What is Hereditary Nephritis?

Hereditary Nephritis
Secondary FSGS. (A) An image showing a biopsy with interstitial fibrosis, tubular atrophy, and a segmentally sclerosed glomerulus (H& E 100×). (B) PAS stain showing extensive tubular atrophy (PAS 100×). (C) PAS stain showing two glomeruli, one larger than the other with the smaller one showing an area of sclerosis associated with epithelial cell proliferation in that area (PAS 200×). (D) A silver stain showing segmental sclerosis in the perihilar region (Jones 200×). (E, F) Electron micrographs showing two images of the basement membranes with variable diameters showing prominent splitting of lamina densa with a basketweave appearance characteristic of hereditary nephritis, proven by collagen studies. Note also the microvillous transformation of foot processes and effacement in this patient with nephrotic range proteinuria (11,500×). Pathology of Podocytopathies Causing Nephrotic Syndrome in Children: Ranganathan S - Frontiers in pediatrics (2016). Not altered. CC.

Hereditary Nephritis is also known as Alport syndrome. A group of diseases caused by gene mutation

What is the Pathology of Hereditary Nephritis?

The pathology of hereditary nephritis is:

-Etiology: The cause of hereditary nephritis is genetic factors.

-Genes involved: COL4A1-A6 genes.

-Pathogenesis: The sequence of events that lead to hereditary nephritis is poorly understood but maybe due to the mutation of genes.

-Morphology: Not applicable. 

-Histology: The histology associated with hereditary nephritis shows thin GBM with thickening.

How does Hereditary Nephritis Present? 

Patients with hereditary nephritis typically are males present at the first years of life. The symptoms, features, and clinical findings associated with hereditary nephritis include hematuria, renal dysfunction, proteinuria, deafness,edema, and vision changes.

How is Hereditary Nephritis Diagnosed? 

Hereditary nephritis is diagnosed through history taking, physical examination, urinalysis, renal biopsy, hearing and vision tests, genetic testing.

How is Hereditary Nephritis Treated? 

Hereditary nephritis is treated mainly by managing the symptoms, diuretics, limiting sodium intake, and managing high blood pressure.

What is the Prognosis of Hereditary Nephritis?

The prognosis of hereditary nephritis is poor.

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Last updated on May 24, 2022