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What Syndrome?
due to defective proximal tubular reabsorption
-Type II renal tubular Acidosis (RTA) form bicarbonaturia
Fanconi's Syndrome
Tubulointerstitial Disease
Renal Ultrasound may reveal changes of "medical renal disease":
1. increase echogenicity of the renal parenchyma with loss of _________
2. Prominence of the renal pyramids ________ in some conditions.
1. loss of corticomedullary differentiation
2. cortical scarring
Predominant pathology in Chronic TIN is________ with:
-patchy mononuclear cell infiltration and widespread tubular atrophy
-luminal dilation
-thickening of tubular basement membranes
interstitial fibrosis
What nephritis?
Clinical features of fever, rash, peripheral eosinophilia and oliguric renal failure occuring after 7-10 days of treatment with methicclin or another B-lactam antibiotic?
Allergic Interstitial Nephritis
What medication can cause atypical reaction of AIN in which fever, rash and eosinophilia are rare, but acute renal failure with heavy proteinuria is common?
ARF with features of AIN
If no improvement in 1 week OR rapid progression
Management of:
1. classic allergic AIN
2. Atypical features
1. Corticosteroids
2. Renal biopsy
Absolute indications for corticosteroids and immunosuppresive in Interstitial Nephritis?
1. Sjogren's Syndrome
2. Sarcoidosis
3. SLE interstitial nephritis
4. Adults with TINU
5. idiopathic and other granulomatous interstitial nephritis
Relative indications for corticosteroids and immunosuppressive in interstitial nephritis?
1. Drug Induced or idiopathic AIN with
-Rapid progression of renal failure
-Diffuse infiltrate of renal failure
-Impending need for dialysis
-Delayed recovery
2. Children with TINU
3. Postinfectious AIN with delayed recovery
-is a systemic autoimmune disorder that primarily targets the exocrine glands, especially the lacrimal and salivary glands, and thus results in symptoms such as dry eyes and mouth, that constitute the _______.
-Sjogren's Syndrome
-Sicca Syndrome
The most common renal manifestation of Sjogren's Syndrome?
predominant lymphocytic infiltrate
Sjogren's Syndrome
can be assoc. with:
1. distal RTA
2. nephrogenic diabetes insipidus
3. moderate renal failure
Sjogren's Syndrome
Diagnosis is strongly supported by positive serologic testing for:
______1. (SS-A) antibodies
______2. (SS-B) antibodies
1. anti-Ro
2. anti-La
Sjogren's syndrome
Initial treatment is ___________.
May require maintenance therapy with ________ or ________ to prevent relapse.
1. glucocorticoids
2. azathioprine or mycophenolate mofetil
Hallmark feature, in addition to lymphocyte-predominant interstitial nephritis is
-painful anterior uveitis, often bilateral
& accompanied by:
-blurred vision
Tubulointerstitial nephritis with Uveitis (TINU)

additional extrarenal features:
fever, anorexia, weight loss, abd. pain, arthralgia

elevated crea, sterile pyuria, mild proteinuria, features of Fanconi's syndrome
renal and ocular manifestations generally respond well to?
Oral glucocorticoids
Granulomatous Interstitial Nephritis
Renal biopsy of chronic inflammatory infiltrate with ________ and _________.
1. granulomas
2. multinucleated giant cells
A form of AIN
variably present with:
-autoimmune pancreatitis
-sclerosing cholangitis
-retroperitoneal fibrosis
-chronic sclerosing siladenitis (mimicking Sjogren's syndrome)
IgG4-related systemic Disease
In IgG4-related systemic Disease

_______ that form pseudomotors in the affected organs soon replace the initial inflammatory infiltrates and often lead to biopsy or excision for fear of true malignancy
Fibrotic lesions
IgG4-related systemic disease
________-successfully used as first-line treatment in this group of disorders, once they are correctly diagnosed.
-With typical clinical or histologic features of AIN but have no evidence of drug exposure or clinical or serologic features?
idiopathic AIN

responsive to glucocorticoid
Oliguric acute renal failure, often accompanied by flank pain from tubular obstruction may occur in patient with:
_______1. toxoplasmosis
_______2. HIV
_______3. severe herpesvirus infection
1. Sulfadiazine
2. Indinavir and atazanavir
3. IV acyclovir
What crystals with urinalysis reveals?
_______1. "sheaf of wheat"
_______2. "needle shaped"
_______3. "red-green birefringement needle-shaped"
1. Sulfadiazine
2. Indinavir
3. Acyclovir
Adverse effect of crystal deposition d/o and obstructive tubulopathies is generally precipitated by?______

and is reversible with saline volume repletion and drug withdrawal
Prophylactic _________ reduces the risk of uric acid nephropathy but is of no benefit once tumor lysis has occured.
A recombinant urate oxidase, is usually required to rapidly lower uric acid levels and restore renal function
MC phenotype shared by many ciliopathies?
Kidney Cyst
What mutations in ADPKD?
____1. large 11-transmembrane protein that function like G protein-coupled receptor. -expression is high in development and low in adults.
____2. Calcium permeable 6-transmembrane protein that structurally belongs to transient receptor potential (TRP) cation channel family. -relatively constant
1. PKD 1 polycystin 1 (PC1)
2. PKD 2 polycstin 2 (PC2)
MC life-threatening monogenic disease?
-commonly known as myeloma kidney
-considered who fail to recover when the precipitating factor is corrected or in elderly pt. with otherwise unexplained renal failure
Light Chain Cast Nephropathy
Filtered monoclonal immunoglobulin light chains ________ proteins form intratubular aggregates with secreted ________ protein in the distal tubule.
Bence-Jones proteins
Tamm-Horsfall protein
What Nephropathy
-bone pain
-abnormally narrow anion gap d/t hypoalbuminemia
Urinary dipstick detect _____ but not immunoglobulin light chains.

Serum and urine should both be sent for ______& for _____ for the detection and identification of a potential monoclonal band.

protein electrophoresis
In Lymphomatous infiltration of the kidney, interstitial infiltration by _________ is a common autopsy finding in pts dying of chronic lymphocytic leukemia and non-hodgkin\'s lymphoma, however, this is usually an incidental finding.
malignant B lymphocyte
Banning of OTC _____-containing analgesics has led to dramatic decrease in the incidence of CIN from heavy metal: lead & cadmium exposure analgesic nephropathy in N. America
Previously called chronic pyelonephritis bec. it was believed to result from recurrent UTI\'s in childhood.
Vesicoureteral reflux and reflux nephropathy
What nephropathy?
affected adults are freq. asympt, but may give Hx of prolonged bed-wetting or recurrent UTIs during childhood.

Renal Ultrasound:
-asym. small kidneys w/ irregular outlines
-thinned cortices
-regions of compensatory hypertrophy
Vesicoureteral Reflux and Reflux Nephropathy
In sickle cell Nephropathy
Evidence of tubular injury may be evident in childhood and early adoleacence in the form of:

years before there is significant nephron loaa and proteinuria from secondary FSGS
1. polyuria
d/t decreased conc. ability
2. type IV RTA
What necrosis may result from ischemia d/t sickling of red cell a in the relatively hypoxemic and hypertonic medullary vasculature and present with gross hematuria and ureteric obstruction by sloughed ischemic papillae
Papillary necrosis
Analgesic nephropathy
long-term use of compound analgesic preparations containing

characterized by:
renal insufficiency
papillary necrosis
1. phenacetin
2. aspirin
3. caffeine
What is the radiographic constellation of Analgesic nephropathy, best appreciated by Computed Tomography?
small, scarred kidney with papillary calcifications
2 other term for Aristolochic acid nephropathy?
-Chinese Herbal Nephropathy
-Balkan Endemic Nephropathy
Aristolochic acid, after prolonged exposure, produce ________ with a relative paucity of cellular infiltrates
renal interstitial fibrosis
Aristolochic Acid Nephropathy
Definitive Diagnosis req. 2 of the ff. 3 features?
-char. histology of kidney biopsy
-confirmation of aristolochic acid
-detection of aristolactam-DNA adducts in kidney or urinary tract dis.
What cancer is very high incidence in Aristolochic acid nephropathy, with risk related to cumulative dose?
Upper Urinary Tract Urothelial Cancers
Unusual form of slowly progressive CKD with:
-mild proteinuria
-interstitial fibrosis
-tubular atrophy
-oddly enlarged nuclei of proximal tubular epithelial cells
Karyomegalic interstitial Nephritis
MC manif. of Lithium assoc. Nephropathy
Nephrogenic Diabetes Insipidus

manif. as polyuria and polydipsia
Lithium accumulates in _____ cells in the collecting duct by entering through the ______
principal cells
What enzyme is inhibited by Lithium?
glycogen synthase kinase 3 B
What Nephropathy?
Renal biopsy:
-interstitial fibrosis
-tubular atrophy that are out of proportion to the degree of glomerulosclerosis or vascular dis.
-sparse lymphocytic infiltrate
-small cysts or dilation of the distal tubule and collecting ducts that are highly characteristic of this d/o
Lithium-assoc. Nephropathy
Lithium adjust to avoid toxic levels preferably ____
less than 1 meq/L
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