- Pathogenesis of Acute Post streptococcal Glomerulonephritis (APSGN).

Pathogenesis of Acute Post streptococcal Glomerulonephritis (APSGN).

Evidence of immune mechanism: 

1. Latent period between strept infection and onset of the disease
2. ↓Serum C3
3. Detection of immune reactants in glomeruli by immunologic techniques 

By Ibrahim samaha

- Causes of Hematuria in Children.

 Causes of Hematuria in Children

UPPER URINARY TRACT DISEASE
Isolated renal disease

• Immunoglobulin (Ig) A nephropathy (Berger disease)
• Alport syndrome (hereditary nephritis)
• Thin glomerular basement membrane nephropathy
• Postinfectious GN (poststreptococcal GN)*
• Membranous nephropathy
• Membranoproliferative GN*
• Rapidly progressive GN
• Focal segmental glomerulosclerosis
• Anti–glomerular basement membrane disease

Multisystem disease

• Systemic lupus erythematosus nephritis*
• Henoch-Schönlein purpura nephritis
• Granulomatosis with polyangiitis (formerly Wegener granulomatosis)
• Polyarteritis nodosa
• Goodpasture syndrome
• Hemolytic-uremic syndrome
• Sickle cell glomerulopathy
• HIV nephropathy

- Urine analysis Differentiation between Golmeular and Non-Glomerular hematuria

 Urine analysis Differentiation between

 Golmeular and Non-Glomerular hematuria

	Golmeular	Non-Glomerular
Colour	brown, smoky, cola-colored, or tea-colored as a result of the hematin formation from hemoglobin in the acidic environment.	bright red or pink
RBCs morphology	Dysmorphic > 80% Acanthocytes > 5%	Normal
proteinuria	>100 mg/dL via dipstick,	minimal proteinuria on dipstick (<100 mg/dL).
Clots	Absent	May be Present
RBCs cast	May be present due to squeezing of RBCs and sticking together and entangled in the protein matrix	Absent
Pain	Painless	Painful

- Cystinosis (liginac Syndrome).

Cystinosis (liginac Syndrome)

A/E:  defect in the metabolism of cysteine that results in accumulation of cystine crystals in most of the major organs of the body, notably the kidney, liver, eye, and brain.

Incidence:

-       It occurs at an incidence of 1 : 100,000 to 1 : 200,000.

-       Is the most common cause of Fanconi syndrome in children.

-       In certain populations, such as French Canadians, the incidence is much higher.

clinical patterns:

1.    Infantile or nephropathic cystinosis: the most severe form present in the 1st 2 yr of life with severe tubular dysfunction and growth failure. If the disease is not treated, the children develop end-stage renal disease by the end of their 1^st decade.

2.    A milder form of the disease manifests in adolescents and is characterized by less-severe tubular abnormalities and a slower progression to renal failure.

3.    A benign adult form with no renal involvement also exists.

-  Patients with nephropathic cystinosis present with clinical manifestations reflecting their pronounced tubular dysfunction and Fanconi syndrome…?

-  Patients are typically fair skinned and blond because of diminished pigmentation.

-  Ocular presentations include photophobia, retinopathy, and impaired visual acuity.

-  Patients also can develop hypothyroidism, HSM, and delayed sexual maturation.

-  With progressive tubulointerstitial fibrosis, renal insufficiency is invariant.

- Lowe Syndrome.

Lowe Syndrome

Lowe syndrome (oculocerebrorenal syndrome of Lowe) is a rare X-linked disorder characterized by:

-       congenital cataracts , glaucoma and blindness often develop.

-       mental retardation, hypotonia and hyporeflexia,

-       The renal abnormalities are tubular and include: hypophosphatemic rickets with ↓ serum phosphorus levels, low to normal serum calcium Levels,↑ serum alkaline phosphatase levels,pRTA, and aminoaciduria.

-  Significant proteinuria is common.

-  The disease is caused by mutations in the OCRL1 gene, which codes for a Golgi aparatus phosphatase.

-  There is no specific therapy for the renal disease or neurologic deficits.

-  Renal treatment may include alkali therapy, phosphate replacement, and vitamin D support.

-  Cataract removal is generally required.

-  Genetic counselling.

Death occur during childhood.