Acquired von Willebrand Syndrome in an Infant With Coarctation of the Aorta and Williams Syndrome.

An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.

Long-Term Study of Children With ROME III Functional Gastrointestinal Disorders Managed Symptomatically in a Biopsychosocial Model.

BACKGROUND: Our study evaluated progression of and identified potential factors contributing to outcomes of ROME III defined-functional gastrointestinal disorders (FGIDs) in children treated symptomatically in a biopsychosocial model of care with a long-term follow-up. METHODS: We performed a retrospective review of pediatric patients who were diagnosed with ROME III defined-FGIDs including functional abdominal pain, functional dyspepsia, irritable bowel syndrome and abdominal migraine. Patients were managed symptomatically in a biopsychosocial model of care from the time of initial diagnosis. Demographics, management, progression and response to treatment assessed as complete, partial, and no improvement were reviewed. RESULTS: Two hundred fifty-eight patients were included with mean age of 10.6 years, female 55.4%, mean number of encounters 3.3 visits, and mean follow-up was 18.7 months (range 2 - 59, SD 15.8). Diagnoses were functional abdominal pain 45%, irritable bowel syndrome 20.9%, multiple 13.2%, functional dyspepsia 12.8%, and abdominal migraine 8.1%. Investigations were performed in most patients: laboratory studies in 93.4% (non-contributory abnormal 23.6%), imaging studies in 45.3% (non-contributory abnormal 5%) and endoscopies in 43.0% (non-contributory abnormal 1.2%). Treatment included medication in 93.7%, and surgery in 1.9% (normal pathology). There were new functional gastrointestinal diagnosis in 11.6%, evolution of FGIDs, from one to another in 12.0%, and recurrence found in 35.7% of patients. There were 60.1% patients in the complete improvement group (CIG) and 39.1% in the partial/no improvement group (PIG/NIG). No statistical difference was found between CIG and PIG/NIG regarding demographics or evaluation. PIG/NIG had more encounters (mean 3.63 vs. 3.11; P = 0.03), had non-contributory lab abnormalities (34.4% vs. 20.0%; P = 0.01), needed more endoscopies (52.4% vs. 36.8%; P = 0.02), required more treatment changes (mean 1.41 vs. 0.81; P < 0.01) and developed new functional gastrointestinal diagnoses (19.4% vs. 6.5%; P < 0.01) with long-term follow-up. CONCLUSIONS: Patients with ROME III defined-FGIDs who experience partial or no improvement with treatment develop new FGID diagnosis, need more number of follow-up visits, require more number of endoscopies, need more treatment changes, and have more non-contributory laboratory abnormalities, compared to those who experience complete improvement. Symptomatic treatment offered in a biopsychosocial model of care is possibly beneficial in managing children with FGIDs.