A woman with multicentric reticulohistiocytosis, having symptoms including bleeding and a borderline level of factor VIII, was given an i.v. infusion of DDAVP as a pharmacological test to evaluate the release of factor VIII-related properties from storage sites. The results show a reduced response to DDAVP in the patient in comparison with a normal control subject. This could be due either to a defect in the release of the factor VIII complex after DDAVP or to a reduced synthesis and/or storage capacity by the storage cells.

Adolescent↗

PubMedDate not supplied

Humeral head osteonecrosis caused by electrical injury: a case report.

Various musculoskeletal abnormalities caused by electrical injury are described. Such abnormalities usually include fractures or dislocation of adjacent bones and joints. Osteonecrosis is a noteworthy, but less common, consequence of electric shock. The case is discussed of a 52-year-old woman who had received an electric shock (220-V alternating household current) to the right hand and developed osteonecrosis in the ipsilateral humeral head, most likely caused by bone "melting." An osteonecrotic lesion may therefore develop in a joint at a distance from the point of electrical contact, and this must always be kept in mind in diagnosis and treatment.

Electric Injuries↗

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[Autologous stem cell transplantation in a patient with diffuse systemic sclerosis].

Systemic Sclerosis (SSc) is a systemic disease of unknown etiology presenting with disseminated skin thickening and fibrotic impairment of various organs including lung and kidney. According to the rate and degree of skin involvement, SSc can be classified in a limited and a diffuse form, the latter showing a severe and progressive lung involvement, which is responsible for its high related morbidity and mortality along with resistance to standard therapeutic protocols. High dose chemotherapy, followed by autologous stem cell transplantation, is a standard therapeutic regimen for haematological diseases: re-infusion of mobilised peripheral blood progenitor cells overcomes the myeloablative effect of super-maximal eradicative doses of chemotherapeutic agents. Recently, this therapeutic approach has been applied in some cases of resistant SSc and, albeit the low number of cases, it has been proven effective in early diagnosed and rapidly progressive forms of the disease showing a clinical improvement and an instrumentally detectable decrease of fibrosis extent. We report the case of a young woman affected by diffuse SSc with a rapid progression of clinical signs and instrumentally detectable lesions who underwent a conditioning regimen with fludarabine, cyclophosphamide and anti-thymoglobulines followed by re-infusion of autologous peripheral blood stem cells. Two years after transplantation a clinical and instrumental evidence of treatment was observed, with good control of disease evolution. The only sign of disease resumption was a slow worsening of skin involvement.

Adult↗

PubMedDate not supplied

[The evaluation of neuropsychiatric lupus erythematosus by functional neuroimaging. Preliminary results].

OBJECTIVE: The aim of this study was to investigate the performance of functional neuro-imaging for describe neurological involvement in Lupus Erithematosus Systemicus. METHODS: 20 SLE patients were included into this study (18 females / 2 males). Median age was 40.5 years (range 16 -66 ys), 9 patients with a clear neurological involvement, 7 with aspecific neurological symptoms and 4 were asymptomatics, according to 1999 ACR Classification. All patients were underwent to conventional resonance imaging (RM-FLAIR), RM perfusion ( RM-PWI), RM diffusion (RM-DWI) and cerebral SPECT. The RM techniques was performed using a 1 Tesla "Signa-Horizon" Tomograph by General Electric: The data analysis was performed from two independent neuroradiologist and than trough coordinated evaluation after coregistration of acquired volumes. RESULTS: In 11/20 patients (55%) lesions were demonstrated in RM-FLAIR evaluation, more frequent in cases with focal symptoms than in diffuse. RM-PWI was positive in 50% of cases. SPECT analysis was altered in 85 % of patients. In all patients RM-DWI evaluation was negative. 5 of 29 lesional areas (3 patients) showed by SPECT analysis were positive in RM-PWI. None of them was positive in RM-FLAIR study. After coordinated evaluation of RM-FLAIR, SPECT and RM-PWI, 7 findings were considered false positive. 6 Of patients with negative RM-FLAIR were positive in SPECT and 3 in RM-PWI. Only 1 patient was positive in SPECT and RM-PWI. CONCLUSIONS: According to the literature, the RM-FLAIR is a very sensitive procedure to describe the lesional charge, especially in patients with focal symptoms. All lesions was considered as stable outcomes due to negativity of RM-DWI analysis. The SPECT is a sensitive technique to individuate cerebral areas of altered perfusion. The coregistration seems to be an helpful method to improve the explanation of uncertain cases. e the sections are prepared for the microscopic analysis of the various histomorphometric parameters.

Adolescent↗

PubMedDate not supplied

[Pain patterns in Italian patients with osteoarthritis: preliminary results of the MI.D.A. Study (Misurazione del Dolore nell'Artrosi)].

OBJECTIVES: To evaluate the characteristics of pain in a cohort of Italian patients with osteoarthritis (OA) of the hip and knee. METHODS: The 657 general practitioners participating in the study were asked to enroll 10 consecutive patients with OA diagnosed according to the American College of Rheumatology (ACR) clinical criteria. A questionnaire evaluating demographic data, clinical characteristics of OA, including the "Questionario Semantico Reumatologico" (QSR) pain questionnaire, the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) and the Lequesne indices, and information on previous diagnostic and therapeutic interventions was administered. RESULTS: A total of 4,109 patients were enrolled. Of them, 2356 were affected by knee OA and 1817 by hip OA. There were 2863 (69.7%) women and 1246 (30.3%) men. Median age was 68.2 years (range 50-103 years). Of the 4109 enrolled subjects, 3128 (76.1%) reported one or more medical comorbidities, mostly cardiovascular (52.7%), endocrinological (14.7%), gastrointestinal (13.4%), and respiratory (11.2%) disorders. The median pain visual analogue scale (VAS) score was 58.1+/-22.6 mm, higher in women (60.2+/-22.3 mm) than in men (53.3+/-22.6mm) (p<0.00001). OA pain was also higher in patients from Southern Italy (p<0.00001). NSAIDs were administered to nearly 70% of patients, COX-2 inhibitors to 55%, disease-modifying anti OA drugs to 19% and analgesics to 28.2%. Differences in drugs utilization were associated with OA localization and patient's geographical origin. Results of the WOMAC index were similar throughout groups. Responses to the QSR pain questionnaire showed differences, which are related to OA localization and geographical origin of the patients. CONCLUSIONS: The MI.D.A. study can help to better understand the patterns of pain in osteoarthritis and the associated treatment.

Age Factors↗

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[Transient regional osteoporosis].

Transient osteoporosis of the hip and regional migratory osteoporosis are uncommon and probably underdiagnosed bone diseases characterized by pain and functional limitation mainly affecting weight-bearing joints of the lower limbs. These conditions are usually self-limiting and symptoms tend to abate within a few months without sequelae. Routine laboratory investigations are unremarkable. Middle aged men and women during the last months of pregnancy or in the immediate post-partum period are principally affected. Osteopenia with preservation of articular space and transitory edema of the bone marrow provided by magnetic resonance imaging are common to these two conditions, so they are also known by the term regional transitory osteoporosis. The appearance of bone marrow edema is not specific to regional transitory osteoporosis but can be observed in several diseases, i.e. trauma, reflex sympathetic dystrophy, avascular osteonecrosis, infections, tumors from which it must be differentiated. The etiology of this condition is unknown. Pathogenesis is still debated in particular the relationship with reflex sympathetic dystrophy, with which regional transitory osteoporosis is often identified. The purpose of the present review is to remark on the relationship between transient osteoporosis of the hip and regional migratory osteoporosis with particular attention to the bone marrow edema pattern and relative differential diagnosis.

Diagnosis, Differential↗

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[Diffuse idiopathic skeletal hyperostosis (D.I.S.H.)].

Diffuse idiopathic skeletal hyperostosis (D.I.S.H.) is a common disorder of unknown aetiology characterized by exuberant hyperostosis of the antero-lateral aspect of the spinal column, that sometimes leads to bone ankilosis, and by ossification of extra-spinal entheses. This condition is often associated with the metabolic derangement of type 2 diabetes. Primary hypertension, its cardiovascular aftereffects and lithiasis are also often present in these patients. D.I.S.H. has to be distinguished from osteoarthritis, although they often coexist in the same patient. The mean difference lies in the anatomical target of the pathological process, that is represented by articular cartilage in osteoarthritis and by entheses in diffuse idiopathic skeletal hyperostosis. The enthesopathy leads to the ossification of the anterior longitudinal ligament of the spine and causes the formation of flowing osteophytes, while intervertebral disc space is quite preserved in early phases of the disease. Symptoms of spine involvement are not typical of the disease and consist of pain and stiffness, usually worsened by inaction and damp. It has also been described the ossification of posterior longitudinal ligament which can lead to medullary canal stenosis. Appendicular skeleton is symmetrically involved in early phases of the disease, the most distinctive affected sites being feet, olecranus and patella. Hip involvement is also frequent and may lead to severe disability and represents an important cause of invalidity. The purpose of the present review is to remark on aetiopathogenetic and clinical aspects of diffuse idiopathic skeletal hyperostosis.

Humans↗

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Elderly onset rheumatoid arthritis: clinical aspects.

The presentation, severity and prognosis of rheumatoid arthritis (RA) differ depending on the age of disease onset. Elderly onset RA (EORA: age of onset > 60 years) has been reported to differ from younger-onset RA (YORA) by a more balanced gender distribution, a higher frequency of acute onset often associated with systemic features, more frequent involvement of the shoulder girdle and higher disease activity. To add to our knowledge of this disease, 101 EORA and 88 YORA patients, not previously treated with DMDARs or steroids, were studied and compared, paying particular attention to the onset. The female to male ratio was higher in the YORA group (4.4:1 vs 1.6:1; p < 0.05). The disease duration was similar: 5.6 +/- 3.3 months in EORA and 7.9 +/- 3.8 months in YORA. EORA presented a more frequent acute onset (33.6% vs 13.6%; p < 0.05) especially if rheumatoid factor was absent. This subset also showed more frequent polymyalgic onset. Constitutional symptoms (fever, weight loss, fatigue) were more frequent in EORA patients without differences between seropositive and seronegative patients. The distribution of involved joints showed a significantly higher frequency of shoulder involvement in EORA (64% vs 38%; p < 0.05) and of feet involvement in YORA (25% vs 52%; p < 0.05). Hands and wrists were the most frequently involved joints in all patients.

Age Distribution↗

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Evaluation with MR, perfusion MR and cerebral flowSPECT in NPSLE patients.

PURPOSE: To compare the performance of MR, cerebral flow SPECT and perfusion MRI (PWI) in NPSLE patients by using image co-registration. MATERIALS AND METHODS: 20 SLE patients underwent MR (T2-weighted FLAIR), perfusion and diffusion MR, and SPECT (after ( 99m)Tc-HMPAO intravenous injection). Two experienced operators analysed the images both independently and jointly after multi-modal volumetric co-registration ("Statistical Parametric Mapping" software, based on the Maximization of mutual information method). RESULTS: The FLAIR examination depicted 82 small lesions in 11/20 patients. Perfusion SPECT showed 43 hypoperfused areas in 17/20 patients. PWI showed 13 hypoperfused areas in 10/20 patients. After co-registration of images, anatomical agreement between SPECT and PWI was found in 10 hypoperfused areas (8 patients). Co-registration with FLAIR showed some false positives, more frequent in SPECT (9/43 areas) than in PWI (2/13 areas). DISCUSSION AND CONCLUSIONS: The FLAIR examination confirmed its high sensitivity in detecting lesions. Perfusion SPECT confirmed high sensitivity for the detection of hypoperfused cerebral areas. PWI showed fewer areas of cerebral hypoperfusion than did SPECT. The disagreement between SPECT and PWI may be related to the different modalities for disease detection. The anatomical agreement of hypoperfused areas between SPECT and PWI, assessed after co-registration, can suggest the prognostic hypothesis of delayed appearance of permanent parenchymal lesions. Co-registration modality, being able to show some false positives, seems to be a valid support for the interpretation of SPECT and PWI findings; the joint analysis of SPECT and PWI highlighted the capability of PWI for the interpretation of uncertain cases.

Adult↗

PubMedDate not supplied

Beta-thalassaemic trait and systemic lupus erythematosus.

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Adult↗