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==Overview==
==Overview==
'''Renal papillary necrosis''' is a form of [[nephropathy]] involving the [[ischemic]] [[necrosis]] of the [[renal papilla]], which is supplied by the [[vasa recta]].
'''Renal papillary necrosis''' is a form of [[nephropathy]] involving the [[ischemic]] [[necrosis]] of the [[renal papilla]], which is supplied by the [[vasa recta]].
[[Renal papillary necrosis]] is a significant [[kidney]] disorder, which is associated with chronic conditions such as [[diabetes]], [[sickle cell disease]], [[urinary tract obstruction]], [[urinary tract infection]] and long term use of [[analgesics]] containing [[phenacetin]].
==Historical Perspective==
[[Renal papillary necrosis]] was first introduced by Friedrich in 1877, following [[urinary obstruction]]. Then, other associated factors such as diabetes, sickle cell disease and analgesics have been found.
==Pathophysiology==
It is thought that [[Renal papillary necrosis]] is the result of damage to interstitial medullary and papillary cells due to direct effect of diseases such as [[diabetes]], [[sickle cell disease]], [[urinary tract obstruction]], [[renal vein thrombosis]], upper [[urinary tract infection]], [[tuberculosis]], [[vasculitis]], [[renal]] [[transplant rejection]] and long term [[analgesics]] use such as [[acetaminophen]] and [[NSAIDs]]. Decreased [[prostaglandin]] synthesis and subsequent reduced [[renal]] blood flow is an important mechanism for developing [[renal papillary necrosis]]. Accumulation of sickle-shaped [[RBCs]] in patients with [[sickle cell disease]] can lead to [[intravascular]] [[obstruction]], blood [[stasis]] and [[thrombosis]] that results in [[ischemic necrosis]] of [[renal]] [[papillary]] and [[medullary]] cells.
==Causes==
[[Renal papillary necrosis]] is associated with damaging the [[renal]] [[medullary]] and [[papillary]] cells due to many diseases such as [[diabetes]], [[sickle cell disease]], [[urinary tract obstruction]], [[renal vein thrombosis]], [[pyelonephritis]], [[tuberculosis]], [[vasculitis]], [[cirrhosis]], [[renal]] [[transplant rejection]] and long term [[analgesics]] and [[alcohol]] abuse. In infants, it is associated with [[asphyxia]], [[dehydration]], and [[sepsis]].
==Classification==
[[Renal papillary necrosis]] may be classified according to the severity of [[renal]] parenchymal lesions into two subtypes: [[Medullary]] and [[Papillary]].
==Differential Diagnosis==
[[Renal papillary necrosis]] should be differentiated from other conditions presenting with [[Flank pain|acute flank]] or [[upper abdominal pain]], [[hematuria]], [[nausea and vomiting]]. The differentials include: [[nephrolithiasis]], [[pyelonephritis]], [[renal infarct]], [[renal cell carcinoma]], [[pelvic inflammatory disease]], [[ovarian torsion]], [[testicular torsion]], [[prostatitis]], [[prostatic cancer]], [[ectopic pregnancy]], [[orchitis]], [[appendicitis]], [[cholecystitis]], [[ischemic colitis]], [[peptic ulcer disease]], [[portal vein thrombosis]], [[diverticulitis]], [[abdominal aortic aneurysm]].
==Epidemiology and Demographics==
The prevalence and demographic characteristics of [[renal papillary necrosis]] depend on the causative agent.*[[Renal papillary necrosis]] due to [[analgesic]] abuse is more prevalent in Australia and England and less in the US. The prevalence of [[renal papillary necrosis]] was reported 65,000 per 100,000 patients with [[sickle cell disease]].In developing countries, the incidence of [[renal papillary necrosis]] is 3900 per 100,000 persons.The prevalence of [[renal papillary necrosis]] increases with age. It commonly affects the [[elderly]] (>60 years).[[Renal papillary necrosis]] due to [[sickle cell disease]] commonly affects [[adults]] in the third and fourth decades.[[Renal papillary necrosis]] is more prevalent in [[women]].
==Risk Factors==
==Natural History, Complications & Prognosis==
Common complications of [[renal papillary necrosis]] include [[Kidney infection]] and [[stones]], [[chronic renal failure]], [[kidney]] [[cancers]], [[hypertension]], [[anemia]] and [[ESRD]]. Prognosis depends on the underlying condition.
==Diagnosis==
The diagnosis of renal papillary necrosis is made by a history of having high-risk conditions such as [[analgesic]] abuse and urinalysis and Ultrasound, CT, or other imaging tests of the kidneys.
===History and Symptoms===
The most common symptoms of [[renal papillary necrosis]] include [[back pain]], [[fever]], [[bloody urine]], presence of [[tissues]] in [[urine]], Cloudy [[urine]], [[dysuria]], [[frequency]], [[urgency]] and [[urinary incontinency]]. History of medical conditions such as [[diabetes]] and [[sickle cell disease]] and
long term [[analgesics]] use such as [[acetaminophen]] and [[NSAIDS]] should be considered. If left untreated or progressed, symptoms of [[chronic renal failure]] may be developed.
===Physical Examination===
Common physical examination findings of [[renal papillary necrosis]] include [[fever]], [[costovertebral angle]] [[tenderness]], and [[hematuria]].
===Laboratory Findings===
=== X-ray===
=== CT Scan===
[[CT]] [[urography]] is helpful in the diagnosis of [[renal papillary necrosis]] that shows multiple small accumulations of [[contrast media]] in the [[papillary]] regions near the [[calyx]] system.
The lobster claw sign is a diagnostic finding on [[CT]] [[urography]] in the papillary subtype of [[renal papillary necrosis]] that develops due to elongation of the [[minor calyx]] fornices following [[ischemia]] and destruction of the [[renal]] [[papilla]].
=== MRI ===
MRI can be used as a helpful alternative imaging test in children suspected [[renal papillary necrosis]]. On Magnetic resonance urography, [[renal papillary necrosis]] is characterized by classic signs of [[papillary necrosis]] including candle-flame appearance, Golf ball on a tee sign, lobster claw sign and [[clubbing]] of calyces.
===Other Imaging Findings===
===Biopsy===
On [[renal]] biopsy, [[renal papillary necrosis]] is characterized by [[necrosis]] of [[papilla]], swelling and sloughing of [[papilla]], mild [[inflammation]] and [[microcalcification]] at [[necrotic]] area.
==Treatment==
The mainstay of therapy in [[renal papillary necrosis]] is [[supportive care]] and treating the underlying cause such as discontinuation of [[analgesics]]. In severe cases, [[dialysis]] or [[renal replacement therapy]] may be required.
===Medical Therapy===
The mainstay of therapy is [[supportive care]] and treating the underlying cause.
===Surgery===
In severe cases of [[renal papillary necrosis]] leading to [[chronic kidney disease]], [[dialysis]] or [[renal replacement therapy]] may be required.


It is primarily caused by [[diabetes mellitus]], in which it is related to renal infection or vascular disease. It can also occur as a result of acute [[pyelonephritis]]. Lastly, it can be an outcome of chronic analgesic nephritis, with persistent use or abuse of [[phenacetin]]. This is less common since phenacetin is no longer available over the counter.
===Prevention===
Effective measures for the primary prevention of [[renal papillary necrosis]] include controlling underlying diseases such as [[diabetes]] or [[sickle cell anemia]], avoid taking over-the-counter [[analgesics]] and using the minimum dose of [[NSAIDs]] within the shortest time.


==References==
==References==

Latest revision as of 09:08, 31 August 2020


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Nasrin Nikravangolsefid, MD-MPH [2]

Overview

Renal papillary necrosis is a form of nephropathy involving the ischemic necrosis of the renal papilla, which is supplied by the vasa recta. Renal papillary necrosis is a significant kidney disorder, which is associated with chronic conditions such as diabetes, sickle cell disease, urinary tract obstruction, urinary tract infection and long term use of analgesics containing phenacetin.

Historical Perspective

Renal papillary necrosis was first introduced by Friedrich in 1877, following urinary obstruction. Then, other associated factors such as diabetes, sickle cell disease and analgesics have been found.

Pathophysiology

It is thought that Renal papillary necrosis is the result of damage to interstitial medullary and papillary cells due to direct effect of diseases such as diabetes, sickle cell disease, urinary tract obstruction, renal vein thrombosis, upper urinary tract infection, tuberculosis, vasculitis, renal transplant rejection and long term analgesics use such as acetaminophen and NSAIDs. Decreased prostaglandin synthesis and subsequent reduced renal blood flow is an important mechanism for developing renal papillary necrosis. Accumulation of sickle-shaped RBCs in patients with sickle cell disease can lead to intravascular obstruction, blood stasis and thrombosis that results in ischemic necrosis of renal papillary and medullary cells.

Causes

Renal papillary necrosis is associated with damaging the renal medullary and papillary cells due to many diseases such as diabetes, sickle cell disease, urinary tract obstruction, renal vein thrombosis, pyelonephritis, tuberculosis, vasculitis, cirrhosis, renal transplant rejection and long term analgesics and alcohol abuse. In infants, it is associated with asphyxia, dehydration, and sepsis.

Classification

Renal papillary necrosis may be classified according to the severity of renal parenchymal lesions into two subtypes: Medullary and Papillary.

Differential Diagnosis

Renal papillary necrosis should be differentiated from other conditions presenting with acute flank or upper abdominal pain, hematuria, nausea and vomiting. The differentials include: nephrolithiasis, pyelonephritis, renal infarct, renal cell carcinoma, pelvic inflammatory disease, ovarian torsion, testicular torsion, prostatitis, prostatic cancer, ectopic pregnancy, orchitis, appendicitis, cholecystitis, ischemic colitis, peptic ulcer disease, portal vein thrombosis, diverticulitis, abdominal aortic aneurysm.

Epidemiology and Demographics

The prevalence and demographic characteristics of renal papillary necrosis depend on the causative agent.*Renal papillary necrosis due to analgesic abuse is more prevalent in Australia and England and less in the US. The prevalence of renal papillary necrosis was reported 65,000 per 100,000 patients with sickle cell disease.In developing countries, the incidence of renal papillary necrosis is 3900 per 100,000 persons.The prevalence of renal papillary necrosis increases with age. It commonly affects the elderly (>60 years).Renal papillary necrosis due to sickle cell disease commonly affects adults in the third and fourth decades.Renal papillary necrosis is more prevalent in women.

Risk Factors

Natural History, Complications & Prognosis

Common complications of renal papillary necrosis include Kidney infection and stones, chronic renal failure, kidney cancers, hypertension, anemia and ESRD. Prognosis depends on the underlying condition.

Diagnosis

The diagnosis of renal papillary necrosis is made by a history of having high-risk conditions such as analgesic abuse and urinalysis and Ultrasound, CT, or other imaging tests of the kidneys.

History and Symptoms

The most common symptoms of renal papillary necrosis include back pain, fever, bloody urine, presence of tissues in urine, Cloudy urine, dysuria, frequency, urgency and urinary incontinency. History of medical conditions such as diabetes and sickle cell disease and long term analgesics use such as acetaminophen and NSAIDS should be considered. If left untreated or progressed, symptoms of chronic renal failure may be developed.

Physical Examination

Common physical examination findings of renal papillary necrosis include fever, costovertebral angle tenderness, and hematuria.

Laboratory Findings

X-ray

CT Scan

CT urography is helpful in the diagnosis of renal papillary necrosis that shows multiple small accumulations of contrast media in the papillary regions near the calyx system. The lobster claw sign is a diagnostic finding on CT urography in the papillary subtype of renal papillary necrosis that develops due to elongation of the minor calyx fornices following ischemia and destruction of the renal papilla.

MRI

MRI can be used as a helpful alternative imaging test in children suspected renal papillary necrosis. On Magnetic resonance urography, renal papillary necrosis is characterized by classic signs of papillary necrosis including candle-flame appearance, Golf ball on a tee sign, lobster claw sign and clubbing of calyces.

Other Imaging Findings

Biopsy

On renal biopsy, renal papillary necrosis is characterized by necrosis of papilla, swelling and sloughing of papilla, mild inflammation and microcalcification at necrotic area.

Treatment

The mainstay of therapy in renal papillary necrosis is supportive care and treating the underlying cause such as discontinuation of analgesics. In severe cases, dialysis or renal replacement therapy may be required.

Medical Therapy

The mainstay of therapy is supportive care and treating the underlying cause.

Surgery

In severe cases of renal papillary necrosis leading to chronic kidney disease, dialysis or renal replacement therapy may be required.

Prevention

Effective measures for the primary prevention of renal papillary necrosis include controlling underlying diseases such as diabetes or sickle cell anemia, avoid taking over-the-counter analgesics and using the minimum dose of NSAIDs within the shortest time.

References

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