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==Pathogenesis==
{| class="infobox bordered" style="width: 15em; text-align: left; font-size: 90%; background:AliceBlue"
* 17 alpha-hydroxylase deficiency results in a decreased synthesis of both [[cortisol]] and [[sex steroid]]s, in addition to an increase in [[mineralocorticoid]] production. Hypertension and hypokalemia occur due to the accumulation of cortisol precursors with mineralocorticoid activity upstream of the block, plus sexual infantilism due to inability to synthesize androgens and estrogens. Unlike most other forms of CAH, mineralocorticoid excess and high corticosterone production [9] mitigate the clinical consequences of cortisol deficiency, and symptomatic adrenal insufficiency is rare
|-
* This form of congenital adrenal hyperplasia results from deficiency of the [[enzyme]] [[17α-hydroxylase]] (also called [[CYP17A1]]).<ref name="w">Congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency. Wikipedia (2016. https://en.wikipedia.org/wiki/Congenital_adrenal_hyperplasia_due_to_17_alpha-hydroxylase_deficiency  Accessed on February 4, 2016</ref>
| colspan="1" style="text-align:center; background:LightGrey" |
* 17α-hydroxylase deficiency impairs the efficiency of [[cortisol]] synthesis, resulting in high levels of [[adrenocorticotropic hormone]] secretion and hyperplasia of the adrenal glands.
 
* Clinical effects of this condition include overproduction of [[mineralocorticoid]]s and deficiency of prenatal and [[puberty|pubertal]] [[sex steroid]]s. CYP17A1 functions in [[steroidogenesis]], where it converts [[pregnenolone]] and [[progesterone]] to their 17-hydroxy forms. The enzyme itself is attached to the smooth [[endoplasmic reticulum]] of the steroid-producing cells of the [[adrenal gland|adrenal cortex]] and [[gonad]]s. CYP17A1 functions as both a 17α-hydroxylase and a 17,20-lyase. The dual activities mediate three key transformations in [[cortisol]] and [[sex steroid]] synthesis:
'''Diabetes mellitus Main page'''
* As 17α-hydroxylase it mediates [[pregnenolone]] [[17-hydroxypregnenolone]] and [[progesterone]] [[17-hydroxyprogesterone]].
|- bgcolor="LightGrey"
* As 17,20-lyase it mediates 17-hydroxypregnenolone → [[DHEA|Dehydroepiandrosterone]].
!
* An expected second 17,20-lyase reaction (17-hydroxyprogesterone → [[androstenedione]]) is mediated so inefficiently in humans as to be of no known significance.
 
* The hydroxylase reactions are part of the synthetic pathway to cortisol as well as sex steroids, but the lyase reaction is only necessary for sex steroid synthesis.
|- bgcolor="Pink"
* The dual enzyme activities were for many decades assumed to represent two entirely different genes and enzymes: 7α-hydroxylase deficient congenital adrenal hyperplasia, and a distinct and even rarer defect of sex steroid synthesis termed 17,20-lyase deficiency. In the last decade, it has become clear that the two diseases are different forms of defects of the same gene. However, the clinical features of the two types of impairment are distinct enough that they are described separately in the following sections.
!
===Mineralocorticoid Effects===
Patient Information
* The adrenal cortex is hyperplastic and overstimulated, with no impairment of the mineralocorticoid pathway. Consequently, levels of deoxycorticosterone, [[corticosterone]], and 18-deoxycorticosterone are elevated. Although these precursors of [[aldosterone]] are weaker mineralocorticoids, the extreme elevations usually provide enough volume expansion, blood pressure elevation, and potassium depletion to suppress [[renin]] and aldosterone production. Some persons with 17α-hydroxylase deficiency develop [[hypertension]] in infancy, and nearly 90% do so by late childhood. The low-renin [[hypertension]] is often accompanied by [[hypokalemia]] due to urinary potassium wasting and [[metabolic alkalosis]]. These features of mineralocorticoid excess are the major clinical clue distinguishing the more complete 17α-hydroxylase deficiency from the 17,20-lyase deficiency, which only affects the sex steroids. Treatment with glucocorticoid suppresses ACTH, returns mineralocorticoid production toward normal, and lowers blood pressure.
: [[Diabetes mellitus type 1 (patient information)|Type 1]]
[[Image:Steroidogenesis.png|thumb|center|800px|Production of DHEA from Cholesterol. ([[Cortisol]] is a [[glucocorticoid]].)]]
: [[Diabetes mellitus type 2 (patient information)|Type 2]]
===Glucocorticoid Effects===
|-
* Although production of cortisol is inefficient enough to normalize ACTH, the 50-100-fold elevations of [[corticosterone]] have enough weak [[glucocorticoid]] activity to prevent glucocorticoid deficiency and adrenal crisis.
!
===Sex Steroid Effects===
 
* Genetic XX females affected by 17α-hydroxylase deficiency are born with normal female internal and external anatomy. At the expected time of [[puberty]], neither the adrenals nor the ovaries can produce sex steroids, so neither breast development nor pubic hair appears. Investigation of delayed puberty yields elevated [[gonadotropin]]s and normal karyotype while imaging confirms the presence of ovaries and an infantile uterus. Discovery of hypertension and hypokalemic [[alkalosis]] usually suggests the presence of one of the proximal forms of congenital adrenal hyperplasia, and the characteristic mineralocorticoid elevations confirm the specific diagnosis.
|- bgcolor="Pink"
* A few milder forms of this deficiency in genetic females have allowed relatively normal breast development and irregular menstruation. Evidence suggests that only 5% of normal enzyme activity may be enough to allow at least the physical changes of female puberty, if not [[ovulation]] and fertility. In these girls, the elevated blood pressure was the primary clinical problem.
!
* 17α-Hydroxylase deficiency in genetic males (XY) results in moderate to severe reduction of fetal [[testosterone]] production by both adrenals and testes. [[virilization|Undervirilization]] is variable and sometimes complete. The appearance of the external [[genitalia]] ranges from normal female to ambiguous to mildly underdeveloped male. The most commonly described phenotype is a small [[phallus]], [[perineum|perineal]] [[hypospadias]], small blind pseudovaginal pouch, and intra-abdominal or [[inguinal canal|inguinal]] testes. [[Wolffian duct]] derivatives are hypoplastic or normal, depending on the degree of testosterone deficiency. Some of those with partial virilization develop [[gynecomastia]] at puberty even though masculinization is reduced. The presence of hypertension in the majority distinguishes them from other forms of partial androgen deficiency or [[androgen insensitivity syndrome|insensitivity]]. Fertility is impaired in those with more than minimal testosterone deficiency.
[[Diabetes mellitus#Overview|Overview]]
|-
!
 
|- bgcolor="Pink"
!
[[Diabetes mellitus#Classification|Classification]]
: [[Diabetes mellitus type 1]]
: [[Diabetes mellitus type 2]]
: [[Gestational diabetes]]
|-
!
 
|- bgcolor="Pink"
!
[[Diabetes mellitus#Differential diagnosis|Differential Diagnosis]]
|-
!
 
|- bgcolor="Pink"
!
[[Diabetes mellitus#Complications|Complications]]
|-
!
 
|- bgcolor="Pink"
!
[[Diabetes mellitus#Screening|Screening]]
|-
!
 
|- bgcolor="Pink"
!
[[Diabetes mellitus#Diagnosis|Diagnosis]]
|-
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|- bgcolor="Pink"
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[[Diabetes mellitus#Prevention|Prevention]]
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|}
---------------------------
 
<div style="-webkit-user-select: none;">
{| class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;"
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=zucxZw069kw|350}}
|-
|}
__NOTOC__
 
{{CMG}}
{{Glomerulonephritis}}
 
==Pathophysiology==
===Microscopic Pathology===
 
[http://www.peir.net Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
 
<div align="left">
<gallery heights="175" widths="175">
image:Acute GN 1.jpg|Glomerulonephritis: Micro H&E med mag; an excellent example of AGN with many neutrophils
image:Acute GN 2.jpg|Acute Glomerulonephritis: Micro H&E high mag; an  excellent example of acute exudative glomerulonephritis.
</gallery>
</div>
 
<br>
 
===Glomerulonephritis Videos===
====Rapidly progressive glomerulonephritis====
 
{{#ev:youtube|CqSyj4cVZPE}}
 
 
====Chronic glomerulonephritis====
 
{{#ev:youtube|eA1vYarRAWo}}
 
===Images===
 
[http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 1.jpg|This is a low-power photomicrograph of a saggital section of end stage chronic glomerulonephritis (GN). Note the marked thinning of the cortex (arrow).
Image:Glomerulonephritis case 2.jpg|This is a higher-power photomicrograph of hyalinized glomeruli (arrows) and glomeruli with thick basement membranes.
</gallery>
</div>
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 3.jpg|This is a higher-power photomicrograph of hyalinized glomeruli (1) and glomeruli with thickened basement membranes (2).
Image:Glomerulonephritis case 4.jpg|This is a photomicrograph of interstitial and vascular lesions in end stage renal disease.
</gallery>
</div>
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 5.jpg|This is an immunofluorescent photomicrograph of granular membranous immunofluorescence (immune complex disease). The antibody used for these studies was specific for IgG.  
Image:Glomerulonephritis case 6.jpg|This is an electron micrograph of subepithelial granular electron dense deposits (arrows) which correspond to the granular immunofluorescence seen in the previous image.
</gallery>
</div>
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 7.jpg|This is a photomicrograph of a glomerulus from another case with acute poststreptococcal glomerulonephritis. In this case the immune complex glomerular disease is ongoing with necrosis and accumulation of neutrophils in the glomerulus.
Image:Glomerulonephritis case 8.jpg|This immunofluorescent photomicrograph of a glomerulus from a case of acute poststreptococcal glomerulonephritis shows a granular immunofluorescence pattern consistent with immune complex disease. The primary antibody used for this staining was specific for IgG; however antibodies for complement would show a similar pattern.
</gallery>
</div>
 
<div align="left">
<gallery heights="175" widths="175">
Image:Glomerulonephritis case 9.jpg|This electron micrograph demonstrates scattered subepithelial dense deposits (arrows) and a polymorphonuclear leukocyte in the lumen.
Image:Glomerulonephritis case 10.jpg|For comparison this is an immunofluorescent photomicrograph of a glomerulus from a patient with Goodpasture's syndrome. The linear (arrows) immunofluorescence is characteristic of Goodpasture's syndrome.
</gallery>
</div>
 
===Images:===
 
*[http://www.pathologyatlas.ro/Crescentic%20Glomerulonephritis.html Crescentic GN]
 
*[http://www.pathologyatlas.ro/Chronic%20Glomerulonephritis1.html Chronic GN]
 
==References==
{{Reflist|2}}
 
[[Category:Disease]]
[[Category:Organ disorders]]
[[Category:Inflammations]]
[[Category:Kidney diseases]]
 
[[Category:Needs overview]]
 
{{WH}}
{{WS}}
--------------------------------------------------
===Common Causes===
*[[Churg-strauss syndrome]]
*[[Cryoglobulinaemia]]
*[[Diabetes mellitus type 2]]
*[[Dibasic aminoaciduria type 2]]
*[[Endocarditis]]
*[[Glycogenosis type 1a]]
*[[Henoch-schönlein purpura ]]
*[[Hepatitis b]]
*[[Hereditary onycho-osteodysplasia]]
*[[Hypersensitivity vasculitis]]
*[[Iga nephropathy]]
*[[Lepromatous leprosy]]
*[[Mixed essential cryoglobulinaemia]]
*[[Myeloma]]
*[[Paraneoplastic syndrome]]
*[[Polyarteritis nodosa]]
*[[Radiotherapy]]
*[[Schimke immunoosseous dysplasia]]
*[[Secondary syphilis]]
*[[Serum sickness]]
*[[Sickle cell disease]]
*[[Systemic lupus erythematosus]]
*[[Vasculitis]]
*[[Wegener's granulomatosis]]
*[[Wiskott-aldrich syndrome]]
 
 
--------------------------------------
 
__NOTOC__
 
{{Glomerulonephritis}}
{{CMG}}; {{AE}}{{HK}}
 
==Overview==
Glomerulonephritis may be proliferative or non-proliferative and may be associated with [[Nephrotic syndrome|nephrotic]] or [[Nephritic syndrome|nephritic]] features. The various types of glomerulonephritides should be differentiated from each other based on associations, presence of [[pitting edema]], hemeturia, [[hypertension]], [[hemoptysis]], [[oliguria]], peri-orbital edema, [[hyperlipidemia]], type of [[antibodies]], [[Light microscope|light]] and [[Electron microscopy|electron microscopic]] features.  
 
==Differential Diagnosis==
The following table differentiates between various types of glomerulonephritides:
{| class="wikitable"
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Glomerulonephritis
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Sub-entity
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Causes and associations
! colspan="7" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History and Symtoms
! colspan="9" align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
|-
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Hyperlipidemia and hypercholesterolemia
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephrotic features
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Nephritic features
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |ANCA
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Anti-glomerular basement membrane antibody (Anti-GBM antibody)
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Immune complex formation
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Light microscope
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Electron microscope
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Immunoflourescence pattern
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Pitting edema
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemeturia (pre-dominantly microscopic)
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hypertension
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Hemoptysis
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Oliguria
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Peri-orbital edema
|-
| rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Non-proliferative
!Minimal change disease
|
* Idiopathic
* Protein tyrosine phosphatase receptor type O (glomerular epithelial protein 1- GLEPP1)
|
* Young children
* Recent infection and immunization
* Atopy
* Hodgkin lymphoma
* Thrombosis (due to urinary loss of antithrombin-III)
|
+
|
-
|
-
|
-
|
+/-
|
-
|
+
|
+
|
-
|
-
|
-
|
-
|
* Normal
|
* Fusion of podocytes
|
-
|-
!Focal segmental glomerulosclerosis
|
* Idiopathic
* HIV
* Heroine use
* Sickle cell disease
* Interferon
* Severe obesity
* Mixed cryoglobunemia (Hepatitis C)
|
* Adults
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
| +
| +
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* Focal (some glomeruli) and segmental (only part of glomerulus)
|
* Effacement of podocytes
|<nowiki>-</nowiki>
|-
!Membranous glomerulonephritis
|
* Idiopathic
* Hepatitis B and C
* Solid tumors
* Systemic lupus erythmatosus
* Drugs (NSAIDS, penclliamine, gold, captopril)
|
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+/-</nowiki>
|<nowiki>-</nowiki>
| +
| +
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* Thick glomerular basement membrance
|
* Sub-epithelial immune complex depositis with 'spike and dome' appearance
|<nowiki>-</nowiki>
|-
| rowspan="7" align="center" style="background:#4479BA; color: #FFFFFF;" + |Proliferative
!IgA nephropathy
|
* Idiopathic
* Viral infections
|
* Young children
* History of mucosal infections (e.g. gastroenteritis) and upper respiratory tract infection
* 2-3 days after infection (synpharyngitic)
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+/-</nowiki>
| -
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| +
|
* Crescent formation
|
* Mesangial proliferation
|<nowiki>-</nowiki>
|-
! rowspan="5" |Rapidly progressive glomerulonephritis
|
* Goodpasture syndrome
|
* Young adults
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
| -
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|
* Hypercellular and inflamed glomeruli (Crescent formation)
|
*  Diffuse thickening of the glomerular basement membrane with absence of subepithelial and subendothelial deposits 
|<nowiki>+ (Linear)</nowiki>
|-
|
* Post infectious glomerulonephritis
|
* Streptococcal skin infections
* Streptococcal pharyngitis
* 2-3 weeks after infection
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* Hypercellular and inflamed glomeruli
|
* Sub-epithelial immune complex deposits
| + (Granular)
|-
|
* Granulomatosis with polyangitis (Wegner's granulomatosis)
|
* Necrotizing granulomas (Nasopharynx, lungs, kidneys)
* [[Conjunctivitis]]
* Ulceration of the [[cornea]]
* [[Episcleritis]]
* Peripheral neuropathy
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+ (C-ANCA)</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* Hypercellular and inflamed glomeruli (Crescent formation)
|<nowiki>-  (pauci-immune)</nowiki>
|<nowiki>+/-</nowiki>
|-
|
* Churg Strauss syndrome
|
* Necrotizing granulomas (Lungs and kidneys)
* Asthma
* Peripheral neuropathy
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
+ (C-ANCA)
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* Hypercellular and inflamed glomeruli (Crescent formation)
|<nowiki>- (pauci-immune)</nowiki>
|<nowiki>-</nowiki>
|-
|
* Microscopic polyngitis
|
* Necrotizing vasculitis (no granuloma)
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
| +
|
+ (P-ANCA)
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|
* Hypercellular and inflamed glomeruli (Crescent formation)
|<nowiki>- (pauci-immune)</nowiki>
|<nowiki>-</nowiki>
|-
!Membranoproliferative glomerulonephritis
|
* Idiopathic
* Hepatitis B and C (Type 1)
* C3 nepritic factor (Type2)
|
* Hemeturia
* Oliguria
* Periorbital edema
* Hypertension
|<nowiki>+/-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
|
* Thick glomerular basement membrane (Tram-track appearance)
|
* Mesangial proliferation and leukocyte infiltration
|<nowiki>+ (Granular)</nowiki>
|}
 
==References==
{{Reflist|2}}
 
{{WH}}
{{WS}}
 
[[Category:Needs content]]
 
[[Category:Disease]]
[[Category:Organ disorders]]
[[Category:Inflammations]]
[[Category:Kidney diseases]]

Latest revision as of 06:42, 28 July 2020

Diabetes mellitus Main page

Patient Information

Type 1
Type 2

Overview

Classification

Diabetes mellitus type 1
Diabetes mellitus type 2
Gestational diabetes

Differential Diagnosis

Complications

Screening

Diagnosis

Prevention


https://https://www.youtube.com/watch?v=zucxZw069kw%7C350}}


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Glomerulonephritis Main page

Glomerulonephritis patient information

Overview

Classification

[[]]
[[]]
[[]]

Pathophysiology

Differential Diagnosis

Screening

Diagnosis

Prevention

Pathophysiology

Microscopic Pathology

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology


Glomerulonephritis Videos

Rapidly progressive glomerulonephritis

{{#ev:youtube|CqSyj4cVZPE}}


Chronic glomerulonephritis

{{#ev:youtube|eA1vYarRAWo}}

Images

Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Images:

References

Template:WH Template:WS


Common Causes




Glomerulonephritis Main page

Glomerulonephritis patient information

Overview

Classification

[[]]
[[]]
[[]]

Pathophysiology

Differential Diagnosis

Screening

Diagnosis

Prevention

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [3]

Overview

Glomerulonephritis may be proliferative or non-proliferative and may be associated with nephrotic or nephritic features. The various types of glomerulonephritides should be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features.

Differential Diagnosis

The following table differentiates between various types of glomerulonephritides:

Glomerulonephritis Sub-entity Causes and associations History and Symtoms Laboratory Findings
Hyperlipidemia and hypercholesterolemia Nephrotic features Nephritic features ANCA Anti-glomerular basement membrane antibody (Anti-GBM antibody) Immune complex formation Light microscope Electron microscope Immunoflourescence pattern
History Pitting edema Hemeturia (pre-dominantly microscopic) Hypertension Hemoptysis Oliguria Peri-orbital edema
Non-proliferative Minimal change disease
  • Idiopathic
  • Protein tyrosine phosphatase receptor type O (glomerular epithelial protein 1- GLEPP1)
  • Young children
  • Recent infection and immunization
  • Atopy
  • Hodgkin lymphoma
  • Thrombosis (due to urinary loss of antithrombin-III)

+

-

-

-

+/-

-

+

+

-

-

-

-

  • Normal
  • Fusion of podocytes

-

Focal segmental glomerulosclerosis
  • Idiopathic
  • HIV
  • Heroine use
  • Sickle cell disease
  • Interferon
  • Severe obesity
  • Mixed cryoglobunemia (Hepatitis C)
  • Adults
+ - - - +/- - + + - - - -
  • Focal (some glomeruli) and segmental (only part of glomerulus)
  • Effacement of podocytes
-
Membranous glomerulonephritis
  • Idiopathic
  • Hepatitis B and C
  • Solid tumors
  • Systemic lupus erythmatosus
  • Drugs (NSAIDS, penclliamine, gold, captopril)
+ - - - +/- - + + - - - +
  • Thick glomerular basement membrance
  • Sub-epithelial immune complex depositis with 'spike and dome' appearance
-
Proliferative IgA nephropathy
  • Idiopathic
  • Viral infections
  • Young children
  • History of mucosal infections (e.g. gastroenteritis) and upper respiratory tract infection
  • 2-3 days after infection (synpharyngitic)
+/- + + - + +/- - - + - - +
  • Crescent formation
  • Mesangial proliferation
-
Rapidly progressive glomerulonephritis
  • Goodpasture syndrome
  • Young adults
+/- + + + + + - - + - + +
  • Hypercellular and inflamed glomeruli (Crescent formation)
  •  Diffuse thickening of the glomerular basement membrane with absence of subepithelial and subendothelial deposits 
+ (Linear)
  • Post infectious glomerulonephritis
  • Streptococcal skin infections
  • Streptococcal pharyngitis
  • 2-3 weeks after infection
+/- + + + + + - - + - - +
  • Hypercellular and inflamed glomeruli
  • Sub-epithelial immune complex deposits
+ (Granular)
  • Granulomatosis with polyangitis (Wegner's granulomatosis)
+/- + + + + + - - + + (C-ANCA) - -
  • Hypercellular and inflamed glomeruli (Crescent formation)
- (pauci-immune) +/-
  • Churg Strauss syndrome
  • Necrotizing granulomas (Lungs and kidneys)
  • Asthma
  • Peripheral neuropathy
+/- + + + + + - - +

+ (C-ANCA)

- -
  • Hypercellular and inflamed glomeruli (Crescent formation)
- (pauci-immune) -
  • Microscopic polyngitis
  • Necrotizing vasculitis (no granuloma)
+/- + + + + + - - +

+ (P-ANCA)

- -
  • Hypercellular and inflamed glomeruli (Crescent formation)
- (pauci-immune) -
Membranoproliferative glomerulonephritis
  • Idiopathic
  • Hepatitis B and C (Type 1)
  • C3 nepritic factor (Type2)
  • Hemeturia
  • Oliguria
  • Periorbital edema
  • Hypertension
+/- + + + + + - + - - - +
  • Thick glomerular basement membrane (Tram-track appearance)
  • Mesangial proliferation and leukocyte infiltration
+ (Granular)

References

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