Disease Information for Nephritis, familial interstitial

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Clinical Manifestations
Signs & Symptoms
Excess Thirst in Children
Excessive thirst/polydipsia
Muscle weakness
Acutely ill patient/signs
Fever in kids
Thirst Increased
Weakness
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Family History
Family history/Kidney disease
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Microbiology & Serology Findings
Microlab/Sterile urine
Abnormal Lab Findings (Non Measured)
Renal function abnormalities (Lab)
URINE Dipstick/Leukocytes Positive
URINE Pyuria
URINE Pyuria with negative cultures
URINE Sediment/Urinalysis abnormal
Abnormal Lab Findings - Decreased
BUN/Creatinine ratio (Lab)
Creatine clearance (Lab)
Potassium, serum (Lab)
URINE Specific gravity
Abnormal Lab Findings - Increased
BUN/Blood urea nitrogen (Lab)
Fractional excretion Sodium (Lab)
URINE Casts/Protein casts
URINE Eosinophiles
URINE Granular Casts
URINE Leukocyte casts
URINE Leukocytes
URINE Protein (Albumin)
Diagnostic Test Results
X-RAY
Xray/Small kidneys/KUB
Associated Diseases & Rule outs
Rule Outs
IGA nephropathy/Bergers disease
Associated Disease & Complications
Acute anuria/renal failure
Acute Tubulointerstitial Nephropathy
Azotemia in Children
Azotemia/Acute
Hypervolemia
Hypokalemia
Hypomagnesemia
Isosthenuria syndrome
Nephritis tubulointerstitial
Nephritis, interstitial
Proteinuria
Hematuria
Proteinuria in Children
Pyuria
Disease Mechanism & Classification
Class
CLASS/Renal/kidney involvement/disorder (ex)
CLASS/Urologic (category)
Pathophysiology
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Defective Potassium excretion/renal
Pathophysiology/Hereditary disease renal effect
Process
PROCESS/Hereditofamilial (category)
Synonyms
Synonym
Alport, Alport syndrome, Alport syndrome (AS ATS), Alport Syndromes, Alports Syndrome, Alport's syndrome, congenital hereditary hematuria, deafness nephritis syndrome, Dickinson syndrome, hearing loss nephritis syndrome, hematuria nephropathy deafness syndrome, hematuric familial nephropathy, hematuric hereditary nephritis, hemorrhagic familial nephritis, hemorrhagic hereditary nephritis, hereditary familial congenital hemorrhagic nephritis, hereditary hematuria syndrome, hereditary interstitial pyelonephritis, hereditary nephritis, HEREDITARY NEPHRITIS (ALPORTS SYNDROME), HEREDITARY NEPHRITIS ALPORTS SYNDROME, hereditary nephritis deafness abnormal thrombogenesis syndrome, hereditary nephritis deafness syndrome, NEPHRITIS WITH NERVE DEAFNESS HEREDITARY, NEPHROPATHY AND DEAFNESS HEREDITARY, Syndrome Alport, Syndrome Alport's, Syndromes Alport, Synonym/Acute tubulointerstitial nephritis syndrome
Definition

Diagnosis: Fever; Transient maculopapular rash; Acute renal insufficiency; Pyuria (including eosinophiluria), white blood cell casts, and hematuria; Acute interstitial nephritis accounts for 10-15% of cases of intrinsic renal failure; An interstitial inflammatory response with edema and possible tubular cell damage is the typical pathologic finding; Cell-mediated immune reactions prevail over humoral responses; T lymphocytes can cause direct cytotoxicity or release lymphokines that recruit monocytes and inflammatory cells; Although drugs account for over 70% of cases, acute interstitial nephritis also occurs in infectious diseases, immunologic disorders, or as an idiopathic condition; The most common drugs are penicillins and cephalosporins, sulfonamides and sulfonamide-containing diuretics, NSAIDs, rifampin, phenytoin, and allopurinol; Infectious causes include streptococcal infections, leptospirosis, cytomegalovirus, histoplasmosis, and Rocky Mountain spotted fever; Immunologic entities are more commonly associated with glomerulonephritis, but systemic lupus erythematosus, Sjögren"s syndrome, sarcoidosis, and cryoglobulinemia can cause interstitial nephritis; Clinical features can include fever (> 80%), rash (25- 50%), arthralgias, and peripheral blood eosinophilia (80%); The urine often contains red cells (95%), white cells, and white cell casts; Proteinuria can be a feature, particularly in NSAID-induced interstitial nephritis but is usually modest; Eosinophiluria can be detected by Wright"s or Hansel"s stain; Acute interstitial nephritis often carries a good prognosis; Recovery occurs over weeks to months, but acute dialytic therapy may be necessary in up to one-third of all patients before resolution; Patients rarely progress to ESRD; Those with prolonged courses of oliguric failure and advanced age have a worse prognosis; Treatment consists of supportive measures and removal of the inciting agent; If renal failure persists after these steps, a short course of corticosteroids can be given; Short-term, high-dose methylprednisolone 4 days or prednisone 2 weeks followed by a prednisone taper can be used in these more severe cases of drug-induced interstitial nephritis; [CMDXRX2005]

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Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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