The Increase of Thyroid Abnormalities, Elevated Serum Beta Globulins and Type A Blood Group in Paget's Disease.

Paget’s disease of the bone (PDB) or osteitis deformans is a chronic metabolic bone disorder, characterized by focal increase in bone resorption, followed by a rapid and abnormal bone repair with a disorganized and excessive bone formation, leading to pain, bone deformities and, eventually, fractures. Although the exact etiology of the disease remains controversial, genetic background and environmental factors (viral infections) are typically involved in the complex pathobiology pathways of PDB. The diagnosis of PDB, subsequent classification in disease subtype (monostotic or polyostotic), activity and severity, and complications (fracture, deformities, osteoarthritis, secondary development of tumors, hearing loss, neural compression, high output congestive cardiac failure) are usually made using a sequence of a blood tests (biochemical markers of bone turnover, especially elevated serum alkaline phosphatase) and imaging (x-ray or bone scan). Bisphosphonates are widely used for the management aiming to reduce bone turnover, promote healing of osteolytic lesions and improve bone pain. We report the case of a polyostotic Paget’s disease of bone as an unusual case of low back pain.

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Asymptomatic Paget's disease of bone in a 62-year-old Nigerian man: three years post-alendronate therapy

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-16-0005 ◽

2016 ◽

Vol 2016 ◽

Author(s):

Clement Olukayode Aransiola ◽

Arinola Ipadeola

Keyword(s):

Alkaline Phosphatase ◽

Reference Range ◽

Paget’S Disease ◽

Elevated Serum ◽

Paget's Disease ◽

Paget’S Disease Of Bone ◽

List Type ◽

Paget's Disease Of Bone ◽

Total Alkaline Phosphatase ◽

Total Alkaline

Summary Paget's disease is a chronic and progressive disorder of bone characterized by focal areas of excessive osteoclastic resorption accompanied by a secondary increase in the osteoblastic activity. Paget's disease of bone (PBD) is a rare endocrine disease especially among Africans and Asians. Hence the detection of a case in a middle-aged Nigerian is of interest. We present the case of a 62-year-old Nigerian man in apparent good health who was found to have a markedly elevated serum total alkaline phosphatase (ALP) of 1179 U/l (reference range, 40–115 U/l) 4 years ago during a routine medical check-up in the USA. He had no history suggestive of PDB and also had no known family history of bone disease. Examination findings were not remarkable except for a relatively large head. A repeat ALP in our centre was 902 U/l (reference range, 40–120 U/l). Cranial CT scan showed diffuse cranial vault thickening consistent with Paget's disease which was confirmed by Tc-99m hydroxymethylene diphosphonate. He was placed on 40 mg alendronate tablets daily for 6 months. The patient has remained asymptomatic and has been in continuing biochemical remission during the 3-year follow-up period. The most recent ALP result is 88 U/l (reference range, 30–132 U/l) in April 2015. Learning points Serum total alkaline phosphatase remains a sensitive marker of bone turnover and an isolated increase above the upper limit of normal warrants more intense scrutiny in form of investigations targeted at excluding PD. Paget's disease is very rare but can occur in the Africans as seen in this Nigerian man and most patients are asymptomatic. Asymptomatic patients can benefit from treatment if disease is active, polyostotic or the lesions are located in bones with future risk of complications such as long bones, vertebrae and skull. Bisphosphonates are still the mainstay of treatment and alendronate is a useful therapeutic option for treatment.

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Paget ‘s Disease: Not So Typical for Atypical Femur Fracture

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.426 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A209-A210

Author(s):

Ashima Mittal ◽

Murray B Gordon

Keyword(s):

Task Force ◽

Paget’S Disease ◽

Distal Tibia ◽

Elevated Serum ◽

Hip Osteoarthritis ◽

Paget's Disease ◽

Cortical Thickening ◽

Task Force Report ◽

Femur Fractures ◽

History Of

Abstract Introduction: Atypical femur fractures (AFF) are reported in patients taking prolonged bisphosphonate therapy, but Paget’s disease (PD) has been rarely reported as a cause of AFF. Case: 71-year-old female with past medical history of right hip osteoarthritis, seizure disorder, hypertension, and Hashimoto’s thyroiditis presented with persistent right hip pain. There was no history of trauma or fall. She had otosclerosis with bilateral hearing loss and bilateral stapedectomies. Her medications were primidone, levothyroxine, lisinopril-hydrochlorothiazide, and vitamin D. She did not smoke tobacco or drink alcohol. She had elevated serum alkaline phosphatase of 300 U/L (35–104). The X-ray of the skull was negative for any cortical thickening. CT of right femur revealed cortical thickening and coarsening of trabeculae of the proximal right femur consistent with PD and incomplete atypical subtrochanteric proximal fracture. Urine NTx 303 BCE/mM Cr (<89). Bone scan showed uptake in the R proximal femur, L distal tibia, and L3-L4 vertebral bodies suspicious of PD. DXA showed osteopenia. She was given zoledronic acid 5 mg IV. Discussion: PD leads to an increased incidence of fractures particularly of the lower extremities with most fractures transverse in nature. Non-union is not uncommon1. Stress fractures in PD are caused by disorganized bone remodeling due to excessive breakdown and formation of bone. Our patient met the major criteria for AFF as per the ASBMR 2010 task force report but there was no exposure to bisphosphonates2. The ASBMR task force recommended that bisphosphonates should be discontinued in patients with bisphosphonate-associated AFF due to their severely suppressed bone turnover status. On the other hand, the AFF in patients with PD may heal in response to bisphosphonate treatment. References: 1. Singer FR. Bone Quality in Paget’s Disease of Bone. Curr Osteoporos Rep. 2016;14(2):39–42. DOI:10.1007/s11914-016-0303-62. Shane E, Burr D, Ebeling PR, Abrahamsen B, Adler RA et.al. Atypical subtrochanteric and diaphyseal femoral fractures: report of a task force of the American Society for Bone and Mineral Research. J Bone Miner Res. 2010; 25:2267–2294.

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