RENAL Diseases

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RENAL Diseases

• Investigation of renal system
• Haematuria/ PSGN
• ARF/CRF
• Proteinuria/ Nephrotic syndrome
• UTI
• Congenital Anomalies

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HEMATURIA

• Hematuria is one of the most common urinary
  findings that bring children to the attention of
  the pediatric nephrologist.
• Generally, hematuria is defined as the presence
  of 5 or more red blood cells (RBCs) per
  high-power field in fresh centrifuged speciment.

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• Hematuria
• can be gross Macroscopic
• (ie, the urine is overtly bloody, smoky, or tea
  colored) or microscopic.
• It may be symptomatic or asymptomatic,
  transient or persistent, and either isolated or
  associated with proteinuria and other urinary
  abnormalities.

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• The role of the primary care physician in the
  management of a child with hematuria includes the
  following
• Recognize and confirm the finding of hematuria.
• Identify common etiologies.
• Select patients who have significant urinary
  system disease that might require further
  expertise in either diagnosis or management and
  referral.

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Causes of RED urine

• 1- Heme-negative (no RBC or Hb).
• 2- Heme-positive ( Hb no RBC).
• 3- True Hematuria ( RBC - Hb).

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Causes of Heme-positive ( no RBC but positive Hb)

• 1- Hemoglobinuria. ( As in Hemolytic Anemia)
• 2- Myoglobinuria (Resulting from muscle injury
  or secondary to viral infection, cruch injury, or
  DIC)

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Causes of Heme-negative (no RBC or Hb).

• 1- Drugs
• 2- Dyes
• 3- Metabolites.

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Red urine without RBCs
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Causes of True Hematuria

• 1- Glomerular hematuria
• Thin basement membrane disease (benign familial
  hematuria)
• Alport syndrome
• IgA nephropathy
• Hemolytic uremic syndrome
• Postinfectious glomerulonephritis
• Membranoproliferative glomerulonephritis
• Lupus nephritis
• Anaphylactoid purpura (Henoch-Schönlein purpura).

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• 2- Nonglomerular hematuria
• Fever
• Strenuous exercise
• Mechanical trauma (masturbation)
• Menstruation
• Foreign bodies
• Urinary tract infection
• Hypercalciuria/urolithiasis
• Sickle cell disease/trait
• Coagulopathy
• Tumors
• Drugs/toxins (NSAIDs, anticoagulants,
  cyclophosphamide, ritonavir, indinavir)
• Anatomic abnormalities (hydronephrosis,
  polycystic kidney disease, vascular
  malformations)
• Hyperuricosuria

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APPROACH TO HEMATURIA

• .
• HISTORY
• clots in urine ? Extraglomerular causes
• fever, abdominal pain, dysuria, frequency,
  and recent enuresis in older children (? UTI).
• recent trauma to the abdomen
  ?Hydronephrosis
• early-morning periorbital puffiness, weight
  gain, oliguria, the presence of dark-colored
  urine, and the presence of edema or hypertension
  (?Glomerular causes)
• Is painless (? Glomerular causes)
• Recent throat or skin infection (?
  Postinfection glomerulonephritis).
• Joint pains, skin rashes, and prolonged
  fever in adolescents (? Coollagen Vascular
  Disorders).
• Skin rashes and arthritis (? Henoch
  Schonelein Purpura or SLE).
• exercise, menstruation, recent bladder
  catheterization, intake of certain drugs or toxic
  substances, or passage of a calculus
• Family history (? Inherited or Familial
  Renal diseases).

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• 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

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• Studies performed in selected patients
• Dnase B titer or streptozyme lt 6 months duration
• Skin or throat culture
• ANA titer
• Urine analysis looking for cast
• Coagulation study/ platelet count
• Sickle cell screen
• Audiogram
• Renal biopsy
• Microscopic haematuria plus any of the following
• Diminished renal function
• Proteinuria
• Persistant microscopic haematuria (gt1 year)
• Second episode of gross haematuria
• Cystoscopy
• Pink o microscopic haematuria, dysuria and
  sterile urine culture

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• Glomerular brownish or cola-coloured and may
  contain RBCs cast, proteinuria
• Lower urinary tract red to pink color urine and
  may contain clots

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• 7 years old boy
• Frank haematuria (smokey or tea colored)
• H/O throat infection 2 weeks ago
• O/E peri-orbital edema, BP 140/90 (hypertension)
• What is the most likely diagnosis
• What investigations

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Post-streptococcal glomerulonephritis

• Rare before the age of 3 years
• Nephritic picture Gross haematuria, edema ,
  hypertension, renal insufficiency (normal RF-ARF)
• Complications of hypertension encephalopathy,
  congestive heart failure
• Rarely nephritic- nephrotic picture.

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Diagnosis

• Urine analysis RBCs, RBCs cast, proteinuria
• Low complement C3
• Evidence of streptococcal infection throat
  culture, ASO titer and DNase B antigen and
  streptozyme test
• Mild normochromic anaemia
• Renal function

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Complications

• ARF ? volume overload, hypertension, fits,
  hyperkalemia, hyperphosphatemia, hypocalcaemia
  and acidosis
• Treatment
• Antibiotics
• Management of ARF
• Treat Hypertension.

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Prognosis

• Complete recovery 95
• Infrequently severe acute phase leading to
  chronic renal insufficiency
• Recurrence are extremely rare.

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Case History

• 5 years old boy
• Generalized malaise, abdominal pain, joints pain,
  peri orbital oedoma, skin rash (as seen in the
  picture).
• He presented with Hematuria.
• What is the most likely diagnosis?

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Henoch-Schönlein Purpura or (Anaphylactoid
Purpura)

• Renal involvement occurs in 2550 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

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Recurrent Gross Haematuria or Persistent
Microscopic Haematuria

• IgA nephropathy (Berger)
• Alport syndrome
• Familial idiopathic haematuria
• Idiopathic hypercalciuria.

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IgA Nephropathy (Berger)

• Glomerulonephritis with IgA as the predominant
  immunoglobulin in mesangial deposits, in the
  absence of any systemic disease
• Haematuria minimal proteinuria
• Normal C3 usually normal RF
• Diagnosis renal biopsy

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IgA Nephropathy (Berger)

• Treatment supportive
• Prognosis mainly good, only 30 has progressive
  diseasehypertension, diminished renal function,
  or proteinuria exceeding 1 g/24 hr between
  episodes of gross hematuria

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ALPORT Syndrome.

• Hereditary nephritis. (85X-linked, ARAD).
• Haematuria proteinuria sensorineural hearing
  loss (minority) eye abnormalitie (10) Corneal
  erosione.
• 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.

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Idiopathic Familial Benign Haematuria

• No proteinuria
• All investigations normal
• Urine test of the parents and siblings
• An excellent prognosis, but long-term follow-up
  is required to exclude Alport syndrome

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Idiopathic Hypercalciuria

• This entity may be inherited as AD,and may
  present with recurrent gross hematuria or
  persistent microscopic hematuria, or dysuria in
  the absence of stone formation, or sandy urine.
• Hypercalciuria (without hypercalcemia)
• Diagnosis 24-hr urinary calcium excretion
  exceeding 4 mg/kg, urine calcium to creatinine
  ratio (mg/mg)gt0.2
• Hypercalciuria may lead to nephrolithiasis
• RX Oral thiazide decrease Ca intake.

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Membranous 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.
• Diagnosis Renal biopsy.

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MEMBRANOPROLIFERATIVE (MESANGIOCAPILLARY)
GLOMERULONEPHRITIS

• 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.

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RAPIDLY PROGRESSIVE (CRESCENTIC)
GLOMERULONEPHRITIS

• Nephritis with rapid progression to end-stage
  renal failure
• Causes poststreptococcal, lupus,
  membranoproliferative, 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

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Acute 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).

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Etiology

• Prerenal causes
• Hypovolemia, hypotension, hypoxia
  (dehydration,Hemorrhage,Sepsis,H.F)
• Renal causes
• Acute tubular necrosis
• Acute interstitial nephritis
• Glomerulonephritis
• Localized intravascular coagulation
• Tumors
• Developmental abnormalities
• Hereditary nephritis
• Postrenal causes
• Obstructive uropathy, stone, blood clot.

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CLINICAL MANIFESTATIONS OF ACUTE RENAL FAILURE

• 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

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Diagnosis

• Careful history
• Examination
• Investigation 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)
  (FENa (UNa x PCr/PNaxUCr) X100).

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Urine Analysis

• Renal
• osmolality less than 350 mOsm/kg mmol/l H2O
• Usually exceeds 40 mEq/l (mmol/l)
• Usually exceeds 1

• 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 Na x
  plasma Cr divided by the plasma Na x urine
  creatinine concentration X 100) is usually less
  than 1.

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Treatment

• Pre-renalHypovolemia 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
• gt 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

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Treatment

• 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.

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Prognosis

• 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.

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