Transient osteoporosis of the hip: A case report

We report a case of transient osteoporosis of the hip (TOH) in a 59-year-old man including the clinical presentation, diagnostic studies, management and clinical progress. TOH is a rare self-limiting condition that typically affects middle-aged men or, less frequently, women in the third trimester of pregnancy. Affected individuals present clinically with acute hip pain, limping gait, and limited ranges of hip motion. TOH may begin spontaneously or after a minor trauma. Radiographs are typically unremarkable but MR imaging studies yield findings consistent with bone marrow edema. TOH is referred to as regional migratory osteoporosis if it travels to other joints or the contralateral hip. TOH often resembles osteonecrosis but the two conditions must be differentiated due to different prognoses and management approaches. The term TOH is often used interchangeably and synonymously with transient bone marrow edema.     

髋部暂时性骨质疏松症发病机制研究进展

髋关节暂时性骨质疏松症(transient osteoporosis of the hip,TOH)被归类为骨髓水肿综合征的一种。TOH既往研究不少,对其发病机制存在争议。近年来随着影像学、病理学、分子生物学等多学科的发展,研究发现该病发病机制较复杂且不明确,本文从神经源性、骨坏死、血管功能异常、软骨下骨折、遗传、区域性加速现象等方面对TOH的相关发病机制的研究进展进行了概括。     

A new hypothesis for the bone marrow edema pathogenesis during transient osteoporosis

Transient osteoporosis is an infrequent condition of uncertain etiology with pain, limited range of motion and radiographic evidence of osteoporosis affecting one or more joints. It is self-limited, reversible and can involve only the hip (transient osteoporosis of the hip, TOH) or, less frequently, one or more joints contemporaneously or at different times (regional migratory osteoporosis, RMO). We studied four men with transient osteoporosis, including two with TOH and two with RMO. All patients underwent a standard radiographic work-up of the affected joints, arteriovenous Doppler US, computed tomography, magnetic resonance imaging (MRI) and three-phase bone scanning. In all patients, symptoms were related to bone marrow edema demonstrated at MRI and to a transitory regional arterial hyperflow observed at the early scintigraphic analysis. On the basis of our observations, we hypothesize that regional arterial hyperflow may be the cause of the bone marrow edema and therefore of the transient osteoporosis.     

8例髋关节暂时性骨质疏松症的临床诊断与治疗

目的探讨髋关节暂时性骨质疏松症（transient osteoporosis of the hip,TOH）的临床表现、诊断、鉴别诊断、治疗及预后。方法2003年8月至2009年8月治疗8例TOH患者,男2例,女6例;年龄22—43岁,平均34．9岁。患者均为单侧发病,左侧5例,右侧3例。患者的患髋均表现有不同程度的突发急性疼痛或渐进性疼痛,同时伴有显著的跛行。患髋内旋轻度受限是主要的体征。症状出现后3—6周,x线片可见股骨头均匀广泛的骨质疏松,MR表现为弥散且一致的等低T1、长T2异常信号,脂肪抑制序列呈高信号。嘱患者在疼痛可忍受的条件下半负重行走,避免出现骨折等并发症;口服二膦酸盐和钙剂;剧烈疼痛时,可口服非甾体类抗炎药物缓解疼痛。结果临床治疗3个月后疼痛明显减轻,10个月后疼痛完全缓解,复查MR示病变完全消失。结论TOH是一种少见的疾病,无原因出现的髋关节疼痛和跛行是主要症状,X线片和MR可分别看到暂时性的骨质疏松和骨髓水肿。此病具有自限性,保守治疗可获得良好的结果,明确诊断是治疗的关键。     

Tyrosine hydroxylase activity in sympathetic nervous system of rats with streptozocin-induced diabetes

The activity of tyrosine hydroxylase (TOH), the rate-limiting enzyme in norepinephrine biosynthesis, was measured in selected sympathetic ganglia to develop a quantitative measure of sympathetic autonomic neuropathy in streptozocin-induced diabetic rats. Surprisingly, TOH activity was elevated twofold in diabetic prevertebral ganglia innervating the alimentary tract (i.e., superior mesenteric, celiac, and inferior mesenteric), which has terminal processes that develop neuroaxonal dystrophy in this model system. TOH activity of paravertebral ganglia (i.e., superior cervical and stellate) with nonalimentary targets was not increased in the same animals. Increased TOH activity in the prevertebral ganglia 1) developed within the 1st wk of diabetes and persisted for 10 mo, 2) did not represent a change in TOH affinity for d-1,6-methyl-5,6,7,8- tetrahydropterine cofactor, 3) was prevented by both nicotinamide pretreatment and early institution of insulin therapy, and 4) did not develop as a result of classical transsynaptic induction. Pair-feeding experiments confirmed that the most likely cause of increased TOH activity in this system was the marked hypertrophy and hyperplasia of the diabetic bowel resulting from compensatory hyperphagia. We conclude that TOH activity does not represent a suitable marker for sympathetic autonomic neuropathy in this experimental system. Rather, the increase appears to be an example of a selective increase in the synthesis of neurotransmitter enzymes, possibly in response to increased trophic support provided by the expanded target, i.e., the hypertrophic gut. The additional synthetic stress imposed on prevertebral neurons by the expansion of the innervation of the alimentary target coupled with the complex diabetic metabolic milieu may contribute to the development and selective distribution of dystrophic axonopathy to the innervation of the alimentary tract.     

Transanal hemorrhoidopexy: Why it makes sense

AimTransanal open hemorrhoidopexy (TOH) was developed as an alternative to stapled hemorrhoidopexy and THD/mucopexy. The aim of this article is to review the technical aspect and results of this technique.MethodsA single coloproctology center experience with this technique was reviewed retrospectively. All consecutive patients who underwent TOH were analyzed. Short and long-term results are reported. TOH involves placing a Z-suture in the low rectum above the hemorrhoidal cushions and excising a 1 centimeter strip of rectal mucosa between the upper and lower aspects of the suture. Tightening of the suture provides an effective upward life of the hemorrhoidal bundle. The procedure is performed in 3 or 4 quadrants of the anal canal (as needed) at the point of maximal prolapse.ResultsFrom 2006 until 2013, 217 patients with 2nd and 3rd degree internal hemorrhoids were operated. Short-term complications included major bleeding in 5 patients (2%) and severe pain in 19 patients (9%). Long-term follow-up was obtained in 169 of 217 patients (78%) either through clinic visits or phone interview. Available patients were followed up for a minimum period of 60 months after TOH (median 113 months). 109 patients (64%) were symptom free and 147 patients (87%) indicated that they would consent to TOH again.ConclusionsTOH is a safe operation for hemorrhoidal prolapse with encouraging short and long-term results. It is a safe and inexpensive alternative to stapled hemorrhoidopexy or THD/mucopexy and it should be included in the treatment algorithm of patients with symptomatic hemorrhoidal prolapse.     

Radiological morphology variances of transient osteoporosis of the hip

Background

Although several morphological abnormalities or variances of the hip joint have been proposed to be associated with hip joint degeneration, few studies have investigated any radiological features in patients with transient osteoporosis of the hip (TOH). The purpose of this study is to evaluate the morphological variances of the hip joint in TOH patients radiographically.

Distinguishing transient osteoporosis of the hip from avascular necrosis.

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.     

Pregnancy-related hip diseases: incidence and diagnoses

BackgroundPregnancy-related hip diseases epidemiology has been poorly evaluated. We report our experience of gestational and postpartum hip diseases and evaluate their incidence.Methods(1) Prospective survey: all pregnant or early postpartum women suspected to have hip involvement during their follow-up in an Obstetric unit were referred to a rheumatologist. If clinically confirmed, magnetic resonance imaging (MRI) and additional investigations as needed were performed. This survey had 2 years duration. (2) Retrospective study: all cases of definite (with MRI confirmation) pregnancy-related hip disease referred to our Rheumatology unit during the past 15 years were analyzed.ResultsDuring the 2-year prospective survey, 3 patients (4 hips) of pregnancy-related hip disease were observed over 4900 pregnancies (1 case of transient osteoporosis of the hip (TOH) and 2 cases of occult fracture of the femoral head). During the 15-year retrospective study, 12 patients (17 hips) with hip diseases during pregnancy or early postpartum were identified. There were 6 patients (9 hips) with TOH, 4 patients (6 hips) with occult fracture of the femoral head, 1 patient with osteonecrosis of the femoral head, and 1 coxitis in a patient with ankylosing spondylitis. Differentiating diagnosis between TOH and occult fractures could only be made by MRI. Five of the 6 women with TOH had osteopenia at the lumbar spine at dual energy X-ray absorptiometry (DEXA). The 4 women with occult fractures had either osteopenia or osteoporosis at the lumbar spine.ConclusionHip diseases are infrequent during pregnancy and early postpartum. Transient osteoporosis of the hip and occult stress fractures of the femoral head appear the main causes and those diagnoses justify evaluation for an underlying bone fragility. Osteonecrosis is very rare in this setting.     

Intravenous pamidronate in the treatment of transient osteoporosis of the hip

The aim of this study was to evaluate the efficacy of intravenous pamidronate in patients with transient osteoporosis of the hip (TOH). Thirteen men and three women (mean age 38.3 years, range 30-49) were recruited. The diagnosis was made by means of radiographs, bone scintigraphy, and magnetic resonance imaging (MRI). Pamidronate (45 mg) was intravenously administered three times, once every third day. The outcome measures included a clinical assessment using a pain visual analog scale (VAS; range 0-100), and the WOMAC functional impairment score (FUI; range 0-100). The bone mineral density (BMD) of the total hip and femoral neck was measured using dual-energy X-ray absorptiometry (DXA). Clinical assessments were made before treatment (T(0)) and 1 month later (T(1)), and the densitometric measurements at T(0), and then after 2 (T(2)) and 4 months (T(4)). A further MRI scan was made 3 months after treatment. In comparison to the unaffected side, there was a significant decrease at T(0) in the BMD of both the total hip (median 16.6%, range 8.5%-29.1%, p < 0.00001) and femoral neck (median 22.5%, range 12.0%-34.2%, p < 0.00001). By T(1), both VAS and FUI had decreased significantly (p < 0.00001). By T(2), the total hip and femoral neck BMD had increased by 10.9% (range 2.7%-23.6%, p < 0.00001) and 12.3% (range 7.8%-26.9%, p < 0.00001), respectively, and all patients were asymptomatic. By T(3), the MRI findings had normalized in all patients and, at T(4), there was a further increase in BMD. None of the patients experienced symptom relapse during the follow-up of 39.5 +/- 17.7 months. These results suggest that a short course of pamidronate is effective in treating TOH, and leads to a prompt and long-lasting recovery.     

Fibrogenesis imperfecta ossium with early onset: observations after 20 years of illness

Fibrogenesis imperfecta ossium is a rare, acquired disorder of bone mineralization characterized by a morphologic abnormality of bone collagen that presents with bone pain and tenderness and usually results in the patient becoming bedridden. Onset of symptoms in the six previously reported cases of this disorder occurred in patients over 50 years of age. We report a case of fibrogenesis imperfecta ossium with symptoms starting at age 39 where the diagnosis was not made even after three bone biopsies because of the failure to recognize the characteristic morphologic abnormality of collagen. Elevated serum alkaline phosphatase, increased urinary hydroxyproline, and numerous osteoclasts on a bone biopsy are compatible with increased bone turnover. There was no apparent abnormality of vitamin D metabolism contributing to this disorder. Treatment with sodium fluoride, synthetic salmon calcitonin, and 24,25-dihydroxyvitamin D did not result in any apparent benefit.     

Three children with lower limb fractures and a mineralization defect: a novel bone fragility disorder?

In this report, we describe three unrelated children with an apparently novel bone fragility disorder that is associated with an idiopathic mineralization defect. Recurrent lower limb fractures started with weight bearing. The patients had none of the phenotypic, radiological, or histomorphometric features classically associated with known bone fragility disorders such as osteogenesis imperfecta (OI), idiopathic juvenile osteoporosis (IJO), or mild autosomal dominant osteopetrosis. Radiologically, there was increased metaphyseal trabeculation, normal to increased cortical thickness, and no evidence of rickets or osteomalacia. Areal and volumetric bone mineral density (BMD) of the lumbar spine did not show any major alteration. Peripheral quantitative computed tomography of the radius showed elevated cortical thickness and total and trabecular volumetric bone mineral density in one patient. Qualitative histology of iliac bone biopsy specimens showed a paucity of the birefringent pattern of normal lamellar bone. Quantitative histomorphometric analysis demonstrated osteomalacia with a prolonged mineralization lag time in the presence of a decreased mineral apposition rate. There was no biochemical evidence of abnormal calcium or phosphate metabolism. Type I collagen mutation analysis was negative. We conclude that this is a bone fragility disorder of moderate severity that tends to cause fractures in the lower extremities and is associated with the accumulation of osteoid due to an intrinsic mineralization defect. The pathogenetic basis for this disorder remains to be elucidated.     

Craniometaphyseal dysplasia associated with hydrocephalus: case report

Craniometaphyseal dysplasia is a rare bone disorder that may cause a variety of neurological abnormalities; hydrocephalus has not been observed with this disorder, however. A case of craniometaphyseal dysplasia associated with hydrocephalus is reported, and possible causes are discussed.     

Mandibular osteomyelitis and fracture successfully treated with vascularised iliac bone graft in a patient with pycnodysostosis.

Pycnodysostosis is a rare sclerosing bone disorder. Complications such as osteomyelitis and fracture of the jaws are not uncommon and difficult to treat. Treatment by reconstruction with a reconstruction plate and an iliac bone graft fails in most cases. We report a case of pycnodysostosis with osteomyelitis and fracture of the mandible that was successfully treated with vascularised iliac bone graft. We believe that this procedure is the best method for the treatment of this condition.     

Osteoporosis: differences and similarities in male and female patients

Osteoporosis is associated with decreased bone strength as a consequence of decreased bone density and altered quality. It is a result of a disruption of balance between bone breakdown and bone formation, caused by increased bone resorption by osteoclasts or without appropriate screening, one's first awareness of the disease is a fracture. It results in increased mortality and significant morbidity. In the last decade, great strides have been made in defining the diagnosis and establishing effective modes of treatment for this disorder. Our current state of knowledge indicates that although this disease affects both sexes, there are clear differences that have clinical importance.     

Regional migratory osteoporosis: a case report

We describe a case of regional migratory osteoporosis (RMO) with clinical images clearly illustrating the migratory behavior of this unusual disorder. RMO is a relatively rare disorder that manifests as rapidly developing, self-limiting, reversible osteoporosis typically seen in the lower limbs of middle-aged men. In our case, the lesion was observed migrating not only from the knee to the ankle within the same limb but also within two compartments of the same knee. To our knowledge, this is the first case showing migration of a lesion both within the same joint and to the adjacent joint. We also present computed tomography images showing characteristic spotty bone defects that are rarely described in the literature, along with magnetic resonance imaging scans revealing bone marrow edema in the affected region. Although little is understood about the etiology or treatment of RMO, knowledge of this disorder is mandatory to avoid its misdiagnosis as some other, more aggressive disease, leading to unnecessary treatment.     

股骨头缺血性坏死骨髓活性的研究

目的：股骨头缺血性坏死的病因及发病机制不明。要研究旨在探讨因非创伤所致股骨头缺血性坏死骨髓活性有无异常，揭示骨坏死发生机理。方法：对1999年9月～2000年4月间骨坏死病人骨髓成纤维样细胞集落形成单位（FCFUs）进行培养，观察其生长特点及集落数目，观察骨坏死病人与对照组之间有无差异。结果：骨坏死组骨髓成纤维样细胞集落形成单位的贴壁生长增殖速度慢于对照组，FCFUs形成集落数目与对照组相比存在明显差异。结论：非创伤性股骨头缺血性坏死存在骨髓活性下降，成骨能力降低。     

Parathyroid hormone-independent osteoclastic resorptive bone disease: a new variant of adynamic bone disease in haemodialysis patients.

BACKGROUND: Osteitis fibrosa cystica (OFC) caused by secondary hyperparathyroidism is the pre-eminent form of uraemic osteodystrophy. In recent years, however, new bone abnormalities have been described. Among them adynamic bone disease (ABD) has become a focus of growing interest. Marked suppression of dynamic bone measurements with normal or near-normal static bone-forming parameters are the hallmarks of this disorder. Depressed parathyroid hormone (PTH) levels, frequently evident in this entity, have been linked causally with low bone turnover. METHODS: We reviewed bone biopsy specimens from 96 patients with end-stage renal disease undergoing chronic haemodialysis. RESULTS: We found OFC in 50% of our patients, 20% had mixed bone disease, 24% showed bone morphology of ABD and a minority (6%) had osteomalacia, mostly due to aluminium accumulation. In the patients that were affected by ABD there was a distinct subgroup with bone morphology featuring a striking increase in osteoclast number and osteoclast surface, whereas the osteoid volume, osteoid thickness, osteoblast surface, tetracycline uptake and bone formation rates were diminished as in ordinary ABD. Similarly the PTH levels in this subgroup were low or undetectable. CONCLUSION: We describe patients undergoing chronic haemodialysis with static and dynamic bone forming parameters, indistinguishable from that of ABD, but differing from the classic ABD by the presence of increased osteoclastic bone resorption. The suppressed PTH levels in this subgroup suggests that factors other than PTH activate osteoclasts in some patients on chronic haemodialysis. Uraemic cytokines and/or toxic metabolites, including beta-microglobulin, may be involved in this disorder. The precise nature of this bone abnormality remains to be defined by further studies.     

Bone Metabolism in Anorexia Nervosa

Anorexia nervosa (AN), a psychiatric disorder predominantly affecting young women, is characterized by self-imposed, chronic nutritional deprivation and distorted body image. AN is associated with a number of medical comorbidities including low bone mass. The low bone mass in AN is due to an uncoupling of bone formation and bone resorption, which is the result of hormonal adaptations aimed at decreasing energy expenditure during periods of low energy intake. Importantly, the low bone mass in AN is associated with a significant risk of fractures and therefore treatments to prevent bone loss are critical. In this review, we discuss the hormonal determinants of low bone mass in AN and treatments that have been investigated in this population.     

Chronic kidney disease-mineral-bone disorder: a new paradigm

Disturbances in mineral and bone metabolism are prevalent in chronic kidney disease (CKD) and an important cause of morbidity, decreased quality of life, and extraskeletal calcification that have been associated with increased cardiovascular mortality. These disturbances have traditionally been termed renal osteodystrophy and classified on the basis of bone biopsy. Kidney Disease: Improving Global Outcomes (KDIGO) recently sponsored a Controversies Conference to evaluate this definition. The recommendations were that (1) the term renal osteodystrophy be used exclusively to define alterations in bone morphology associated with CKD and (2) the term CKD-mineral and bone disorder (CKD-MBD) be used to describe the broader clinical syndrome that develops as a systemic disorder of mineral and bone metabolism as a result of CKD. CKD-MBD is manifested by an abnormality of any one or a combination of the following: laboratory-abnormalities of calcium, phosphorus, PTH, or vitamin D metabolism; bone-changes in bone turnover, mineralization, volume, linear growth, or strength; and calcification-vascular or other soft-tissue calcification. The pathogenesis and clinical manifestations of these components of CKD-MBD are described in detail in this issue of Advances in Chronic Kidney Disease.