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Cancer is the second leading cause of death among children aged 5 to 14, after accidents. We conducted a study on the epidemiology of childhood cancer in the university pediatric oncology department of the CHU-CHR in Liège, Belgium. We studied a cohort of 662 patients between the ages of 0 and 17 whose malignancy diagnosis was made between 1985 and 2016. The analyzes were performed retrospectively using medical files. The number of new cases, the proportion of different cancers, sex ratio, age at diagnosis and survival at 5 and 10 years were the epidemiological factors studied.We have been able to show an increase in the number of new diagnoses per year. More than 40 % of childhood cancers occur before the age of five. The most common neoplasias are leukemias, tumors of the central nervous system and lymphomas. This distribution is influenced by age. All malignant tumours combined, we observed a slightly larger proportion of affected boys than girls. Overall survival at 5 years reaches 80.2 %. However, it varies according to the type of tumour from 59.3 % for malignant soft tissue tumors up to 100 % for hepatoblastomas.Le cancer est la deuxième cause de décès chez les enfants de 5 à 14 ans, après les accidents. Nous avons réalisé une étude sur l’épidémiologie des cancers de l’enfant au sein du service universitaire d’oncologie pédiatrique du CHU-CHR de Liège. Nous avons étudié une cohorte de 662 patients, âgés de 0 à 17 ans, dont le diagnostic de tumeur maligne a été posé entre 1985 et 2016. Le nombre de nouveaux cas, la proportion des différents cancers, le sex ratio, l’âge au diagnostic et la survie à 5 et 10 ans ont été les facteurs épidémiologiques étudiés. Nous avons pu démontrer une augmentation du nombre de nouveaux diagnostics par an. Plus de 40 % des cancers de l’enfant surviennent avant l’âge de 5 ans. Les néoplasies les plus fréquentes sont les leucémies, les tumeurs du système nerveux central et les lymphomes. Cette répartition est néanmoins influencée par l’âge. Toutes tumeurs malignes confondues, nous avons observé une proportion légèrement plus grande de garçons atteints que de filles. La survie globale à 5 ans s’élève à 80,2 %. Elle varie cependant selon le type de tumeur de 59,3 % pour les tumeurs malignes des tissus mous jusqu’à 100 % pour les hépatoblastomes.

Bone pain associated with bone marrow infiltration is often present at diagnosis of pediatric acute lymphoblastic leukemia (ALL). It sometimes signs the presence of pathological fracture, lytic lesions, arthritis, or osteitis associated to ALL that can delay the diagnosis. During treatment, bone complications (pain, osteopenia, fracture, avascular necrosis, ...) are also reported. In order to describe bone involvement (BI) of pediatric LLA, we reviewed the records of 104 patients followed in our unit. The overall incidence of BI was 67 %. At diagnosis, 50 % of patients had BI and in 19 %, the diagnosis of ALL was delayed. During and after treatment, respectively 28 % and 37 % of patients presented bone complications (pain, fractures, avascular necrosis, osteopenia). Patients with BI had a lower leukocytosis inferior to 10x109/l (p = 0.005) and an ALL of average risk (p = 0.019). 38 % of patients with BI during treatment were over 10 years old and 55 % were girls (vs. 21 % and 38 % in the entire cohort, respectively). Osteoporosis was more severe at diagnosis than during treatment, suggesting the presence of constitutional promoting factors. In our cohort, the majority of BI was resolved at the end of treatment with no long-term sequelae.Des douleurs osseuses sont souvent présentes au diagnostic des leucémies lymphoblastiques aiguës (LLA) pédiatriques. Elles signent aussi parfois la présence de fracture pathologique, de lésions lytiques, d’arthrite, ou d’ostéite associées qui peuvent retarder le diagnostic. Lors des traitements, des complications osseuses (douleurs, ostéopénie, fracture, nécrose avasculaire, …) sont également rapportées. Afin de décrire les atteintes osseuses (AO) des LLA pédiatriques, nous avons revu les dossiers de 104 patients suivis dans notre unité. L’incidence globale des AO était de 67 %. Au diagnostic, 50 % des patients avaient une AO et, chez 19 % d’entre eux, le diagnostic de LLA a été retardé. Pendant et après les traitements, respectivement 28 et 37 % des patients ont présenté des complications osseuses (douleurs, fractures, nécrose avasculaire, ostéopénie). Les patients avec AO avaient une leucocytose plus basse inf�rieur a 10x109/l (p = 0,005) et une LLA de risque moyen (p = 0,019). Chez les patients avec AO pendant les traitements, 38 % avaient plus de 10 ans et 55 % étaient des filles (vs 21 % et 38 % dans la cohorte entière). L’ostéoporose était plus sévère au diagnostic que pendant les traitements, suggérant la présence de facteurs favorisants constitutionnels. Dans notre cohorte, la majorité des AO étaient résolues après les traitements de LLA sans séquelles à long terme.

Objective To describe the epidemiology of Guyana9s road traffic injuries and perform the first geocoding of road traffic injuries in this setting. Methods This was a registry-based retrospective cross-sectional study investigating collisions resulting in serious and fatal injuries. Police reports from two police divisions were used to identify victim, second party (ie, non-victim) and collision characteristics of all serious and fatal collisions between January 2012 and June 2015. Collisions with available location data were geocoded using Geographic Information Systems. Distributions of characteristics were compared for urban and rural areas. Multivariable logistic regression was used to assess variables associated with fatal collisions. Results The study included 751 collisions, resulting in 1002 seriously or fatally injured victims. Fatally injured victims tended to be older, male and either pedestrians or cyclists. Fatal collisions tended to take place in rural areas, occur on weekends and involve speeding. Fifty-three per cent of fatalities occurred due to non-motorised road users being struck by motorised road users, and the most common fatal collision type was between pedestrians and motor vehicles (35%). The distribution of collisions was similar for urban (43.8%) and rural (56.2%) areas. Fatal collisions were more likely to occur in rural settings. Conclusions Road traffic injuries pose a considerable public health burden in Guyana. These results suggest a pattern of high mortality in rural collisions and a disproportionate burden of injuries on vulnerable road users. The spatial distribution of collisions should be considered in order to target interventions and improve road traffic safety.

Treating chronic neuropathic pain remains a challenge, despite the existing therapies. Recent years have seen the emergence of promising new technologies, such as the neurostimulation of the dorsal root ganglion (DRG). In the present article, we review the clinical evidence for the efficacy and safety of DRG neurostimulation in the treatment of chronic pain. While the results from a number of small observational studies are promising, it is not yet possible to conclude on the long-term effectiveness and safety of DRG stimulation and it is too early to recommend its widespread use outside of a research protocol. To improve the level of proof, larger randomized controlled trials are needed. These should include well-described populations, a sufficiently long follow-up and a detailed description of concurrent treatments (pharmacologic and patient integration in a multidisciplinary approach).

Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency in Dutch neonates.Spaapen LJ, Bakker JA, Velter C, Loots W, Rubio-Gonzalbo ME, Forget PP, Dorland L, De Koning TJ, Poll-The BT, Ploos van Amstel HK, Bekhof J, Blau N, Duran M.Department of Biochemical Genetics, Stichting Klinische Genetica Zuid-Oost Nederland, Maastricht. leo.spaapen@gen.unimaas.nlFour neonates with a positive phenylalanine screening test (Phe concentrations between 258 and 1250 micromol/L) were investigated further to differentiate between phenylalanine hydroxylase (PAH) deficiency and variant hyperphenylalaninaemia (HPA) forms. In patients 1 and 2 a tetrahydrobiopterin (BH4) load caused a significant decrease of the plasma Phe levels. A combined phenylalanine/BH4 loading test was performed in patients 2, 3 and 4. In the latter two patients, plasma Phe concentrations completely normalized within 8 h after the BH4 load (20 mg/kg). Basal urinary pterins were normal in all four patients. The activity of dihydropteridine reductase (DHPR) was normal in patients 1, 2 and 3 and 50% of control values in patient 4 (not in the range of DHPR-deficient patients). In patient 3 a subsequent phenylalanine loading test with concomitant analysis of plasma biopterins revealed a normal increase of biopterin, excluding a BH4 biosynthesis defect. Pterins and neurotransmitter metabolites in CSF of patients 1, 3 and 4 were normal. DNA mutations detected in the PAH gene of patients 1-4 were A313T, and L367fsinsC; V190A and R243X; A300S and A403V; R241C and A403V. The results are suggestive for mutant PAH enzymes with decreased affinity for the cofactor BH4.

Acid steatocrit during infancy.Van den Neucker A, Forget P, Veneberg JA, Schutten BJ, van Kreel B.Department of Paediatrics and Chemistry, University Hospital of Maastricht, The Netherlands.The faecal acid steatocrit is an improved steatocrit method for the evaluation of faecal fat. The present study was set up in order to establish reference values during the first year of life. Faecal acid steatocrit values were determined in 58 healthy full term and in 16 healthy prematurely born infants. Very high acid steatocrit results (above 60%) were found in all premature and many formula-fed term infants during the first 6 months of age, thereafter with values below 10%. In 40- to 120-day-old infants acid steatocrit results of human milk-fed infants were significantly lower than those of formula-fed infants (p < 0.01). We conclude that high acid steatocrit results after the age of 6 months are indicative of fat malabsorption, while before this age high values can be due to "physiological steatorrhoea". The acid steatocrit should be useful for the evaluation of milk fat absorption in infants.

Background Intimate partner violence (IPV) is prevalent throughout the world and is a devastating public health problem. Healthcare workers (HCWs) are tasked with treating victims of IPV but may be victims themselves. Guyana is a lower-middle income country in South America. This study sought to determine the knowledge and attitudes of Guyanese HCWs and their perceived barriers to providing care in addition to determining the prevalence of IPV victimization and perpetration among HCWs. Methods HCWs at the only tertiary care hospital in the Guyana completed an anonymous survey that comprised 30 questions relating to IPV. Results The survey was completed by 87.5% of eligible HCWs. Of the respondents, 81.8% were female, 49.9% had ever experienced abuse and 21% admitted to perpetrating violence. Multivariate analysis found that the age groups 31-40 years (OR 2.3, 95% CI 1.1-4.6) and 41-50 years (OR 2.3, 95% CI 1.2-4.7) had higher odds of accepting justification for physical violence, and so did nursing staff (OR 4.3, 95% CI 1.4-13.1). Overall, 29.9% of HCWs accepted justification for physical violence in at least one of the named scenarios. Conclusion This study demonstrates a high prevalence of IPV among HCWs and identifies prevailing attitudes regarding IPV. This knowledge is essential in developing effective, appropriate training programs and identifies a need to address IPV among the healthcare workforce. Language: en

Lipid, nitrogen, water and energy content of a single stool sample in healthy children and children with cystic fibrosis.Van den Neucker AM, Forget PP, van Kreel B.Department of Paediatrics, University Hospital Maastricht, P Debyelaan 25, 6229 HX Maastricht, The Netherlands. AVN@paed.azm.nlTo evaluate the usefulness of "single stool sample" analysis in the investigation of steatorrhoea instead of 72-h stool collection, we examined 57 stool samples of patients with cystic fibrosis (CF) while on pancreatic enzyme therapy and 29 stool samples of healthy children. We compared results of fat, nitrogen, water and energy content. Fat was determined by the method of Van de Kamer et al. and the Acid Steatocrit (AS) method, water by vacuum drying, nitrogen by the Dumas method and energy was obtained using a bomb calorimeter. Results (median) for CF patients and healthy controls were significantly different (P< or =0.0001) for fat respectively: 8.90 and 4.75/100 g wet weight (ww) stool as measured by the method of Van de Kamer et al. and 28.6% and 7.2% by the AS method. Energy results for energy were also significantly (P< or =0.0001) different: 742.96 and 549.32 kJ/100 g ww stool for CF patients and healthy controls respectively. Results of single stool sample water and nitrogen were similar in both groups. These results were comparable to those of a 3-day stool collection, as reported in the literature. CONCLUSION: Results from single stool samples yield clinically useful information concerning fat and energy. Single stool sample analysis is therefore useful for frequent monitoring of faecal fat and energy

The combination of Pleth Variability Index (PVI) and passive leg raising (PLR)-induced pulse pressure variation may help to diagnose hypovolemia in spontaneously breathing patients. In 44 subjects, PVI and Pulse Pressure (PP) variation after PLR were measured before and after induced hypovolemia (blood gift or hemodialysis session). PVI values were significantly greater after hemodialysis session or blood gift (22% vs 18%, P = 0.03); in contrast PP variation did not change significantly (7% vs 4%, P = 0.49). The accuracy of these parameters or of their combination to identify the "after hypovolemia induction" period was weak. In spontaneous ventilation, PVI value is greater after induced hypovolemia, whereas PP variation does not change significantly. The combination of PVI and PLR does not improve the accuracy of the detection of induced hypovolemia.

OBJECTIVES: To assess the collective hearing hazard entailed by occupational noise exposure. METHODS: The Late Audio-Sonometric Index (LASI) is a global score recommended for following up hearing loss in groups of workers exposed to traumatic noise, combining: daily noise exposure level (L(EX,8h) in dB(A)); individual hearing threshold level at 3kHz (HTL (3kHz) in dB); HTL(0)(3)(kHz) targeted at retirement; and 5 (or 10) years age bracket between 50 and 59 years of age. LASI=HTL(0)(3kHz) - HTL (3kHz)+10 log (age - 20) - 10 log (0.23×L(EX,8h) - 17.36). RESULTS: When L(EX,8h) exceeds 80dB(A) and HTL(0)(3kHz) is at 25dB, occasional audiometric surveillance may be recommended for workers exposed to hazardous impulse noise: e.g., should the median and the mean LASI values fall below 20 for men or 23 for women (indicative threshold values). CONCLUSION: This exponential model thus integrates the fact that daily exposure to impulse noise may be more harmful for hearing than eight hours exposure to continuous noise of equivalent energy. Language: en

In order to illustrate epidemiologic features and survival rate, 31 Wilm's tumours treated in our institution have been retrospectively studied. The mean age at diagnosis in our series was surprisingly higher than usually described: 25% of the patients were older than 8 years. Moreover, a mesoblastic nephroma congenital kidney tumour--appeared in a 10 month old girl. Symptoms were usually abdominal mass or gross hematuria. Young children are often brought to medical attention because of abdominal swelling: a large flank mass was palpable in all children under 5 years. Our study shows that preoperative chemotherapy results in a less intensive treatment regimen conducting probably to less sequellae. Event free survival and overall survival were respectively 87% and 94% at 10 years after diagnosis. Renal insufficiency was the most important side effect: 2 children required renal transplantation.

Department of Pediatrics, University Hospital, Maastricht, The Netherlands.Magnetic resonance cholangiopancreaticography (MRCP) was used to visualize the biliary tract in two children, aged 7 weeks and 10 years, with a choledochal cyst. MRCP was successful in both cases and the findings were confirmed by intraoperative cholangiography.

We report the successful use of nasal intermittent positive pressure ventilation (NIPPV) in the perioperative period of a 51 yr-old woman with a type II spinal muscular atrophy (SMA II). The patient was treated chronically with nocturnal NIPPV at home and scheduled for endoscopic retrograde cholangiopancreatography (ERCP) under general anesthesia. Some criteria of difficult intubation were present (forced mouth opening of 1.5 cm, short neck and thyromental distance of 5 cm). Nasal endotracheal fiberoptic intubation during spontaneous breathing under sedation with propofol was performed. The ERCP procedure was conducted without complications. At the end of the procedure, IPPV was maintained until recovery of respiratory function. After extubation, NIPPV was continued in the recovery room. The patient was discharged from the post-anesthesia care unit 4 hours after the procedure. Management of patients with SMA remains a challenge and clinicians must be aware that the use of NIPPV may be a useful and life-saving tool in the perioperative period for these patients.

Making use of ({sup 51}Cr)EDTA as a permeability marker, we measured intestinal permeability in a group of 20 children with proven cow's milk intolerance (CMI), a group of 17 children with similar complaints where CMI was excluded (sick controls), and a group of 12 control children. ({sup 51}Cr)EDTA test results (mean +/- SD) were 6.85 +/- 3.64%, 3.42 +/- 0.94%, and 2.61 +/- 0.67% in the group with CMI, the sick control, and the control group, respectively. When compared to both control groups, patients with cow's milk intolerance (CMI) showed a significantly increased small bowel permeability. We conclude that the ({sup 51}Cr)EDTA test can be helpful for the diagnosis of cow's milk intolerance.

We present the experience of the Citadelle Hospital (Liege, B) in the diagnosis, treatment and follow-up of medulloblastoma in children. A retrospective study of 10 cases of medulloblastoma was performed. Five years after diagnosis, the event-free survival was 77%.

In this retrospective study, we analyse epidemiology, clinical symptoms and therapeutic results in a group of 23 children with neuroblastoma. Half of them were less than 2 years of age; in 19 of 23, the primitive tumour was abdominal; 35% of them were initially metastatic. The overall survival was 83% at 5 years and the event free survival, 75% at 5 years. Pronostic factors are age, extension of the disease at diagnosis, biologic parameters and genetic study of the neuroblast cells (amplification of N-myc oncogen). Our study shows the deleterious effect of bone lesions.

We report our experience over the last seventeen years (1985-2002) of the treatment of acute lymphoblastic leukemia (ALL) in children at the University of Liege Pediatric Department of Hematogy-Oncology (CHU-Sart Tilman and CHR-Citadelle). Seventy seven children are enrolled in our study; the mean follow-up is 6.7 years. The 5 years over all survival and the disease free survival for the entire group are respectively 83% and 79%. Prognostic factors shown in our study are sex, high white blood cells counts at diagnosis and immunophenotypes.

This study compared the acid steatocrit (AS) results of healthy children with those of sick children with and without gastrointestinal involvement. Stool samples of 166 children were investigated, comprising 50 healthy children, 26 asthma patients, and 90 patients with gastrointestinal problems divided into 34 treated cystic fibrosis (CF) patients with exocrine pancreatic insufficiency, 16 untreated coeliac disease (CD) patients and 40 patients with various gastrointestinal problems. The median values (5th-95th percentile) of AS results were 3.3% (0.0-21%) for healthy children, 4.5% (1.8-22.5%) for asthma patients, 24.7% (2.6-68.2%) for treated CF patients with exocrine pancreatic insufficiency, 19.8% (3-77.7%) for untreated CD patients and 5.5% (1.8-29%) for patients with various gastrointestinal diseases.The AS results of treated CF patients with exocrine pancreatic insufficiency and untreated CD patients were similar and significantly higher than those of healthy children and asthma patients. AS can be considered to be a reliable tool in screening for steatorrhoea in paediatric patients.

SUMMARY. In this prospective open study of 14 children with cystic fibrosis (CF), we evaluated the effect of 1 year adjuvant therapy with lansoprazole, a proton pump inhibitor (PPI), on growth, fecal fat loss, body composition and lung function. Only stable patients with pancreatic insufficiency were included, and their data were compared to those of a large Dutch pediatric normal reference population. During the use of the PPI, mean weight and height did not change significantly, while body mass index improved (P0.05). An immediate significant and persistent reduction of fecal acid steatocrit (P0.05) was demonstrated. Compared to normal Dutch children, the CF patients showed significantly decreased standard deviation scores (SDS) for total body fat (TBF, -0.966) and fat-free mass (FFM, -1.826). Under lansoprazole, TBF improved significantly (P0.05), while mean FFM remained unchanged. A significant improvement in total lung capacity (P0.05), residual volume (P = 0.055), and maximal inspiratory mouth pressure (P = 0.002) was also demonstrated. Hyperinflation tended to decrease during the use of a PPI. Daily recordings of peak expiratory flow (PEF) showed a maximal diurnal variability of 28% of recent best PEF and minimal morning PEF of 72% of recent best PEF, confirming that bronchial hyperresponsiveness is increased in CF. We conclude that adjuvant therapy with lansoprazole in young CF patients with persistent fat malabsorption, decreased fat losses and improved total body fat. Lung hyperinflation decreased, which may partly explain the improvement in inspiratory muscle performance. The simultaneous improvements in body composition and lung hyperinflation suggest a relationship between these two parameters. Further research is necessary to confirm such a relationship and to elucidate the mechanisms involved.

Elevated NO production mediated by activation of the enzyme iNOS is thought to play a central role in the development of tissue damage observed during septic shock. IFN-gamma, TNF-alpha and IL-10 have been shown to be involved in the regulation of LPS-induced serum levels of the NO-oxidation products nitrate and nitrite. Therefore, in the present study, we investigated the role of endogenous IFN-gamma, TNF-alpha and IL-10 in the regulation of LPS-induced tissue iNOS expression in the major organs. To this end, mice were pre-treated with anti-IFN-gamma, anti-TNF-alpha, anti-IL-10 monoclonal antibodies, or combinations of these, two hours before intraperitoneal LPS-challenge. Immunohistochemical staining for iNOS and determination of iNOS activity indicated that iNOS expression was mainly upregulated in the small intestine, lung and heart, and that IFN-gamma, TNF-alpha as well as IL-10 are involved in the regulation of iNOS expression and enzyme activity. Whereas blocking either IFN-gamma or TNF-alpha did not affect iNOS expression, iNOS enzymatic activity seems to be inhibited. In contrast, blocking both mediators nearly completely prevents iNOS expression after LPS challenge, suggesting that the presence of either IFN-gamma or TNF-alpha is essential for LPS-induced iNOS expression in these organs. Combined treatment of these monoclonal antibodies revealed that whereas on the one hand IL-10 inhibits LPS-induced iNOS expression, on the other hand IL-10 or an IL-10 inducible factor is also involved in the upregulation of iNOS expression after LPS challenge.

We evaluated the reliability and immediate results of celioscopic management of acute small bowel obstruction. From January 1995 to April 1998, 39 patients underwent a primary celioscopic procedure for small bowel obstruction. The most common etiology was post operative adhesions (34 patients). The whole operation could be carried out exclusively by celioscopy in 22 patients (56%). A laparotomy had to be performed in 17 patients due to: impossibility to identify or treat the cause of obstruction, bowel necrosis or intraoperative complication (3 bowel wounds). Post operative complications were: 1 death (not directly related to the surgical procedure), 2 early recurrences of obstruction after exclusive celioscopy, 1 evisceration after laparotomy and 1 small bowel fistula after conversion to laparotomy. Mean hospital stay was 5 days after exclusive celioscopy and 9.5 days after conversion to laparotomy. Celioscopic management of small bowel obstruction is feasible, but it is often difficult and may be hazardous; a careful selection of patients must be made, based on the importance of obstruction and the type of previous abdominal surgery.

Nitric oxide (NO) production catalyzed by iNOS (inducible NO synthase) is thought to take place mainly in macrophages after activation by inflammatory mediators. NO is subsequently oxidized to nitrite and nitrate, which are excreted in urine. The concentration of inflammatory mediators in small bowel biopsy specimens from patients with coeliac disease is increased. The latter could induce increased NO production by stimulation of intestinal macrophage iNOS, resulting in high levels of urinary NO oxidation products, nitrite and nitrate (NOx).In the present study we evaluated the urinary NOx/creatinine ratios in children with active coeliac disease (n = 22), coeliac disease patients on a gluten-free diet (n = 9), healthy (n = 11) and sick control children (n = 18).The Griess reagent method was used for measuring urinary NOx.Median NOx/creatinine ratios of active coeliac disease patients, coeliac disease patients on a gluten-free diet, healthy and sick control patients were 1.21, 0.19, 0.10 and 0.13 mmol/mmol, respectively. All active coeliac disease patients showed increased NOx/ creatinine ratios. Urinary NOx/creatinine ratios of the active coeliac disease patients were significantly higher than those of healthy controls (p0.0001), sick controls (p0.0001) and coeliac disease patients on a gluten-free diet (p0.0001).The urinary NOx/creatinine ratio is increased in patients with active coeliac disease and reverts to normal on a gluten-free diet.

This retrospective study evaluates long-term growth of children with congenital heart disease (CHD) and looks for possible relationships between postsurgical catch-up growth and both severity of preoperative growth failure and operation age. Growth data of 123 children with isolated CHD were available. Mean z-scores and 95% confidence intervals for weight, height and weight-for-height were plotted for age-periods as well as for pre- and postoperative periods. Growth of children with a large VSD or a Tetralogy of Fallot was most abnormal and improved but did not normalize after operation. Catch-up growth for length was strongly correlated with severity of the preoperative growth failure (r = 0.92, p0.05) but not with operation age (r = 0.20, NS). We conclude that surgical correction results in catch-up growth for most individuals. Catch-up growth is positively correlated with the severity of the initial growth disturbance and not with age at the moment of surgical correction.

To compare three techniques of brachial plexus blockade for emergency surgery of the upper limb.Prospective, randomised study.One hundred eleven patients admitted to an emergency surgical service, randomly assigned to three groups.The patients were given 2% lidocaine with epinephrine 20 mL and 0.5% bupivacaine 20 mL. The three groups were as follows: brachial plexus block using a peripheral nerve stimulator (group St, n = 38); transarterial brachial plexus blockade with injection of 2/3 of the anaesthetic in back of and 1/3 in front of the artery (group TAP, n = 36); transarterial brachial plexus blockade with one single injection in back of the artery (group TP, n = 37). The success rate, time required to perform the technique, latency of analgesia, quality of motor blockade, and adverse effects were compared between the three groups. Analysis of variance was used to compare quantitative data and chi 2 test were used for qualitative data.Rates of success varied between 65 and 75%. Success rates, latency of analgesia and quality of motor blockade were not significantly different between groups. Time to perform the technique was longer when using a nerve stimulator.As these three techniques for brachial plexus block in emergency surgery are comparable, no one can be recommended instead of the others.

To assess incidence of secondary brain insults of systemic origin (SBISOs) such as arterial hypotension, hypoxaemia, hypercarbia, and anaemia in severely head injured children; to assess their impact on mortality and morbidity in the short- and long-term.Prospective, open study covering a 24-month period.Seventy-one children, under 15 years of age, admitted to a trauma centre for severe brain injury.Analysis of SBISOs and outcome.Twenty-five children were admitted with SBISOs. The mortality rate was 37%. After hospitalization, 84% of the children with SBISOs vs 46% without SBISOs had severe disability (Glasgow outcome score = 1, 2 and 3). After 1 year, 20 out of the 45 children still alive were contacted. One of the four with SBISOs communicated a bad recovery. Fifteen children without SBISOs presented good recovery: GOS = 4-5, paediatric overall performance category (POPC scale) = 1-2.Hypotension was associated with significant increase in mortality (x 3.6) in children with severe head injury. The consequences were worse when anaemia was associated.

Celiac disease is characterized by severe inflammation of the small intestine, and inflammation is known to often be associated with enhanced nitric oxide (NO) production. The aim of the present study was to determine whether children with active celiac disease show increased duodenal inducible nitric oxide synthase (iNOS) expression by immunohistochemistry. For this purpose, NO activity was assessed by detection of nitrotyrosine, which is an indicative marker for the formation of the NO- and superoxide-derived oxidant peroxynitrite. Serial staining with the macrophage markers CD68 and CD14 was performed to assess whether intestinal macrophages are involved in intestinal NO production. Duodenal biopsies from seven children with normal biopsy findings were used for comparison. Intense iNOS staining of enterocytes was observed in 10 of 11 celiac disease biopsies but in only 1 of 7 controls (p0.002). In addition, nitrotyrosine staining was detected in the enterocytes of celiac disease patients and was associated with iNOS staining. Moreover, the number of iNOS-positive cells in the lamina propria was significantly (p0.002) enhanced in patients with celiac disease. Serial staining for iNOS, CD68, and CD14 revealed an increase in CD68/CD14 double-positive monocytes and colocalization of iNOS and CD14 expression associated with this disease. Collectively, these data suggest that in patients with active celiac disease, synthesis of iNOS is induced in the intestine in association with the formation of peroxynitrite and nitration of cellular proteins. Furthermore, the increase in intestinal CD14-positive macrophages suggests a role for these cells in the pathophysiology of the disease.

The steatocrit method has recently been introduced as a simple screening test for steatorrhea. As it seemed likely that separation of fecal homogenate by centrifugation into a lipid phase, a watery phase, and a solid phase would be pH-dependent, we evaluated the effect of fecal acidification on steatocrit results. We also compared classical and acid steatocrit results in healthy children and in patients with cystic fibrosis and studied the relationship between two steatocrit methods and fecal fat content as measured by a reference chemical method. Steatocrit results increased with the degree of fecal acidification, and maximal results were obtained at the lowest fecal pH values. Means and SEM for classical and acid steatocrit values were 1.1 +/- 0.4% (classical) versus 3.8 +/- 1% (acid) in controls (n = 6) and 5.4 +/- 1.9% (classical) versus 26.9 +/- 4.3% (acid) in cystic fibrosis patients (n = 9). The correlations between fecal fat content measured chemically and steatocrit results were 0.18 (p = 0.35) and 0.81 (p < 0.0001) for classical and acid steatocrit, respectively. We conclude that acidification of fecal homogenates leads to a marked improvement in the steatocrit method.

In a prospective study we looked for the presence of both IgE plasma cells in small bowel mucosa and specific serum IgE antibodies to cow milk in children suspected of cow milk protein intolerance. Thirty-one children with complaints possibly due to cow milk intolerance were submitted to two consecutive cow milk elimination/challenge tests. The diagnosis of cow milk protein intolerance was confirmed in 16 of our 31 patients on the basis of two positive elimination/challenge tests. IgE plasma cells were found in nine of 16 patients with proven cow milk protein intolerance and in only one of the 15 patients without cow milk protein intolerance (p.01). The RAST for cow milk was positive in six of 16 infants with cow milk protein intolerance and in two of the 15 other infants. Serum IgE level was of no value for the diagnosis of cow milk protein intolerance. Neither of these diagnostic procedures was sensitive enough to be used as a screening test for cow milk protein intolerance. Furthermore, the relationship between specific IgE antibodies for cow milk and the presence of mucosal IgE plasma cells was poor: five of nine infants with cow milk protein intolerance and the presence of mucosal IgE plasma cells had negative RASTs for cow milk.

The polyamine concentration in rat milk and food, human milk, and infant formulas was estimated by HPLC. In rat milk, the concentration of putrescine and spermine was low (generally under 2.5 nmol.mL-1 for putrescine and under 1 nmol.mL-1 for spermine). The spermidine concentration was higher and seemed to increase during lactation. The rat food was richer in polyamines than the rat milk (about 150 times for putrescine and spermine, about 30 times for spermidine). We already proved that ingestion of spermine or spermidine can induce precocious maturation of the rat intestine. The present observations suggest that polyamines contained in rat food could play an important role in postnatal maturation of the rat intestine. The polyamine concentration of human milk was measured from 60 different mothers during a period extending from the 1st wk to the 6th mo of lactation. Great variation was observed. During the 1st mo of lactation, the general pattern was as follows: putrescine concentration generally varied little (from 1 to 3 nmol.mL-1), spermine and spermidine concentrations showed a similar pattern (the highest values appeared at the end of the 1st wk of suckling). After the 4th mo of lactation, putrescine concentration increased slightly, whereas spermine and spermidine concentration stayed almost stable. The concentrations of polyamines in 18 powdered milks for babies were estimated. Spermine and spermidine contents were lower than those in human milk. A protective effect of spermine or spermidine against alimentary allergies is suggested.

In this study we investigated the presence of gastroesophageal reflux in children with recurrent abdominal pain and its possible relationship to food intolerance-associated duodenal inflammation. Twenty-four-hour intra-esophageal pH monitoring, an endoscopic duodenal biopsy and a small bowel 51Cr-EDTA permeability test were performed in 25 children with recurrent abdominal pain. In 14 cases (56%) the pH monitoring was abnormal, pointing to the presence of pathological gastroesophageal reflux. Treatment of gastroesophageal reflux in the latter patients resulted in resolution or improvement of abdominal pain in 10 cases (71%). Gastroesophageal reflux did not appear to be associated with either intestinal permeability to 51Cr-EDTA or duodenal biopsy findings. We conclude that pathological gastroesophageal reflex is a frequent finding in children with recurrent abdominal pain, that it is unrelated to duodenal inflammation and that there might be a causal relationship between pathological gastroesophageal reflux and recurrent abdominal pain in children.

A 7-year-old white girl presented with recurrent abdominal pain because of gastroesophageal reflux. At endoscopy, we found intestinal lymphangiectasia in the duodenal biopsy. There were no physical signs, nor any laboratory evidence of enteric protein loss.

Random fecal chymotrypsin activity and fecal alpha 1-antitrypsin (FA-1-AT) concentrations were determined in 11 children with cystic fibrosis, 5 children with Crohn's disease, 9 children with chronic aspecific diarrhea, 85 children with acute gastroenteritis, and 54 control children. Cystic fibrosis patients showed only very low fecal chymotrypsin values that did not overlap with values obtained in patients with either acute or chronic diarrhea. When compared with our control group, a significant increase of FA-1-AT concentrations was found only in children with Crohn's disease. Normal values were found in all patients with either chronic aspecific diarrhea or cystic fibrosis, while 12 of 85 children with acute gastroenteritis showed FA-1-AT concentrations above the 95th percentile of control children. We conclude that diarrhea (either acute or chronic) does not significantly decrease the clinical usefulness of fecal chymotrypsin activity measurements in the diagnosis of pancreatic insufficiency, while acute (gastroenteritis) but not chronic (chronic aspecific diarrhea, cystic fibrosis) diarrhea can give rise to protein losing and FA-1-AT concentrations similar to those found in Crohn's disease.

The lactulose-rhamnose intestinal permeability test was performed in 10 cystic fibrosis (CF) children. Urine was collected for 5 h after oral intake of the test solution. A single thin-layer chromatography followed by densitometry was used for the measurement of lactulose and rhamnose concentrations in the urine specimens. The excretion of each molecule was expressed as the percentage of the orally administered dose excreted and the lactulose-rhamnose ratio was the ratio of the percentage quantities of each probe molecule excreted. The mean lactulose-rhamnose excretion ratio in CF patients and controls was 0.16 and 0.038, respectively (p less than 0.001). The mean 5-h lactulose excretion was 2% and 0.28% in CF patients and control subjects, respectively (p less than 0.001). The mean 5-h rhamnose excretion was 12.8% and 7.6% in CF patients and control subjects (p less than 0.05). We conclude that CF patients have an increased intestinal permeability to lactulose and rhamnose. Further studies are needed in order to elucidate the mechanisms involved as well as the eventual nutritional implications.

Fifty-four children who had chronic bronchopulmonary disease (CBPD) were investigated for the incidence of gastroesophageal reflux (GER). An activity score for CBPD and a numerical reflux score using criteria from a pH probe study were established. Results of barium esophagography, endoscopy, and prolonged esophageal pH probe monitoring were abnormal in 67, 78, and 53% of the patients, respectively. A significant relationship was found between the CBPD activity score and the reflux score; this strongly suggests that, in the patients studied, GER was responsible for the bronchopulmonary symptoms. Antireflux therapy resulted in an improvement of the CBPD activity score in all patients who had abnormal reflux scores. The mean CBPD activity score was 4.4 and 1.3 before and after antireflux therapy, respectively (P less than 0.01). Most patients who had normal reflux scores did not improve on antireflux therapy.

Intestinal permeability (IP) to /sup 51/Cr ethylenediamine tetraacetic acid was investigated in 47 children with chronic obstructive respiratory disease (CORD). Endoscopic duodenal biopsies were performed in 22 of these patients. IP was significantly increased in CORD patients when compared to either control children or adults (P less than 0.001). Mean +/- 1 SD were 4.3 +/- 1.71%, 2.5 +/- 0.78%, and 2.3 +/- 0.77% in the three groups, respectively. IP was not related to the presence of atopy. Significant differences in IP results were found between CORD children with abdominal pain (4.5 +/- 1.4%) and both control children and CORD patients without abdominal pain (2.5 +/- 0.78% and 3.2 +/- 1.49%, respectively). A significant correlation was found between small bowel injury on the one hand and IP on the other hand (P less than 0.02). Furthermore, small bowel injury was significantly related to the presence of abdominal pain (P less than 0.05). We speculate that in CORD patients with abdominal pain, a factor exists that causes small bowel injury responsible for both abdominal pain and increased small bowel permeability. Food intolerance could, presumably, play a role in the mucosal damage-linked IP increase found in the subset of CORD patients whomore » complain of abdominal pain.« less

The definition of "insufficient" small bowel lactase activity varies greatly among authors. The present study is aimed at redefining lactase insufficiency by comparing intestinal lactase activity and results of the lactose breath hydrogen test. Primary "insufficient" lactase activity was considered to be present when a child with a normal small bowel histology showed lactose malabsorption as measured by the lactose breath hydrogen test. The lactase activity of 22 "normal" children ranged from 0.77 to 4.57 U/g wet weight, while five children showed primary lactase insufficiency as defined above. Small bowel lactase activity in the latter patients was less than 0.74 U/g wet weight. Sucrase and maltase activities were similar in both groups of patients. We conclude that children with a normal small bowel histology should be considered to have primary lactase insufficiency when small bowel lactase activity is below 0.75 U/g wet weight.

Serum diamine oxidase (DAO) activities were measured in 9 pregnant women and in 33 neonates during the 1st week of life. The radiometric method was used. Very high values were found in the mothers (mean ± SE: 720 ± 143 pmol ml-1 h-1) Neonates showed significantly elevated values (mean ± SE: 178 ± 54 pmol ml-1 h-1) on the 1st postnatal day when compared to the 7th day values (mean ± SE: 27 ± 5.7 pmol ml-1 h-1)·Serial determinations showed a progressive decline from day 1 to day 7 after birth. We suggest the high serum DAO levels in neonates during the first days of life are due to leakage of placental enzyme into the circulation either at birth or during fetal life. Consequently, serum DAO cannot be used as a marker of small bowel functional integrity in the 1 st postnatal week.

Forget, P., Fernandes, J. and Haverkamp Begemann, P. (Department of Paediatrics, Sophia Children's Hospital and Neonatal Unit, Erasmus University, Rotterdam, the Netherlands). Utilization of fat emulsion during total parenteral nutrition in children. Acta Paediatr Scand, 64: 377, 1975.–Tolerance for Intralipid fat emulsion during total parenteral nutrition (PN) was studied in 6 children. The Intralipid dose was monitored by the daily determination of plasma Intralipid levels. Fat removal was investigated at the start of and during the PN period by the intravenous fat tolerance test (IVFTT) and by determining the plasma postheparin lipoprotein lipase (LPL) activity. When the plasma Intralipid levels exceeded a value of 100 mg/100 ml, hyper pre-βT lipoproteinaemia, hypertriglyceridaemia, hypercholesterolemia and hyperphospholipidaemia appeared. During PN most patients showed marked increases of postheparin LPL. Return to normal values occurred after discontinuation of PN. Maximal LPL activities were found to correlate significantly with total daily caloric intake (r=0·95, 0.05

Serum diamine oxidase (DAO) activities were measured in 20 control patients and in 24 patients with gastroenteritis. Results (mean +/- SE) were as follows: 1) control patients (n = 20), 36.2 +/- 5.7 pmol h-1 ml-1; 2) gastroenteritis (acute phase) (n = 11), 31 +/- 4.9 pmol h-1 ml-1; 3) gastroenteritis (healing phase) (n = 21), 18 +/- 1.9 pmol h-1 ml-1. Patients with gastroenteritis in the healing phase had significantly lower DAO values when compared to control patients (p less than 0.001) and to gastroenteritis patients in the acute phase (p less than 0.05). In eight patients where both acute and healing phase values could be measured, a significant decrease between acute and healing phase was found (p less than 0.001). Patients with severe gastroenteritis tended to have lower DAO activity values than patients with moderate gastroenteritis (p = 0.10). Our results support the hypothesis that serum DAO activity is a marker of the total mass of functional enterocytes, the decrease of which during gastroenteritis is reflected in a decrease of serum DAO activity values.

Forget, P. P. F. X., Fernandes, J. and Haverkamp Begemann, P. (Department of Paediatrics, Sophia Children's Hospital and Neonatal Unit, Erasmus University, Rotterdam, the Netherlands). Enhancement of fat utilization during prolonged intravenous feeding. AdaPaediatrScand, 63:750, 1974.-An 8-year-old girl with severe underweight caused by anorexia nervosa was treated with total intravenous nutrition for 4 weeks. During this period the Intralipid dose was stepwise increased, the doses of Vamin and glucose were kept constant. The Intralipid dose was monitored by the determination of the serum Intralipid levels. The fat utilization was investigated by intravenous fat tolerance tests and the estimation of postheparin lipoprotein lipase activity of the plasma. The Intralipid elimination constant increased from 796 to 2296/min, the postheparin lipoprotein lipase activity increased from 50 to 317 μEq fatty acid/min/l. These data enabled us to increase the Intralipid dose from 3 g fat/kg/per day to 8 g fat/kg/per day, without an increase of the triglyceride blood levels. We may conclude that lipoprotein lipase is an inducible enzyme. It is not clear which component of the hyper-caloric intravenous regime causes this induction.

A relationship between disaccharidase and diamine oxidase (DAO) activities was looked for by measuring these enzyme activities in histologically normal small intestinal biopsies of 18 children. The range for disaccharidase activities expressed in U g-1 wet weight was 0.1-5.7, 7.1-36.7, and 2.3-8.5 for lactase, maltase, and sucrase, respectively. The range for DAO activities expressed in nmol h-1 g-1 wet weight was 202-974. Significant correlations were found between disaccharidase and DAO activities (lactase versus DAO: n = 17, r = 0.80, P less than 0.001; maltase versus DAO: n = 18, r = 0.70, P less than 0.001; sucrase versus DAO: n = 18, r = 0.55, P less than 0.05). Our results further support the hypothesis that DAO is a marker of small intestinal functional integrity in children.

Intestinal permeability was investigated in 10 normal young adults, in 11 control children and in 9 children presenting with either Berger disease (4 cases) or Henoch-Schönlein nephritis (5 cases), making use of (51 Cr) EDTA as a probe molecule. All subjects exhibited a normal creatinine clearance at the time of testing. After oral administration of (51 Cr) EDTA, 24-hour urine radioactivity was measured and results were expressed in percentage of the oral dose administered. Means and SD were 2.35% +/- 0.77, 2.51% +/- 0.70, and 5.10% +/- 2.35 for normal adults, control children and patients with IgA-associated nephropathies, respectively. The differences of permeability between controls and patients were statistically significant (p less than 0.01). In addition, a significant, direct, linear correlation has been established between the percentage of (51 Cr) EDTA excreted in 24-hour urine and IgA immune complex-plasma levels. Our results therefore support the hypothesis that increased gut permeability could play a role in the pathogenesis of IgA-associated nephropathies.

In the present study, the protective effect of PGE/sub 2/ on intestinal damage in indomethacin-treated adult rats was investigated. Ileal integrity was evaluated making use of different biochemical and histological parameters : activities of sucrase, maltase and diamine oxidase; concentrations of DNA, putrescine, spermidine and spermine; incorporation of /sup 3/H-thymidine into DNA; mitotic index and mucosal thickness. Results expressed per g of mucosal weight, showed that : - maltase and diamine oxidase activities as well as DNA, spermidine and spermine concentrations decreased markedly in indomethacin-treated rats when compared to control rats; - the decrease of maltase activity as well as DNA, spermidine and spermine concentration was less pronounced in PGE/sub 2/-treated rats when compared to indomethacin-treated rats; - /sup 3/H-thymidine incorporation into DNA and mitotic index values showed no significant variation in the course of different treatments; - mucosal thickness increased strongly, the PGE/sub 2/-protected rats. The authors suggest that PGE/sub 2/ could protect the rat's intestinal mucosa against the effects of indomethacin through a trophic action on intestinal villi. 15 references, 3 tables.

Extract: Peripheral uptake of triglyceride from plasma was investigated by intravenous fat tolerance tests and by postheparin lipoprotein lipase measurements in children with different types of glycogen storage disease. The patients with a glucose 6-phosphatase deficiency were characterized by a significantly diminished triglyceride elimination rate (5.79 ± 2.78%/min) and 5-min postheparin lipoprotein lipase activity (40.2 ± 23 μEq fatty acid (FA)/liter/min). The patients with a deficiency of debranching enzyme showed a significantly diminished triglyceride elimination rate (4.84 ± 1.61%/min) whereas the 5-min postheparin lipoprotein lipase activities did not significantly differ from the control values (49.6 ± 27.7 μEq FA/liter/min). The patients with a deficiency of the phosphorylase system showed neither a significantly diminished triglyceride elimination rate (7.34 ± 2.65%/min) nor a diminished 5-min postheparin lipoprotein lipase activity (61.7 ± 30.1 μEq FA/liter/ min). Triglyceride elimination rates were correlated positively with plasma lipoprotein lipase activities (r = 0.55, P < 0.05). Speculation: The dietary treatment of hyperlipidemia in hepatic glycogenosis might be based on the outcome of the intravenous fat tolerance test, a low fat clearance being an indication for a low fat, high carbohydrate diet, a normal fat tolerance for a high fat low carbohydrate diet.