Blue kidney in a pale patient--a case for a causal association between renal haemosiderosis in paroxysmal nocturnal haemoglobinuria and chronic kidney disease

Asim, Muhammad; Iqbal, Zafar; Mujeeb, Imaad

doi:10.1093/ndtplus/sfp057

Cited by 2 publications

(2 citation statements)

References 8 publications

(6 reference statements)

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“…Renal hemosiderosis was ruled out because the dead animal was not anemic; this was indicated by a lack of pallor. Anemia is a consistent characteristic feature of hemosiderosis [14,[18][19][20][21][22]. Hemochromatosis was excluded due to the lack of liver fibrosis, a gross pathological finding involving the liver that has been consistently reported in cases of hemochromatosis [12,15,[23][24][25][26][27].…”

Section: An Image Of a Left Kidney Showing Dark Blue Discoloration Of...mentioning

confidence: 99%

Bovine Renal Lipofuscinosis in a Tanzanian Crossbred Yearling Male Calf: A Case Report

Masola

2024

Adv. Res.

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Aim: To report a rare case of bovine renal lipofuscinosis (BRL) that was encountered incidentally at necropsy. Presentation of the Case: In June 2023 an yearling male calf was found dead in one of the dairy cattle farms in Mbeya city, Tanzania; after being sick for about a week. The owner requested a necropsy service to establish the cause of the death of the animal. Necropsy revealed significant gross pathological changes in a liver that was cooked-like and shrunken, with flabby consistence and large pale necrotic foci. Furthermore, dark blue discoloration of both kidneys with normal morphology was evident. Based on the history and gross pathological findings, the animal was diagnosed to have died from acute hepatitis. Differential diagnosis for kidney discoloration included hemachromatosis, renal hemosiderosis, BRL, and melanotic renal cell carcinoma (MRCC). After ruling out hemachromatosis, renal hemosiderosis and MRCC in the list of possible causes of kidney discoloration based on the lack of gross pathological findings, particularly hepatic fibrosis, pallor, and a mass of tumor tissue in the kidneys that are characteristic of hemachromatosis, renal hemosiderosis and MRCC, respectively; the cause of kidney discoloration was diagnosed as BRL. Discussion: In this necropsy, the history and gross pathology suggested that the animal’s death could be attributed to acute liver failure, which is one of the sequelae of acute hepatitis. Kidney discoloration was not considered an attribute factor to the animal’s death because, with the exception of discoloration, the kidneys did not have gross pathological changes. Moreover, to date there has been no report of a clinical disease in cattle associated with BRL. However, BRL is an important disorder because it leads to condemnation of the affected kidneys as unfit for human consumption. Conclusion: The case of BRL reported here was an incidental finding at necropsy, and is not associated with the death of the animal.

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Section: An Image Of a Left Kidney Showing Dark Blue Discoloration Of...mentioning

confidence: 99%

Bovine Renal Lipofuscinosis in a Tanzanian Crossbred Yearling Male Calf: A Case Report

Masola

2024

Adv. Res.

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“…Renal hemosiderosis is not only a rare cause of AKI; it can also induce chronic kidney failure [62]. Asim et al [63] reported a case of detailed evidence of CKD progressing over a few years, associated with pancytopenia, in a patient who had no previous admissions for hemolytic crisis or AKI. His only relevant findings at renal biopsy were hemosiderosis, interstitial fibrosis and tubular atrophy, and he was later diagnosed with PNH.…”

Section: Pnh and Renal Tubulopathiesmentioning

confidence: 99%

Complement Inhibition Therapy and Dialytic Strategies in Paroxysmal Nocturnal Hemoglobinuria: The Nephrologist’s Opinion

Gembillo

Siligato

Cernaro

et al. 2020

JCM

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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease that presents an estimated incidence of 1.3 cases per million per year, with a prevalence of 15.9 cases per million. It is characterized by hemolysis, bone marrow dysfunction with peripheral blood cytopenia, hypercoagulability, thrombosis, renal impairment and arterial and pulmonary hypertension. Hemolysis and subsequent hemosiderin accumulation in tubular epithelium cells induce tubular atrophy and interstitial fibrosis. The origin of PNH is the somatic mutation in the X-linked phosphatidylinositol glycan class A (PIG-A) gene located on Xp22: this condition leads to the production of clonal blood cells with a deficiency in those surface proteins that protect against the lytic action of the activated complement system. Despite the increased knowledge of this syndrome, therapies for PNH were still only experimental and symptomatic, until the introduction of the C5 complement blockade agent Eculizumab. A second generation of anti-complement agents is currently under investigation, representing future promising therapeutic strategies for patients affected by PNH. In the case of chronic hemolysis and renal iron deposition, a multidisciplinary approach should be considered to avoid or treat acute tubular injury or acute kidney injury (AKI). New promising perspectives derive from complement inhibitors and iron chelators, as well as more invasive treatments such as immunoadsorption or the use of dedicated hemodialysis filters in the presence of AKI.

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Blue kidney in a pale patient--a case for a causal association between renal haemosiderosis in paroxysmal nocturnal haemoglobinuria and chronic kidney disease

Cited by 2 publications

References 8 publications

Bovine Renal Lipofuscinosis in a Tanzanian Crossbred Yearling Male Calf: A Case Report

Bovine Renal Lipofuscinosis in a Tanzanian Crossbred Yearling Male Calf: A Case Report

Complement Inhibition Therapy and Dialytic Strategies in Paroxysmal Nocturnal Hemoglobinuria: The Nephrologist’s Opinion

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