DOCSLIB.ORG

Osteomalacia and Osteoporosis D

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Osteomalacia and Osteoporosis D Postgrad. med.J. (August 1968) 44, 621-625. Postgrad Med J: first published as 10.1136/pgmj.44.514.621 on 1 August 1968. Downloaded from Osteomalacia and osteoporosis D. B. MORGAN Department of Clinical Investigation, University ofLeeds OSTEOMALACIA and osteoporosis are still some- in osteomalacia is an increase in the alkaline times confused because both diseases lead to a phosphatase activity in the blood (SAP); there deficiency of calcium which can be detected on may also be a low serum phosphorus or a low radiographs of the skeleton. serum calcium. This lack of calcium is the only feature Our experience with the biopsy of bone is that common to the two diseases which are in all a large excess of uncalcified bone tissue (osteoid), other ways easily distinguishable. which is the classic histological feature of osteo- malacia, is only found in patients with the other Osteomalacia typical features of the disease, in particular the Osteomalacia will be discussed first, because it clinical ones (Morgan et al., 1967a). Whether or is a clearly defined disease which can be cured. not more subtle histological techniques will detect Osteomalacia is the result of an imbalance be- earlier stages of the disease remains to be seen. tween the supply of and the demand for vitamin Bone pains, muscle weakness, Looser's zones, D. The the following description of disease is raised SAP and low serum phosphate are the Protected by copyright. based on our experience of twenty-two patients most reliable aids to the diagnosis of osteomalacia, with osteomalacia after gastrectomy; there is no and approximately in that order. evidence that the features vary with the cause The interpretation of a raised SAP is difficult of the disease. because the 'normal values' for SAP are not The clinical features are nearly specific; the clearly defined. The particular problem is that common ones are bone pains and muscle weak- the SAP increases with age in the normal popula- ness. The bone pains are generalized, often worse tion (Hobson & Jordan 1959; Klaasen & Sier- at night, and sometimes associated with tender- stema, 1964); values of 16 or even 25 KA units/ ness of the bones. The bone pains have been 100 ml of serum are not uncommon in well mistakenly attributed to rheumatoid arthritis, persons over 60 years of age. Even if the SAP osteoarthritis or even neurosis, and the diagnosis of is above these values it may indicate some other osteomalacia may be delayed for years. disease of bone, for example Paget's disease, or The muscle weakness is most obvious in the even chronic liver disease, rather than osteo- muscle around the pelvis, and a common symp- malacia. http://pmj.bmj.com/ tom is difficulty in climbing stairs and in sitting A raised SAP together with generalized bone up in bed without the aid of the arms. pains, could be the result of Paget's disease of The muscles may be wasted as well as weak bone rather than osteomalacia and it may be but there is usually no evidence of neurological impossible to distinguish these two diseases even damage; the weakness is a symptom of a primary on biopsy of the bone (Paterson, Woods & muscle disorder. The radiological signs of the Morgan, 1968). That Paget's disease is radiolog- disease may be no more than a generalized ically detectable is no proof that it is the cause lack of calcium in the skeleton indistinguish- of the symptoms and the presence of Paget's on September 28, 2021 by guest. able from that in osteoporosis. In addition, disease which is common does not exclude osteo- Looser's nodes are a radiological finding which malacia which is rare. is almost specific for osteomalacia. However, For these reasons we have come to regard a the nodes are not always present, and similar response to vitamin D as the essential and final lesions arise in Paget's disease of bone, hyper- diagnostic criterion of osteomalacia. parathyroidism and thyrotoxicosis (Camp & Historically, osteomalacia was recognized as McCullogh, 1941). the adult counterpart of rickets in children, that Looser's nodes are also known as pseudofrac- is to say osteomalacia was the result of a simple tures because they look like fractures on the deficiency of vitamin D usually the result of a radiographs, but they are often painless. poor dietary intake of the vitamin. Osteomalacia The common and constant biochemical change thus defined still occurs (Gough, Lloyd & Wills, 622 D. B. Morgan Postgrad Med J: first published as 10.1136/pgmj.44.514.621 on 1 August 1968. Downloaded from 1964; Andersson et al., 1966; Chalmers et al., For the osteomalacia after gastrectomy we 1967). This condition can usually be cured with have given one dose of 10 mg of vitamin D2 by 3000 i.u. of vitamin D a day. The daily require- intramuscular injection. This dose will relieve ment for the vitamin in the diet is probably of the bone pains, heal the Looser's nodes, and the order of 100 i.u. (25,ug) in adults. Unfor- the SAP and serum phosphorus and calcium tunately, the term osteomalacia has been applied return to normal. The SAP may rise after the to those conditions which simulate nutritional vitamin D, before it begins to fall towards nor- osteomalacia in their clinical, biochemical, and mal values. The increase in the serum phos- histological signs, but which are not the result phorus may be the most dramatic and reliable of a simple deficiency of vitamin D. measure of a response to vitamin D but only if These 'osteomalacia-like' diseases fall into the measurements are made in the fasting patient. several groups according to the dose of vitamin The frequency at which further injections are D required to achieve some response and the required varies much from one patient to fundamental abnormality in the disease. another. We have controlled the frequency of At one extreme is the group of diseases which injection by the symptoms and the SAP. Some includes familial hypophosphataemia. These of our patients required the injections every 4 diseases are not cured by vitamin D however months, but one of our patients with severe much of the vitamin is given. osteomalacia, who had only one injection almost The osteomalacia of chronic glomerular failure 3 years ago, became symptom-free in a few heals with vitamin D but only in very large months and has remained well since. A safe doses (150,000i.u. a day or more). Stanbury & prescription and a liberal one would be an injec- Lumb (1966), therefore, suggested that this condi- tion of 1 mg of vitamin D2 every month. tion is the result of a resistance to the action Once the diagnosis is established it is essential of the vitamin and thus an increased demand to supplement the diet with calcium, otherwise Protected by copyright. for it. This demand is not met by the amount the bone may heal at the expense of the calci- of the vitamin in the diet. fied skeleton, and the osteomalacia may become The position within this spectrum of the osteoma- osteoporosis. lacia of intestinal disease depends on the form Treatment should be continued for at least 2 of the latter. Osteomalacia after resection of years, even if the symptoms have disappeared the distal intestine or in association with regional earlier and the biochemistry has returned to ileitis is usually the result of a simple deficiency normal. of vitamin D, perhaps due to a failure to absorb A recurrence of the disease can probably be the vitamin. On the other hand, the osteomalacia prevented by 1000 i.u. of vitamin D by mouth associated with malabsorption due to gluten sen- every day for life. Supplementation of the diet sitivity may be partly the result of a resistance with calcium is not necessary after the bones to the vitamin (Nassim et al., 1959). have healed. In order to establish the nature of the osteo- malacia after gastrectomy we gave patients with The cause of the vitamin deficiency http://pmj.bmj.com/ the clinical and biochemical features of the We get our vitamin D from the diet by mak- disease, small doses of the vitamin of the order ing the vitamin in the skin through a trapping of the physiological requirement for the vitamin; of the energy in the ultraviolet light from the in this case 1000 i.u. (25 ,ug) weekly by intra- sun. muscular injection (Morgan et al., 1965b). The Taking into account the many factors which patients responded clinically, biochemically, diminish the amount of ultraviolet light that radiologically and histologically to this treat- reaches us, the amount of the vitamin we make ment. Such a response not only established the in the skin is probably small and may be insigni- on September 28, 2021 by guest. presence of osteomalacia in these patients but ficant in relation to our need for the vitamin. also demonstrated that the osteomalacia was Tthe deficiency of vitamin D in patients with the result of a simple deficiency of vitamin D. osteomalacia after gastrectomy is, therefore, the These small doses were chosen to establish result of too small an intake of the vitamin or the presence of a deficiency of the vitamin; we a failure to absorb the vitamin or both. would not of course suggest that they represent There is no simple explanation of the defic- the most efficient treatment for the disease. iency which applies to all patients with osteo- malacia after gastrectomy, and it seems likely The treatment of osteomalacia that a deficiency only arises when all the factors The dose of vitamin D which is effective which can diminish the supply of the vitamin clearly depends on the cause of the osteomalacia.
Load more

Recommended publications
  • Severe Septicaemia in a Patient with Polychondritis and Sweet's
    81 LETTERS Ann Rheum Dis: first published as 10.1136/ard.62.1.88 on 1 January 2003. Downloaded from Severe septicaemia in a patient with polychondritis and Sweet’s syndrome after initiation of treatment with infliximab F G Matzkies, B Manger, M Schmitt-Haendle, T Nagel, H-G Kraetsch, J R Kalden, H Schulze-Koops ............................................................................................................................. Ann Rheum Dis 2003;62:81–82 D Sweet first described an acute febrile neutrophilic dermatosis in 1964 characterised by acute onset, fever, Rleucocytosis, and erythematous plaques.1 Skin biopsy specimens show infiltrates consisting of mononuclear cells and neutrophils with leucocytoclasis, but without signs of vasculi- tis. Sweet’s syndrome is frequently associated with solid malig- nancies or haemoproliferative disorders, but associations with chronic autoimmune connective tissue disorders have also been reported.2 The aetiology of Sweet’s syndrome is unknown, but evidence suggests that an immunological reaction of unknown specificity is the underlying mechanism. CASE REPORT A 51 year old white man with relapsing polychondritis (first diagnosed in 1997) was admitted to our hospital in June 2001 with a five week history of general malaise, fever, recurrent arthritis, and complaints of morning stiffness. Besides Figure 1 autoimmune polychondritis, he had insulin dependent Manifestation of Sweet’s syndrome in a patient with relapsing polychondritis. diabetes mellitus that was diagnosed in 1989. On admission, he presented with multiple small to medium, sharply demarked, raised erythematous plaques on both fore- dose of glucocorticoids (80 mg) and a second application of http://ard.bmj.com/ arms and lower legs, multiple acne-like pustules on the face, infliximab (3 mg/kg body weight) were given.
    [Show full text]
  • Hypophosphatasia Could Explain Some Atypical Femur Fractures
    Hypophosphatasia Could Explain Some Atypical Femur Fractures What we know Hypophosphatasia (HPP) is a rare genetic disease that affects the development of bones and teeth in children (Whyte 1985). HPP is caused by the absence or reduced amount of an enzyme called tissue-nonspecific alkaline phosphatase (TAP), also called bone-specific alkaline phosphatase (BSAP). The absence of TAP raises the level of inorganic pyrophosphate (Pi), which prevents calcium and phosphate from creating strong, mineralized bone. Without TAP, bones can become weak. In its severe form, HPP is fatal and happens in 1/100,000 births. Because HPP is genetic, it can appear in adults as well. A recent study has identified a milder, more common form of HPP that occurs in 4 of 1000 adults (Dahir 2018). This form of HPP is usually seen in early middle aged adults who have low bone density and sometimes have stress fractures in the feet or thigh bone. Sometimes these patients lose their baby teeth early, but not always. HPP is diagnosed by measuring blood levels of TAP and vitamin B6. An elevated vitamin B6 level [serum pyridoxal 5-phosphate (PLP)] (Whyte 1985) in a patient with a TAP level ≤40 or in the low end of normal can be diagnosed with HPP. Almost half of the adult patients with HPP in the large study had TAP >40, but in the lower end of the normal range (Dahir 2018). The connection between hypophosphatasia and osteoporosis Some people who have stress fractures get a bone density test and are treated with an osteoporosis medicine if their bone density results are low.
    [Show full text]
  • Upper Jaw Chronic Osteomyelitis. Report of Four Clinical Cases Osteomielitis Crónica Maxilar
    www.medigraphic.org.mx Revista Odontológica Mexicana Facultad de Odontología Vol. 16, No. 2 April-June 2012 pp 105-111 CASE REPORT Upper jaw chronic osteomyelitis. Report of four clinical cases Osteomielitis crónica maxilar. Informe de 4 casos clínicos Alberto Wintergerst Fish,* Carlos Javier Iturralde Espinosa,§ Vladimir de la Riva Parra,II Santiago Reinoso QuezadaII ABSTRACT RESUMEN Osteomyelitis is an infl ammatory bone disease commonly related La osteomielitis es una enfermedad ósea infl amatoria, comúnmente to an infectious origin caused by germs, mainly pyogenic staphy- relacionada a un origen infeccioso por gérmenes piógenos funda- lococcus, and occasionally, streptococci, pneumococci and en- mentalmente estafi lococos y en algunas ocasiones por estreptoco- terobacteriae. Several treatments and classifi cations for osteomy- cos, neumococos y enterobacterias. Se han establecido diversas elitis have been established. These are based on clinical course, clasifi caciones y tratamientos para la osteomielitis, basadas en el pathologic-anatomical or radiologic features, etiology and patho- comportamiento clínico, características anatomo-patológicas, ra- genesis. Chronic osteomyelitis is a complication of non-treated or diográfi cas, etiología y patogenia. La osteomielitis crónica es una inadequately treated acute osteomyleitis. It can also be caused by a complicación de la osteomielitis aguda no tratada, manejada inade- low grade prolonged infl ammatory reaction. This study presents four cuadamente o como una reacción infl amatoria prolongada de bajo cases of maxillary osteomyelitis treated between 2007 and 2009. grado. Se presentan 4 casos de osteomielitis crónica en el maxilar Cases were treated with antimicrobial therapy. Preoperatively, pa- tratadas entre 2007 y 2009 mediante terapia antimicrobiana preo- tients were prescribed Clindamycin, 300 mg every eight hours, Ce- peratoriamente con clindamicina 300 mg, IV cada 8 h y ceftriaxona friaxone, 1 g IV every 12 hours.
    [Show full text]
  • Hypophosphatasia: Current Literature for Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment
    Open Access Review Article DOI: 10.7759/cureus.8594 Hypophosphatasia: Current Literature for Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment Abdulai Bangura 1 , Lisa Wright 2 , Thomas Shuler 2 1. Department of Research, Trinity School of Medicine, Ratho Mill, VCT 2. Department of Orthopaedics, Carilion Clinic, Roanoke, USA Corresponding author: Abdulai Bangura, [email protected] Abstract Hypophosphatasia (HPP) is a rare inherited bone disorder identified by impaired bone mineralization. There are seven subtypes of HPP mainly characterized by their age of onset. These subtypes consist of perinatal (prenatal) benign, perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and pseudohypophosphatasia. Due to limited awareness of the condition, either misdiagnosis or delayed diagnosis is common. Furthermore, the condition is frequently treated with contraindicated drugs. This literature illustrates the most recent findings on the etiology, pathophysiology, clinical manifestations, diagnosing, and treatment for HPP and its subtypes. The etiology of the disease consists of loss-of-function mutations of the ALPL gene on chromosome one, which encodes for tissue nonspecific isoenzyme of alkaline phosphatase (TNAP). A decrease of TNAP reduces inorganic phosphate (Pi) for bone mineralization and allows for an increase in inorganic pyrophosphate (PPi) and phosphorylated osteopontin (p-OPN), which further reduces bone mineralization. The combination of these processes softens bone and mediates a clinical presentation similar to rickets/osteomalacia. HPP has an additional wide range of clinical features depending on its subtype. Although a concrete diagnostic guideline has not yet been established, many studies have supported a similar method of identifying HPP. Clinical features, radiological findings, and/or biomarker levels of the disorder should raise suspicion and encourage the inclusion of HPP as a differential diagnosis.
    [Show full text]
  • Metabolic Bone Disease 5
    g Metabolic Bone Disease 5 Introduction, 272 History and examination, 275 Osteoporosis, 283 STRUCTURE AND FUNCTION, 272 Investigation, 276 Paget’s disease of bone, 288 Structure of bone, 272 Management, 279 Hyperparathyroidism, 290 Function of bone, 272 DISEASES AND THEIR MANAGEMENT, 280 Hypercalcaemia of malignancy, 293 APPROACH TO THE PATIENT, 275 Rickets and osteomalacia, 280 Hypocalcaemia, 295 Introduction Calcium- and phosphate-containing crystals: set in a structure• similar to hydroxyapatite and deposited in holes Metabolic bone diseases are a heterogeneous group of between adjacent collagen fibrils, which provide rigidity. disorders characterized by abnormalities in calcium At least 11 non-collagenous matrix proteins (e.g. osteo- metabolism and/or bone cell physiology. They lead to an calcin,• osteonectin): these form the ground substance altered serum calcium concentration and/or skeletal fail- and include glycoproteins and proteoglycans. Their exact ure. The most common type of metabolic bone disease in function is not yet defined, but they are thought to be developed countries is osteoporosis. Because osteoporosis involved in calcification. is essentially a disease of the elderly, the prevalence of this condition is increasing as the average age of people Cellular constituents in developed countries rises. Osteoporotic fractures may lead to loss of independence in the elderly and is imposing Mesenchymal-derived osteoblast lineage: consist of an ever-increasing social and economic burden on society. osteoblasts,• osteocytes and bone-lining cells. Osteoblasts Other pathological processes that affect the skeleton, some synthesize organic matrix in the production of new bone. of which are also relatively common, are summarized in Osteoclasts: derived from haemopoietic precursors, Table 3.20 (see Chapter 4).
    [Show full text]
  • A Case of Osteitis Fibrosa Cystica (Osteomalacia?) with Evidence of Hyperactivity of the Para-Thyroid Bodies
    A CASE OF OSTEITIS FIBROSA CYSTICA (OSTEOMALACIA?) WITH EVIDENCE OF HYPERACTIVITY OF THE PARA-THYROID BODIES. METABOLIC STUDY II Walter Bauer, … , Fuller Albright, Joseph C. Aub J Clin Invest. 1930;8(2):229-248. https://doi.org/10.1172/JCI100262. Research Article Find the latest version: https://jci.me/100262/pdf A CASE OF OSTEITIS FIBROSA CYSTICA (OSTEOMALACIA?) WITH EVIDENCE OF HYPERACTIVITY OF THE PARA- THYROID BODIES. METABOLIC STUDY IIF By WALTER BAUER,2 FULLER ALBRIGHT3 AND JOSEPH C. AUB (From the Medical Clinic of the Massachutsetts General Hospital, Boston) (Received for publication February 5, 1929) INTRODUCTION In a previous paper (1) we have pointed out certain characteristic responses in the calcium and phosphorus metabolisms resulting from parathormone4 administration to essentially normal individuals. In the present paper, similar studies will be reported on a patient who presented a condition suggestive of idiopathic hyperparathyroidism. CASE HISTORY The patient, Mr. C. M., sea captain, aged 30, was transferred from the Bellevue Hospital Service to the Special Study Ward of the Massachusetts General Hospital through the courtesy of Dr. Eugene F. DuBois, for further investigation of his calcium metabolism and for consideration of parathyroidectomy. His complete case history has been reported by Hannon, Shorr, McClellan and DuBois (2). It describes a man invalided for over three years with symptoms resulting from a generalized skeletal decalcification. (See x-rays, figs. 1 to 4.) 1 This is No. VII of the series entitled "Studies of Calcium and Phosphorus Metabolism" from the Medical Clinic of the Massachusetts General Hospital. 2 Resident Physician, Massachusetts General Hospital. ' Research Fellow, Massachusetts General Hospital and Harvard Medical School.
    [Show full text]
  • Rheumatology Certification Exam Blueprint
    Rheumatology Certification Examination Blueprint Purpose of the exam The exam is designed to evaluate the knowledge, diagnostic reasoning, and clinical judgment skills expected of the certified rheumatologist in the broad domain of the discipline. The ability to make appropriate diagnostic and management decisions that have important consequences for patients will be assessed. The exam may require recognition of common as well as rare clinical problems for which patients may consult a certified rheumatologist. Exam content Exam content is determined by a pre-established blueprint, or table of specifications. The blueprint is developed by ABIM and is reviewed annually and updated as needed for currency. Trainees, training program directors, and certified practitioners in the discipline are surveyed periodically to provide feedback and inform the blueprinting process. The primary medical content categories of the blueprint are shown below, with the percentage assigned to each for a typical exam: Medical Content Category % of Exam Basic and Clinical Sciences 7% Crystal-induced Arthropathies 5% Infections and Related Arthritides 6% Metabolic Bone Disease 5.5% Osteoarthritis and Related Disorders 5% Rheumatoid Arthritis 13% Seronegative Spondyloarthropathies 6.5% Other Rheumatic and Connective Tissue Disorders (ORCT) 16.5 % Lupus Erythematosus 9% Nonarticular and Regional Musculoskeletal Disorders 7% Nonrheumatic Systemic Disorders 9% Vasculitides 8.5% Miscellaneous Topics 2% 100% Exam questions in the content areas above may also address clinical topics in geriatrics, pediatrics, pharmacology and topics in general internal medicine that are important to the practice of rheumatology. Exam format The exam is composed of multiple-choice questions with a single best answer, predominantly describing clinical scenarios.
    [Show full text]
  • An Unusual Cause of Back Pain in Osteoporosis: Lessons from a Spinal Lesion
    Ann Rheum Dis 1999;58:327–331 327 MASTERCLASS Series editor: John Axford Ann Rheum Dis: first published as 10.1136/ard.58.6.327 on 1 June 1999. Downloaded from An unusual cause of back pain in osteoporosis: lessons from a spinal lesion S Venkatachalam, Elaine Dennison, Madeleine Sampson, Peter Hockey, MIDCawley, Cyrus Cooper Case report A 77 year old woman was admitted with a three month history of worsening back pain, malaise, and anorexia. On direct questioning, she reported that she had suVered from back pain for four years. The thoracolumbar radiograph four years earlier showed T6/7 vertebral collapse, mild scoliosis, and degenerative change of the lumbar spine (fig 1); but other investigations at that time including the eryth- rocyte sedimentation rate (ESR) and protein electophoresis were normal. Bone mineral density then was 0.914 g/cm2 (T score = −2.4) at the lumbar spine, 0.776 g/cm2 (T score = −1.8) at the right femoral neck and 0.738 g/cm2 (T score = −1.7) at the left femoral neck. She was given cyclical etidronate after this vertebral collapse as she had suVered a previous fragility fracture of the left wrist. On admission, she was afebrile, but general examination was remarkable for pallor, dental http://ard.bmj.com/ caries, and cellulitis of the left leg. A pansysto- lic murmur was heard at the cardiac apex on auscultation; there were no other signs of bac- terial endocarditis. She had kyphoscoliosis and there was diVuse tenderness of the thoraco- lumbar spine. Her neurological examination was unremarkable. on September 29, 2021 by guest.
    [Show full text]
  • Adult Osteomalacia a Treatable Cause of “Fear of Falling” Gait
    VIDEO NEUROIMAGES Adult osteomalacia A treatable cause of “fear of falling” gait Figure Severe osteopenia The left hand x-ray suggested the diagnosis of osteomalacia because of the diffuse demineralization. A 65-year-old man was hospitalized with a gait disorder, obliging him to shuffle laterally1 (video on the Neurology® Web site at www.neurology.org) because of pain and proximal limb weakness. He had a gastrectomy for cancer 7 years previously, with severe vitamin D deficiency; parathormone and alkaline phosphatase were increased, with reduced serum and urine calcium and phosphate. There was reduced bone density (figure). He was mildly hypothyroid and pancytopenic. B12 and folate levels were normal. Investigation for an endocrine neoplasm (CT scan, Octreoscan) was negative. EMG of proximal muscles was typical for chronic myopathy; nerve conduction studies had normal results. After 80 days’ supplementation with calcium, vitamin D, and levothyroxine, the patient walked properly without assistance (video); pancytopenia and alkaline phosphatase improved. Supplemental data at This unusual but reversible gait disorder may have resulted from bone pain and muscular weakness related to www.neurology.org osteomalacia2 and secondary hyperparathyroidism, with a psychogenic overlay. Paolo Ripellino, MD, Emanuela Terazzi, MD, Enrica Bersano, MD, Roberto Cantello, MD, PhD From the Department of Neurology, University of Turin (P.R.), and Department of Neurology, University of Eastern Piedmont (E.T., E.B., R.C.), AOU Maggiore della Carità, Novara, Italy. Author contributions: Dr. Ripellino: acquisition of data, video included; analysis and interpretation of data; writing and editing of the manuscript and of the video. Dr. Terazzi: analysis and interpretation of data.
    [Show full text]
  • Osteopetrosis J
    Arch Dis Child: first published as 10.1136/adc.46.247.257 on 1 June 1971. Downloaded from Archives of Disease in Childhood, 1971, 46, 257. Osteopetrosis J. S. YU,* R. K. OATES, K. HELEN WALSH, and SUSAN J. STUCKEY From the Department of Child Health, University of Sydney, and Department of Dietetics, Royal Alexandra Hospital for Children, Camperdown, N.S.W., Australia Yu, J. S., Oates, R. K., Walsh, K. H., and Stuckey, S. J. (1971). Archives of Disease in Childhood, 46, 257. Osteopetrosis. The clinical histories of nine children with osteopetrosis are reported. Two of them had the malignant infantile variety of the disease: one died within 3 months of birth and the other has survived 20 months on a regimen of a low calcium intake, cellulose phosphate, and steroids. The beneficial effect of a low calcium intake, in early infancy, is supported by the clinical course in the infant with the malignant variety and in another child with the more benign form of the disease. No calcium balance studies were performed. This study suggests that the active measures outlined may favourably influence the haematological and osteosclerotic course of the disease, pending further know- ledge of its aetiological basis, and more specific therapy. Osteopetrosis or marble bone disease is a rare positive calcium balance with a diet low in calcium metabolic bone disease characterized by dense and with steroids (Morrow et al., 1967). This bones (Albers-Schonberg, 1904; Karshner, 1926). approach to management has been recently criti- copyright. Two varieties have been clearly defined-an cized by Cohen (Children's Hospital Medical infantile progressive disease and a milder benign Center, Boston, 1965).
    [Show full text]
  • Distinguishing Transient Osteoporosis of the Hip from Avascular Necrosis
    Original Article Article original Distinguishing transient osteoporosis of the hip from avascular necrosis Anita Balakrishnan, BMedSci;* Emil H. Schemitsch, MD;* Dawn Pearce, MD;† Michael D. McKee, MD* Introduction: To review the circumstances surrounding the misdiagnosis of transient osteoporosis of the hip (TOH) as avascular necrosis (AVN) and to increase physician awareness of the prevalence and diagnosis of this condition in young men, we reviewed a series of cases seen in the orthopedic unit at St. Michael’s Hospital, University of Toronto. Methods: We studied the charts of patients with TOH referred between 1998 and 2001 with a diagnosis of AVN for demographic data, risk factors, imaging results and outcomes. Results: Twelve hips in 10 young men (mean age 41 yr, range from 32–55 yr) were identified. Nine men underwent magnetic resonance imaging (MRI) before referral, which showed characteristic changes of TOH. All 10 patients were referred for surgical intervention for a diagnosis of AVN. The correct diagnosis was made after reviewing patients’ charts and the scans and was confirmed by spontaneous resolution of both symptoms and MRI findings an average of 5.5 months and 7.5 months, respectively, after consultation. Conclusions: Despite recent publications, the prevalence of TOH among young men is still overlooked and the distinctive MRI appearance still misinterpreted. Symptoms may be severe but resolve over time with reduced weight bearing. The absence of focal changes on MRI is highly suggestive of a transient lesion. A greater level of awareness of this condition is needed to differentiate TOH from AVN, avoiding unnecessary surgery and ensuring appropriate treatment.
    [Show full text]
  • Hematological Diseases and Osteoporosis
    International Journal of Molecular Sciences Review Hematological Diseases and Osteoporosis , Agostino Gaudio * y , Anastasia Xourafa, Rosario Rapisarda, Luca Zanoli , Salvatore Santo Signorelli and Pietro Castellino Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy; [email protected] (A.X.); [email protected] (R.R.); [email protected] (L.Z.); [email protected] (S.S.S.); [email protected] (P.C.) * Correspondence: [email protected]; Tel.: +39-095-3781842; Fax: +39-095-378-2376 Current address: UO di Medicina Interna, Policlinico “G. Rodolico”, Via S. Sofia 78, 95123 Catania, Italy. y Received: 29 April 2020; Accepted: 14 May 2020; Published: 16 May 2020 Abstract: Secondary osteoporosis is a common clinical problem faced by bone specialists, with a higher frequency in men than in women. One of several causes of secondary osteoporosis is hematological disease. There are numerous hematological diseases that can have a deleterious impact on bone health. In the literature, there is an abundance of evidence of bone involvement in patients affected by multiple myeloma, systemic mastocytosis, thalassemia, and hemophilia; some skeletal disorders are also reported in sickle cell disease. Recently, monoclonal gammopathy of undetermined significance appears to increase fracture risk, predominantly in male subjects. The pathogenetic mechanisms responsible for these bone loss effects have not yet been completely clarified. Many soluble factors, in particular cytokines that regulate bone metabolism, appear to play an important role. An integrated approach to these hematological diseases, with the help of a bone specialist, could reduce the bone fracture rate and improve the quality of life of these patients.
    [Show full text]