Skeletal Blood Flow in Paget's Disease of Bone and its Response to Calcitonin Therapy

1978 ◽  
Vol 54 (1) ◽  
pp. 69-74 ◽  
Author(s):  
R. Wootton ◽  
J. Reeve ◽  
E. Spellacy ◽  
M. Tellez-Yudilevich
Keyword(s):  
Alkaline Phosphatase ◽  
Blood Flow ◽  
Serum Alkaline Phosphatase ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Short Term ◽  
Urinary Hydroxyproline ◽  
Rapid Fall ◽  
Hydroxyproline Excretion

1. Blood flow to the skeleton was measured by the 18F clearance method of Wootton, Reeve & Veall (1976) in 24 patients with untreated Paget's disease. In every patient but one, resting skeletal blood flow was increased. There was a significant positive correlation between skeletal blood flow and serum alkaline phosphatase and between skeletal blood flow and urinary total hydroxyproline excretion. 2. Fourteen patients were re-studied after they had received short-term (7 days or less) or long-term (7 weeks or more) calcitonin. Skeletal blood flow, alkaline phosphatase and urinary hydroxyproline excretion fell towards normal in every case. There was some evidence from the short-term studies that calcitonin produced a more rapid fall in skeletal blood flow than in alkaline phosphatase. 3. Glomerular filtration rate appeared to increase transiently in response to calcitonin.

Paget’s disease: clinical update

2011 ◽  
Vol 152 (33) ◽  
pp. 1337-1346
Author(s):  
Judit Donáth ◽  
Gyula Poór
Keyword(s):  
Alkaline Phosphatase ◽  
Serum Alkaline Phosphatase ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Etiologic Role ◽  
Osteoblast Activity ◽  
Chronic Disorder ◽  
Abnormal Increase ◽  
Osteoclast Function ◽  
High Level

Paget’s disease is a chronic disorder of bone remodeling, characterized by an abnormal increase of osteoclast and, hence, osteoblast activity. The imbalance of bone turnover results in the formation of unhealthy and fragile bone. It also leads to impairment of adjacent joints and to a risk of various complications. Current research focuses on the elucidation of the etiologic role viral infection and predisposing genetic factors. Paget’s disease is commonly discovered by chance; its suspicion is raised either by high level of alkaline phosphatase or by the X-ray of the pathological bone. Bisphosphonates have proven to be effective in controlling disease activity because they inhibit osteoclast function. Their use is recommended when bone-derived serum alkaline phosphatase is high and/or when disease localizations are highly suspected for the development of complications. Orv. Hetil., 2011, 152, 1337–1346.

Effect of Salmon Calcitonin on Cardiac Output, Oxygen Transport and Bone Turnover in Patients with Paget's Disease

1975 ◽  
Vol 48 (6) ◽  
pp. 537-540 ◽  
Author(s):  
W. A. Crosbie ◽  
S. M. Mohamedally ◽  
N. J. Y. Woodhouse
Keyword(s):  
Cardiac Output ◽  
Pain Relief ◽  
Oxygen Transport ◽  
Salmon Calcitonin ◽  
Bone Pain ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Urinary Hydroxyproline ◽  
The Mean ◽  
Hydroxyproline Excretion

1. Twelve patients with symptomatic Paget's disease were studied before starting treatment with salmon calcitonin (12.5 μg) subcutaneously twice daily. Eleven of them were studied again after 3 months on this therapy. 2. Although pretreatment values for urinary total hydroxyproline excretion and cardiac output were considerably increased in some patients, there was no correlation between these two variables in the group as a whole. 3. Treatment resulted in a striking reduction in disease activity; the mean urinary hydroxyproline decreased 67%. 4. There was, however, no significant fall in cardiac output or change in oxygen transport during treatment. 5. Of the eight patients with bone pain who received treatment, five claimed complete pain relief.

scholarly journals Juvenile Paget’s disease: Report of a family with a novel missense mutation and unique radiological manifestations in a heterozygote

QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
O Khalifa ◽  
K Ramzan ◽  
A S Moustafa ◽  
K AlMane ◽  
M Abdelfattah ◽  
...  
Keyword(s):  
Alkaline Phosphatase ◽  
Bone Turnover ◽  
Skeletal Dysplasia ◽  
Serum Alkaline Phosphatase ◽  
Phenotypic Variability ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Homozygous Mutation ◽  
Markers Of Bone Turnover ◽  
Juvenile Paget’S Disease

Abstract Juvenile Paget’s disease (JPD) is a rare, generalized skeletal disorder characterized by markedly increased bone turnover secondary to enhanced osteoclastic activity. The patients with JPD develop progressive, widespread skeletal involvement and non-skeletal manifestations. Objectives To identify the characteristic molecular, biochemical, radiological, and audiological findings in three siblings with JPD associated with intrafamilial phenotypic variability. Patients and Methods A Saudi family with 3 affected siblings was recruited. Biochemical measurement of serum alkaline phosphatase and markers of bone turnover were performed. Genetic testing and sequencing of a panel of 22 known genes for skeletal dysplasia with increase bone density were performed by next generation sequencing (NGS). Radiological and audiological assessment were also performed. Results Patients showed marked elevation of serum alkaline phosphatase and markers of bone turnover. A novel homozygous mutation c.433T>G (p.Cys145Gly) in exon 3 of TNFRSF11B gene was identified in the 3 affected siblings. Both parents were heterozygous for the mutation. The heterozygote father had thickening of calvarium, a radiological manifestation of JPD. The eldest child (index case) had a unique striking distortion of the skull bones as evidenced by 3-D computed tomography of skull. He had also bilateral inner ear structural deformity and severe sensorineural hearing loss. Conclusions: JPD is a rare disorder that can be highly overlooked due to phenotypic overlap with other disorders of skeletal dysplasia. Mutations affecting cysteine residues result in the most severe JPD phenotypes. NGS is useful for early diagnosis of JPD, a prerequisite for successful treatment strategy.

scholarly journals Maladie de Paget au Sénégal : à propos de deux cas dans une population noire africaine

2016 ◽  
Vol 3 (2) ◽  
pp. 121-124
Author(s):  
Ismaïla Diédhiou ◽  
◽  
Awa-Ndao Fall ◽  
Thierno-Oumar Soko ◽  
Abdou-Rajack NDIAYE
Keyword(s):  
Alkaline Phosphatase ◽  
Physical Examination ◽  
Serum Alkaline Phosphatase ◽  
Paget’S Disease ◽  
Joint Space ◽  
Paget's Disease ◽  
Pelvic Bone ◽  
Cortical Thickening ◽  
Maladie De Paget ◽  
The Right

Mr. MD is a 72-years-old man, admitted for spontaneous, permanent, crushing type pain on the pelvis above the right hip evolving for two years, without night or morning stiffness but increasing with hearing loss, temporal and parietal headache. Physical examination showed a painful hip in active and passive mobilization. Pressure on iliac spines and lower lumbar and sacrococcygeal bones was painful. The patient showed no inflammatory syndrome. Serum calcium was normal. We noted an isolated increase in alkaline phosphatase levels to 401 IU/l. Radiographs showed bilateral heterogeneous sclerosis of the iliac bone with thickening lines and and almost disappearance of the right hip joint space. There was a marked thickening of the cortex on the femoral proximal third and thickening of the cranial vault. MRI showed cortical thickening of the right pelvic bone, a T1 hyper signal, and an intermediate T2 signal with fat/sat. This was pathognomonic of Paget's disease. The second patient is a 72-year-old man with no history, having intense pain on the right side of the lower limb. Physical examination showed no musculoskeletal deformity, but pain on palpation and mobilization of the right hip. Serum alkaline phosphatase (ALP) was raised to 4 times the normal range. Radiography showed cortical thickening of the ischial pubic branch, a heterogeneous sclerosis gypsy moth of the iliac wing, a steady narrowing of the femoral hip-spaced lines and thickening of the iliac ischial pubic. This aspect is pathognomonic of Paget’s disease, the patient underwent treatment with zoledronic acid intravenously at 5 mg. The outcome was favorable up to 10 months with reduced pain (VAS = 2/10) and normal PAL.

Possible role for glucagon in the control of Paget’s disease of bone

2019 ◽  
Vol 95 (1120) ◽  
pp. 85-90
Author(s):  
John Rhys Condon
Keyword(s):  
Serum Alkaline Phosphatase ◽  
Paget’S Disease ◽  
Vascular Complications ◽  
Paget's Disease ◽  
Paget’S Disease Of Bone ◽  
Response To Treatment ◽  
Vertebrobasilar Insufficiency ◽  
Paget's Disease Of Bone ◽  
Trigeminal Nerves

Paget’s disease of bone is characterised by overactive osteoclasts that resorb bone at a higher rate than normal. Osteoblasts attempt to repair the damage by laying down new bone which in turn is resorbed leaving a chaotic pattern of lytic and dense sclerotic bone behind. Deformed bone enlarges, becomes vascularised, bends and fractures. No bone is exempt but the skull, pelvis, vertebrae and long bones are commonly affected. Pressure from pagetic bone impinges on the auditory, facial, optic, trigeminal nerves and the spinal cord, risking paraplegia or quadriplegia. Vascular complications include cardiac failure and vertebrobasilar insufficiency. Serum alkaline phosphatase and urine N-telopeptide were used to assess response to treatment with porcine, salmon and human calcitonins, glucagon and bisphonates given alone or in combination. Glucagon has few side effects and controls the disease very rapidly. It can be given alone but because remissions last a few months, repeat courses may be necessary to achieve a long-term permanent quiescent bone state. If complete disease remission is not achieved with the hormone alone, an oral or intravenous bisphosphonate is given at the end of glucagon treatment. Other options are a second-generation bisphosphonate given orally to patients who decline parenteral medication. It remains to be seen whether glucagon affects other bone disorders.

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