weeks from infection became normal in 38% of cases on the third study, while only 1/10 abnormal DMSA scans performed more than 20 weeks after infection became normal after 1.5‐3.9 y. Persistent renal changes were more common in children > 4 y of age than in children 1 y of age. Two months after the presenting infection, it was unusual to see a normal DMSA scan in a child with a VUR gr. ≥ 3. The study suggests that DMSA changes after an index UTI may be transient for a longer period of time than has been previously considered. Therefore, in order to detect persistent changes, a DMSA scan should be performed more than 5 months after UTI.
The efficacy of sucrose for relieving procedural pain in neonates—a systematic review and meta‐analysis
Authors: Stevens B , Taddio A , Ohlsson A , Einarson T
Published: August 2010

Abstract The objective was to determine the efficacy and optimal dose of sucrose for relieving procedural pain in neonates. Data were obtained using MEDLINE, EMBASE, Reference Update and personal files and assessed for quality of the methods. Data from all randomized controlled trials where term and preterm neonates received a heelstick or venipuncture were examined for the efficacy of different sucrose doses (0.18 g, 0.24 g, 0.48 g or 0.50 g, 1.0 g) and water (placebo). The primary outcome was the proportion of time crying during 3 min after the painful stimulus. Data were combined across studies using a random effects model, adapted for use with single groups, producing a point estimate and 95% confidence interval (CI). Thirteen trials were identified; eight were rejected as data were inappropriate, non‐extractable, or the primary outcome was not measured. Five studies provided data on 271 infants. The proportion of time crying did not differ between 0.18 g of sucrose and water (p > 0.05) but was significantly lower in all other sucrose groups. There were no differences in proportion of time crying between term and preterm neonates. Sucrose reduced the proportion of time crying during painful procedures in neonates. The 0.18 g dose of sucrose was ineffective. Doses of 0.24 g (2 ml of 12% sucrose solution) were most effective. A dose of 0.50 g provided no additional benefit.
Familial liability to complications after BCG vaccination
Authors: Banac S , Franulović J
Published: August 2010

Abstract Adverse reactions induced by vaccination with bacille Calmette‐Guerin (BCG) were observed in three infants from two blood‐related families. A boy and a girl showed disseminated BCG infection with a fatal course in the former case. The third infant had regional suppurative lymphadenitis. Cases of two other children from the same families are also described: a boy who received BCG vaccine after 5 y of age without complications, and a girl who has not yet been vaccinated. The clinical course and immunological evaluation of the reported cases suggest a congenital immunodeficiency of an unusual transient nature. The role of genetic factors in familial susceptibility to mycobacterial infection also needs to be taken into consideration.
An 11‐year‐old girl with reflex sympathetic dystrophy successfully treated by thoracoscopic sympathectomy
Authors: Honjyo K , Hamasaki Y , Kita M , Harano K , Totoki T , Miyazaki S
Published: August 2010

Abstract We report on an 11‐year‐old girl with reflex sympathetic dystrophy (RSD) complaining of severe pain in her right upper extremity. Oral administration of narcotics or non‐steroid anti‐inflammatory drugs gave no relief in pain. Thoracoscopic electrocauterization of the thoracic sympathetic ganglion at the level of T3 was performed 3 months after the start of symptoms, and brought complete resolution of pain.
Bone age in 116 untreated patients with Turner's syndrome rated by a computer‐assisted method (CASAS)
Authors: Schwarze CP , Arens D , Haber HP , Wollmann HA , Binder G , Mayer EIE , Ranke MB
Published: August 2010

Bone age maturation in 116 untreated patients with Turner's syndrome was evaluated in a cross‐sectional and longitudinal analysis. A total of 265 radiographs were rated using the TW2‐RUS method on the computer‐assisted skeletal age score (CASAS) system. Bone age was found to be retarded from the chronological age of 3 to 6y. Between the ages of 7 and 12y bone age almost equalled chronological age and progressed normally at a rate of 1 y y−1. Bone maturation slowed down thereafter and epiphyseal closure was not reached before the age of 17 y. Reference data are presented on bone age and a bone age maturation curve for untreated patients with Turner's syndrome to be used in clinical practice. In the assessment of bone age and bone age velocity in Turner's syndrome the CASAS system produced reliable and valid results. The absolute difference between repeated bone age ratings was 0.26“y”(median) with a range of 0.00‐0.56“y”. Future studies evaluating the effect of growth‐promoting treatment in Turner's syndrome should use a computerized method for the determination of bone age.
Intensified treatment of acute childhood lymphoblastic leukaemia has improved prognosis, especially in non‐high‐risk patients: the Nordic experience of 2648 patients diagnosed between 1981 and 1996
Authors: Gustafsson G , Kreuger A , Clausen N , Garwicz S , Kristinsson J , Lie SO , Moe PJ , Perkkiö M , Yssing M , Saarinen‐Pihkala UM ,   the Nordic Society of Paediatric Haematology and Oncology (NOPHO)
Published: August 2010

In a multinational, population‐based study from the five Nordic countries (Denmark, Finland, Iceland, Norway and Sweden), 2648 children below 15 y of age were diagnosed with acute lymphoblastic leukaemia (ALL) in the years 1981‐1996. The annual incidence was 3.9/100 000 children and was stable throughout the study period. The development from regional or national protocols to common Nordic treatment protocols for all risk groups was completed in 1992 through a successive intensification of therapy, based on multidrug chemotherapy including pulses of methotrexate in high doses and avoidance of cranial irradiation in most children. For children with non‐B‐cell ALL (n= 2602), the event‐free survival (p‐EFS) increased from 0.53 ± 0.02 (diagnosed 7/81‐6/86) to 0.67 ± 0.02 (7/86‐12/91) to 0.78 ± 0.02 (1/92‐12/96). The corresponding p‐EFS values at 5 y were 0.57, 0.70 and 0.78, respectively. The main improvements were seen in the group of children with non‐high risk leukaemia, with 5‐y p‐EFS values increasing from 0.60 to 0.76 and 0.85 for the three periods. In high‐risk patients, progress has been moderate, especially in children with high white blood cell values at diagnosis. During the last 5‐y period, only 10% of the patients received cranial irradiation in first remission while 90% of the patients received high doses of cytostatic infusions (methotrexate isolated or combined with cytarabinoside) and multiple intrathecal injections of methotrexate as CNS‐adjusted treatment without any indication of an increased CNS relapse rate.
Lack of precision in neonatal death classifications based on the underlying causes of death stated on death certificates
Authors: Winbo IGB , Serenius FH , Källén BAJ
Published: August 2010

Large‐scale analyses of causes of neonatal deaths are usually based on death‐certificate information. A new computer‐based method has been introduced to define the cause of stillbirths and neonatal deaths in large amounts of material and to classify them according to two different models [Wigglesworth and Neonatal and Intrauterine death Classification according to (a)Etiology (NICE)]. The method is based on a combination of detailed information from health care registries and the death‐certificate information. The present study aimed to compare these two classification models with a previously published method based solely on death certificate information [International Collaborative Effort (ICE)]. The study population comprised 2378 neonatal deaths in Sweden between 1987 and 1992. Cross‐tabulation was made between the ICE classification and the other two classification models. In addition, case examples are presented in detail, exemplifying how classification errors arose. The ICE classification gives a rather low precision, notably for two important causes of death: asphyxia and immaturity. Among 328 infants dying from asphyxia according to computerized Wigglesworth classification, ICE classified 59% as asphyxia and 22% were labelled immaturity. When ICE classified the deaths as due to asphyxia, this was verified in only 50%. Among 792 infants dying from immaturity according to computerized Wigglesworth classification, 64% were classified as such by ICE. The findings cast doubts on the results of studies based exclusively on death‐certificate information. Whenever possible in the analysis of neonatal deaths, death‐certificate information should be supplemented with more detailed data. The computer‐based method introduced here makes such analyses possible for large databases.
Transepidermal water loss in infants born at 24 and 25 weeks of gestation
Authors: Ågren J , Sjörs G , Sedin G
Published: August 2010

The rate of evaporation of water from the skin of 13 infants born at 24 (n= 3) and 25 (n= 10) weeks of gestation was measured on the first day after birth and at postnatal ages of 1,3,7 and 28 d, using the gradient method. Transepidermal water loss was estimated from this rate and corrected to an ambient relative humidity (RH) of 50%. Transepidermal water loss, corrected to 50% RH, was high on the first day after birth (58.4 ± 14.8gm 2 h?1) and remained at the same level during the second day (59.3 ± 17.6gm?2h?1). It then decreased significantly to 43.8 ± 9.5 at a postnatal age of 3 d, 36.1 ± 12.6 at 7 d and 24.2 ± 7.7gm?2h?1 at 28 d (p < 0.001). Within the group investigated, there was no significant correlation between transepidermal water loss and body or skin temperature, birth weight, gender, mode of delivery or gestational age. Transepidermal water loss on the first day after birth was somewhat lower than the highest losses previously found in infants born at 25 weeks of gestation, and of the same magnitude as previously reported for infants born at 25‐27 weeks. Transepidermal water loss at postnatal ages of 1,3,7 and 28 d in the present study was higher than that previously found in the group of infants born at 25‐27 weeks. In conclusion, in infants born at 24‐25 completed weeks of gestation transepidermal water loss was high immediately after birth and decreased with increasing postnatal age, but at a slower rate than previously reported for slightly more mature infants.
Coma in a premature infant associated with the transdermal absorption of propylene glycol
Authors: Peleg O , Bar‐Oz B , Arad I
Published: August 2010

A case of propylene glycol intoxication in a premature infant is reported. The infant went into a state of coma after treatment for burns with antiseptic dressings. Cessation of the topical treatment resulted in complete recovery. An exceptionally high level of the dressings' solvent, propylene glycol, found in the urinary chromatogram, was believed to be the causative agent. It is suggested that topical preparations containing propylene glycol should not be used in premature infants during the first weeks of life.
Is homozygous α‐thalassaemia a lethal condition in the 1990s?
Authors: Ng PC , Fok TF , Lee CH , Cheung KL , Li CK , So KW , Wong W , Yuen PMP
Published: August 2010

Two cases of homozygous α‐thalassaemia who received active treatment in accordance with parental wishes are reported. One infant survived and the other, although successfully weaned off mechanical respiratory support, unexpectedly developed portal vein thrombosis and died. Homozygous a‐thalassaemia, a condition previously considered to be universally fatal, and an indication for therapeutic abortion, is now potentially curable with advances in diagnostic technology and treatment. However, active management of these cases raises serious ethical questions and has major financial implications on the health‐care system. Invasive prenatal and intensive postnatal interventions should remain experimental and cannot be recommended as routine clinical practice until the questions of long‐term neurodevelopmental outcome, and the morbidity and mortality associated with bone‐marrow transplantation have been fully addressed. As a result of advances in information technology, more and more parents of affected foetuses are likely to request active treatment.
Mediastinal lymphadenopathy: a variant of incomplete Kawasaki disease
Authors: Marcet J Bosch , Boira M Peñas , Créixams X Serres , Martínez L Inaraja
Published: August 2010

A 14‐month‐old girl presented with a 4‐d history of fever and generalized exanthema. Four characteristic symptoms of incomplete Kawasaki disease (KD) were present on admission (fever, rash, non‐purulent conjunctival injection, oropharyngeal changes) and then followed by oedema of the hands and feet and mild plantar desquamation. The typical laboratory features of KD, such as elevated erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and positive C‐reactive protein were also seen. Ultrasound examination of the mediastinum revealed the presence of a lymph node, 30 mm in diameter, below the tracheal carina. Thoracic CT scan confirmed the mediastinal lymph node. The patient was treated with aspirin and intravenous γ‐globulin. Ultrasound study of the mediastinum, which was carried out 6 weeks after hospital discharge, showed that the lymph node had disappeared. This case illustrates that lymph nodes other than cervical lymphadenopathy should be sought when the diagnosis of classical or atypical KD is suspected.
Breastfeeding and neurological outcome at 42 months
Authors: Lanting CI , Patandin S , Weisglas‐Kuperus N , Touwen BCL , Boersma ER
Published: August 2010

This study investigated the effect of early feeding mode on the neurological condition at 42 months. For this purpose, healthy pregnant women were recruited in Groningen and Rotterdam, The Netherlands. Children were healthy and born at term. At 42 months, the children were neurologically examined by means of the Touwen/Hempel technique. In addition to the clinical diagnosis, the neurological findings were interpreted in terms of optimality. Special attention was paid to the quality of movements in terms of fluency. In total, 200 (51%) exclusively breastfed (for ≥ 6 weeks) and 194 (49%) formula‐fed children were studied. Twelve (3%) 42‐month‐old children were considered to be neurologically mildly abnormal and 1 child was diagnosed as abnormal. No effect of the type of feeding was found on the clinical diagnosis or the neurological optimality. After adjustments for study centre and social, obstetric, perinatal and neonatal neurological differences, a beneficial effect of breastfeeding on the fluency of movements was found (odds ratio for non‐optimal fluency 0.56; 95% confidence interval 0.37‐0.85). The prolongation of full breastfeeding beyond 6 weeks did not influence the quality of movements. In conclusion, among Dutch preschool children, there was a small advantageous effect of full breastfeeding during the first 6 weeks of life on the fluency of movements.