[Epidemiology of celiac disease]. / Épidemiologie de la maladie cœliaque.

Celiac disease is an autoimmune disorder induced by gluten in genetically susceptible individuals. Patients may present with typical symptoms of enteropathy with diarrhoea and failure to thrive, but atypical symptoms, or even silent forms are more often recognized since serologic markers are available. This progress led to the increase of the incidence of the celiac disease during the last 30 years, which reflect probably the improvement in diagnosing atypical forms of the disease. The prevalence is comparable from a country to another, around 0,7 to 2%. There is a progression with time from latent to silent form, and then to the active disease. The exclusion of the gluten from the diet leads to the regression from the active to the latent form of the disease.

Proton pump inhibitors are still overprescribed for hospitalized children.

OBJECTIVE: The use of proton pump inhibitors has increased exponentially over the past 20 years. Several side effects have been reported and concerns exist about the consequences of long-term proton pump inhibitors on health, leading to limitation of their use. The present study analyzed prescriptions of proton pump inhibitors at inpatient units and assessed their compliance with current recommendations. METHODS: This single-center, observational, retrospective study reviewed medical file of patients hospitalized at the pediatric medical departments of the Bordeaux University Hospital between April 1 and September 30, 2019. Patients younger than 18 years, hospitalized in the pediatric hospital units and treated with proton pump inhibitors were included. Prescriptions of proton pump inhibitors were compared with French and international guidelines. RESULTS: Proton pump inhibitors were prescribed for 251 of 2237 children (11%), mainly for gastroesophageal reflux disease (47%) and prevention of peptic ulcer disease (32.7%). Proton pump inhibitor prescription complied to recommendations in 34.5% of cases, less often in children aged younger than 1 year (13.5%) than in older children. Compliance to recommendations was lower when proton pump inhibitors were indicated for the prevention of peptic disease (5%) than for gastroesophageal reflux disease (48%). CONCLUSIONS: Proton pump inhibitors are frequently prescribed for hospitalized children, and indications comply with recommendations in only 35% of the cases. Efforts in spreading awareness of the recommendations on the use of proton pump inhibitors in children are mandatory among hospital pediatricians.

Satisfaction of mothers regarding human milk donation.

In France, human milk banks are in charge of the collection, analysis, processing, and distribution of human milk to neonatology centers for preterm infants. Knowledge of what motivates mothers to donate their milk could lead to better communication regarding human milk donation. A satisfaction survey was conducted among mothers who were donating their milk to a human milk bank. In total, 214 mothers answered a questionnaire in the presence of the collector during a home visit. The median age of the mothers was 31 years (18-46), mainly high school (19%) or university (65%) graduates, and the median duration of donation was 3 months (0.5-22). At the time of the study, the median age of infants was 3 months (0.5-25), and 88% of infants were exclusively breastfed. About three quarters of mothers were motivated by willingness to help others, a quarter of them being especially sensitive to premature neonatal care; 30% of mothers were motivated by having a high supply of milk. Around 25% of mothers were given information on human milk donation during pregnancy, and two thirds after delivery, mainly by the maternity ward midwives (53.4%) or by collectors during their visit (14.1%). Most mothers (72%) found the human milk donation process easy and most of them (92.5%) were willing to donate their milk again after their next pregnancy. This survey shows that more than 90% of mothers are satisfied with donation to human milk banks. However, efforts should be made to provide information on breastfeeding and human milk donation to the general population and health professionals.

Serum fructosamine predicts macrosomia in well-controlled hyperglycaemic pregnant women: An observational cross-sectional study.

AIM: While serum fructosamine may be a good marker of glucose control in pregnant women with diabetes, its relationship with macrosomia is still uncertain. METHODS: In 130 hyperglycaemic women with singleton pregnancies (117 gestational diabetes mellitus, 13 pregestational diabetes), serum fructosamine and HbA1c levels were measured at 25±7 weeks of gestation. Levels in mothers of infants with and without macrosomic newborns (birth weight>4000g and/or large-for-gestational-age birth weight>90th percentile) were compared using logistic regression analysis adjusted for macrosomia risk factors. RESULTS: These 130 pregnant women were 33±5 years old; their BMI before pregnancy was 27.7±6.9kg/m2, and they gained 7.5±5.1kg during the first 6 months of gestation. Glucose control was good according to HbA1c levels (5.3±0.3%; 34±2mmol/mol), yet 17/130 (13%) newborns had macrosomia: 3900±227g vs 3057±512g (P<0.001) in the others. These mothers were older and had higher parity, whereas their BMI scores before pregnancy and gestational weight gains did not differ. Fructosamine levels were also higher at 221±40µmol/L vs 192±22µmol/l (P<0.001), respectively, and remained significant even after adjusting for maternal age, BMI, parity, type of diabetes, antecedents of macrosomia and excessive gestational weight gain. By contrast, HbA1c did not differ between the two groups. In fact, nearly two-thirds (64.7%) of the mothers of macrosomic newborns had fructosamine levels>200µmol/l vs 31.9% of mothers with non-macrosomic newborns (P<0.05). CONCLUSION: High fructosamine levels are associated with macrosomia in the newborns of well-controlled hyperglycaemic pregnant women.

Prevention of relapse by mesalazine (Pentasa) in pediatric Crohn's disease: a multicenter, double-blind, randomized, placebo-controlled trial.

AIM: This study aimed to test the efficacy of mesalazine in maintaining remission in pediatric Crohn's disease (CD) following successful flare-up treatment. METHODS: In this double-blind, randomized, placebo-controlled trial, 122 patients received either mesalazine 50mg/kg per day (n=60) or placebo (n=62) for one year. Treatment allocation was stratified according to flare-up treatment (nutrition or medication alone). Recruitment was carried out over two periods, as the first period's results showed a trend favoring mesalazine. Relapse was defined as a Harvey-Bradshaw score more than or equal to 5. Time to relapse was analyzed using the Cox model. RESULTS: The one-year relapse rate was 57% (n=29) and 63% (n=35) in the mesalazine and placebo groups, respectively. We demonstrated a twofold lower relapse risk (P<0.02) in patients taking mesalazine in the medication stratum (first recruitment period), and a twofold higher risk in patients taking mesalazine in the nutrition stratum (second recruitment period), compared with the other groups. None of the children's characteristics, which differed across the two recruitment periods, accounted for the between-period variation in mesalazine efficacy. One serious adverse event was reported in each treatment group. CONCLUSION: Overall, mesalazine does not appear to be an effective maintenance treatment in pediatric CD.

[Chronic exanthema in a child with congenital rubella]. / Exanthème chronique chez un enfant atteint de rubéole congénitale.

BACKGROUND: Since congenital rubella has become extremely uncommon following the introduction of rubella vaccination, cutaneous signs are currently rarely reported. PATIENTS AND METHODS: An infant, presenting congenital rubella (seroconversion of the mother for rubella at 11 weeks' amenorrhoea), presented diffuse exanthema between the ages of seven and 24 months. In a setting of congenital rubella syndrome, the infant presented psychomotor retardation, deafness, hypoplasia of the pulmonary artery and under-nourishment. Humoral and cell-mediated immunodeficiency was also noted. DISCUSSION: The cutaneous signs of congenital rubella were first described in the 1960s and 1970s; they are rare and appear after a symptom-free period before resolving spontaneously several months later. Standard findings include chronic exanthema of the face and extremities associating reticulated erythema and pigmented macular papules. Exanthema indicates chronic and persistent viral infection, a common situation in newborn babies and infants following maternal-foetal infection. The persistence of viral infection in infants is attributed to immature cellular immunity, which would otherwise either eradicate the virus or ensure passage to the latency phase. In the present case, there was also relative humoral immunodeficiency resulting from foetal rubella infection. The symptom-free interval before the onset of rash and other clinical signs may be due to the relative transient protection afforded by the presence of maternal immunoglobulines G.

[Rotavirus vaccination in France: recommendations of the French Speaking Group of Pediatric Gastroenterology, Hepatology and Nutrition]. / La vaccination rotavirus en France: position du Groupe Francophone d'Hépatologie, Gastroentérologie et Nutrition Pédiatriques.

The rotavirus is the major cause of infantile gastroenteritis worldwide. In Europe and in industrialized countries, rotavirus infections bring out considerable economic expenses. In France, the burden of health expenses is particularly marked during winter period, the peak of rotavirus infections coinciding with that of the respiratory syncitial virus bronchiolitis, this specificity results in an increase of nosocomial infections during this period and a saturation of beds in paediatric hospitals. Vaccination likely represents the only efficacious adapted response to rotavirus infections. Two vaccines have recently been developed and licensed in France. The development of these two vaccines has followed two different strategies. Despite their differences, these vaccines are both safe and efficient in protecting young infants against severe rotavirus acute gastroenteritis. French Health Authorities decided to postpone rotavirus vaccination for at least two years. According scientific and economic data, the French Speaking Group of Gastroenterology Hepatology and Nutrition disagrees with this decision and states that rotavirus vaccination is universally recommended for healthy infants under six months.

Liver transplantation for cirrhosis in cystic fibrosis.

BACKGROUND: Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure. AIMS: The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population. PATIENTS: Five children with CF, at a mean age of 16.5 years, underwent liver transplantation. RESULTS: All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure. CONCLUSION: Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.

Evaluation of nutritional status and support in children with congenital heart disease.

BACKGROUND/OBJECTIVES: The objective of this disease was to determine the prevalence of malnutrition in children with congenital heart disease (CHD). SUBJECTS/METHODS: A total of 125 children with CHD, under 6 months of age, were divided into four groups: no pulmonary hypertension (PH) or cyanosis (group 1, n=47), isolated cyanosis (group 2, n=52), isolated PH (group 3, n=16), and PH and cyanosis (group 4, n=10). Six children died at 6 months (n=4), 12 months (n=1) and 19 months (n=1). The remaining children were followed-up for 24 months. Prevalence of moderate to severe malnutrition (weight/weight for height <80%), caloric intake and medications were compared between the four groups. RESULTS: Moderate or severe malnutrition was more frequent in group 4 (100%) compared with others groups (group 1, 20%; group 2, 16.7% and group 3, 50%; P<0.05). Low oral caloric intake was more frequent in group 3 (71.4%) and group 4 (75%) than in group 1 (28%) and 2 (28.6%) (P<0.05). Food enrichment was practised in half of the children of group 4 and rarely in other groups (group 1, 15.8%; group 2, 8.6% and group 3, 11.1%; P<0.05). Enteral feeding was used more often in groups 3 (33.3%) and 4 (50%) than in groups 1 (15.8%) or 2 (14.3%; P<0.05). CONCLUSIONS: Moderate or severe malnutrition is present in 15% of children with CHD, and it is more frequent in case of PH. Half of these children demonstrate low caloric intake, whereas few have proper nutritional support.

[Fecal elastase-1: a useful test in pediatric practice]. / Intérêt pratique du dosage de l'élastase-1 fécale chez l'enfant: comparaison au dosage de la chymotrypsine fécale.

AIM: To study fecal elastase-1 (E1F) and chymotrypsin (ChT) in stools for the diagnosis of pancreatic insufficiency in pediatric practice. MATERIALS AND METHODS: E1F and ChT were measured in stools of 198 children divided in 3 groups: 49 children without any digestive disease (group A), 71 children with pancreatic diseases (group B), and 78 children with non-pancreatic digestive diseases (group C). RESULTS: In group B, E1F values were very low in 64 children and normal in 7 children without pancreatic insufficiency (6 children with cystic fibrosis and 1 with chronic pancreatitis). ChT values were normal in children without pancreatic insufficiency but also in half of children treated with pancreatic enzymes. Decreased E1F values were seen in 2 children (4%) in the group A and 22 children (28%) in the group C, especially those with acute gastroenteritis or celiac disease. CONCLUSION: E1F is a simple, non-invasive, useful tool for the diagnosis of pancreatic insufficiency in children with growth failure or chronic diarrhea, and those with cystic fibrosis. Nevertheless, low values may be found in diseases with villous atrophy or very liquid stools.

Management of pancreatic, gastrointestinal and liver complications in adult cystic fibrosis.

INTRODUCTION: The gastrointestinal tract is a major source of morbidity in adults with cystic fibrosis (CF), with a wide range of complications, some of them being specific to the underlying disease. STATE OF KNOWLEDGE: Abnormal CFTR function, with reduced bicarbonate and other ion transport levels through the apical surface of epithelial cells, affects the intestinal tract including the pancreas and the liver. Similarly to what is observed in the respiratory tract, gastrointestinal CFTR dysfunction leads to mucus accumulation, dysmotility, small bowel bacterial overgrowth and inflammation with alteration of innate immune responses, all of which being likely to be interrelated. In developed countries, almost half of patients with CF are adults followed in multidisciplinary CF care centres by pneumologists who often have to manage gastrointestinal complications. CONCLUSION: It therefore appears essential that adult gastroenterologists develop the expertise needed for managing CF gastrointestinal complications in close collaboration with multidisciplinary CF care centre teams to improve the quality of life of adults with CF.

Acute urticaria in infancy and early childhood: a prospective study.

OBJECTIVES: To establish the clinical, etiological, and prognostic features of acute urticaria in infancy and early childhood and to define its optimal management. DESIGN: Prospective study. The inception cohort was collected from April 1, 1992, through March 31, 1994. After initial evaluation, the course of the disease was assessed at 2 months and after 1 to 2 years. SETTING: Emergency department of a regional teaching pediatric hospital (referral center), which is also the only pediatric hospital for the general community in the city (population, 600,000 inhabitants). PATIENTS: Fifty-seven consecutive infants, aged 1 to 36 months, hospitalized with a final diagnosis of acute urticaria. Follow-up at 1 to 2 years was available in 40 of 57 patients. INTERVENTION: Oral antihistamines (dexchlorpheniramine maleate, terfenadine, or hydroxyzine hydrochloride) for 2 weeks. MAIN OUTCOME MEASURES: Recurrence and chronicity. RESULTS: Annular or geographic papules and plaques with hemorrhagic lesions were seen in 28 patients (49% of cases) and angioedema in 34 patients (60% of cases). An underlying cause was suspected or identified in 52 patients (91% of cases). Infection, either associated or not with drug intake, was the cause in 46 patients (81%) and foods were the cause in 6 (11%). Parasitic infestations were noncontributory. Hemorrhagic lesions and association with articular symptoms were statistically more frequent in urticaria caused by infections. Atopy in the patient or family was associated in 33 patients (58% of cases), and particularly atopic dermatitis was associated with urticaria caused by food. At 1- to 2-year follow-up, 12 (30%) of 40 patients surveyed had chronic or recurrent urticaria. CONCLUSIONS: Causative factors in urticaria are dominated by benign viral illnesses, often associated with antibiotic drug therapy. In most patients, laboratory investigations are not required. Twenty percent to 30% of cases evolve into chronic or recurrent disease.

Scoliosis at less than 30 degrees. Properties of the evolutivity (risk of progression).

In an attempt to find prognostic factors for mild scoliosis, the first clinical and radiologic data of 159 subjects with idiopathic scoliosis less than 30 degrees were correlated with the annual speed of progression estimated graphically by further examinations. Thoracic and double major are more progressive than thoracolumbar and lumbar. The correlation with rate of progression is better for supine angle than for standing angle. Curves with low supine angles are those that have great relative collapse and low risk of progression. Only the rib hump of thoracic and thoracolumbar are correlated with evolutivity. Predictive equations give less approximation for individual prognosis than a previous graphic method, so they are not used. Approximately 73% of scoliosis have evolutivities less than 6 degrees per year and required nocturn corrective treatment.

Abdominal lymphatic dysplasia and 22q11 microdeletion.

We report the case of a child with 22q11 microdeletion who presented with abdominal lymphatic dysplasia resulting in exsudative enteropathy. This primitive and localized lymphatic malformation is consistent with the vascular theory in the velocardiofacial syndrome.

A short-stay observation unit improves care in the paediatric emergency care setting.

The aim of this study was to evaluate the influence of a short-stay observation unit (SSOU) on the hospitalization rate, the authors have analysed the activity of a SSOU opened in 1992 in a paediatric emergency department (PED) and the number of children hospitalized between 1987 and 1996. Among 2321 patients admitted to the SSOU in 1996, 644 medical patients have been analysed, showing a young population (55% under 3 years old), living in the town or surroundings (70%) and usually have not been referred by a general practitioner (64%). Twenty per cent were admitted for accurate diagnosis (group A), 49% for treatment and observation of a recognized acute pathology before decision of discharge (group B), and 31% waiting for a bed in a paediatric ward (group C). Sixty-eight per cent of children spent less than 6 hours in the SSOU, and 79% of those from groups A and B were thereafter discharged home. Between 1987 and 1991, the number of children hospitalized increased 5% per year on average. After the opening of the SSOU in 1992, this increase has been stopped although the number of medical referrals to PED was still going up. The SSOU in a PED can provide comprehensive care to young children requiring short-term treatment or observation, and is effective in limiting unappropriate hospitalizations.

Epidemiology of poisoning in children: a 7-year survey in a paediatric emergency care unit.

Acute poisoning in children is still a major public health problem, and represents a frequent cause of admission in emergency departments. We carried out an epidemiological study of poisonings leading to admission to a paediatric emergency care unit (PECU). We analysed data from 2988 children who were admitted to the PECU of Bordeaux, France with acute poisoning from 1989 through 1995. During the 7-year period, the poison exposure numbers decreased slightly from 490 to 382 (6% vs. 3% of total medical emergencies). This represented a mean annual incidence of 1.4 poison exposures per 1000 children younger than 18 years of age and living in Bordeaux and its surroundings. Characteristics of the study population, circumstances of poisoning and substances involved were similar to those previously described. Eighty per cent of children were younger than 5 years of age, presented with a benign course. Forty per cent were not treated and 75% were discharged home either immediately or within 24 hours of admission. Only 1.5% of cases, mainly adolescent girls who attempted suicide, were admitted to a paediatric intensive care unit. Overall mortality rate was 0.33/1000. In children, most cases of acute poisoning are accidental, benign, and mainly attributed to the ingestion of a non-toxic substance. This points to the need for better information of the population on availability of poison control centre calling facilities, in order to decrease the number of admissions to the PECU. Patients suspected of having ingested a potentially dangerous substance can be managed in short-stay observation units, thus avoiding unnecessarily prolonged hospitalization. Acute poisoning in children remains a frequent problem, highlighting the need to develop an education programme on primary prevention in our region.

[Helicobacter pylori gastritis in children]. / Gastrite à Helicobacter pylori chez l'enfant.

BACKGROUND: The role of Helicobacter pylori in chronic gastric and duodenal ulcer disease has now been proven in adults as well as in children. MATERIAL AND METHODS: H pylori has been looked for during endoscopy in 130 children suffering from recurrent abdominal pain (78 patients) or other symptoms (52 patients). Biopsy samples were taken from the antrum and the fundus, and were examined for histology and bacteriology (Gram staining, urease test, culture). RESULTS: H pylori was found in 30 cases (H pylori+ group, medium age: 12 years) and was absent in the 100 other cases (H pylori- group, medium age: 6 years). H pylori was present in 27% of children suffering from recurrent abdominal pain and 17% of children with other symptoms. Epigastric location of the pain and vomiting were equally frequent in both groups. Endoscopic gastritis, often nodular, was noted in 60% of the cases in the H pylori+ group and in 46% in the H pylori- group (NS). Chronic gastritis was noted in 90% of the cases in the H pylori+ group, and in 32% of the cases in the H pylori- group (p < 0.01). Culture was positive in 27 out of 28 cases (97%) in the H pylori+ group, including 3 cases with negative histologic examination. Twenty-two children in the H pylori+ group were treated with amoxicillin and metronidazole. Endoscopy performed about 2 months later showed eradication of H pylori in 63% of the cases, associated with disappearance of histologic lesions in 6 out of 14 cases and of symptoms in 5 out of 7 cases. CONCLUSIONS: H pylori is associated with some pediatric cases of antral gastritis. Further studies are needed to determine the influence of its eradication on symptoms and the interest to search this pathology (by serology) in children suffering from chronic abdominal pain.

[Mechanisms of hepatic fibrogenesis]. / Mécanismes de la fibrogénèse hépatique.

Hepatic fibrosis is a scaring process leading to cirrhosis, a major complication of numerous chronic liver diseases. Hepatic stellate cells play a central role in the fibrotic process. After parenchymal or biliary injury, cytokines and growth factors allow the recruitment, proliferation, and activation, of stellate cells toward myofibroblasts, which secrete the extracellular matrix. Fibrosis, resulting from the failure of the balance between synthesis and degradation of extracellular matrix, is an evolutive and potentially reversible process. Histological examination is the main investigation to quantify fibrosis. Serological tests are warranted to allow a non invasive follow up of patients. Development of antifibrotic therapies should soon permit to slow down the evolution toward cirrhosis, limiting the needs for hepatic transplantation.

[Acute severe asthma in children. Epidemiological, physiopathological and clinical aspects]. / Asthme aigu grave de l'enfant. Aspects épidémiologiques, physiopathologiques et cliniques.

Therapy for asthma is being prescribed more frequently. However, asthma mortality remains high in many countries. Fatal outcome is not always related to inadequate follow-up. In this article we report that children with mild to moderate symptoms may present a fatal attack. Many factors are responsible for such an outcome: asphyxia +3 due to ventilation/perfusion mismatch and/or bronchospasm, cardiac failure, cardiac arrhythmia, intrinsic positive expiratory pressure, or metabolic disturbances (hypokalemia, for example). Such problems can occur in predisposed patients: it has been shown that the chemosensitivity to hypoxia and the perception of dyspnoea are altered in certain patients with near-fatal asthma. It is very important to identify children at risk of severe asthma and to organize care so as to optimize the management of such children.