Introduction: Calciphylaxis occurring after kidney transplantation is rare and rarely reported. It results in chronic non-healing wounds and is associated with a poor prognosis and is often fatal. We present a case of proximal lower limb calciphylaxis that occurred early after kidney transplantation. The patient had no classic associated risk factors. He had previously had a total parathyroidectomy but had normal serum calcium-phosphate product and parathyroid hormone levels. The clinical outcome of this case was favorable and highlights some fundamental issues relating to management. 
 
Case prsentation: A 70-year-old British Caucasian man with end-stage renal failure secondary to IgA nephropathy presented six months post kidney transplantation with cutaneous calciphylaxis lesions involving the medial aspect of the thigh bilaterally. 
 
Conclusion: To the best of our knowledge, this is the first reported case of rapid onset cutaneous calciphylaxis occurring soon after kidney transplantation that was associated with a favorable outcome. Cutaneous calciphylaxis lesions should be promptly managed with meticulous wound care, antimicrobial therapy and the correction of calcium-phosphate product where indicated

Andrew R Ready

C Shapiro

H Vanbelleghem

IA Mohammed

M Angelis

Michael A Silva

MR Hayden

N Velasco

Nicholas Inston

Paul Cockwell

R Fox

Rahul Hejmadi

Rajesh Hanvesakul

Steve Mellor

SU Nigwekar

UC Brewster

English

PubMed

Hanvesakul, Rajesh

Silva, Michael A

Hejmadi, Rahul

Mellor, Steve

Ready, Andrew R

Cockwell, Paul

Inston, Nicholas

University of Birmingham Research Archive, E-prints Repository

BioMed CentralJournal of Medical Case Reports
ssOpen AcceCase report
Calciphylaxis following kidney transplantation: a case report
Rajesh Hanvesakul*1, Michael A Silva1, Rahul Hejmadi2, Steve Mellor1, 
Andrew R Ready1, Paul Cockwell1 and Nicholas Inston1
Address: 1Department of Nephrology, University Hospital Birmingham, Birmingham B15 2TH, UK and 2Department of Pathology, University 
Hospital Birmingham, Birmingham B15 2TH, UK
Email: Rajesh Hanvesakul* - rhanvesakul@doctors.org.uk; Michael A Silva - michael.silva@uhb.nhs.uk; 
Rahul Hejmadi - rahul.hejmadi@uhb.nhs.uk; Steve Mellor - steve.mellor@uhb.nhs.uk; Andrew R Ready - andrew.ready@uhb.nhs.uk; 
Paul Cockwell - paul.cockwell@uhb.nhs.uk; Nicholas Inston - nicholas.inston2@uhb.nhs.uk
* Corresponding author    
Abstract
Introduction: Calciphylaxis occurring after kidney transplantation is rare and rarely reported. It
results in chronic non-healing wounds and is associated with a poor prognosis and is often fatal.
We present a case of proximal lower limb calciphylaxis that occurred early after kidney
transplantation. The patient had no classic associated risk factors. He had previously had a total
parathyroidectomy but had normal serum calcium-phosphate product and parathyroid hormone
levels. The clinical outcome of this case was favorable and highlights some fundamental issues
relating to management.
Case presentation: A 70-year-old British Caucasian man with end-stage renal failure secondary
to IgA nephropathy presented six months post kidney transplantation with cutaneous calciphylaxis
lesions involving the medial aspect of the thigh bilaterally.
Conclusion: To the best of our knowledge, this is the first reported case of rapid onset cutaneous
calciphylaxis occurring soon after kidney transplantation that was associated with a favorable
outcome. Cutaneous calciphylaxis lesions should be promptly managed with meticulous wound
care, antimicrobial therapy and the correction of calcium-phosphate product where indicated.
Introduction
Calciphylaxis or calcific uraemic arteriolopathy (CUA) is
a disease that involves calcium deposition in the walls of
small- and medium-sized arteries with consequent ischae-
mic necrosis and gangrene of the supplying tissue. This
condition was first described in the dialysis population
[1] and contrary to its name, is not exclusive to uraemic
patients but may occur in nonuraemic conditions such as
primary hyperparathyroidism, malignancy, alcoholic liver
disease, connective tissue disease, diabetes mellitus,
Crohn's disease and corticosteroid use [2]. CUA has a slow
and indolent onset that classically presents in the distal
lower limbs. However, it may occur in any part of the
body, including visceral tissue [3,4]. In general, systemic
calciphylaxis has a poor prognosis and is often fatal. The
pathogenesis of this disease is not well understood but
may involve abnormal calcium phosphate homeostasis.
There are also several recognized risk factors associated
with the development of CUA, such as hyperparathy-
roidism, elevated calcium-phosphate product (multiply-
Published: 24 November 2009
Journal of Medical Case Reports 2009, 3:9297 doi:10.1186/1752-1947-3-9297
Received: 22 January 2009
Accepted: 24 November 2009
This article is available from: http://www.jmedicalcasereports.com/content/3/1/9297
© 2009 Hanvesakul et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3
(page number not for citation purposes)
Journal of Medical Case Reports 2009, 3:9297 http://www.jmedicalcasereports.com/content/3/1/9297ing serum calcium and phosphate values [normal range
less than 4.5]), diabetes mellitus, obesity, coagulopathies,
warfarin or iron dextran treatment.
There have been a few reported cases of CUA occurring in
patients late after kidney transplantation [4-6]. We
describe an unusual case of rapid onset CUA with super-
imposed infection in a patient six months after kidney
transplantation but with normal serum calcium levels
despite having had a total parathyroidectomy.
Case presentation
A 70-year-old British Caucasian man with end stage renal
failure (ESRF) secondary to IgA nephropathy received a
deceased donor kidney transplant on the 14th January
2008. He had been diagnosed with ESRF 15 years previ-
ously, and was initially dialysing via peritoneal dialysis
and later via haemodialysis. At the time of transplant, his
only remaining vascular access was a long-term intrave-
nous catheter. Many years previously he had undergone a
total parathyroidectomy for tertiary hyperparathyroidism
and therefore took regular calcium supplements and alfa-
calcidol at a dose of 0.25 μg/day. Both pre- and post-kid-
ney transplant parathyroid hormone (PTH) and serum
calcium levels were within normal ranges with a calcium-
phosphate product of less than 4. Post-transplant recovery
was complicated by delayed graft function requiring dial-
ysis for 14 days, transplant wound dehiscence requiring
V.A.C.® dressing, and bladder outflow obstruction requir-
ing intermittent self-catheterization. Despite these com-
plications, he continued to improve with a good urine
output and a glomerular filtration rate of 38.9 ml/min
and was discharged home.
During routine follow-up at the outpatient clinic, the
patient's calcium supplements were modified. He was
admitted four months later with a complaint of sudden
onset of painless lesions bilaterally on the medial aspect
of his thighs. They appeared within the 48 hours prior to
admission and were not associated with a history of
trauma. Serum levels of calcium, phosphate, PTH and cal-
cium-phosphate product were found to be normal. Clini-
cally, both lesions were well demarcated, gangrenous in
appearance and foul smelling (Figure 1). Peripheral
pulses were intact. Swabs taken from this lesion grew Pseu-
domonas and coliforms sensitive to meropenem. A wedge
biopsy of the lesion confirmed the diagnosis of calciphy-
laxis (Figure 2). Intravenous antibiotic was immediately
introduced and all calcium supplements were stopped.
Over a period of one month, the lesions had fully
resolved.
Conclusion
We describe an unusual presentation of proximal lower
limb calciphylaxis with superimposed infection. Interest-
ingly, this patient did not have any of the recognized risk
factors associated with the development of CUA. The clin-
ical outcome in this case has been favorable and empha-
sizes the importance of early diagnosis, aggressive
antimicrobial therapy and wound management. CUA
may present as mild erythematous patches, livido reticu-
laris, painful nodules or necrotic ulcerating lesions, giving
various possible differential diagnoses. Gold standard
treatment for CUA includes diagnostic biopsy to refute
other causes of cutaneous lesions, treatment of hyperpar-
athyroidism, reduction of calcium-phosphate product,
anti-microbial therapy and meticulous wound care. Sev-
eral therapeutic strategies for the treatment of CUA have
been tested including parathyroidectomy, hyperbaric oxy-
gen therapy, sodium thiosulfate infusion, tissue plas-
minogen activator and bisphosphonates but none have
shown any consistent benefit [7]. Cinacalcet has been
On the right leg, a well-demarcated and gangrenous lesion is characteristic of calciphylaxisFigur 1
On the right leg, a well-demarcated and gangrenous 
lesion is characteristic of calciphylaxis. In comparison, 
the lesions on the left leg are those in the early changes of 
calciphylaxis.
Histology showing ischaemic skin changes with ulceration, inflammation, scarr ng (right) and alcification in th  medium-sized arteries in the subcutaneous fat (l ft), typical of calciphylaxis (H & E ×200)Figure 2
Histology showing ischaemic skin changes with ulcer-
ation, inflammation, scarring (right) and calcification 
in the medium-sized arteries in the subcutaneous fat 
(left), typical of calciphylaxis (H & E, ×200).Page 2 of 3
(page number not for citation purposes)
Journal of Medical Case Reports 2009, 3:9297 http://www.jmedicalcasereports.com/content/3/1/9297Publish with BioMed Central   and  every 
scientist can read your work free of charge
"BioMed Central will be the most significant development for 
disseminating the results of biomedical research in our lifetime."
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Your research papers will be:
available free of charge to the entire biomedical community
peer reviewed and published immediately upon acceptance
cited in PubMed and archived on PubMed Central 
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used in the treatment of CUA in individuals who were
anaesthetically unfit for parathyroidectomy. It inhibits
PTH release and has been shown to be effective in the
treatment of CUA in cases where PTH levels were found to
be high (secondary hyperparathyroidism) [8,9]. In gen-
eral, CUA is associated with a poor prognosis if not aggres-
sively managed.
List of abbreviations
CUA: calcific uraemic arteriolopathy; ESRF: end stage
renal failure; GFR: glomerular filtration rate; PTH: parath-
yroid hormone.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
RH, MAS, SM, ARR, PC and NI all made substantial intel-
lectual contributions to the preparation of the manu-
script. RHe performed the histological examination of the
lesion. All authors read and approved the final manu-
script.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
References
1. Angelis M, Wong LL, Myers SA, Wong LM: Calciphylaxis inpa-
tients on hemodialysis: A prevalence study.  Surgery 1997,
122:1083-1089.
2. Nigwekar SU, Wolf M, Sterns RH, Hix JK: Calciphylaxis from non-
uremic causes: A systematic review.  Clin J Am Soc Nephrol 2008,
3:1139-1143.
3. Shapiro C, Coco M: Gastric calciphylaxis in a patient with a
functioning renal allograft.  Clin Nephrol 2007, 67(2):119-125.
4. Brewster UC, Perazella MA: Calcific uremic arteriolopathy in a
transplanted kidney.  Am J Med Sci 2005, 329(2):102-103.
5. Vanbelleghem H, Terryn W, Van Leuven L, Van Caesbroeck D,
Demetter P, Lameire N: A dramatic case of calciphylaxis 20
years after kidney transplantation.  Nephrol Dial Transplant 2004,
19(12):3183-3185.
6. Fox R, Banowsky LH, Cruz AB Jr: Post-renal transplant calciphy-
laxis: successful treatment with parathyroidectomy.  J Urol
1983, 129(2):362-363.
7. Hayden MR, Goldsmith D, Sowers JR, Khanna R: Calciphylaxis: cal-
cific uremic arteriolopathy and the emerging role of sodium
thiosulfate.  Int Urol Nephrol 2008, 40(2):443-451.
8. Mohammed IA, Sekar V, Bubtana AJ, Mitra S, Hutchison AJ: Proximal
calciphylaxis treated with calcimimetric 'cinacalcet'.  Nephrol
Dial Transplant 2008, 23(1):387-389.
9. Velasco N, MacGregor MS, Innes A, MacKay IG: Successful treat-
ment of calciphylaxis with cinacalcet - an alternative to par-
athyroidectomy?  Nephrol Dial Transplant 2006, 21(7):1999-2004.Page 3 of 3
(page number not for citation purposes)


Calciphylaxis following kidney transplantation: a case report

Introduction: Calciphylaxis occurring after kidney transplantation is rare and rarely reported. It results in chronic non-healing wounds and is associated with a poor prognosis and is often fatal. We present a case of proximal lower limb calciphylaxis that occurred early after kidney transplantation. The patient had no classic associated risk factors. He had previously had a total parathyroidectomy but had normal serum calcium-phosphate product and parathyroid hormone levels. The clinical outcome of this case was favorable and highlights some fundamental issues relating to management. Case presentation: A 70-year-old British Caucasian man with end-stage renal failure secondary to IgA nephropathy presented six months post kidney transplantation with cutaneous calciphylaxis lesions involving the medial aspect of the thigh bilaterally. Conclusion: To the best of our knowledge, this is the first reported case of rapid onset cutaneous calciphylaxis occurring soon after kidney transplantation that was associated with a favorable outcome. Cutaneous calciphylaxis lesions should be promptly managed with meticulous wound care, antimicrobial therapy and the correction of calcium-phosphate product where indicated

Hanvesakul, R

Silva, Michael

Mellor, S

Ready, Andrew

University of Birmingham Research Portal

  Calciphylaxis following kidney transplantation: acase report.Hanvesakul, R; Silva, Michael; Hejmadi, Rahul; Mellor, S; Ready, Andrew; Cockwell, Paul;Inston, NicholasDOI:10.1186/1752-1947-3-9297Document VersionPublisher's PDF, also known as Version of recordCitation for published version (Harvard):Hanvesakul, R, Silva, M, Hejmadi, R, Mellor, S, Ready, A, Cockwell, P & Inston, N 2009, 'Calciphylaxis followingkidney transplantation: a case report.', Journal of medical case reports, vol. 3, pp. 9297.https://doi.org/10.1186/1752-1947-3-9297Link to publication on Research at Birmingham portalGeneral rightsUnless a licence is specified above, all rights (including copyright and moral rights) in this document are retained by the authors and/or thecopyright holders. The express permission of the copyright holder must be obtained for any use of this material other than for purposespermitted by law.•	Users may freely distribute the URL that is used to identify this publication.•	Users may download and/or print one copy of the publication from the University of Birmingham research portal for the purpose of privatestudy or non-commercial research.•	User may use extracts from the document in line with the concept of ‘fair dealing’ under the Copyright, Designs and Patents Act 1988 (?)•	Users may not further distribute the material nor use it for the purposes of commercial gain.Where a licence is displayed above, please note the terms and conditions of the licence govern your use of this document.When citing, please reference the published version.Take down policyWhile the University of Birmingham exercises care and attention in making items available there are rare occasions when an item has beenuploaded in error or has been deemed to be commercially or otherwise sensitive.If you believe that this is the case for this document, please contact UBIRA@lists.bham.ac.uk providing details and we will remove access tothe work immediately and investigate.Download date: 01. Feb. 2019BioMed CentralJournal of Medical Case ReportsssOpen AcceCase reportCalciphylaxis following kidney transplantation: a case reportRajesh Hanvesakul*1, Michael A Silva1, Rahul Hejmadi2, Steve Mellor1, Andrew R Ready1, Paul Cockwell1 and Nicholas Inston1Address: 1Department of Nephrology, University Hospital Birmingham, Birmingham B15 2TH, UK and 2Department of Pathology, University Hospital Birmingham, Birmingham B15 2TH, UKEmail: Rajesh Hanvesakul* - rhanvesakul@doctors.org.uk; Michael A Silva - michael.silva@uhb.nhs.uk; Rahul Hejmadi - rahul.hejmadi@uhb.nhs.uk; Steve Mellor - steve.mellor@uhb.nhs.uk; Andrew R Ready - andrew.ready@uhb.nhs.uk; Paul Cockwell - paul.cockwell@uhb.nhs.uk; Nicholas Inston - nicholas.inston2@uhb.nhs.uk* Corresponding author    AbstractIntroduction: Calciphylaxis occurring after kidney transplantation is rare and rarely reported. Itresults in chronic non-healing wounds and is associated with a poor prognosis and is often fatal.We present a case of proximal lower limb calciphylaxis that occurred early after kidneytransplantation. The patient had no classic associated risk factors. He had previously had a totalparathyroidectomy but had normal serum calcium-phosphate product and parathyroid hormonelevels. The clinical outcome of this case was favorable and highlights some fundamental issuesrelating to management.Case presentation: A 70-year-old British Caucasian man with end-stage renal failure secondaryto IgA nephropathy presented six months post kidney transplantation with cutaneous calciphylaxislesions involving the medial aspect of the thigh bilaterally.Conclusion: To the best of our knowledge, this is the first reported case of rapid onset cutaneouscalciphylaxis occurring soon after kidney transplantation that was associated with a favorableoutcome. Cutaneous calciphylaxis lesions should be promptly managed with meticulous woundcare, antimicrobial therapy and the correction of calcium-phosphate product where indicated.IntroductionCalciphylaxis or calcific uraemic arteriolopathy (CUA) isa disease that involves calcium deposition in the walls ofsmall- and medium-sized arteries with consequent ischae-mic necrosis and gangrene of the supplying tissue. Thiscondition was first described in the dialysis population[1] and contrary to its name, is not exclusive to uraemicpatients but may occur in nonuraemic conditions such asprimary hyperparathyroidism, malignancy, alcoholic liverdisease, connective tissue disease, diabetes mellitus,Crohn's disease and corticosteroid use [2]. CUA has a slowand indolent onset that classically presents in the distallower limbs. However, it may occur in any part of thebody, including visceral tissue [3,4]. In general, systemiccalciphylaxis has a poor prognosis and is often fatal. Thepathogenesis of this disease is not well understood butmay involve abnormal calcium phosphate homeostasis.There are also several recognized risk factors associatedwith the development of CUA, such as hyperparathy-roidism, elevated calcium-phosphate product (multiply-Published: 24 November 2009Journal of Medical Case Reports 2009, 3:9297 doi:10.1186/1752-1947-3-9297Received: 22 January 2009Accepted: 24 November 2009This article is available from: http://www.jmedicalcasereports.com/content/3/1/9297© 2009 Hanvesakul et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3(page number not for citation purposes)Journal of Medical Case Reports 2009, 3:9297 http://www.jmedicalcasereports.com/content/3/1/9297ing serum calcium and phosphate values [normal rangeless than 4.5]), diabetes mellitus, obesity, coagulopathies,warfarin or iron dextran treatment.There have been a few reported cases of CUA occurring inpatients late after kidney transplantation [4-6]. Wedescribe an unusual case of rapid onset CUA with super-imposed infection in a patient six months after kidneytransplantation but with normal serum calcium levelsdespite having had a total parathyroidectomy.Case presentationA 70-year-old British Caucasian man with end stage renalfailure (ESRF) secondary to IgA nephropathy received adeceased donor kidney transplant on the 14th January2008. He had been diagnosed with ESRF 15 years previ-ously, and was initially dialysing via peritoneal dialysisand later via haemodialysis. At the time of transplant, hisonly remaining vascular access was a long-term intrave-nous catheter. Many years previously he had undergone atotal parathyroidectomy for tertiary hyperparathyroidismand therefore took regular calcium supplements and alfa-calcidol at a dose of 0.25 μg/day. Both pre- and post-kid-ney transplant parathyroid hormone (PTH) and serumcalcium levels were within normal ranges with a calcium-phosphate product of less than 4. Post-transplant recoverywas complicated by delayed graft function requiring dial-ysis for 14 days, transplant wound dehiscence requiringV.A.C.® dressing, and bladder outflow obstruction requir-ing intermittent self-catheterization. Despite these com-plications, he continued to improve with a good urineoutput and a glomerular filtration rate of 38.9 ml/minand was discharged home.During routine follow-up at the outpatient clinic, thepatient's calcium supplements were modified. He wasadmitted four months later with a complaint of suddenonset of painless lesions bilaterally on the medial aspectof his thighs. They appeared within the 48 hours prior toadmission and were not associated with a history oftrauma. Serum levels of calcium, phosphate, PTH and cal-cium-phosphate product were found to be normal. Clini-cally, both lesions were well demarcated, gangrenous inappearance and foul smelling (Figure 1). Peripheralpulses were intact. Swabs taken from this lesion grew Pseu-domonas and coliforms sensitive to meropenem. A wedgebiopsy of the lesion confirmed the diagnosis of calciphy-laxis (Figure 2). Intravenous antibiotic was immediatelyintroduced and all calcium supplements were stopped.Over a period of one month, the lesions had fullyresolved.ConclusionWe describe an unusual presentation of proximal lowerlimb calciphylaxis with superimposed infection. Interest-ingly, this patient did not have any of the recognized riskfactors associated with the development of CUA. The clin-ical outcome in this case has been favorable and empha-sizes the importance of early diagnosis, aggressiveantimicrobial therapy and wound management. CUAmay present as mild erythematous patches, livido reticu-laris, painful nodules or necrotic ulcerating lesions, givingvarious possible differential diagnoses. Gold standardtreatment for CUA includes diagnostic biopsy to refuteother causes of cutaneous lesions, treatment of hyperpar-athyroidism, reduction of calcium-phosphate product,anti-microbial therapy and meticulous wound care. Sev-eral therapeutic strategies for the treatment of CUA havebeen tested including parathyroidectomy, hyperbaric oxy-gen therapy, sodium thiosulfate infusion, tissue plas-minogen activator and bisphosphonates but none haveshown any consistent benefit [7]. Cinacalcet has beenOn the right leg, a well-demarcated and gangrenous lesion is characteristic of calciphylaxisFigur 1On the right leg, a well-demarcated and gangrenous lesion is characteristic of calciphylaxis. In comparison, the lesions on the left leg are those in the early changes of calciphylaxis.Histology showing ischaemic skin changes with ulceration, inflammation, scarr ng (right) and alcification in th  medium-sized arteries in the subcutaneous fat (l ft), typical of calciphylaxis (H & E ×200)Figure 2Histology showing ischaemic skin changes with ulcer-ation, inflammation, scarring (right) and calcification in the medium-sized arteries in the subcutaneous fat (left), typical of calciphylaxis (H & E, ×200).Page 2 of 3(page number not for citation purposes)Journal of Medical Case Reports 2009, 3:9297 http://www.jmedicalcasereports.com/content/3/1/9297Publish with BioMed Central   and  every scientist can read your work free of charge"BioMed Central will be the most significant development for disseminating the results of biomedical research in our lifetime."Sir Paul Nurse, Cancer Research UKYour research papers will be:available free of charge to the entire biomedical communitypeer reviewed and published immediately upon acceptancecited in PubMed and archived on PubMed Central yours — you keep the copyrightSubmit your manuscript here:http://www.biomedcentral.com/info/publishing_adv.aspBioMedcentralused in the treatment of CUA in individuals who wereanaesthetically unfit for parathyroidectomy. It inhibitsPTH release and has been shown to be effective in thetreatment of CUA in cases where PTH levels were found tobe high (secondary hyperparathyroidism) [8,9]. In gen-eral, CUA is associated with a poor prognosis if not aggres-sively managed.List of abbreviationsCUA: calcific uraemic arteriolopathy; ESRF: end stagerenal failure; GFR: glomerular filtration rate; PTH: parath-yroid hormone.Competing interestsThe authors declare that they have no competing interests.Authors' contributionsRH, MAS, SM, ARR, PC and NI all made substantial intel-lectual contributions to the preparation of the manu-script. RHe performed the histological examination of thelesion. All authors read and approved the final manu-script.ConsentWritten informed consent was obtained from the patientfor publication of this case report and accompanyingimages. A copy of the written consent is available forreview by the Editor-in-Chief of this journal.References1. Angelis M, Wong LL, Myers SA, Wong LM: Calciphylaxis inpa-tients on hemodialysis: A prevalence study.  Surgery 1997,122:1083-1089.2. Nigwekar SU, Wolf M, Sterns RH, Hix JK: Calciphylaxis from non-uremic causes: A systematic review.  Clin J Am Soc Nephrol 2008,3:1139-1143.3. Shapiro C, Coco M: Gastric calciphylaxis in a patient with afunctioning renal allograft.  Clin Nephrol 2007, 67(2):119-125.4. Brewster UC, Perazella MA: Calcific uremic arteriolopathy in atransplanted kidney.  Am J Med Sci 2005, 329(2):102-103.5. Vanbelleghem H, Terryn W, Van Leuven L, Van Caesbroeck D,Demetter P, Lameire N: A dramatic case of calciphylaxis 20years after kidney transplantation.  Nephrol Dial Transplant 2004,19(12):3183-3185.6. Fox R, Banowsky LH, Cruz AB Jr: Post-renal transplant calciphy-laxis: successful treatment with parathyroidectomy.  J Urol1983, 129(2):362-363.7. Hayden MR, Goldsmith D, Sowers JR, Khanna R: Calciphylaxis: cal-cific uremic arteriolopathy and the emerging role of sodiumthiosulfate.  Int Urol Nephrol 2008, 40(2):443-451.8. Mohammed IA, Sekar V, Bubtana AJ, Mitra S, Hutchison AJ: Proximalcalciphylaxis treated with calcimimetric 'cinacalcet'.  NephrolDial Transplant 2008, 23(1):387-389.9. Velasco N, MacGregor MS, Innes A, MacKay IG: Successful treat-ment of calciphylaxis with cinacalcet - an alternative to par-athyroidectomy?  Nephrol Dial Transplant 2006, 21(7):1999-2004.Page 3 of 3(page number not for citation purposes)

Calciphylaxis following kidney transplantation: a case report.

Abstract Introduction Calciphylaxis occurring after kidney transplantation is rare and rarely reported. It results in chronic non-healing wounds and is associated with a poor prognosis and is often fatal. We present a case of proximal lower limb calciphylaxis that occurred early after kidney transplantation. The patient had no classic associated risk factors. He had previously had a total parathyroidectomy but had normal serum calcium-phosphate product and parathyroid hormone levels. The clinical outcome of this case was favorable and highlights some fundamental issues relating to management. Case presentation A 70-year-old British Caucasian man with end-stage renal failure secondary to IgA nephropathy presented six months post kidney transplantation with cutaneous calciphylaxis lesions involving the medial aspect of the thigh bilaterally. Conclusion To the best of our knowledge, this is the first reported case of rapid onset cutaneous calciphylaxis occurring soon after kidney transplantation that was associated with a favorable outcome. Cutaneous calciphylaxis lesions should be promptly managed with meticulous wound care, antimicrobial therapy and the correction of calcium-phosphate product where indicated.</p

Hanvesakul Rajesh

Silva Michael A

Hejmadi Rahul

Mellor Steve

Ready Andrew R

Cockwell Paul

Inston Nicholas

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Journal of Medical Case Reports

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  University of BirminghamCalciphylaxis following kidney transplantation: acase report.Hanvesakul, R; Silva, Michael; Hejmadi, Rahul; Mellor, S; Ready, Andrew; Cockwell, Paul;Inston, NicholasDOI:10.1186/1752-1947-3-9297Document VersionPublisher's PDF, also known as Version of recordCitation for published version (Harvard):Hanvesakul, R, Silva, M, Hejmadi, R, Mellor, S, Ready, A, Cockwell, P & Inston, N 2009, 'Calciphylaxis followingkidney transplantation: a case report.', Journal of medical case reports, vol. 3, pp. 9297.https://doi.org/10.1186/1752-1947-3-9297Link to publication on Research at Birmingham portalGeneral rightsUnless a licence is specified above, all rights (including copyright and moral rights) in this document are retained by the authors and/or thecopyright holders. 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Mar. 2020BioMed CentralJournal of Medical Case ReportsssOpen AcceCase reportCalciphylaxis following kidney transplantation: a case reportRajesh Hanvesakul*1, Michael A Silva1, Rahul Hejmadi2, Steve Mellor1, Andrew R Ready1, Paul Cockwell1 and Nicholas Inston1Address: 1Department of Nephrology, University Hospital Birmingham, Birmingham B15 2TH, UK and 2Department of Pathology, University Hospital Birmingham, Birmingham B15 2TH, UKEmail: Rajesh Hanvesakul* - rhanvesakul@doctors.org.uk; Michael A Silva - michael.silva@uhb.nhs.uk; Rahul Hejmadi - rahul.hejmadi@uhb.nhs.uk; Steve Mellor - steve.mellor@uhb.nhs.uk; Andrew R Ready - andrew.ready@uhb.nhs.uk; Paul Cockwell - paul.cockwell@uhb.nhs.uk; Nicholas Inston - nicholas.inston2@uhb.nhs.uk* Corresponding author    AbstractIntroduction: Calciphylaxis occurring after kidney transplantation is rare and rarely reported. Itresults in chronic non-healing wounds and is associated with a poor prognosis and is often fatal.We present a case of proximal lower limb calciphylaxis that occurred early after kidneytransplantation. The patient had no classic associated risk factors. He had previously had a totalparathyroidectomy but had normal serum calcium-phosphate product and parathyroid hormonelevels. The clinical outcome of this case was favorable and highlights some fundamental issuesrelating to management.Case presentation: A 70-year-old British Caucasian man with end-stage renal failure secondaryto IgA nephropathy presented six months post kidney transplantation with cutaneous calciphylaxislesions involving the medial aspect of the thigh bilaterally.Conclusion: To the best of our knowledge, this is the first reported case of rapid onset cutaneouscalciphylaxis occurring soon after kidney transplantation that was associated with a favorableoutcome. Cutaneous calciphylaxis lesions should be promptly managed with meticulous woundcare, antimicrobial therapy and the correction of calcium-phosphate product where indicated.IntroductionCalciphylaxis or calcific uraemic arteriolopathy (CUA) isa disease that involves calcium deposition in the walls ofsmall- and medium-sized arteries with consequent ischae-mic necrosis and gangrene of the supplying tissue. Thiscondition was first described in the dialysis population[1] and contrary to its name, is not exclusive to uraemicpatients but may occur in nonuraemic conditions such asprimary hyperparathyroidism, malignancy, alcoholic liverdisease, connective tissue disease, diabetes mellitus,Crohn's disease and corticosteroid use [2]. CUA has a slowand indolent onset that classically presents in the distallower limbs. However, it may occur in any part of thebody, including visceral tissue [3,4]. In general, systemiccalciphylaxis has a poor prognosis and is often fatal. Thepathogenesis of this disease is not well understood butmay involve abnormal calcium phosphate homeostasis.There are also several recognized risk factors associatedwith the development of CUA, such as hyperparathy-roidism, elevated calcium-phosphate product (multiply-Published: 24 November 2009Journal of Medical Case Reports 2009, 3:9297 doi:10.1186/1752-1947-3-9297Received: 22 January 2009Accepted: 24 November 2009This article is available from: http://www.jmedicalcasereports.com/content/3/1/9297© 2009 Hanvesakul et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3(page number not for citation purposes)Journal of Medical Case Reports 2009, 3:9297 http://www.jmedicalcasereports.com/content/3/1/9297ing serum calcium and phosphate values [normal rangeless than 4.5]), diabetes mellitus, obesity, coagulopathies,warfarin or iron dextran treatment.There have been a few reported cases of CUA occurring inpatients late after kidney transplantation [4-6]. Wedescribe an unusual case of rapid onset CUA with super-imposed infection in a patient six months after kidneytransplantation but with normal serum calcium levelsdespite having had a total parathyroidectomy.Case presentationA 70-year-old British Caucasian man with end stage renalfailure (ESRF) secondary to IgA nephropathy received adeceased donor kidney transplant on the 14th January2008. He had been diagnosed with ESRF 15 years previ-ously, and was initially dialysing via peritoneal dialysisand later via haemodialysis. At the time of transplant, hisonly remaining vascular access was a long-term intrave-nous catheter. Many years previously he had undergone atotal parathyroidectomy for tertiary hyperparathyroidismand therefore took regular calcium supplements and alfa-calcidol at a dose of 0.25 μg/day. Both pre- and post-kid-ney transplant parathyroid hormone (PTH) and serumcalcium levels were within normal ranges with a calcium-phosphate product of less than 4. Post-transplant recoverywas complicated by delayed graft function requiring dial-ysis for 14 days, transplant wound dehiscence requiringV.A.C.® dressing, and bladder outflow obstruction requir-ing intermittent self-catheterization. Despite these com-plications, he continued to improve with a good urineoutput and a glomerular filtration rate of 38.9 ml/minand was discharged home.During routine follow-up at the outpatient clinic, thepatient's calcium supplements were modified. He wasadmitted four months later with a complaint of suddenonset of painless lesions bilaterally on the medial aspectof his thighs. They appeared within the 48 hours prior toadmission and were not associated with a history oftrauma. Serum levels of calcium, phosphate, PTH and cal-cium-phosphate product were found to be normal. Clini-cally, both lesions were well demarcated, gangrenous inappearance and foul smelling (Figure 1). Peripheralpulses were intact. Swabs taken from this lesion grew Pseu-domonas and coliforms sensitive to meropenem. A wedgebiopsy of the lesion confirmed the diagnosis of calciphy-laxis (Figure 2). Intravenous antibiotic was immediatelyintroduced and all calcium supplements were stopped.Over a period of one month, the lesions had fullyresolved.ConclusionWe describe an unusual presentation of proximal lowerlimb calciphylaxis with superimposed infection. Interest-ingly, this patient did not have any of the recognized riskfactors associated with the development of CUA. The clin-ical outcome in this case has been favorable and empha-sizes the importance of early diagnosis, aggressiveantimicrobial therapy and wound management. CUAmay present as mild erythematous patches, livido reticu-laris, painful nodules or necrotic ulcerating lesions, givingvarious possible differential diagnoses. Gold standardtreatment for CUA includes diagnostic biopsy to refuteother causes of cutaneous lesions, treatment of hyperpar-athyroidism, reduction of calcium-phosphate product,anti-microbial therapy and meticulous wound care. Sev-eral therapeutic strategies for the treatment of CUA havebeen tested including parathyroidectomy, hyperbaric oxy-gen therapy, sodium thiosulfate infusion, tissue plas-minogen activator and bisphosphonates but none haveshown any consistent benefit [7]. Cinacalcet has beenOn the right leg, a well-demarcated and gangrenous lesion is characteristic of calciphylaxisFigur 1On the right leg, a well-demarcated and gangrenous lesion is characteristic of calciphylaxis. In comparison, the lesions on the left leg are those in the early changes of calciphylaxis.Histology showing ischaemic skin changes with ulceration, inflammation, scarr ng (right) and alcification in th  medium-sized arteries in the subcutaneous fat (l ft), typical of calciphylaxis (H & E ×200)Figure 2Histology showing ischaemic skin changes with ulcer-ation, inflammation, scarring (right) and calcification in the medium-sized arteries in the subcutaneous fat (left), typical of calciphylaxis (H & E, ×200).Page 2 of 3(page number not for citation purposes)Journal of Medical Case Reports 2009, 3:9297 http://www.jmedicalcasereports.com/content/3/1/9297Publish with BioMed Central   and  every scientist can read your work free of charge"BioMed Central will be the most significant development for disseminating the results of biomedical research in our lifetime."Sir Paul Nurse, Cancer Research UKYour research papers will be:available free of charge to the entire biomedical communitypeer reviewed and published immediately upon acceptancecited in PubMed and archived on PubMed Central yours — you keep the copyrightSubmit your manuscript here:http://www.biomedcentral.com/info/publishing_adv.aspBioMedcentralused in the treatment of CUA in individuals who wereanaesthetically unfit for parathyroidectomy. It inhibitsPTH release and has been shown to be effective in thetreatment of CUA in cases where PTH levels were found tobe high (secondary hyperparathyroidism) [8,9]. In gen-eral, CUA is associated with a poor prognosis if not aggres-sively managed.List of abbreviationsCUA: calcific uraemic arteriolopathy; ESRF: end stagerenal failure; GFR: glomerular filtration rate; PTH: parath-yroid hormone.Competing interestsThe authors declare that they have no competing interests.Authors' contributionsRH, MAS, SM, ARR, PC and NI all made substantial intel-lectual contributions to the preparation of the manu-script. RHe performed the histological examination of thelesion. All authors read and approved the final manu-script.ConsentWritten informed consent was obtained from the patientfor publication of this case report and accompanyingimages. A copy of the written consent is available forreview by the Editor-in-Chief of this journal.References1. Angelis M, Wong LL, Myers SA, Wong LM: Calciphylaxis inpa-tients on hemodialysis: A prevalence study.  Surgery 1997,122:1083-1089.2. Nigwekar SU, Wolf M, Sterns RH, Hix JK: Calciphylaxis from non-uremic causes: A systematic review.  Clin J Am Soc Nephrol 2008,3:1139-1143.3. Shapiro C, Coco M: Gastric calciphylaxis in a patient with afunctioning renal allograft.  Clin Nephrol 2007, 67(2):119-125.4. Brewster UC, Perazella MA: Calcific uremic arteriolopathy in atransplanted kidney.  Am J Med Sci 2005, 329(2):102-103.5. Vanbelleghem H, Terryn W, Van Leuven L, Van Caesbroeck D,Demetter P, Lameire N: A dramatic case of calciphylaxis 20years after kidney transplantation.  Nephrol Dial Transplant 2004,19(12):3183-3185.6. Fox R, Banowsky LH, Cruz AB Jr: Post-renal transplant calciphy-laxis: successful treatment with parathyroidectomy.  J Urol1983, 129(2):362-363.7. Hayden MR, Goldsmith D, Sowers JR, Khanna R: Calciphylaxis: cal-cific uremic arteriolopathy and the emerging role of sodiumthiosulfate.  Int Urol Nephrol 2008, 40(2):443-451.8. Mohammed IA, Sekar V, Bubtana AJ, Mitra S, Hutchison AJ: Proximalcalciphylaxis treated with calcimimetric 'cinacalcet'.  NephrolDial Transplant 2008, 23(1):387-389.9. Velasco N, MacGregor MS, Innes A, MacKay IG: Successful treat-ment of calciphylaxis with cinacalcet - an alternative to par-athyroidectomy?  Nephrol Dial Transplant 2006, 21(7):1999-2004.Page 3 of 3(page number not for citation purposes)

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Hutchison AJ: Proximal calciphylaxis treated with calcimimetric 'cinacalcet'. Nephrol Dial Transplant

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Lameire N: A dramatic case of calciphylaxis 20 years after kidney transplantation. Nephrol Dial Transplant

Perazella MA: Calcific uremic arteriolopathy in a transplanted kidney.

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Wong LM: Calciphylaxis inpatients on hemodialysis: A prevalence study. Surgery

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Calciphylaxis following kidney transplantation: a case report

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