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Ontology Browser

Term:
Parent Terms Term With Siblings Child Terms
kidney disease +     
vascular disease +     
adenovirus renal infection 
Adult familial nephronophthisis - spastic quadriparesia 
aneurysm  
Aneurysm, False 
angioosteohypertrophic syndrome 
anuria 
arterial calcification of infancy +  
arterial disorder +   
arterial occlusive disease +   
arterial tortuosity syndrome 
Arterio-Arterial Fistula 
atheroembolism of kidney 
Autosomal dominant medullary cystic kidney disease with hyperuricemia 
autosomal dominant progressive nephropathy with hypertension 
Bannayan-Riley-Ruvalcaba syndrome 
Behcet's syndrome  
bleeding disorder, vascular-type 
blood vessel injury 
blood vessel neoplasm +   
capillary disorder +  
capillary malformation +  
cardiac embolism  
cerebral arteriopathy with subcortical infarcts and leukoencephalopathy +  
Cerebro-reno-digital syndrome 
Char syndrome 
cholesterol embolism +  
chronic kidney disease +   
congenital primary megaureter +  
Coronary-Subclavian Steal Syndrome 
cystic kidney disease +   
diabetes insipidus +  
duplication of urethra 
Ehlers-Danlos syndrome, vascular type +  
Ehlers-Danlos syndrome, vascular-like type 
Embolism, Paradoxical 
endothelial dysfunction 
epistaxis 
exstrophy-epispadias complex +   
familial thoracic aortic aneurysm and aortic dissection +  
fetal lower urinary tract obstruction +   
glomerular disease +   
hantavirus hemorrhagic fever with renal syndrome +  
Hemangiopericytic Neoplasm +  
hemoglobinuria +  
hemorrhagic fever with renal syndrome  
Hepatic fibrosis - renal cysts - intellectual disability 
hepatic vascular disorder +   
Hepatopulmonary Syndrome 
hereditary arterial and articular multiple calcification syndrome 
hydronephrosis +   
hypertensive nephropathy +   
impaired renal function disease +   
Infantile nephronophthisis 
inherited kidney disorder +   
ischemic colitis 
ischemic disease +   
Juvenile nephronophthisis 
kidney cortex necrosis 
kidney failure +   
kidney neoplasm +   
kidney papillary necrosis +  
lethal arteriopathy syndrome due to fibulin-4 deficiency 
Loeys-Dietz syndrome +  
lymphatic malformation +  
lymphatic vessel neoplasm +   
macrovascular complications of diabetes  
multiple cutaneous and mucosal venous malformations 
multisystemic smooth muscle dysfunction syndrome 
neonatal Marfan syndrome 
nephritis +   
nephrocalcinosis +  
nephrolithiasis +   
nephrosis +   
neurovascular disorder +   
ocular vascular disease +   
patent ductus arteriosus 2 
PDA1 
perinephritis 
peripheral vascular disease +   
polymyositis +   
post-operative acute kidney injury  
pulmonary venoocclusive disease +  
renal aminoaciduria 
renal artery disease +  
renal dysplasia +  
renal glycosuria 
renal hypertension +  
renal hypoplasia +  
renal nutcracker syndrome 
A rare, syndromic renal disease characterized by the entrapment of left renal vein (LRV) between the superior mesenteric artery (SMA) and the abdominal aorta, resulting in increased luminal pressure, renal hilar varices, hematuria and, at the microscopic level, rupture of thin-walled veins into the collecting system in renal fornices. Renal nutcracker syndrome (NCS) is a condition that occurs when the left renal vein (the vein that carries blood purified by the left kidney) becomes compressed between the aorta, abdominal and superior mesenteric artery. The signs and symptoms of the condition can vary from person to person. Some affected people may be asymptomatic while others develop severe and persistent symptoms. When present, features of NCS may include blood in the urine (hematuria), renovascular hypertension, varicose veins, orthostatic proteinuria, flank pain and/or abdominal pain. Some cases of mild NCS in children may be due to changes in body proportions associated with growth. Why NCS occurs or becomes symptomatic in adults is less clear. Treatment ranges from surveillance to surgical intervention and is based on the severity of symptoms and their expected reversibility when considering the affected person's age and stage of the syndrome.
renal tuberculosis +  
renal tubular dysgenesis +  
renal tubular transport disease +  
renal tubule disease +  
skin vascular disease +   
small artery occlusion  
STING-associated vasculopathy with onset in infancy 
stricture or kinking of ureter  
Superior Vena Cava Syndrome 
Systemic capillary leak syndrome 
systemic lupus erythematosus +   
systemic scleroderma +   
Takayasu arteritis  
temporal arteritis  
thoracic outlet syndrome +  
thrombotic disease +   
ureteral disorder +   
vascular anomaly +  
vascular disorder of penis 
vascular ectasia +  
vascular hemostatic disease +  
vascular insufficiency disorder +   
vascular malformation +  
vascular occlusion disorder +  
vascular toxicity 
vasculitis +   
vein disorder +   
venous thromboembolism  
X-linked diffuse leiomyomatosis-Alport syndrome 

Synonyms
Exact Synonyms: Left renal vein entrapment syndrome ;   NCS ;   Orphanet:71273 ;   RNS ;   Renal Nutcracker Phenomenon ;   http://identifiers.org/medgen/465238 ;   http://identifiers.org/mesh/D059228 ;   http://identifiers.org/snomedct/717267005 ;   http://linkedlifedata.com/resource/umls/id/C3178770
Related Synonyms: nutcracker syndrome
Xrefs: DOID:9007228 ;   GARD:11971 ;   MEDGEN:465238 ;   MESH:D059228 ;   MONDO:0019105 ;   ORDO:71273 ;   ORDO:Orphanet_71273 ;   SCTID:717267005 ;   UMLS:C3178770
Definition Sources: Orphanet:71273

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