Paediatric Nephrology (I)Paediatric Nephrology (I) HEAMTOURIA & RENAL FAILUREHEAMTOURIA & RENAL FAILURE

Jameela Kari, FRCP (UK), CABP, MD, CCST, FRCPCH

King AdulAziz University Hospital, Jeddah, Saudi Arabia

RENAL SYSTEMRENAL SYSTEM

Investigation of renal system

Haematuria/ PSGN ARF/CRF Proteinuria/ Nephrotic

syndrome UTI Congenital Anomalies

Investigation of the renal systemInvestigation of the renal system

Urine analysis Urine electrolytes: fraction excretion of sodium

(pre-renal <1%, renal >1-2%) 24 hours urine for protein, Ca etc Cultures: older children: midstream

Younger children:SPA, Catheter, Bag Urea & creatinine GFR (creatinine clearance= ml/1.73m2/minute)

Imaging: US, KUB, MCUG, CT scan, isotope scan (DMSA, DTPA), IVP

Renal biopsy

HAEMATURIAHAEMATURIA

Macroscopic = frank = gross Microscopic > 5 RBCs per high powered field Red urine:

– Blood (RBCs= haematuria, haemoglobin= haemoglobinuria)– Myoglobin– Food: blackberries– Aniline dyes used for coloring candy– Drugs: Rifampicin, phenolphthalein– Urate– Porphyrins

HaematuriaHaematuria

CausesCauses

Glomerular: brownish or cola-coloured and may contain RBCs cast, proteinuria

Lower urinary tract: red to pink color urine and may contain clots

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

Haematological: thrombocytopaenia, DIC, coagulopathies, renal vein thrombosis, SCD

Glomerular: brownish or cola-coloured and may contain RBCs cast, proteinuria

Stones and hypercalciuria Infection: bacterial, TB, viral Lower urinary tract: red to pink color urine and may

contain clots Anatomic abnormalities: congenital, trauma, polycystic

kidneys, tumors, vascular abnormalities Exercise and drugs

Glomerular DiseasesGlomerular Diseases

7 years old boyFrank haematuria (smokey or tea colored)H/O throat infection 2 weeks agoO/E peri-orbital edema, BP 140/90

(hypertension)– What is the most likely diagnosis– What investigations

Post-streptococcal glomerulonephritisPost-streptococcal glomerulonephritis::

Rare before the age of 3 yearsNephritic picture: Gross haematuria,

edema , hypertension, renal insufficiency (normal RF-ARF)

Complications of hypertension: encephalopathy, congestive heart failure

Rarely: nephritic- nephrotic picture.

Diagnosis:Diagnosis:

Urine analysis: RBCs, RBCs cast, proteinuria

Low complement C3Evidence of streptococcal infection: throat

culture, ASO titer and DNase B antigen and streptozyme test

Mild normochromic anaemia Renal function

Complications:Complications:ARF volume overload, hypertension, fits, hyperkalemia, hyperphosphatemia, hypocalcaemia and acidosis

Treatment• Antibiotics• Management of ARF

PrognosisPrognosis

Complete recovery 95%Infrequently: severe acute phase leading to

chronic renal insufficiency Recurrence are extremely rare

What is this?

RBCs castGlomerulonephriti

s or vasculitisExclude extra-

renal disorders

Glomerular DiseasesGlomerular Diseases

MPGN Rapidly progressive glomerulonephritits SLE Shunt nephritis Goodpasture disease Membranous Anaphlactoid purpura IgA nephropathy Idiopathic (benign familial) Alport syndrome

EVALUATION OF A CHILD EVALUATION OF A CHILD WITH HAEMATURIAWITH HAEMATURIA History Examination Investigation:

– Studies performed in all patients: urine microscopy and culture CBC serum creatinine serum C3 level US kidneys urine protein = urine albumin/creatinine ratio calcium = urine calcium/creatinine ratio

– Studies performed in selected patients: Dnase B titer or streptozyme < 6 months duration Skin or throat culture ANA titer Urine analysis looking for cast Coagulation study/ platelet count Sickle cell screen in black patients Audiogram

– Renal biopsy Microscopic haematuria plus any of the following:

– Diminished renal function– Proteinuria

Persistant microscopic haematuria (>1 year) Second episode of gross haematuria

– Cystoscopy Pink o microscopic haematuria, dysuria and sterile urine culture

Case History:Case History:

5 years old boyGeneralized malaise, abdominal pain, joints

pain, peri orbital oedoma

Henoch-Schönlein Purpura or Henoch-Schönlein Purpura or (Anaphylactoid Purpura)(Anaphylactoid Purpura)

Renal involvement occurs in 25–50% of children during the acute phase

Haematuria with or without casts or proteinuria during the first few weeks of illness

The nephrotic syndrome, moderate azotemia, hypertension, oliguria, and hypertensive encephalopathy may occasionally occur.

Most children with renal involvement recover

Recurrent Gross Haematuria or Recurrent Gross Haematuria or Persistent Microscopic HaematuriaPersistent Microscopic Haematuria

IgA nephropathy (Berger)Alport syndromeFamilial idiopathic haematuriaIdiopathic hypercalciuria.

IgA Nephropathy (Berger)IgA Nephropathy (Berger)

Glomerulonephritis with IgA as the predominant immunoglobulin in mesangial deposits, in the absence of any systemic disease

Haematuria + minimal proteinuriaNormal C3 + usually normal RFDiagnosis: renal biopsy

IgA Nephropathy (Berger)IgA Nephropathy (Berger)

Treatment: supportivePrognosis: mainly good, only 30% has

progressive disease:hypertension, diminished renal function, or proteinuria exceeding 1 g/24 hr between episodes of gross hematuria

ALPORT SyndromeALPORT Syndrome..

Hereditary nephritis. Haematuria + proeinuria + sensorineural hearing

loss (minority) + eye abnormalitie (10%). Diagnosis: renal biopsy. Males with Alport syndrome commonly develop

end-stage renal failure in the 2nd or 3rd decade of life, occasionally in association with hearing loss. Females usually have a normal life span and only subclinical hearing loss.

Idiopathic Familial Benign Idiopathic Familial Benign HaematuriaHaematuriaNo proteinuriaAll investigations normalUrine test of the parents and siblingsAn excellent prognosis, but long-term

follow-up is required to exclude Alport syndrome

Idiopathic HypercalciuriaIdiopathic Hypercalciuria

RGH, persistent microscopic hematuria, or dysuria in the absence of stone formation

Hypercalciuria (without hypercalcemia)Diagnosis: 24-hr urinary calcium excretion

exceeding 4 mg/kg, urine calcium to creatinine ratio (mg/mg)

Hypercalciuria may lead to nephrolithiasisRX: Oral thiazide

Membranous GlomerulopathyMembranous Glomerulopathy

Uncommon in childhood and a rare cause of haematuria.

The most common cause of nephrotic syndrome in adults.

Associated with systemic lupus erythematosus, cancer, gold or penicillamine therapy, and syphilis and hepatitis B virus infections.

MEMBRANOPROLIFERATIVE MEMBRANOPROLIFERATIVE (MESANGIOCAPILLARY) (MESANGIOCAPILLARY) GLOMERULONEPHRITISGLOMERULONEPHRITIS Chronic glomerulonephritis that frequently leads

to glomerular destruction and end-stage renal failure.

Most common in the second decade of life. Presentation: nephrotic syndrome, gross hematuria

or asymptomatic microscopic hematuria, proteinuria and hypertension . renal function may be normal to depressed. Low C3 complement level.

Diagnosis by renal biopsy.

RAPIDLY PROGRESSIVE RAPIDLY PROGRESSIVE (CRESCENTIC) (CRESCENTIC) GLOMERULONEPHRITISGLOMERULONEPHRITIS Nephritis with rapid progression to end-stage renal

failure Causes: poststreptococcal, lupus,

membranoproliferative, and the glomerulonephritides of Goodpasture disease, anaphylactoid purpura, and other forms of vasculitis

Acute renal failure, often after an acute nephritic or nephrotic episode

Diagnosis: Renal biopsy Paediatric Nephrology Emergency

Acute Renal FailureAcute Renal Failure

Develops when renal function is diminished to the point at which body fluid homeostasis can no longer be maintained.

Oliguria (daily urine volume less than 400 ml/m2) is common, the urine volume may approximate normal.

Nonoliguric renal failure: in certain types of acute renal failure (aminoglycoside nephrotoxicity).

EtiologyEtiology

Prerenal causes– Hypovolemia, hypotension, hypoxia

Renal causes– Acute tubular necrosis– Acute interstitial nephritis– Glomerulonephritis– Localized intravascular coagulation– Tumors – Developmental abnormalities– Hereditary nephritis

Postrenal causes– Obstructive uropathy, stone, blood clot

CLINICAL MANIFESTATIONSCLINICAL MANIFESTATIONS

Diminished urine output Oedema (salt and water overload) Hypertension, vomiting, and lethargy (uremic

encephalopathy). Complications of acute renal failure: volume overload

with congestive heart failure and pulmonary edema, arrhythmias, gastrointestinal bleeding due to stress ulcers or gastritis, seizures, coma, and behavioral change– Life threatening: GIT bleed, pericarditis and encephalopathy

DiagnosisDiagnosis

Careful historyExaminationInvestigation: CBC, urea and electrolytes,

PO4, Ca, blood gases, C3, US kidneys, urine electrolytes ( Na and creatinine), fractional excretion of Na (less than 1% in hypovalaemia)

Urine AnalysisUrine Analysis

Prerenal. Urine osmolality exceeds 500

mOsm/kg [mmol/l] H2O. Sodium content is usually less

than 20 mEq/l (mmol/l). The fractional excretion of

sodium (urine/plasma sodium concentration divided by the urine/plasma creatinine concentration X 100) is usually less than 1%.

Renal osmolality less than 350

mOsm/kg [mmol/l] H2O Usually exceeds 40 mEq/l

(mmol/l) Usually exceeds 1%

TreatmentTreatment

Pre-renal=Hypovolemia: volume replacement may be critical

Renal:– Fluid restriction: input= output + 400 ml/m2/24 hr

(insensible losses)– Hyperkalemia: no potassium-containing fluid, foods, or

medications until adequate renal function is re-established > 7 mEq/L (mmol/L): Nebulised salbutamul, IV Calcium

gluconate, sodium bicarbonate, ca resonium, glucose and insulin

– Moderate acidosis is common in renal failure: Na bicarbonate

TreatmentTreatment Hypocalcemia and hyperphosphataemia: Ca

binders (Ca carbonate). Hypertension:

– The primary disease process (nifedipine, diazoxide, sodium nitroprusside or labetalol as a continuous intravenous infusion is indicated for hypertensive crises).

– Expansion of the extracellular fluid volume (salt and water restriction is critical).

Indications for dialysis: fluid overload, and congestive heart failure, electrolyte abnormalities (especially hyperkalemia), central nervous system disturbances, hypertension.

PrognosisPrognosis

In general, recovery of function is likely following renal failure resulting from prerenal causes, the hemolytic-uremic syndrome, acute tubular necrosis, acute interstitial nephritis, or uric acid nephropathy.

On the other hand, recovery of renal function is unusual when renal failure results from most types of rapidly progressive glomerulonephritis, bilateral renal vein thrombosis, or bilateral cortical necrosis.

Chronic Renal FailureChronic Renal Failure

In children under 5 yr of age is commonly the result of anatomic abnormalities (hypoplasia, dysplasia, obstruction, malformations)

After 5 yr of age acquired glomerular diseases (glomerulonephritis, hemolytic-uremic syndrome) or hereditary disorders (Alport syndrome, cystic disease) predominate

UT malformation + Glomerulonephritis + Pyelonephritis….. > 50% of causes

Clinical ManifestationsClinical Manifestations

Nonspecific symptoms (headache, fatigue, lethargy, anorexia, vomiting, polydipsia, polyuria, growth failure).

Physical examination: pallor and weakness, hypertension, growth retardation and signs of renal osteodystrophy.

InvestigationsInvestigations

– CBC: anaemia.– Electrolytes: hyponatremia, hyperkalemia,

acidosis.– BUN and creatinine (nitrogen accumulation and

level of renal function).– hypocalcemia, hyperphosphatemia,

osteodystrophy.– High of intact parathyroid hormone levels.

MANAGAMENTMANAGAMENTdepends upon the degree of renal insufficiency (CRD)depends upon the degree of renal insufficiency (CRD)

STAGE of CKD GFR ML/MINUTE/1.73M2 FEATURES

1 : chronic changes with normal GFR

90-120 None

2 :Impaired RF (mild) 60-89 None

3 : Moderate 30-59 None, short stature, PTH

4 : Severe

(pre-terminal)

15-29 Acidosis, anaemia, BP, lethargy, etc

5 :ESRF < 15% Dialysis or RX

ManagementManagement

Diet in chronic renal failure. Water and electrolyte management in chronic

renal failure (fluid, K, Na). Acidosis in chronic renal failure. Renal Osteodystrophy. Anemia in chronic renal failure. Hypertension in chronic renal failure. Drug dosage in chronic renal failure.

End-stage Renal FailureEnd-stage Renal Failure

Dialysis is generally initiated when the patient's GFR < 15 ml/1.73m2/minute– Continuous ambulatory peritoneal dialysis

(CAPD)– Continuous cyclic peritoneal dialysis (CCPD)– Haemodialysis– Haemofiltration

Renal transplantation

PDPD

Peritoneal cavity- has a semipermeable membrane that surrounds intestine and other organs in abdominal cavity.

PDPD

CAPD: dialysate flows in, dwells, then drain; this process is repeated 4-5 times

While it dwells, osmosis and diffusion occurs

No machine used Fill volume: up to

50mls/kg = 1100ml/m2

Peritoneal Dialysis=CAPDPeritoneal Dialysis=CAPD

APD=CCPDAPD=CCPD

HaemodialysisHaemodialysis

HaemodialysisHaemodialysis

HaemodialysisHaemodialysis

HaemodialysisHaemodialysis

السليب اقصانا اسر فك اللهمنبيينا ومسري األولي قبلتنا وطهر

الحبيب ... نصرك اللهم القريب نصرك اللهم

القريب