bluuebunny/crossref_metadata_2025 huggingface dataset:
ON GANGLIONEUROBLASTOMA SYMPATHICUM
Authors: BUSCH B.
Published: August 2010

A case of ganglioneuroblastoma sympathicum is reported — the 6′ surely substantiated and the largest known — in a 30 years old woman, who dies with recidive and in cachexia in spite of attempted operative removal and intensive X‐ray treatment.

The macro‐ and microscopical appearance of the tumour and the numerous glandular metastases is described.

The previously published cases are reported.

The exstirpation at as early a moment as possible of any tumour containing ganglion‐cells is advocated, because the possibility of a «malignant degeneration» can not be excluded.

The factors causing this «malignant degeneration» are totally unknown.

Mitteilung Über ein Fall von ganglioneuroblastoma sympathicum — der sechste und grøsste der bisher bekannten — bei einer 30‐jährigen Frau, die stirbt durch Rezidiven und Kachexie trotz Versuch auf Exstirpation und intensive RÖntgenbehandlung.

Das makro‐ und mikroskopische Aussehen des Tumors und der zahlreichen Glandelmetastasen wird beschrieben.

Die frÜhere Fälle werden zusammengefasst.

Es wird die mÖglichst frÜhe Exstirpation jedes ganglienzellenhaltigen Tumour angeräthet, da die MÖglichkeit einer späteren «malignen Degeneration» nie ausgeschlossen werden kann.

Die Ursachen einer solchen «malignen Degeneration» sind vÖllig unbekannt.

Resumé

Rapport sur un cas de ganglioneuroblastome sympathique — le 6′ et le plus grand des cas encore communiqués — chez une femme de 30 ans, qui meurt par récidives et cachexie, un attempt d'exstirpation et traitement intensive avec des X‐rays prouvant sans éffet.

L'apparence macro‐ & microscopique du tumeur et des metastases aux glandes très nombreuses est décrie.

Les cas jusque á ce date communiqués sont resumés.

L'exstirpation plutôt possible est recommandée de tout tumeur contenant des cellules du ganglion, la possibilité d'une «degeneration maligne» future ne pouvant pas être exclue.

Les causes d'une telle «degeneration maligne» sont tout à fait inconnuees.
ÜBER DIE GONOKOKKEN‐KOMPLEMENTBINDUNGS‐REAKTION BEI MEDIZINISCHEN GELENKLEIDEN
Authors: HOLBÖLL S. A.
Published: August 2010

Unter 230 Patienten mit medizinischen Gelenkkrankheiten ist die kliuische Diagnose in 29 Fällen (= 12.6%) gonorrhoisehe Arthroitis.

Die Gonokokken‐Koinplementbindungs‐Reaktion erwies sich bei alien Patienten mit gonorrhoischer Arthroitis als positiv, während bei ungefähr 200 anderen Gelenkpatienten nnr 4 schwache und zweifelhafte Reaktionen beobachtet wurden.

Die Reaktion kann während der ersten Krankheitstage negativ bleiben, sie wird erst zu Anfang der 2ten Woche positiv und steigt alsdann bis zu einem Maximum, das für die einzelnen Fälle verschieden ist.

Bei 4 Patienten, die früher eine gonorrhoische Arthroitis gehabt haben und jetzt chronische Gelenksymptome aufweisen, ist die Reaktion nach 5–7 Jahren positiv.

Die Gonokokken‐Komplementbindungs‐Reaktion ist als eine siehere spezifische Reaktion zu bezeiehnen, die in einigen Fällen für die klinische Diagnose der gonorrhoischen Gelenk‐krankheiten eine gute Stütze bildet.
Environmental constraints for plumage melanization in the northern goshawk Accipiter gentilis
Authors: Galván Ismael , Bijlsma Rob G. , Negro Juan J. , Jarén Manuel , Garrido‐Fernández Juan
Published: August 2010

Although it is recognized that certain environmental factors are important determinants of the expression of melanin‐based traits, their influence in wild populations of animals is poorly known. One of these factors is the availability of amino acids that serve as precursors of melanins. Here we measured eumelanin and pheomelanin content in feathers of northern goshawk Accipiter gentilis nestlings, hypothesizing that, if the availability of melanin precursors is related to food abundance and habitat quality, plumage melanization should be affected by those variables. Although the eumelanin content increased with food abundance as predicted, the levels of this variable were higher in low‐quality habitats (homogeneous coniferous forests) and in nestlings in poor condition, and the pheomelanin content and eumelanin:pheomelanin ratio were lower and higher, respectively, in subpopulations where nestlings were in poorer condition. Therefore, environmental availability of melanin precursors seems to determine plumage melanization in goshawks, but our findings may also be explained by the differential effects of environmental oxidative stress on both forms of melanin, as eumelanin and pheomelanin production are favoured under high and low levels, respectively, of oxidative stress.
Systemic and splanchnic hemodynamic changes in patients with hepatic schistosomiasis
Authors: Denié Cécile , Vachiery Florence , Elman Annie , Soupison Thierry , Gadano Adrian , Moreau Richard , Lebrec Didier
Published: August 2010

Abstract: Although hepatic schistosomiasis is a common cause of portal hypertension, only a few hemodynamic studies, in humans, have been published on this subject. The aim of this study was to determine the systemic and splanchnic hemodynamic changes in hepatic schistosomiasis and to evaluate the influence of liver fibrosis on these changes. A retrospective analysis of a series of 13 patients with hepatic schistosomiasis who had undergone hemodynamic studies was performed. Portal or perisinusoidal fibrosis was present at liver biopsy in 8 patients. The control group included 22 patients with chronic hepatitis and normal hepatic venous pressure gradients. Patients with schistosomiasis exhibited high cardiac index (4.11±1.15 1 · min‐1 · m‐2 vs 2.99±0.85 1 · min‐1 · m‐2; p<0.05) and low systemic vascular resistance (1039±316 dyn · s · cm‐5 vs 1334±336 dyn · cm‐5; p<0.05). The hepatic venous pressure gradient and hepatic blood flow were normal. Azygos blood flow was markedly increased (0.90±0.66 1 · min‐1 vs 0.13±0.04 1 · min‐1; p<0.05). Hemodynamic values were not significantly different between patients with liver fibrosis and those without fibrosis at liver biopsy. In conclusion, patients with hepatic schistosomiasis had a hyperkinetic systemic and splanchnic circulation. In patients with esophageal varices, a normal hepatic venous pressure gradient confirmed presinusoidal portal hypertension. The presence of portal or perisinusoidal fibrosis did not influence hyperdynamic splanchnic state.
Periodontal inflammation and attachment loss: a critical problem for biological studies
Authors: Watts TLP
Published: August 2010

BACKGROUND: In biological studies of periodontitis, there has been long‐standing confusion between the ubiquitous phenomena of inflammation and the essential criterion of attachment loss. This is partly attributable to inadequate methods of clinical measurement, but seems also to be a consequence of an unproven and usually unstated assumption that the same biological processes underlie both inflammation and attachment loss. Developments in unidimensional clinical probing methods have helped in studies of periodontal treatment. However, such methods are intrinsically unsuitable in studies of periodontal diseases, and may have given them a false sense of security. The confusion has been compounded by inappropriate use of statistical techniques in an attempt to solve problems which do not arise from mathematical models but are intrinsic to measurement methods.

OBJECTIVE: This paper is a clinician's attempt to state the current difficulties and suggest some ways forward, including the development of three‐dimensional measurement, non‐invasive probing, and several objectives for biochemical, microbiological and immunolog‐ical research.
Prevalence of oral leukoplakia in 1000 Berliners
Authors: Reichart PA , Kohn H
Published: August 2010

OBJECTIVE: The aim of this study was to determine the prevalence of oral leukoplakia and other oral white lesions in an urban population of non‐referred patients to a department of oral surgery in the city of Berlin.

DESIGN: A total of 1000 patients over the age of 16 were evaluated for oral leukoplakia and other oral white lesions. Age, sex, and smoking as well as alcohol habits were recorded.

RESULTS: Of 506 men (50.6%) and 494 women (49.4%), 0.9% showed oral leukoplakia. Men were more frequently affected (I.6%) than women (0.2%). Patients of older age groups were more frequently affected than younger patients. Other white oral lesions were recorded such as leukoedema (8.3%), smoker's palate (0.I%), frictional white lesions (2.6%) and lichen planus (0.6%) with equal distribution between men and women.

CONCLUSION: The prevalence of oral leukoplakia in this limited group of urban patients was low, however comparable to that of other neighbouring west European countries. Association with tobacco and alcohol consumption was demonstrated as in most other studies.
Hepatitis C virus infection and lichen planus: a short review
Authors: Lodi G. , Porter SR
Published: August 2010

OBJECTIVE: To review the current literature regarding the association of lichen planus (LP) and liver disease, with particular attention to the association of the oral variant of the disease with hepatitis C virus (HCV) infection.

MATERIALS AND METHODS: Available literature of the possible association of LP with systemic disorders, in particular chronic hepatic disease, has been reviewed. RESULTS: LP is sometimes associated with infectious or autoimmune disease and/or neoplasia, however an aetio‐logical association between LP and these disorders seems unlikely. A more consistent association exists between LP and chronic hepatic disease. The precise cause of this association is not known. However, in the last 6 years a notable association between HCV infection and LP has been observed, particularly in patients in Spain, Italy and Japan. The pathogenesis of this possible HCV‐associated LP is not known, but it may involve a cell mediated response to an altered epithelial antigen.

CONCLUSION: There is now evidence to suggest a significant association between HCV infection and LP in some groups of patients.
Parathyroid hormone‐related protein (PTHrP) as a regulating factor of endochondral bone formation
Authors: Suda N.
Published: August 2010

Parathyroid hormone‐related protein (PTHrP) was first identified as a pathogenetic factor for the hypercalcemia of malignancy. Recently PTHrP is focused as a physiological paracrine factor regulating cell proliferation and differentiation in many tissues during fetal and postnatal growth. Evidence for the skeletal origin of PTHrP comes from several sources and targeted disruption of the PTHrP gene in mice has resulted in a phenotype with accelerated endochondral bone formation, suggesting PTHrP as a factor regulating chondrocyte differentiation. Indian hedgehog, one of the conserved family of hedgehog regulating segmentation of Drosophila, is found to be an upstream factor of PTHrP in a regulating pathway of chondrocyte differentiation. Moreover, Bcl‐2, a protein that controls programmed cell death in several cell types, is suggested to lie downstream of PTHrP in this pathway. A point mutation of PTH/PTHrP receptor is identified in a patient with Jansen‐type metaphyseal chondrodysplasia and constitutive, ligand independent activation is indicated in this mutant receptor.
Accumulation of strontium and fluoride in approximal dental plaque and changes in plaque microflora after rinsing with chlorhexidine‐fluoride‐strontium solution
Authors: Spets‐Happonen S , Seppa L , Korhonen A , Alakuijala P
Published: August 2010

OBJECTIVES: To find out if strontium is incorporated into plaque and enamel in vivo during a 2‐week rinsing period with a chlorhexidine‐fluoride‐strontium solution and to determine the effects of the rinsing on the numbers of mutans streptococci and lactobacilli in plaque. SUBJECTS AND METHODS: A total of 18 adult participants rinsed their mouths twice a day for 2 weeks, first with a placebo solution and subsequently, separated by a I‐week interval without rinsing, with a chlorhexidine gluconate (0.05%)‐sodium fluoride (0.04%)‐strontium (100 ppm as SrCI2) rinsing solution (CXFSr) for another 2 weeks.

RESULTS: After the CXFSr rinsing period the strontium and fluoride contents (μg g‐plaque dry weight; mean ± s1) of approximal plaque were 32.5 ± 4.7 and 72.8 ± 9.0, compared with the respective contents of 8.4 ± 1.2 and 42.0 ± 4.8 after placebo rinsing (P = 0.0001 for both comparisons). The strontium content remained elevated for 6 weeks. The median proportion of mutans streptococci of approximal plaque of the total viable count of bacteria was 1% after placebo rinsing but decreased to 0.2% after CXFSr rinsing. The proportion of mutans streptococci remained low at 3 weeks (P = 0.018 vs placebo) but had reached the placebo level at 6 weeks. Rinsing with CXFSr solution did not reduce lactobacilli in plaque. The strontium or fluoride contents of the enamel surfaces subjected to tooth brushing did not significantly change.

CONCLUSIONS: Strontium and fluoride accumulated in dental plaque during a 2‐week CXFSr rinsing period and the proportion of mutans streptococci in approximal plaque was reduced at least for 3 weeks after completion of the rinsing.
Elimination of hypersensitive gagging reaction to dentistry by propofol at subhypnotic doses
Authors: Tomioka S , Uchida D , Eguchi S , Nakajo N
Published: August 2010

We describe a 44‐year‐old male patient with severe gagging reaction successfully treated without sedative effect by a continuous infusion of propofol. Propofol at subhypnotic doses seemed to be effective in eliminating hypersensitive gagging reaction to dentistry.
Cranio‐facial fibrous dysplasia in a 38‐year‐old African woman: a case history
Authors: Simon E , Matee M , Shubi F , Mselle T
Published: August 2010

A 38–year‐old woman sought treatment with cranio‐facial dysplasia involving the cranium, maxilla and the mandible. Her chief complaint was a mandibular swelling, which had appeared about 2 years previously, had gradually enlarged, and was associated with spontaneous pain. X‐ray film examination revealed a ground‐glass opacity with blurred demarcation and a 99 Tc medronate bone scan disclosed an increase in tracer uptake in the cranium, maxilla and mandible. The rest of the skeleton was not affected. Histological examination of the lesions revealed solid proliferation of spindle‐shaped cells associated with islands of osteid and bone trabecullae with Chinese letter pattern and numerous multinucleated giant cells consistent with fibrous dysplasia. The continued osteoblastic activity of involved bones, coupled with the medical condition of the patient, restricted the management of the patient to largely supportive and palliative care.
Number II Pemphigus vulgaris
Authors: Black M , Mignogna MD , Scully C
Published: August 2010

Pemphigus is a group of potentially life‐threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering. Pemphigus vulgaris (PV), the most common variant, is characterized by circulating IgG antibodies directed against desmoglein 3 (Dsg3), with about half the patients also having Dsg1 autoantibodies. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles and ethnic groups such as Ashkenazi Jews and those of Mediterranean and Indian origin, are especially liable. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examination are essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected using both normal human skin and monkey oesophagus or by enzyme‐linked immunosorbent assay. Before the introduction of corticosteroids, PV was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using corticosteroids, with azathioprine or other adjuvants or alternatives but newer therapies with potentially fewer adverse effects, also appear promising.
Assessing the levels of immunoglobulins in the saliva of diabetic individuals with periodontitis using checkerboard immunodetection
Authors: Bachrach G , Muster Z , Raz I , Chaushu G , Stabholz A , Nussbaum G , Gutner M , Chaushu S
Published: August 2010

Background:  Current methods for determining salivary antibodies are cumbersome for large‐scale screenings.

Objectives:  To test checkerboard immunodetection for monitoring salivary antibodies and to profile them in diabetic individuals with periodontitis.

Methods:  Salivary anti‐Porphyromonas gingivalis, anti‐Actinobacillus actinomycetemcomitans and total IgA levels of 10 individuals were compared using checkerboard immunoblotting and enzyme‐linked immunosorbent assay (ELISA).

Results:  Close correlation between both methods was found in anti‐P. gingivalis IgA and total IgA, but not in anti‐A. actinomycetemcomitans IgA, because of high background levels in ELISA. Thereafter, checkerboard immunodetection was used to compare salivary antibodies of 20 adult type II diabetic with 32 non‐diabetic individuals with (n = 22) or without (n = 10) periodontitis. Patients with periodontitis (regardless of their diabetic condition) expressed increased levels of total IgA in both whole and parotid saliva, but reduced levels of anti‐A. actinomycetemcomitans IgA in whole saliva. Consequently, the proportion of anti‐A. actinomycetemcomitans IgA in the total IgA was lower in saliva of patients with periodontitis compared with healthy controls.

Conclusions:  Checkerboard immunodetection was reliable and economical for screening saliva samples for multiple antibody reactions. Our results support previous reports which suggested that patients with periodontitis are able to secrete high levels of salivary Ig, but are hampered in targeting their salivary response toward A. actinomycetemcomitans.
Dwarfism in Siblings: Two Case Histories With Comments By Carl von Linné
Authors: WALLGREN ARVID
Published: August 2010

Comments on a case report on dwarfism in siblings from 1745 sponsored by Carl von Linné and by him regarded as due to alcoholism in the children.
A Case of Superior Vena Caval Thrombosis Associated with Periarteritis Nodosa
Authors: KOMROWER G. M. , MACGREGOR E. U. , MARSDEN H. B.
Published: August 2010

1. A case of superior vena oaval thrombosis is described in a child of nine years.

2. The clinical progress suggested the possibility of a necrotizing angiitis and autopsy examination disclosed a periarteritis nodosa.

3. The relationship of periarteritis nodosa and superior vena caval thrombosis due to “non‐specific mediastinitis” is discussed.

We are most grateful to Professor A. C. P. Campbell, Professor of Pathology, University of Manchester, for his criticism and advice.

Un cas de thrombose dans la veine cave supérieure, associée avec une périartérite noueuse.

Un cas de thrombose de la veine cave supérieure chez un enfant de 9 ans, est décrit. La progression clinique a suggéré la possibilité d'une angiite nécrotique et l'autopsie a révélé une périartérite noueuse. Le rapport existant entre la périartérite noueuse et la thrombose de la veine cave supérieure dûe à une « médiastinite non spécifique », est développé.

Ein Fall von Thrombose der oberen Hohlvene in Zusammenhang mit Periarteriitis nodosa.

Thrombose der Vena cava superior bei einem 9jährigen Kind ist beschrieben. Der klinische Verlauf wies auf die Möglichkeit einer nekrotisierenden Angioitis hin, und die Autopsie enthüllte die Anwesenheit einer Periarteriitis nodosa. Die Beziehung von Periarteriitis nodosa zur Thrombose der oberen Hohlvene infolge einer “unspezifischen Mediastinitis” wird einer Brörterung unterzogen.

Un caso de trombosis en la vena cava superior, asociada con periarteritis nodular.

Descríbese un caso de trombosis en la vena cava superior de un niño de 9 años de edad. La progresión clínica sugirió la posibilidad de angiítis necrótica, y la autopsia reveló periarteritis nodular. Discútese la relación existente entre la periarteritis nodular y la trombosis en la vena cava superior debida a “mediastinitis no específica”.
THE VENOUS PLASMA FREE AMINO ACID LEVELS DURING THE FIRST HOURS OF LIFE
Authors: LINDBLAD B. S. , RAHIMTOOLA RAZIA J. , KHAN NAFEES
Published: August 2010

The plasma aminogram was investigated in newborns from a low socio‐economical group of West Pakistan with an increased frequency of the “small for dates” syndrome.

The plasma levels of proline and glycine increased during the first hours of life to levels significantly higher than those of a Swedish normal material. The alanine levels showed a “resistance to decline” during this period. The branch‐chained amino acid levels of plasma showed a fast decline after a temporary “resistance to decline”.

The plasma free amino acid levels changed during the first hours of life to a pattern, which is the same as that seen in protein deficiency.
C‐REACTIVE PROTEIN IN SERUM FROM INFANTS AS DETERMINED WITH IMMUNODIFFUSION TECHNIQUES
Authors: SAXSTAD J. , NILSSON L.‐Å. , HANSON L. Å.
Published: August 2010

Using sensitive, and accurate immunodiffusion methods CRP could be demonstrated in 17 of 98 infants apparently healthy upon clinical examination. Thirteen of these sera only contained trace amounts of CRP (0.5‐1 μg/ml). The only case which had quantifiable amounts of CRP and which did not become negative upon control turned out to have kidney abnormalities and pyelonephritis.
CLINICAL AND BIOCHEMICAL‐GENETIC ASPECTS OF INTERMITTENT BRANCHED‐CHAIN KETOACIDURIA
Authors: GOEDDE H. W. , LANGENBECK U. , BRACKERTZ D. , KELLER W. , ROKKONES T. , HALVORSEN S. , KIIL RAGNHILD , MERTON BRITA
Published: August 2010

Intermittent branched‐chain ketoaciduria was detected in two Norwegian families. All three patients had normal intelligence. One died during an acute acidotic attack at the age of 8 years.

The biochemical and clinical data in the two families suggest a separate variant of branched chain ketoaciduria. Possible mechanisms of genetic heterogeneity in this disorder are discussed.
BIOCHEMICAL DIAGNOSIS OF CYSTINOSIS USING LEUCOCYTES
Authors: WILLCOX P. , PATRICK A. D.
Published: August 2010

Abstract. Willcox, P. and Patrick, A. D. (Institute of Child Health, London, England). Biochemical Diagnosis of Cystinosis using leucocytes. Acta Paediatr Scand 64:132, 1975. –A simple method for the biochemical diagnosis of cystinosis involves incubating leucocytes from a small volume of blood with 35S‐cystine. Non‐protein 35S‐IabeIled compounds are then extracted from the leucocytes and separated by thin‐layer chromatography. The excessive incorporation of 35S‐cystine into the increased cystine pool of cystine cells is easily detected in autoradiography of the chromatograms.
SUDDEN INFANT DEATH SYNDROME IN SWEDEN IN 1973–1977 AND 1979
Authors: Norvenius S. Gunnar
Published: August 2010

The aim of the investigation has been to describe Sudden Infant Death Syndrome, SIDS, in Sweden, its background, true incidence and epidemiological characteristics, and finally to offer possibilities of prophylaxis.

The method used has entailed a retrospective survey of all 1,873 post‐perinatal (1 ‐ 51 weeks) deaths from six birth cohorts in 1973–1977 and 1979. Through comprehensive documentation, 334 deaths have been isolated as SIDS.

The result was an incidence of 0.54/1000 live births. Internationally, this incidence is low, but the study could establish that SIDS increased during 1973–1977 and 1979 in Sweden. The epidemiological characteristics shown by the study agree to an extent with American and British investigations, but reported Swedish incidence i s lower. An assessment of the total post‐perinatal mortality has also been made to demonstrate that there is no communication between SIDS with a uniform diagnosis and other causes of death. Analysis has been binary: in relation t o factors in society, and on an individual basis. Geographical or social bases did not affect incidence of SIDS in Sweden, which is remarkable when comparing these findings with other reports on the subject.

In regards to individuals, the analysis has been based upon the place where the infant died.

Deaths in the infant's own bed showed a clear rise on Saturdays, but the incidence here remained constant over the period studied. Deaths in bed with one/two adults showed epidemiological findings which indicated disadvantageous family and social factors in many cases; this group increased from year to year. Deaths outdoors were common in winter; a certain increase towards the end of the week as well as from year t o year was indicated. No social strain was shown. Deaths during car rides clearly increased from year to year, but showed no particular socio‐familial profile nor weekday variation.

Findings analyzing location of death have indicated that the different places have their own epidemiological characteristics distribution. Analyses of this sort are scarce. There were no certain indications of SIDS occuring more often in socially disadvantageous circumstances: 4/5 of the SIDS families had no social strain. The study has also included medico‐historical notes, e.g. ‘over‐laying’ and a description of epidemiological/pathological and forensic aspects of sudden infant death from Stockholm during the 1840s.

SIDS means that neither anamnesis nor a thorough autopsy can explain the death. SIDS may be considered a dysfunction syndrome, in which endogenous and exogenous factors act together and silently lead to death. These factors exist in the normal infant's development and in normal environments. The study has also shown that variables i n neonatal data or maternal factors cannot as yet be used as risk indicators. Generally a single factor may not be used to identify a child who is at increased risk of SIDS.

On the basis of the findings of the study, prophylactic advice is presented.
The Role of Hypothalamic Hormones in the Control of Growth Hormone Secretion and of Growth
Authors: FROHMAN L.A.
Published: August 2010

GHRH and somatostatin have major integrative roles in the control of GH secretion. Alterations in the secretion of each hypothalamic hormone have profound effects on GH secretion. On the basis of current information, it appears that disturbances in GHRH secretion provide a most convincing argument for the pathophysiological role of this hypothalamic hormone in clinically recognized disorders of GH secretion. Thus, the potential use of GHRH and its agonists and antagonists in the treatment of patients with both deficient and excessive GH secretion is based on a solid framework of physiological and pathophysiological studies.
Treatment of Turner's Syndrome with Recombinant Human Growth Hormone (Somatrem)
Authors: LIPPE B. , ROSENFELD002C R.G. , HINTZ R.L. , JOHANSON A.J. , FRANE J. , SHERMAN B. , GROUP GENENTECH COLLABORATIVE
Published: August 2010

This report extends to 3 years the prospective study of the effects of somatrem alone or in combination with oxandrolone on growth in Turner's syndrome. Sixty‐seven patients completed the 1‐year study period during which all treatment groups had statistically increased height velocities as compared to the control group. Oral glucose tolerance and insulin responses remained unchanged after 1 year of somatrem treatment. The group Receiving oxandrolone experienced an increase in integrated glucose response and the group receiving combined therapy an increase in both integrated glucose and insulin responses. During the second and third years the somatrem group remained on the same dose and treatment schedule and grew at mean velocities of 5.4 ± 1.1 and 4.6 ± 1.4 cmiyear. The dose of oxandrolone was reduced by 50% during the second and third years for the combination group. The somatrem dnse remained unchanged. This group had height velocities of 7.4 ± 1.4 cm and 6.1 ± 1.5 cm/year. The control group and the group treated with oxandrolone alone were converted to combined therapy at the lowered oxandrolone dose. Their growth rates during the second year were 8.3 ± 1.2 and 7.1 ± 1.6 cm/year, respectively. Using bone age determinations and the methods of Bayley and Pinneau, allgroups currently show predicted increases in final adult height.
Accelerated Growth Following Treatment of Children with Chronic Renal Failure with Recombinant Human Growth Hormone (Somatrem): a Preliminary Report
Authors: LIPPE B. , FINE R.N. , KOCH V.H. , SHERMAN B.M.
Published: August 2010

Five male children with chronic renal failure (CRF) and growth retardation were treated with recombinant human GH (somatrem) three times weekly for 6 months. The patients ranged in age from 35 to 91 months and had a mean SD score for height of ‐3.03 ± 1.0 at initiation of therapy. Their mean pretreatment height velocity was 4.94 ± 1.40 cm/year for the year prior to somatrem treatment. Following 6 months of treatment all children had a significant increase in annual height velocity, with the mean annual value for the group being 10.08 ± 1.97 cm/year (p <0.01). Glucose tolerance was monitored and was not significantly affected nor were there any other complications of note. These data indicate that somatrem can produce short‐term accelerated height velocity in the child with growth retardation associated with CRF, The long‐term benefits of therapy in this group of children is under investigation.
Postnatal Changes in Intracranial Blood Flow Velocity in Preterm Infants
Authors: WINBERG P. , SONESSON S. ‐E. , LUNDELL B. P. W.
Published: August 2010

ABSTRACT. Postnatal changes in intracranial arterial blood flow velocity, were studied in preterm infants of less than 34 weeks of gestation. The blood flow velocity was measured in an artery on the base of the skull, using a range‐gated Doppler ultrasound velocimeter. Ten healthy infants (mean gestational age 32.5 weeks), and ten infants with transitional respiratory disease (mean gestational age 31.3 weeks) were studied at 1, 2, 5, 24 h, and 2, 3, 5 and 10 days after birth. The healthy infants showed a consistent pattern of changes on the first day, with an average reduction in mean flow velocity of 29% between 1 and 5 h. At 24 h after birth, mean flow velocity had almost returned to the level of the 1 h recording. After 24 h there was a gradual increase in systolic and mean flow velocity until 10 days, while diastolic flow velocity remained unchanged. In the infants with respiratory disease there were no systematic changes in mean flow velocity on the first day, although large individual changes were seen. After 24 h no differences were seen between the healthy infants and the infants with respiratory disease. These findings indicate a transient decrease in cerebral perfusion during early circulatory transition in healthy preterm infants, and that mild to moderate respiratory disease causes larger individual variations in intracranial blood flow velocity.
Blindness and Partial Sight Due to Retinopathy of Prematurity in the Netherlands: 1975‐1987
Authors: CATS B. P. , TAN K. E. W. P.
Published: August 2010

ABSTRACT. In 1975–1987 at least 84 children developed blindness or impaired vision due to retinopathy of prematurity (ROP) in the Netherlands. Neonatal data were obtained on 74 (86%). Unexpectedly most children (40 i.e. 54%) had been in level I and II hospitals after birth. Screening for ROP had been either insufficient or not timed according to present recommendations in 33 out of 40 children in level I and II hospitals and 25 out of 34 in level III hospitals. As the number of children with ROP has not changed appreciably over the last 40 years, while birth rates in the Netherlands have steadily decreased, the incidence of blindness/impaired vision due to ROP has probably increased. In the current population at risk of ROP which differs considerably from the one during the first epidemic, the disease may not be completely preventable. We conclude that improvements in screening procedures and timely treatment are crucial in order to improve the present situation.
BOOK REVIEW
Authors: GREEVES L. G.
Published: August 2010

Book review in this articles:

Jordan SC, Scott O. Heart Disease in Paediatrics, 3 ed.
Design and Performance of Blanket Heat Shields for Neonates
Authors: WALTERSPIEL JUAN N.
Published: August 2010

ABSTRACT. Background: Several designs of plastic blanket heat shields are in use. This study was done to compare different designs for their efficiency in reducing heat loss. Methods: Four heat shield designs were tested by sequentially covering each of 14 infants (wt. 640–2030 g) cared for under radiant warmers. The power consumption of the radiant warmers was measured as a surrogate for. heat loss. All designs were tested for a total of 20 min on all infants. Results were calculated as percent change in power consumption from shield to shield. The most efficient design was further modified and evaluated in another group of 14 infants (wt. 700–1180 g). Results: The relative reductions in power consumption were: no shield (control) –0%, a plastic foil over the side rails: –17%, a single layer close to the infant but excluding the head: –34%, the same as double layer –37% and the most efficient one, a single layer covering the whole infant –42%. A modification of this design, tested in the second group of infants, reduced power consumption by 13% (95% CI –5.9/–19.7), (p < 0.004) when compared to the single layer covering the whole infant. It was tucked under the connecting tubes to the ventilator. It also reduced the risk for displacement and allowed for the endotracheal tube to be suctioned without removing the blanket. Conclusion: Modifications of the design of heat shield blankets for infants resulted in significant increases in efficiency.
Types of Fluid Disorder in Children with Bacterial Meningitis
Authors: LAINE J. , HOLMBERG C. , ANTTILA M. , PELTOLA H. , PERHEENTUPA J.
Published: August 2010

ABSTRACT. As part of a prospective study of children with bacterial meningitis we analyzed in 36 patients of our hospital the fluid balance on admission and during the first three days of treatment. On admission 10 of them (28%) had inappropriate antidiuretic hormone secretion SIADH, 10 (28%) hypo‐osmolal and 10 (28%) iso‐osmolal contraction. Six patients (17%) had no clear fluid disorder. The patients with SIADH had significantly lower mean serum NA’(127 vs. 132 mEq/l, p < 0.0l) and higher mean urine Na+ (111 vs. 26 mEq/l, p < 0.01) concentration as well as higher mean urinary Na+/K+ ratio (2.23 vs. 0.365, p< 0.005) than the patients with hypo‐osmolal contraction. They also tended to be younger and have a shorter history of fever. The patients with SIADH had a less strict fluid restriction than the patients with hypo‐osmolal contraction, and their fluid balance normalized more slowly. Our findings support initial water restriction for all children with bacterial meningitis.
Psychological and Respiratory Physiological Effects of a Physical Exercise Programme on Boys with Severe Asthma
Authors: ENGSTRÖM I. , FÄLLSTRÖM K. , KARLBERG E. , STEN G. , BJURE J.
Published: August 2010

ABSTRACT. Ten boys 9–12 years of age with severe perennial asthma participated in a physical exercise programme lasting 8 months. Pulmonary function and psychological tests were performed before training, immediately after, and one year after the end of the exercise programme. Static lung volumes, flow‐volume variables and histamine tolerance were used as indicators of pulmonary function. Ego structure, body image, social development and concentration capacity were used as indicators of personality development. Before the study, the group had high FRC (p < 0.05) and RV (p < 0.001), low FEV1, MEF50 and MEF25 (all p < 0.001) and low histamine tolerance. They showed marked disturbances in their personality development with low scores in psychological variables. During the training period, MEF50 and MEF25 increased slightly (p < 0.01). Marked improvement was observed in all psychological variables (p < 0.001). The positive effects remained during the following year. The marked and lasting improvement in personality development was regarded as an essential factor behind the more modest positive clinical and pulmonary function changes. The results emphasize the importance of including exercise programmes in the treatment of children with asthma.
Effects of recombinant human insulin‐like growth factor I administration in adults with growth hormone deficiency
Authors: Thorén M , Wivall‐Helleryd I‐L , Hall K
Published: August 2010

Recombinant human insulin‐like growth factor I (IGF‐I), 40 μg/kg/body weight, was administered subcutaneously at 08.00 hours to six adult patients with growth hormone deficiency (GHD). The mean maximal IGF‐I concentrations were found 2–6 hours after injection. Concentrations then gradually declined, though mean values were still above basal 24 hours after the injection. Only one patient maintained IGF‐I levels above the lower normal range throughout 24 hours. There was a significant decrease in mean IGF‐II concentrations when measured 4 and 24 hours after injection of IGF‐I. The diurnal variations of insulin and IGF binding protein‐1 were preserved. There were no side‐effects, and blood glucose remained normal. These results show that in patients with low IGF‐I levels resulting from GHD, it is necessary to administer IGF‐I at intervals of less than 24 hours to obtain IGF‐I levels that are within the normal range.
Prognostic impact of bone marrow karyotype in childhood acute lymphoblastic leukaemia: Swedish experiences 1986‐91
Authors: Forestier E , Gustafsson G , von Heideman A , Heim S , Hernell O , Mitelman F , Nordenson I , Swolin B , Söderhäll S
Published: August 2010

AbstractThe prognostic value of cytogenetic classification in acute lymphoblastic leukaemia (ALL) was evaluated in Swedish children below 16 years of age (n= 372) diagnosed between 1986 and 1991. A bone marrow karyotype was obtained in 281 cases, of which 149 (53%) showed clonal abnormalities. Event‐free survival (p‐EFS) was 0.64–0.69 in patients with diploid and pseudodiploid karyotype. Patients with massive hyperdiploidy (> 50 chromosomes) had the best outcome (p‐EFS = 0.76) and those with hypodiploidy (< 46 chromosomes) had the worst (p‐EFS = 0.33). White blood cell count and age were the strongest predictors of outcome. The karyotype reached borderline significance. The diagnostic karyotype was also a predictor of outcome after relapse, with hyperdiploid patients doing better than the others. The presence of a structural chromosomal abnormality did not constitute a negative prognostic factor when intensive chemotherapy was given.
Endothelium‐derived relaxing and contracting factors during the early neonatal period
Authors: Endo A , Ayusawa M , Minato M , Takada M , Takahashi S , Harada K
Published: August 2010

AbstractThe interaction and relative potency of nitric oxide, an endothelium‐derived relaxing factor, and endothelin‐1, an endothelium‐derived contracting factor, may be important in the transition from fetal to extrauterine life. The change in level of nitric oxide during the early neonatal period has not been measured. Accordingly, the serum levels of nitric oxide metabolites (the sum of nitrite and nitrate) and plasma endothelin‐1 were determined in 20 healthy neonates at birth, at 12 and 24 h postnatally, and at the age of 5 d. The lowest serum concentration of nitric oxide metabolites was observed at birth (26.2 ± 9.1 µmol 1‐1, mean ± SD) and increased with age, whereas the highest plasma concentration of endothelin‐1 was observed at birth (14.0 ± 6.7 pg ml‐1) and decreased with age. These changes suggest that nitric oxide and endothelin‐1 play roles in the circulatory adaptation of the neonate to extrauterine life.
Inaccuracy of portable peak flow meters: correction is not needed
Authors: Brand PLP , Waalkens HJ , Duiverman EJ , van Essen‐Zandvliet EEM , Group Dutch CNSLD Study
Published: August 2010

Abstract This study examined whether correction of peak expiratory flow (PEF) values for the inaccuracy of the meter would affect asthma management in 102 children (7–14 y old). PEF was recorded with a mini Wright meter twice daily for 2 weeks. As expected, measured PEF overestimated PEF level and asthma control in these children on many diary days. The actual numerical differences between measured and corrected PEF on these days were very small (>5% in only five patients, maximum 10%). It is unlikely that such small changes in PEF justify changes in asthma management, even if these changes cause PEF levels to cross arbitrary borders between various levels of asthma control used in self‐management plans. The clinical importance of the inaccuracy of portable PEF meters is negligible.
Selective compartmentalization of γσ‐T lymphocytes in human breastmilk
Authors: Lindstrand A , Smedman L , Gunnlaugsson G , Troye‐Blomberg M
Published: August 2010

Abstract In human breastmilk, T lymphocytes with γδ‐receptor (TCR) are more frequent than those with αβ‐TCR, in comparison with peripheral blood. Differential representation has also been demonstrated for subpopulations of γδ‐T cells. Reactivity was visualized with three monoclonal antibodies against Vδ1, Vδ2 and Vγ2 on T cells from the breastmilk and peripheral blood of 12 women who had recently given birth. Confirming the results with Vδ1, it was found that Vδ1 (p < 0.01) and Vγ2 (p < 0.05) but not Vδ2 were overrepresented on T cells in milk as compared with blood. This selective compartmentalization seems to reflect the homing of certain cells to the mammary gland.
Helicobacter pylori infection in children
Authors: Vandenplas Y , Blecker U
Published: August 2010

Helicobacter pylori colonizes the human stomach, especially during childhood. However, a variety ofH. pylori strains exists, with major differences in virulence characteristics which probably account for different clinical symptoms, and the majority of infected subjects remains asymptomatic. Helicobacter pylori infection is correlated with socioeconomic conditions and hygienic circumstances, resulting in an extremely high prevalence in children in developing countries. Commercial screening tests are not capable of separating the more virulent strains (type I with vacuolating toxin VacA and CagA protein) from the less virulent strains (type II, VacA and CagA negative). Type I strains, but not type II, are associated with an increased risk for duodenal ulcer and gastric cancer. Therefore, future screening tests and vaccinations should focus on the type I strains.
Transient trimethylaminuria in childhood
Authors: Mayatepek E , Kohlmüller D
Published: August 2010

Trimethylaminuria, also called fish‐odour syndrome, is an inherited disorder caused by deficient N‐oxidation and increased excretion of trimethylamine. This study reports on two unrelated and otherwise healthy children with transient trimethylaminuria, a hitherto unknown abnormality, without N‐oxidation deficiency. This demonstrates that a diagnosis offish‐odour syndrome should include the analysis of urinary excretion not only of trimethylamine but also of trimethylamine‐N‐oxide. Since transient trimethylaminuria may be a common condition and no cause for it can presently be recognized, such patients will require careful follow‐up.
Growth and development four years after treatment for the Trichuris dysentery syndrome
Authors: Callender JE , Walker SP , Grantham‐McGregor SM , Cooper ES
Published: August 2010

A follow‐up study is reported of 18 children 4 y after treatment for the Trichuris dysentery syndrome (TDS) and matched control children. The TDS children were initially severely stunted and had extremely low developmental levels. They showed catch‐up in height of 1.9 z‐scores even though they remained in very poor environments. Their intelligence quotients, school achievement and cognitive function remained significantly lower than those of the controls. Controlling for their earlier developmental levels, the TDS children showed a small improvement in mental development relative to the controls.
At risk for language disorders? Correlates and course of language disorders in preschool children born at risk
Authors: Weindrich D , Jennen‐Steinmetz Ch , Laucht M , Esser G , Schmidt M‘H
Published: August 2010

The language abilities of 324 children of an at‐risk population were investigated at age 2 and 4.5 y. Modified research criteria of the ICD‐10 for specific developmental disorders of speech and language were applied. Frequencies between 4% and 7%, depending on age and type of disorder, were diagnosed among children whose performance on the language measure was only 1 instead of ICD‐10′s 2 SD below group mean, but the discrepancy measure of 1 SD between non‐verbal language score and language measure was retained. Psychosocial aspects of a child's environment proved to be better predictors of later language disorders than obstetric complications. Stability of specific language disorders was on the whole fairly low, but children who perform below age level on language measures remained at risk. Gender differences are almost compensated by the age 4.5 y.
Pseudotumour cerebri complicating varicella in a child
Authors: Lahat E , Leshem M , Barzilai A
Published: August 2010

Pseudotumour cerebri is a clinical syndrome characterized by increased intracranial pressure in the absence of intracranial pathology. The possible association between this condition and infections has already been mentioned in a few case series. This paper describes a 6‐y‐old girl with a classical picture of pseudotumour cerebri which developed 1 week following varicella. To the authors' knowledge, this is the first description suggesting this association. Additional reports are required to support the possible relationship between the two conditions.
Fatty acid balance studies in term infants fed formula milk containing long‐chain polyunsaturated fatty acids
Authors: Morgan C , Davies L , Corcoran F , Stammers J , Colley J , Spencer SA , Hull D
Published: August 2010

Long‐chain polyunsaturated fatty acids (LCP) are thought to be required for optimal nervous system development in the newborn. A commercial milk formula containing LCP (Aptamil‐LCP) with a fatty acid profile closely resembling breast milk, has recently been introduced for term infants. The absorption of fatty acids in term infants was examined in a double‐blind randomized controlled trial comparing Aptamil‐LCP (n= 20) and standard Aptamil (n= 20). Formula‐fed newborn infants were studied from birth for 14 d. Fat balances (3 d) were performed from d 10. A 3‐d stool collection was performed from d 10 in a parallel breastfed group (n= 21). Plasma samples were taken on d 6. Median fat excretion (mg kg−1) was 897.1, 615.0 and 355.2 with Aptamil, Aptamil‐LCP and breastfeeding, respectively. The median total fat absorption coefficient in Aptamil‐LCP‐fed infants was higher than in those fed standard Aptamil (p < 0:01). These findings were accounted for by differences in the excretion and absorption of long‐chain saturated fatty acids (C14:0, C16:0 and C18:0). Higher fat excretion was associated with bulkier and firmer stools. Only trace amounts of LCP were detected in the stools of all groups. This accounted for less than 4% of dietary intake in Aptamil‐LCP‐fed infants. No differences in the utilization of LCP from Aptamil‐LCP and breast milk feeding were apparent. Plasma phospholipid fatty acid composition data reflected differences in dietary LCP intake. Thus, PL LCP levels were highest in the breastfed infants and lowest in the Aptamil‐fed infants, with values for the Aptamil‐LCP‐fed group falling in between.