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pediatric

Alport Syndrome (Hereditary Nephritis)

ICD-10 Code
Q87.81

Genetic disease of collagen IV (mutations in COL4A3, COL4A4, or COL4A5) leading to abnormal glomerular basement membrane (GBM) structure. Characterized by hematuria, progressive renal failure, sensorineural hearing loss, and pathognomonic ocular findings (anterior lenticonus). Electron microscopy shows alternating thinning and splitting of the GBM.

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents for evaluation of persistent hematuria, noted on routine urinalysis. Family history is significant for hereditary nephritis, progressive renal insufficiency, and sensorineural hearing loss. Patient reports no current gross hematuria, dysuria, or flank pain. No history of hypertension or edema. Genetic testing confirms mutation in [COL4A3/COL4A4/COL4A5].

Clinical Examination Findings

Vitals: BP [value] mmHg (percentile for age/height). HEENT: Ocular exam reveals [normal/anterior lenticonus/macular flecks]. Audiometry demonstrates [normal/bilateral high-frequency sensorineural hearing loss]. Cardiovascular: S1/S2 regular, no murmurs. Abdomen: Soft, non-tender, no palpable masses or hepatosplenomegaly. Extremities: No peripheral edema. Skin: No rashes or lesions.

Treatment Protocol

Management plan: 1. Initiation of ACE inhibitor (e.g., Lisinopril) for renoprotection and reduction of proteinuria. 2. Strict blood pressure control targeting <50th percentile for age. 3. Biannual monitoring of serum creatinine, BUN, and urine protein-to-creatinine ratio. 4. Annual ophthalmology and audiology screening. 5. Referral to nephrology and genetic counseling.

Detailed clinical guide coming soon.