Influenza-like Viral Illnesses and Flare-ups of Fibrodysplasia Ossificans Progressiva

Scarlett, Richard F; Rocke, David M.; Kantanie, Sharon; Patel, Jean B.; Shore, Eileen M.; Kaplan, Frederick S.

doi:10.1097/01.blo.0000129557.38803.26

Cited by 67 publications

(46 citation statements)

References 19 publications

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“…However, the natural progression of the disease can be altered by environmental factors, such as soft tissue injury. Tissue damage and/or the wound healing response that follows are often associated with induction of heterotopic bone formation at the site of injury [10,[19][20][21][22][23].…”

Section: Clinical Description and Diagnostic Criteria For Classic Fopmentioning

confidence: 99%

Insights from a rare genetic disorder of extra-skeletal bone formation, fibrodysplasia ossificans progressiva (FOP)

Shore

Kaplan

2008

Bone

114

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Fibrodysplasia ossificans progressiva (FOP) is a rare human genetic disorder of extensive and debilitating extra-skeletal bone formation. While the challenges of investigating a rare condition are many, the potential benefits are also great -not only for the specific disease under investigation, but also for the unique perspective on how cells normally function and the mechanisms that underlie more common disorders. This review will illustrate some of the many insights that we have gained by studying FOP.

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Section: Clinical Description and Diagnostic Criteria For Classic Fopmentioning

confidence: 99%

Insights from a rare genetic disorder of extra-skeletal bone formation, fibrodysplasia ossificans progressiva (FOP)

Shore

Kaplan

2008

Bone

114

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“…Although there is no effective treatment, it is important to avoid soft-tissue trauma, including biopsies, 7 intramuscular injections, 9 surgical procedures, 7,10 and mandibular blocks for dental procedures, 11 as well as viral illnesses. 12 All of these are likely to induce episodes of rapidly progressive heterotopic ossification, with resultant permanent loss of motion in the affected area. Therefore, trauma prevention is a major aspect of care, and the cost to the patient of misdiagnosis and inappropriate intervention is high.…”

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confidence: 99%

Iatrogenic Harm Caused by Diagnostic Errors in Fibrodysplasia Ossificans Progressiva

et al. 2005

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ABSTRACT. Background. Little is known about diagnostic errors for a disease worldwide. Such errors could alter the disease's natural history, especially if unwarranted interventions cause irreversible harm. Fibrodysplasia ossificans progressiva (FOP), a rare, autosomal dominant genetic disease characterized by episodes of permanent heterotopic ossification of soft tissues, occurs worldwide without racial, ethnic, or geographic predilection. There is no effective treatment, and soft-tissue trauma (eg, biopsies, surgical procedures, intramuscular injections, or mandibular blocks for dental procedures) and viral illnesses are likely to induce episodes of rapidly progressive heterotopic ossification, with resultant permanent loss of motion in the affected area. Accurate diagnoses can be made on the basis of the clinical findings of tumor-like swellings on the head, neck, back, or shoulders and characteristic short great toes with hallux valgus-like malformations and missing interphalangeal joints. On the basis of conversations with numerous individuals with FOP, we suspected that diagnostic errors with FOP are common and often associated with inappropriate and harmful diagnostic and therapeutic procedures.Objective. To document the frequency of diagnostic errors with FOP and complications resulting from misdiagnoses.Design. A questionnaire requesting detailed demographic, diagnostic, and treatment information was sent to all 269 patient-members of the International FOP Association; the sampling frame included >90% of all known FOP patients worldwide. We received 138 replies (51% response) from 25 countries. The age range was 2 to 71 years; there were 78 female subjects and 60 male subjects. In addition, to assess the availability and adequacy of information about FOP, we reviewed 184 English-language textbooks in relevant specialties published in the past 20 years.Results. Incorrect diagnoses were given initially to 87% of individuals with FOP. This astonishing rate of diagnostic errors occurred worldwide, regardless of ethnicity, geographic background, or misdiagnosing physician's specialty. The most common incorrect diagnosis was cancer (32%). The mean period from the onset of symptoms to correct diagnosis was 4.1 years, and the median number of physicians consulted before the correct diagnosis of FOP was 6. For 67% of patients, unnecessary invasive procedures (biopsies) were performed; 68% received inappropriate therapies. Forty-nine percent of all patients reported permanent loss of mobility resulting from invasive medical interventions that caused posttraumatic ossification. Notably, only 8% of the 184 textbooks that were reviewed contained adequate descriptions of FOP, including the caution that trauma can accelerate the process of heterotopic ossification.Conclusions. T he accurate diagnosis of disease has been a basic principle of medical care since antiquity. Mistaken or delayed diagnosis can lead to morbidity or death through use of inappropriate procedures or through withholding of necessary treatments. R...

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“…Such trauma may be as subtle as needle sticks, bumps, or major injuries such as catastrophic falls. Even viral influenza illness has been an inciting event in the flare-up of FOP [2,3]. Fibrodysplasia ossificans progressiva is classically characterized as having malformations of the great toes and heterotopic ossification of tissues, including tendons, ligamentum, and skeletal muscle.…”

Section: Introductionmentioning

confidence: 99%

Fibrodysplasia ossificans progressiva: anesthetic management in complex orthopedic spine procedures

Gorji

Nastasi

et al. 2011

Journal of Clinical Anesthesia

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Influenza-like Viral Illnesses and Flare-ups of Fibrodysplasia Ossificans Progressiva

Abstract: disease activity in this population of patients genetically susceptible to progressive heterotopic ossification.

Cited by 67 publications

References 19 publications

Insights from a rare genetic disorder of extra-skeletal bone formation, fibrodysplasia ossificans progressiva (FOP)

Insights from a rare genetic disorder of extra-skeletal bone formation, fibrodysplasia ossificans progressiva (FOP)

Iatrogenic Harm Caused by Diagnostic Errors in Fibrodysplasia Ossificans Progressiva

Fibrodysplasia ossificans progressiva: anesthetic management in complex orthopedic spine procedures

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