Hematuria

Presence of at least 5RBC/Microliter of urine, micro(less than 100), macro(more than 100)

Prevalence 0.5-2%among school children

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 Hematuria

1- UTI, 2- meatal stenosis 3- perinatal irritation 4- trauma 5- stone 6- coagulopathy 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

Acute post sterptoccocal GN(PSGN)

Sudden onset of Grosse hematuria(30-50%), edema(85%) , HT, degree of renal insufficiency(usaualy mild). 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(less than 5%).

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) Magnetic resonance imaging of the brain is indicated in patients with severe neurologic symptoms and can demonstrate reversible posterior leukoencephalopathy in the parieto-occipital areas on T2-weighted images. Chest x-ray is indicated in those with signs of heart failure or respiratory distress, or physical exam findings of a heart gallop, decreased breath sounds, rales, or hypoxemia.

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 Hemolytic-uremic syndrome (HUS) is one of the most common causes of community-acquired acute kidney failure in young children. It is characterized by the triad of 1- microangiopathic hemolytic anemia 2- thrombocytopenia 3- renal insufficiency

CLINICAL DESCRIPTION Hemolytic uremic syndrome (HUS) is characterized by the acute onset of microangiopathic hemolytic anemia, renal injury, and a low platelet count. Thrombotic thrombocytopenic purpura (TTP) also is characterized by these features but can include central nervous system (CNS) involvement and fever and can have a more gradual onset. Most cases of HUS (but few cases of TTP) occur after an acute gastrointestinal illness (usually diarrheal).

DEFINITION OF POSTDIARRHEAL

HEMOLYTIC UREMIC SYNDROME DISEASE

LABORATORY CRITERIA FOR DIAGNOSIS The following are both present at some time during the illness: • Anemia (acute onset) with microangiopathic changes (i.e., schistocytes, burr cells, or helmet cells) on peripheral blood smear and • Renal injury (acute onset) evidenced by hematuria, proteinuria, or elevated creatinine level: ≥1.0 mg/dL in a child <13 yr or ≥1.5 mg/dL in a patient 13 yr or older or ≥50% increase over baseline. Note: A low platelet count can usually, but not always, be detected early in the illness, but it can then become normal or even high. If a platelet count obtained within 7 days after onset of the acute gastrointestinal illness is not <150,000/mm3, other diagnoses should be considered.

CASE CLASSIFICATION Probable • An acute illness diagnosed as HUS or TTP that meets the laboratory criteria in a patient who does not have a clear history of acute or bloody diarrhea in the preceding 3 wk, or • An acute illness diagnosed as HUS or TTP that (a) has onset within 3 wk after onset of an acute or bloody diarrhea and (b) meets the laboratory criteria except that microangiopathic changes are not confirmed. Confirmed • An acute illness diagnosed as HUS or TTP that both meets the laboratory criteria and began within 3 wk after onset of an episode of acute or bloody diarrhea

Etiology The various etiologies of HUS allow classification into 1-infection-induced 2- genetic 3-medication- induced, and 4-HUS associated with systemic diseases characterized by microvascular injury The most common form of HUS is caused by toxin-producing Escherichia coli that cause prodromal acute enteritis and is commonly termed diarrhea-associated HUS. In the subcontinent of Asia and southern Africa, the shiga toxin of Shigella dysenteriae type 1 is causative, whereas in Western countries verotoxin- producing E. coli (VTEC) are the usual causes. Several serotypes of E. coli can produce verotoxin, and the O157:H7 type is most common in Europe and the Americas. The reservoir of VTEC is the intestinal tract of domestic animals, usually cows. Disease is usually transmitted by undercooked meat or unpasteurized milk or apple cider. Epidemics have followed ingestion of undercooked, contaminated hamburger at fast food restaurant

s

Pathogenesis

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 -.

Clinical Manifestations HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. Persons with this condition may urinate less than normal. Urine output may almost stop.

.anemia Red blood cell destruction leads to symptoms of Early symptoms: Blood in the stools Irritability Fever Lethargy

diarrhea and Vomiting Weakness Later symptoms: Bruising Decreased consciousness Low urine output No urine output Pallor

rare -- Seizures )petechiaeSkin rash that looks like fine red spots (

Yellow skin (jaundice) Liver or spleen swelling Nervous system changes

The majority of patients with HUS have some central nervous system (CNS) involvement. Most have mild manifestations, with significant irritability, lethargy, and nonspecific encephalopathic features. Severe CNS involvement occurs in ≤20% of cases. Seizures and significant encephalopathy are the most common manifestations in those with severe CNS involvement and result from focal ischemia secondary to microvascular CNS thrombosis.

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 The acute prognosis, with careful supportive care, 1-For diarrhea-associated HU has <5% mortality in most major medical centers. 2-Half of the patients require dialysis support during the acute phase of the disease. 3-Most recover renal function completely, but of surviving patients, 5% remain dependent on dialysis, 4-And up to 20-30% are left with some level of chronic renal insufficiency. 5-The prognosis for HUS not associated with diarrhea is more severe. Pneumococci-associated HUS causes increased patient morbidity, with mortality reported as 20%. 6-The familial, genetic forms of HUS can be insidiously progressive or relapsing diseases and have a poor prognosis The primary approach that has substantially improved acute outcome in HUS is early recognition of the disease, monitoring for potential complications, and meticulous supportive care

Supportive care includes careful management of fluid and electrolytes including correction of volume deficit , control of hypertension , and early institution of dialysis if the patient becomes anuric or significantly oliguric . Red cell transfusions are usually required because hemolysis can be brisk and recurrent until the active phase of the disease has resolved . In pneumococci-associated HUS, it is recommended that any administered red cells be washed before transfusion to remove residual plasma

Anticoagulation, antiplatelet, and fibrinolytic therapy is specifically contraindicated because they increase the risk of serious hemorrhage. Antibiotic therapy to clear the toxigenic organisms can result in increased toxin release, potentially exacerbating the disease, and therefore is not recommended. Prompt treatment of any underlying pneumococcal infection is importan

Plasma infusion or plasmapheresis has been proposed for 1-patients suffering severe manifestations of HUS, 2- primarily serious CNS involvement. 3-other genetic forms of HUS, such as undefined familial (recessive or dominant) 4-or sporadic but recurrent HUS.