ABSTRACT
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is relatively uncommon and generally seen in the fourth decade of life. There are very few pediatric cases with the diagnosis of HUVS in the literature. In this report, we describe the first familial cases of HUVS in three siblings. The disease onset was during childhood period in all patients. One of them developed severe renal involvement and died. The other two had ongoing skin and eye manifestations and the elder one developed lupus. Presence of these three patients is a strong evidence for the role of genetic factors in the pathogenesis of this rare vasculitis.
Subject(s)
Urticaria/genetics , Vasculitis, Leukocytoclastic, Cutaneous/genetics , Child , Child, Preschool , Female , Humans , Siblings , Syndrome , Urticaria/complications , Vasculitis, Leukocytoclastic, Cutaneous/complicationsABSTRACT
Angiodysplasia is a frequent cause of gastrointestinal bleeding in adults with chronic renal failure (CRF); however, there is no data about this association in children. The mechanism of this association is not known. We report a 4.5-year-old boy with CRF presenting with hematochezia due to colonic angiodysplasia. He was on hemodialysis for the previous 9 months. Treatment with argon plasma coagulation (APC) was commenced following a short course of octreotide therapy. During the 3 years of follow-up, no occult or gross bleeding occurred. This case illustrates that octreotide and APC therapy seems to be useful for arresting bleeding from angiodysplasia and prevention of recurrent bleeding in children with CRF.
Subject(s)
Angiodysplasia/complications , Gastrointestinal Hemorrhage/etiology , Kidney Failure, Chronic/complications , Child, Preschool , Humans , MaleABSTRACT
The nutcracker syndrome, caused by compression of the left renal vein between the superior mesenteric artery and the aorta, usually manifests with hematuria, flank pain and proteinuria. We report on a 9-year-old boy who was previously diagnosed with urolithiasis but had significant proteinuria, not explained by this diagnosis. On further investigation by renal Doppler ultrasonography it was found that he had nutcracker syndrome. We would like to emphasize that this syndrome should be considered where there is no obvious cause of proteinuria and hematuria.
