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Look at how the capillaries are thickened

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Minimal change disease & primary FSGS present with fulminant (acute) nephrotic syndrome, while membranous nephropathy and secondary FSGS present with a more insidious and chronic version of nephrotic syndrome.

Those guys have a really interesting collection of gods 🍻

Secondary FSGS has an important clinical significance because a variety of renal diseases progress to ESRD through the form of secondary FSGS.

Diabetic nephropathy is a clinical syndrome characterized by the following:

• Persistent albuminuria (>300 mg/d or >200 μg/min) that is confirmed on at least 2 occasions 3-6 months apart.

• Progressive decline in the glomerular filtration rate (GFR).

• Elevated arterial blood pressure

A 2-years old boy presents to pediatric clinic without having been seen by a pediatrician since 6 months of age. His parents are concerned about his propensity for biting himself and strange writhing body movements. On physical examination, the patient is quite spastic in his movements with marked hyperreflexia in all limbs. His fingers are notably disfigured from his constant selfbiting. When questioned further, the mother admits that the child produces reddish-orange urine in collect urine from the child. Suspecting marked hyperuricemia and uric crystals on analysis.
The doctor started the child on allopurinol (for excess uric acid production), and suggested that the parents see a dentist about removing the child newly forming front teeth.

It’s a classical picture of lesch nyhan syndrome
It’s basically x linked recessive disorder, it’s a kind of spectrum of disorders and the cause is HGPRT (Hypoxanthine-guanine phosphoribosyltransferase) deficiency.
Which means more purine degradation and so more uric acid production.
Well, actually there are 2 pathways for purine synthesis, one of them is salvage pathway which is the affected one because it depends on HGPRT enzyme, and some organs depend only on this pathway for synthesis of purine, these organs include: brain, bone marrow and leukocytes.
So the main manifestations of this syndrome are related to these organs.

A child who eating himself >>

- So according as the uric acid is increasing this leads to Gout and nephrolithiasis.
- 90-100% of patients get megaloblastic anemia ( because as we know purine is essential for DNA synthesis).
- and there are a lot of manifestations in the brain starting from the childhood, self mutilation behavior which is characteristic feature, dystonic movements and others.