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PREPARED BY: MA’AM JENNIFER GAYTANO

PART II. RENAL DISEASES TUBULAR DISEASES
Acute Tubular Necrosis
CLASSIFIED into 4 TYPES: ➔ Destruction of RENAL TUBULAR Epithelial Cells
Based on MORPHOLOGIC COMPONENT initially ➔ 2 Distinct Types: Ischemic & Toxic ATN
AFFECTED ISCHEMIC Acute Tubular Necrosis
Glomerular ➔ Most often are IMMUNE- ➔ Follows a HYPOTENSIVE event that result in
MEDIATED decrease perfusion of the kidneys followed by
Tubular ➔ Result from INFECTIOUS or renal tissue ischemia
TOXIC SUBSTANCES ➔ 3 principal causes:
Interstitial
❑ Sepsis
Vascular ➔ Causes a renal perfusion that
❑ Shock
subsequently induces both
❑ Trauma
MORPHOLOGIC &
FUNCTIONAL changes in the TOXIC Acute Tubular Necrosis
kidney ➔ Results from exposure to NEPHROTOXIC
AGENTS
➔ Caused by variety of agents separated into @
GLOMERULAR DISEASES CATEGORIES:
❑ Endogenous nephrotoxin
Nephrotic Syndrome ❑ Exogenous nephrotoxin
➔ INCREASED permeability of the GLOMERULI to
the passage of plasma proteins (ALBUMIN)
ENDOGENOUS NEPHROTOXIN
➔ Heavy PROTEINURIA (3.5g/day) ➔ Normal solutes or substances that become toxic
➔ HYPOPROTEINEMIA (Plasma albumin usually when their concentration in the bloodstream is
<3g/dL → LIVER SYNTHESIS unable to excessive:
compensate for the large amount of protein a) hemoglobin – hemoglobinuria – severe
EXCRETED in the urine. hemolytic events
b) myoglobin – myoglobinuria –
➔ HYPERLIPIDEMIA - (INCREASED plasma levels
rhabdomyolyis
of TRIGLYCERIDES, CHOLESTEROL,
c) uric acid
PHOSPHOLIPIDS & VLDL)
d) immunoglobulin light chain
EXOGENOUS NEPHROTOXIN:
TYPES OF GLOMERULONEPHRITIS ➔ Substances ingested or absorbed
a) therapeutic agents
Acute Glomerulonephritis
b) anesthetics
➔ One cause: Post-Streptococcal Infection → c) radiographic contrast media
known as: acute poststreptococcal d) chemotherapeutic drugs
glomerulonephritis (Group A β-hemolytic e) recreational drugs
Streptococci – those with M PROTEIN in their Cell f) industrial chemicals
wall induces this type of nephritis)
➔ Blood: ELEVATED ASO Titer
➔ NON-AGN: non-streptococcal agent
➔ (Bacteria: pneumococci, viruses: mumps, HEPA
TUBULAR DYSFUNCTION
B, parasitic infection: malaria) ➔ Single defect in proximal tubular dysfunction
Renal ➔ Impaired ability to
Focal Segmental Glomerulonephritis REABSORBED GLUCOSE
➔ SCLEROSIS of the GLOMERULI Glucosuria
➔ FOCAL (Occurring in some glomeruli); Cystinuria ➔ Impaired ability to
➔ SEGMENTAL (Affecting a specific area of the (Cystine and REABSORBED SPECIFIC
➔ AMINO ACIDS
glomerulus) Dibasic AA)
➔ Predominant Feature: PROTEINURIA Hartnup
Membranoproliferative Glomerulonephritis
Disease
➔ Cellular Proliferation of the MESANGIUM along
with LEUKOCYTE INFILTRATION & (Monoamino-
THICKENING OF THE GLOMERULAR Monocarboxylic
BASEMENT MEMBRANE AA)
IgA Nephropathy Bartter’s ➔ Impaired ability to
➔ Most prevalent type of glomerulonephritis world Syndrome REABSORB SODIUM
wide ➔ Impaired
Renal Tubular ability to
➔ Deposition of IgA in the GLOMERULAR REABSORB
MESANGIUM Acidosis Type II
BICARBONATE
Chronic Glomerulonephritis ➔ Impaired
Idiopathic ability to
➔ Development: Slow and Silent REABSORB CALCIUM
➔ 80%: have previously some forms of Hypercalciuria
glomerulonephritis Hypocalciuric ➔ Excessive
➔ 20%: form of glomerulonephritis that has been Familial REABSORPTION of
CALCIUM
unrecognized Hypercalcemia

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PREPARED BY: MA’AM JENNIFER GAYTANO
Gordon’s ➔ Excessive Acute Interstitial Nephritis:
Syndrome REABSORPTION of ➔ Nephritis: allergic response to the interstitium of
SODIUM the kidney
Pseudohypo ➔ EXCESSIVE ➔ Most common cause: Acute Allograft Rejection of
parathyroidism REABSORPTION of a transplanted kidney
PHOSPHATE Yeast Infections:
Fanconi ➔ Generalized LOSS OF ➔ Candida species (e.g., Candida
Syndrome PROXIMAL TUBULAR ➔ albicans) – normal flora of GIT & vagina
FUNCTION ➔ Proliferation of yeasts due to: changes in pH,
➔ NOT REABSORBED form adversely disrupted by antibiotics
the ULTRAFILTRATE &
➔ EXCRETED in the URINE:
(AA, Glucose, Water,
VASCULAR DISEASE
Phosphorous, Potassium, &
Acute ➔ Clinically sudden:
Calcium)
Renal a) DECREASE of GFR
b) Azotemia
Failure
c) Oliguria (Urine Output <400Ml)
DISTAL TUBULAR DYSFUNCTION
Mechanism:
Familial ➔ Impaired ability to 1. pre-Renal – result from
Hypophosphatemia REABSORB DECREASE renal blood flow
PHOSPHATE
(Vitamin D (25% of cases) [URINE
Resistant Rickets) SODIUM
Idiopathic ➔ Impaired ability to CONCENTRATION IS LOW
REABSORB CALCIUM – INCREASED AMOUNT OF
Hypercalciuria SODIUM BEING
Renal Tubular ➔ Impaired ability to REABSORBED]
Acidosis, Types I ACIDIFY URINE 2. Renal – (Approx. 65% of
and IV cases) renal damage, can
Renal Salt-Losing ➔ Impaired ability to result from glomerular,
RETAIN SODIUM tubular or vascular disease
Disorder process [INCRASED
Nephrogenic ➔ Impaired ability to URINARY EXCRETION OF
Diabetes CONCENTRATE SODIUM]
URINE 3. Post-Renal – (Approx. 10%
Liddle’s Syndrome ➔ Excessive reabsorption of cases) obstruction in the
of SODIUM urine flow
Renal ➔ Inability to REABSORB ➔ Progressive LOSS of RENAL
Chronic
Phosphaturia INORGANIC FUNCTION caused by:
➔ PHOSPHATES Renal
IRREVERSIBLE & INTRINSIC
Failure
RENAL DISEASE
➔ Decreasing GFR slowly but
TUBULOINTERSTITIAL DISEASE & URINARY continuously DECREASES
TRACT INFECTIONS ➔ “END-STAGE RENAL
Urinary Tract Infections DISEASE” / “END-STAGE
LOWER UTI ➔ KIDNEYS” – CRF that
➔ Urethra (Urethritis) progresses to advanced renal
➔ Bladder (Cystitis) disease
➔ Painful Urination (Dysuria) Calculi ➔ “Stones” – renal calyces, pelvis,
➔ Burning Sensation bladder, ureter
➔ Frequent urge to urinate Calcium 75%
UPPER UTI ❑ With oxalate 35%
➔ Renal Pelvis Alone (Pyelitis) ❑ With phosphate 15%
➔ Renal Pelvis including Interstitium ❑ With others 25%
(Pyelonephritis) Magnesium ammonium 15%
phosphate
Acute Pyelonephritis
Uric acid 6%
➔ Bacterial infection that involves the renal tubules,
Cystine 2%
interstitium, & renal pelvis
➔ Mechanism:
rinary tract to the kidney
oodstream in the kidneys (hematogenous infection) PART III. METABOLIC DISEASES
Chronic Pyelonephritis
➔ Develops when permanent inflammation of renal QUALITATIVE TEST: SCREENING OF
tissue causes permanent scarring that involves METABOLIC DISORDERS
the renal calyces and pelvis

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PREPARED BY: MA’AM JENNIFER GAYTANO
Ferric Chloride ➔ Alkaptonuria Enzymes
Test (Homogentesic Acid) Responsible for
➔ MSUD Galactosemia:
➔ Melanoma (Melanin) 3.
➔ PKU 4. 1.Galactose 1-
Ammoniacal ➔ Alkaptonuria phosphate
Silver Nitrite (Homogentesic Acid) uridylyltransferase
➔ Alkaptonuria (GALT)
Benedict’s Test 5. 2.Galactokinase
(Homogentesic Acid)
(GALK)
Nitrosonaphthol ➔ Tyrosinuria
6. 3.Uridine
Test diphosphate
Hoesch Test ➔ Porphyria (Porphobilinogen) galactose-4-
Watson- ➔ Porphyria (Porphobilinogen) epimerase
Schartz 7. (GALE)
Secondary ➔ Induced by: SEVERE
Test
Aminoaciduria LIVER DISEASE or
GENERALIZED TUBULAR
DYSFUNCTION (e.g.,
AMINO ACID DISORDERS Fanconi Syndrome)
Liver & Kidney ➔ Actively involved in the Cystinosis ➔ MOI: autosomal recessive
metabolism of Amino Acids ➔ Lysosomal storage disease
➔ TRANSAMINATION: → results in →
Interconvertion of AA DEPOSITION OF CYSTINE
➔ DEAMINATION: in the LYSOSOMES of cells
Degradation of AA throughout the body
➔ AA → results in formation of (Kidneys, Eyes, Bone
AMMONIUM IONS → this AI Marrow & Spleen)
is used to form → UREA 3 DISTINCT TYPES
(subsequently eliminated ➔ Most common & SEVERE
Nephropathic
from the body by the FORM
KIDNEYS) Cystinosis
➔ Accumulated CYSTINE
Aminoaciduria ➔ Overflow Aminoaciduria: CRYSTALLIZES within the
➔ Cause: INCREASE in the PROXIMAL TUBULAR
plasma levels of AA CELLS of the NEPHRONS
➔ Renal Threshold for AA → causing →
reabsorption: EXCEEDED GENERALIZED TUBULAR
→ additional AA are DYSFUNCTION →
EXCRETED in the URINE development of →
➔ No-Threshold FANCONI SYNDROME
Aminoaciduria: ➔ Evident: FIRST YEAR OF
➔ AA not reabsorbed by the LIFE
tubules ➔ RARE FORM
Intermediate
➔ INCREASE in blood = ➔ Clinical Features: SAME
INCREASE in urine Cystinosis
with Nephropathic
➔ Renal Aminoaciduria: Cystinosis
➔ Plasma levels of AA: ➔ Evident: ADOLESCENCE
NORMAL ➔ RARE FORM
Ocular
➔ Cause: Defect in tubules ➔ CYSTINE DEPOSITION in
(congenital/acquired) → Cystinosis
the CORNEA → Ocular
➔ NOT Reabsorbed by the Impairment
tubules = INCREASE ➔ MOI: Autosomal Recessive
Cystinuria
amount in URINE ➔ Due to: NEPHRONES
Primary ➔ a.k.a. “inborn errors of (PTC) = UNABLE to
Aminoaciduria metabolism REABSORBED AA
➔ Result from: INHERITED (cysteine, dibasic AA e.g.,
DEFECT arginine, lysine, ornithine)
➔ 2 Types of Defects:
1. Enzyme is
DEFECTIVE
Maple Syrup ➔ MOI: Autosomal Recessive
(DEFICIENT) in
the SPECIFIC AA Urine Disease ➔ Accumulation of branched-
metabolic pathway chain AA (leucine,
2. Tubular isoleucine, valine) and their
corresponding α-keto acids
Reabsorptive
Dysfunction in BLOOD, URINE & CSF

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PREPARED BY: MA’AM JENNIFER GAYTANO
➔ Deficient Enzyme: 3. 3.Uridine
Branched-Chain α-Keto diphosphate
Acid Dehydrogenase galactose-4-
(BCKD) epimerase (GALE)
Phenylketonuria ➔ MOI: Autosomal Recessive Porphyrias ➔
➔ INCREASED urinary
excretion of
PHENYLPYRUVIC ACID (a
ketone) and its metabolites
➔ Deficient Enzyme:
PHENYLALANINE
HYDROXYLASE
Alkaptonuria ➔ Urine, Sweat & Breath:
Characteristic
➔ MOUSY/MUSTY ODOR →
caused by →
PHENYLACETIC ACID
➔ MOI: Autosomal Recessive
➔ Excretion of large amount of
HOMOGENTISIC ACID
(HGA) in the urine
➔ Unusual darkening of the
urine when alkali is added
➔ Deficient Enzyme:
HOMOGENTISIC ACID
OXIDASE
Tyrosinuria ➔ Increased amount of
TYROSINE in the urine →
occurs when → tyrosine
levels in PLASMA is
abnormally high
Melanuria ➔ Melanin: Color of hair, eyes
& skin
➔ Hypomelanosis / Albinism:
Defective melanin
production
➔ Melanuria (Increased
excretion of urinary melanin)
= INCREASED production
of MELANIN & its colorless
precursors (e.g., 5,6-
dihydroxyindole)

CARBOHYDRATE DISORDERS
Diabetes ➔ “Diabetes” → copious amount
Mellitus of urine (POLYURIA) that this
disorder produces
➔ “mellitus” → means “sweet”
➔ Polyuria with presence of
glucose in the urine
➔ HIGH:SG
Diabetes ➔ “Diabetes” → copious amount
Insipidus of urine (POLYURIA) that this
disorder produces
➔ “insipidus” → refers to “bland
taste” of urine produced
➔ LOW: SG
Galactosemia ➔ Enzymes Responsible for
Galactosemia:
1. Galactose 1-
phosphate
uridylyltransferase
(GALT)
2. 2.Galactokinase
(GALK)

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