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עמוד בית
Sun, 23.06.24

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November 2008
Eyal Shteyer, MD and Michael Wilschanski, MD.
Management of inflammatory bowel disease in childhood poses great challenges. Apart from the disease complications, the drugs' adverse affects, especially corticosteroids, are significant. In the past decade there was major progress in elucidating the pathogenesis of IBD[1], which led to new treatment options aiming to achieve better control of the disease and decrease the various complications of therapy currently used. In this review we provide an overview of novel therapies for IBD, their efficacy, safety and their current use in children.





[1] IBD = inflammatory bowel disease


July 2008
C. Hartman, D. Berkowitz, B. Weiss, R. Shaoul, A. Levine, O. Eshach Adiv, R. Shapira, A. Fradkin, M. Wilschanski, A. Tamir and R. Shamir

Background: A polymeric diet rich in transforming growth factor-beta 2 used as a single nutrient has been shown to induce remission in 79% of children with Crohn's disease.

Objectives: To summarize the experience of several pediatric gastroenterology units in Israel using a TGFβ2[1]-enriched polymeric diet (Modulen IBD) supplementation in children and adolescents with Crohn's disease.

Methods: In a retrospective study we reviewed the charts of 28 children with Crohn's disease (10 girls, 18 boys) who received, in addition to conventional treatment, Modulen IBD™ as a supplement to their regular nutrition. These children were compared with 18 children supplemented with standard polymeric formula (Ensure Plus®) and 18 children without formula supplementation. We recorded clinical manifestations, growth, and the Pediatric Crohn's Disease Activity Index before and after initiation of the polymeric diet.

Results: The Modulen-treated children showed a significant decrease in PCDAI[2] from 34.3 to 15.7 (P < 0.0001). A significant decrease in PCDAI was recorded also in the Ensure Plus group, from 35 to 22 (P = 0.02) but not in the non-supplemented group. Significant improvements in body mass index (P = 0.01) and erythrocyte sedimentation rate (P = 0.03) were recorded at follow-up (median 3.4 months) only in the Modulen IBD group.

Conclusions: In this cohort of children with Crohn's disease, supplementation of the diet with Modulen IBD as well as supplementation with Ensure Plus was associated with a decrease in PCDAI. The children supplemented with Modulen IBD also showed improvement in BMI[3], suggesting an additional advantage of nutritional therapy in children with this disease.






[1] TGF-β2 = transforming growth factor-β2

[2] PCDAI = Pediatric Crohn's Disease Activity Index

[3] BMI = body mass index


February 2000
Yitzhak Lotem MD, Asher Barak MD, Huda Mussaffi MD, Mordechai Shohat MD, Michael Wilschanski MD, Yakov Sivan MD and Hannah Blau MD

Background: Cystic fibrosis is the most common life-limiting autosomal recessive genetic disorder in Caucasians. Typically it is a multisystem disease diagnosed by increased chloride levels on sweat testing, with mortality due mainly to progressive respiratory disease. The clinical spectrum of CF has recently been much expanded.

Genetic testing for mutant CF transmembrane regulator has revealed atypical cases where sweat test results are borderline or normal. In other patients, genetic mutations cannot be identified but abnormal CFTR function is shown using nasal potential difference measurement.

Objectives: To highlight the diagnostic and therapeutic dilemmas in cases of atypical cystic fibrosis.

Methods: We reviewed patients with atypical CF and widely varying phenotype who are managed at Schneider Children’s Medical Center of Israel. 

Results: Two patients had severe lung disease but little expression in other organs. Accurate diagnosis was essential to enable aggressive therapy in a specialized center. Four other patients are in excellent general health but have symptoms limited to male infertility, heat exhaustion, pancreatitis or transient liver dysfunction, while lung disease is minimal. For these patients, careful counseling is needed to avoid unnecessary upheaval, inappropriately aggressive management, and the psychosocial implications of a CF diagnosis. These dilemmas have increased considerably in our center, as in others worldwide.

Conclusion: It is our obligation as clinicians - at the level of both primary physician and referral center - to maintain an ever higher index of suspicion for CF, tempered by a rational program of counseling and management appropriate to the individual.

 

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CF= cystic fibrosis

CFTR= CF transmembrane regulator

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