Cardiac malformations in children with congenital hypothyroidism.

Congenital hypothyroidism (CH) is the most common endocrine disease in children, according to literature, infants with CH have an increased risk of associated congenital malformations (CM), especially cardiac defects (CD), compared to the general population. We retrospectively analyzed medical records of 255 patients with a positive screening result for CH in the period 1991-2016 followed at our Center. At the time of enrollment, the clinical examination included looking for the presence of heart murmurs and dysmorphic features. In all patients an echocardiography with cardiological evaluation were performed. Of all patients, 191 were included in the final analysis. Of these, 51.3% (98/191) presented an eutopic normally sized thyroid gland while 48.7% (93/191) showed a thyroid dysgenesis. Among the studied infants, 13.6% (26/191) presented CD. The most frequent cardiac anomaly was atrial septal defect (ASD) which was found in 65.4% (17/26) of patients with CD. Other defects were ventricular septal defect (VSD), patent ductus arteriosus (PDA), pulmonary valve stenosis (PvS), transposition of the great vessels (TGV), aortic valve stenosis (AvS). Six patients had multiple defects. In the analysed group, there was no significant relation with sex, type of CH, median blood-TSH (b-TSH) and serum-TSH (s-TSH) values and frequency of CD. There is a high prevalence of CD in CH, indicating the need of routine echocardiography in these patients to achieve an early diagnosis and management of CD.

Cardiac involvement in Lysosomal Storage Diseases.

Lysosomal storage diseases (LSDs) include a heterogeneous group of rare, inborn, metabolic diseases characterized by deficiency of lysosomal enzymes or of other proteins involved in lysosomal function, leading to multi organ system substrates accumulation, with consequent multi systemic clinical presentation. Cardiac disease is particularly important in some group of LSDs as glycogen storage diseases (Pompe), mucopolysaccharidoses and in glycosphingolipidoses (Anderson-Fabry disease and less frequently Gaucher disease). Various cardiac manifestations may be observed including hypertrophic and dilated cardiomyopathy, coronary artery disease and valvular disease. The availability of enzyme replacement therapy (ERT) has changed the natural history of some LSDs such as Pompe disease, thanks to the significant effects on cardiological involvement. In other LSDs such as MPSs or Fabry disease, ERT has been shown to stabilize or slow the progression of heart damage. This imposes the need for a timely diagnosis that allows a rapid onset of ERT.

Prevalence of elevated pulmonary artery systolic pressure in Down Syndrome young patients with and without congenital heart disease.

This study examined the prevalence and distribution of elevated systolic pulmonary arterial pressure, measured by echocardiography, in young patients with down syndrome associated or not with congenital heart disease and surgical correction during childhood. Pulmonary artery systolic pressure, computed by regurgitant tricuspid flow velocity evaluation, is the most frequently used parameter for the screening of pulmonary hypertension. Down syndrome and congenital heart disease often coexist and the probability to detect elevated systolic pulmonary arterial pressure in this setting is high. However, little is known about the evaluation of pulmonary arterial pressure during growth of patients with down syndrome with or without congenital heart disease. We enrolled 47 young patients (55% of male sex; mean age: 18.4 ± 6.0 years), 40 with congenital heart disease and 7 without a cardiac defect. Systolic pulmonary arterial pressure was assessed by echocardiography. No difference was found in the population dichotomized by presence or absence of CHD. Only male sex (p=0.000), highly sensitive troponin-T (P=0.027), tricuspid annular plane systolic excursion (TAPSE, p=0.045) and sPAP (p=0.004) were elevated in surgical group. The ASD was found as, the most common structural abnormality in our patients (50%), followed by VSD (27.5%) and complex CHD (such as complete atrioventricular canal defect, CAVC = 25% and Fallot disease = 15%). Furthermore, about 45% of patients had the combined defect. Only 37.5% of patients underwent to corrective surgery during the first months of life. We observed a significantly increase of sPAP values in patients with complex CHD, such as CAVC (p=0.019) and Fallot disease (p=0.001) but, in the following multivariate analysis performed in the patients with CHD, only Fallot disease remains as independent predictors of elevated values of sPAP (p=0.022). An elevated systolic pulmonary arterial pressure may represent the key screening tool in the diagnostic assessment of suspect pulmonary arterial hypertension in high risk population with down syndrome regardless the presence of congenital heart disease.

Dilated cardiomyopathy in mucolipidosis type 2.

Mucolipidosis II and III are lysosomal storage diseases caused by pathogenetic mutations in GNPTAB and GNPTG genes which cause an impaired activity of the lysosomal hydrolase N-acetylglucosamine- 1-phosphotransferase, a key enzyme in the synthesis of the mannose-6-phosphate targeting signals on lysosomal enzymes. Patients with MLII alpha/beta present coarse facial features, cessation of statural growth, important skeletal manifestations, impaired neuromotor development and cardiorespiratory involvement. All children appear to have cardiac involvement, but severe dilated cardiomyopathy is uncommon. In this report we describe the case of an 11-month-old girl who is affected by a MLII. Analysis of the GNPTAB gene identified at a heterozygous level the previously described gene variants c. 2693delA p(Lys898Serfs*13) and c. 2956C>T p(Arg986Cys). Her main clinical features were coarse face with gingival hypertrophy, dysostosis multiplex, recurrent respiratory infection and an early onset of dilated cardiomyopathy, an uncommon feature for MLII. To our knowledge, dilated cardiomyopathy has been previously described in literature in only two cases of MLII and in one patient affected by MLIII.

Unusual presentation of Henoch-Schönlein purpura.

Henoch Schonlein Purpura (HSP) is a systematic IgA-mediated vasculitic disease that affects the small vessels of the skin, the joints, the gastrointestinal tract and the kidneys (1). It is the most common childhood vaculitis, with an incidence estimated at 3-26 per 100,000 children, and with a male-to-female ratio of 2:1 (2-6). The 90% of patients are under 10 years of age, with a mean age of 4 years (4). It seems to be most common in fall and winter in children, and summer and winter in adults (7). Recent studies suggested a strong genetic predisposition in individuals with immunoglobulin Avasculitis (IgAV) associated to HLA class II region. Clinically, the non-thrombocytopenic purpura often located on lower extremities and buttocks is the essential element for the diagnosis of HSP. Treatment is supportive, because the disease is usually benign and self-limited. Indeed, in children, the prognosis is good, with a self-limited course and without any complications and after a median follow-up of 12 months, complete recovery was obtained in 83% of the IgAV patients (4, 8). The aim of our study is to describe some atypical presentations of the HSP in children.

Renal involvement in paediatric Fabry disease.

Anderson-Fabry Disease (AFD) is a rare, X-linked inborn error of glycosphingolipid catabolism caused by a deficient or absent activity of the lysosomal enzyme, α-galactosidase A, resulting in the progressive multisystem lysosomal accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3). Among the wide spectrum of clinical signs and symptoms and the life-threatening complications of Fabry disease, renal failure causes significant morbidity and mortality. Various evidence shows that the accumulation of Gb3 in different renal cells is present since the first years of life, many years and usually decades before manifest symptoms and signs of renal involvement. Early renal damage can be demonstrated by clinical signs as microalbuminuria and proteinuria, developing as early as in the second decade of life. A decline in GFR is uncommon at paediatric ages but may be seen as early as adolescence. Renal biopsy is rarely used in paediatric patients with Fabry disease although evidence shows that it may be considered a valid tool for the diagnosis of early and potentially reversible nephropathy, as well as for the evaluation of the effectiveness of enzyme replacement therapy (ERT). Although there is consensus in considering the early initiation of ERT as the only tool able to prevent the progression of nephropathy, the issue on the correct timing for the onset of ERT in pediatric age remains open in the management of this chronic and progressive disease.

Pediatric urolithiasis.

Urolithiasis is a well-known condition that can affect any part of the urinary tract. With a rate of 3-5% the incidence of upper urinary tract for long has been higher in adults (1-3), but recently it has increased among children reaching 3,3% . Indeed, more than 1% of all urinary stones are seen in patients aged less than 18 years (4). Pediatric urolithiasis is endemic in Turkey and Far East and it is probably due to malnutrition and racial factors (5). The spontaneous stone passage is more likely in children than in adults, indeed ureteral calculi spontaneously pass into 41-63% of children (1). Rate of stone passage depends on size and stone location in the urinary system. Stones sized less than 5 mm have a passage rate ranging from 40% to 98%, whilst stones > 5 mm have between 55% and 50% (6). In the last decade, the use of alpha blockers has proven well efficacious in helping spontaneous passage of distal ureteric stones in adults (7-9). The latest EAU guidelines support their use in adults while remain vague about their use in children because of unclear safety and efficacy (4). In search of evidence supporting or not the use of medical expulsive therapy in children we reviewed the literature dealing with the management of urolithiasis in pediatric patients. The primary aim of the present study was to evaluate the efficacy of medical expulsive therapy (MET), defined as stone expulsion rate, with a-blockers compared to a control group. The secondary aim was to assess the safety, defined as side effects rate, of MET compared to a control group.

Alport's syndrome.

Alport's syndrome (AS, OMIM 301050) is a hereditary disorder characterized by progressive renal failure, hearing impairment and ocular changes. It is clinically and genetically heterogeneous and in its natural history, renal disease progresses from microscopic haematuria to proteinuria, and finally to progressive renal insufficiency. AS is caused by an inherited defect in a type IV collagen, a structural material, expressed in many tissues that is essential for the normal function of different parts of the body. In most of cases, about the 85%, Alport's syndrome is X-linked and is originated by mutations in the COL4A5 gene. In the remaining cases, it may be inherited in either an autosomal recessive, or rarely in an autosomal dominant manner. Mostly, the condition is caused by mutations in the COL4A3 or COL4A4 genes. Coexisting mutations in COL4A3, COL4A4, COL4A5 or COL4A6 were found to cause an Alport's syndrome phenotype with digenic inheritance. Diagnosis of the condition is based on family history, clinical signs, and specific procedures such as a kidney biopsy. The diagnosis can be confirmed by genetic testing. Treatment may include use of a hearing aid, hemodialysis, and peritoneal dialysis to treat those with end-stage renal failure, and, as the last step, kidney transplantation. Firstly described by Arthur C. Alport's, in 1927, over the years it has become a pathology of high scientific interest. At the moment, thanks to advances in diagnostic techniques, it is possible to make an early diagnosis avoiding irreversible damages and life -threatening complications.

Rotavirus gastroenteritis: precursor of functional gastrointestinal disorders?

BACKGROUND AND AIMS: : Abdominal pain-related functional gastrointestinal disorders (AP-FGIDs) following bacterial acute gastroenteritis (AGE) have been demonstrated in adults and children. An adult study demonstrated AP-FGIDs resulting from an outbreak of viral AGE. Viral AGEs are common in children. Thus, the demonstration of AP-FGIDs occurring after a viral infection in children could constitute a significant finding. The aim of the study was to investigate the development of FGIDs following an episode of acute rotavirus gastroenteritis in children. This is the first pediatric multicenter study designed to assess postviral AP-FGIDs. PATIENTS AND METHODS: : It is a cohort study. Inclusion criteria of the study are children ages 4 to 18 years with history of AGE secondary to rotavirus. Sample size is 44 exposed and 44 controls (unidirectional alpha of 0.05, power of 0.80). Children consulting at 2 hospitals (Chicago, IL, and Naples, Italy) for AGE (2002-2004) who tested positive for rotavirus were randomly contacted by telephone >2 years after the episode. Each exposed child who visited the emergency department or outpatient site for acute trauma or well-child visit within 4 weeks of the index case was matched with a control of the same age and sex. Gastrointestinal symptoms and disability were evaluated with a validated pediatric questionnaire. RESULTS: : Eighty-eight patients (46 boys, mean age 5.3 years) were recruited. Contacted patients presented with AGE in 2002 (9), 2003 (11), and 2004 (24). Seven (16%) exposed patients and 3 (7%) controls reported AP-FGIDs (P = 0.31). CONCLUSIONS: : Our study suggests that rotavirus infection does not seem to place children at increased risk for AP-FGIDs at long-term follow-up. Larger, prospective studies should be conducted to evaluate whether rotavirus gastroenteritis leads to AP-FGIDs in children.

Research and clinical challenges in paediatric inflammatory bowel disease.

Inflammatory bowel disease in childhood has become the subject of intense scientific debate during the last two decades, when there has been a significant rise in its incidence. There is a commonly agreed view that the disorder in children has peculiarities both in terms of underlying mechanisms and clinical management. This review highlights the emerging pathophysiologic concepts and clinical issues in paediatric inflammatory bowel disease and their effects on the management of children with this disorder are discussed. Particular emphasis is given to the link between the improvement of the research in the pathogenetic mechanisms and the development of novel therapeutic strategies able to promote a change in the natural course of the disorder.

Overlap between functional abdominal pain disorders and organic diseases in children. / Sobreposición entre los trastornos funcionales de dolor abdominal y enfermedades orgánicas en niños.

Functional abdominal pain disorders are highly prevalent in children. These disorders can be present in isolation or combined with organic diseases, such as celiac disease and inflammatory bowel diseases. Intestinal inflammation (infectious and non-infectious) predisposes children to the development of visceral hypersensitivity that can manifest as functional abdominal pain disorders, including irritable bowel syndrome. The new onset of irritable bowel syndrome symptoms in a patient with an underlying organic disease, such as inflammatory bowel disease, is clinically challenging, given that the same symptomatology may represent a flare-up of the inflammatory bowel disease or an overlapping functional abdominal pain disorder. Similarly, irritable bowel syndrome symptoms in a child previously diagnosed with celiac disease may occur due to poorly controlled celiac disease or the overlap with a functional abdominal pain disorder. There is little research on the overlap of functional abdominal disorders with organic diseases in children. Studies suggest that the overlap between functional abdominal pain disorders and inflammatory bowel disease is more common in adults than in children. The causes for these differences in prevalence are unknown. Only a handful of studies have been published on the overlap between celiac disease and functional abdominal pain disorders in children. The present article provides a review of the literature on the overlap between celiac disease, inflammatory bowel disease, and functional abdominal pain disorders in children and establish comparisons with studies conducted on adults.

Genetics and Gene Therapy in Hunter Disease.

Mucopolysaccharidosis type II or Hunter syndrome is an X-linked lysosomal storage disease caused by a mutation in the gene encoding the lysosomal enzyme iduronate-2-sulfatase. The consequent enzyme deficiency causes a progressive, multisystem accumulation of glycosaminoglycans, which is the cause of the clinical manifestations involving also Central Nervous System for patients with the severe form of disease. The limits of the currently available therapies for Hunter syndrome, hematopoietic stem cell transplantation and recombinant enzyme replacement therapy, mainly regarding brain achievement, have encouraged several studies which recognized gene therapy as a potential therapeutic option for this condition. In vitro studies firstly aimed at the demonstration that viral vector- mediated IDS gene expression could lead to high levels of enzyme activity in transduced cells. The encouraging results obtained allowed the realization of many preclinical studies investigating the utilization of gene therapy vectors in animal models of Mucopolysaccharidosis II, together with a phase I clinical trial approved for Hunter patients affected by the mild form of the disease. Together to in vivo studies in which recombinant vectors are directly administered, systematically or by direct injection into Central Nervous System, also ex vivo gene therapy, consisting in transplantation of autologous hematopoietic stem cells, modified in vitro, into the animal or patient, has been tested. A wider clinical application of the results obtained so far is essential to ensure that gene therapy can be definitively validated as a therapeutic option available and usable for this rare but life-threatening disorder.

Mind the gut: probiotics in paediatric neurogastroenterology.

The gut-brain axis has recently emerged as a key modulator of human health and the intestinal microbiome has a well-recognised pivotal role in this strong connection. The aim of this narrative review is to update and summarise the effect and clinical applicability of probiotics in paediatric neurogastroenterology. The Cochrane Database and PubMed were searched using keywords relating to different subtypes of functional gastrointestinal disorders (FGIDs) and their symptoms, those relating to the CNS and related neurological or behavioural dysfunction as well as 'probiotic' OR 'probiotics'. Included papers were limited to those including children (aged 0-18 years) and using English language. Although significant effects of specific strains have been reported in infants with FGIDs, heterogeneity amongst the studies (different products and concentrations used and FGID subtypes), has limited the ability to draw an overall conclusion on the clinical value of probiotics. According to different meta-analyses of randomised controlled trials, the use of Lactobacillus reuteri (DSM 17938) was associated with a significant decrease in average crying time in infantile colic. There is moderate evidence for this strain and LGG and limited evidence (based on one study each) for the beneficial effect of VSL#3 and a three-strain bifidobacteria mix in abdominal pain FGIDs, particularly in the irritable bowel disease subgroup of children, but not in functional dyspepsia. There is currently no clear evidence of positive effects of oral probiotics in autistic spectrum disorder. Efficacy and safety of other strains or beneficial effects in other conditions still need to be proven, as probiotic properties are strain-specific, and data cannot be extrapolated to other brain-gut or mood diseases or to other probiotics of the same or different species. To transform the use of probiotics from a tempting suggestion to a promising treatment modality in neurogastroenterological disorders more accurate differentiation of the efficacy-proven strains, clarification of dose, duration, and outcome and a careful selection of the target patients are still necessary.

Home infusion program with enzyme replacement therapy for Fabry disease: The experience of a large Italian collaborative group.

Fabry disease (FD) [OMIM 301500] is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A, resulting in progressive multisystem accumulation of globotriaosylceramide (Gb3). Although the introduction of Enzyme Replacement Therapy (ERT) resulted in a variety of clinical benefits, life-long intravenous (IV) treatment with ERT with an every other week schedule, may interfere with daily life activities and impact on QoL. We report here a multicentric, observational, longitudinal data analysis on a large cohort of 85 Italian FD patients (45 males, 40 females) from 11 out of 20 Italian regions, who received a cumulative number of 4269 home infusions of agalsidase alfa. For the whole cohort, the average duration of home therapy was 1 year and 11 months (range 3 months-4 years and 6 months), and during this period, compliance to treatment (number of infusions performed vs scheduled) reached 100%. The EQ-5 VAS scale was administered to patients to evaluate the self-reported QoL, 58% of patients showing an increase of EQ-5 VAS score at follow up compared to baseline (home treatment start) or remaining stable. A mild increase of average disease severity, measured through Mainz Severity Score Index (MSSI), was found during hospital treatment (p < 0,007), while it remained stable between the first home therapy infusion and last follow up. Interestingly, 4 out of 7 (57%) patients, showing an improvement in FD-related clinical status after starting home therapy, had previously a sub-optimal compliance to treatment during the period of hospital treatment management. Only 4 adverse non serious reactions (0,093%) were reported totally in 2 patients during home treatment. We conclude that home infusions in eligible patients with FD are safe, contribute to improve treatment compliance and therapeutic clinical outcomes, and may have a positive impact on self-perceived QoL.

Evidence favouring the gastro-oral route in the transmission of Helicobacter pylori infection in children.

OBJECTIVE: Several studies support the view that Helicobacter pylori is acquired in early life and within families. However, the exact route of transmission remains unknown. Given that H. pylori colonizes only the human gastric mucosa, the hypothesis that history of vomiting in siblings may be a relevant risk factor was tested in a paediatric setting. METHODS: One hundred urban children (age range 0.8-16.6 years, median 9), 44% with evidence of active H. pylori infection, were recruited. A structured questionnaire dealing with socio-economic issues was completed. Vomiting siblings and siblings of vomiting index children were screened for H. pylori by means of (13)C-urea breath test. Serum samples from index children were assayed for immunoglobulin G to hepatitis A (HAV) and Epstein-Barr virus (EBV) in order to check for faecal-oral and oral-oral exposure, respectively. RESULTS: Vomiting siblings of H. pylori-infected index children and siblings of H. pylori-infected vomiting index children had a high rate of active H. pylori infection (60 and 67%, respectively). History of vomiting in siblings was positively associated with active H. pylori infection in the index children (multivariate odds ratio 2.4, 95% confidence interval 1.3-4.3). Seropositivity for HAV and EBV was found in 1 and 68 index children, respectively. The agreement between active H. pylori infection and EBV seropositivity was not significant (kappa = 0.26). CONCLUSIONS: History of vomiting in siblings is an independent risk factor for H. pylori. Nowadays, transmission of H. pylori in urban children may involve the gastro-oral route more than the faecal-oral or oral-oral pathways.

Expression of proinflammatory and Th1 but not Th2 cytokines is enhanced in gastric mucosa of Helicobacter pylori infected children.

BACKGROUND: Helicobacter pylori-induced gastric inflammation is thought to be largely regulated by cytokines. PATIENTS AND METHODS: The expression of interferon-gamma, interleukin-12, interleukin-4, interleukin-10, interleukin-8, and interleukin-17 mRNA was examined on gastric mucosal samples from 24 children by semiquantitative reverse transcription polymerase chain reaction and southern blotting. Biopsy-based tests, serology, and urea 13C breath test were used to assess Helicobacter pylori status. Gastric biopsies were also evaluated for bacterial density, chronic inflammation, and acute inflammatory activity. RESULTS: Interferon-gamma, interleukin-12, interleukin-8 and interleukin-17 expression was higher in Helicobacter pylori-infected (n=13) than uninfected (n=11) children. Conversely, interleukin-4 and interleukin-10 expression did not differ between Helicobacter pylori-infected and uninfected children. In Helicobacter pylori-infected children, interferon-gamma, interleukin-12, interleukin-8 and interleukin-17 expression correlated with bacterial density, and Interferon-gamma and interleukin-12 expression with chronic inflammation score. CONCLUSIONS: The findings of this study indicate that, in children, Helicobacter pylori-induced inflammatory response would favour production of proinflammatory cytokines and development of cell-mediated immunity, namely Th1 response.

[Success of antegrade enemas in children with functional constipation]. / Efficacia di clisteri "anterogradi" in bambini con stipsi funzionale.

AIM: To assess the benefit of antegrade enemas via cecostomy in children with functional constipation unresponsive to medical treatment. METHODS: A retrospective chart review identified 19 children (12 male, age 10.4 +/- 4.3 yrs) who from 1998-2001 underwent placement of a cecostomy for administration of antegrade enemas. All children were neurologically normal, suffered from functional constipation; all had a history of using multiple medications, having daily soiling, and 65% had prior hospitalizations due to fecal impaction. Sixteen of 19 children had undergone colonic manometry which ruled out colonic inertia. One of 3 irrigation solutions: polyethylene glycol (65%), saline and glycerin solution mix (10%) and phosphate enema (25%) administered through the cecostomy everyday in 14 children or every other day in 5 children. We used a questionnaire to interview caregivers, a mean of 21.1 +/- 24.9 mo after cecostomy placement. RESULTS: In all patients antegrade enemas led to significant improvement of: bowel movements/wk (7.4 vs. 1.4, p < 0.001), soiling accidents/wk (1.1 vs. 6.1, p < 0.001), emotional health score (3.8 vs. 1.8, p < 0.001), overall health score (3.7 vs. 1.8, p < 0.001), number of medications for constipation (0.8 vs. 4.2, p < 0.001), number of missed school days/mo (1.3 vs. 10.5, p < 0.001), and number of physician office visits/yr (7.7 vs. 24.1, p < 0.002). Eight patients have been able to discontinue the use of the antegrade enemas within a mean of 19.9 +/- 14.2 mo after beginning treatment. CONCLUSION: Antegrade enemas are an alternative effective way for treating severe cases of functional constipation unresponsive to medical management.

[Oral rehydration in infants with acute diarrhea: using a new preparation of reduced osmolarity]. / Reidratazione orale in bambini con diarrea acuta: utilizzo di una preparazione a ridotta osmolarità.

Oral rehydration solutions (ORS) are widely employed in the treatment of acute diarrhea in children. Although several studies in the recent literature prove the efficacy and safety of newly formulated, reduced-osmolarity solutions, and a specific working group of the European Society for Pediatric Gastroenterology and Nutrition (ESPGAN) has made similar recommendations, such evidence in our Country is lacking. To this aim, we enrolled 38 children (age from 3 to 24 months, average: 8.2) with acute diarrhea lasting from no more than 5 days (average: 2.5). At observation, dehydration was found to be: absent in 12 (31.6%), mild in 19 (50%), moderate in 5 (13.2%), severe in 2 (5.2%). The last 2 patients were admitted and treated i.v., thus excluded from the study. In the 36 remaining children, a pathogen was identified in 25 (69.4%): Rotavirus in 18, Salmonella in 4, EPEC in 2, Cryptosporidium in 1. Stool Clinitest was positive in 7/31 children, all with Rotavirus infection. All the patients received ad libitum for the first 4-6 hours exclusively a reduced-osmolarity ORS formulated according to the ESPGAN criteria. Afterwards, they were fully re-fed and were also given the same ORS until diarrhea stopped. Most of the children accepted the ORS; those who refused it were either not dehydrated and/or older than 15 months. Thirty-three out of 36 children were fully rehydrated after 12 hours, without any side effects. We conclude that such an ORS is safe and effective in the treatment of children with acute diarrhea in our settings.

Helicobacter pylori infection in children with celiac disease: prevalence and clinicopathologic features.

BACKGROUND: Celiac disease is frequently associated with chronic gastritis. Helicobacter pylori is the main etiologic agent of chronic gastritis. The aim of this study was to assess the prevalence of H. pylori, the related symptoms, and the endoscopic and histologic gastric features in children with celiac disease. METHODS: Eight-one (24 boys, 57 girls; age range: 1.4-17.7 years, median 6.8) children with celiac disease were studied. All children had a blood sample taken. In a subgroup of 30 children who underwent endoscopy, three gastric biopsy specimens were taken for histology (hematoxylin and eosin, Giemsa, immunohistochemistry) and urease quick test. Symptom complaints were recorded. Age- and sex-matched (one case, one control) children without celiac disease were used for comparison. Serum H. pylori IgG were measured by means of a locally validated commercial enzyme-linked immunoassay. RESULTS: Overall, 15 of 81 (18.5%) children with celiac disease and 14 of 81 (17.3%) control children were positive for H. pylori. The percentage of H. pylori positivity was similar in children with untreated and treated celiac disease. Recurrent abdominal pain was the only symptom that helped to distinguish between H. pylori-positive and H. pylori-negative children. However, symptoms disappeared in patients with celiac disease after gluten withdrawal, irrespective of H. pylori status. All endoscopic (erythema, nodularity) and histologic (superficial-, interstitial-, lymphocytic-gastritis, activity, lymphoid follicles) findings did not differ between celiac and nonceliac H. pylori-positive children. CONCLUSIONS: Prevalence and clinical expressivity of H. pylori infection is not increased in children with celiac disease. The clinicopathologic pattern of the infection is not specifically influenced in this condition.