Therapeutic approach to pediatric patients with acute chest syndrome: national multicenter survey of non invasive ventilation (NIV) and transfusion.

BACKGROUND: Intensive care units (ICUs) have seen a spike in the use of noninvasive ventilation (NIV) for many medical conditions. We sought to investigate the attitudes and clinical practice regarding the management of acute chest syndrome (ACS) with a focus on NIV in pediatric ICUs. METHOD: Members of the French Group for Pediatric Intensive Care Emergencies (GFRUP) were asked to complete an online survey on physicians' attitudes toward children with ACS admitted to the PICU during 2015. RESULTS: The survey was answered by teams from 17 PICUs (240 beds). In total, 15 centers (88%) had a local transfusion unit and 14 (82%) worked in connection with a sickle cell disease (SCD) reference center. During 2015, 360 patients with SCD were managed (median: 7 per center; 21) of whom 137 (38%) for an ACS (median: 4 ACS per center; 8). The median length of PICU stay for ACS was 5 days (3.1). Among the 137 patients who presented with ACS, 73 (53%) received simple blood transfusion and 16 (12%) received exchange transfusion. For patients who required noninvasive ventilatory support, NIV with bilevel pressure (BiPAP) was the most frequent method (n = 68, 50%), followed by continuous positive airway pressure (CPAP) (n = 23, 17%) and high-flow oxygen (n = 21, 15%). The proportion of patients on BiPAP was up to 71% in the centers most frequently managing ACS patients. CONCLUSION: BiPAP is commonly used in PICUs for SCD patients with ACS, especially in trained centers. Future physiological studies and randomized controlled trials might help to choose the best ventilatory support for ACS.

[Extracorporeal membrane oxygenation in critically ill neonates and children]. / Place de l'assistance respiratoire et circulatoire extracorporelle de courte durée (ECMO), post-cardiotomie exclue, dans la prise en charge des défaillances graves du nouveau-né et de l'enfant.

Extracorporeal membrane oxygenation is used as a last resort during neonatal and pediatric resuscitation in case of refractory circulatory or respiratory failure under maximum conventional therapies. Different types of ECMO can be used depending on the initial failure. The main indications for ECMO are refractory respiratory failure (acute respiratory distress syndrome, status asthmaticus, severe pneumonia, meconium aspiration syndrome, pulmonary hypertension) and refractory circulatory failure (cardiogenic shock, septic shock, refractory cardiac arrest). The main contraindications are a gestational age under 34 weeks or birth weight under 2kg, severe underlying pulmonary disease, severe immune deficiency, a neurodegenerative disease and hereditary disease of hemostasis. Neurological impairment can occur during ECMO (cranial hemorrhage, seizure or stroke). Nosocomial infections and acute kidney injury are also frequent complications of ECMO. The overall survival rate of ECMO is about 60 %. This survival rate can change depending on the initial disease: from 80 % for meconium aspiration syndrome to less than 10 % for out-of-hospital refractory cardiac arrest. Recently, mobile ECMO units have been created. These units are able to perform ECMO out of a referral center for untransportable critically ill patients.

A pilot study comparing two polymethylpentene extracorporeal membrane oxygenators.

OBJECTIVE: We compared two polymethylpentene oxygenators being used in our unit: the Maquet Quadrox-iD paediatric and the Medos Hilite 800LT. STUDY DESIGN: A mono-centric, prospective pilot study was conducted on ten consecutive newborn patients who had been admitted to our hospital service for extracorporeal circulation (EC) treatment. We examined the rate of oxygen transfer, the CO2 removal capacity and the average sweep gas flow required to produce this result. We also assessed the disturbances of haemostasis, the need for labile blood products and the membrane oxygenator lifetime and cost of use. CONCLUSIONS: According to our study, it seems to us that Medos Hilite 800LT membrane oxygenators demonstrate greater oxygen transfer and CO2 removal capacity than Maquet Quadrox-iD paediatric membrane oxygenators, at a similar cost. These results lead us to conclude that it is reasonable to continue using Medos Hilite 800LT membrane oxygenators. A broader comparison study would be necessary in order to support these initial results.

Azathioprine for prevention of postoperative recurrence in Crohn's disease: a retrospective study.

BACKGROUND: The efficacy of azathioprine (AZA) in chronically active Crohn's disease (CD) is well established. Whether this drug is also useful to prevent recurrences after surgery is unknown. We report here our experience of AZA in this therapeutic goal. METHODS: Between 1987 and 1996, 38 patients with CD were treated with AZA to prevent postoperative recurrence. Twenty-three of these had undergone a curative resection with removal of all previously involved parts of the gut. In the other 15 patients, resection was limited to the parts of the gut macroscopically abnormal at the time of surgery; those parts that were previously involved but normal at this time were conserved. The operative procedures were ileocolonic resection (n = 18), subtotal colectomy with ileorectal anastomosis (n = 12), coloproctectomy with ileo-anal anastomosis (n = 4) or ileostomy (n = 2), ileal resection (n = 1) and segmental colectomy (n = 1). Twelve patients had been treated previously with AZA before surgery; in 26 patients, AZA was started within the 2 months following surgery. RESULTS: The median duration of postoperative follow-up was 29 months. Probabilities of clinical recurrence according to the Kaplan-Meier method were 9, 16 and 28% at 1, 2 and 3 years, respectively. For the 25 patients who had a colonoscopy or a small bowel barium X-ray during the follow-up, probabilities of anatomical recurrence were 16, 36 and 59% at 1, 2 and 3 years, respectively. The probability of anatomical recurrence was significantly higher in patients who had segments of the gut previously involved but not removed because they were macroscopically normal at the time of surgery. CONCLUSION: In patients treated with AZA, the rate of postoperative endoscopic recurrence was lower than that previously reported in untreated patients. Our results suggest that AZA should be evaluated prospectively for prevention of postoperative CD recurrence, at least in high-risk patients.

Gastrointestinal lymphoma: prevention and treatment of early lesions.

Gastrointestinal lymphomas comprise a group of distinct clinicopathological entities. Differences in lifestyle and environmental factors between countries could account for the variety in the distribution of the main subtypes: low-grade B-cell lymphomas of the mucosa-associated lymphoid tissue type, alpha-chain disease and enteropathy (coeliac disease)-associated T-cell lymphoma (EATL). The possibility of preventing these lymphomas implies a knowledge of their natural history together with an identification of potential predisposing factors. The development of the lymphoid hyperplasia and subsequently low-grade lymphoma with the possibility of high-grade transformation is a multifactorial process involving both antigenic and host-related factors. The pathogenic role of Helicobacter pylori and gluten has been demonstrated in gastric lymphoma and enteropathy-associated T-cell lymphoma respectively, while environmental factors, especially non-specific bacterial ones, may play a major role in the pathogenesis of alpha-chain disease. The most difficult task in preventing these lymphomas is the recognition of early lesions likely to regress after the removal of the exogenous stimulus.

Intraluminal modulation of gastric sensitivity to distension: effects of hydrochloric acid and meal.

Conscious sensations in response to gut distensions may be modulated by temporospatial interactions among different stimuli. This study investigated whether symptoms induced by gastric distension may be modified by hydrochloric acid (HCl) gastric infusion and meal ingestion. In nine healthy subjects, fixed pressure (isobaric) and fixed volume (isovolumetric) distensions were performed during continuous (4 ml/min) intragastric saline or HCl infusion, during fasting and after meal ingestion, until the maximal distension step defined as discomfort or a predefined maximal volume. During fasting isobaric distensions, the maximal distension step was significantly decreased during HCl compared with saline. The intragastric volumes were not significantly different, but the wall tension was significantly lower during HCl than saline. HCl increased gastric compliance. Meal ingestion relaxed the stomach and decreased the pressure at the maximal distension step during saline, but HCl did not further decrease it compared with fasting. During isovolumetric distensions, HCl also increased gastric compliance, but in both fasted and fed states it did not modify the maximal distension steps. In conclusion, sensations in response to gastric isobaric distensions, but not to isovolumetric distensions, are influenced by gastric acid infusion and meal ingestion. The effects of HCl might be related to a sensitization of mucosal mechanoreceptors.

Resistance of t(11;18) positive gastric mucosa-associated lymphoid tissue lymphoma to Helicobacter pylori eradication therapy.

20-30% of gastric mucosa-associated lymphoid tissue (MALT) lymphoma associated with Helicobacter pylori do not regress after antibiotic therapy. Regression can be assessed only by extended follow-up. To assess whether t(11;18, q21;q21), which results in a chimeric transcript between the AP12 and MLT genes, predicts lymphoma resistance to antibiotic therapy, we screened for the fusion transcript with RT-PCR in ten responsive and 12 non-responsive gastric MALT lymphomas. The AP12-MLT transcript was detected in nine (75%) of 12 patients non-responsive to antibiotic therapy but not in responsive patients. Most H pylori-associated gastric MALT lymphomas that do not respond to antibiotic therapy are associated with t(11;18, q21;q21).

Predictive factors for regression of gastric MALT lymphoma after anti-Helicobacter pylori treatment.

BACKGROUND AND AIMS: Discrepant remission rates (41-100%) have been reported for patients with localised low grade gastric mucosa associated lymphoid tissue (MALT) lymphoma after eradication of Helicobacter pylori. The aim of this study was to explain these discrepancies and to determine the predictive factors of gastric lymphoma regression after anti- H pylori treatment. PATIENTS AND METHODS: Forty six consecutive patients with localised gastric MALT lymphoma (Ann Arbor stages I(E) and II(E)) were prospectively enrolled. All had gastric endoscopic ultrasonography and H pylori status assessment (histology, culture, polymerase chain reaction, and serology). After anti-H pylori treatment, patients were re-examined every four months. RESULTS: Histological regression of the lymphoma was complete in 19/44 patients (43%) (two lost to follow up). Median follow up time for these 19 responders was 35 months (range 10-47). No regression was noted in the 10 H pylori negative patients. Among the 34 H pylori positive patients, the H pylori eradication rate was 100%; complete regression rate of the lymphoma increased from 56% (19/34) to 79% (19/24) when there was no nodal involvement at endoscopic ultrasonography. There was a significant difference between the response of the lymphoma restricted to the mucosa and other more deep seated lesions (p<0.006). However, using multivariate analysis, the only predictive factor of regression was the absence of nodal involvement (p<0.0001). CONCLUSION: In H pylori positive patients with localised gastric MALT lymphoma, carefully evaluated and treated without any lymph node involvement assessed by endoscopic ultrasonography, complete remission of lymphoma was reached in 79% of cases.

Methotrexate in Crohn's disease: long-term efficacy and toxicity.

OBJECTIVE: A 16-wk, placebo-controlled trial has recently shown weekly low-dose methotrexate to be an effective treatment for patients with chronically active Crohn's disease. The long-term efficacy and safety of this antimetabolite drug, however, are not yet well established and are assessed in this study. METHODS: A total of 49 patients with Crohn's disease who were treated with methotrexate for > or =6 months were studied. All patients had been chronically treated with steroids; but at the time of initiation, only 27 were still on steroids. Of the 49 patients, 42 had previously taken azathioprine but were no longer on this drug because of intolerance or failure. Clinical remission was defined as a Harvey-Bradshaw index of <4. RESULTS: In all, 41 patients achieved complete clinical remission and were maintained on methotrexate for a median of 18 months (range, 7-59 months). In these patients the probabilities of relapse were 29%, 41%, and 48% at 1, 2, and 3 yr, respectively. A higher rate of relapse was observed in women and in patients with ileocolitis. Adverse reactions were recorded in 24 patients, requiring discontinuation of methotrexate in five. A liver biopsy was performed in 11 patients; a mild steatosis was found in five, a slight dilation of the sinusoids in one, a granulomatous hepatitis with a mild portal fibrosis in one, and a slight periportal fibrosis in one patient. CONCLUSIONS: This study suggests a long-term benefit of maintenance treatment with methotrexate in patients with chronically active Crohn's disease, with side effects that are usually only moderate.

Management and long-term results of surgery for localized gastric lymphomas.

BACKGROUND: High- and low-grade gastric lymphomas (GL) differ in their behavior and chemosensitivity. Surgery has to be reevaluated according to the histologic grade of malignancy. We aimed to assess the place of surgery in the management of GL and its results after long-term follow-up. METHODS: Among 54 patients with primary GL prospectively enrolled from 1984 to 1990, 45 with localized disease were studied. Primary resection was done whenever safe. All patients received chemotherapy adapted to the grade of malignancy and/or to the completeness of the resection. RESULTS: Among 18 low- and 27 high-grade GL, 35 patients had primary resections; of those, 23 were complete. The complete response rate for all patients with low- and high-grade GL was 67% and 89%, respectively. After a median follow-up of 8 years, the disease-free survival rates for low-grade GL and high-grade GL were 94% and 89%, respectively. It was better after complete resection. CONCLUSION: Complete resection is a major determinant of prolonged complete remission.

[Isolated jejunal tuberculosis mimicking Crohn disease. Diagnosis by push video-enteroscopy]. / Tuberculose jéjunale isolée simulant une maladie de Crohn. Diagnostic par vidéo-entéroscopie poussée.

Intestinal tuberculosis is relatively unfrequent in Western countries, but immigrants and AIDS patients remain groups at particular risk for this disease. The diagnosis of intestinal tuberculosis is often difficult to establish because of close similarities with other conditions, in particular Crohn's disease. We report a case of jejunal tuberculosis in a 33-year-old man with severe weight loss and unexplained fever. The diagnosis was obtained on histological examination of the distal jejunum biopsies performed during pushed video-enteroscopy. Interestingly, culture of the biopsies and specific PCR remained negative. Dramatic improvement was observed during the first days of antituberculous treatment. The main clinical and paraclinical manifestations of intestinal tuberculosis are also reviewed, as well as recent epidemiologic observations and new developments in diagnosis and treatment.

The clinicopathological features of extensive small intestinal CD4 T cell infiltration.

METHODS: Four patients with clinicopathological features suggesting a new distinct entity defining extensive small intestinal CD4 T cell infiltration were observed. RESULTS: All four patients presented with chronic diarrhoea, malabsorption, and weight loss. Biopsy specimens of the small intestine disclosed extensive and diffuse infiltration of the lamina propria by pleomorphic small T lymphocytes, which were positive for CD3, CD4, CD5, and the beta chain of T cell receptor in all three cases studied and negative for CD103 in all three cases studied. It is notable that, in all invaded areas, the infiltrating cells showed no histological change throughout the whole evolution. In three patients, lymphocyte proliferation was monoclonal and there was extraintestinal involvement. In one patient, lymphoproliferation was oligoclonal and confined to the small intestine. In all four patients, there was no evidence of coeliac disease. Although none of the four patients responded to single or multiple drug chemotherapy, median survival was five years. CONCLUSION: Extensive small intestinal CD4 T cell infiltration is a rare entity, distinct from coeliac disease and associated with prolonged survival.

Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome.

BACKGROUND & AIMS: The short bowel syndrome (SBS) may be associated with either transient or permanent intestinal failure, presently treated by parenteral nutrition (PN). Survival and PN-dependence probabilities, taking into account both small bowel remnant length and the type of the digestive circuit of anastomosis, are not known in adult SBS patients. The aim of this study was to assess such prognostic factors. METHODS: A total of 124 consecutive adults with nonmalignant SBS were enrolled from 1980 to 1992 at 2 home PN centers. They were analyzed for survival and PN-dependence probabilities using the Cox model and for PN dependence using linear discriminant analysis. Data were updated in April 1996. RESULTS: Survival and PN-dependence probabilities were 86% and 49% and 75% and 45% at 2 and 5 years, respectively. In multivariate analysis, survival was related negatively to end-enterostomy, to small bowel length of <50 cm, and to arterial infarction as a cause of SBS, but not to PN dependence. The latter was related negatively to postduodenal small bowel lengths of <50 and 50-99 cm and to absence of terminal ileum and/or colon in continuity. Cutoff values of small bowel lengths separating transient and permanent intestinal failure were 100, 65, and 30 cm in end-enterostomy, jejunocolic, and jejunoileocolic type of anastomosis, respectively. CONCLUSIONS: In adult SBS patients, small bowel length of <100 cm is highly predictive of permanent intestinal failure. Presence of terminal ileum and/or colon in continuity enhances both weaning off PN and survival probabilities. After 2 years of PN, probability of permanent intestinal failure is 94%. These rates may lead to selection of other treatments, especially intestinal transplantation, instead of PN, for permanent intestinal failure caused by SBS.

Antibiotic efficacy in small intestinal bacterial overgrowth-related chronic diarrhea: a crossover, randomized trial.

BACKGROUND & AIMS: No controlled trial has examined the clinical efficacy of antibiotics in small bowel bacterial overgrowth. METHODS: Ten patients with bacterial overgrowth-related diarrhea underwent the following five 7-day treatment periods: untreated (control period), then placebo, and subsequently, in random order and blinded fashion, norfloxacin (800 mg/day), amoxicillin-clavulanic acid (1500 mg/day), and Saccharomyces boulardii (1500 mg/day). A hydrogen breath test was performed on the first and last day of each period. RESULTS: Daily stool frequency was similar during the control and placebo periods (4.2 +/- 0.6 vs. 3.9 +/- 0.6 [mean +/- SEM], respectively). Norfloxacin and amoxicillin-clavulanic acid led to a significant reduction in daily stool frequency (2.3 +/- 0.4 and 3.0 +/- 0.5, respectively; P < 0.01 vs. placebo period) after 2.0 +/- 1.4 and 1.2 +/- 0.4 days, which was maintained for 6.1 +/- 3.7 and 6.0 +/- 9.6 days, respectively. Breath-expired H(2) volume decreased with norfloxacin (37 +/- 8 to 12 +/- 5 mL per 2 hours; P < 0.01) and amoxicillin-clavulanic acid (24 +/- 6 to 8 +/- 4 mL per 2 hours, respectively; P = 0.01), but H(2) breath test result was negative in only 3 and 5 patients. CONCLUSIONS: Norfloxacin and amoxicillin-clavulanic acid are effective in the treatment of bacterial overgrowth-related diarrhea.

[Pregnancy outcome in inflammatory bowel diseases]. / Pronostic de la grossesse au cours des maladies inflammatoires intestinales.

OBJECTIVE: To assess pregnancy outcome in relation to disease activity and maintenance therapy in patients with inflammatory bowel disease. METHODS: A postal questionnaire was sent to every woman of child-bearing age followed for inflammatory bowel disease in three referral centers (Rothschild, Saint-Lazare, Saint-Louis). Response rate was 65%. RESULTS: One hundred and forty-four pregnancies (153 fetuses) in 138 women (122 had Crohn's disease) were reported. Outcome of pregnancy was normal (baby > 2500 g, without malformation) in 115 cases (77%). There were 17 cases of preterm birth (11.5%), 3 cases of hypotrophy, and 14 pregnancy losses (9 miscarriages (6%), 4 therapeutic abortions for major malformation (2.8%)). Percentages of normal pregnancy outcome were not different between patients who continued maintenance therapy and those who stopped: respective percentages were 75 vs 73% in patients receiving mesalamine or olsalazine (n = 30), and 60 vs 75% in patients receiving azathioprine (n = 22). CONCLUSION: In a selected series of women with inflammatory bowel disease, pregnancy outcome is approaching that observed in a normal population, except for an elevated rate of preterm births. There is no need to stop maintenance therapy with 5-aminosalicylates or azathioprine during pregnancy.