Presentation
Three cases of particular interest are presented in detail. In one, both pituitary gigantism (in addition to severe deformity of facial bones) and hyperthyroidism were present. [amjmed.com]
Degree of confidence Plain radiographs are highly specific when characteristic features are present in a lesion. [emedicine.medscape.com]
The patient to be presented has been under constant medical scrutiny since the onset of symptoms at the age of three, and [annals.org]
It is clinically characterized by a triad of Pigmentation ( café-au-lait macule), polyostotic/monostotic fibrous dysplasia (FD) and precocious puberty (PP). [1], [2] A full presentation is rare, and it is more common in females [3], [4], [5] who present [ijpd.in]
If Albright's syndrome is present, an operation on the parathyroids can be avoided, since these glands are not involved in... Bibliography 1. [acpjournals.org]
Entire Body System
- Falling
Her disease process has been characterized by early breast development at 3 years of age, menarche at the age of 6, and a pathologic subtrochanteric fracture after a trivial fall at age 8. She now complains of a limp and pain after long walks. [gait.aidi.udel.edu]
Über einen Fall von Albrightschem Syndrom. Z. Orthop. 80, 308–319 (1951). Google Scholar McCune, D. J., and H. [link.springer.com]
- Dentist
Dentists should familiarize themselves with this disease, as it can be adequately treated by conservative surgical and prosthetic techniques. To read this article in full you will need to make a payment References Lichtenstein L. [oooojournal.net]
Gastrointestinal
- Abdominal Pain
Abdominal ultrasound or MRI was performed in additional three of the seven Finnish patients for other reasons, for example, due to unspecific abdominal pain, and they all showed normal findings. [frontiersin.org]
[…] unilateral and may be overlying involved bone) Not dependent on the hypothalamic/anterior pituitary axis Endocrine abnormalities: hyperthyroidism (M=F), hyperparathyroidism, hypophosphatemia, osteoporosis, kidney stones, anorexia, nausea, vomiting, abdominal [orthopaedicsone.com]
Ears
- Hearing Problem
Despite the extended fibrous dysplasia, the boy faced no vision or hearing problems and experienced no fractures or any other kinetic problem. Four years after diagnosis the boy displays an uneventful course. REFERENCES 1. [hormones.gr]
- Tinnitus
GHH Micro – 8 M 35 33 1.72 M – – Hyposmia Unilateral GHH, PH Macro – 9 M 46 46 3.05 P + + – – GHH Micro HTN, DM 10 M 43 26 2.72 P + – – GHH, PH Macro – 11 M 47 46 0.05 P + – Unilateral GHH Macro – 12 M 9 9 1.43 P + + – – GHH N/A – 13 M 22 22 2.05 P + Tinnitus [eje.bioscientifica.com]
Musculoskeletal
- Fracture
We hereby present the case of a 23-year-old male patient who is treated several times for recurrent pathological fractures of the femur at our center. [scirp.org]
Progressive deformity may be produced by malunion of specific fractures or gradual "bending" of the bone due to subclinical chronic insufficiency fractures. TREATMENT: Observation -- with prevention of deformity and fracture. [gait.aidi.udel.edu]
Fibrous dysplasia of the bone can involve one or several bones and is characterized by bone deformities, pain and fractures. [indianpediatrics.net]
Acromegaly, pathologic fractures, spinal instability, scoliosis, hyperthyroidism, hypophosphatemia, liver disease, obstructive sleep apnea, neonatal Cushing’s syndrome, neuropathy, hypertension and arrhythmia can also accompany MAS. [ai-online.info]
Your history reveals that 18 months ago, he sustained a midshaft fracture of the right tibia with minimal trauma. While under treatment in a long leg cast, he fractured his femur at the proximal edge of the cast. The fracture healed in a spica cast. [healio.com]
- Osteoporosis
[…] yes : flogosis => hyperemia => demineralization (this is not osteoporosis of disuse !) [ufrgs.br]
Primary and secondary osteoporosis, symptomatic Paget disease of bone, osteogenesis imperfecta both in children and adults and fibrous dysplasia are currently the major indications for bisphospho-nates. [indianpediatrics.net]
المحتويات Chapter 18 The Spectrum of Pediatric Osteoporosis 439 Chapter 19 Osteogenesis Imperfecta 511 Chapter 20 Sclerosing Bone Dysplasias 541 Chapter 21 Parathyroid Disorders 557 Chapter 22 Fibrous Dysplasia 589 Chapter 23 Nutritional Rickets 625 Chapter [books.google.com]
Bisfosfonatos: aplicaciones actuales en osteoporosis y cáncer. An Fac Med 1999; 60: 61-5. 26) Behrman RE, Liegman RM, Arvin AM. Nelson’s Textbook of Pediatrics. Philadelphia. WB Saunders, 15th ed. 1996, pág. 1584. [sisbib.unmsm.edu.pe]
[…] hypothalamic-pituitary-ovarian axis during early infancy in female patients with saltlosing 21-hydoxylase deficiency Maesaka H, Suwa S, Tachibana K, Katsumata N, Pediatr Res 1985 19 1258-1262 en Characteristics and bisphosphonate treatment of a patient with juvenile osteoporosis [eamas.net]
- Bone Pain
It is most common in older individuals, and may lead to bone pain, deformities, and fractures. Also called Paget's disease of bone. [kmle.co.kr]
Fibrous dysplasia of the bone can involve one or several bones and is characterized by bone deformities, pain and fractures. [indianpediatrics.net]
Bisphosphonates are helpful in relieving bone pain, but it is no longer believed that they prevent progression of the disease. [en.wikipedia.org]
Relapse of bone pain is more common. Stronger bisphosphonate medications such as zoledronic acid may be used in such cases and may be most effective in improving bone pain. [rarediseases.org]
These patients may experience severe bone and joint pain and painful calcifications in soft tissue and tendons. Serum phosphate levels rise in these patients as calcium levels drop, a potent stimulus for PTH production. [emedicine.medscape.com]
Neurologic
- Confusion
The first great advance toward clarifying this confusing situation was made when it was recognized that hyperparathyroidism was present in certain cases of so-called osteitis fibrosa. [pubs.rsna.org]
[…] echography or MRI > conventional X-rays (arthrography is no longer used) yes : subcutaneous edema and effusion/hypertrophy of synovial membrane => increased density of periarticular soft tissues; spindle, nodular, enlengthened morphology with net or confused [ufrgs.br]
Osteitis fibrosa cystica Osteitis fibrosa cystica ə, abbreviated OFC, also known as osteitis fibrosa, osteodystrophia fibrosa, and Von Recklinghausen`s disease of bone (not to be confused with Von Recklinghausen`s disease, neurofibromatosis type I), is [encyclo.co.uk]
McCune-Albright syndrome is most commonly confused with neurofibromatosis (NF), especially when a child presents with large café-au-lait macules (CALMs). [ijpd.in]
Workup
"Parathyroid Carcinoma: Differential Diagnoses & Workup". eMedicine. Archived from the original on 2009-05-27. Retrieved 2009-01-28. ^ a b Arabi, A. (2006). "Regression of Skeletal Manifestations of Hyperparathyroidism with Oral Vitamin D". [en.wikipedia.org]
Treatment
We analyze the difficulties associated with treating a patient with poor bone quality over several years, discuss our treatment options, review the literature for similar cases and look at what we could have done differently. [scirp.org]
Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout. [books.google.com]
Generally, no treatment is required in asymptomatic cases. [dovemed.com]
Our patient received external irradiation treatment and long-acting octreotide therapy (Sandostatin LAR). With this treatment, serum growth hormone and serum IGF-1 levels decreased markedly but not to normal range. [turkjem.org]
Treatment with medication is also of vast value. [eje.bioscientifica.com]
Prognosis
The prognosis is dependent upon the severity of the disorder Currently, there are no known methods to prevent Fibrous Dysplasia The types of Fibrous Dysplasia include: Monostotic Fibrous Dysplasia: When only a single bone is involved Polyostotic Fibrous [dovemed.com]
Diagnosis - McCune Albright syndrome Prognosis - McCune Albright syndrome The long-term outlook (prognosis) for people with McCune Albright syndrome (MAS) varies depending on the nature and severity of features in each affected person. [checkorphan.org]
Part I explores basic techniques to researching mccune-albright syndrome (e.g. finding guidelines on diagnosis, treatments, and prognosis), followed by a number of topics, including information on how to get in touch with organizations, associations, [amazon.in]
Prognosis Fibrous dysplasia has a good prognosis with low rates of malignant transformation. [eyewiki.aao.org]
A report showed that in 8 out of 11 instances where cavities caused by OFC were filled with transplanted bone, the lesion healed and the transplanted bone blended rapidly and seamlessly with the original bone.[37] Prognosis[edit] Almost all who undergo [en.wikipedia.org]
Etiology
It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Fibrous dysplasia represents about 2, 5% of all bone tumors and over 7% of all benign tumours. [journal-imab-bg.org]
Another author in 1938 Lichtenstein coined the term 'Fibrous Dysplasia of bone' and described it as a different entity of unknown etiology. [omicsonline.org]
Etiology of fibrous dysplasia and McCune-Albright syndrome. Int J Oral Maxillofac Surg 1999;28:366–371. 20. Zimmerman DC, Dahlin DC, Stafne EC. Fibrous dysplasia of the maxilla and mandible. Oral Surg Oral Med Oral Pathol 1958;11:55–68. 21. [synapse.koreamed.org]
Etiology The etiology of hyperparathyroidism in sporadic cases is unknown; however, hereditary aspects of the disease have been identified. Details of the disorders, related conditions, and treatment considerations are discussed below. [emedicine.medscape.com]
[…] possibly hypogonadotrophic hypogonadism[8] and soft tissue myxomas.[9] Association of multiple endocrinopathies is a rare occurrence, although gigantism and hyperthyroidism in one case of Albright syndrome was recorded by Firat and Stutzman.[5] The etiology [jpgmonline.com]
Epidemiology
Metyrapone may also be used for treatment. [12] Epidemiology [ edit ] McCune–Albright syndrome is estimated to occur at a frequency between 1 person in 100,000 to 1 person in 1,000,000 individuals worldwide. [15] Notable cases [ edit ] Mauricio Saravia [en.wikipedia.org]
Pathophysiology
Pathophysiology, evaluation, and treatment. J Bone Joint Surg [Am]. 2005;87:1848-64. Han I, Choi ES, Kim HS. Monostotic fibrous dysplasia of the proximal femur: natural history and predisposing factors for disease progression. [ijoro.org]
Surgical treatment of hyperparathyroidism requires a thorough understanding of the pathophysiology of the disease and the embryologic basis of parathyroid gland surgical anatomy. [emedicine.medscape.com]
"The Role of Type 1 and Type 2 5′-Deiodinase in the Pathophysiology of the 3,5,3′-Triiodothyronine Toxicosis of McCune-Albright Syndrome". The Journal of Clinical Endocrinology and Metabolism. 93 (6): 2383–2389. doi : 10.1210/jc.2007-2237. [en.wikipedia.org]
[…] of all orbital tumors (0.6-2%). [4] Fibrous dysplasia represents approximately 18% of all primary orbital bone tumors. [4] The majority of cases with orbital involvement have monostotic fibrous dysplasia of the frontal, sphenoid or ethmoid bones. [4] Pathophysiology [eyewiki.aao.org]
Prevention
The prognosis is dependent upon the severity of the disorder Currently, there are no known methods to prevent Fibrous Dysplasia The types of Fibrous Dysplasia include: Monostotic Fibrous Dysplasia: When only a single bone is involved Polyostotic Fibrous [dovemed.com]
Alendronate is at present indicated for use in prevention and treatment of osteoporosis and Paget’s disease. It should be used in conjunction with adequate amounts of vitamin D and calcium in the prevention of progressive loss of bone mass. [indianpediatrics.net]
Bisphosphonates are helpful in relieving bone pain, but it is no longer believed that they prevent progression of the disease. [en.wikipedia.org]
Albright like syndrome, Osteitis fibrosa disseminata, Polyostotic, fibrous dysplasia, Precocious puberty with polyostotic fibrosis and abnormal pigmentation, MAS, PFD, POFD Symptoms - McCune Albright syndrome Causes - McCune Albright syndrome Prevention [checkorphan.org]
No known prevention exists for fibrous dysplasia. [eyewiki.aao.org]