Urea resolves gross hematuria in a 15 year old with hemoglobin C trait

Hematuria is a rare complication seen in patients with hemoglobin C trait. We report a 15-year-old African-American female with hemoglobin C trait, who presented with persistent hematuria. None of the urological, serological or histological workups revealed any other pathology. Hematuria failed to respond to all conventional modalities used in the treatment of the same condition seen in sickling hemoglobinopathies. This case is the first known case of persistent hematuria in a pediatric patient with hemoglobin C trait, which resolved with intravenous urea administration. Received: 27 June 2000 / Revised: 18 October 2000 / Accepted: 19 October 2000     

Recurrent hematuria in 4 white patients with sickle cell trait

Extensive investigations failed to disclose the etiology of recurrent gross hematuria in 4 white patients of Algerian descent. Hemoglobin electrophoresis revealed sickle cell trait in all cases. The hematuria ceased after bed rest and hydration in 3 patients, and following partial nephrectomy after visualization of the bleeding site at operative nephroscopy in 1. We recommend that hemoglobin electrophoresis be considered when evaluating every patient, black or white, presenting with unexplained hematuria.     

Medical management of refractory hematuria in sickle-cell trait

Hematuria secondary to sickle-cell trait has on occasion proved refractory to medical management. We have adopted a new six-drug regimen to deal with this difficult problem. Herein is reported our experience with this protocol in 2 patients. Our results have been excellent, with hematuria clearing within twenty-four hours. The drug regimen is used in an attempt to alter the conditions that are favorable for sickling in the renal medulla.     

Gross hematuria associated with sickle cell trait and von Willebrand's disease.

A case of recurrent gross hematuria, sickle cell trait and von Willebrand's disease is reported. The gross hematuria abated promptly after the institution of cryoprecipitate therapy. The importance of considering von Willebrand's disease in the differential diagnosis in patients with sickle cell trait and hematuria is discussed.     

Two cases of severe sepsis successfully treated with activated protein C

A 64-year-old man and a 52-year-old woman in shock with multiple organ failure and worsening of sepsis related organ failure assessment SOFA scores in the early days of care were treated with recombinant human activated protein C (rhAPC). In the woman sepsis was associated with reversible heart failure, with decreased ejection fraction, biventricular dilatation, and a sharp increase of troponin I, observations that have been linked to a higher rate of multiorgan failure and higher mortality. The man began to improve after 24 hours and the woman after 48 hours of rhAPC treatment, with both continuing to improve after withdrawal of treatment. Severe sepsis remains a therapeutic challenge. Among the many treatments based on the physiopathology of the disease, so far only rhAPC seems to improve outcome and reduce mortality.     

Sickle cell trait: forgotten cause of hematuria in white patients.

Extensive evaluations for intermittent gross hematuria, including selective renal arteriograms, failed to reveal the etiology of bleeding in 2 whtie patients. Sickle cell preparation and hemoglobin electrophoresis, obtained as long shot tests, revealed sickle cell trait in both patients. Both patients were treated successfully with low doses of epsilon aminocaproic acid. Sickle cell prepration and hemoglobin electrophoresis should be included in the evaluation of every patient, white or black, with unexplained hematuria.     

C1q nephropathy in a child presenting with recurrent gross hematuria

C1q nephropathy is a rare glomerular disease characterized by mesangial immune deposits with dominant or codominant staining for C1q. The exact pathogenesis leading to the mesangial immune deposits of C1q remains unknown. C1q nephropathy often presents with proteinuria in the nephrotic range, with an unpredictable or poor response to corticosteroid therapy. It is seen more commonly in older children and young adults and is more common in African Americans compared with Caucasians. We present a 4-year-old African American girl who presented with recurrent gross hematuria in the absence of proteinuria or hypertension and whose renal biopsy demonstrated dominant mesangial deposits of C1q. We conclude that C1q nephropathy should be considered in patients who present with recurrent gross hematuria.     

Vascular C3 deposits on renal biopsy in pediatric patients with hematuria

Isolated deposition of the third component of complement (C3) in the renal arterioles has been noted on biopsy specimens in patients with hematuria. This entity is of little known significance and reports of this finding in pediatric patients with hematuria are rare. We reviewed the clinical histories and biopsies of 17 children with hematuria and vascular C3 deposition on biopsy at Texas Children’s Hospital over an 14-year period. The mean age of presentation was 10.8 (range 4.5–16.6) years with a male preponderance (71%). Family history for hematuria was positive in 6 of 17 patients (35%). Light microscopy was normal or showed only minor abnormalities. Immunofluorescence was negative for IgA and IgG in all patients. Seven patients (41.1%) were noted to have diffuse or focally thin basement membranes on electron microscopy in addition to positive C3 immunofluorescence. The mean follow-up time was 23.8 months, during which 2 of 17 patients (12%) developed worsening proteinuria. The etiopathogenesis of this condition remains unclear, but an underlying immunological process cannot be ruled out. It is possible that this condition represents a stage of an acute glomerulonephritis. Clinical follow-up of these patients is warranted, as the long-term prognosis remains unclear. Received: 30 April 1999 / Revised: 3 December 1999 / Accepted: 7 December 1999