Marked Osteoporosis Research Articles

Clinico-radiological characteristics of two patients with geroderma osteodysplasticum caused by new nucleotide variants in the GORAB gene

Geroderma osteodysplasticum (OMIM #231070) is a rare autosomal recessive disorder characterized by congenital wrinkled skin on the dorsal surface of the limbs and abdomen; marked osteoporosis in childhood. Pathogenic variants of the nucleotide sequence in the GORAB gene, which encodes the GORAB protein located in the Golgi apparatus and plays a crucial role in vesicle transport in the Golgi complex, lead to the disease. This is the first report of 2 patients with this disease in Russia. A detailed description of the clinical-radiological and genetic characteristics of these patients with new nucleotide variants in the GORAB gene in a compound heterozygous state is presented: c.170C>G (p.Ser57Ter) and c.790G>C (p.Ala264Pro); c.295C>T (p.Gln99Ter) and heterozygous deletion on chromosome 1 (chr1:g.170531967–170539494del), affecting exons 1 and 2 of the gene. The main clinical manifestations of the syndrome were congenital wrinkled skin, sagging cheeks, hypoplasia of the cheekbones, prognathism, muscle hypotonia and joint hypermobility. Radiological signs included congenital bilateral dislocation of the hip, compression fractures of the vertebral bodies, and disruption of the continuity of the growth plate of the distal femur and proximal tibia, referred to as “insertion.” The rarity of the syndrome, as well as the similarity of clinical manifestations with a heterogeneous group of hereditary diseases accompanied by decreased skin elasticity, leads to a late correct diagnosis. This necessitates the description of the clinical-genetic characteristics of the disease, the study of the dynamics of the formation of its phenotypic manifestations, and the methods of molecular-genetic diagnostics.

Osteoporotic vertebral compression fractures caused by Cushing’s syndrome in young women: case report and literature review

BackgroundCushing’s syndrome is known as an important cause of secondary osteoporosis, characterized by reduction of bone mineral density and potential occurrence of fragility fractures before diagnosis in young population. Therefore, for young patients with fragility fractures, especially in young women, more attention should be paid on glucocorticoid excess caused by Cushing’s syndrome, due to relatively higher rate of misdiagnosis, distinct pathological characteristics and different treatment strategies compared with violent fractures and primary osteoporosis related fractures.Case presentationWe presented an unusual case of a 26-year-old woman with multiple vertebral compression fractures and pelvis fractures, subsequently diagnosed as Cushing’s syndrome. On admission, the radiographic results showed fresh second lumbar vertebra fracture, and old fourth lumbar vertebra and pelvic fractures. The dual energy X-ray absorptiometry of lumbar spine revealed marked osteoporosis, and her plasm cortisol was extremely high. Then, Cushing’s syndrome, caused by left adrenal adenoma, was diagnosed by further endocrinological and radiographic examinations. After receiving left adrenalectomy, her plasma ACTH and cortisol values returned to normal level. In term of OVCF, we adopted conservative treatments, including pain management, brace treatment, and anti-osteoporosis measures. Three months after discharge, the patient’s low back pain was in complete remission without new onset of pain, and returned to normal life and work. Furthermore, we reviewed the literatures on advancements in the treatment of OVCF caused by Cushing’s syndrome, and based on our experiences, proposed some additional perspectives to guide treatment.ConclusionIn term of OVCF secondary to Cushing’s syndrome without neurological damage, we prefer systematic conservative treatments, including pain management, brace treatment, and anti-osteoporosis measures, to surgical treatment. Among them, anti-osteoporosis treatment has the highest priority because of the reversibility of osteoporosis caused by Cushing’s syndrome.

Resolution of a Clinical Case of Severe Bone Deficit in a Patient with Marked Osteoporosis with the use of Growth Factors

This clinical case report describes and demonstrates how advanced modern implantology can successfully handle severe vertical and horizontal bone defects.

Inflammatory Skin-Derived Cytokines Accelerate Osteoporosis in Mice with Persistent Skin Inflammation.

Secondary osteoporosis can also be caused by chronic inflammatory skin disease as well as rheumatoid arthritis or inflammatory bowel disease. However, the exact role of osteoporosis in inflammatory skin conditions has not been elucidated. Using a mouse model of dermatitis, we investigated the pathophysiology of osteoporosis in inflammatory skin conditions and the therapeutic impact of osteoporosis medication on inflammatory skin disease. We employed model mice of spontaneous skin inflammation, specifically overexpressing human caspase-1 in the epidermis. Bone density and the expression of various mRNAs in the femur were examined by micro CT and RT-PCR. The effects of minodronate and anti-RANKL antibody on bone structure, histology, and femur blood flow were studied. The mouse model of skin inflammation showed a marked decrease in bone density compared to wild-type littermates with abnormalities in both bone resorption and formation. Minodronate improved bone density by decreasing osteoclasts, but anti-RANKL antibody did not improve. In the dermatitis model, the blood flow in the bone marrow was decreased, and minodronate restored this parameter. A model of persistent dermatitis exhibited marked osteoporosis, but the impact of chronic dermatitis on osteoporosis has not been thoroughly investigated. We should explore the pathogenesis of osteoporosis in skin inflammatory diseases.

SUN-162 Isolated Primary Pigmented Micronodular Adrenal Disease. Unilateral or Bilateral Adrenalectomy? Two Cases Report

Background: Isolated primary pigmented nodular adrenal disease (i-PPNAD) is a particular case of micronodular bilateral hyperplasia (MiBAH) leading to Cushing syndrome (CS). We present two patients with i-PPNAD who undergone unilateral adrenalectomy with different disease outcomes. Case 1: A 56 yo man presented with typical signs and symptoms of CS, including central weight gain, proximal muscle weakness, hypertension and marked osteoporosis. A biochemical investigation showed an assessment of late-night salivary cortisol - 23 nmol/l (n <9), 24h urinary free cortisol (UFC)- 1206 nmol/24h (n-60–413). The 1 mg DST showed post-DST cortisol 617 nmol/l (n<50). ACTH concentration was suppressed at 1.1 pg/ml (n-7–66). Adrenal CT-scan was normal. PPNAD was assumed and the screening for Carney complex components was negative. After left adrenalectomy, histological examination confirmed the diagnosis of PPNAD. However, biochemical remission wasn’t achieved: postoperative UFC-860 nmol/24h. Thus, the right adrenalectomy was conducted. Case 2: A 40 yo female presented with long term drug-corrected (beta-blockers, ACE inhibitor) arterial hypertension, menstrual dysfunction and clinical features of CS (central obesity, proximal muscle weakness, hirsutism), without osteoporosis. Baseline hormonal evaluation documented slightly increased level of salivary cortisol- 9,8 nmol/l (n <9), post 1 mg DST cortisol - 470 nmol/l (n<50) and ACTH-concentration below detection <1 pg/ml (n-7–66). However, UFC was normal – 215 nmol/l (n-60–413). Abdominal CT-scan revealed micronodular hyperplasia of the left adrenal and was suggestive of possible right adrenal gland hyperplasia. We performed left adrenalectomy. In the early postoperative period, adrenal insufficiency was diagnosed and thus it required hydrocortisone replacement. Discussion: We obtained two opposite results: in case 1, an expected remission after unilateral adrenalectomy was not achieved, while patient No.2 developed adrenal insufficiency, which was a more favourable prognostic sign and attested to the successful outcome of surgical treatment. A possible explanation of the ineffectiveness of surgical intervention in patient No.1 was primary more severe hypercortisolism. It is also necessary to take into account that according to the CT data of the patient No.1, both adrenal glands were within normal ranges and did not differ from one another, while in the patient No.2, the lesion was more prominent on the right side. Conclusion: Diagnostic of i-PPNAD and choice of intervention can be precarious, consequently more studies are needed to define to which patients unilateral adrenalectomy could be an efficient treatment modality. It is possible to perform unilateral adrenalectomy with subsequent assessment of cortisol levels, in case of lack of remission the contralateral adrenalectomy is required.

Тренировка дыхательной мускулатуры у пациентов с хронической обструктивной болезнью легких

Chronic obstructive pulmonary disease (COPD) is an important issue in modern healthcare. According to GOLD (2018), great attention is paid to the programs of pulmonary rehabilitation (PR), which also include respiratory muscle training (RMT). Indications for RMT. According to the latest research data, the routine use of RMT is not advised for patients with COPD who can properly participate in PR programs with a full range of necessary general physical exercises. RMT can be considered as a compound to treatment in patients with COPD who cannot fully participate in general physical training due to the presence of comorbid diseases. Contraindications for RMT. In most studies of RMT in patients with COPD, the exclusion criteria were the following: the need for long-term oxygen therapy, a recent exacerbation of the disease, severe concomitant cardiovascular or neurological pathology. Also, RMT is not recommended for patients with: 1) a history of spontaneous pneumothorax; 2) large bullae on chest x-ray films; 3) marked osteoporosis with history of rib fractures; 4) lung surgery within the last 12 months. The choice of training methods. It is recommended to use devices with an adjustable threshold applied force. Despite the fact that the use of devices with fixed inspiratory resistance is described in the literature as a possible RMT technique, the use of such devices requires special attention to breathing pattern, since a patient can reduce the training load by decreasing the inspiratory flow. Conclusion. RMT is recommended for those patients with COPD, who have significant limitations for general physical training, usually due to concomitant diseases.

High lumbar noninstrumented fusion rates using lamina autograft and Nanoss/bone marrow aspirate.

Background:Patients with marked osteoporosis and/or obesity/morbid obesity and severe multilevel lumbar stenosis and other pathology often undergo multilevel laminectomies with non instrumented posterolateral fusions (PLF). The other pathology may include combinations of degenerative spondylolisthesis/lysis, foraminal/far lateral discs, and/or synovial cysts requiring more extensive facet resections. Presently, spine surgeons often use bone graft expanders to supplement the lamina autograft harvested in the course of laminectomy/decompressions for the PLF mass.Methods:In 59 patients, we prospectively analyzed the fusion rates following multilevel laminectomies/noninstrumented fusions using lamina autograft and the bone graft expander Nanoss (RTI Surgical Alachua, FL, and USA) with autogenous bone marrow aspirate (BMA). Patients averaged 66.1 years of age; many exhibited marked osteoporosis (48 patients) and obesity (13 of 27 morbidly obese). Magnetic resonance (MR) and computed tomography (CT) studies documented stenosis/ossified yellow ligament (OYL) and degenerative spondylolisthesis (51 patients)/lysis (2 patients), synovial cysts (32 patients), and disc herniations (10 of 21 far lateral). Patients were followed remove up for an average of 3.12 years.Results:Average 4.0 level laminectomies/1.2 level noninstrumented fusions utilized lamina autograft and Nanoss/BMA. Both X-ray/CT studies performed an average of 4.9 months postoperatively documented a 97% fusion rate (57 of 59 patients). Two patients with severe osteoporosis, morbid obesity, and smoking histories exhibited pseudarthroses; neither was sufficiently symptomatic to require secondary surgery.Conclusions:Fifty-nine patients with multilevel lumbar stenosis/OYL and other pathology underwent multilevel lumbar laminectomies/noninstrumented fusions using lamina autograft and Nanoss/BMA. Both dynamic X-ray/CT studies confirmed a 97% fusion rate an average of 4.9 months postoperatively. Nanoss/BMA contributed to a high posterolateral lumbar non instrumented fusion rate without complciations.

Russian

Osteoporosis is a metabolic bone multifactorial disease characterized by increased bone fragility due to a decrease in bone mass and microarchitectural spongy and compact bone tissues. The major complications of osteoporosis are fractures of the hip bone and vertebral bodies, the treatment of which is accompanied by significant difficulties and possible complications in the postoperative period because of the poor bone quality. Particularly acute this problem is the use of bone grafts and prostheses – marked osteoporosis can significantly reduce the osseointegration of materials. To improve the biocompatibility of the implants a lot of techniques used in modern medicine sach as modification the geometry and topology of the implant surface and deposition on the surface of biologically active molecules. One of the most studied compounds, which are used to cover the implants and independently as osteoplastic material is hydroxyapatite. The purpose of our study was to investigate changes in the periimplant area of the distal epiphysis of femur after implantation of nanocomposite material based on chitosan and hydroxyapatite. Implantation of chitosan-apatite nanocomposite material into the distal femoral epiphysis with signs of osteoporosis leads to normalization of trabecular structure and mineral saturation in periimplant zone. Given the data, the apatite-chitosan composite can be considered as the applicant for the surface modification of bone implants.

Severe Symptomatic Hypocalcemia after Denosumab Administration in an End-Stage Renal Disease Patient on Peritoneal Dialysis with Controlled Secondary Hyperparathyroidism

We report the 1 case of severe, symptomatic hypocalcemia after denosumab (RANKL inhibitor) treatment in a peritoneal dialysis patient with secondary hyperparathyroidism and osteoporosis. A 58-year-old Caucasian female has been receiving chronic ambulatory peritoneal dialysis for four years secondary to polycystic kidney disease. Laboratory studies revealed: albumin-corrected calcium 9.0 mg/dL, phosphorus 5 mg/dL, alkaline phosphatase (ALP) 58 U/L [normal, 40-105], albumin 3.4 gm/dL [normal, 3.6-5.4] and intact parathyroid hormone (PTH) 315 pg/mL [normal, 40-72]. Marked osteoporosis was noted on the DXA scan, preventing her from renal transplantation considerations. She had failed conventional medical treatment, including per os calcium, monthly ergocalciferol (50,000 units/month), activated vitamin-D analog (doxercalciferol) and Case Study British Journal of Medicine & Medical Research, 3(4): 1398-1406, 2013 1399 renal-failure adjusted alendronate (70 mg twice a month). She was started on subcutaneous denosumab 60 mg every 6 months. After her first dose, she developed a progressive drop of calcium, phosphorus, bicarbonate and magnesium, in spite of massive escalation of doxercalciferol and calcium supplementation. Hypocalcemia nadired at 6.3 mg/dL with symptomatic tetany, requiring a brief hospitalization approximately 7 weeks after denosumab treatment. Her elevated PTH rose further transiently (647 pg/mL), along with ALP (123 U/L). Bone-mineral parameters normalized approximately 3 months after denosumab administration. The observed phenomenon resembled the phenotype of “hungry bone syndrome” observed after surgical parathyroidectomy. Conclusion: Treatment decisions based on bone densitometry results alone are not transposable between patients with or without end-stage renal disease. Denosumab may lead to critical hypocalcemia in dialysis patients and further aggravate existing secondary hyperparathyroidism.

Flexible intramedullary nailing for distal femoral fractures in patients with myopathies

Distal femoral fractures are quite common in nonambulating patients with myopathies, as they present marked osteoporosis. The deterioration of preexisting knee flexion contracture is a known problem, as these fractures are usually angulated posteriorly. The goals of treatment are to reduce immobilization and bed rest to a minimum, prevent function loss, and prevent refracture. The aim of our work was to investigate if these goals can be achieved by an operative treatment with closed reduction and flexible intramedullary nailing (FIN). Six distal femoral fractures in four nonambulating patients with myopathies (three Duchenne muscular dystrophy and one nemaline myopathy) were treated with FIN between 2005 and 2011. Patient charts and radiographs were reviewed to determine if intra- or postoperative complications occurred and to detect the interval to wheelchair mobilization and hospital discharge. Pre- and postoperative knee flexion contracture was noted from the patient charts of our reeducation unit, where patients were already known preoperatively. Wheelchair mobilization without further immobilization after an interval of 2-3days was possible. No aggravation of knee flexion contracture was detected in our patient series. No complications associated to the operative treatment itself and no refractures in the follow up occurred. Our experience showed that FIN is a low invasive and sufficiently stable osteosynthesis in such fractures. Left in place, nails will reinforce mechanical stability.

Low Bone Mass in Ehlers-Danlos Syndrome

A 14-year-old girl with type-III Ehlers-Danlos syndrome (EDS) underwent a dual energy X-ray absorptiometry (DXA) evaluation of the bone mineral density (BMD) in the scoliotic lumbar spine (L-spine), which measured 0.464 g/ cm (T-score: -5.3, Z-score: -4.23) (Picture 1). The BMD was found to be 0.230 g/cm (T-score: -4.5) in the deformed distal forearm and 0.181 g/cm (T-score: -4.1) in the ultradistal forearm using single energy X-ray absorptiometry (SXA) (Picture 2). The patient was treated with teriparatide and her BMD in the distal radius thereafter consistently increased without showing any increase in the BMD in the Lspine. The total BMD was found to be 458 mg/cm (T-score: 1.2, Z-score: 1.4) at the radius-4% distal site, and the trabecular BMD was found to be 220.5 mg/cm (T-score: 0.5, Zscore: 0.6) at the same site using peripheral quantitative CT (pQCT). The volumetric BMD was found to be 71.2 mg/cm (T-score: -3.74, Z-score: -4.51) in the lordotic L-spine on QCT (Picture 3). We herein report the occurrence of marked osteoporosis in the peripheral skeleton of EDS patients as an additional, yet heretofore unrecognized, manifestation of

Cathepsin K and matrix metalloproteases activity in bone tissue of OXYS rats with osteoporosis development

The comparative study of activity of cysteine protease cathepsin K and matrix metalloproteases (MMPs) in bone tissue of accelerated senescent OXYS rats with early ageing comparatively to Wistar rats of the same age was performed. Early development osteoporosis is a typical feature of OXYS rats. In bone tissue of 3 month old OXYS rats, before appearance of osteoporosis manifestation cathepsin K activity was higher, whereas MMPs activity was lower than in Wistar rats. In Wistar rats (3 and 14 months old) cathepsin K activity of spine was shown to increase, and MMPs activity to decrease. In OXYS rats age-related change of cathepsin K and MMPs activity in bone tissue had the opposite direction. As a result of this there were no differences between Wistar and OXYS rats 14 months old despite the marked osteoporosis in OXYS rats as revealed our early researches. Serum Q2-macroglobulin activity was higher in 14 months old OXYS rats. The role of activation of cathepsin K in bone resorption in the development of osteoporosis in early ageing OXYS rats is discussed.

Das Kallmann-Syndrom – eine Form des hypogonadotropen Hypogonadismus

Two men (aged 44 and 47 years) were admitted with the diagnosis of Kallmann's syndrome, but they had widely different symptoms. The first patient (A) suffered from significant secondary symptoms, i.e. weight gain and skeletal pain, which had caused him to seek medical assistance. The second patient (B) had been admitted because of symptoms of depression, caused by separation from his female partner.Patient A showed major somatic symptoms characteristic of the syndrome: eunuchoidism (physique, boyish voice, gynecomastia, micropenis and absent secondary sex characteristics). Further diagnostic tests revealed low testosterone concentrations (0.958 ng/ml; reference range 1.8 - 7.58 ng/ml) and low luteinizing hormone (LH) concentrations (<0.7 IU/l; reference range 0.8 - 7.6 IU/l), as well as infertility. Radiology showed marked osteoporosis, providing the indication for total hip replacement. Magnetic resonance imaging (MRI) of the skull and chromosomal analysis gave normal results. The physical development of patient B had progressed ever since hormone substitution after a suspected diagnosis of mumps orchitis in early childhood. However infertility was still present. Abnormal laboratory findings at admission: LH 0.10 IU/l (reference range 0.8 - 7.6 IU/l), follicle stimulating hormone 0.10 IU/l (reference range 1.2 - 10.1 IU/l), testosterone 10.0 ng/ml (reference range 1.8 - 7.58 ng/ml). A hypoplastic olfactory sulcus was shown by MRI, but no olfactory bulb. Mineral density of the femur was slightly diminished. Combined stimulation test of the pituitary gland revealed hypogonadotropic hypogonadism and anosmia in both patients.In patient A administration of testosterone, calcium, colecalciferol and biphosphonates improved virilization and reduced skeletal pain. Continuation of testosterone, calcium and colecalciferol treatment, psychotherapy and antidepressive medication with paroxetine were initiated in patient B. His symptoms of depression were treated successfully, but personal and sexual relationships remained difficult and had many problems.Early diagnosis of Kallmann's syndrome and symptomatic treatment with hormone replacement prevent patients from developing pernicious sequelae. However, sexual identity will be difficult even for patients treated after early diagnosis.

THE CLASSIC: Intrapelvic Protrusion of the Acetabular Component Following Total Hip Replacement

THE CLASSIC: Intrapelvic Protrusion of the Acetabular Component Following Total Hip Replacement

Primary hip stem stability: the effect of bone pathology on micromotion

The effect of bone quality on the success of hip arthroplasty remains a topic of debate. Skeletal disease such as osteoporosis cause a significant loss of cancellous bone stock and structural deterioration of bone tissue. The reduced bone quality affects the decision made by surgeons in terms of selection of suitable hip stem as osteoporosis increases the likelihood of fracture and instability. It has been suggested that patients with poor bone stock would be better served having cemented hip stems to ensure strong primary fixation. However, there are reports that also cementless stems are reliable for patients suffering osteoporosis. A finite element model in conjunction with a novel methodology for predicting hip stem stability was experimentally validated in a previous study. In this study the methodology was applied to two ct datasets from osteoarthritic patients about to undergo hip replacement. Based on DEXA scans of the two patients, the Young Adult T-score showed in one case marked osteoporosis in all regions of the femur. 3D models were constructed from these two CT datasets and the material properties were assigned based on the grey-scale values on an element-by-element basis. A third femur model was created as ‘non-pathological’ control using the Visible Human Project (VHP) CT dataset. During the analyses, all interfacial contacts on surfaces with micromotion larger than a threshold limit for bone ingrowth of 50 mikro meter were removed, and the iteration continues until either a stable-state condition is achieved or instability occurred. The results showed that the stems fixed in the control and osteoarthritic bones were stable. For the osteoporotic bone, the stem was found to be unstable due to a progressive reduction in surface area feasible for bone ingrowth. The results showed that bone quality affects the stability and therefore the potential success of hip stems.

Outcome measures of bipolar hip arthroplasty for atraumatic hip disorders -A preliminary report

Background: Bipolar hip arthroplasty was devised for fracture neck femur in elderly patients. Subsequently, indications have been expanded to include conditions affecting acetabulum like rheumatoid arthritis, osteoarthritis and avascular necrosis of femoral head. Materials and methods: We have studied the results of bipolar hip arthroplasty in 38 such patients, with severely involved acetabulum due to rheumatoid arthritis, avascular necrosis of femoral head and primary osteoarthritis. Acetabulum was reamed to get a tight 'equatorial' or 'rim' fit of the prosthesis. Prosthesis selected was 1 mm bigger than the maximum size of reamer used. Cement was used in femur whenever there was marked osteoporosis or wide medullary canal. Post operatively all patients were regularly screened for pain, range of movement, protrusio acetabuli, loosening / sinking of prosthesis and radiographic assessment of movement in the two bearings of prosthesis. Results: Overall results achieved were good to excellent in 80% of patients. Conclusions: The ultimate outcome is comparable to total hip arthroplasty. The added advantage is of low cost, simplicity of procedure and easy future revision.

Estrogen Receptor (ER) Gene Polymorphism May Predict the Bone Mineral Density Response to Raloxifene in Postmenopausal Women on Chronic Hemodialysis

The estrogen receptor (ER) gene has been considered as a candidate genetic marker for osteoporosis, and PvuII and XbaI polymorphisms of the ERα gene have been associated with low bone mineral density (BMD). We investigated whether ER polymorphism could predict the response of BMD in 28 postmenopausal women on hemodialysis with marked osteopenia or osteoporosis, randomized to receive raloxifene, a selective estrogen receptor modulator (SERM), or placebo for 1 year. BMD was assessed by dual X-ray absorptiometry and PvuII and XbaI restriction fragment-length polymorphism of the ER gene was determined using polymerase chain reaction. Baseline lumbar spine or femoral neck BMD parameters were not different between patients presenting either homozygous PP or xx when compared with heterozygous Pp or Xx genotypes. After 1 year, patients on raloxifene, presenting with PP or xx genotypes (but not those with Pp or Xx), showed a significantly higher mean lumbar spine BMD (0.942 ± 0.18 vs. 0.925 ± 0.17 g/cm2, p < .01) and lower serum pyridinoline (19.7 ± 9.7 vs. 30.6 ± 16.5 nmol/L, p < .02) when compared with baseline values. No changes were detected in the placebo-treated patients or in the femur neck sites. In conclusion, after 1 year on raloxifene, postmenopausal osteoporotic women on chronic hemodialysis, homozygous for the P or x (PP or xx) alleles of the ER, exhibited a better lumbar spine BMD response and decreased serum pyridinoline values when compared with heterozygous women (Pp or Xx), suggesting that ERα allelic variants may explain, at least in part, the different outcomes after treatment of osteoporosis with SERM.

A case of systemic mastocytosis associated with severe osteoporosis and pathologic fractures

We describe the case of a 56-year-old man with rapidly progressive osteoporosis culminating in pathologic fractures of multiple lumbar vertebrae. With the exception of discrete brown-reddish cutaneous lesions, low back pain was the only prominent symptom. Laboratory evaluations excluded metabolic or endocrinological abnormalities. Serum tryptase was highly increased, as was urinary excretion of histamine. Iliac crest biopsy demonstrated mastocytosis, which was confirmed by skin biopsy and which was consistent with teleangiectasia macularis eruptiva perstans, a rare form of cutaneous mastocytosis. In cases of unusually marked osteoporosis, especially in men, mastocytosis should be considered in the differential diagnosis.

Treatment of Proximal Femur Fractures on the Background of Osteoporosis

Analysis of treatment of 526 patients with proximal femur fractures (362 patients with femoral neck fractures and 164 patients with trochanteric zone fractures) was performed. Patients' age ranged from 34 to 92 (mean 67). Algorithm of differentiated choice of operative tactics depending on fracture characteristics and injury term was presented. Low invasive osteosynthesis of femoral neck with bundle of stressed V-shaped pins was suggested. Theoretical and practical ground of that technique was given. Stress within fixative-bone system created after osteosynthesis by V-shaped pins, provided stable fixation even in marked osteoporosis. The importance of bone mineral density evaluation for the choice of treatment tactics as well as the necessity of medicamental correction of the disturbed bone remodeling after surgical treatment was noted.

Unilateral hinged external fixator of the elbow in complex elbow injuries

We present the outcome of treating complex injuries of the elbow with the “Orthofix” external fixator in patients where the condition of the soft tissues did not permit extensile surgical approach or where the internal fixation would not be stable enough to permit safe early joint mobilization postoperatively. Eight patients were treated, three with supra-intercondylar fracture in the presence of marked osteoporosis and five with fracture–dislocation of the elbow and excessive soft tissue impairment. The fixator was applied for a mean of 8.5 weeks (5–13 weeks). Great care was taken to use the appropriate technique in applying the fixator, especially to define the correct centre of rotation. All the fractures were united, and no instability of the joint was noted. All the patients maintained a functional range of motion, with lack of extension less than 30° and flexion more than 120°. One patient who developed transient palsy of the radial nerve and two patients who developed pin track infection were treated with oral antibiotics. Despite the technical difficulties, external fixation of the elbow could be a salvage procedure in difficult cases of elbow trauma, where open procedures are not indicated.