Updated: 10/15/2018

Sickle Cell Nephropathy

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Snapshot
  • An 18-year-old African American man with a history of sickle cell trait presents with 1 week of painless gross hematuria. On review of systems, he endorses a 10 pound weight loss within the past 2 months. Physical exam reveals a left-sided abdominal mass. His serum creatinine is found to be elevated. He is sent to the emergency room for urgent CT imaging. (Renal medullary carcinoma secondary to sickle cell trait).
Introduction
  • Clinical definition
    • nephropathy associated with sickle cell disease or trait
      • vaso-occlusion
      • renal medullary carcinoma
  • Epidemiology
    • incidence
      • proteinuria
        • 20-25% in sickle cell disease
      • renal insufficiency
        • 5-30% in sickle cell disease
    • risk factors
      • vaso-occlusion
        • sickle cell disease > trait
      • renal medullary carcinoma
        • sickle cell trait > disease
        • patients of African ancestry
  • Pathogenesis
    • normal ↓ O2 tension in the renal medulla is low enough to induce RBC sickling
    • sickling of red blood cells in medulla capillaries cause increased viscosity
    • increased viscosity causes ischemia and infarction in renal medulla
    • this can lead to renal infarcts and papillary necrosis
  • Prognosis
    • prognostic variable
      • negative
        • renal failure
    • increased mortality risk for those with renal failure, regardless of treatment
    • prognosis is < 1 year for renal medullary carcinoma
      • commonly very aggressive and metastasizes early
Presentation
  • Symptoms
    • vaso-occlusion
      • nausea
      • vomiting
      • nocturia
      • polyuria
      • painless hematuria
      • history of urinary tract infections
    • renal medullary carcinoma
      • gross painless hematuria
      • weight loss
  • Physical exam
    • vaso-occlusion
      • flank or abdominal pain
      • hypertension
        • renin-mediated
    • renal medullary carcinoma
      • abdominal mass
Imaging
  • Ultrasound
    • indications
      • to exclude other abnormalities such as nephrolithiasis
    • recommend views
      • renal
    • findings
      • can detect renal papillary necrosis
  • CT
    • indications
      • if renal medullary carcinoma is suspected (young patient with sickle cell trait and gross hematuria)
    • findings
      • mass in kidney
Studies
  • Labs
    • electrolyte abnormalities
      • impaired distal H+ and K+ secretion can cause a renal tubular acidosis
        • hyperkalemia
        • metabolic acidosis
    • ↑ creatinine
  • Urinalysis
    • proteinuria
    • low specific gravity and osmolarity
      • indicates loss of urine concentrating ability
  • Hemoglobin electrophoresis
    • presence of HbS 
  • Diagnostic criteria
    • generally, this is a diagnosis of exclusion
Differential
  • Nephrolithiasis
    • seen on imaging
Treatment
  • Conservative
    • hydration and blood transfusions as needed
      • indications
        • isolated hematuria
        • hydration can dislodge blood clots
  • Medical
    • dialysis
      • indications
        • end-stage renal disease
  • Operative
    • renal transplant
      • indications
        • end-stage renal disease not responsive to dialysis
Complications
  • End-stage renal disease
  • Renal medullary carcinoma with metastases
 

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Questions (1)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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