scholarly journals Endovascular treatment of intrarenal aneurysms bleeding and angiomyolipomas in a patient with tuberous sclerosis and polycystic kidney disease

2021 ◽  
Author(s):  
Túlio Leite ◽  
Lucas Vatanabe Pazinato ◽  
Maria Juliana de Aquino Vidal ◽  
Danielo de Freitas ◽  
Joaquim Mauricio da Motta Leal Filho
Keyword(s):  
Kidney Disease ◽  
Tuberous Sclerosis ◽  
Polycystic Kidney Disease ◽  
Endovascular Embolization ◽  
Renal Tumors ◽  
Polycystic Kidney ◽  
Nephron Sparing ◽  
Contiguous Gene ◽  
Life Threatening ◽  
Autosomal Dominant Polycystic Kidney

Abstract Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are conditions related to renal failure that can rarely occur in association as a contiguous gene syndrome. Angiomyolipomas (AMLs) are renal tumors strongly related to TSC that may rupture and cause life-threatening bleedings. We present a patient with TSC, ADPKD, and renal AMLs with persistent hematuria requiring blood transfusion. The persistent hematuria was successfully treated through endovascular embolization, a minimally invasive nephron sparing technique.

scholarly journals Tuberous sclerosis associated with autosomal dominant polycystic kidney disease: Case report about of the TSC2/PKD1 contiguous gene syndrome

2016 ◽  
Vol 49 (6) ◽  
pp. 583
Author(s):  
Rodolfo M. Queiroz ◽  
Michela P. Gomes ◽  
Marcus V. N. Valentin ◽  
Cecília H. Miyake ◽  
Lucas G. Abud ◽  
...  
Keyword(s):  
Case Report ◽  
Kidney Disease ◽  
Tuberous Sclerosis ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Contiguous Gene ◽  
Disease Case ◽  
Autosomal Dominant Polycystic Kidney

Relatamos o caso de mulher jovem portadora de doença renal crônica, com antecedentes de crises convulsivas, episódios de pneumotórax espontâneos e nefrectomia à esquerda. O estudo retrospectivo dos seus exames de imagem evidenciaram cistos hepáticos, renais e pulmonares; além de túberes corticais e nódulos subependimários no encéfalo. A avaliação anatomopatológica do rim removido cirurgicamente caracterizou doença policística renal do adulto. A revisão clínica em conjunto com esses exames revelou o diagnóstico de esclerose tuberosa e doença renal policística autossômica dominante, sugerindo síndrome do gene contíguo TSC2/PKD1

scholarly journals Tuberous sclerosis complex and polycystic kidney disease together: An exception to the contiguous gene syndrome

2006 ◽  
Vol 8 (3) ◽  
pp. 197-198 ◽  
Author(s):  
Audrey C Woerner ◽  
Kit-Sing Au ◽  
Aimee T Williams ◽  
Peter C Harris ◽  
Hope Northrup
Keyword(s):  
Kidney Disease ◽  
Tuberous Sclerosis ◽  
Polycystic Kidney Disease ◽  
Tuberous Sclerosis Complex ◽  
Polycystic Kidney ◽  
Contiguous Gene

scholarly journals Tranexamic Acid Treatment of Life-Threatening Hematuria in Polycystic Kidney Disease

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Turki AlAmeel ◽  
Michael West
Keyword(s):  
Kidney Disease ◽  
Tranexamic Acid ◽  
Polycystic Kidney Disease ◽  
Polycystic Kidney ◽  
Acid Therapy ◽  
Chronic Renal Impairment ◽  
High Attenuation ◽  
Complex Interactions ◽  
Life Threatening ◽  
Autosomal Dominant Polycystic Kidney

A 41-year-old woman with autosomal dominant polycystic kidney disease had chronic kidney disease class IV. She presented 10 days postpartum with a 4-day history of severe hematuria, left flank pain, and anemia, hemoglobin 62 g/L. CT scan showed massively enlarged kidneys with multiple cysts; several cysts bilaterally had high attenuation consistent with hemorrhage. Hematuria persisted over several days despite intensive conservative measures that included vitamin K1, 4 units of plasma, transfusion of 10 units of packed RBCs, Darbopoeitin, and DDAVP. Antifibrinolytic therapy was given with tranexamic acid 1000 mg p.o. t.i.d for one day then OD. The hematuria stopped within 24 hours and did not recur after tranexamic acid therapy ended. Over the next 4 years there were 3 hospitalizations each with severe gross hematuria requiring blood transfusion for acute anemia. The hematuria responded well to further treatment with tranexamic acid. Tranexamic acid produces antifibrinolytic effects via complex interactions with plasminogen, displacing plasminogen from the fibrin surface. Chronic renal impairment is considered a relative contraindication to use of tranexamic acid due to reports of ureteric clots and acute renal failure from cortical necrosis. We conclude that tranexamic acid can be used safely in some patients with CKD and polycystic kidney disease to treat severe hematuria.

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