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A 40-year-old woman with end-stage renal disease (ESRD) due to lupus nephritis presented with arteriovenous graft infection. She was receiving hemodialysis 3-times a week for 13 years, but reported non-adherence to medications and dietary restrictions. Physical examination showed prominent jaw bones consistent with uremic leontiasis ossea (Figure 1A arrow heads) [1,2]. Skull radiograph also showed thickening of calvaria and classic "salt-and-pepper sign". Hand radiographs showed diffuse demineralization and extensive acro-osteolysis of the distal phalanges (Figure 1B solid arrows) and cortical tunneling (panel b dotted arrows). She also had severe kypho-scoliosis (Figure 1C) which had progressed over years (upper panels Figures 1A, 1B and 1C and Figures 2D, 2E and 2F taken 6 years apart) leading to restrictive lung disease and chronic hypercapnic respiratory failure. Her serum parathormone (PTH) level was higher than 1900 pg/mL (201 pmol/L) and serum phosphorous level was consistently above 5.5 mg/dL (1.78 mmol/L). These findings were consistent with advanced chronic kidney disease-mineral and bone disorder (CKD-MBD). She was treated with antibiotics, arterio-venous graft resection, and was discharged on sevalamer.

Figure 1: Clinical picture, labelled image

Figure 2: Clinical picture, unlabelled image

CKD-MBD is a systemic disease characterized by abnormalities in mineral hemostasis leading to biochemical abnormalities (serum calcium and phosphorous), endocrine dysfunction (PTH and vitamin D metabolism), anomalous bone turnover and extra-skeletal calcification [3]. It is associated with increased cardiovascular calcification, morbidity and mortality [4,5]. Renal osteodystrophy is the skeletal component of CKD-MBD and refers to the alterations in bone morphology [6]. Since bone biopsy is invasive and expensive, circulating PTH levels are commonly used as surrogate indicator for bone turnover. Proactive management, including optimal control of serum calcium, phosphate, PTH levels and, sometimes timely parathyroidectomy can prevent such severe manifestations [3].

Over the following two years, the patient required multiple hospitalizations for hypercapnic respiratory failure needing mechanical ventilation and eventually passed away in hospice.

References

• 1. Chang JI, Som PM, Lawson W (2007) Unique imaging findings in the facial bones of renal osteodystrophy. AJNR Am J Neuroradiol 28: 608-609.
• 2. Dimkovic N, Piscevic V, Jankovic A, Djuric P (2015) Fatal uremic leontiasis ossea in long-lasting uncontrolled hyperparathyroidism: a case report. Hippokratia 19: 266-267.
• 3. Chapter 1 (2009) Introduction and definition of CKD–MBD and the development of the guideline statements. Kidney International 76: 3-8.
• 4. London GM, Marchais SJ, Guérin AP, Métivier F (2005) Arteriosclerosis, vascular calcifications and cardiovascular disease in uremia. Curr Opin Nephrol Hypertens 14: 525-531.
• 5. Ketteler M, Gross ML, Ritz E (2005) Calcification and cardiovascular problems in renal failure. Kidney Int Suppl 120-127.
• 6. Moe S, Drüeke T, Cunningham J (2006) Definition, evaluation, and classification of renal osteodystrophy: a position statement from Kidney Disease: Improving Global Outcomes (KDIGO). Kidney Int 69: 1945-1953.