Table of Contents
Patients, Families and Friends
Health Care Providers
|Specific Problems: Hematuria and Nephropathy|
|Health Care Providers - Problem Oriented Clinical Guidelines|
Kidney damage starts very early and progresses throughout life causing major complications in many individuals with sickle syndromes. Hyposthenuria, which starts in the first ten years of life, causes nocturia and enuresis, predisposes to dehydration, and reduces resistance to urosepsis. Hematuria and papillary necrosis are common. With advancing age, Proteinuria, renal tubular acidosis, nephrotic syndrome, glomerulosclerosis, and renal insufficiency occur.
by Antonio Guasch, MD
Edited by James Eckman, M.D. and Allan Platt, PA-C
Hematuria is a relatively common disorder in sickle cell anemia and may be a manifestation of the disease itself, or result from other problems such as urinary infections, renal papillary necrosis, renal stones, tumors or glomerulonephritis..
Present Illness. Note the onset, amount (gross vs microscopic), and associated symptoms (dysuria, fever, nausea, vomiting, back or flank pain, or other systemic symptoms). Previous episodes, treatment and precipitating factors. Obtain a detailed sexual history and, in females, determine the last menstrual period.
Past Medical History. Define previous hospitalizations, surgery, present medications, and drug allergies. Family history of stones, renal or bladder tumors.
Review of symptoms. Note change in weight and general ROS to sdetect systemic disease
- General. Degree of distress, weight change, hydration.
- Vital Signs. Temperature, Blood pressure, Pulse.
- Abdomen. Costovertebral angle tenderness, masses, palpable kidneys, or supra-pubic tenderness.
- Genitourinary. Vaginal bleeding, urethral irritation, bladder or pelvic masses, prostate enlargement and tenderness, or rectal masses.
- Minimum Evaluation. CBC with differential, reticulocyte and platelet counts. Urinalysis and culture. SMA 7, PT, PTT. Ferritin should be considered
- Additional Evaluation. Chest and Abdominal x-ray. Consider IVP, computerized tomography, renal ultrasound, or retrograde imaging studies.
First differentiate hematuria from hemoglobinuria by finding red cells on a fresh urine sample. Hemoglobinuria may be seen in hemolytic states or secondary to a delayed tranfusion reaction.
- Hematuria secondary to sickle cell syndrome. Painless, gross hematuria may occur in all sickle cell syndromes including sickle trait. Other causes must be excluded and the first episode of hematuria thoroughly investigated.
- Renal Papillary Necrosis. Occurs with increased frequency in sickle syndromes. May present with painless hematuria or with flank pain. A history of passing tissue is occasionally elicited.
- Urinary Tract Infection. Cystitis may cause dysuria, frequency, and hematuria. Infection is suggested by finding >5 WBCs/HPF and (+) leukoesterase or (+) nitrite on urinalysis is confirmed by culture.
- Renal Stone. May present as a renal colic (flank pain with radiation to groin), and associated symptoms (nausea, vomiting, and diaphoresis). The main differential diagnosis is with papillary necrosis and pyelonephritis.
- Glomerulonephritis. May present with hematuria, variable proteinuria, increased serum creatinine, hypertension and abnormal urinary sediment (RBC casts, WBCs, etc).
- Malignancy. Renal or bladder tumors may present with painless gross hematuria, with or without associated findings. As life expectancy increases in the sickle cell anemia population, urinary tract malignancies (and prostatic hypertrophy) are expected to become more common and should be ruled out. Patients with sickle genes may be predisposed to unusual renal tumors.
The decision to admit patients with gross hematuria depends on the severity of the problem and/or the findings of a previous evaluation. In general, first episodes should be admitted to identify the cause and to institute intravenous hydration. Associated findings like pain, fever or the development of renal insufficieny are indications for admission. Mild cases with a previous negative work-up may be managed as outpatient. Periodic reevaluation to rule out malignancies may be needed, especially in the elderly..
- Secondary to sickle cell anemia. This is a diagnosis of exclusion after other causes are eliminated. Treatment included vigorous hydration with hypotonic fluids, diuretics, urine alkalinization and bed rest. Epsilon aminocaproic acid may be administered at a dose of 3-8 grams/day in six divided doses after adequate diuresis has been initiated. Chronic relapsing hematuria can be treated with exchange transfusion, EACA, iron, and the avoidance of intense physical activities. Severe cases may need urological intervention, and, occasionally, selective embolization, but nephrectomy is not indicated since it would recur in the contralateral kidney.
- Renal Papillary Necrosis. Admit for evaluation and treatment. Retrograde pyelogram is most sensitive, but an IVP or ultrasound may provide the diagnosis. Treatment is with intravenous hydration and straining of urine to document the passage of tissue. A urine culture should always be obtained to rule out associated infection. Nephrotoxic medications (especially NSAIDs) should be avoided.
- Infection: Follow treatment schedules as outlined in UTI chapter.
- Renal Stone: Admit for pain control, intravenous hydration, and strain of urine to document stone passage and stone analysis. A kidney ultrasound or CT scan may revealed the stone/associated obstruction. Urology consultation is recommended for persistent pain, infection or obstruction.
- Glomerulonephritis. Nephrology consultation is necessary for further evaluation and treatment.
- Malignancy. Urology consultation and appropriate staging is necessary
The mainstem of prevention is to maintain an adequate hydration and urine output, specially those instances that seems to be induced by physical activity. Bed rest and adequate hydration at the onset of symptoms may prevent severe hematuria.
Patient and Parent Education
The etiology of the problem must be established and clear explained to the patient and family. The benign nature of sickle associated hematuria must be stressed because this is a very frighteningcomplication. The relapsing nature of the disorder must be made clear. Precipitating activities should be identified and avoided. Bed rest and vigorous hydration as initial therapy of relapsing hematuria is taught. The lack of benefit of nephrectomy should be told up front to prevent unnecessary and inappropriate surgery. Often the patient will have to be taught to accept the hematuria as a chronic or recurrent problem and adjust lifestyle with this in mind.
Buckalew VM Somern A. Renal manifestations of sickle cell disease. Arch. Intern. Med. 133:660-669, 1974.
Alleyne GAO, Statius Van Eps LW, Addae SK et al. The kidney in sickle cell anemia. Kidney Int. 7:371-379, 1975.
Pandya KK, Koshey M, Brown N, Presman D. Renal papillary necrosis in sickle cell hemoglobinopathies. J. Urol. 115:497-501, 1976.
Black WD, Hatch FE, Acchiardo S. Aminocaproic acid in prolonged hematuria of patients with sicklemia. Arch. Intern. Med. 136:678-681, 1976.
SP, Ramsey EJ, Knochel JP. The effect of bicarbonate and distilled water on sickle cell trait hematuria and in vitro studies on the interaction of osmolality and pH on erythrocyte sickling in sickle cell trait. J. Urol. 118:793-795, 1977.
Eknoyan G, Qunibi WY, Grissom RT, Tuma SN, Ayus JC. Renal papillary necrosis: An update. Medicine 61:55-73, 1982.
Barakat AY, Noubani H. The kidney in hematologic disease. A Review. Int. J. Pediatr. Nephrol. 67:46-49, 1986.
Allon M: Renal Abnormalities In Sickle Cell Disease. Arch Intern Med 150:501-504, 1990
Buckalew Vm, Jr., Someren A: Renal Manifestations Of Sickle Cell Disease. Arch Intern Med 133:660-669, 1974
Guasch A, Cua M, Mitch We: Extent And The Course Of Glomerular Injury In Patients With Sickle Cell Anemia. Kidney Int 49:786-791, 1996
Black Wd, Hatch Fe, Acchiardo S: Aminocaproic Acid In Prolonged Hematuria Of Patients With Sicklemia. Arch Intern Med 136:678-681, 1976
Zayas Cf, Platt J, Eckman Jr, Elsas L, Clark Ws, Mitch We, Guasch A: Prevalence And Predictors Of Glomerular Involvement In Sickle Cell Anemia. J Am Soc Nephrol 7:1401, 1996 (Abstract).
|Last Updated on Thursday, 14 May 2009 17:07|