Management of nephrotic syndrome; has four elements 1. establish the cause; there is important age – related causes e.g. in neonate congenital aetiologies are most common , in older children minimal changes nephropathy is the dominent cause of nephrotic syndrome .

In later life the most cause of nephroic syndrome is membranous nephropathy focal segmental glomerulosclerosis , diabetes mellitus and amyloidosis rarely cause nephrotic syndrome in childhood ( MOSTLY IN OLDER PATIENTS)

2. Treat the cause of nephrotic syndrome if possible ; In children with minimal change nephropathy initial treatment with cortico steroid ( 2 mg\kg) for few months and decrease the dose to the half till control of the symtoms with follow up

the symtoms with follow up In older patient and in children unresponsive to corticosteroid renal biopsy is necessary unless there is specific cause (i.e diabetes mellitus , hypertention )

3. treat the symyoms : edema is treated and chould be controlled with low- sodium diet ( no added satls) with diuretics , in severe cases of nephrotic syndrome large doses of combionation of diuretics acting on different parts of nephron may be required ( loop diuretic plus thiazide plus amiloride).

In occasional patient with evidence of hypovolemia intravenous salt free albumin infusion may be needed Over diuresis may lead to renal impairment through hypovolemia

4. prevent complication ; venous thrombosis can be prevented by anticoagulation , there is need for routine anticoagulation in all patients with chronic or servere nephrotic syndrome

Hypercholestremia is treated with lipid- lowering drugs The risk of infection with pneumococci can be prevented with immunization

Classification of glomerulonephritis It is confusing the original classification nephrotic and nephritic has given way to histological classification

  1:Minimal change nephropathy. It is the most cause of N.S in childhood and in one fourth of adult patients.nephrotic syndrome in those patients usually do not progress to renal impairment.

  2:focal segmental glomerulosclerosis Presented with idiopathic N.S shows poor response to corticosteroid and often progress to renal impairment and usually recure after renal transplantation.

  3:Membranous nephropathy: It is regarded as most common cause of N.S in adulthood A proportion of cases associated with known causes as a.persons with certain HLA and DR b.certain drugs and heavy metals c.H.B .V d. certain malignancy as lymphoma but most cases are idiopathic .Membranous glomerulonephritis showed the following course 1.third of the cases remit spontaneously 2.third of the cases remain in nephrotic state 3.third of the cases progress to renal failure. They found that short term with high dose of corticosteroid and alkylating agents may improve nephrotic state and long term prognosis.

  4.IgA nephropathy: is the most recognized type of glomerulonephritis and can be present in many ways ; Hematuria is almost universal Proteinuria is usual and may be severe proteinurea Hypertention is very common The disease is a common cause of E.S.R.D One type of IgA nephropathy is Henoch- shoenlion purpura; Her systemic vasculitis occur in response to upper respiratory tract infection .mostly occur in children and rare in adult. There is characteristic petechial rash, cutaneous vasculitis, abdominal pain ,with gastro-intestinal vasculitis with mild G.N presented as hematuria.

  5. Acute post-infective glomerulonephritis ; Mostly seen after streptococcus infection but can occur after other infection.mostly in children occur after 10 days of infection which indicate immune mechanism certain strains are accused Clinical features; Acute nephritis with varing severity occur

  1.Na and water retention with edema. 2. Hypertension 3.Decrease of G.F.R 4.proteinuria but rarely exceed 2g/24 hours 5.hematuria 6.decrease in urine volume( oliguria) and the urine become red or smoky 7. low serum C 3, C 4 8. evidence of streptococcus infection like increase A.S.O titer or culture of throat swab renal function begin to improve spontaneously After 10 -14 days Treatment; a.antibiotic b.restriction of Na and fluid c.use of diuretic and hypotensive drugs renal lesion is almost in all children and most adult resolve compeletly

  6.Glomerulonephritis associated with chronic diseases like a.G.N with malaria b.HB virus c.Visceral leishmaniasis in those conditions of G.N the most histological pattern are membranous nephropathy and membrano- proliferative focal –segmental glomerulosclerosis with HIV infection is highly prevalent.

Cystic diseases of the kidney   Polycystic kidney disease Type I ; Infantile polycystic kidney disease It is 1. rare 2. autosomal resessive 3.associated with hepatic fibrosis. 4.fatal in first year of life.

  Type II ; Adult poly cystic kidney disease It is 1. common 2.incidence 80 / 100,000 3.autosomal dominent

  There are two types of APKD according to gene affected 1.type one gene affected on chromosome 16 and this type is common 2.type two gene affected on chromosome 4 and this type is rare. Pathology; small cysts appear in proximal tubular epithelium Which are numerous in number and variable sizes.

  Clinical features ; 1.may be asympyomic till twenty year of age 2.hypertension usually after 20 year 3.patients may complain of vague abdominal discomfort 4.acute loin pain or renal colic 5.hematuria 6.urinary tract infection 7. may present with renal failure.

  Associated features with APKD 1.30% of cases have hepatic cysts but there are no disturbance in liver functions 2. berry aneurysm of cerebral vessels and 10% of cases may suffer from subarachnoid hemorrhage 3.mitral and aortic regurgitation is frequent 4.colonic diveriticulation may occur 5. we may see abdominal herniae.

  Investigations and diagnosis ; 1. Family history . 2.Clinical examination 3.Ultrasound study of abdomen. 4.Genetic defect detection Management; 1. good control of blood pressure 2. treatment of urinary tract infection 3.if chronic renal failure developed we start treatment of it Screening and genetic counselling

  Interstitial Nephritis Acute interstitial nephritis Refers to acute inflammation within the tubule-interstitium Aetiology; 1. drugs ; penicillins ,non-steroidal anti-inflammatory drugs Allopurinol, frusimide. 2.systemic diseases like sarcoidosis ,SjogrenS disease 3.infections ; like a.leptospirosis b.tuberculosis c.pyelonephritis d.cytomegalovirus

  Dx: 1-30% of pt. with drug induced AIN have generalize drug hypresene like fever ;rash ;and eosinophilia 2-70%shows eosinophilia in urine 3-hx. And examination and specific test may point to Dx. 4-renal Biopsy has definit Dx.

  Management :- 1-if ARF due to Rx is usually conservative and dialysis needed only in sever case . 2-in drug induced AIN withdrawal of drug is sufficient 3- cortico steroid are frequently used but there effect is doubtful .

  Chronic interstitial nephritis :- Is a hetergenous group of disease causing chronic inflammation with the tubules and interstitium . Classification of CIN:- Type of disease : 1-chronic glomerular disease -----in all type of glomerular nephritis avariable degree of IN is associated .

  2-immune –inflammatory disease ------sarcoidosis ;sjogrens syndrome;SLE ;transplantation rejection ;Amyloidosis . 3-Tumors -----Myloma 4-Drugs -----all drug causing AIN esp.NSAID 5-Metabolic or congenital ---wilsons dis. Hypokalemia ;hypercalciuria ;hyper oxaluria Sickle cell anemia 6- Toxins ---mushrom poison ;lead poison ;chinase herps ;Balkan nephropathy .

Nephropathy tubules