MEDICATION
- Antibiotic recommendations for UTI are discussed in the relevant chapters.
- Steroids for rapidly progressing glomerulonephropathy should be discussed with a nephrologist.
FOLLOW-UP
DISPOSITION
Admission Criteria
- Acute renal failure:
- Azotemia/uremia/hyperkalemia
- Hemodynamic instability
- Hematuria with traumatic injuries
- Obstructing ureteral stones with evidence of systemic infection or renal failure
- Hypertensive emergency
- Oliguria/anuria
- Pregnant with preeclampsia, pyelonephritis, obstructing nephrolithiasis
- Intractable pain
- Intolerance of PO fluids and medications
Discharge Criteria
- Hemodynamically stable without life-threatening issues
- Infected ureteral stones without renal failure or obstruction
- Mild hematuria or proteinuria without renal failure
- Hematuria:
- Gross hematuria, except in young women with proven UTIs, needs urology follow-up.
- Microscopic hematuria needs repeated U/As and PCP follow-up, and may need urology/nephrology in the future.
- Pregnant patients with possible infections should have close follow-up to ensure treatment success.
- Proteinuria:
- Mild cases should be referred to their primary care physician for further workup in an outpatient setting.
- Nephrotic-range proteinuria, as well as proteinuria with renal failure and no alternative explanation, should be referred to a nephrologist promptly.
PEARLS AND PITFALLS
- Missing acute glomerulonephritis in children, either by misdiagnosing as a UTI based on the U/A, or by failing to consider it in the differential is a pitfall.
- Spot urine protein/creatinine ratio correlates well with 24 hr urine protein (i.e., ratio of 3.5 is roughly equivalent to 24 hr protein excretion of 3.5 g)
- Periorbital edema can be a sign of nephritic syndrome, not just allergic reaction.
- Failing to ensure follow-up for asymptomatic hematuria, especially in patients >40 yr is a pitfall.
ADDITIONAL READING
- McDonald MM, Swagerty D, Wetzel L. Assessment of microscopic hematuria in adults.
Am Fam Physician
. 2006;73(10):1748–1754.
- Naderi AS, Reilly RF. Primary care approach to proteinuria.
J Am Board Fam Med
. 2008;21(6):569–574.
- Rao PK, Jones JS. How to evaluate ‘dipstick hematuria’: What to do before you refer.
Cleve Clin J Med
. 2008;75:227–233.
- Wolfson AB, Hendey GW, Ling LJ, et al., eds.
Harwood-Nuss’ Clinical Practice of Emergency Medicine.
5th ed. Philadelphia, PA: J.B. Lippincott Publishers; 2009.
See Also (Topic, Algorithm, Electronic Media Element)
- Urinary Tract Infection, Adult
- Urinary Tract Infection, Pediatric
- Preeclampsia/Eclampsia
- Renal Failure
- Renal Calculus
CODES
ICD9
- 599.70 Hematuria, unspecified
- 791.0 Proteinuria
- 599.71 Gross hematuria
ICD10
- R31.9 Hematuria, unspecified
- R80.9 Proteinuria, unspecified
- R31.0 Gross hematuria
HEMOPHILIA
Steven H. Bowman
•
Christina H. Georgopoulos
BASICS
DESCRIPTION
- Caused by deficiency of functional factor VIII or factor IX
- Lack of functional factor causes partial inactivation of coagulation cascade and impaired hemostasis.
- 2 types:
- Hemophilia A: Factor VIII deficiency
- Hemophilia B (Christmas disease): Factor IX deficiency
- Symptoms dependent on factor activity:
- 5–30% factor activity (mild hemophilia):
- Bleeding with major trauma or surgery
- 1–5% factor activity (moderate hemophilia):
- Bleeding secondary to trauma/surgery
- Occasional spontaneous hemarthroses (<1 time per month)
- <1% factor activity (severe):
- Spontaneous bleeding from infancy
- May bleed as often as 1–2 times per week, often requiring factor replacement
- Complications:
- Death from hemorrhage
- Recurrent joint bleeding leads to joint destruction and loss of function
- Transfusion-transmitted infections (risk reduced with purification of concentrates)
- Development of inhibitors (IgG antibodies to factors), which prevent hemostasis
ETIOLOGY
Genetics
- X-linked recessive, though 1/3 have a spontaneous mutation
- Rare disease:
- Hemophilia A: 1 in 5--7,000 males
- Hemophilia B: 1 in 30,000 males
- Prevalence of inhibitors: 20% of severe hemophilia A and <5% of severe hemophilia B
DIAGNOSIS
SIGNS AND SYMPTOMS
- Bleeding:
- Hemarthrosis (most common):
- Knee (most common) > elbow > ankle > shoulder > wrist
- Muscle hemorrhage
- Bleeding from soft tissue lacerations
- Postextraction or oral mucosal bleeding
- Epistaxis (only in severe disease)
- Hematuria
- Intracranial hemorrhage
- GI bleeding
- Pseudotumors (blood cysts)
ESSENTIAL WORKUP
Thorough history and physical exam
DIAGNOSIS TESTS & NTERPRETATION
Lab
- PLT count: Normal
- Bleeding time: Normal
- PT: Normal
- PTT: Increased
- Urinalysis: Asymptomatic hematuria (often)
- Specific factor assays:
- Factor VIII:Ag (measures quantity): Decreased
- Factor VIII:c (measures activity): Decreased
- vWF:Ag and vWF: Normal
Imaging
Radiographic studies may be required in certain circumstances:
- Head CT to evaluate for intracranial bleed
- Renal US/cystoscopy to evaluate excessive hematuria or renal trauma
- Abdominal CT to evaluate for retroperitoneal bleeding
DIFFERENTIAL DIAGNOSIS
- Von Willebrand disease
- Anticoagulant drugs
- Antiplatelet agents
- Thrombocytopenia
- Hepatic dysfunction
TREATMENT
INITIAL STABILIZATION/THERAPY
- Control bleeding proximally.
- Establish IV access, draw type and screen.
- Consider PRBCs for transfusion.
ED TREATMENT/PROCEDURES
General
- Patients generally know their doses, type of factor, and whether or not they have inhibitors. Have low threshold for factor replacement when they present with any symptom.
- Coordinate ED care with patient’s hematologist.
- Start replacing factors immediately, even before imaging/consults.
Approach to Factor Replacement
- 1 U in 1 mL of normal plasma is considered 100% clotting factor activity.
- People without hemophilia have factor levels between 60% and 150%.
- Step 1: Determine% activity desired based on location/system involved in bleeding:
- Low to moderate bleeding – want 30–50% activity
- Soft tissue injury/lacerations
- Joint or muscle bleeding (except iliopsoas)
- Moderate to severe bleeding – want 50–100% activity
- GI or GU bleeding
- Major muscle bleeds (iliopsoas)
- Severe to life-threatening bleeding – want 100% activity
- CNS injury/intracranial bleed
- Major bleeding from trauma or postsurgery
- Intra-abdominal/retroperitoneal bleeding
- Throat/neck bleeds compromising airway
- Step 2: Calculate factor dose for level of activity desired.
- Factor VIII required (in U) = wt (kg) × 0.5 × (% factor activity desired). 1 IU/kg raises activity by 2%. Give every 12 hr for 1–2 days.
- Factor IX required (in U) = wt (kg) × 1 × (% factor activity desired). 1 IU/kg raises activity by 1%. Give every 24 hr for 1–2 days.
- Factor dose given IV push over 1–2 min