hematurea

 ? Presence of at least 5RBC/Microliter of urine, micro(less than 100), macro(more than 100) ? Prevalence 0.5-2%among school children, screening urine analysis should be obtained of well child once at 5years old then once during 2nd decade of life. ? Haematuria either heme +ve (RBC, myoglobin) , heme-ve(just red discoloration) caused by ? 1-drugs(deferoxamine, ibubrufin, metranidazole, iron sorbitol, nitrfurantion, refampcin, salicylic acid ? 2- dyes (beets, food coloring, ? 3- hemogenstic acid, urate, porphyrin ?

 Common Causes OF Grosse Haematuria ? 1- UTI, 2- meatal stenosis, 3- perinatal irritation, 4- trauma, 5- stone, 6- caogulopathy, 7- tumor,8- glom ruler diseases(IgA nephropathy, post infectious GN, HSP, SLE)

 Evaluation ? 1- careful history,physical examination, GUA ? -level of Haematuria upper=brown in color, more than2++ protienurea, RBCcast, deformed RBC(glomerl, tubuler, interstitial), ? Lower=pc system, ureter, blader, urethra)(gross, clot, less protienurea, normal shape RBC) ? -tea color, edema, HT, hiegh blood urea suggest acute GN(PSGN) ? -Reccurent URI, GI system suggest acute GN, HUS ? -frequency , dysurea, fever suggest UTI ? -renal colick, renal stone ? -flank mass hydronephrosis, cystic disease, RVT ? -headache, visual disturbances, epistaxis, significant HT, suggest PSGN ? -family history of deafness Alport syndrome ?

-the child of persistent asymptomatic isolated microscopical Haematuria more than 2weeks need further evaluation.

 IgA nephropathy(Beurger disease)

 ? common chronic glo.disease ? -predominate IgA within messengeal deposits of glo with abscense of systemic disease like SLE, HSP ?

 Clinical feature ? Micro Haematuria, protienurea , nephritis, nephrotic, male more than female, Crosse ? Hematuria associated with URI, GIT disorder, mild to moderate HT, normal C3 level incontrast to PSGN, serum IgA IS NOT DIAGNOSTIC, ONLY 50% (increased) ?

 Prognosis and treatment ? NOT lead to significant renal damage, progressive disease in 20-30% ? Poor prognostic factors ? 1- persistent HT 2- decrease in renal function 3- heavy and persistent protienurea ? The proper management of HT is the aim, immune suppressive , steroid some benefit

 Alport syndrome(hereditary nephritis) ? Is genetically heterogeneous disease caused by mutation in gene coding for type 4 collagen which is the main component of basement membrane ? Genetic ? 85% sex linked (dominant, recessive), autosomal also, ?

 C/F ? Intermittent micro, recurrent macro, 1-2 days of URI, protienurea, in the 2nd decade nephritic syndrome, bilateral SN deafness 90% never congenital, ocular (30-40%)macular flecks, corneal erosion , anterior lenticonus(extrusion of central portion of the lens in to anterior chamber) ?

 DIAGNOSIS ? Carful family history , screening GUA, of 1st degree relative , audiogram, ophthalmologic examination, are critical in diagnosis, anterior lenticonus is pathognmonic ? Prognosis and treatment ? High risk of progression to end stage renal disease (heterozygous, recessive), nephrotic syndrome ?

 NOsp. Treatment

 Acute post sterptoccocal GN(PSGN) ?

 Sudden onset of Grosse hematuria, edema , HT, degree of renal insufficiency. Is the most common G-disease supper passed by IgA nephropathy. ?

 Etiology and Epidemiology ? Flow throat (serotype 12)in winter, and skin(serotype 49) in summer, infection by certain nephrogenic strain of GABH strept. ?

 Pathology ? Both kidneys enlarged symmetrically, on light microscope all glomeruli appears enlarged , diffuse messengeal cell proliferation with increase in matrix. ?

 Pathogenesis ? Precise mechanism is still undetermined, although low C3 level strongly suggestive of immune e mediated by activation of alternative pathway of complement rather than classical. ?

 C/F ? -most common age (5-15)years,and uncommon less than 3 years ? -1-2week after throat(strept.pharangitis) and 3-6weeks after skin infection(pyioderma) ? -the severity of renal range from asymptomatic hematuria with normal renal function to acute renal failure with hematuria ? -various degree of edema, HT, oligurea, encephalopathy or and heart failure owing to HT, or hypervolemia. ? - encephalopathy result from HT or toxic material of strept. ? -Edema result from salt and water retention or nephrotic(10-20%). ? Fever, malaise, lethargy , flank pain are common. ? - the acute phase resolved at 6-8 weeks, although protienurea and HT usually resolved by 4-6 weeks after onset, persistent microscopical hematuria may persist up to 1-2years. ?

 Diagnosis ? 1- GUA RBC, RBC cast, protienurea, WBC, ?

 2- CBP mild anemia(hemodilution, low grade hemolysis) 

? 3- RFT b.urea and s.cr either normal; or increased ?

 4- C3 level decreased markedly (return to normal at 6-8 weeks) ?

 5- clear evidence of invasive st.pharangitis, by +ve throat culture or Antistreptolysin test(ASO) commonly increased markedly after URI but not skin infection (normally 200 TOD) ?

 6- the best one after skin infection is Dexoyribonuclase B(DANase B) ?

 7- the streptozyme test include all (ASO, DANase B, streptokinase, hyalinrridase). ?

 The clinical diagnosis child with acute nephritis, low C3 level , evidence of recent strpt. Infection. ?

 DDX ? IgA , focal segemental GN, membroprolefrative, SLE, HSP, acute exacerbation of chronic GN. ?

 Indication of renal biopsy ? 1- in the presence of renal failure ? 2- in the presence of nephrotic syndrome ? 3- absence of evidence of recent strpt. Infection. ? 4- normal C3,C4 level ? 5- hematuria, decrease renal function, low C3 level persist more than 8 weeks after the onset. ?

 NOTE ? Acute GN, may flow other infection(coagalase –ve staph, st.pneumonia, G-ve, fungal, viral(influenza), ricketetial. ? Complication ? 1- mainly due to HT 60%, 10% encelopathy. ? 2- acute renal failure acidosis, hyperkalemia, hypocalcaemia, hyperphosphetemia, seizure. ?

 Prevention ? 1- Early institution of AB for st.pharangitis, skin infection, does not eliminate the risk of GN ? 2- family member of patient with PSGN should be cultured for GABH st. if +ve , give treatment ?

 Treatment ? 1-Proper management of HT, RF, ? 2-10 days course of treatment with penicilline to prevent the spread of infection and dose not affect the natural history of the disease. ? 3- salt restriction , diuretics, CA cannel blocker , ACE, are standard treatment of HT. ?

 Prognosis ? 95%complete recovery , mortality rate decreased by appropriate management of HT, HF, RF. ? Acute phase may pass to chronic renal disease ? Recurrence are extremely rare.

 Hemolytic uremic syndrome(HUS) 

? Most common cause of acute renal failure in young children, ch.ch by the following ? 1- microangiopathic hemolytic anemia ? 2- uremia ? 3- thrombocytopenia ? ?

 Aetiology ? 1- acute enteritis caused by Shiga- toxin producing E coli 0157:H7 precede the HUS in 80%of cases, usually transmitted by undercooked meat, unpastruiezed milk. ? 2- other infection(shigella, salmonella, strept.pnuemonia, campylobacter, viral agent(eacho, influenza, varicella, EPV, HIV) ? 3- Drugs(cyclosporine, pills ? SLE, malignant HT, radiation nephritis. ? 5-familiar ?

 Pathogenasis ? 1-Endothelial cell damage localized thrombus, ? 2- anemia mechanical damage of RBC, due to passing via altered vasculature ? 3- thrombocytopenia intrarenal platelets adhetion ? 4- HSM engulfed damaged RBC, platelets by liver and spleen ?

 C/F ? -common in young age group (less than 4 ) ? -preceded by GI(often bloody) most commonly and less by URI. ? -sudden onset of paler, irritability, weekness, lethargy, oligurea, in 5-10 days after GI, URI, ? - physical finding dehydration, edema, petechee, HSM, marked irritability. ?

DIAGNOSIS ?

 1- CBP HB=5-9 G/L, wbc up to 30000, platelet 20000-100000, retic increased, coombs test -ve, The blood peripheral smear reveals helmet cells, burr cells, and fragmented RBCs ?

 2- GUA low grade protienurea, micro.hematuria ?

 3- PT, PTT normal ?

4- RFT mild to moderate RF require dialysis ?

 DDX ? TTP ? SLE, malignant HT, RVT,(marked renal enlargement, absent of renal vien flow via Doppler US. ? Complication ?

 1- renal acidosis, hyperkalemia, hypocalcaemia, hyperphosphetemia, seizure ? 2- extrarenal GIT, intessuception, perforation ? CNS, infarction, cortical blindness ? HEART pericarditis, arrythemia.

 Prognosis and Treatment ?

 1- supportive care, meticulous attention to fluid and electrolyte, ?

 2- control of HT, aggressive nutritional support, ?

 3- early institution of dialysis(MR from 80% to 10%) ?

 4- AB should not be given in a patient with enteritis suppose to be due to Shiga- toxin producing E coli, (increase the risk of HUS) ?

 5- Plasma pharesis and FFP indicated in HUS without diarrheal prodrome and in CNS problems, familiar,TTP, DRUGS

 ? 6- HUS recover in about 90% and in 9% lead to end stage renal disease ?

 7- in patient passing acute phase , should flow because HT, decrease in renal function may be seen 20 years after. ? . ?