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of or relating to or resembling a cyst
of or relating to a normal cyst (as the gallbladder or urinary bladder)
inflammation of a bone as a consequence of infection or trauma or degeneration

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胞嚢(ほうのう)性の

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/10/06 22:47:20」(JST)

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Osteitis fibrosa cystica /ˌɒstiːˈaɪtɨs faɪˈbroʊsə ˈsɪstɨkə/,^[1] abbreviated OFC, and also known as osteitis fibrosa, osteodystrophia fibrosa, Von Recklinghausen's Disease of Bone, not to be confused with Von Recklinghausen's disease (neurofibromatosis type I). Osteitis Fibrosa Cystica is a skeletal disorder caused by a surplus of parathyroid hormone from over-active parathyroid glands. This surplus stimulates the activity of osteoclasts, cells that break down bone, in a process known as osteoclastic bone resorption. The over-activity of the parathyroid glands (hyperparathyroidism) can be triggered by parathyroid adenoma, hereditary factors, parathyroid carcinoma, or renal osteodystrophy. Osteoclastic bone resorption releases minerals, including calcium, from the bone into the bloodstream. In addition to elevated blood calcium levels, over-activity of this process results in a loss of bone mass, a weakening of the bones as their calcified supporting structures are replaced with fibrous tissue (peritrabecular fibrosis), and the formation of cyst-like brown tumors in and around the bone. The symptoms of the disease are the consequences of both the general softening of the bones and the excess calcium in the blood, and include bone fractures, kidney stones, nausea, moth-eaten appearance in the bones, appetite loss, and weight loss.

First described in the nineteenth century, OFC is currently detected through a combination of blood testing, X-rays, and tissue sampling. Before 1950, around half of those diagnosed with hyperparathyroidism in the United States saw it progress to OFC, but with early identification techniques and improved treatment methods, instances of OFC in developed countries are increasingly rare. Where treatment is required, it normally involves addressing the underlying hyperparathyroidism before commencing long-term treatment for OFC—depending on its cause and severity, this can range from hydration and exercise to surgical intervention.

Classification[edit]

Osteitis fibrosa cystica is defined as the classic skeletal manifestation of advanced hyperparathyroidism. Under the ICD-10 classification system, established by the World Health Organization, OFC is listed under category E21.0, primary hyperparathyroidism.^[2]

Signs and symptoms[edit]

The major symptoms of OFC are bone pain or tenderness, bone fractures, and skeletal deformities such as bowing of the bones. The underlying hyperparathyroidism may cause kidney stones, nausea, constipation, fatigue and weakness. X-rays may indicate thin bones, fractures, bowing, and cysts. Fractures are most commonly localized in the arms, legs, or spine.^[3]^[4]

The addition of weight loss, appetite loss, vomiting, polyuria, and polydipsia to the aforementioned symptoms may indicate that OFC is the result of parathyroid carcinoma.^[5] Parathyroid carcinoma, an uncommon cancer of the parathyroid glands, is generally indicated by serum calcium levels higher than usual, even in comparison to the high serum calcium levels that OFC generally presents with. Symptoms are also often more severe.^[6] Generally, the presence of a palpable neck mass is also indicative of the cancer, occurring in approximately 50% of sufferers, but virtually nonexistent in individuals with OFC with a different origin.^[7]

Causes[edit]

Diagram showing position of the parathyroid glands beside the thyroid

Osteitis fibrosa cystica is the result of unchecked hyperparathyroidism, or the overactivity of the parathyroid glands, which results in an overproduction of parathyroid hormone (PTH). PTH causes the release of calcium from the bones into the blood, and the reabsorption of calcium in the kidney. Thus, excess PTH in hyperparathyroidism causes elevated blood calcium levels, or hypercalcemia.^[8] There are four major causes of primary hyperparathyroidism that result in OFC:

Parathyroid Adenoma

The vast majority of cases of hyperparathyroidism are the result of the random formation of benign, but metabolically active, parathyroid adenoma swellings. These instances comprise approximately 80–85% of all documented cases of hyperparathyroidism.^[9]

Hereditary factors

Approximately 1 in 10 documented cases of hyperparathyroidism are a result of hereditary factors. Disorders such as familial hyperparathyroidism, multiple endocrine neoplasia type 1 (MEN Type 1) and hyperparathyroidism-jaw tumor syndrome can, if left unchecked, result in OFC.^[9] MEN Type 1 is an autosomal dominant disorder and the most common hereditary form of hyperparathyroidism, affecting about 95% of genetic cases of OFC, and also tends to affect younger patients than other forms. Major mutations which can lead to hyperparathyroidism generally involve the parathyroid hormone receptor, G proteins, or adenylate cyclase.^[10]^[11] Certain genetic mutations have been linked to a higher rate of parathyroid carcinoma occurrence, specifically mutations to the gene HRPT2, which codes for the protein parafibromin.^[12]

Parathyroid carcinoma

Parathyroid carcinoma (cancer of the parathyroid gland) is the rarest cause of OFC, accounting for about 0.5% of all cases of hyperparathyroidism. OFC onset by parathyroid carcinoma is difficult to diagnose.^[9]

Renal complications

OFC is a common presentation of renal osteodystrophy, which is a term used to refer to the skeletal complications of end stage renal disease (ESRD). OFC occurs in approximately 50% of patients with ESRD.^[13] ESRD occurs when the kidneys fail to produce calcitriol, a form of Vitamin D, which assists in the absorption of calcium into the bones. When calcitriol levels decrease, parathyroid hormone levels increase, halting the storage of calcium, and instead triggering its removal from the bones.^[14]

Pathophysiology[edit]

The effects of OFC on bone are largely dependent on the duration of the disease and the level of parathyroid hormone (PTH) produced.^[15] PTH is responsible for maintaining a homeostatic calcium concentration in the blood. It activates the parathyroid-hormone related protein receptor located on osteoblasts and osteocytes, both of which are responsible for the building and calcification of bone. Abnormalities affecting the parathyroid glands cause a surplus of PTH, which, in turn, increases the activity and frequency of osteoblasts and osteocytes.^[16] Increased PTH levels trigger the release of stored calcium through the dissolution of old bone, as well as the conservation of serum calcium through a cessation in the production of new bone.^[17]^[18]

Generally, the first bones to be affected are the fingers, facial bones, ribs, and pelvis.^[19]^[20] Long bones, which are longer than they are wide, are also among the first affected.^[20] As the disease progresses, any bone may be affected.^[15]

X-ray of the hands showing brown tumors in the long bones of the fingers

Diagnosis[edit]

OFC may be diagnosed using a variety of techniques. Muscles in patients afflicted with OFC can either appear unaffected or "bulked up." If muscular symptoms appear upon the onset of hyperparathyroidism, they are generally sluggish contraction and relaxation of the muscles.^[21] Deviation of the trachea (a condition in which the trachea shifts from its position at the midline of the neck), in conjunction with other known symptoms of OFC can point to a diagnosis of parathyroid carcinoma.^[20]

Blood tests on patients with OFC generally show high levels of calcium (normal levels are considered to range between 8.5 and 10.2 mg/dL,^[22] parathyroid hormone (levels generally above 250 pg/mL, as opposed to the "normal" upper-range value of 65 pg/mL^[23]),^[24] and alkaline phosphatase^[3](normal range is 20 to 140 IU/L^[25]).

X-rays may also be used to diagnose the disease. Usually, these X-rays will show extremely thin bones, which are often bowed or fractured. However, such symptoms are also associated with other bone diseases, such as osteopenia or osteoporosis.^[26] Generally, the first bones to show symptoms via X-ray are the fingers.^[19] Furthermore, brown tumors, especially when manifested on facial bones, can be misdiagnosed as cancerous.^[26] Radiographs distinctly show bone resorption and X-rays of the skull may depict an image often described as "ground glass" or "salt and pepper".^[27]^[28] Dental X-rays may also be abnormal.^[3]

Histology of bone showing osteitis fibrosa cystica. (Fibrosis and intratrabecular tunnels are seen).

Cysts may be lined by osteoclasts and sometimes blood pigments, which lend to the notion of "brown tumors." Such cysts can be identified with nuclear imaging combined with specific tracers, such as sestamibi.^[4] Identification of muscular degeneration or lack of reflex can occur through clinical testing of deep tendon reflexes, or via photomotogram (an achilles tendon reflex test).^[21]^[29]

Fine needle aspiration (FNA) can be used to biopsy bone lesions, once found on an X-ray or other scan. Such tests can be vital in diagnosis and can also prevent unnecessary treatment and invasive surgery.^[30] Conversely, FNA biopsy of tumors of the parathyroid gland is not recommended for diagnosing parathyroid carcinoma and may in fact be harmful, as the needle can puncture the tumor, leading to dissemination and the possible spread of cancerous cells.^[31]

The brown tumors commonly associated with OFC display many of the same characteristics of osteoclasts.^[20] These cells are characteristically benign, feature a dense, granular cytoplasm, and a nucleus that tends to be ovular in shape, enclosing comparatively fine chromatin. Nucleoli also tend to be smaller than average.^[15]

Comparison of bone pathology
view talk edit Condition	Calcium	Phosphate	Alkaline phosphatase	Parathyroid hormone	Comments
Osteoporosis	unaffected	unaffected	normal	unaffected	decreased bone mass
Osteopetrosis	unaffected	unaffected	elevated	unaffected	thick dense bones also known as marble bone
Osteomalacia and rickets	decreased	decreased	elevated	elevated	soft bones
Osteitis fibrosa cystica	elevated	decreased	elevated	elevated	brown tumors
Paget's disease of bone	unaffected	unaffected	variable (depending on stage of disease)	unaffected	abnormal bone architecture

Management[edit]

Medical[edit]

Medical management of OFC consists of Vitamin D treatment, generally alfacalcidol or calcitriol, delivered intravenously. Studies have shown that in cases of OFC caused by either end-stage renal disease or primary hyperparathyoidism, this method is successful not only in treating underlying hyperparathyoidism, but also in causing the regression of brown tumors and other symptoms of OFC.^[32]

Surgery[edit]

In especially severe cases of OFC, parathyroidectomy, or the full removal of the parathyroid glands, is the chosen route of treatment. Parathyroidectomy has been shown to result in the reversal of bone resorption and the complete regression of brown tumors.^[32] In situations where parathyroid carcinoma is present, surgery to remove the tumors has also led to the regression of hyperparathyroidism as well as the symptoms of OFC.^[33]

Bone transplants have proven successful in filling the lesions caused by OFC. 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.^[34]

Prognosis[edit]

Almost all who undergo parathyroidectomy experience increased bone density and repair of the skeleton within weeks. Additionally, patients with OFC who have undergone parathyroidectomy begin to show regression of brown tumors within six months.^[35]^[36] Following parathyroidectomy, hypocalcaemia is common. This results from a combination of suppressed parathyroid glands due to prolonged hypercalcaemia, as well as the need for calcium and phosphate in the mineralization of new bone.^[37]

Thirty percent of patients with OFC caused by parathyroid carcinoma who undergo surgery see a local recurrence of symptoms. The post-surgical survival rate hovers around seven years, while patients who do not undergo surgery have a survival rate of around five years.^[20]

Epidemiology[edit]

Osteitis fibrosa cystica has long been a rare disease.^[38] Today, it appears in only 2% of individuals diagnosed with primary hyperparathyroidism, which accounts for 90% of instances of the disease.^[20] Primary hyperparathyroidism is three times as common in individuals with diabetes mellitus.^[39]

The hospitalization rate for hyperparathyroidism in the United States in 1999 was 8.0 out of 100,000.^[40] The disease has a definite tendency to affect younger individuals, typically appearing before the age of 40, with a study in 1922 reporting that 70% of cases display symptoms before the age of 20, and 85% before 35.^[41] Primary hyperparathyoidism, as well as OFC, is more common in Asiatic countries.^[19] Before treatment for hyperparathyroidism improved in the 1950s, half of those diagnosed with hyperparathyroidism saw it progress into OFC.^[3]

Rates of OFC increase alongside cases of unchecked primary hyperparathyroidism. In developing countries, such as India, rates of disease as well as case reports often mirror those published in past decades in the developed world.^[42]^[43]

The other 10% of cases are primarily caused by primary hyperplasia, or an increase of the number of cells. Parathyroid carcinoma accounts for less than 1% of all cases,^[20] occurring most frequently in individuals around 50 years of age (in stark contrast to OFC as a result of primary hyperparathyroidism) and showing no gender preference.^[20] Approximately 95% of hyperparatyhroidism caused by genetic factors is attributed to MEN type 1. This mutation also tends to affect younger individuals.^[9]

History[edit]

Friedrich Daniel von Recklinghausen, who is creited, along with Gerhard Engel, in naming OFC

The condition was first described by Gerhard Engel in 1864 and Friedrich Daniel von Recklinghausen in 1890, though William Hunter, who died in 1783, is credited with finding the first example of the disease.^[44]^[45] "von Recklinghausen's disease" (without the qualification "of bone") is a completely unrelated disorder, nowadays termed neurofibromatosis.^[8] In 1884, Davies Colley delivered a presentation to the Pathological Society of London that detailed the manifestation of hyperparathyroidism into a brown tumor of the mandible, as well as the histological makeup of the tumor.^[46]

The discovery and subsequent description of the parathyroid glands is credited to Ivar Sandstrom, though his publication, On a New Gland in Man and Several Mammals-Glandulae Parathyroideae, received little attention. Gustaf Retzius and Eugene Gley compounded his research, the latter credited with the discovery of the function of the parathyroid glands.^[46] This research cumulated in the first surgical removal of a parathyroid tumor by Felix Mandel in 1925. A 2.5 × 1.5-inch (64 × 38 mm) tumor was removed from the thyroid artery of a man suffering from advanced OFC. The patient's symptoms disappeared, only to return in approximately six years as a result of renal stones that were diagnosed only after the patient had died. In 1932, blood tests on a female patient suffering from renal stone-based OFC revealed extremely high blood calcium levels. Fuller Albright diagnosed and treated the woman, who suffered from a large tumor of the neck as well as renal stones.^[8]

The first published literature to describe a brown tumor (which was linked to OFC) was published in 1953, though clinical reports from before 1953 do draw a correlation between the disease and tumors previous to the publication.^[47]

The advent of the multichannel autoanalyzer in the 1960s and 70s led to an increase in early diagnosis of primary hyperparathyroidism. This increase led to a sharp decline in the prolonged manifestation of the disease, leading to a drop in the number of cases of OFC due to the early detection of hyperparathyroidism.^[48] Before this invention, the diagnosis of primary hyperparathyroidism was generally prolonged until the emergence of severe manifestations, such as OFC.^[49]

References[edit]

^ US dict: ŏs′·tē·īt′·əs fī·brō′·sə sĭs′·tə·kə
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Patton, Kevin T.; Thibodeau, Gary A. (1996). Anthony's Textbook of Anatomy & Physiology. St. Louis: Mosby. ISBN 0-8151-8861-7

UpToDate Contents

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1. 原発性副甲状腺機能亢進症の臨床症状 clinical manifestations of primary hyperparathyroidism
2. 腎性骨異栄養症の骨生検および診断 bone biopsy and the diagnosis of renal osteodystrophy
3. 小児における、慢性腎臓病に伴う骨ミネラル代謝異常（CKD-MBD） pediatric chronic kidney disease mineral and bone disorder ckd mbd
4. 慢性腎臓病患者における骨粗鬆症：診断、評価およびマネージメント osteoporosis in patients with chronic kidney disease diagnosis evaluation and management
5. 末期腎不全における副甲状腺摘出の適応 indications for parathyroidectomy in end stage renal disease

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Santiago Chinchilla A, Ramos Font C, Muros de Fuentes MA, Navarro-Pelayo Lainez M, Palacios Gerona H, Moreno Caballero M, Llamas Elvira JM.SourceServicio de Medicina Nuclear, Hospital Universitario Virgen de las Nieves, Granada, Spain. aliciasantiagochinchilla@gmail.com
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PMID 21342724

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O-228 REMARKABLE BUT DISORDERLY RECOVERY OF OSTEITIS FIBROSA CYSTICA FOLLOWING PARATHYROIDECTOMY.

AGARWAL G.,MISHRA S. K.,MITHAL A.,ARYA V.
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★リンクテーブル★

リンク元	「慢性腎不全」「嚢胞性線維性骨炎」
拡張検索	「osteitis fibrosa cystica generalizata」
関連記事	「cystic」

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Osteitis fibrosa cystica
	Classification and external resources
	; Osteitis fibrosa cystica of the tibia. Arrows point to the brown tumors which are typically present in bones of people with OFC.
ICD-10	E21.0
ICD-9	252.01
OMIM	603233
DiseasesDB	30721
MedlinePlus	001252
MeSH	D010002

　　[★]

英: chronic renal failure, CRF
関: 腎不全、急性腎不全

概念

数ヶ月から数十年に及ぶ緩徐な経過で腎機能障害が進行し、末期腎不全に至る不可逆的疾患。

濾過される原尿の量が減少するので、血液中の不要物を排泄したり、電解質・水分の調節能が低下する。

定義 (日本人腎臓学会 CKD診断ガイド)

1.-2.のいずれか、または両方が3ヶ月以上持続する。
1. 尿異常、画像診断、血液、病理で腎障害の存在が明らか。特に尿蛋白が存在
2. GFR < 60 ml/min/1.73m³

病態

水、Naの貯留→循環血液量↑→高血圧、肺水腫、心不全
電解質の異常→K↑(高カリウム血症), P↑, Mg↑，Ca↓

カリウム↑：GFR低下によりカリウム分泌が不十分となる。
リン↑：近位尿細管での排泄障害(YN.E-25)？、酸分泌障害？
カルシウム↓：尿細管機能低下？？？により、PTHの作用を受けて遠位尿細管で再吸収されるべきカルシウムが排泄される。

尿毒素の貯留→尿毒症
水素イオンの貯留→代謝性アシドーシス
内分泌の異常

エリスロポエチン低下　→　腎性貧血
活性化ビタミンD₃が産生されない　→　低カルシウム血症　→　副甲状腺ホルモン↑　→　腎性骨異栄養症
レニン高値　→　高血圧
インスリン代謝の低下 (イン不倫は腎臓で分解される)

細胞性免疫能の低下　→　結核のリスク(FSRFでは正常人比10倍)
出血傾向　→　尿毒症により凝固系機能低下
低カルシウム血症：

尿細管での再吸収不全、1,25-dihydroxy-vitamin D3不足により腸管からのCa吸収低下、無機リン増加によりカルシウムの減少([Ca][P]積が一定になるように保たれているらしい)、骨のPTHに対する感受性低下？？？(なぜ？)