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1.
Arch Pediatr ; 30(5): 307-313, 2023 Jul.
Article in English | MEDLINE | ID: mdl-37236887

ABSTRACT

BACKGROUND: Collagen gastritis is a rare disease that manifests in children mainly as isolated gastric involvement associated with martial deficiency anemia. There are no recommendations for the management and follow-up of these patients. We aimed to describe the clinical data, endoscopic findings, and treatments deployed in France's children with collagenous gastritis. METHODS: All French pediatric gastroenterology centers and pediatric centers for rare digestive diseases (Centres de Maladies Rares Digestives) were contacted to collect cases of collagenous gastritis, defined on gastric biopsies and diagnosed before 18 years of age. RESULTS: A total of 12 cases diagnosed (4 males and 8 females) between 1995 and 2022 could be analyzed. The median age at diagnosis was 12.5 years (7-15.2). The most frequent clinical presentation was abdominal pain (6/11) and/or nonspecific symptomatology attributed to anemia (8/10). Anemia was present in all children (11/11; Hb 2.8-9.1 g/dL). Nodular gastritis was present in 10 patients (antrum: 2; fundus: 4; in antrum and fundus: 4). All patients had a basement membrane thickening (from 19 to 100 µm). The treatments received were PPI (11), oral or intravenous martial supplementation (12), budesonide (1), and prednisone (1). Martial supplementation improved anemia in all cases. At discontinuation, nine of 10 patients had a recurrence of anemia. CONCLUSION: Collagenous gastritis is an exceptional condition, clinically manifested in children as abdominal pain and iron deficiency anemia probably of hemorrhagic origin. Patients require long-term follow-up and monitoring of their disease to describe the risk of progression better.


Subject(s)
Anemia , Gastritis , Malabsorption Syndromes , Male , Female , Humans , Child , Gastritis/complications , Gastritis/diagnosis , Gastritis/therapy , Biopsy , Malabsorption Syndromes/complications , Anemia/complications , Abdominal Pain/etiology
2.
Eur J Pediatr Surg ; 2023 Apr 11.
Article in English | MEDLINE | ID: mdl-36882155

ABSTRACT

OBJECTIVE: The aim of this retrospective study was to describe the risk of postoperative recurrence (POR) after ileocecal resection, the occurrence of surgical complications, and identify predictors of these adverse postoperative outcomes in pediatric Crohn's disease (CD). PATIENTS AND METHODS: All the children less than 18 years of age with a diagnosis of CD, who underwent primary ileocecal resection for CD between January 2006 and December 2016 in our tertiary center, were considered for inclusion. Factors related to POR were investigated. RESULTS: A total of 377 children were followed for CD between 2006 and 2016. During this period, 45 (12%) children needed an ileocecal resection. POR was diagnosed in 16% (n = 7) at 1 year and 35% (n = 15) at the end of the follow-up, with a median follow-up of 2.3 years (Q1-Q3 1.8-3.3). Median duration of the postoperative clinical remission was 1.5 years (range 0.5-2). Multivariate Cox regression analysis identified only young age at diagnosis as a risk factor for POR.In total, 7 of the 43 patients (16%) developed severe postoperative complications, defined as requiring surgical, endoscopic, or radiological intervention. The only risk factor was intraoperative abscess. CONCLUSION: Only young age at diagnosis was associated with POR. This information could be useful to develop targeted therapeutic strategies for young CD children. At the end of follow-up with a median follow-up of 2.3 years (Q1-Q3 1.8-3.3), there was no surgical POR: endoscopic dilatation for POR should be considered in order to delay or prevent surgery.

4.
J Bacteriol ; 199(14)2017 07 15.
Article in English | MEDLINE | ID: mdl-28484043

ABSTRACT

The phospholipid (PL) composition of bacterial membranes varies as a function of growth rate and in response to changes in the environment. While growth adaptation can be explained by biochemical feedback in the PL synthesis pathway, recent transcriptome studies have revealed that the expression of PL synthesis genes can also be tuned in response to various stresses. We previously showed that the BasRS two-component pathway controls the expression of the diacylglycerol kinase gene, dgkA, in Escherichia coli (A. Wahl, L. My, R. Dumoulin, J. N. Sturgis, and E. Bouveret, Mol Microbiol, 80:1260-1275, 2011, https://doi.org/10.1111/j.1365-2958.2011.07641.x). In this study, we set up a strategy to identify the mutation responsible for the upregulation of pssA observed in the historical pssR1 mutant and supposedly corresponding to a transcriptional repressor (C. P. Sparrow and J. Raetz, J Biol Chem, 258:9963-9967, 1983). pssA encodes phosphatidylserine synthase, the first step of phosphatidylethanolamine synthesis. We showed that this mutation corresponded to a single nucleotide change in the anti-Shine-Dalgarno sequence of the 16S rRNA encoded by the rrnC operon. We further demonstrated that this mutation enhanced the translation of pssA Though this effect appeared to be restricted to PssA among phospholipid synthesis enzymes, it was not specific, as evidenced by a global effect on the production of unrelated proteins.IMPORTANCE Bacteria adjust the phospholipid composition of their membranes to the changing environment. In addition to enzymatic regulation, stress response regulators control specific steps of the phospholipid synthesis pathway. We wanted to identify a potential regulator controlling the expression of the phosphatidylserine synthase gene. We showed that it was not the previously suggested hdfR gene and instead that a mutation in the anti-Shine-Dalgarno sequence of 16S RNA was responsible for an increase in pssA translation. This example underlines the fact that gene expression can be modulated by means other than specific regulatory processes.


Subject(s)
Escherichia coli Proteins/metabolism , Escherichia coli/metabolism , Phospholipids/biosynthesis , Ribosomes/metabolism , Transcription Factors/metabolism , Escherichia coli/genetics , Escherichia coli Proteins/genetics , Gene Expression Regulation, Bacterial/physiology , Ribosomes/genetics , Transcription Factors/genetics
5.
Arch Pediatr ; 24(4): 391-398, 2017 Apr.
Article in French | MEDLINE | ID: mdl-28279616

ABSTRACT

Small-bowel capsule endoscopy (CE) has recently been used in children. During the past few years, an intense research activity has defined the advantages and limitations of CE. Its uses have been established in several small-bowel pathologies such as obvious or obscure digestive bleeding, Crohn disease, and hereditary polyposis. Although the absence of biopsy reduces the specificity of CE findings, small-bowel exploration using CE achieves better accuracy in detecting lesions than most radiological examinations. In children, swallowing problems and the risk of retention due to stenosis are the main concerns when using CE: these problems can be solved using endoscopic delivery of the capsule and luminal diameter calibration, respectively. This review focuses on the evidence making CE indispensable to small-bowel exploration.


Subject(s)
Capsule Endoscopy/methods , Intestinal Diseases/diagnosis , Intestine, Small , Adenomatous Polyposis Coli/diagnosis , Child , Crohn Disease/diagnosis , Gastrointestinal Hemorrhage/etiology , Humans , Intestinal Obstruction/etiology , Risk Factors , Sensitivity and Specificity
6.
Arch Pediatr ; 23(11): 1141-1145, 2016 Nov.
Article in French | MEDLINE | ID: mdl-27692552

ABSTRACT

Desmoid tumors (DT) are rare and nonmetastasizing fibroblastic neoplasms, characterized by local invasiveness. They occur sporadically or arise in the context of familial adenomatous polyposis (FAP; 5-10% of cases). Most cases develop sporadically in young adults, but some cases also occur in children. We report the case of an adolescent girl with FAP and DT, and we discuss the therapeutic strategies. An adolescent girl with FAP underwent surgery at the age of 14 years with total proctocolectomy. She had a neo-mutation in the APC gene at codon 1068, which is not usually associated with DT. Three years later, she had painful defecations. Imaging showed two abdominal DT. After a multidisciplinary team meeting, the patient was refused for surgery, and medical treatment with antihormonal agents and nonsteroidal anti-inflammatory drugs was started. Imaging 18 months later showed DT stabilization, but the patient had difficulties to control chronic pains, which required morphine treatment, hypnotic sessions, and transcutaneous electric nerve stimulation. This case highlights the importance of DT screening in patients with FAP, mainly after surgery, regardless of their age and genetic mutation. Progress remains to be made in determining DT risk factors and in developing treatment. DT are still difficult to cure because of their potential for local invasion and local recurrence, and need to be managed by a multidisciplinary team.


Subject(s)
Abdominal Neoplasms/pathology , Adenomatous Polyposis Coli/pathology , Fibromatosis, Aggressive/pathology , Neoplasms, Multiple Primary/pathology , Adenomatous Polyposis Coli/genetics , Adolescent , Female , Genes, APC , Humans
8.
J Bacteriol ; 197(11): 1862-72, 2015 Jun.
Article in English | MEDLINE | ID: mdl-25802297

ABSTRACT

UNLABELLED: In Escherichia coli, the FadR transcriptional regulator represses the expression of fatty acid degradation (fad) genes. However, FadR is also an activator of the expression of fabA and fabB, two genes involved in unsaturated fatty acid synthesis. Therefore, FadR plays an important role in maintaining the balance between saturated and unsaturated fatty acids in the membrane. We recently showed that FadR also activates the promoter upstream of the fabH gene (L. My, B. Rekoske, J. J. Lemke, J. P. Viala, R. L. Gourse, and E. Bouveret, J Bacteriol 195:3784-3795, 2013, doi:10.1128/JB.00384-13). Furthermore, recent transcriptomic and proteomic data suggested that FadR activates the majority of fatty acid (FA) synthesis genes. In the present study, we tested the role of FadR in the expression of all genes involved in FA synthesis. We found that FadR activates the transcription of all tested FA synthesis genes, and we identified the FadR binding site for each of these genes. This necessitated the reassessment of the transcription start sites for accA and accB genes described previously, and we provide evidence for the presence of multiple promoters driving the expression of these genes. We showed further that regulation by FadR impacts the amount of FA synthesis enzymes in the cell. Our results show that FadR is a global regulator of FA metabolism in E. coli, acting both as a repressor of catabolism and an activator of anabolism, two directly opposing pathways. IMPORTANCE: In most bacteria, a transcriptional regulator tunes the level of FA synthesis enzymes. Oddly, such a global regulator still was missing for E. coli, which nonetheless is one of the prominent model bacteria used for engineering biofuel production using the FA synthesis pathway. Our work identifies the FadR functional dual regulator as a global activator of almost all FA synthesis genes in E. coli. Because FadR also is the repressor of FA degradation, FadR acts both as a repressor and an activator of the two opposite pathways of FA degradation and synthesis. Our results show that there are still discoveries waiting to be made in the understanding of the genetic regulation of FA synthesis, even in the very well-known bacterium E. coli.


Subject(s)
Bacterial Proteins/metabolism , Escherichia coli/metabolism , Fatty Acids/biosynthesis , Gene Expression Regulation, Bacterial , Repressor Proteins/metabolism , Bacterial Proteins/chemistry , Bacterial Proteins/genetics , Base Sequence , Binding Sites , Escherichia coli/chemistry , Escherichia coli/genetics , Molecular Sequence Data , Promoter Regions, Genetic , Repressor Proteins/chemistry , Repressor Proteins/genetics , Transcription, Genetic
9.
Arch Pediatr ; 22(1): 32-8, 2015 Jan.
Article in French | MEDLINE | ID: mdl-25500066

ABSTRACT

Digestive complications related to the ingestion of magnetic foreign bodies in children are increasing, especially in Asia and North America. In France, several case reports have been reported since 2008. We conducted a retrospective multicentric study to evaluate the frequency of ingestion of magnet foreign bodies and to describe the complicated cases that have occurred in France over the last 5 years. We report 40 cases of which 60% were multiple magnet ingestions. Eighty-eight percent of the children of the group who had swallowed multiple magnets needed interventional management by endoscopy (33%) or surgery (58%). Only two children (12.5%) of the group who swallowed one magnet required removal. This problem is not uncommon in France (2% of the 1132 foreign bodies investigated in the Toulouse center over 5 years), which justifies clear information for healthcare professionals and caregivers in order to avoid potential intestinal complications. We suggest interventional management or very close monitoring in the cases of multiple magnet ingestion. Meanwhile, in the majority of confirmed cases of simple magnet ingestions, we propose home monitoring.


Subject(s)
Foreign Bodies/epidemiology , Foreign Bodies/surgery , Magnets , Age Distribution , Child , Child, Preschool , Endoscopy/statistics & numerical data , Female , France/epidemiology , Humans , Infant , Laparoscopy/statistics & numerical data , Male , Retrospective Studies , Sex Distribution
11.
Arch Pediatr ; 17(9): 1264-72, 2010 Sep.
Article in French | MEDLINE | ID: mdl-20627490

ABSTRACT

BACKGROUND AND STUDY AIMS: Capsule endoscopy (CE) is a novel and noninvasive means of investigating the small bowel. In children, the best CE indications have not yet been fully appraised. The aim of this study was to evaluate the diagnostic yield of CE in different pediatric pathologies. PATIENTS AND METHODS: We retrospectively reviewed every CE performed in children in two French pediatric hospitals between March 2002 and June 2009. Seventy-nine CEs were performed on 70 children (mean age, 10.6 years; range, 2.2-18.0); 52 boys and 18 girls. The indications were iron deficiency anemia (24%), obscure gastrointestinal bleeding (14%), polyposis syndromes (16%), suspected Crohn disease (15%), unresponsive Crohn disease (10%), graft-versus-host disease (10%), and other (10%). RESULTS: Of the 79 CEs, 69 reached the cecum (87%). Only one occlusion occurred in a case of stenosing Crohn disease, requiring surgical removal. In addition, technical difficulties led to an incomplete small bowel study in 12 cases (16%). The CE showed small bowel lesions in 42 cases (53%). The diagnostic yield was 27% in obscure gastrointestinal bleeding, 37% in iron-deficiency anemia, 42% in suspected Crohn disease, 88% in unresponsive Crohn disease, 62% in polyposis syndromes, and 88% in graft-versus-host disease. CONCLUSION: In children, CE is well tolerated and can be performed in children as young as 2.2 years of age. Its diagnostic yield is highest in polyposis syndromes, unresponsive Crohn disease, and graft-versus-host disease.


Subject(s)
Capsule Endoscopy , Gastrointestinal Diseases/diagnosis , Adolescent , Anemia, Iron-Deficiency/diagnosis , Capsule Endoscopy/adverse effects , Child , Child, Preschool , Crohn Disease/diagnosis , Diagnosis, Differential , Female , France , Gastrointestinal Hemorrhage/diagnosis , Graft vs Host Disease/diagnosis , Hospitals, Pediatric , Humans , Intestinal Polyposis/diagnosis , Male , Predictive Value of Tests , Retrospective Studies , Sensitivity and Specificity
12.
Gastroenterol Clin Biol ; 34(3): 202-8, 2010 Mar.
Article in English | MEDLINE | ID: mdl-20303225

ABSTRACT

AIM: The treatment of achalasia consists of reducing distal esophageal obstruction by either Heller myotomy surgery or endoscopic pneumatic dilatation. The aim of the present study was to evaluate the short- and middle-term results of these procedures in children. METHODOLOGY: For technical reasons, children under six years old (n=8) were treated by surgery only, whereas patients over six years old (n=14) were treated by either Heller myotomy or pneumatic dilatation. RESULTS: Of the children aged under six years, 75% were symptom-free at six months and 83% at 24 months of follow-up. Of the patients aged over six years, complete remission was achieved by Heller myotomy in 44.5% vs. 55.5% by pneumatic dilatation after six months, and in 40% vs. 65%, respectively, after 24 months. Both pneumatic dilatation and Heller myotomy showed significant rates of failure. CONCLUSION: These results suggest that pneumatic dilatation may be considered a primary treatment in children over six years old. Also, where necessary, Heller myotomy and pneumatic dilatation may be used as complementary treatments.


Subject(s)
Catheterization , Esophageal Achalasia/therapy , Esophageal Sphincter, Lower/surgery , Esophagectomy/methods , Adolescent , Child , Child, Preschool , Digestive System Surgical Procedures/methods , Esophageal Achalasia/physiopathology , Esophageal Achalasia/surgery , Female , Humans , Infant , Male , Manometry , Quality of Life , Retrospective Studies , Treatment Outcome
15.
Arch Pediatr ; 16(4): 368-71, 2009 Apr.
Article in French | MEDLINE | ID: mdl-19250810

ABSTRACT

Duodenal duplication is a rare congenital disorder of the gastrointestinal tract. The presentation is highly variable. We report a case of duodenal duplication presenting with hemorrhagic ascites in a 3-month-old girl. The diagnosis of duodenal duplication can be made preoperatively by resonance magnetic imaging. Surgical resection of the duplication was performed. Microscopic examination of the specimen confirmed the duodenal duplication. To our knowledge, this is the 1st reported case of hemorrhagic ascites caused by duodenal duplication and demonstrated by resonance magnetic imaging.


Subject(s)
Ascites/etiology , Duodenum/abnormalities , Hemorrhage/etiology , Duodenum/pathology , Female , Humans , Infant , Magnetic Resonance Imaging
17.
J Neuroradiol ; 35(2): 121-4, 2008 May.
Article in French | MEDLINE | ID: mdl-17904638

ABSTRACT

This paper describes MRI aspects of a leukodystrophy due to the Mitochondrial Neurogastrointestinal Encephalomyopathy syndrome in an adolescent girl investigated for nocturnal recurrent emesis leading to major cachexia.


Subject(s)
Gastrointestinal Diseases/diagnosis , Magnetic Resonance Imaging , Mitochondrial Encephalomyopathies/diagnosis , Adolescent , Cachexia/etiology , Diagnosis, Differential , Female , Gastrointestinal Diseases/complications , Humans , Mitochondrial Encephalomyopathies/complications , Vomiting/etiology
18.
Arch Pediatr ; 14 Suppl 3: S156-8, 2007 Oct.
Article in French | MEDLINE | ID: mdl-17961808

ABSTRACT

Rotavirus is the most frequent virus found in childhood gastroenteritis. A rotavirus viremia is observed in 19 to 63 % of cases, for three days at the beginning of infection. Then, rotavirus can reach several organs as liver (hepatitis in 1/3 of case), nervous central system (2 % of encephalitis could be linked to rotavirus), or more infrequently mesenteric lymph nodes, lung or heart. However, the link between rotavirus and systemic manifestations has not been well established. Further studies are necessary to confirm the role of rotavirus in these organ's lesions.


Subject(s)
Gastroenteritis/virology , Rotavirus Infections/physiopathology , Child , Child, Preschool , Encephalitis, Viral/physiopathology , Focal Infection/virology , Hepatitis, Viral, Human/physiopathology , Humans , Infant , Infant, Newborn , Viremia/virology
19.
Arch Pediatr ; 14 Suppl 3: S169-75, 2007 Oct.
Article in French | MEDLINE | ID: mdl-17961811

ABSTRACT

Acute infectious diarrhea in children remain still a frequent cause of morbidity. 50 % of them are due to rotavirus. Oral rehydration therapy and early realimentation have drastically reduced their mortality and morbidity. Beside oral or eventually IV rehydration therapy no medication has proven its efficacy based on the main HMO criteria (reduction of over 30 % of the stool output) except racecadotril and loperamide which is contre-indicated for the last one in children less than 2 years old. Other medications such as silicates or some probiotics have shown efficacy on diarrhea duration or stool consistency but not on stool output. They have so no formal indication in infectious diarrhea and should be considered as "comfort" treatment. Antibiotics, beside their indication in shigella, cholera and amibiasis could be used in invasive diarrhea in some debilating conditions or infants less than 3 months.


Subject(s)
Diarrhea, Infantile/drug therapy , Diarrhea/drug therapy , Acute Disease , Anti-Bacterial Agents/therapeutic use , Antidiarrheals/therapeutic use , Child , Child, Preschool , Dysentery/drug therapy , Fluid Therapy , Humans , Infant , Rehydration Solutions/therapeutic use
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