What is the diagnosis for a 9-year-old boy presenting with hematuria (presence of blood in urine), impaired renal function (elevated serum creatinine), and hypertension (elevated blood pressure), with a history of sensorineural hearing loss and a family history of renal transplant complications?

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Differential Diagnosis for a 9-year-old boy with dark brown urine

  • Single most likely diagnosis
    • Alport syndrome: This condition is characterized by sensorineural hearing loss, hematuria, and a family history of renal disease, which aligns with the patient's symptoms and family history. The worsening hearing loss over the past 4 years and the maternal uncle's death from complications of a renal transplant also support this diagnosis.
  • Other Likely diagnoses
    • IgA nephropathy: Although the patient does not have the typical symptoms of IgA nephropathy, such as abdominal pain or fever, it is still a possible cause of hematuria, especially given the recent upper respiratory tract infection.
    • Post-streptococcal glomerulonephritis (PSGN): The patient's recent sore throat and improving symptoms could suggest a post-infectious glomerulonephritis, although the lack of fever, dysuria, or urinary frequency makes this less likely.
  • Do Not Miss diagnoses
    • Goodpasture syndrome: This autoimmune disease can cause rapidly progressive glomerulonephritis and pulmonary hemorrhage, which would be catastrophic if missed. Although the patient does not have the typical symptoms of Goodpasture syndrome, such as cough or shortness of breath, it is essential to consider this diagnosis due to its potential severity.
    • Vasculitis (e.g., ANCA-associated vasculitis): Vasculitis can cause renal disease and hearing loss, and it is crucial to rule out this diagnosis due to its potential for severe complications.
  • Rare diagnoses
    • Thin basement membrane nephropathy: This condition can cause hematuria and is often associated with a family history of renal disease. However, it is less likely than Alport syndrome given the patient's sensorineural hearing loss.
    • Fabry disease: This rare genetic disorder can cause renal disease, hearing loss, and other systemic symptoms. Although it is unlikely, it should be considered in the differential diagnosis due to the patient's combination of symptoms.

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This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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