By

Mahmoud Samir Foda

Ass. Lecturer of Internal Medicine and Nephrology

Faculty of Medicine

Tanta University

Male patient, 43 years old, HTN and ESRD maintained

on regular HD from AVF 6 years ago in HD unit at Tanta

University Hospital.

He is farmer from rural area, married with no special

habits.

Complaint:

Feet pain mainly in lt foot since 9 months ago.

left big toe ulcer 1 month ago.

History of present illness:

The complaint started 9 months ago with gradual onset

and progressive course of feet pain mainly on lt foot pain

burning in character ↑ by touch and dependent position, ↓

by elevation with little improvement by analgesics.

8 months later patient developed left big toe ulcer begin

to appear with erythema of the other toes and erythema of

the Right foot. The patient presented also by Pain in Rt

thigh, weakness and inability to go upstairs.

HD session prescription:

• Session 2.5 hour,3 times /week , pump 270 ml/minute ,

low flux polysulphone 1.2 m2 hemodialyzer using

commercially available dialysate with average Kt/v 0.9.

Past history :

2 years after starting the dialysis, kidney transplantation was

done (1 year and 3 months without dialysis), but unfortunately

chronic graft rejection occurred and the patient had

hemodialysis again.

HTN since 7 years.

history of Cellecpt and Corticosteroids intake during the

period of transplantation .

Family history :

Irrelevant

Drug history

Calcium acetate 2gm/day.

Alphacalcidol 1 micogm/day.

Multivitamins.

Amlodipine 5 mg/day.

IV iron sucrose.

The patient was conscious, pale with uremic facies,

average body built and no special attitude in bed.

Vital signs: blood pressure: 110/70 mmHg.

pulse: 90 b/m, regular, average volume &

dorsalis pedis pulsation felt bilaterally.

Temp: 37 C.

R.R: 14 c/m.

Abdominal examination: ascites.

Extremities: - No lower limb edema.

- left big toe ulcer ( oval in shape ,elevated

margins, with dirty floor) erythema of both

feet.

Chest: Unremarkable

Heart: Unremarkable

pictures of the patient

Liver function tests:

Bilirubin= 1 mg/dl

AST= 30 IU/L ALT= 25 IU/L.

S. albumin= 3 g/dl.

CBC:

• Hb= 10 g/dl (microcytic hypochromic).

• platelets= 150,000/mm³.

• WBCs= 10,000/mm³ (Neut. 88%).

• ESR: 1st h= 60 mm 2nd h= 95 mm.

Prothrombin activity: 95%. APTT: 37 sec.

S. creatinin: 4.4 mg/dl.

B. urea: 66 mg/dl (before) 35 mg/dl (after).

URR: 46.9 %

Uric acid: 6 mg/dl.

FBG: 100 mg/dl. PPBG: 130 mg/dl.

Viral markers:

• HBs Ag: Negative.

• HCV Ab: positive.

• HIV Ab : Negative.

S.Ferritin= 25.9 ng/ml. TSAT% :10%.

Electrolytes & autoimmune markers:

• Na= 132 mmol/L

• K= 4.5 mmol/L

• Po4= 6.0 mg/dl

• i Ca= 0.9 mmol/L

PTH= 3100 pg/ml

ANCA (p&c)= -ve

ANA= -ve

Anti Ds DNA= -ve

Pelvi-Abd US: Atrophic renal graft and moderate ascites.

Chest x ray: - NAD

systemic vasculitis.

peripheral vascular disease.

Atheroemboli.

warfarin-induced skin necrosis.

Cryoglobulinemia.

Calcific Uremic Arteriolopathy(Calciphylaxis).

Hypercoagulability as protein c def.

DD

• Serum Cryoglobline :-ve.

• Rheumatoid factor:-ve .

• Alkaline phosphatase:233u/l (N upto 187)

• protein C level, protein S level, anticardiolipin level,

lupus anticoagulant level: normal.

Lateral x- ray on the abdomen

• Echocardiography :

average L V internal dimensions with

good systolic function EF=74% with

diastolic dysfunction, Dilated left

atrium.

X –ray on the hands and the feet

Duplex on the arterial and venous

system of both lower limbs

• Diffuse arterial atherosclerotic

changes with chronic occlusion of the

tibial arteries.

• The patient received cinacalcet 30mg bid for 2

weeks then once daily and sevelamer 2.4 gm/d.

His investigations after 2 weeks and 1 month

• ionized Ca 1.0 →0.99 mmol/L

• PO4 5.3 → 3.4mg/dL

• PTH 2513 →1489 pg/mL.

• After stopping of cinacalcet there is rising in the PTH level

(PTH=2980 pg/mL) and now the patient is

prepared for parathyrodectomy.

DEFINATION

• Calciphylaxis is a poorly understood

and highly morbid syndrome of

vascular calcification and skin necrosis.

DIAGNOSISHistory:

• A long-standing history of CKD and renal

replacement therapy.

• Very rarely, it may occur in an individual without a

history of chronic renal failure.

• Many persons who develop calciphylaxis have

undergone renal allograft transplantation.

• There are triggers to calciphylaxis :

Long-term obesity.

• Infusion of medications such as iron dextran.

• Remote and/or recent use of immunosuppressive agents,

especially corticosteroids.

• Diabetes mellitus and insulin injections.

• Use of vitamin D and calcium-based phosphate binders.

• Elevated aluminum levels.

• Concomitant vascular disease.

• Concurrent use of warfarin anticoagulation:

Current data suggest that warfarin therapy may

lower protein C concentrations, leading to a

procoagulant condition in the calcified vessel.

• Warfarin may also inhibit carboxylation of matrix

Gla protein, an important inhibitor of calcification,

thus promoting calcification.

EXAMINATION

• Early lesions of calciphylaxis manifest as

nonspecific violaceous mottling; as livedo

reticularis; or as erythematosus papules, plaques,

or nodules.

• More developed lesions have a stellate purpuric

configuration with central cutaneous necrosis .

Lesions may be singular or numerous, and they

generally occur on the lower extremities however,

lesions also may develop on the hands.

Intense pain is a constant finding.

An intact peripheral pulse helps to distinguish acral

calciphylaxis from atherosclerotic peripheral vascular

disease.

TREATMENT

• Reduce serum phosphate level.

• Limit excessive calcium loading.

• Reduce vitamin D analog dose in patients with

suppressed PTH levels.

• Calcimimetics ?? in patients with elevated PTH

levels.

• Parathyroidectomy.