ABSTRACT
Therapeutic plasma exchange (PE) or plasma-pheresis has been used in recent years in the treatment of severe hemolytic uremic syndrome (HUS) in children. We analyzed the benefit of PE and peritoneal dialysis (PD) in 9 children, 6 boys and 3 girls, aged 1-10 years, from 1983-1993. All children came from different geographical regions, and all had the sporadic form of the illness. Three patients had the gastrointestinal form, 5 had respiratory prodromes while 1 child developed HUS during the course of varicella. Seven children were hypertensive, but only in 3 was hypertension persistent. The child with varicella had a transient complement decrease. Five children were treated with PE. In 4 children, fresh frozen plasma (FFP) was used as replacement fluid, and human albumin was used in 1 child. Four children were treated with PD and infusions of FFP. Rapid recovery of renal function was observed in 5 patients whereas in 2 oliguric children the recovery of renal function ensued within 1 and 2 months, respectively. Two children developed terminal renal failure (TRF) (in 1 child the treatment was very delayed, and in other child HUS developed following varicella). Only 1 boy had relapses of the disease followed by impairment of renal function from which he gradually recovered. During the 3-10 year follow-up period, only the child with relapses was hypertensive while the others had normal clinical and laboratory parameters. We suggest that PE plays an important role in the early treatment of severe forms of HUS in children.
Subject(s)
Hemolytic-Uremic Syndrome/therapy , Plasmapheresis , Case-Control Studies , Child , Child, Preschool , Diuresis/physiology , Female , Humans , Hypertension/physiopathology , Hypertension/therapy , Infant , Longitudinal Studies , Male , Peritoneal Dialysis , Plasma , Renal Insufficiency/therapy , Retrospective Studies , Serum Albumin/metabolism , Treatment OutcomeABSTRACT
A case of an extremely rare type of cloacal exstrophy in a male infant with a normally developed subvesical part of the urinary system and external genitalia but absent distal colon segment is presented. The patient also had omphalocele, upper urinary tract anomalies and sacrococcygeal teratoma.
Subject(s)
Abnormalities, Multiple , Cloaca/abnormalities , Abnormalities, Multiple/surgery , Cloaca/surgery , Hernia, Umbilical/surgery , Humans , Infant, Newborn , MaleABSTRACT
The authors present 16 patients with the idiopathic forms of hemorrhagic disease in newborns, none of which, after birth, having prophylactically received K-vitamin. Seven of them had an early form, four a classical form, and five a late form of hemorrhagic disease. All of them were born at term, four were female and 12 male newborns. The diagnosis was established on the basis of the decreased prothrombin time (PV) and the activated partial thromboplastin time (APTV), as well as their normalization following the applied therapy. The PV values in these patients ranged from 0.009 to 0.65 and the APTV from 33 to 120. The authors point out the significance of the prophylaxis of hemorrhagic diseases in the newborn and the still inconclusive state of scientific dilemmas and clinical practice in this connection.
