Renal Diseases Flashcards
Examples of Glomerular Diseases
Acute Post- Streptococcal glomerulonephritis, Rapidly progressive glomerulonephritis, Goodpasture Syndrome, Wegener granulomatosis, Henoch-Schonlein purpura, Membranous Glomerulonephritis, Membranoproliferative glomerulonephritis, Chronic Glomerulonephritis, Ig A nephropathy (Berger’s Disease), Minimal Change disease, Focal Segmental Glomerulosclerosis, Diabetic Nephropathy (Kimmelstiel-Wilson Disease), Alport Syndrome
Etiology: Formed in conjunction w/ Grp A Streptococcus infection in the glomerular membranes
Acute Post- Streptococcal glomerulonephritis
Etiology: Immune complex decomposition from systemic immune disorders
Rapidly progressive glomerulonephritis
Attachments of a cytotoxic antibody formed during viral respiratory infections to glomerular & alveolar basement membranes
Goodpasture Syndrome
Etiology: Antineutrophilic cytoplasmic autoantibody (ANCA) binds to neutrophils in vascular walls producing damage to small vessels in the lungs and glomerulus
Wegener granulomatosis
Etiology: Occurs primarily in children following viral respiratory infections
Henoch-Schonlein Purpura
Etiology: Thickening of glomerular membrane
Membranous Glomerulonephritis
Etiology: Cellular proliferation
affecting the capillary walls or the glomerular basement membrane, possibly immune- mediated
Membranoproliferative glomerulonephritis
Etiology: Marked decrease in renal function resulting from glomerular damage precipitated by other renal disorders
Chronic Glomerulonephritis
Etiology: Deposition of IgA on the glomerular membrane resulting from increased levels of serum IgA
Ig A nephropathy (Berger’s Disease)
Etiology: Disruption of the shield of negativity and damage to the right podocyte barrier resulting in massive loss of protein and lipids
NephroticSyndrome
Etiology: Disruption of the podocytes occurring primarily in children following allergic reactions and immunizations
Minimal change disease
Etiology: Disruption of podocytes in certain areas of glomeruli associated with heroin and analgesic abuse and AIDS
Focal Segmental Glomerulosclerosis
Etiology: Deposition of glycosylated
proteins on the glomerular basement membranes caused by poorly controlled glucose levels
Diabetic Nephropathy (Kimmelstiel-Wilson Disease)
Etiology: Genetic disorder showing lamelled of thinning glomerular basement membrane (Prob: Collagen Type IV)
Alport Syndrome
UA TEST/FINDINGS: Mousy odor/ Ferricchloride
Tube test- used to detect presence of urine phenyl pyruvic acid (+) permanent (Blue green color)
PHENYLKETONURIA
Most well-known of the aminoaciduria
PHENYLKETONURIA
ENZYME DEF: Fumarylacetoacetate hydrolase (TYPE 1) / Tyrosine Aminotransferase (TYPE 2) / p-hydroxyphenylpyruvic acid dioxygenase (type 3)
TYROSYLURIA
UA TEST/FINDINGS: Rancidodor / Nitroso-Naphthol test- orange-red color (tyrosine metabolites)
TYROSYLURIA
accumulation of excess tyrosine in the plasma (tyrosinemia) producing urinary overflow
TYROSYLURIA
UA TEST/FINDINGS: Dark urine
MELANURIA
Darkening appears after the urine is exposed to air / Elevatedurinary melanin is a serious finding that indicates proliferation of the normal melanin- producing cells (melanocytes), producing a malignant melanoma
MELANURIA
Homogentisic Acid Oxidase
ALKAPTONURIA
UA TEST/FINDING: Blackurine / Homogentisic
Acid test- (+) black color
ALKAPTONURIA
Etiology: Failure to inherit the gene for the enzyme necessary to produce oxidative decarboxylation of these keto acids (a- ketoisovaleric, a-ketoisocaproic, and a-keto-b-methylvaleric)
MAPLE SYRUP URINE DISEASE
UA TEST/FINDINGS: Maple syrup odor / 2,4-dinitrophenyl- hydrazine (DNPH) test – (+ Yellow turbidity or precipitate)
MAPLE SYRUP URINE DISEASE
Amino acids involved are leucine, isoleucine and valine
MAPLE SYRUP URINE DISEASE
Etiology: Deficiency of Isovaleryl coenzyme A in the leucine pathway
ISOVALERIC ACIDEMIA
Sweaty feet odor
ISOVALERIC ACADEMIA
Etiology: errors in the metabolic pathway converting isoleucine, valine, threonine, and methionine to succinyl coenzyme A
PROPIONIC & METHYLMALONIC ACIDEMIA
Methylmalonic academia- detected using p- nitroaniline test= (+) EMERALD GREEN COLOR
PROPIONIC & METHYLMALONIC ACIDEMIA
Etiology: excess indole is then reabsorbed from the intestine into the blood-stream and circulated to the liver, where it is converted to indican and then excreted in the urine
INDICANURIA
UA TEST/ FINDINGS: Hartnup’s disease- blue staining of infant’s diaper / OBERMAYER TEST
FeCl3 + urine + Chloroform = (+) violet color
INDICANURIA
increased amounts of tryptophan are converted to indole
INDICANURIA
Etiology: due to excess amounts of serotonims
5-HYDROXYINDOLE- ACETIC ACID
UA TEST: Silver Nitroprusside Test- (+) Purple-black color
5-HYDROXYINDOLE- ACETIC ACID
5-HIAA is a degradation product of serotonin / excretion of greater than 25 mg/24 h can be an indication of argentaffin cell tumors / Patient should avoid eating bananas, pineapple & tomatoes prior to testing 5-HIAA
5-HYDROXYINDOLE- ACETIC ACID
Etiology: Inability of renal tubules to reabsorb cystine filtered by the glomerulus
CYSTINURIA
UA TEST: Rotten egg URINE odor / Cyanide Nitroprusside test- (+) Red purple color
CYSTINURIA
Etiology: defect in the lysosomal membranes prevents the release of cystine into the cellular cytoplasm for metabolism / The incomplete metabolism of cystine results in crystalline deposits of cystine in many areas of the body
CYSTINOSIS
UA TEST: Infantile nephropathic cystinosis / Polyuria Generalized aminoaciduria (+) Clinitest for urinary substances Lack of urinary concentration
CYSTINOSIS
Etiology: Defects in the metabolism of the amino acid Methionine
HOMOCYSTINURIA
UA TEST: Cyanide Nitroprusside test- (+) red purple color / Silver Nitroprusside Test- (+)red purple color
HOMOCYSTINURIA
increased homocystine can result in failure to thrive, cataracts, mental retardation, thromboembolic problems, and death / included in Newborn Screening test
HOMOCYSTINURIA
disorders of porphyrin metabolism
PORPHYRIAS
can be inherited or acquired from erythrocytic and hepatic malfunctions or exposure to toxic agents
PORPHYRIAS
Common causes of acquired porphyrias include lead poisoning, excessive alcohol intake, iron deficiency, chronic liver disease, and renal disease.
PORPHYRIAS
free erythrocyte protoporphyrin (FEP) as a screening test for lead poisoning, whole blood is analyzed
PORPHYRIAS
Abnormal skeletal structure
Hurler
Severe mental retardation
Hurler
Mental retardation
San Filippo
Etiology: Failure to inherit the gene to produce the enzyme hypoxanthine phosphoriboxyl transferase
LESCH- NYHAN SYNDROME
Clinical manifestation: motor defects, mental retardation, a tendency toward self-destruction, gout, and renal calculi
LESCH- NYHAN SYNDROME
Lab result: Increased uric acid
LESCH- NYHAN SYNDROME
