Extreme scoliosis from CKD-MBD resulting in respiratory fail
The present case has been reported in BMJ.

A 64-year-old man presented to the hospital because of dyspnoea. Twenty years ago, he had started peritoneal dialysis (PD) for chronic kidney disease. He had been diagnosed with secondary hyperparathyroidism 7 years before his presentation to our hospital, with an intact parathyroid hormone (PTH) 1227 pg/mL (normal range 10–60 pg/mL), calcium level of 10.1 mg/dL (normal range 8.8–10.5 mg/dL), phosphorus level of 5.5 mg/dL (normal range 2.4–4.1 mg/dL) and alkaline phosphatase level of 377 IU/L (normal range 140–338 IU/L).

His medications included cinacalcet. Ultrasonography showed a 5-milimetre nodule at the lower pole of the parathyroid, but 99mTc-hexakis2- methoxyisobutylisonitrile (MIBI)scintigraphy revealed no abnormal accumulation.

The patient’s intact PTH increased to 2606 pg/mL 6 years prior to presentation. At that time, the patient deferred parathyroidectomy. A year later, he switched from PD to haemodialysis. Intact PTH remained elevated to more than 4000 pg/mL. His thorax became scoliotic curving to the left. He had experienced dyspnoea on exertion for 1 year, presenting to our hospital for an exacerbation of these symptoms.

His vital signs on presentation were as follows: body temperature 36.1°C, blood pressure 88/40 mm Hg, heart rate 108/min, a respiratory rate of 24/min and an ambient air oxygen saturation of 88%. His chest examination revealed bilateral diffuse inspiratory crackles. His arterial blood gas analysis values were: pH 7.14, pCO2 89.6 mm Hg, HCO3- 29.8 mEq/L.

Chest radiography demonstrated cardiomegaly and remarkable thoracic deformity. His echocardiography revealed mild mitral and severe tricuspid regurgitation with probable pulmonary hypertension.

The patient was diagnosed with type 2 respiratory failure and pulmonary hypertension due to severe thoracic deformity. He was intubated and placed on mechanical ventilation for 2 days. After receiving 75 mg of cinacalcet, the patient recovered and was discharged home in 2 weeks.

A month later on repeat admission, he died secondary to pneumonia and an exacerbation of his pulmonary hypertension.

Learning points
• Untreated chronic kidney disease-mineral bone disorder (CMD-MBD) causes life-threatening bone deformity.

• Repeated education and proper medications are needed for management of CKD-MBD including adjustment of calcium, phosphorus and hormonal abnormalities.

Read more here: https://pxmd.co/jSVWQ
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