Systemic Mastocytosis Presenting as Pathologic Intertrochanteric Femur Fracture

Shah, Aadit; Bhan, Rohit; Pey, Eduard Praiss; Riordan, Haley; Khan, Farhat Ullah

doi:10.5435/jaaosglobal-d-21-00137

Cited by 3 publications

(2 citation statements)

References 27 publications

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“…For patients with poor physical conditions, blind pursuit of anatomic reduction is not desirable. Repeated continuous reduction and prolonged surgery and anesthesia may lead to a significant increase in postoperative hidden blood loss in patients, which in turn leads to an increased risk of complications in patients (23)(24)(25). Therefore, priority should be given to the alignment of the fracture rather than complete reduction for frail elderly patients.…”

Section: Discussionmentioning

confidence: 99%

Observation of the clinical efficacy of percutaneous reduction by leverage combined with intramedullary nail internal fixation in the treatment of irreducible femoral intertrochanteric fracture: a retrospective single-arm cohort study

Jiang¹,

Han²,

Liu³

et al. 2022

Ann Transl Med

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Background: There are few reports on the efficacy and safety of percutaneous lever reduction combined with intramedullary nailing in the treatment of irreducible functional intertrochanteric fracture. This study was designed to investigate the clinical effect of percutaneous reduction by leverage combined with intramedullary nail internal fixation in the treatment of irreducible femoral intertrochanteric fracture.Methods: A total of 26 patients with irreducible femoral intertrochanteric fracture admitted to Qilu Hospital were included in this study, including 10 males and 16 females. All fractures were reduced through an incision made at the insertion point of the intramedullary nail head or the main nail with the aid of auxiliary equipment such as a periosteal dissector or a bone-holding forcep. Indicators such as operative time, blood loss, and complications were recorded, and the quality of fracture reduction was evaluated by the Baumgaetner modified method. All the patients were followed up regularly for 3 months, 1 year, and 2 years postoperatively, and the Zuckerman Functional Recovery Scale (FRS) for Hip Fracture was utilized to evaluate the hip function of the patients. The Euro-Quality of Life-5 Dimension (EQ-5D) was used to evaluate the quality of life of patients. Hip pain was assessed by the visual analog scale (VAS) at the last follow-up.Results: According to the Evans-Jensen classification of fractures, 12 cases were classified as type III, 10 as type IV, and 4 as type V. The mean operation time was 67.9±16.4 min and the intraoperative blood loss was 165.8±58.3 mL. All fractures healed completely. In terms of fracture reduction quality, 14 cases were excellent and 11 cases were good. At the last follow-up, the FRS scores decreased from 93.3±5.7 preoperatively to 81.5±18.5 postoperatively, and the EQ-5D index decreased from 0.95±0.05 preoperatively to 0.86±0.14 postoperatively.Conclusions: With mini-incision assisted reduction combined with intramedullary nail internal fixation to treat of irreducible femoral intertrochanteric fracture, favorable clinical results can be obtained, and the walking ability and quality of life of the patients can be improved postoperatively.

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Section: Discussionmentioning

confidence: 99%

Observation of the clinical efficacy of percutaneous reduction by leverage combined with intramedullary nail internal fixation in the treatment of irreducible femoral intertrochanteric fracture: a retrospective single-arm cohort study

Jiang¹,

Han²,

Liu³

et al. 2022

Ann Transl Med

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“…There are reports regarding the management of osteoporosis in patients with mastocytosis [31][32][33][34][35]. Bisphosphonates are recommended as first-line treatment in osteoporotic patients with systemic mastocytosis [33].…”

Section: Management Of Systemic Mastocytosis In Terms Of Osteoporosismentioning

confidence: 99%

Osteoporosis in Patients With Systemic Mastocytosis

Coskun Benlidayi,

Boga Vijdan

2023

CAJMHE

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Mastocytosis is a disease characterized by abnormal proliferation and accumulation of clonal mast cells. One of the systems that may be affected in patients with mastocytosis is the skeletal system. Osteolysis, osteopenia, osteoporosis and osteosclerosis may occur as a result of skeletal system involvement. Osteoporosis is seen in more than 1/5 of these patients and the disease may even present with multiple fractures. Underlying factors of the deterioration of bone health in systemic mastocytosis include mast cell infiltration and systemic release of mast cell mediators, increased number of osteoclasts, and overproduction of proinflammatory cytokines. Taking preventive measures for bone health disorders in patients with systemic mastocytosis, implementing screening protocols and treating osteoporosis appropriately if it develops are extremely important in preventing fragility fractures. The aim of this review is to provide an insight to the changes in bone tissue in patients with mastocytosis.

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Mastocytose als oorzaak van osteoporotische fracturen: casusbespreking en literatuurstudie

Jonckheere¹,

Breynaert²,

Gielen³

2023

Tijdschr Geneesk

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Mastocytosis as a cause of osteoporotic fractures: a case report and literature review Systemic mastocytosis is a rare cause of secondary osteoporosis. Mastocytosis is a myeloproliferatic disease characterized by abnormal growth and proliferation of mast cells. The disease is associated with activating KIT mutations. There are 2 major types of mastocytosis: cutaneous mastocytosis and systemic mastocytosis with extracutaneous infiltration. Systemic mastocytosis needs to be considered in the differential diagnosis of unexplained osteoporosis, unexplained spinal fractures (especially in men and premenopausal women) and in patients with osteoporosis with severe bone pain. Bone involvement is one of the most frequent expressions of systemic mastocytosis, especially in adults. The clinical presentation is diverse: from osteoporosis with fragility fractures and poorly localized bone pain to asymptomatic osteolytic and/or focal sclerotic lesions and diffuse osteosclerosis. Osteoporosis in patients with systemic mastocytosis is caused by neoplastic infiltration or, more likely, local release of mediators. Screening for mastocytosis is possible by determining the serum basal tryptase and searching for a p.D816V KIT mutation, but the final diagnosis requires a bone marrow examination. The first-line treatment of osteoporosis secondary to mastocytosis consists of bisphosphonates and denosumab. Teriparatide can be considered in patients with new vertebral fractures under treatment with antiresorptive therapy, but more research is needed to evaluate its effectivity and safety. In case of refractory osteoporosis caused by mastocytosis, cytoreductive therapy, whether or not in combination with bisphosphonates, can be administered. In this article, the authors report a case of a 48-year-old patient with osteoporosis as the first manifestation of underlying mastocytosis. A literature review was performed to describe the etiology, pathophysiology, diagnostic process and treatment options of bone involvement in patients with mastocytosis.

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Systemic Mastocytosis Presenting as Pathologic Intertrochanteric Femur Fracture

Cited by 3 publications

References 27 publications

Observation of the clinical efficacy of percutaneous reduction by leverage combined with intramedullary nail internal fixation in the treatment of irreducible femoral intertrochanteric fracture: a retrospective single-arm cohort study

Observation of the clinical efficacy of percutaneous reduction by leverage combined with intramedullary nail internal fixation in the treatment of irreducible femoral intertrochanteric fracture: a retrospective single-arm cohort study

Osteoporosis in Patients With Systemic Mastocytosis

Mastocytose als oorzaak van osteoporotische fracturen: casusbespreking en literatuurstudie

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