European journal of pediatrics
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Randomized Controlled Trial
Brief hypnotherapeutic-behavioral intervention for functional abdominal pain and irritable bowel syndrome in childhood: a randomized controlled trial.
Functional abdominal pain and irritable bowel syndrome are two prevalent disorders in childhood which are associated with recurrent or chronic abdominal pain, disabilities in daily functioning, and reduced quality of life. This study aimed to evaluate a brief hypnotherapeutic-behavioral intervention program in a prospective randomized controlled design. Thirty-eight children, 6 to 12 years of age, and their parents were randomly assigned to a standardized hypnotherapeutic-behavioral treatment (n = 20) or to a waiting list condition (n = 18). Both groups were reassessed 3 months after beginning. Primary outcome variables were child-completed pain measures and pain-related disability. Secondary outcome variables were parent-completed measures of their children's pain and pain-related disability. Health-related quality of life from both perspectives also served as a secondary outcome. In the treatment group, 11 of 20 children (55.0%) showed clinical remission (>80% improvement), whereas only one child (5.6%) in the waiting list condition was classified as responder. Children in the treatment group reported a significantly greater reduction of pain scores and pain-related disability than children of the waiting list condition. Parental ratings also showed a greater reduction of children's abdominal pain and pain-related disability. Health-related quality of life did not increase significantly. ⋯ Hypnotherapeutic and behavioral interventions are effective in treating children with long-standing AP. Treatment success of this brief program should be further evaluated against active interventions with a longer follow-up.
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A term infant was noted to have right-sided foot drop. We discuss the role of neurophysiology and diagnostic imaging.
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Donohue syndrome (leprechaunism; OMIM *246200) is a rare, recessively inherited disorder of extreme insulin resistance due to mutations in the insulin receptor gene (INSR) causing either defects in insulin binding or receptor autophosphorylation and tyrosine kinase activity. We report a patient with pronounced clinical picture of leprechaunism who developed severe progressive hypertrophic obstructive cardiomyopathy (HOCM) and renal tubular dysfunction which improved on continuous subcutaneous infusion of recombinant human insulin-like growth factor-1 (rhIGF-I). INSR gene molecular analysis and insulin receptor (IR) autophosphorylation on cultured fibroblasts were performed. A novel homozygous missense mutation p.Leu795Pro was found, located in the extracellular portion of the β subunit of the insulin receptor. The post-binding defect of the insulin receptor signaling in cultured fibroblasts demonstrated decreased insulin receptor autophosphorylation. ⋯ Treatment with rhIGF-I partially reversed severe progressive HOCM and renal tubular dysfunction in a patient with Donohue syndrome associated with a novel p.Leu795Pro INSR gene mutation causing a severe decrease in IR autophosphorylation.