Discussion: A 25 year old male, presented in OPD with complain of pain at the middle of the front of both legs about 10 years. [ecommons.aku.edu]

An 11-year-old boy presented with the chief complaint of difficulty looking straight with both eyes. [ajnr.org]

Bullough, MB, ChB, presents a unique, lavishly illustrated account of the pathology of arthritic disorders, metabolic disturbances, and soft tissue and bone tumors. [books.google.com]

some skull enlargement, kyphosis and anterior bowing of both femurs is present. [healio.com]

The patients affected with JPD present an altered bone turnover, therefore, show a phenotype characterized by progressive bone deformities, fractures, and short stature. [ncbi.nlm.nih.gov]

• Failure to Thrive

  She presented with fractures during the first year‐of‐life followed by bone deformities, delayed development, failure‐to‐thrive, and pneumonias. [ingentaconnect.com]

  Clinical manifestations present from early infancy and include: skeletal deformity and failure to thrive followed by skull enlargement, difficulty in walking, progressive sensorineural deafness (due to cochlear involvement), kyphosis and acetabular protrusion [iofbonehealth.org]

  Presentation The patient with Juvenile Paget disease present in early childhood Bone pain and bone deformities [Bowed limbs] Failure to thrive. Dwarfism. Progressive enlargement of the head. Difficulty walking. Pigeon chest deformity. [boneandspine.com]

  Failure to thrive. Dwarfism. Progressive enlargement of the head. Bowed limbs. Difficulty walking. Pigeon breast deformity. Muscular weakness. Premature shedding of the teeth. [ 3 ] Associated diseases Retinal degeneration (± angioid streaks). [patient.info]

• Hearing Impairment

  impairment Deafness Hearing defect [ more ] 0000365 Hypertension 0000822 Optic atrophy 0000648 Pectus carinatum Pigeon chest 0000768 5%-29% of people have these symptoms Macular scar 0200056 Melanocytic nevus Beauty mark 0000995 Motor delay 0001270 Retinopathy [rarediseases.info.nih.gov]

  Hearing impairment and tooth loss is common. Apart from juvenile-onset Paget's disease (PDB5), the condition is inherited as an autosomal dominant trait. [genome.jp]

• Bone Disorder

  Overview A rare genetic bone disorder involving abnormal loss of bone mineralization and remineralization, broadened bone shafts and high levels of alkaline phosphatase in the blood. [checkorphan.org]

  Paget’s disease, also known as osteitis deformans, is the second most common bone disorder in the United States (behind osteoporosis). [spineuniverse.com]

  disorder, characterized by excessive bone resorption and bone formation. [thieme-connect.com]

  Paget's disease of bone is an uncommon, chronic bone disorder that occurs in only about one percent of people in the United States and slightly more often in men than in women (three to two). [rheumatology.org]

  Paget's disease of bone is a frequent bone disorder which usually starts after the age of 40 and which is characterized by bone pain and deformities. [clinicaltrials.gov]

• Arthralgia

  […] diagnosis of the disease; essentially Behçet's disease does not always follow the International Study Group guidelines and so a high degree of suspicion for a patient who presents having any number of the following findings is necessary: arthritis / arthralgia [en.wikipedia.org]

• Headache

  Headaches In addition there may be headaches if the skull is involved due to pressure on nerves and blood vessels. Some patients also develop a larger size of the head. [news-medical.net]

  Headaches and hearing loss may occur when Paget’ disease affects the skull. The diagnosis is made by correlating the clinical findings with the blood level of alkaline phosphatase and in many cases the X-ray findings. [rheum-care.com]

  Headache, loss of vision, and hearing loss can occur when bones of the skull are affected. With very widespread Paget's disease, it is possible to develop congestive heart failure due to an increased workload on the heart. [medicinenet.com]

  An overgrowth of bone in the skull can cause hearing loss or headaches. Spine. If your spine is affected, nerve roots can become compressed. This can cause pain, tingling and numbness in an arm or leg. Leg. [mayoclinic.org]

  Having Paget’s in the skull may cause an enlarged head, headaches and hearing loss. In severe Paget’s, heart, kidney and nervous systems may occur. [arthritis.org]

(See Workup .) Medical therapy is principally with bisphosphonates; surgical therapy may be indicated. (See Treatment and Medication .) Pathophysiology Three phases of Paget disease have been described: lytic, mixed lytic and blastic, and sclerotic. [emedicine.medscape.com]

Recombinant OPG would represent a replacement treatment, but it is unavailable for clinical use. It seems that life-long treatment with anti-resorptives is required, since the disease is reactivated after treatment discontinuation. [ncbi.nlm.nih.gov]

Her bone disease remained active despite treatment. [academic.oup.com]

Treatment - Juvenile Paget disease Primary treatment consists of drug therapy and includes one of the following: * Calcitonin (subcutaneously or intranasally) is used to retard bone resorption (which relieves bone lesions) and reduce levels of serum alkaline [checkorphan.org]

Make the best clinical decisions with an enhanced emphasis on evidence-based practice and expert opinions on treatment strategies. Zero in on the most relevant and useful references with the aid of a more focused, concise bibliography. [books.google.com]

Paget’s Disease of Bone: Prognosis and Complications Abstract Introduction Prognosis of Paget’s Disease of Bone Bone Complications Secondary Osteoarthritis Neoplastic Degeneration Neurological Complications Cardiovascular Complications Other Complications [elsevier.com]

Prognosis - Juvenile Paget disease Not supplied. [checkorphan.org]

Deafness Osteoporosis Delayed motor development Prognosis Severity of the disease increases as the child progresses towards adolescence. Some patients will survive beyond the age of 50 years. [boneandspine.com]

Treatment and prognosis Traditionally, treatment has been a mastectomy with nodal dissection. However, with MR, breast-conserving therapy is more frequently being used. [radiopaedia.org]

[…] lead to osteopetrosis. [ 5, 9 ] Recombinant versions of osteoprotegerin are under research. [ 10 ] Complications Bone pain Pathological fractures Kyphoscoliosis Acetabular protrusion Sensorineural deafness Osteoporosis Delayed motor development [ 11 ] Prognosis [patient.info]

AO and auricular calcification refer interchangeably to rigid pinnae, sparing the ear lobe, from various etiologies. [ncbi.nlm.nih.gov]

Viral Etiology of Paget’s Disease of Bone Abstract Introduction Paramyxo-Viral Etiology Viral and Genetic Interactions References Chapter 3. [elsevier.com]

Other etiologies have also been proposed, such as neoplastic, vascular, endocrinologic, immunologic, traumatic, and hereditary. [radiologykey.com]

The condition was initially named by Paget "osteitis deformans", implying an inflammatory etiology. The term "osteodystrophica deformans" is now preferred. [radiopaedia.org]

Skowronska-Jozwiak E, Lorenc RS ; Metabolic bone disease in children : etiology and treatment options. Treat Endocrinol. 20065(5):297-318. [patient.info]

Clinical Perspectives of Paget’s Disease of Bone Abstract Introduction Natural History Clinical Features and Complications Diagnosis Pathology Biochemistry Epidemiology Secular Change in Paget’s Disease References Chapter 2. [elsevier.com]

[…] locus 8q24 and results from mutations or deletions in the TNFRSF11B gene. [ 3 ] These lead to a deficiency of osteoprotegerin, a member of the TNF-receptor superfamily. [ 4 ] Osteoprotegerin inhibits osteoclast differentiation and bone resorption. [ 5 ] Epidemiology [patient.info]

Epidemiological aspects of Paget's disease: family history and relationship to other medical conditions. Semin Arthritis Rheum. 1994;23:222-5. [ Links ] 36. Lever JH. [scielo.org.co]

Epidemiology of Paget's disease of bone: a systematic review and meta-analysis of secular changes. Bone. 2... [5minuteconsult.com]

[…] biochemistry of nutrition 180 Nutritional disorders and their management 200 Clinical biochemistry of the gastrointestinal tract 214 Assessment of hepatic function and investigation of jaundice 231 Acute and chronic liver disease 250 Glucose metabolism and the pathophysiology [books.google.com]

Rare in African Americans and Asians Incidence and prevalence have decreased in recent years; likely due to genetic and environmental factors Etiology and Pathophysiology Unknown, although several theories proposed: Slow viral infection (controversial [unboundmedicine.com]

Advances in Pathobiology and Management of Paget’s Disease of Bone presents an essential collection of up-to-date knowledge about the pathophysiology, genetics, and treatment of Paget’s disease. [elsevier.com]

Relative or absolute secondary increases in PTH may occur in 12–18% of moderately active PDB in the setting of a skeletal metabolic balance favoring bone formation. (32,33) This may be of pathophysiological importance, because higher concentrations of [onlinelibrary.wiley.com]

Bisphosphonate associated osteonecrosis of the jaw: an update on pathophysiology, risk factors, and treatment. Int J Dent. 2014. 2014:471035. [Medline]. [Full Text]. [emedicine.medscape.com]

Calcitonin did not prevent the occurrence or progression of angioid streaks, and Argon laser photocoagulation of complicating subretinal neovascular membranes failed to prevent visual loss. [healio.com]

NNO is a night to celebrate safety and crime prevention successes – and to expand and strengthen programs for the next 364 days,” he said. [taku105.com]

Prevention - Juvenile Paget disease Not supplied. Diagnosis - Juvenile Paget disease Not supplied. Prognosis - Juvenile Paget disease Not supplied. [checkorphan.org]

Treatment is primarily preventive physical exercise and proper nutrition. [medicineonline.es]

To have strong bones when you are young, and to prevent bone loss when you are older, you need to get enough calcium, vitamin D, and exercise. You should also avoid smoking and drinking too much alcohol. Bone diseases can make bones easy to break. [medlineplus.gov]