Glomerular diseases typical case reports morphology Doc. MUDr. Zdeňka Vernerová, CSc., MUDr. Martin Havrda

Glomerular disorders.

Case 1

Boy, 4 years, complaints: Pharyngitis or impetigo 2 weeks ago Hematuria Oliguria Edema Headache Nauzea, vomiting Weakness

Boy, 4 years, physical examination: Edema Hypertension Pale appearance (pulmonary edema)

Boy, 4 years, DIAGNOSIS? ACUTE GLOMERULONEPHRITIS.

Hematuria

Boy, 4 years, confirmation of the diagnosis: Glomerular hematuria, red blood cell casts Proteinuria Renal failure ↓ C3 ↑ ASLO Renal biopsy – endocapillary GN.

Boy, 4 years, follow-up: Supportive and symptomatic treatment. Dialysis if necessary. Risk of death in the acute phase: –Renal failure –Brain edema –Pulmonary edema –Infection Spontaneous recovery common. Hypertension or residual urinary abnormality common.

Case 2

Man, 21 years, complaints: In case of respiratory infections, fever is often accompanied by macroscopic hematuria (dark coloured urine). With cessation of fever urine becomes normal. Laboratory tests reveal protein and blood in the urine. No other complaints.

Man, 21 years, physical examination: Mild hypertension (140/90mmHg). Otherwise normal.

Man, 21 years, DIAGNOSIS? IgA NEPHROPATHY.

IgA

Man, 21 years, confirmation of the diagnosis: Glomerular hematuria, red blood cell casts Proteinuria common Loss of GFR in some cases Maybe ↑ IgA in serum Renal biopsy – commonly mesangioproliferative GN.

Man, 21 years, follow-up: Treatment depends on morphological and clinical presentation. –Mild cases – observation only –Hypertension, if present, must always be treated aggresively!!! –Severe cases – steroids, sometimes with cytotoxic drugs (cyklofosfamide) Variable prognosis, disease progression in years –Blood pressure level essential for prognosis

Case 3

Girl, 7 years, complaints: Edema of the face, legs, all body.

Girl, 7 years, physical examination : Massive whole body edema.

Girl, 7 years, DIAGNOSIS? MINIMAL CHANGE DISEASE

Girl, 7 years, confirmation of the diagnosis: Proteinuria > 3,5 g/24h per 1,73 m² Hypoalbuminemia Hypercholesterolemia Renal biopsy – diffuse flattening of the foot processes of podocytes

Girl, 7 years, follow-up: Treatment with steroids. Supportive and symptomatic treatment. Resolution common with steroid treatment. Relapses are quite common. Good prognosis related to renal function. In some cases, long-term steroid treatment or use of cytotoxic and immunosupressive drugs may be necessary (cyclosporin, cyklophosfamide).

Case 4

Man, 18 years, complaints: Massive whole body edema.

Man, 18 years, physical examination: Gross generalised edema. Hypertension may be present.

Man, 18 years, DIAGNOSIS? Focal and segmental glomerulosclerosis (primary).

Man, 18 years, confirmation of the diagnosis? Nephrotic syndrome. Loss of GFR in some cases. Renal biopsy.

Man, 18 years, follow-up: Treatment rarely effective (steroids, cytotoxic drugs). Poor prognosis. –Severe nephrotic syndrome with complications. –Progressive loss of GFR. –Renal failure in several years common. Risk of disease relapse after transplantation.

Case 5

Female, 50 years, complaints: Edema of the legs.

Female, 50 years, physical examination: Edema. Hypertension in some cases.

Female, 65 years, DIAGNOSIS? Membranous nephropathy (idiopathic).

Female, 65 years, confirmation of the diagnosis? Nephrotic syndrome. Microscopic glomerular hematuria common. Renal biopsy. 20% secondary...?

Female, 50 years, follow-up: Treatment with steroids and cytotoxic drugs (cyklofosfamide, chlorambucil). Supportive and symptomatic treatment. Remission common with treatment. Risk of relapse. Renal failure uncommon.