Category: Calcium/Bone Disorders

Monitor: 14

14 - PAGET DISEASE OF BONE WITH NORMAL ALKALINE PHOSPHATASE

Friday, Apr 26
12:30 PM – 1:00 PM

Objective :

Paget Disease of Bone (PDB) is a disorder of abnormal bone metabolism, in which there is a dysregulated bone remodeling causing bone overgrowth and deformity on affected sites with impaired bone integrity. Accelerated bone turnover is typically found in these patients, and serum alkaline phosphatase (sAP) levels tend to correlate with extent and activity of disease. Although PDB with normal sAP levels has been described, mostly in monostotic (single site) disease, is not frequently recognized. 


Methods :  


Results : Case of a 54-year-old man with medical history of Osteoarthritis and Osteopenia who was referred to Endocrinology clinics due to suspected PDB. Patient presented with a three-year history of persistent right knee discomfort, with no trauma. At that time, knee X-ray resulted on right fibular head markedly expanded with lucent and sclerotic changes. Subsequent right knee MRI suggested fibular head calcified chondroid tumor with disruption of fibular head cortex and extension of gastrocnemius muscle, worrisome for chondrosarcoma. Patient evaluated by Oncology service whom order bone scan, resulting in increased activity over right fibula and left hemipelvis.  Due to above findings, left hip MRI was done, showing no acute bone pathology. Evaluated by Orthopedic Oncologist, a left iliac crest biopsy was performed resulting in medullary bone and fibromuscular tissue, ruling out malignancy. Upon evaluation, physical exam unremarkable and biochemical work-up showed normal calcium and phosphorus, as well as normal renal function, transaminases and sAP levels. Bone tumor markers: Pro-collagen type 1 at 96 (nl 22-57ug/dL), N-terminal pro-peptide at (nl 22−87μg/L)and serum C-telopeptide levels at 804 (nl 38-724pg/ml) came back elevated, again with normal sAP levels. As discussed with Nuclear Medicine, bone scan repeated, showing persistent unchanged findings from prior bone scans that correlated with subsequent left hip/pelvis X-ray that showed sclerosis of left iliac bone as well as the ischium and pubis suggesting PDB. Patient was diagnosed with polyostotic PDB.


Discussion :  


Conclusion :

Serum alkaline phosphatase is the most useful biochemical marker to assess and correlate degree of bone disease in PDB; usually it is the first sign found in asymptomatic individuals raising index of suspicion for condition. In most cases, if the disease affects several bones (polyostotic disease), this will result in higher sAP. PDB with normal sAP is less common, especially in those with multiple bone sites involved. This case illustrates the importance of prompt suspicion by clinical findings, and correlation with imaging studies such as bone scans and plain radiographs, as they may be diagnostic. 

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Jinetsy I. Rivera-Ortiz

Endocrinology Fellow
Univeristy of Puerto Rico- Medical Science Campus
Vega Alta, Puerto Rico

I am first year Endocrinology fellow in-training from Puerto Rico. I did my Internal Medicine specialty in the University of Puerto Rico-Medical Science Campus, and I'm currently Internal Medicine Board Certified. My interest are General Endocrinology, Thyroid Disease, Diabetes, Adrenal Disease and Bone.

Milliette Alvarado-Santiago

Attending Physician
Univeristy of Puerto Rico- Medical Science Campus

Dr. Milliette Alvarado Santiago is an Endocrinology Specialist in Rio Piedras, Puerto Rico. She graduated with honors from University Of Puerto Rico School Of Medicine in 2010. Having more than 8 years of diverse experiences, especially in ENDOCRINOLOGY, INTERNAL MEDICINE, Dr. Milliette Alvarado Santiago has dedicated her early career to the academic field, being full time assistant professor at the Endocrinology Fellowship at the University of Puerto Rico.

Margarita Ramirez-Vick

Attending Physician
University of Puerto Rico - Medical Science Campus

Dr. Margarita Ramirez-Vick is an endocrinology specialist in San Juan, PR and has been practicing for 29 years. She graduated from University of Puerto Rico School of Medicine in 1989 and received her medical degree from University of Puerto Rico School of Medicine. She specializes in endocrinology, diabetes & metabolism.

Naomi Collazo-Gutierrez

Endocrinology Fellow
University of Puerto Rico-Medical Science Campus

Dr. Naomi Collazo Gutierrez, MD is an internal medicine specialist in San Juan, PR. Currently, second year endocrinology fellow in training at University of Puerto Rico-Medical Science Campus.

Loida A. Gonzalez-Rodriguez

Assistant Professor
University of Puerto Rico School of Medicine - Endocrinology, Diabetes and Metabolism Division
San Juan, Puerto Rico

Loida A. Gonzalez-Rodriguez, MD is an Endocrinology, Diabetes & Metabolism specialist in San Juan, Puerto Rico. She graduated from University of Puerto Rico - School of Medicine in 2008, completed her specialty in Internal Medicine in 2011 and her sub-specialty in Endocrinology in 2014 at the University Hospital of Puerto Rico. Since then she has been Assistant Professor at the Endocrinology, Diabetes and Metabolism Division of the University of Puerto Rico. Dr. Gonzalez-Rodriguez also participates in different research projects in the area of endocrinology.

Jinetsy I. Rivera-Ortiz

Endocrinology Fellow
Univeristy of Puerto Rico- Medical Science Campus
Vega Alta, Puerto Rico

I am first year Endocrinology fellow in-training from Puerto Rico. I did my Internal Medicine specialty in the University of Puerto Rico-Medical Science Campus, and I'm currently Internal Medicine Board Certified. My interest are General Endocrinology, Thyroid Disease, Diabetes, Adrenal Disease and Bone.