Myositis ossificans: magnetic resonance images

International Nuclear Information System (INIS)

Dosda, R.; Marti-Bonmati, L.; Concepcion, L.; Galant, J.

1999-01-01

Myositis ossificans is characterized by a benign, self-limiting, ossifying mass of the white tissue. In the present report, we describe the magnetic resonance (MR) images in three cases of myositis ossificans in pediatric patients, correlating the MR findings with those obtained with other radiological studies. The lesions were detected in three patients, two boys and one girl, ranging in age between 10 and 14 years. The nature of the lesion was confirmed histologically in all three cases. The MR images were obtained using superconductive units at 0.5 Teslas, with T1 and T2-weighted spin-echo and STIR sequences. In two patients, gadolinium-enhanced T1-weighted images were also obtained. As in any process of maturation, the proliferation/maturation ratio depends on the moment in the course of the lesion, which affects its MR features,. In acute phases, the soft tissue mass with an intraosseous, perilesional adematous reaction predominates, while annular calcification and lesser edema are characteristic of subacute episode. Myositis ossificans is very rare in children. The inflammatory response may present a radiological pattern difficult to distinguish from that of aggressive tumor or infection, especially in the acute phase. (Author) 7 refs

Adductor muscle pyo-myositis simulating appendicitis: CT and MR imaging findings

International Nuclear Information System (INIS)

Coumbaras, M.; Le Hir, P.; Jomaah, N.; Arrive, L.; Tubiana, J.M.

2001-01-01

Pyo-myositis is a primary bacterial infection of skeletal muscle. This infection tends to occur in the large muscles of the lower extremity. Pyo-myositis of the proximal muscles of the thigh can simulate acute abdominal disease. Early diagnosis improves the outcome. Delayed diagnosis may lead to septicemia and shock. We report the CT and MRI findings in a patient with pyo-myositis of the proximal muscles of the thigh. (authors)

Myositis Mimics.

Science.gov (United States)

Michelle, E Harlan; Mammen, Andrew L

2015-10-01

Patients with autoimmune myositis typically present with muscle weakness, elevated serum levels of muscle enzymes, and abnormal muscle biopsies. However, patients with other acquired myopathies or genetic muscle diseases may have remarkably similar presentations. Making the correct diagnosis of another muscle disease can prevent these patients from being exposed to the risks of immunosuppressive medications, which benefit those with myositis, but not those with other types of muscle disease. Here, we review some of the most common acquired and inherited muscle diseases that can mimic autoimmune myositis, including inclusion body myositis, limb girdle muscular dystrophies, metabolic myopathies, mitochondrial myopathies, and endocrine myopathies. We emphasize aspects of the medical history, physical exam, laboratory evaluation, and muscle biopsy analysis that can help clinicians distinguish myositis mimics from true autoimmune myositis.

Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature.

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Binns, E L; Moraitis, E; Maillard, S; Tansley, S; McHugh, N; Jacques, T S; Wedderburn, L R; Pilkington, C; Yasin, S A; Nistala, K

2017-10-31

Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation. Three new patients are reported. All 3 cases presented with profound, rapid-onset, proximal myopathy and markedly raised CK, but no rash. Histology revealed a destructive myopathy characterized by scattered atrophic and necrotic fibres with little or no inflammatory infiltrate. All 3 patients responded to induction with cyclophosphamide, IVIG and rituximab, in conjunction with intensive physiotherapy and methotrexate as the maintenance agent. Our patients regained near-normal strength (MMT > 70/80), in contrast with the current literature where >50% of cases reported severe residual weakness. A literature search on paediatric anti-SRP myositis was performed to June 2016; PubMed was screened using a combination of the following terms: signal recognition particle, autoantibodies, antibodies, myositis, muscular diseases, skeletal muscle, childhood, paediatric, juvenile. Articles in a foreign language were excluded. Nine case studies were found. This paper supports the hypothesis that anti-SRP myositis is distinct from other JIIM. It is an important differential to JDM and should be considered where there is severe weakness without rash or if highly elevated muscle enzymes (CK > 10,000 U/l) are found. Early identification is essential to initiate aggressive medical and physical therapy. Greater international collaboration and long-term follow-up data is needed to establish the most effective treatment strategy for this rare group of patients.

Ultrasound and MR imaging of acute myositis

International Nuclear Information System (INIS)

Schedel, H.; Reimers, C.D.; Vogl, T.; Lissner, J.

1992-01-01

Ultrasound and MR imaging are both methods suitable for imaging neuromuscular diseases; however, contrast media like Gd-DTPA are, to our knowledge, not used so far. In this article we report about our experience of the use of Gd-DTPA in imaging myositis. (orig.)

Purulent myositis of the thigh as a presentation of perforated low rectal cancer.

Science.gov (United States)

Jenkins, V; Steinke, J; Rajendran, N; Kumar, D

2018-03-01

Purulent myositis is an acute, intramuscular bacterial infection involving abscess formation most commonly affecting the quadriceps, hamstring and gluteal muscles. We present a case of extensive purulent myositis of the thigh and lower leg caused by bowel perforation below the peritoneal reflection secondary to rectal cancer. Cases of lower limb and perineal purulent myositis should raise suspicion of rectal perforation and should prompt investigations to exclude rectal malignancy.

NONBACTERIAL MYOSITIS

OpenAIRE

Crum-Cianflone, Nancy F.

2010-01-01

Infectious myositis is defined as an infection of a skeletal muscle. Infectious myositis is most commonly caused by bacteria; however, a variety of viral, parasitic, and fungal agents may also cause myositis. The pathogenesis of nonbacterial infectious myositis is via direct infection of the musculature or immune mechanisms. Symptoms typically include muscular pain, tenderness, swelling, and/or weakness. The diagnosis of the specific microbe is often suggested by the presence of concordant cl...

Focal myositis

International Nuclear Information System (INIS)

Kransdorf, M.J.; Temple, H.T.; Sweet, D.E.

1998-01-01

Focal myositis is a pseudotumor of soft tissue that typically occurs in the deep soft tissue of the extremities, and is a relatively rare lesion. There is a wide clinical spectrum, with approximately one-third of patients with focal myositis subsequently developing polymyositis, and clinical symptoms of generalized weakness, fever, myalgia, and weight loss, with elevation of creatine phosphokinase. We report the case of a patient with focal myositis who subsequently developed myositis ossificans-like features. (orig.)

Focal myositis

Energy Technology Data Exchange (ETDEWEB)

Kransdorf, M.J. [Saint Mary`s Hospital, Richmond, VA (United States). Dept. of Radiol.]|[Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC (United States); Temple, H.T. [Department of Orthopedic Surgery, University of Virginia Health Sciences Center, Charlottesville, Virginia (United States)]|[Department of Orthopedic Pathology, Armed Forces Institute of Pathology, Washington, DC (United States); Sweet, D.E. [Department of Orthopedic Pathology, Armed Forces Institute of Pathology, Washington, DC (United States)

1998-05-01

Focal myositis is a pseudotumor of soft tissue that typically occurs in the deep soft tissue of the extremities, and is a relatively rare lesion. There is a wide clinical spectrum, with approximately one-third of patients with focal myositis subsequently developing polymyositis, and clinical symptoms of generalized weakness, fever, myalgia, and weight loss, with elevation of creatine phosphokinase. We report the case of a patient with focal myositis who subsequently developed myositis ossificans-like features. (orig.) With 3 figs., 25 refs.

Myositis

Science.gov (United States)

Myositis means inflammation of the muscles that you use to move your body. An injury, infection, or ... weakness, plus a skin rash. Other symptoms of myositis may include Fatigue after walking or standing Tripping ...

Myositis-specific autoantibodies: an important tool to support diagnosis of myositis.

Science.gov (United States)

Betteridge, Z; McHugh, N

2016-07-01

The idiopathic inflammatory myopathies are characterized by muscle weakness, skin disease and internal organ involvement. Autoimmunity is known to have a role in myositis pathogenesis, and myositis-specific autoantibodies, targeting important intracellular proteins, are regarded as key biomarkers aiding in the diagnosis of patients. In recent years, a number of novel myositis autoantibodies including anti-TIF1, anti-NXP2, anti-MDA5, anti-SAE, anti-HMGCR and anti-cN1A have been identified in both adult and juvenile patients. These autoantibodies correlate with distinct clinical manifestations and importantly are found in inclusion body, statin-induced, clinically amyopathic and juvenile groups of myositis patients, previously believed to be mainly autoantibody negative. In this review, we will describe the main myositis-specific and myositis-associated autoantibodies and their frequencies and clinical associations across different ages and ethnic groups. We will also discuss preliminary studies investigating correlations between specific myositis autoantibody titres and clinical markers of disease course, collectively demonstrating the utility of myositis autoantibodies as both diagnostic and prognostic markers of disease. © 2015 The Association for the Publication of the Journal of Internal Medicine.

NONBACTERIAL MYOSITIS

Science.gov (United States)

Crum-Cianflone, Nancy F.

2010-01-01

Infectious myositis is defined as an infection of a skeletal muscle. Infectious myositis is most commonly caused by bacteria; however, a variety of viral, parasitic, and fungal agents may also cause myositis. The pathogenesis of nonbacterial infectious myositis is via direct infection of the musculature or immune mechanisms. Symptoms typically include muscular pain, tenderness, swelling, and/or weakness. The diagnosis of the specific microbe is often suggested by the presence of concordant clinical signs and symptoms, a detailed medical/travel history, and laboratory data. For example, immunocompromised hosts have a heightened risk of fungal myositis, whereas the presence of a travel history to an endemic location and/or eosinophilia may suggest a parasitic cause. Definitive diagnosis requires detecting the organism by specific laboratory testing including serologies, histopathology, and/or cultures. Treatment entails antimicrobial agents against the pathogen, with consideration for surgical drainage for focal purulent collections within the musculature. PMID:21308520

Myositis ossificans: radiologic evaluation of two cases with diagnostic computed tomograms

International Nuclear Information System (INIS)

Zeanah, W.R.; Hudson, T.M.

1982-01-01

Although most physicians associated myositis ossificans with recent, acute trauma, only 40%-60% of patients give such a history. The appearance of a soft tissue mass without a clear history of trauma may suggest a diagnosis of sarcoma, especially because results of a biopsy of the central portion of an area of myositis ossificans may yield immature, undifferentiated tissue resembling a sarcoma. Pain and rapid growth of a mass are more usual in myositis ossificans than in sarcomas, and careful inquiry may reveal stretching injury or chronic trauma associated with normal, vigorous, physical activities. Recognizing the characteristic histologic zoning phenomenon (immature tissue centrally surrounded by more mature tissue and a peripheral shell of benign bone) during the biopsy procedure permits the correct diagnosis of myositis ossificans. Plain radiographs or conventional tomograms may reflect this histologic zoning by demonstrating the typical, mature, outer shell of bone. Although additional radiographic studies are not usually necessary, they may be obtained when the mass is suspected to be a sarcoma. In two patients computed tomographic scans clearly demonstrated well-defined, peripheral shells of mature bone, diagnostic of myositis ossificans

Pure White Cell Aplasia and Necrotizing Myositis

Directory of Open Access Journals (Sweden)

Peter Geon Kim

2016-01-01

Full Text Available Pure white cell aplasia (PWCA is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. PWCA has been associated with autoimmune, drug-induced, and viral exposures. Here, we report a case of a 74-year-old female who presented with severe proximal weakness without pain and was found to have PWCA with nonspecific inflammatory necrotizing myositis and acute liver injury on biopsies. These findings were associated with a recent course of azithromycin and her daily use of a statin. Myositis improved on prednisone but PWCA persisted. With intravenous immunoglobulin and granulocyte-colony stimulating factor therapies, her symptoms and neutrophil counts improved and were sustained for months.

Myositis ossificans within the intercondylar notch treated arthroscopically

International Nuclear Information System (INIS)

Leung, Allen H.; Desai, Panna; Rybak, Leon D.; Rose, Donald J.

2010-01-01

We present a case of intraarticular myositis ossificans in the right knee of a child. Myositis ossificans (MO), though relatively rare in childhood and even more uncommon within a joint, should be included in the differential diagnosis of an intra-articular mass when indicated by the typical clinical, radiographic, and histologic findings. An 11-year-old male presented with a history of trauma to his right knee. Four weeks after the initial injury, an MRI demonstrated evidence of an ACL rupture with a ''cystic mass'' within the intercondylar notch along the anterior surface of the torn ligament. At subsequent arthroscopy, the mass noted on MRI was removed. The histology was consistent with MO. The authors believe this to be the first case of MO in the intercondylar notch detected by MRI, treated by arthroscopy, and confirmed by histology. (orig.)

Myositis ossificans within the intercondylar notch treated arthroscopically

Energy Technology Data Exchange (ETDEWEB)

Leung, Allen H.; Desai, Panna [Hospital for Joint Diseases/New York University, Department of Pathology, New York, NY (United States); Rybak, Leon D. [Hospital for Joint Diseases/New York University, Department of Radiology, New York, NY (United States); Rose, Donald J. [Hospital for Joint Diseases/New York University, Department of Orthopedic Surgery, New York, NY (United States)

2010-09-15

We present a case of intraarticular myositis ossificans in the right knee of a child. Myositis ossificans (MO), though relatively rare in childhood and even more uncommon within a joint, should be included in the differential diagnosis of an intra-articular mass when indicated by the typical clinical, radiographic, and histologic findings. An 11-year-old male presented with a history of trauma to his right knee. Four weeks after the initial injury, an MRI demonstrated evidence of an ACL rupture with a ''cystic mass'' within the intercondylar notch along the anterior surface of the torn ligament. At subsequent arthroscopy, the mass noted on MRI was removed. The histology was consistent with MO. The authors believe this to be the first case of MO in the intercondylar notch detected by MRI, treated by arthroscopy, and confirmed by histology. (orig.)

Merits of magnetic resonance imaging (MRI) for the diagnosis of myositis ossificans circumscripta

International Nuclear Information System (INIS)

Bouchardy, L.; Garcia, J.

1994-01-01

A retrospective study of 5 cases, 4 of myositis ossificans circumscripta (MOC) and 1 of non-ossificans myositis, is presented. The clinical presentation was a painful soft-tissues swelling, and the final diagnosis was established by biopsy in 3 cases and clinical evolution in 2 cases. Different types of imaging techniques were performed: 4 MRI, 2 arteriographies, 3 Tc 99m scintigraphies, 3 US, 3 CT and plain film radiographs for all patients. MOC is a benign process (as opposed to myositis ossificans progressiva, which is an hereditary pathology with a fatal prognosis) with 3 phases of evolution: an acute or pseudo-inflammatory phase, a sub-acute or pseudo-tumoral phase and a chronic phase with a spontaneous healing. The radiologic diagnostic findings are dependent of the phase of the disease. The calcifications are seen earlier with scintigraphy than plain films, with US being less helpful. A heterogenous mass and calcifications are seen with CT. MRI allows the characterization of oedema during the acute phase and sometimes can exclude a malignant process. MRI is the best method for an early diagnosis, the differential diagnosis and to follow the evolution. (authors). 26 refs., 8 figs

Myositis-specific and myositis-associated autoantibodies in Indian patients with inflammatory myositis.

Science.gov (United States)

Srivastava, Puja; Dwivedi, Sanjay; Misra, Ramnath

2016-07-01

We aimed to study the prevalence and clinical associations of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) in a large cohort of Indian patients with idiopathic inflammatory myositis (IIM). Clinical details and serum samples were collected from patients with IIM (satisfying Bohan and Peter Criteria, 1975) and CTD-associated myositis. Sera were analysed for antibodies against SRP, Mi2, Jo1, PL7, PL12, EJ, OJ, Ro52, Ku, Pm-Scl 75 and PM-Scl 100, using immunoblot assay. The cohort comprised 124 patients with IIM (M:F = 1:3.6). Fifty-five of them had dermatomyositis (DM), 22 had juvenile dermatomyositis (JDM), 25 had polymyositis (PM) and 22 had connective tissue disease-associated myositis (CTD myositis). Mean disease duration was 10.9 months. ANA was positive in 84 (68.9 %), and MSAs in 61 (49.2 %) patients. Among MSAs, autoantibodies to Mi2, synthetase (Jo1, PL7, PL12, EJ) and SRP were present in 26 (20.9 %), 29 (23.4 %) and 6 (4.8 %) patients, respectively. Prevalence of MAAs was as follows: antibodies to Ro52 in 45 (36.3 %), Ku and PM-Scl 75 in 13 (10.5 %) and PM-Scl 100 in 5 (4 %) patients. Anti-Mi2 antibodies were positively associated with DM (21/55, 38.2 %; p < 0.0001) and pharyngeal weakness (13/34, 38.2 %; p = 0.004) and negatively associated with ILD (0/28; p = 0.001). ILD and mechanics' hands were significantly more in patients with anti-synthetase antibodies (16/28, 57 % and 14/22, 63.6 %; p < 0.0001). Four of six patients with anti-SRP antibody showed poor response to multiple drugs. Higher prevalence of anti-Mi2 is probably related to higher proportion of patients with DM. Absence of ILD in patients with anti-Mi2 antibody suggests that it may protect against ILD. In Indian population also, anti-synthetase antibodies are associated with ILD, and anti-SRP antibodies with poor response to treatment.

Myositis Ossificans.

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Walczak, Brian E; Johnson, Christopher N; Howe, B Matthew

2015-10-01

Myositis ossificans is a self-limiting, benign ossifying lesion that can affect any type of soft tissue, including subcutaneous fat, tendons, and nerves. It is most commonly found in muscle as a solitary lesion. Ossifying soft-tissue lesions historically have been inconsistently classified. Fundamentally, myositis ossificans can be categorized into nonhereditary and hereditary types, with the latter being a distinct entity with a separate pathophysiology and treatment approach. The etiology of myositis ossificans is variable; however, clinical presentation generally is characterized by an ossifying soft-tissue mass. Advanced cross-sectional imaging alone can be nonspecific and may appear to be similar to more sinister etiologies. Therefore, the evaluation of a suspicious soft-tissue mass often necessitates multiple imaging modalities for accurate diagnosis. When imaging is indeterminate, biopsy may be required for a histologic diagnosis. However, histopathology varies based on stage of evolution. The treatment of myositis ossificans is complex and is often made in a multidisciplinary fashion because accurate diagnosis is fundamental to a successful outcome. Copyright 2015 by the American Academy of Orthopaedic Surgeons.

[New insights of myositis-specific and -associated autoantibodies in juvenile and adult type myositis].

Science.gov (United States)

Váncsa, Andrea; Dankó, Katalin

2016-07-01

Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179-1184.

Magnetic resonance imaging of myositis ossificans: Analysis of seven cases

International Nuclear Information System (INIS)

De Smet, A.A.; Norris, M.A.; Fisher, D.R.

1992-01-01

Since magnetic resonance imaging (MRI) is commonly used to evaluate soft tissue masses, we analyzed eight MR examinations in seven patients with myositis ossificans to determine if typical patterns were present. One acute lesion had homogeneous intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Two subacute lesions had low signal intensity margins with slightly increased signal intensity centers on T1-weighted images and very high signal intensity on T2-weighted images. Five chronic lesions had two different patterns. All five were well-defined with low signal intensity borders. Three had signal intensity patterns characteristic of fat on T1-weighted and T2-weighted images. The other two lesions had intermediate signal intensity on T1-weighted images and slightly increased signal intensity on T2-weighted images. We conclude that typical MR appearances of myositis ossificans do exist. A low signal intensity rim is a common finding. However, these patterns are not unique to myositis ossificans and resemble those that have been reported in other lesions. It is important to be aware of the spectrum of MR findings of myositis ossificans when considering the differential diagnosis of a soft tissue mass. (orig./MG)

Global Characteristics of Childhood Acute Promyelocytic Leukemia

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Zhang, L; Samad, A; Pombo-de-Oliveira, MS; Scelo, G; Smith, MT; Feusner, J; Wiemels, JL; Metayer, C

2014-01-01

Acute promyelocytic leukemia (APL) comprises approximately 5–10% of childhood acute myeloid leukemia (AML) cases in the US. While variation in this percentage among other populations was noted previously, global patterns of childhood APL have not been thoroughly characterized. In this comprehensive review of childhood APL, we examined its geographic pattern and the potential contribution of environmental factors to observed variation. In 142 studies (spanning >60 countries) identified, variation was apparent—de novo APL represented from 2% (Switzerland) to >50% (Nicaragua) of childhood AML in different geographic regions. Because a limited number of previous studies addressed specific environmental exposures that potentially underlie childhood APL development, we gathered 28 childhood cases of therapy-related APL, which exemplified associations between prior exposures to chemotherapeutic drugs/radiation and APL diagnosis. Future population-based studies examining childhood APL patterns and the potential association with specific environmental exposures and other risk factors are needed. PMID:25445717

Inclusion-Body Myositis: Diagnosis

Science.gov (United States)

... for MDA Blog Donate Search MDA.org Close Inclusion-Body Myositis (IBM) Diagnosis As with other muscle diseases, a doctor diagnoses inclusion-body myositis (IBM) by considering the individual’s personal ...

Antibodies in juvenile-onset myositis.

Science.gov (United States)

Tansley, Sarah L

2016-11-01

Juvenile-onset myositis is a highly heterogeneous disease. Myositis-specific and associated autoantibodies provide a potential means of subdividing patients into clinically homogenous subgroups. Given the increasing availability of autoantibody testing, this review explores the phenotypes associated with different autoantibodies in juvenile-onset myositis and the potential clinical utility of autoantibody testing. Autoantibodies can be identified in 60-70% of children with myositis and the recent discovery of novel myositis-associated autoantibodies in adult patients suggests this may increase in the near future. Detailed phenotype descriptions are now known for several autoantibodies commonly identified in juvenile-onset disease. Whilst there is insufficient evidence to recommend a differential treatment approach based on autoantibody status, it is becoming increasingly clear that some autoantibody subgroups are often treatment resistant and may benefit from a more aggressive approach. The validation of nonspecialised methods for myositis-specific autoantibody detection should lead to more widely available testing. In juvenile-onset disease, this will provide detailed prognostic information and in the future may also influence approach.

Adductor muscle pyo-myositis simulating appendicitis: CT and MR imaging findings; Pyomyosite des mulcles adducteurs mimant une appendicite aigue: aspects tomodensitometriques et IRM

Energy Technology Data Exchange (ETDEWEB)

Coumbaras, M.; Le Hir, P.; Jomaah, N.; Arrive, L.; Tubiana, J.M. [Hopital Saint Antoine, 75 - Paris (France)

2001-04-01

Pyo-myositis is a primary bacterial infection of skeletal muscle. This infection tends to occur in the large muscles of the lower extremity. Pyo-myositis of the proximal muscles of the thigh can simulate acute abdominal disease. Early diagnosis improves the outcome. Delayed diagnosis may lead to septicemia and shock. We report the CT and MRI findings in a patient with pyo-myositis of the proximal muscles of the thigh. (authors)

Radiological evaluation of myositis ossificans

International Nuclear Information System (INIS)

Kwon, Yang Sook; Kim, Soo Han; Lim, Young Chae; Shin, Hyun Ja

1986-01-01

The 35 patients who have suffered from myositis ossificans were investigate for 11 years. They were post traumatic patients. They trauma was divided into 3 groups; spinal cord injury, direct injury (fracture) and mixed type involving both. We evaluated the age distribution, the frequency of myositis ossificans, predilection site, bilaterally and relation between predilection site and bilaterally to the type of trauma. The results are as follows; 1. The age distribution was highest in 4th decades (12 among 35 patients). 2. In regard to distribution of 35 patients, spinal cord injury were most common (15 cases, 43%), the fracture nextly common (14, 40%) and then mixed type (6, 17%). 3. Among 51 cases of myositis ossificans, the frequency was highest in spinal cord injury (25 cases, 49%), next was fracture (14, 27%). 4. The predilection site of myositis ossificans were thigh (18 cases, 35%), hip (17, 33%) and buttock (6, 12%). 5. In correlation between predilection site of myositis ossificans and level of spinal cord injury, hip is the most frequent site in thoracic injury. 6. The bilaterally of myositis ossificans is 39% (20 among 51 lesions).

Acute hemiplegia in childhood

International Nuclear Information System (INIS)

Okuno, Takehiko; Takao, Tatsuo; Itoh, Masatoshi; Konishi, Yukuo; Nakano, Shozo

1983-01-01

The results of CT in 100 patients with acute hemiplegia in childhood are reported here. The etiology was various: 2 patients had infratentorial brain tumors, 56 had cerebral vascular diseases, 3 had head injuries, 16 had intracranial infectious diseases, one had postinfectious encephalomyelitis, one had multiple sclerosis, 2 had epilepsy, and the diagnosis of 19 were unknown. Eleven patients had a normal CT and a good prognosis. As for the type of onset, there were patients of type 1 with fever and 42 with convulsions and unconsciousness; those of type 2 with convulsions and unconsciousness were 12, and those of type 3 without fever and convulsions were 46. This classification is assumed to be useful, as the type of onset is characteristic of the etiology. Six patients were diagnosed correctly by repeated examinations, although the first CT did not reveal any remarkable findings. Capsular infarction, occlusion of the posterior cerebral artery in acute hemiplegia in childhood, abnormal findings of the internal capsule, thalamus, and midbrain in a patient with postinfectious encephalomyelitis, and a diffuse low density in the CT of the unilateral hemisphere in the patients with acute encephalopathy and acute hemiplegia of an obscure origin have been found after the introduction of computerized tomography. (author)

Childhood Acute Lymphoblastic Leukemia

DEFF Research Database (Denmark)

Pui, Ching-Hon; Yang, Jun J; Hunger, Stephen P

2015-01-01

PURPOSE: To review the impact of collaborative studies on advances in the biology and treatment of acute lymphoblastic leukemia (ALL) in children and adolescents. METHODS: A review of English literature on childhood ALL focusing on collaborative studies was performed. The resulting article...

Acute Non-Traumatic Abdominal Pain in Childhood at Kenyatta ...

African Journals Online (AJOL)

Background The assessment and diagnosis of acute abdominal pain in childhood is clinically challenging. The epidemiologic correlates differ for different paediatric age groups and settings. Objectives To determine the clinical spectrum of acute abdominal pain in childhood at a referral Kenyan public hospital. Design

Magnetic resonance imaging of infectious myositis

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Yun, Ji Young; Kim, Jee Young; Kim, Sang Heum; Jung, Youn Ju; Cha, Eun Suk; Park, Joung Mi; Park, Young Ha [The Catholic Univ., College of Medicine, Suwon (Korea, Republic of)

1998-09-01

To describe the findings of magnetic resonance imaging in infectious myositis and to determine their value for differentiation between ruberculous and bacterial myositis. Magnetic resonance images of ten proven cases of infectious myositis (five tuberculous and five bacterial) were retrospectively reviewed in the light of clinical and laboratory findings. On the basis of magnetic resonance images, signal intensity of the mass, the presence or absence of an abscess, signal intensity of the peripheral wall, patterns of contrast enhancement, and associated findings were evaluated. Compared with those of bacterial myositis, the symptoms of tuberculous myositis lasted longer but there were no difinite local inflammatory signs. In three of five cases of bacterial myositis there were specific medical records;trauma in two cases and systemic lupus erythematosus in one. All tuberculous myositis cases involved a single muscle, but bacterial myositis affected multipe muscles in three cases(60%). All but one case showed a mass in the involved muscles. In one bacterial case, there was diffuse swelling in the involved muscle. On T1-weighted images, eight infectious cases showed low signal intensity;two, of the bactrerial type, showed subtle increased signal intensity. all cases demonstrated high signal intensity on t2-weighted images. The signal intensity of peripheral wall was slightly increased on T1-weighted images, but low on T2-weighted. In four cases there was associated cellulitis, and in one case each, adjacent joint effusion and deep vein thrombosis were seen. After gadolinium infusion, peripheral rim enhancement was noted in nine cases and heterogeneous enhancement in one. After magnetic resonance imaging of infectious myositis, the characteristic finding was an abscessed lesion, with the peripheral wall showing high signal intensity on T1-weighted images and low signal intensity on T2 weighted. Although we found it difficult to differentiate bacterial from tuberculous

Magnetic resonance imaging of infectious myositis

International Nuclear Information System (INIS)

Yun, Ji Young; Kim, Jee Young; Kim, Sang Heum; Jung, Youn Ju; Cha, Eun Suk; Park, Joung Mi; Park, Young Ha

1998-01-01

To describe the findings of magnetic resonance imaging in infectious myositis and to determine their value for differentiation between ruberculous and bacterial myositis. Magnetic resonance images of ten proven cases of infectious myositis (five tuberculous and five bacterial) were retrospectively reviewed in the light of clinical and laboratory findings. On the basis of magnetic resonance images, signal intensity of the mass, the presence or absence of an abscess, signal intensity of the peripheral wall, patterns of contrast enhancement, and associated findings were evaluated. Compared with those of bacterial myositis, the symptoms of tuberculous myositis lasted longer but there were no difinite local inflammatory signs. In three of five cases of bacterial myositis there were specific medical records;trauma in two cases and systemic lupus erythematosus in one. All tuberculous myositis cases involved a single muscle, but bacterial myositis affected multipe muscles in three cases(60%). All but one case showed a mass in the involved muscles. In one bacterial case, there was diffuse swelling in the involved muscle. On T1-weighted images, eight infectious cases showed low signal intensity;two, of the bactrerial type, showed subtle increased signal intensity. all cases demonstrated high signal intensity on t2-weighted images. The signal intensity of peripheral wall was slightly increased on T1-weighted images, but low on T2-weighted. In four cases there was associated cellulitis, and in one case each, adjacent joint effusion and deep vein thrombosis were seen. After gadolinium infusion, peripheral rim enhancement was noted in nine cases and heterogeneous enhancement in one. After magnetic resonance imaging of infectious myositis, the characteristic finding was an abscessed lesion, with the peripheral wall showing high signal intensity on T1-weighted images and low signal intensity on T2 weighted. Although we found it difficult to differentiate bacterial from tuberculous

Morphoea with Myositis: A Rare Association

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Mary Sommerlad

2011-01-01

Full Text Available In this case, we describe an unusual presentation of a young woman with a rash typical of morphoea (confirmed on biopsy, who went on to develop myositis in an atypical distribution. Although the association of myositis with diffuse systemic sclerosis is well described, the link with localised scleroderma (morphoea and myositis has not been described.

Recurrent Bilateral Focal Myositis.

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Nagafuchi, Hiroko; Nakano, Hiromasa; Ooka, Seido; Takakuwa, Yukiko; Yamada, Hidehiro; Tadokoro, Mamoru; Shimojo, Sadatomo; Ozaki, Shoichi

This report describes a rare case of recurrent bilateral focal myositis and its successful treatment via methotrexate. A 38-year-old man presented myalgia of the right gastrocnemius in May 2005. Magnetic resonance imaging showed very high signal intensity in the right gastrocnemius on short-tau inversion recovery images. A muscle biopsy revealed inflammatory CD4+ cell-dominant myogenic change. Focal myositis was diagnosed. The first steroid treatment was effective. Tapering of prednisolone, however, repeatedly induced myositis relapse, which progressed to multiple muscle lesions of both lower limbs. Initiation of methotrexate finally allowed successful tapering of prednisolone, with no relapse in the past 4 years.

Idiopathic inflammatory myositis.

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Tieu, Joanna; Lundberg, Ingrid E; Limaye, Vidya

2016-02-01

Knowledge on idiopathic inflammatory myopathy (IIM) has evolved with the identification of myositis-associated and myositis-specific antibodies, development of histopathological classification and the recognition of how these correlate with clinical phenotype and response to therapy. In this paper, we outline key advances in diagnosis and histopathology, including the more recent identification of antibodies associated with immune-mediated necrotising myopathy (IMNM) and inclusion body myositis (IBM). Ongoing longitudinal observational cohorts allow further classification of these patients with IIM, their predicted clinical course and response to specific therapies. Registries have been developed worldwide for this purpose. A challenging aspect in IIM, a multisystem disease with multiple clinical subtypes, has been defining disease status and clinically relevant improvement. Tools for assessing activity and damage are now recognised to be important in determining disease activity and guiding therapeutic decision-making. The International Myositis Assessment and Clinical Studies (IMACS) group has developed such tools for use in research and clinical settings. There is limited evidence for specific treatment strategies in IIM. With significant development in the understanding of IIM and improved classification, longitudinal observational cohorts and trials using validated outcome measures are necessary, to provide important information for evidence-based care in the clinical setting. Crown Copyright © 2016. Published by Elsevier Ltd. All rights reserved.

Myositis specific autoantibodies; specificity and clinical applications.

NARCIS (Netherlands)

Hengstman, G.J.D.

2005-01-01

The sera of about half of the patients with myositis contain autoantibodies that are specific for this group of diseases compared to other inflammatory connective tissue disorders. In a recent study we showed that these myositis specific autoantibodies (MSAs) are also specific for myositis as

Myositis ossificans in rectus abdominis muscle: case report

International Nuclear Information System (INIS)

Ko, Eun Sook; Na, Jae Boem

2004-01-01

Myositis ossificans is an ossifying inflammatory lesion occurring within skeletal muscle. Myositis ossificans usually arises in the large muscles of the extremities and this lesion is characterized by progression of mineralization from periphery to center. In the early phase, myositis ossificans simulates malignant soft tissue tumor without dense mineralization. Traumatic myositis ossificans in rectus abdominis muscle has been reported worldwide. The radiologic findings of early active myositis ossificans in rectus abdominis muscle are ill defined heterogenous hypoechoic mass on US, hemorrhage, early strong enhancement and early peripheral mineralization on CT and MR

Myositis ossificans in rectus abdominis muscle: case report

Energy Technology Data Exchange (ETDEWEB)

Ko, Eun Sook; Na, Jae Boem [Gyeongsang National University College of Medicine, Jinju (Korea, Republic of)

2004-10-15

Myositis ossificans is an ossifying inflammatory lesion occurring within skeletal muscle. Myositis ossificans usually arises in the large muscles of the extremities and this lesion is characterized by progression of mineralization from periphery to center. In the early phase, myositis ossificans simulates malignant soft tissue tumor without dense mineralization. Traumatic myositis ossificans in rectus abdominis muscle has been reported worldwide. The radiologic findings of early active myositis ossificans in rectus abdominis muscle are ill defined heterogenous hypoechoic mass on US, hemorrhage, early strong enhancement and early peripheral mineralization on CT and MR.

Bacterial, Fungal, Parasitic, and Viral Myositis

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Crum-Cianflone, Nancy F.

2008-01-01

Infectious myositis may be caused by a broad range of bacterial, fungal, parasitic, and viral agents. Infectious myositis is overall uncommon given the relative resistance of the musculature to infection. For example, inciting events, including trauma, surgery, or the presence of foreign bodies or devitalized tissue, are often present in cases of bacterial myositis. Bacterial causes are categorized by clinical presentation, anatomic location, and causative organisms into the categories of pyo...

Subacute sarcoid myositis with ocular muscle involvement; a case report and review of the literature.

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Hayashi, Y; Ishii, Yoshiki; Nagasawa, J; Arai, S; Okada, H; Ohmi, F; Umetsu, T; Machida, Y; Kurasawa, K; Takemasa, A; Suzuki, S; Senoh, T; Sada, T; Hirata, K

2016-10-07

Sarcoidosis is a chronic granulomatous disease that can affect multiple organs. The lungs, eyes, and skin are known to be highly affected organs in sarcoidosis. There have been reports based on random muscle biopsy that 32-80% of systemic sarcoidosis comprises noncaseating granulomas; however, muscle involvement in sarcoidosis is generally asymptomatic and has an unknown frequency. We describe a case of acute to subacute sarcoid myositis of the skeletal and extraocular muscles. Typical ophthalmic involvement (manifested by infiltration of the ocular adnexa, intraocular inflammation, or infiltration of the retrobulbar visual pathways) and extraocular sarcoid myositis (as with the present case) is infrequently reported. It is important to keep in mind the rare yet perhaps underestimated entity of sarcoid myositis, and to utilize muscle biopsy and imaging tests for appropriate diagnosis and management of patients with sarcoidosis.

Topical action of Buriti oil (Mauritia flexuosa L.) in myositis induced in rats.

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Barbosa, Marília Ursulino; Silva, Marcello de Alencar; Barros, Esmeralda Maria Lustosa; Barbosa, Margarida Ursulino; Sousa, Rayssilane Cardoso de; Lopes, Mateus Aguiar da Costa; Coelho, Nayana Pinheiro Machado de Freitas

2017-11-01

To analyze the topical effects of Buriti oil (Mauritia flexuosa L.) in induced myositis in rats. Thirty six male rats divided into three groups: Control group (C), induced myositis group (MI) and induced myositis group reated with Mauritia flexuosa L. (MT). After inducing myositis with 1% acetic acid, was topically applied 0.5 ml of Mauritia flexuosa L.extract on the posterior region of the right gastrocnemius muscle in animals belonging to group MT, for 7 and 14 days. The neutrophil number there was statistically significant difference, after 7 and 14 days, between groups C and MI (p <0.001) (p<0.01). The group MT there was a significant difference in relation to MI group in both experimental times with (p<0.001). The number of fibroblasts in the 14 days showed that when comparing the groups M and MT the differences were also significant (p<0.001). As for the DLL, in 7 days, there was a significant difference between group C and MI group (p <0.001). When considering the MT group, there was a significant difference in relation to the MI group (p <0.001). The extract of Mauritia flexuosa L. leaves lessened acute and chronic inflammation, increased fibroblast proliferation and reduced macroscopically edema.

Medical and Surgical Treatment in Pediatric Orbital Myositis Associated with Coxsackie Virus

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Pedro Gil

2015-01-01

Full Text Available Purpose. To report a case of orbital myositis associated with Coxsackie virus and its medical and surgical approach. Methods. Complete ophthalmological examination and imaging and analytical investigation were performed. Results. A 6-year-old male presented with subacute painless binocular horizontal diplopia. Examination revealed bilateral best-corrected visual acuity (BCVA of 20/20 and right eye 45-prism-dioptre (PD esotropia in near and distance fixations, with no motility restrictions. Serologic screening was positive for Coxsackie virus acute infection and computerized tomography (CT suggested right eye medial rectus orbital myositis. An oral corticosteroid 1.0 mg/kg/day regimen was started. A new CT after two months showed symmetrical lesions in both medial rectus muscles. Corticosteroids were increased to 1.5 mg/kg/day. After imagiological resolution on the 4th month, alternating 45 PD esotropia persisted. Bilateral 7 mm medial rectus recession was performed after 1 year without spontaneous recovery. At 1-year follow-up, the patient is orthophoric with 200′′ stereopsis and bilateral 20/20 BCVA. Conclusions. To our knowledge, this is the first reported case of orbital myositis associated with Coxsackie virus. This is also the first reported case of isolated strabismus surgery after orbital myositis in pediatric age, highlighting the favourable aesthetic and functional outcomes even in cases of late ocular motility disorders.

Computed tomography of orbital myositis

International Nuclear Information System (INIS)

Dresner, S.C.; Rothfus, W.E.; Slamovits, T.L.; Kennerdell, J.S.; Curtin, H.D.

1984-01-01

The computerized tomographic (CT) scans of 11 consecutive patients with orbital myositis were reviewed to better characterize the CT appearance of this condition. The findings in this series differed from those of previous reports in several ways. Multiple muscle involvement predominated. Bilateral involvement was more frequent than previously reported. Enlargement of the tendon as well as the muscle was a frequent finding, but a normal tendinous insertion did not preclude the diagnosis of orbital myositis. Although the CT appearance of orbital myositis is often helpful, the findings are not pathognomonic; correlation with history, clinical findings, and therapeutic response must be considered in making the diagnosis

Patients' Experience of Myositis and Further Validation of a Myositis-specific Patient Reported Outcome Measure - Establishing Core Domains and Expanding Patient Input on Clinical Assessment in Myositis. Report from OMERACT 12.

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Regardt, Malin; Basharat, Pari; Christopher-Stine, Lisa; Sarver, Catherine; Björn, Anita; Lundberg, Ingrid E; Wook Song, Yeong; Bingham, Clifton O; Alexanderson, Helene

2015-12-01

The Outcome Measures in Rheumatology (OMERACT) myositis working group was established to examine patient-reported outcomes (PRO) as well as to validate patient-reported outcome measures (PROM) in myositis. Qualitative studies using focus group interviews and cognitive debriefing of the myositis-specific Myositis Activities Profile (MAP) were used to explore the experience of adults living with polymyositis (PM) and dermatomyositis (DM). Preliminary results underscore the importance of patient input in the development of PROM to ensure content validity. Results from multicenter focus groups indicate the range of symptoms experienced including pain, fatigue, and impaired cognitive function, which are not currently assessed in myositis. Preliminary cognitive debriefing of the MAP indicated that while content was deemed relevant and important, several activities were not included; and that questionnaire construction and wording may benefit from revision. A research agenda was developed to continue work toward optimizing PRO assessment in myositis with 2 work streams. The first would continue to conduct and analyze focus groups until saturation in the thematic analysis was achieved to develop a framework that encompassed the patient-relevant aspects of myositis. The second would continue cognitive debriefing of the MAP to identify potential areas for revision. There was agreement that further work would be needed for inclusion body myositis and juvenile dermatomyositis, and that the inclusion of additional contributors such as caregivers and individuals from the pharmaceutical/regulatory spheres would be desirable. The currently used PROM do not assess symptoms or the effects of disease that are most important to patients; this emphasizes the necessity of patient involvement. Our work provides concrete examples for PRO identification.

Genomic signatures characterize leukocyte infiltration in myositis muscles

Science.gov (United States)

2012-01-01

Background Leukocyte infiltration plays an important role in the pathogenesis and progression of myositis, and is highly associated with disease severity. Currently, there is a lack of: efficacious therapies for myositis; understanding of the molecular features important for disease pathogenesis; and potential molecular biomarkers for characterizing inflammatory myopathies to aid in clinical development. Methods In this study, we developed a simple model and predicted that 1) leukocyte-specific transcripts (including both protein-coding transcripts and microRNAs) should be coherently overexpressed in myositis muscle and 2) the level of over-expression of these transcripts should be correlated with leukocyte infiltration. We applied this model to assess immune cell infiltration in myositis by examining mRNA and microRNA (miRNA) expression profiles in muscle biopsies from 31 myositis patients and 5 normal controls. Results Several gene signatures, including a leukocyte index, type 1 interferon (IFN), MHC class I, and immunoglobulin signature, were developed to characterize myositis patients at the molecular level. The leukocyte index, consisting of genes predominantly associated with immune function, displayed strong concordance with pathological assessment of immune cell infiltration. This leukocyte index was subsequently utilized to differentiate transcriptional changes due to leukocyte infiltration from other alterations in myositis muscle. Results from this differentiation revealed biologically relevant differences in the relationship between the type 1 IFN pathway, miR-146a, and leukocyte infiltration within various myositis subtypes. Conclusions Results indicate that a likely interaction between miR-146a expression and the type 1 IFN pathway is confounded by the level of leukocyte infiltration into muscle tissue. Although the role of miR-146a in myositis remains uncertain, our results highlight the potential benefit of deconvoluting the source of

[Autoantibody profile in myositis].

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Allenbach, Y; Benveniste, O

2014-07-01

Patients suffering from muscular symptoms or with an increase of creatine kinase levels may present a myopathy. In such situations, clinicians have to confirm the existence of a myopathy and determine if it is an acquired or a genetic muscular disease. In the presence of an acquired myopathy after having ruled out an infectious, a toxic agent or an endocrine cause, physicians must identify which type of idiopathic myopathy the patient is presenting: either a myositis including polymyositis, dermatomyositis, and inclusion body myositis, or an immune-mediated necrotizing myopathy. Histopathology examination of a muscle biopsy is determinant but detection of autoantibody is now also crucial. The myositis-specific antibodies and myositis-associated antibodies lead to a serologic approach complementary to the histological classification, because strong associations of myositis-specific antibodies with clinical features and survival have been documented. The presence of anti-synthetase antibodies is associated with an original histopathologic pattern between polymyositis and dermatomyositis, and defines a syndrome where interstitial lung disease drives the prognosis. Anti-MDA-5 antibody are specifically associated with dermatomyositis, and define a skin-lung syndrome with a frequent severe disease course. Anti-TIF1-γ is also associated with dermatomyositis but its presence is frequently predictive of a cancer association whereas anti-MI2 is associated with the classical dermatomyositis. Two specific antibodies, anti-SRP and anti-HMGCR, are observed in patients with immune-mediated necrotizing myopathies and may be very useful to distinguish acquired myopathies from dystrophic muscular diseases in case of a slow onset and to allow the initiation of effective therapy. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Acute leukemia in early childhood

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M. Emerenciano

2007-06-01

Full Text Available Acute leukemia in early childhood is biologically and clinically distinct. The particular characteristics of this malignancy diagnosed during the first months of life have provided remarkable insights into the etiology of the disease. The pro-B, CD10 negative immunophenotype is typically found in infant acute leukemia, and the most common genetic alterations are the rearrangements of the MLL gene. In addition, the TEL/AML1 fusion gene is most frequently found in children older than 24 months. A molecular study on a Brazilian cohort (age range 0-23 months has detected TEL/AML1+ve (N = 9, E2A/PBX1+ve (N = 4, PML/RARA+ve (N = 4, and AML1/ETO+ve (N = 2 cases. Undoubtedly, the great majority of genetic events occurring in these patients arise prenatally. The environmental exposure to damaging agents that give rise to genetic changes prenatally may be accurately determined in infants since the window of exposure is limited and known. Several studies have shown maternal exposures that may give rise to leukemogenic changes. The Brazilian Collaborative Study Group of Infant Acute Leukemia has found that mothers exposed to dipyrone, pesticides and hormones had an increased chance to give birth to babies with infant acute leukemia [OR = 1.48 (95%CI = 1.05-2.07, OR = 2.27 (95%CI = 1.56-3.31 and OR = 9.08 (95%CI = 2.95-27.96], respectively. This review aims to summarize recent clues that have facilitated the elucidation of the biology of early childhood leukemias, with emphasis on infant acute leukemia in the Brazilian population.

Diagnosis, pathogenesis and treatment of myositis: recent advances.

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Carstens, P-O; Schmidt, J

2014-03-01

Dermatomyositis (DM), polymyositis (PM), necrotizing myopathy (NM) and inclusion body myositis (IBM) are four distinct subtypes of idiopathic inflammatory myopathies - in short myositis. Recent studies have shed some light on the unique pathogenesis of each entity. Some of the clinical features are distinct, but muscle biopsy is indispensable for making a reliable diagnosis. The use of magnetic resonance imaging of skeletal muscles and detection of myositis-specific autoantibodies have become useful additions to our diagnostic repertoire. Only few controlled trials are available to substantiate current treatment approaches for myositis and hopes are high that novel modalities will become available within the next few years. In this review we provide an up-to-date overview of the pathogenesis and diagnostic approach of myositis. We aim to present a guide towards therapeutic and general management. © 2013 British Society for Immunology.

Value of MRI in diagnostics and evaluation of myositis.

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Pipitone, Nicolò

2016-11-01

This review aims at covering the role of muscle MRI in supporting the diagnosis of myositis, in aiding to differentiate it from other muscle disorders, and in monitoring myositis patients over time by assessing response to treatment and by discriminating between muscle inflammation and chronic damage. MRI can assist in 'pattern recognition' of muscle involvement across numerous myopathies, including myositis. Novel applications of magnetic resonance such as cardiac MRI, MR elastography and blood oxigenation level-dependent magnetic resonance can shed light on different aspects of myositis and usefully complement conventional MRI in assessing patients with myositis. MRI can guide therapy by determining whether muscle weakness is related to edema (active inflammation) or muscle atrophy/fat replacement (chronic damage). There is a need to better standardize the assessment of MRI findings in myositis to provide defined outcome measures for use in clinical trials. VIDEO ABSTRACT.

MANAGEMENT OF ACUTE RENAL FAILURE WITH DELAYED HYPERCALCEMIA SECONDARY TO SARCOCYSTIS NEURONA-INDUCED MYOSITIS AND RHABDOMYOLYSIS IN A CALIFORNIA SEA LION (ZALOPHUS CALIFORNIANUS).

Science.gov (United States)

Alexander, Amy B; Hanley, Christopher S; Duncan, Mary C; Ulmer, Kyle; Padilla, Luis R

2015-09-01

A 3-yr-old captive-born California sea lion (Zalophus californianus) developed Sarcocystis neurona-induced myositis and rhabdomyolysis that led to acute renal failure. The sea lion was successfully managed with fluid therapy, antiprotozoals, antibiotics, anti-inflammatories, antiemetics, gastroprotectants, and diuretics, but developed severe delayed hypercalcemia, a syndrome identified in humans after traumatic or exertion-induced rhabdomyolysis. Treatment with calcitonin was added to the management, and the individual recovered fully. The case emphasizes that animals with rhabdomyolysis-induced renal failure risk developing delayed hypercalcemia, which may be life threatening, and calcium levels should be closely monitored past the resolution of renal failure.

Multimodality imaging of Candida tropicalis myositis

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Schwartz, Daniel M. [Children' s Memorial Hospital, Department of Medical Imaging, 2300 Children' s Plaza, Box 9, Chicago, IL (United States); Morgan, Elaine R. [Children' s Memorial Hospital, Department of Hematology and Oncology, Chicago, IL (United States)

2008-04-15

Fungal myositis is a rare entity that has been described in immunocompromised patients. We present a boy with biopsy proven fungal myositis who was examined with multiple imaging modalities. MR imaging proved to be very effective for diagnostic purposes, while US imaging was able to provide guidance for biopsy. (orig.)

Multimodality imaging of Candida tropicalis myositis

International Nuclear Information System (INIS)

Schwartz, Daniel M.; Morgan, Elaine R.

2008-01-01

Fungal myositis is a rare entity that has been described in immunocompromised patients. We present a boy with biopsy proven fungal myositis who was examined with multiple imaging modalities. MR imaging proved to be very effective for diagnostic purposes, while US imaging was able to provide guidance for biopsy. (orig.)

Myositis specific autoantibodies: changing insights in pathophysiology and clinical associations.

NARCIS (Netherlands)

Hengstman, G.J.D.; Engelen, B.G.M. van; Venrooij, W.J.W. van

2004-01-01

PURPOSE OF REVIEW: Defined autoantibodies are found in about half of the patients with myositis. Traditionally, these autoantibodies have been divided into myositis specific autoantibodies (MSAs) and myositis associated autoantibodies. Several studies have shown that MSAs are associated with

Myositis ossificans and the three-phase bone scan

International Nuclear Information System (INIS)

Drane, W.E.

1984-01-01

Myositis ossificans circumscripta (or paraosteoarthropathy) in paraplegics has been a well known entity since its classic description in 1918 by Dejerine and Ceillier. The disease is characterized by ectopic bone formation, primarily occurring in the particular soft tissues of the hips and knees. Hypotheses concerning its origin have been proposed, but its etiology is still unknown. Myositis ossificans can occur after traumatic injury, but develops in paraplegics without apparent injury in the involved regions. Radionuclide techniques have been used in the evaluation of myositis ossificans, particularly with serial studies to stage the activity of the disease. The author reports a case of myositis ossificans in a paraplegic that emphasizes the benefit of the three-phase bone scan in the early diagnosis of this disorder

MYOSITIS OSSIFICANS TRAUMATICA IN A VAMPIRE BAT (DESMODUS ROTUNDUS).

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Hausmann, Jennifer C; Manasse, Jorden; Churgin, Sarah; Steinberg, Howard; Clyde, Victoria L; Wallace, Roberta

2016-09-01

A 15-yr-old sexually intact female vampire bat ( Desmodus rotundus ) was diagnosed with myositis ossificans traumatica of the abdominal wall. The bat presented with a large ulcerated firm mass along the abdomen. Radiographs and cytology were performed, followed by surgical exploration. The mass was determined to be nonresectable and the bat was euthanized. Histopathology showed severe necrotizing, degenerative, and pyogranulomatous myositis with osseous and cartilaginous metaplasia, fibrosis, and ulceration, which were consistent with myositis ossificans traumatica. Myositis ossificans traumatica is commonly associated with previous trauma to skeletal muscle. Two years prior, this bat had an emergency Caesarian section at this site, which was postulated to elicit a marked tissue response leading to this condition. Myositis ossificans traumatica is infrequently reported in humans, dogs, cats, pigs, and horses. To the author's knowledge, this is the first report of this condition in a bat.

Associated Variables of Myositis in Systemic Lupus Erythematosus: A Cross-Sectional Study.

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Liang, Yan; Leng, Rui-Xue; Pan, Hai-Feng; Ye, Dong-Qing

2017-05-26

BACKGROUND This study aimed to estimate the point prevalence of myositis and identify associated variables of myositis in systemic lupus erythematosus (SLE). MATERIAL AND METHODS Clinical date of patients hospitalized with lupus at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital were collected. Patients were defined as having myositis if they reported the presence of persistent invalidating muscular weakness combined with increased levels of creatine phosphokinase (CPK) and abnormal electromyography (EMG). RESULTS The study sample comprised 1701 lupus patients, of which 44 had myositis. Patients with SLE-associated myositis are more likely to have skin rash, alopecia, pericarditis, vasculitis, anti-Sm, anti-RNP, anti-dsDNA, thrombocytopenia, leukopenia, low C3, low C4, high erythrocyte sedimentation rate (ESR), high D-dimer, and active disease. Multivariate logistic regression found positive associations between leukopenia, alopecia, and active disease with myositis. Negative associations between myositis with the use of corticosteroids or immunosuppressive drugs were revealed in univariate and multivariate analysis. CONCLUSIONS The point prevalence of myositis was 2.6% in SLE patients. The significant association of alopecia, leukopenia, and active disease with myositis suggests that organ damage, hematological abnormality, and high disease activity promote the progression of myositis in lupus patients.

Clinical and laboratory description of a series of cases of acute viral myositis

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Silvana Paula Cardin

2015-10-01

Full Text Available ABSTRACT OBJECTIVE: Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. METHOD: A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK, lactate dehydrogenase (LDH, transaminases (AST and ALT, blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. RESULTS: Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%, rhinorrhea (23%, and fever (63%, with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%, limited ambulation (57%, gait abnormality (40%, and muscle weakness in the lower limbs (71%, with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507 ± 9180 U/L, LDH (827 ± 598 U/L, and AST (199 ± 245 U/L, with a tendency to leukopenia (4590 ± 1420 leukocytes/mm3. The complete recovery of laboratory parameters was observed in 30 days (median, and laboratory and clinical recurrence was documented in one case after 10 months. CONCLUSION: Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses.

Clinical and laboratory description of a series of cases of acute viral myositis.

Science.gov (United States)

Cardin, Silvana Paula; Martin, Joelma Gonçalves; Saad-Magalhães, Claudia

2015-01-01

Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK), lactate dehydrogenase (LDH), transaminases (AST and ALT), blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys) were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%), rhinorrhea (23%), and fever (63%), with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%), limited ambulation (57%), gait abnormality (40%), and muscle weakness in the lower limbs (71%), with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507±9180U/L), LDH (827±598U/L), and AST (199±245U/L), with a tendency to leukopenia (4590±1420) leukocytes/mm(3). The complete recovery of laboratory parameters was observed in 30 days (median), and laboratory and clinical recurrence was documented in one case after 10 months. Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses. Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

Streptococcal necrotizing myositis: a case report and clinical review.

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Hourmozdi, Justin J; Hawley, Dean A; Hadi, Christiane M; Tahir, Bilal; Seupaul, Rawle A

2014-03-01

Streptococcal necrotizing myositis, also known as gangrenous myositis, is a very rare and severe soft tissue infection that predominately involves skeletal muscle and, eventually, superficial fascia and surrounding tissues. The presentation is often nonspecific until the rapidly progressing clinical course becomes apparent. A high morbidity and mortality rate has been reported in the small number of cases since 1900. Despite several attempts to better define the different entities causing necrotizing myositis, no single definitive causal relationship has been defined. A review of the literature is presented here to help clinicians distinguish those with necrotizing myositis from those with nonnecrotizing myositis when the clinician is at all confronted with the suspicion for such an infection. The case presented is that of a 48-year-old woman who had streptococcal necrotizing myositis. She died roughly 72 h after admission. After the patient's death, the clinical team sought consent for autopsy. Hospital staff made contact with family, and information was obtained from the family that the onset of the patient's symptoms was allegedly temporally related to her acquisition of a new tattoo on the right back, where the tattoo process allegedly included injection of cremated ashes of a pet dog. A high level of suspicion for necrotizing myositis must be maintained for a patient with unexplained severe muscle pain and soft tissue swelling accompanied by systemic inflammatory response syndrome. Copyright © 2014 Elsevier Inc. All rights reserved.

Non-infectious chemotherapy-associated acute toxicities during childhood acute lymphoblastic leukemia therapy

DEFF Research Database (Denmark)

Schmiegelow, Kjeld; Müller, Klaus Gottlob; Mogensen, Signe Sloth

2017-01-01

During chemotherapy for childhood acute lymphoblastic leukemia, all organs can be affected by severe acute side effects, the most common being opportunistic infections, mucositis, central or peripheral neuropathy (or both), bone toxicities (including osteonecrosis), thromboembolism, sinusoidal...... useful risk factors, and across study groups there has been wide diversity in toxicity definitions, capture strategies, and reporting, thus hampering meaningful comparisons of toxicity incidences for different leukemia protocols. Since treatment of acute lymphoblastic leukemia now yields 5-year overall...... obstruction syndrome, endocrinopathies (especially steroid-induced adrenal insufficiency and hyperglycemia), high-dose methotrexate-induced nephrotoxicity, asparaginase-associated hypersensitivity, pancreatitis, and hyperlipidemia. Few of the non-infectious acute toxicities are associated with clinically...

Temporal relationship between cancer and myositis identifies two distinctive subgroups of cancers: impact on cancer risk and survival in patients with myositis.

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Kang, Eun Ha; Lee, Sang Jin; Ascherman, Dana P; Lee, Yun Jong; Lee, Eun Young; Lee, Eun Bong; Song, Yeong Wook

2016-09-01

The aim was to compare standardized incidence ratios (SIRs) of cancers temporally related and unrelated to active myositis in patients with myositis. Fifty-two cancer cases were identified in 281 myositis patients. SIRs of cancers having temporal overlap with the active phase of myositis [cancers concurrent with active myositis (CAM), n = 30] and cancers not having such temporal overlap [cancers non-concurrent with active myositis (CNM), n = 22] were compared in 281 patients. Patients with CAM were older at diagnosis of myositis, had a greater tendency to be male, more frequent dysphagia and less frequent interstitial lung disease than patients with CNM. CAM SIR (95% CI) was 1.78 (1.19, 2.56) and CNM SIR 1.23 (0.75, 1.90). The peak SIR was observed in the seventh decade of life for CAM and in the third decade for CNM. When stratified by myositis-cancer intervals, CAM SIR was 9.94 (6.43, 14.67) within 1 year of myositis diagnosis, whereas no temporal relationship was found for CNM. Elevated SIRs were observed for oesophageal cancer [57.77 (11.91, 168.82)], non-Hodgkin's lymphoma [41.43 (13.45, 96.69)], adenocarcinoma of unknown primary origin [67.6 (18.42, 173.07]), lung cancer [7.27 (1.98, 18.61)] and ovarian cancer [19.15 (2.32, 69.17)] within 3 years of CAM diagnosis. The cancer stage at the time of diagnosis was more advanced in CAM than CNM (P < 0.001), with a correspondingly increased hazard ratio of mortality [4.3 (1.5, 12.7)] in patients with CAM vs CNM. A significantly elevated SIR was found for CAM, whereas there was a comparable SIR for CNM relative to the general population. Multiple types of cancers showed elevated SIRs among CAM, but none among CNM. Given that cancer stages in CAM were far advanced at diagnosis, mortality risk was greater in patients with CAM. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Emerging therapeutic options for sporadic inclusion body myositis

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Alfano LN

2015-09-01

Full Text Available Lindsay N Alfano, Linda P Lowes Nationwide Children’s Hospital, Center for Gene Therapy, Columbus, OH, USA Abstract: Sporadic inclusion body myositis is the most common inflammatory muscle disorder preferentially affecting males over the age of 40 years. Progressive muscle weakness of the finger flexors and quadriceps muscles results in loss of independence with activities of daily living and eventual wheelchair dependence. Initial signs of disease are often overlooked and can lead to mis- or delayed diagnosis. The underlying cause of disease is unknown, and disease progression appears refractory to available treatment options. This review discusses the clinical presentation of inclusion body myositis and the current efforts in diagnosis, and focuses on the current state of research for both nonpharmacological and pharmacological treatment options for this patient group. Keywords: myositis, inclusion body myositis, inflammatory myopathy, treatment, function, outcomes

Acute myositis: an unusual and severe side effect of docetaxel: a case report and literature review.

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Rochigneux, Philippe; Schleinitz, Nicolas; Ebbo, Mikael; Aymonier, Marie; Pourroy, Bertrand; Boissier, Romain; Salas, Sébastien; Deville, Jean-Laurent

2018-06-01

Docetaxel is an antimicrotubules cytotoxic agent prescribed widely by medical oncologists in multiple tumor types (breast, lung, prostate, stomach, head, and neck). However, the side effects of docetaxel are numerous (cytopenia, peripheral edema, myalgia, arthralgia, alopecia, and sensitive neuropathy) and recent concerns have been raised about neutropenic enterocolitis in France. Here, we report the case of a 57-year-old patient with metastatic prostatic cancer, who developed a severe myositis and fasciitis grade IV 1 week after his second docetaxel infusion. We reviewed the five cases of docetaxel-related myositis described in the literature, and found that most of them occurred in patients with diabetes (n=5/5) or hypertension (n=4/5). A vascular toxicity may explain this severe complication, and patients with diabetes or hypertension should be monitored closely in the context of a docetaxel chemotherapy.

Myositis Ossificans of Psoas Muscle: Magnetic Resonance Imaging Findings

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Sirvanci, M.; Ganiyusufoglu, A.K.; Karaman, K.; Tezer, M.; Hamzaoglu, A. [Univ. of Kadir Has, Istanbul (Turkey). Dept. of Radiology

2004-08-01

Myositis ossificans is a benign, localized, self-limiting ossifying soft tissue mass with unknown pathogenesis. It may be confused with a malignant tumor of bone or soft tissues, especially in the early stages of the disease. In this report, we present a case of myositis ossificans affecting unilateral psoas muscle, which to our knowledge is a very uncommon location. There was no history of trauma. We describe the imaging findings and clues to early diagnosis of myositis ossificans.

Myositis Ossificans of Psoas Muscle: Magnetic Resonance Imaging Findings

International Nuclear Information System (INIS)

Sirvanci, M.; Ganiyusufoglu, A.K.; Karaman, K.; Tezer, M.; Hamzaoglu, A.

2004-01-01

Myositis ossificans is a benign, localized, self-limiting ossifying soft tissue mass with unknown pathogenesis. It may be confused with a malignant tumor of bone or soft tissues, especially in the early stages of the disease. In this report, we present a case of myositis ossificans affecting unilateral psoas muscle, which to our knowledge is a very uncommon location. There was no history of trauma. We describe the imaging findings and clues to early diagnosis of myositis ossificans

Focal myositis: A review.

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Devic, P; Gallay, L; Streichenberger, N; Petiot, P

2016-11-01

Amongst the heterogeneous group of inflammatory myopathies, focal myositis stands as a rare and benign dysimmune disease. Although it can be associated with root and/or nerve lesions, traumatic muscle lesions and autoimmune diseases, its triggering factors remain poorly understood. Defined as an isolated inflammatory pseudotumour usually restricted to one skeletal muscle, clinical presentation of focal myositis is that of a rapidly growing solitary mass within a single muscle, usually in the lower limbs. Electromyography shows spontaneous activity associated with a myopathic pattern. MRI reveals a contrast enhanced enlarged muscle appearing hyper-intense on FAT-SAT T2 weighted images. Adjacent structures are spared and there are no calcifications. Serum creatine kinase (CK) levels are usually moderately augmented and biological markers of systemic inflammation are absent in most cases. Pathological histological features include marked variation in fibre size, inflammatory infiltrates mostly composed of T CD4+ lymphocytes and macrophages, degenerating/regenerating fibres and interstitial fibrosis. Differential diagnoses are numerous and include myositis of other origin with focal onset. Steroid treatment should be reserved for patients who present with major pain, nerve lesions, associated autoimmune disease, or elevated C reactive protein or CK. Copyright © 2016 Elsevier B.V. All rights reserved.

Unilateral temporal myositis heralding polymyositis: ultrasonographic and elastographic findings. Case report.

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Damian, Laura; Botar Jid, Carolina; Rogojan, Liliana; Dinu, Cristian; Maniu, Alma; Fodor, Daniela; Rednic, Simona; Simon, Siao-Pin

2016-03-01

Temporal myositis is a rare inflammatory disease of the temporal muscle. We report a case of unilateral temporal myositis, in which a polymyositis was diagnosed two years thereafter. Although focal myositis may rarely herald polymyositis, isolated temporal myositis preceding inflammatory myopathies has not been described, to our knowledge. In the setting of a temporal pain and swelling, ultrasonography may help in diagnosis, biopsy guidance, disease extension, and progression assessment. Further studies are necessary to establish the role of elastography in differentiating between muscle inflammation and hypertrophy.

Acute childhood leukemia: Nursing care

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Zietz, Hallie A

1997-01-01

Modern therapy for childhood acute leukemia has provided a dramatically improved prognosis over that of just 30 years ago. In the early 1960's survival rates for acute lymphocytic leukemia (ALL) and acute myelogenous leukemia (AML) were 4% and 3%, respectively. By the 1980's survival rates had risen to 72% for all and 25% to 40% for AML. Today, a diagnosis of all carries an 80% survival rate and as high as a 90% survival rate for some low-risk subtypes. Such high cure rates depend on intense and complex, multimodal therapeutic protocols. Therefore, nursing care of the child with acute leukemia must meet the demands of complicated medical therapies and balance those with the needs of a sick child and their concerned family. An understanding of disease process and principles of medical management guide appropriate and effective nursing interventions. Leukemia is a malignant disorder of the blood and blood- forming organs (bone marrow, lymph nodes and spleen). Most believe that acute leukemia results from a malignant transformation of a single early haematopoietic stem cell that is capable of indefinite self-renewal. These immature cells of blasts do not respond to normal physiologic stimuli for differentiation and gradually become the predominant cell in the bone marrow

Cervical myositis ossificans traumatica: a rare location

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Baysal, T.; Sarac, K.; Kutlu, R.; Baysal, O.; Ersoy, Y.; Elmali, N.

1999-01-01

An unusual case of myositis ossificans traumatica lesion located in the paraspinal region is reported. Despite the contiguity of the lesion with the cervical vertebrae and ominous appearance of the biopsy material, the history of antecedent trauma and computed tomography findings allowed preoperative accurate diagnosis. To our knowledge, myositis ossificans traumatica located in the cervical paraspinal region is very rare. (orig.)

Focal myositis

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Galloway, H.R.; Dahlstrom, J.E.; Bennett, G.M.

2001-01-01

Focal myositis is a rare, benign focal inflammation of muscle. The lesion often presents as a mass that may be mistaken for a soft tissue sarcoma. This report describes the MRI and histopathological features of a case and illustrates how the diagnosis may be suspected on the basis of the MR findings. Copyright (2001) Blackwell Science Pty Ltd

Myositis as the initial presentation of panarteritis nodosa.

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Calvo, Romina; Negri, Melina; Ortiz, Alberto; Roverano, Susana; Paira, Sergio

2017-07-26

A 47-year-old man presented with weight loss, bilateral calf pain, fever, hypertension, orchitis and oligoarthritis. Lab tests: anemia and elevated muscle enzymes. Resonance magnetic imaging: hyperintensity in gastrocnemius muscles (myositis). Histologic exam of the muscles: inflammatory infiltrate with atrophy and perifascicular regeneration. methylprednisone (bolus) and cyclophosphamide. Muscle pain and swelling and difficulty in walking are common in panarteritis nodosa (PAN), whereas histologically demonstrated myositis is not. Even more rare is myositis as the initial presentation of this vasculitis. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

Cervical myositis ossificans traumatica: a rare location

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Baysal, T.; Sarac, K.; Kutlu, R. [Dept. of Radiology, Inonu University, Malatya (Turkey); Baysal, O.; Ersoy, Y. [Dept. of Physical Therapy and Rehabilitation, Inonu Univ., Malatya (Turkey); Elmali, N. [Dept. of Orthopedics and Traumatology, Inonu Univ., Malatya (Turkey)

1999-05-01

An unusual case of myositis ossificans traumatica lesion located in the paraspinal region is reported. Despite the contiguity of the lesion with the cervical vertebrae and ominous appearance of the biopsy material, the history of antecedent trauma and computed tomography findings allowed preoperative accurate diagnosis. To our knowledge, myositis ossificans traumatica located in the cervical paraspinal region is very rare. (orig.) With 4 figs., 16 refs.

[Clinical and histopathological features of myositis associated with anti-mitochondrial antibodies].

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Shimizu, Jun

2013-01-01

Anti-mitochondrial antibodies (AMA) are known to be characteristic markers of primary biliary cirrhosis (PBC). The association of PBC with myositis has been reported mainly as case reports, and comprehensive studies of the clinical and histopathological features of patients with myositis and AMAs or PBC have not been conducted thus far. We retrospectively reviewed 212 patients with inflammatory myopathies in our laboratory and found 24 patients with AMA-positive myositis (11%) (seven patients with PBC and 17 patients without PBC). The analysis of clinical and histopathological features revealed that myositis associated with AMAs frequently include patients with a clinically chronic disease course, muscle atrophy, cardiopulmonary involvement and granulomatous inflammation, regardless of the presence or absence of PBC. We also reviewed and analyzed the clinical features of previously reported patients. The analysis of 75 patients, which have been described in previous case reports including the ones of meeting abstracts, also showed the similar results about clinical features of myositis associated with AMAs and supported our findings. Our study suggests that myositis associated with AMAs form a characteristic subgroup.

Childhood Acute Lymphoblastic Leukemia: Integrating Genomics into Therapy

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Tasian, Sarah K; Loh, Mignon L; Hunger, Stephen P

2015-01-01

Acute lymphoblastic leukemia (ALL), the most common malignancy of childhood, is a genetically complex entity that remains a major cause of childhood cancer-related mortality. Major advances in genomic and epigenomic profiling during the past decade have appreciably enhanced knowledge of the biology of de novo and relapsed ALL and have facilitated more precise risk stratification of patients. These achievements have also provided critical insights regarding potentially targetable lesions for development of new therapeutic approaches in the era of precision medicine. This review delineates the current genetic landscape of childhood ALL with emphasis upon patient outcomes with contemporary treatment regimens, as well as therapeutic implications of newly identified genomic alterations in specific subsets of ALL. PMID:26194091

Overlapping features of polymyositis and inclusion body myositis in HIV-infected patients

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Lloyd, Thomas E.; Pinal-Fernandez, Iago; Michelle, E. Harlan; Christopher-Stine, Lisa; Pak, Katherine; Sacktor, Ned

2017-01-01

Objective: To characterize patients with myositis with HIV infection. Methods: All HIV-positive patients with myositis seen at the Johns Hopkins Myositis Center from 2003 to 2013 were included in this case series. Muscle biopsy features, weakness pattern, serum creatine kinase (CK) level, and anti–nucleotidase 1A (NT5C1A) status of HIV-positive patients with myositis were assessed. Results: Eleven of 1,562 (0.7%) patients with myositis were HIV-positive. Myositis was the presenting feature of HIV infection in 3 patients. Eight of 11 patients had weakness onset at age 45 years or less. The mean time from the onset of weakness to the diagnosis of myositis was 3.6 years (SD 3.2 years). The mean of the highest measured CK levels was 2,796 IU/L (SD 1,592 IU/L). On muscle biopsy, 9 of 10 (90%) had endomysial inflammation, 7 of 10 (70%) had rimmed vacuoles, and none had perifascicular atrophy. Seven of 11 (64%) patients were anti-NT5C1A-positive. Upon presentation, all had proximal and distal weakness. Five of 6 (83%) patients followed 1 year or longer on immunosuppressive therapy had improved proximal muscle strength. However, each eventually developed weakness primarily affecting wrist flexors, finger flexors, knee extensors, or ankle dorsiflexors. Conclusions: HIV-positive patients with myositis may present with some characteristic polymyositis features including young age at onset, very high CK levels, or proximal weakness that improves with treatment. However, all HIV-positive patients with myositis eventually develop features most consistent with inclusion body myositis, including finger and wrist flexor weakness, rimmed vacuoles on biopsy, or anti-NT5C1A autoantibodies. PMID:28283597

Identification of a novel myositis-associated antibody directed against cortactin.

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Labrador-Horrillo, Moisés; Martínez, Maria Angeles; Selva-O'Callaghan, Albert; Trallero-Araguás, Ernesto; Grau-Junyent, Josep M; Vilardell-Tarrés, Miquel; Juarez, Candido

2014-10-01

The aim of this study is to describe a novel myositis-associated autoantibody (anti-cortactin antibody) and assess related clinical and immunological manifestations and its clinical significance. Adult patients with myositis (dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, and inclusion body myositis), as well as patients with other autoimmune diseases and non-inflammatory myopathies were analyzed for the presence of anti-cortactin antibody using in-house developed ELISA and immunoblotting techniques with a commercial source of purified cortactin. The cut-off for positive status was determined in a group of healthy volunteers. Antibody against cortactin was positive in 7/34 (20%) polymyositis patients, 9/117 (7.6%) dermatomyositis, 2/7 (26%) immune-mediated necrotizing myopathy, and none of the 4 patients with inclusion body myositis. The antibody also tested positive in 3/101 patients with other autoimmune diseases (2 systemic sclerosis and 1 systemic lupus erythematosus), and in 1/29 patients with non-inflammatory myopathy. No relevant association with specific clinical features was found in patients with these antibodies. Anti-cortactin antibody was more frequently positive in patients with polymyositis and immune-mediated necrotizing myopathy than in the remaining myositis patients, and was the only myositis autoantibody found in sera of 3 patients from these groups. Our data indicate that cortactin is a novel target antigen in patients with autoimmune diseases, especially patients with polymyositis or immune-mediated necrotizing myopathy. Anti-cortactin can be considered a new myositis-associated antibody. Copyright © 2014 Elsevier B.V. All rights reserved.

Eosinophilic myositis: an updated review.

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Selva-O'Callaghan, A; Trallero-Araguás, E; Grau, J M

2014-01-01

Eosinophilia-associated myopathies are clinically and pathologically heterogeneous conditions characterized by the presence of peripheral and/or muscle eosinophilia. There are at least three distinct subtypes: focal eosinophilic myositis, eosinophilic polymyositis, and eosinophilic perimyositis. Infiltrating eosinophils are not always identified in conventional muscle histologic examination, but the eosinophil major basic protein, whose extracellular diffusion is considered a hallmark of eosinophilic cytotoxicity, is usually detected by immunostaining in muscle biopsy. Whereas focal eosinophilic myositis seems to be a benign and isolated condition, and perimyositis is usually related with the inflammatory infiltrate due to fasciitis, eosinophilic polymyositis can be associated with muscular dystrophy or be a feature of multiorgan hypereosinophilic syndrome. Muscle biopsy remains the cornerstone for the diagnosis. Parasitic infections, connective tissue disorders, hematologic and non-hematologic malignancies, drugs, and toxic substances are the main etiologic agents of eosinophilia-associated myopathy. However, in some cases, no known etiologic factor is identified, and these are considered idiopathic. Glucocorticoids are the mainstay therapy in idiopathic forms. Imatinib and mepolizumab, a humanized anti-interleukin 5 monoclonal antibody, may be useful in patients with eosinophilic myositis as part of a hypereosinophilic syndrome. Copyright © 2014 Elsevier B.V. All rights reserved.

Computed Tomography in the Diagnosis of Myositis Ossificans – Case Report

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Nitek, Żaneta; Czwojdziński, Adam; Wolf-Kuś, Alicja; Walecki, Jerzy

2014-01-01

The term myositis ossificans refers to the formation of ossifications in the muscles, ligaments and fascias, usually as a result of trauma. Fibrodysplasia ossificans progressiva is a rare genetic disease in which heterotopic ossifications appear in early childhood and are accompanied by feet and spine defects. We present a case of a 31-year-old woman with massive heterotopic ossifications who suffered multiple injuries. We would like to emphasize the role of computed tomography in the exact localization of ossifications. Thanks to the volume rendering techniques and 3D image reconstructions, it is possible to precisely determine the position of ossifications in relation to the internal organs and blood vessels, allowing to schedule the surgery to remove the lesions

High concordance of subtypes of childhood acute lymphoblastic leukemia within families

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Schmiegelow, K.; Thomsen, U Lautsen; Baruchel, A

2012-01-01

Polymorphic genes have been linked to the risk of acute lymphoblastic leukemia (ALL). Surrogate markers for a low burden of early childhood infections are also related to increased risk for developing childhood ALL. It remains uncertain, whether siblings of children with ALL have an increased risk...

Diagnostic performance of a commercial immunoblot assay for myositis antibody testing.

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Bundell, Chris; Rojana-Udomsart, Arada; Mastaglia, Frank; Hollingsworth, Peter; McLean-Tooke, Andrew

2016-06-01

The objective of this study was to establish a population based reference range for a commercial immunoblot assay detecting myositis specific autoantibodies (MSAs) and myositis associated autoantibodies (MAAs), and to assess the diagnostic performance of this reference range against the manufacturer's recommended ranges in a myositis patient cohort. A total of 124 patients from a myositis cohort and 197 healthy controls were serologically assessed using a commercial immunoblot containing eleven autoantigens (Jo-1, EJ, OJ, PL7, PL12, Mi-2, SRP, Ku, PMScl75, PMScl100 and Ro52) according to the manufacturer's instructions. Use of the manufacturer's reference ranges resulted in detection of MSAs in 19.4% of myositis patients and 9.1% of controls; MAAs were detected in 41.1% of myositis patients and 14.2% of controls. Reference values derived from the healthy control population resulted in significant differences in cut-off values for some autoantibodies, particularly Ro52 and PMScl75. Use of local reference ranges reduced detection of MSAs to 16.9% of myositis patients and 3% of healthy controls, with MAAs 23.4% of patients and 2% of healthy controls. Application of population based reference ranges resulted in significant differences in detection of MSAs and MAAs compared to the manufacturer's recommended ranges. Cut-off levels should be assessed to ensure suitability for the population tested. Copyright © 2016. Published by Elsevier B.V.

Decay-accelerating factor 1 deficiency exacerbates Trypanosoma cruzi-induced murine chronic myositis.

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Solana, María E; Ferrer, María F; Novoa, María Mercedes; Song, Wen-Chao; Gómez, Ricardo M

2012-10-01

Murine infection with Trypanosoma cruzi (Tc) has been used to study the role of T-cells in the pathogenesis of human inflammatory idiopathic myositis. Absence of decay-accelerating factor 1 (Daf1) has been shown to enhance murine T-cell responses and autoimmunity. To determine whether Daf1 deficiency can exacerbate Tc-induced myositis, C57BL/6 DAF(+/+) and DAF(-/-) mice were inoculated with 5 × 10(4) trypomastigotes, and their morbidity, parasitemia, parasite burden, histopathology, and T-cell expansion were studied in the acute and chronic stages. DAF(-/-) mice had lower parasitemia and parasite burden but higher morbidity, muscle histopathology, and increased number of CD44(+) (activated/memory phenotype) splenic CD4(+) and CD8(+) T-cells. An enhanced CD8(+) T-cell immune-specific response may explain the lower parasitemia and parasite burden levels and the increase in histopathological lesions. We propose that Tc-inoculated DAF(-/-) mice are a useful model to study T-cell mediated immunity in skeletal muscle tissues. Copyright © 2012 Wiley Periodicals, Inc.

Childhood Acute Myeloid Leukemia Treatment (PDQ®)—Patient Version

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Childhood acute myeloid leukemia and other myeloid malignancies treatment may include chemotherapy, radiation therapy, stem cell transplant, and targeted therapy. Learn more about AML and myelodysplastic/myeloproliferative diseases in this expert-reviewed summary.

Merits of magnetic resonance imaging (MRI) for the diagnosis of myositis ossificans circumscripta. Apport de l'imagerie par resonance magnetique (IRM) dans le diagnostic de la myosite ossifiante circonscrite (MOC)

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Bouchardy, L.; Garcia, J. (Hopital Cantonal Geneve, Geneva (Switzerland))

1994-02-01

A retrospective study of 5 cases, 4 of myositis ossificans circumscripta (MOC) and 1 of non-ossificans myositis, is presented. The clinical presentation was a painful soft-tissues swelling, and the final diagnosis was established by biopsy in 3 cases and clinical evolution in 2 cases. Different types of imaging techniques were performed: 4 MRI, 2 arteriographies, 3 Tc 99m scintigraphies, 3 US, 3 CT and plain film radiographs for all patients. MOC is a benign process (as opposed to myositis ossificans progressiva, which is an hereditary pathology with a fatal prognosis) with 3 phases of evolution: an acute or pseudo-inflammatory phase, a sub-acute or pseudo-tumoral phase and a chronic phase with a spontaneous healing. The radiologic diagnostic findings are dependent of the phase of the disease. The calcifications are seen earlier with scintigraphy than plain films, with US being less helpful. A heterogenous mass and calcifications are seen with CT. MRI allows the characterization of oedema during the acute phase and sometimes can exclude a malignant process. MRI is the best method for an early diagnosis, the differential diagnosis and to follow the evolution. (authors). 26 refs., 8 figs.

The EuroMyositis registry

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Lilleker, James B; Vencovsky, Jiri; Wang, Guochun

2018-01-01

AIMS: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. METHODS: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtyp...

Non-infectious chemotherapy-associated acute toxicities during childhood acute lymphoblastic leukemia therapy

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Schmiegelow, Kjeld; Müller, Klaus; Mogensen, Signe Sloth; Mogensen, Pernille Rudebeck; Wolthers, Benjamin Ole; Stoltze, Ulrik Kristoffer; Tuckuviene, Ruta; Frandsen, Thomas

2017-01-01

During chemotherapy for childhood acute lymphoblastic leukemia, all organs can be affected by severe acute side effects, the most common being opportunistic infections, mucositis, central or peripheral neuropathy (or both), bone toxicities (including osteonecrosis), thromboembolism, sinusoidal obstruction syndrome, endocrinopathies (especially steroid-induced adrenal insufficiency and hyperglycemia), high-dose methotrexate-induced nephrotoxicity, asparaginase-associated hypersensitivity, pancreatitis, and hyperlipidemia. Few of the non-infectious acute toxicities are associated with clinically useful risk factors, and across study groups there has been wide diversity in toxicity definitions, capture strategies, and reporting, thus hampering meaningful comparisons of toxicity incidences for different leukemia protocols. Since treatment of acute lymphoblastic leukemia now yields 5-year overall survival rates above 90%, there is a need for strategies for assessing the burden of toxicities in the overall evaluation of anti-leukemic therapy programs. PMID:28413626

Brucellosis presenting as piriformis myositis: a case report

Directory of Open Access Journals (Sweden)

Romanos Odysseas

2011-03-01

Full Text Available Abstract Introduction Myositis is a rare bacterial muscle infection. Involvement of the piriformis muscle has been rarely reported in the literature. In this report we describe a case of piriformis myositis due to Brucella melitensis, which to the best of our knowledge is the first such case presented in the literature. Case presentation We report the case of a 19-year-old Caucasian man who presented to our institution with fever and right hip pain. Brucellosis was suspected, but the clinical suspicion was for spondylodiscitis. A pelvic magnetic resonance imaging scan allowed prompt diagnosis of inflammatory involvement of the right piriformis muscle. Blood culture results were positive for B. melitensis. Our patient was treated with antibiotics, and follow-up magnetic resonance imaging scans showed resolution of the inflammation. Conclusion Brucellosis can present as piriformis myositis. The clinical diagnosis of piriformis myositis is difficult, as it can mimic other common entities such as referred back pain from spondylodiscitis. Magnetic resonance imaging is the method of choice for establishing the diagnosis in the early stages of the disease, as late diagnosis can lead to abscess formation and the need for drainage.

Radiologic Findings in Gabapentin-Induced Myositis.

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Coupal, Tyler Michael; Chang, David Ross; Pennycooke, Kevin; Ouellette, Hugue Alcide; Munk, Peter Loren

2017-04-01

Throughout recent years, Gabapentin has become increasingly used for the treatment of neuropathic pain. We report on a case of a 31 year old female who presented to the emergency department with unilateral leg pain, weakness, and swelling after increasingly titrating her Gabapentin dosage over three weeks. Magnetic resonance imaging confirmed the presence of myositis confined to the left thigh and the patient's symptoms and laboratory abnormalities resolved following Gabapentin cessation. While Gabapentin-induced myositis and rhabdomyolysis is a rare entity, it should be a diagnostic consideration for radiologists, particularly in the absence of infection or trauma.

Management of acute moderate and severe childhood malnutrition

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Acute childhood malnutrition affects about a tenth of the world's children under 5 years of age, particularly those living in circumstances of extreme poverty in the developing world. Malnutrition is typically the result of an inadequate diet and is one of the most common diagnoses in children in he...

Significance of myositis autoantibody in patients with idiopathic interstitial lung disease.

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Song, Ju Sun; Hwang, Jiwon; Cha, Hoon-Suk; Jeong, Byeong-Ho; Suh, Gee Young; Chung, Man Pyo; Kang, Eun-Suk

2015-05-01

Some patients with interstitial lung disease (ILD) related to connective tissue disease (CTD) have a delayed diagnosis of the underlying CTD when the ILD is categorized as idiopathic. In this study, we evaluated the frequency of myositis autoantibodies in patients diagnosed with idiopathic ILD and investigated the clinical significance stemming from the presence of the antibodies. A total 32 patients diagnosed with idiopathic ILD were enrolled in this study. We analyzed a panel of 11 myositis autoantibody specificities in the patients using a line blot immunoassay. Then, we divided them into myositis autoantibody-positive and -negative groups and compared the clinical features and laboratory data between the two groups. Of the 32 idiopathic ILD patients, 12 patients had myositis autoantibodies encompassing 9 specificities, except for anti-Mi-2 and anti-PM-Scl 100 (12/32, 38%). Anti-synthetase autoantibodies including Jo-1, EJ, OJ, PL-7, and PL-12 were present in 7 patients (7/32, 22%). The group with myositis autoantibodies presented more frequently with the symptom of mechanic's hand and showed abnormal pulmonary function test results with low forced vital capacity, diffusing capacity for carbon monoxide, total lung capacity, and high lactate dehydrogenase values in blood when compared with the group without myositis antibodies. We strongly suggest that patients undergo an evaluation of myositis autoantibodies, if they are diagnosed with idiopathic ILD in the presence of clinical characteristics including mechanic's hand, arthralgia, and autoantibodies which are insufficient to make a diagnosis of a specific CTD category.

Childhood Acute Myeloid Leukemia Treatment (PDQ®)—Health Professional Version

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Acute myeloid leukemia (AML), juvenile myelomonocytic leukemia (JMML), acute promyelocytic leukemia (APL) and chronic myeloid leukemia (CML) account for about 20% of childhood myeloid leukemias. Other myeloid malignancies include transient abnormal myelopoiesis and myelodysplastic syndrome. Get detailed information about the classification, clinical presentation, diagnostic and molecular evaluation, prognosis, and treatment of newly diagnosed and recurrent disease in this summary for clinicians.

Treatment-related mortality in relapsed childhood acute lymphoblastic leukemia

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Oskarsson, Trausti; Söderhäll, Stefan; Arvidson, Johan

2018-01-01

BACKGROUND: Treatment of relapsed childhood acute lymphoblastic leukemia (ALL) is particularly challenging due to the high treatment intensity needed to induce and sustain a second remission. To improve results, it is important to understand how treatment-related toxicity impacts survival...

Childhood vaccinations and risk of acute lymphoblastic leukaemia in children

DEFF Research Database (Denmark)

Søegaard, Signe Holst; Rostgaard, Klaus; Schmiegelow, Kjeld

2017-01-01

information on ALL subtypes. Using Cox regression, we estimated hazard ratios (HRs) comparing vaccinated with unvaccinated children.Results: Childhood ALL was diagnosed in 490 children during 10 829 194 person-years of follow-up. Neither the total number of vaccine doses received nor exposure to each......Background: It has been proposed that childhood vaccinations protect against acute lymphoblastic leukaemia (ALL) in children by modulation of future responses to common infections in childhood. However, the available studies provide inconsistent findings, and population-based cohort studies...... with longitudinal information on vaccinations are lacking.Methods: In a register-based cohort of all children born in Denmark from 1 January 1990 to 31 December 2008, followed up until age 15 years or 31 December 2009 (n=1 225 404), we evaluated exposure to childhood vaccination and risk of childhood ALL, including...

Update on the pharmacological treatment of adult myositis.

Science.gov (United States)

Oddis, C V

2016-07-01

The management of patients with idiopathic inflammatory myopathy (IIM) remains a challenge given the systemic features beyond active myositis. That is, recognizing the inflammatory arthropathy, varying dermatomyositis rashes, and overt and occult features of interstitial lung disease in addition to myositis adds to the complexity of diagnosis and treatment of IIM. However, clinicians now have available many more immunosuppressive drugs as well as biologic agents for use in patients with myositis and other autoimmune diseases. Here, the use of these agents is reviewed and support based on available published literature is provided even though many studies have been small and results somewhat anecdotal. Glucocorticoids remain the initial treatment of choice in most instances and methotrexate and azathioprine are often used early in the treatment course. These agents are followed by other immunosuppressive drugs, for example mycophenolate mofetil, tacrolimus, cyclosporine and cyclophosphamide, some of which are used alone while combinations of these agents also provide an effective option. There is more rationale for the use of biologic agents such as rituximab from a mechanistic perspective and, given the incorporation of validated core set measures in assessing myositis patients, we can look forward to better designed clinical trials in the future. © 2016 The Association for the Publication of the Journal of Internal Medicine.

Focal myositis of lower extremity responsive to botulinum A toxin.

Science.gov (United States)

Mitrovic, Josko; Prka, Zeljko; Zic, Rado; Marusic, Srecko; Morovic-Vergles, Jadranka

2014-01-01

Focal myositis is a rare, mostly benign disease (pseudotumor) of skeletal muscle, histopathologically characterized by interstitial myositis and tumorous enlargement of a single muscle. The etiology of focal myositis remains unknown; however, localized myopathy has been postulated to be caused by denervation lesions. This case report describes a patient that presented with clinical, laboratory, electromyoneurography, and magnetic resonance imaging features of focal myositis complicated with intervertebral disk protrusion in the lumbosacral spine affected with radicular distress. In most cases, focal myositic lesions show spontaneous regression, relapses are rare, and long-term prognosis is good. There is a wide spectrum of therapeutic options, from no therapy at all through nonsteroidal antirheumatics and glucocorticoids to radiotherapy, surgical excision, and immunosuppressants. In the patient presented, treatment with glucocorticoids, methotrexate, and surgical excision failed to produce satisfactory results. Clinical improvement, pain relief, and reduction in lower leg volume were only achieved by local infiltration of botulinum A toxin.

Pharmacogenetics Influence Treatment Efficacy in Childhood Acute Lymphoblastic Leukemia

DEFF Research Database (Denmark)

Devidsen, M.L.; Dalhoff, K.; Schmiegelow, K.

2008-01-01

in treatment resistance and toxic side effects. As most childhood acute lymphoblastic leukemia treatment protocols include up to 13 different chemotherapeutic agents, the impact of individual SNPs has been difficult to evaluate. So far Focus has mainly been on the widely used glucocorticosteroids, methotrexate...

MASTICATORY MUSCLE MYOSITIS IN A GRAY WOLF (CANIS LUPUS).

Science.gov (United States)

Kent, Marc; Glass, Eric N; Castro, Fernando A; Miller, Andrew D; de Lahunta, Alexander

2017-03-01

A 10-yr-old male, neutered gray wolf ( Canis lupus ) was presented for atrophy of the temporalis and masseter muscles. Clinical signs and magnetic resonance imaging were consistent with a myopathy. Positive serology for antibody titers directed against Type 2M myofibers, and the observation of a mixed mononuclear inflammatory cell infiltrate along with eosinophils and neutrophils within the temporalis muscle, were diagnostic for masticatory muscle myositis. Importantly, protozoal myositis was excluded based on other clinicopathologic data. The case highlights the potential for immune-mediated polymyositis in canids other than the domesticated dog ( Canis lupus familaris). Additionally, awareness of a diet in which raw meat is used should prompt a thorough investigation for an underlying infectious myositis in the gray wolf.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) presenting as diffuse myositis.

Science.gov (United States)

Parent, Marc-Etienne; Larue, Sandrine; Ellezam, Benjamin

2014-11-21

Eosinophilic granulomatosis with polyangiitis is a complex multisystemic syndrome with heterogeneous presentation. Most often, there is a clinical history of asthma or other atopic conditions, and current presentation generally includes signs of cutaneous or pulmonary involvement. Very few reports described myalgia or weakness as the chief complaint. Of these, only a few included muscle biopsy evaluation and none showed convincing evidence of primary myositis. We believe this report is the first to demonstrate true myositis in the setting of early eosinophilic granulomatosis with polyangiitis. This report describes a 74 year old Caucasian man, with no known allergies, presenting severe myalgia, muscle weakness, jaw claudication, and fever. Blood work showed marked eosinophilia and high creatine kinase levels. Biceps brachialis muscle biopsy revealed eosinophilic necrotizing vasculitis and true myositis with myophagocytosis of non-necrotic fibers and strong sarcolemmal MHC-1 overexpression by immunohistochemistry. This patient was successfully treated with prednisone and azathioprine. Our finding of true myositis in a case of eosinophilic granulomatosis with polyangiitis suggests that primary auto-immunity against muscle fibers, distinct from the secondary effects of vasculitis, can occur in this entity and may represent an overlap syndrome. Early recognition of eosinophilic granulomatosis with polyangiitis in patients presenting with myositis may provide an opportunity to treat the vasculitis before onset of severe multisystemic disease. We recommend the use of muscle biopsy with immunohistochemistry for MHC-1 to confirm the diagnosis of myositis in the setting of eosinophilic granulomatosis with polyangiitis.

Myositis ossificans localisata pseudomalignant Form

International Nuclear Information System (INIS)

Grunt, J.; Jankovich, E.; Vasovicova, M.

1994-01-01

Authors presents computer tomography and angiographic findings of rare pseudomalignant form of myositis ossificans. Correct diagnosis achieved by complex evaluation of ascertain findings, including biopsy, enables proper treatment with excluding too radical therapy. 3 figs., 4 refs

Gemfibrozil-induced myositis in a patient with normal renal function.

Science.gov (United States)

Hahn, Martin; Sriharan, Kalavally; McFarland, M Shawn

2010-01-01

To describe a case of gemfibrozil monotherapy-induced myositis in a patient with normal renal function A 68-year-old white man presented to his primary care clinic complaining of a 6-month history of total body pain. His past medical history was significant for hypertension, diabetes mellitus, hyperlipidemia, gastroesophageal reflux disease, benign prostatic hypertrophy, arthritis, impotence, and pancreatic cancer that required excision of part of his pancreas. His home drug regimen included bupropion 75 mg twice daily, gemfibrozil 600 mg twice daily for the past 8 months, glimiperide 1 mg daily, insulin glargine 5 units at bedtime, insulin aspart 5 units in the evening, lisinopril 10 mg daily, omeprazole 40 mg daily, pregabalin 100 mg daily, and sildenafil 100 mg as needed. Laboratory test results were significant for elevated aspartate aminotransferase (AST) 78 U/L (reference range 15-46 U/L), alanine aminotransferase (ALT) 83 U/L (13-69 U/L), and creatine kinase (CK) 3495 U/L (55-170 U/L). Serum creatinine was normal at 1.19 mg/dL. The physician determined that the elevated CK indicated myositis secondary to gemfibrozil use, and gemfibrozil was subsequently discontinued. The patient returned 1 week later to repeat the laboratory tests. Results were CK 220 U/L, AST 26 U/L, ALT 43 U/L, and serum creatinine 1.28 mg/dL. The patient was asked to return in 3 weeks to repeat the laboratory tests. At that time, CK had continued to decrease to 142 U/L, and the AST and ALT had returned to normal, at 22 and 29 U/L, respectively. The patient reported complete resolution of total body pain 3 weeks after discontinuation of gemfibrozil. Follow-up 5 weeks after discontinuation revealed no change compared to the 3-week follow-up. Myositis most often produces weakness and elevated CK levels more than 10 times the upper limit of normal. The risk of developing myositis, myopathy, or rhabdomyolysis is low (1%) when fibrates such as gemfibrozil are used as monotherapy. Evaluation of

Periostitis and localised myositis in polyarteritis nodosa

International Nuclear Information System (INIS)

Macdonald, W.B.G.; Blake, M.P.

2003-01-01

Full text: A 20-year-old man with previously diagnosed polyarteritis nodosa was referred for a bone scan to investigate longstanding knee and lower leg pain. The patient's symptoms worsened with any reduction of steroid dose and his physician was concerned about avascular necrosis of the hips. Plain x-rays of the lower limbs were normal. The blood pool images showed bilaterally increased activity in the anterior muscle compartments of the lower legs, suggestive of localised myositis. Three-hour delayed images showed widespread, increased subperiosteal activity with no evidence of avascular necrosis. Subsequent MRI scanning showed patchy muscle enhancement in both lower legs, also typical of myositis. A muscle biopsy was performed which demonstrated features of both myositis and vasculitis. The patient remains dependent on high-dose steroids for symptom relief. Localised myositis has previously been reported in polyarteritis nodosa and is a recognised, albeit rare, complication of the disorder, the basis of which is not well understood. Diagnostic muscle biopsy should be directed at involved muscle groups, which are best detected with MRI. Lower limb periostitis is well described in polyarteritis nodosa and may result in gross deformity. Several cases have previously been reported in the literature based on radiographic abnormalities, which were not present in our patient. Bone scanning demonstrated the sub-periosteal activity well in our subject and is suggested as a useful investigation in patients with polyarteritis nodosa who complain of lower limb pain. Copyright (2003) The Australian and New Zealand Society of Nuclear Medicine Inc

Increased visfatin levels are associated with higher disease activity in anti-Jo-1-positive myositis patients.

Science.gov (United States)

Hulejová, Hana; Kryštůfková, Olga; Mann, Heřman; Klein, Martin; Pavlíčková, Klára; Zámečník, Josef; Vencovský, Jiří; Šenolt, Ladislav

2016-01-01

The aim of this study was to evaluate serum levels of visfatin in anti-Jo-1-positive myositis patients, its expression in muscle tissue and to investigate potential relationships between visfatin, B-cell activating factor of the TNF family (BAFF), disease activity and anti-Jo-1 autoantibody levels. Serum levels of visfatin and BAFF were measured in 38 anti-Jo-1 positive myositis patients and 35 healthy subjects. Disease activity was evaluated by myositis disease activity assessment tool (MYOACT) using visual analogue scales (VAS) and by serum muscle enzymes. Visfatin expression was evaluated by immunohistochemistry in muscle tissue of myositis patients (n=10) and compared with non-inflammatory control muscle tissue samples from patients with myasthenia gravis (n=5). Serum visfatin and BAFF levels were significantly higher in myositis patients compared to healthy subjects and were associated with clinical muscle activity assessed by VAS. Only serum BAFF levels, but not visfatin levels, positively correlated with muscle enzyme concentrations and anti-Jo1 antibody levels. There was a positive correlation between visfatin and BAFF serum levels in myositis patients but a negative correlation was observed in healthy subjects. Visfatin expression was up-regulated in endomysial and perimysial inflammatory infiltrates of muscle tissue from myositis patients. Up-regulation of visfatin in myositis muscle tissue and an association between increased visfatin levels and muscle disease activity evaluated by MYOACT in anti-Jo-1 positive myositis patients could support possible role of visfatin in the pathogenesis of myositis.

Lateral rectus myositis mimicking an abducens nerve palsy in a pregnant woman.

Science.gov (United States)

Haslinda, Abd-Rahim; Shatriah, Ismail; Azhany, Yaakub; Nik-Ahmad-Zuky, Nik-Lah; Yunus, Rohaizan

2014-01-01

Myositis is a rare unknown inflammatory disorder of the skeletal muscle tissue. Generalized inflammatory myopathies, polymyositis, and dermatomyositis have been reported during pregnancy. Isolated orbital myositis in pregnancy has not been previously described in the literature. The authors report a case of left isolated orbital myositis in a primigravida at 38 weeks gestation affecting the patient's left lateral rectus muscle. MRI of the orbit was consistent with the diagnosis. She showed remarkable clinical improvement with oral corticosteroids therapy.

TEST performance of a myositis panel in a clinical immunology laboratory in New South Wales, Australia.

Science.gov (United States)

Tan, Teck Choon; Wienholt, Louise; Adelstein, Stephen

2016-10-01

There is increasing recognition of a clinico-serological correlation between the idiopathic inflammatory myopathies and myositis-specific autoantibodies (MSA). We review the use of a line immunoassay-based myositis panel incorporating both MSA and myositis-associated autoantibodies (MAA) in a selected population of patients. A retrospective analysis of patients with myositis panel assays performed in 2013 were reviewed and compared against clinical diagnoses. A total of 96 patient samples were evaluated, the clinical indications include 60 patients with suspected idiopathic inflammatory myositis (IIM), 24 patients with suspected interstitial lung disease (ILD) and 12 patients with suspected systemic autoimmune disease (SAD). In the myositis group, there were 21 patients diagnosed with IIM and 18 patients diagnosed with IIM had a positive myositis panel. Of the 39 patients without IIM, nine of these patients had a positive myositis panel. In the ILD group, 10 of 24 patients had a positive myositis panel; of these, two were diagnosed anti-synthetase syndrome (ASS) and five patients with ILD. In the suspected SAD group, three had positive myositis panel and all did not appear associated with their final diagnoses. In patients with a clinical diagnosis of IIM or ILD-associated SAD, four patients with anti-PL-12 were detected, three patients with anti-signal recognition protein, two patients with anti-Jo-1, and two patients with anti-Mi2. The myositis panel is an objective investigative modality with a sensitivity of 80.00% and a specificity of 75.76% in a setting of high pretest clinical suspicion. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

Relapsed childhood acute lymphoblastic leukemia in the Nordic countries

DEFF Research Database (Denmark)

Oskarsson, Trausti; Söderhäll, Stefan; Arvidson, Johan

2016-01-01

Relapse is the main reason for treatment failure in childhood acute lymphoblastic leukemia. Despite improvements in the up-front therapy, survival after relapse is still relatively poor, especially for high-risk relapses. The aims of this study were to assess outcomes following acute lymphoblastic...... leukemia relapse after common initial Nordic Society of Paediatric Haematology and Oncology protocol treatment; to validate currently used risk stratifications, and identify additional prognostic factors for overall survival. Altogether, 516 of 2735 patients (18.9%) relapsed between 1992 and 2011 and were...

Rare myositis-specific autoantibody associations among Hungarian patients with idiopathic inflammatory myopathy.

Science.gov (United States)

Bodoki, L; Nagy-Vincze, M; Griger, Z; Betteridge, Z; Szöllősi, L; Jobanputra, R; Dankó, K

2015-01-01

Idiopathic inflammatory myopathies are systemic, chronic autoimmune diseases characterized by symmetrical, proximal muscle weakness. Homogeneous groups present with similar symptoms. The response to therapy and prognosis could be facilitated by myositis-specific autoantibodies, and in this way, give rise to immunoserological classification. The myositis-specific autoantibodies are directed against specific proteins found in the cytoplasm or in the nucleus of the cells. To date, literature suggests the rarity of the co-existence of two myositis-specific autoantibodies. In this study the authors highlight rare associations of myositis-specific autoantibodies. Three hundred and thirty-seven Hungarian patients with polymyositis or dermatomyositis were studied. Their clinical findings were noted retrospectively. Specific blood tests identified six patients with the rare co-existence of myositis-specific autoantibodies, anti-Jo-1 and anti-SRP, anti-Jo-1 and anti-Mi-2, anti-Mi-2 and anti-PL-12, anti-Mi-2 and anti-SRP, and anti-SRP and anti-PL-7, respectively. This case review aims to identify the clinical importance of these rare associations and their place within the immunoserological classification.

Pseudomalignant myositis ossificans involving multiple masticatory muscles: Imaging evaluation

International Nuclear Information System (INIS)

Kamalapur, Muralidhar G; Patil, Pritam B; Joshi, Shyamsundar; Shastri, Dinesh

2014-01-01

Myositis ossificans is a rare cause of trismus. We present a case of pseudomalignant myositis ossificans involving medial pterygoid, lateral pterygoid, and temporalis muscles. Patient presented with gross limitation in mouth opening. There was no history of trauma. Computed tomography (CT) images revealed a bone density mass located in the region of medial and lateral pterygoid muscles on the right and temporalis muscle on the left. Magnetic resonance imaging (MRI) showed similar findings. Radiological diagnosis was pseudomalignant myositis ossificans. The masses were resected and histopathologic examination confirmed the above diagnosis. This report describes the characteristic CT and MRI features. The unique feature of this case is the absence of history of trauma with involvement of multiple masticatory muscles, which, to the best of our knowledge, has not been reported before

Scintigraphic evaluation of Lyme disease: Gallium-67 imaging of Lyme myositis

International Nuclear Information System (INIS)

Kengen, R.A.; Linde, M. von der; Sprenger, H.G.; Piers, D.A.

1989-01-01

A patient suffering from Lyme disease had cardiac conduction abnormalities, symptoms of arthritis, and myalgia. A Ga-67 image showed evidence of endomyocarditis, but intense skeletal muscle uptake pointed to Lyme myositis. Reference is made to two other case reports of Lyme myositis

International collaboration including patients is essential to develop new therapies for patients with myositis.

Science.gov (United States)

Lundberg, Ingrid E; Vencovsky, Jiri

2017-05-01

To discuss the needs for international collaborations between investigators in different disciplines working with myositis and with patients with myositis. Recent advances in detection of several myositis-specific autoantibodies that are associated with distinct clinical phenotypes, will enable studies in new well defined clinically homogenous subgroups of myositis This is likely to lead to development of new information on molecular pathogenesis that might be different in different myositis subgroups. Subgrouping patients according to autoantibody profile may also be important to assess outcome, to identify prognostic biomarkers and in clinical trials. As these are rare disorders international collaboration is essential to enrol large enough cohorts of the subgroups. To facilitate such collaboration we have developed a web-based international myositis register, www.euromyositis.eu, which includes validated outcome measures and patient reported outcome measures. This register is to support research but also to support decision-making in the clinic. We welcome investigators to join the Euromyositis register. Myositis is a heterogeneous disorder with varying treatment response and outcome. There is a high unmet need for new therapies which can only be achieved by increased knowledge on molecular disease mechanisms. Subgrouping patients according to autoantibody profile may be a new way forward to get a better understanding on disease mechanisms and to develop novel therapies.

Asparaginase-associated pancreatitis in childhood acute lymphoblastic leukaemia

DEFF Research Database (Denmark)

Wolthers, Benjamin O.; Frandsen, Thomas L.; Baruchel, André

2017-01-01

BACKGROUND: Survival for childhood acute lymphoblastic leukaemia surpasses 90% with contemporary therapy; however, patients remain burdened by the severe toxic effects of treatment, including asparaginase-associated pancreatitis. To investigate the risk of complications and risk of re......-exposing patients with asparaginase-associated pancreatitis to asparaginase, 18 acute lymphoblastic leukaemia trial groups merged data for this observational study. METHODS: Patient files from 26 trials run by 18 trial groups were reviewed on children (aged 1·0-17·9 years) diagnosed with t(9;22)-negative acute...... lymphoblastic leukaemia between June 1, 1996, and Jan 1, 2016, who within 50 days of asparaginase exposure developed asparaginase-associated pancreatitis. Asparaginase-associated pancreatitis was defined by at least two criteria: abdominal pain, pancreatic enzymes at least three times the upper limit of normal...

[Effects of birth order, maternal abortion and mode of delivery on childhood acute leukemia risk: a meta-analysis].

Science.gov (United States)

Zou, Guobin; Sha, Xia

2014-03-01

To evaluate the associations between birth order, maternal abortion and mode of delivery and childhood acute leukemia risk. Multiple electronic databases were searched to identify relevant studies up to March 2013 using the search terms "childhood leukemia", "acute lymphoblastic leukemia", "acute myeloid leukemia","birth order", "abortion", "miscarriage", "cesarean", "birth characteristics" and "prenatal risk factor". Data from cohort and case-control studies were analyzed using the Stata software. Twenty-three studies were included in this meta-analysis according to the selection criteria. No significant associations were identified for birth order and mode of delivery (birth order = 2: OR = 0.97, 95%CI: 0.89-1.05; birth order = 3: OR = 1.00, 95%CI: 0.91-1.11; birth order ≥ 4: OR = 1.02, 95%CI: 0.87-1.20; mode of delivery: OR = 1.05, 95%CI: 0.96-1.15). However, there was a significant association between maternal abortion and childhood acute leukemia risk (spontaneous abortion: OR = 1.21, 95%CI: 1.05-1.41; induced abortion: OR = 1.23, 95%CI: 1.07-1.43). Furthermore, the stratified analysis by disease subtypes showed that spontaneous and induced abortions were significantly associated with the risks of childhood acute myeloid leukemia (OR = 1.71, 95%CI: 1.09-2.70) and acute lymphoblastic leukemia (OR = 1.23, 95%CI: 1.05-1.42), respectively. This meta-analysis revealed that maternal abortion might contribute to the childhood acute leukemia risk.

Focal myositis of the thigh: unusual MR pattern

International Nuclear Information System (INIS)

Llauger, Jaume; Palmer, Jaume; San Roman, Luis; Bague, Silvia; Matias-Guiu, Xavier; Doncel, Antonio

2002-01-01

Focal myositis is a commonly referenced, infrequently reported and poorly documented benign inflammatory pseudotumor which may be misdiagnosed clinically as a malignant tumor. We report the clinicopathologic features and magnetic resonance imaging findings in a case of focal myositis in the thigh of a 55-year-old woman. A different radiologic presentation of this disorder is described. The gross appearance of the lesion, previously undescribed, appears to be rather specific for such a pseudoneoplastic disorder, and correlates very well with the magnetic resonance imaging features. (orig.)

Focal myositis of the thigh: unusual MR pattern

Energy Technology Data Exchange (ETDEWEB)

Llauger, Jaume; Palmer, Jaume; San Roman, Luis [Department of Radiology, Hospital de la Santa Creu i Sant Pau, Barcelona (Spain); Bague, Silvia; Matias-Guiu, Xavier [Department of Pathology, Hospital de la Santa Creu i Sant Pau, Barcelona (Spain); Doncel, Antonio [Department of Orthopedic Surgery, Hospital de la Santa Creu i Sant Pau, Barcelona (Spain)

2002-05-01

Focal myositis is a commonly referenced, infrequently reported and poorly documented benign inflammatory pseudotumor which may be misdiagnosed clinically as a malignant tumor. We report the clinicopathologic features and magnetic resonance imaging findings in a case of focal myositis in the thigh of a 55-year-old woman. A different radiologic presentation of this disorder is described. The gross appearance of the lesion, previously undescribed, appears to be rather specific for such a pseudoneoplastic disorder, and correlates very well with the magnetic resonance imaging features. (orig.)

Necrotizing streptococcal myositis of the upper extremity: a case report.

Science.gov (United States)

Reichert, Johannes C; Habild, Götz; Simon, Paul; Nöth, Ulrich; Krümpelmann, Jan B

2017-08-15

Necrotizing myositis is a rare but life-threatening soft-tissue infection characterized by rapidly spreading inflammation and subsequent necrosis of the affected tissue. The myositis is often caused by toxin-producing, virulent bacteria such as group A β-hemolytic streptococcus and associated with severe systemic toxicity. It is rapidly fatal unless diagnosed promptly and treated aggressively. However, necrotizing myositis is often initially misdiagnosed as a more benign soft-tissue infection as such fulminant, invasive muscle infections are rare with no more than 30 cases reported over the last century. We illustrate the case of a 74-year-old male Caucasian initially presenting with a progressing swelling and gradually oncoming pain of the upper right extremity. Rapidly, livid discolorations of the skin, blisters, hypoesthesia and severe pain resistant to analgesics treatment developed accompanied by disruption of the arterial blood flow. Due to a manifest compartment syndrome the patient was admitted to theater for fasciotomy of the arm. After multiple revision surgeries wound closure was achieved using a pedicled, fasciocutaneous parascapular flap and a free, ipsilateral anterolateral thigh flap. Microbiological analysis revealed group A β-hemolytic streptococcus, histology a bacterial interstitial myositis with necrotic muscular fibers. A high degree of clinical suspicion is necessary to avert potentially disastrous consequences of necrotizing myositis. Timely diagnosis, broad-spectrum antibiotic therapy, and aggressive surgical debridement of affected tissue are keys to the treatment of this serious, often life-threatening infection.

Myositis ossificans progressiva : a report of two cases

Energy Technology Data Exchange (ETDEWEB)

Kim, Eun Ah; Lee, Sun Wha [Ewha Womans Univ., Seoul (Korea, Republic of). Coll. of Medicine; Han, Tae Il [Kyung Hee University, Seoul (Korea, Republic of). Coll. of Medicine

1997-12-01

Myositis ossificans progressive is rare hereditable disorder characterized progressiva heterotopic bone formation in connective tissue and muscles in association with congenital skeletal anomalies. We report the plain radiologic and MR findings of myositis ossificants progressiva in two children. One case showed discrete ossification in the right buttock, neck, and both chest walls on plain radiographs, while the other showed diffuse swelling of the left posterior neck, back, and buttock muscles, which was demonstrated on MR images. Both case showed associated anomalies in the hands and feet. (author). 8 refs., 2 figs.

Myositis ossificans progressiva : a report of two cases

International Nuclear Information System (INIS)

Kim, Eun Ah; Lee, Sun Wha; Han, Tae Il

1997-01-01

Myositis ossificans progressive is rare hereditable disorder characterized progressiva heterotopic bone formation in connective tissue and muscles in association with congenital skeletal anomalies. We report the plain radiologic and MR findings of myositis ossificants progressiva in two children. One case showed discrete ossification in the right buttock, neck, and both chest walls on plain radiographs, while the other showed diffuse swelling of the left posterior neck, back, and buttock muscles, which was demonstrated on MR images. Both case showed associated anomalies in the hands and feet. (author). 8 refs., 2 figs

Inclusion body myositis.

OpenAIRE

Garlepp, M J; Mastaglia, F L

1996-01-01

The idiopathic inflammatory myopathies (IIM) are a heterogenous group of rare disorders that share many similarities. In addition to sporadic inclusion body myositis (IBM), these include dematomyositis (DM), polymyositis (PM), and autoimmune necrotizing myopathy (NM). For discussion of later three disorders, the reader is referred to the IIM review in this issue. IBM is the most common IIM after age 50. It typically presents with chronic insidious proximal leg and/or distal arm asymmetric mus...

Second Malignant Neoplasms After Treatment of Childhood Acute Lymphoblastic Leukemia

DEFF Research Database (Denmark)

Schmiegelow, K.; Levinsen, Mette Frandsen; Attarbaschi, Andishe

2013-01-01

PURPOSE: Second malignant neoplasms (SMNs) after diagnosis of childhood acute lymphoblastic leukemia (ALL) are rare events. PATIENTS AND METHODS: We analyzed data on risk factors and outcomes of 642 children with SMNs occurring after treatment for ALL from 18 collaborative study groups between 19...

Myositis ossificans with atypical clinical, radiographic, or pathologic findings

International Nuclear Information System (INIS)

Nuovo, M.A.; Chumas, J.; Ackerman, L.V.

1992-01-01

Myositis ossificans is a relatively rare, well-defined entity. The 23 cases sent for consultation to two of us (L.V.A. and A. N.) were reviewed. Clinical, radiologic, and microscopic information was reexamined, and special attention was given to features infrequently seen in typical myositis ossificans. Due to the uncommon location of 15 lesions and an unusual presentation in 5, the correct diagnosis was not obvious in these cases. Radiologic studies raised the possibility of a malignant bone-forming tumor in at least three instances; myositis ossificans was originally diagnosed in 6 cases radiologically. In 8 cases, histologic evidence suggested malignancy, including osteosarcoma, either parosteal or extraosseous, in 6. Other diagnoses included epithelioid sarcoma and callus formation. Presentation of these variations from the norm highlights the importance of recognizing the evolution of a nonneoplastic fibro-osseous and cartilaginous entity in which conservative treatment is curative. (orig./GDG)

Brain hypothermia therapy for childhood acute encephalopathy based on clinical evidence

OpenAIRE

IMATAKA, GEORGE; ARISAKA, OSAMU

2015-01-01

Although previous studies have reported on the effectiveness of brain hypothermia therapy in childhood acute encephalopathy, additional studies in this field are necessary. In this review, we discussed brain hypothermia therapy methods for two clinical conditions for which sufficient evidences are currently available in the literature. The first condition is known as hypoxic-ischemic encephalopathy and occurs in newborns and the second condition is acute encephalopathy which occurs in adults ...

The Co-Existence of Myasthenia Gravis in Patients with Myositis: A Case Series

Science.gov (United States)

Paik, Julie J.; Corse, Andrea M.; Mammen, Andrew L.

2014-01-01

Objective Myositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients. Methods We identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features. Results All 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%). High dose steroids were associated with worsening weakness in 2/6 (33%) patients. Conclusions Prominent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis-MG overlap, high dose steroids may exacerbate symptoms and pryidostigmine may play an important therapeutic role. PMID:24412588

Potential Environmental Triggers of Myositis

Science.gov (United States)

2012-10-01

expression disturbances in myositis patient samples at the whole genome level. With our collaborators at the Karolinska Institute and Genpathway, Inc. we... Karolinska Institute in Stockholm, Sweden. Aim 1 Methods: A total of 500 ng of genomic DNA was used for bisulfate conversion using a Bisulfite

Inclusion body myositis. Clinical aspects

NARCIS (Netherlands)

Cox, Fieke Maria Elisabeth

2014-01-01

Sporadische inclusion body myositis (IBM) is een van de meest voor voorkomende verworven spierziekte die ontstaat na het 50e levensjaar. In dit proefschrift worden de klinische aspecten van sporadische IBM beschreven. Uit de studie met betrekking tot het natuurlijk beloop blijkt dat de ziekte niet

Etiology of common childhood acute lymphoblastic leukemia: the adrenal hypothesis

DEFF Research Database (Denmark)

Schmiegelow, K.; Vestergaard, T.; Nielsen, S.M.

2008-01-01

The pattern of infections in the first years of life modulates our immune system, and a low incidence of infections has been linked to an increased risk of common childhood acute lymphoblastic leukemia (ALL). We here present a new interpretation of these observations--the adrenal hypothesis...

Sjögren's syndrome-associated myositis with germinal centre-like structures.

Science.gov (United States)

Espitia-Thibault, Alexandra; Masseau, Agathe; Néel, Antoine; Espitia, Olivier; Toquet, Claire; Mussini, Jean-Marie; Hamidou, Mohamed

2017-02-01

Muscular impairment is a rare systemic manifestation of SS that is rarely described in the literature and classically non-specific, both clinically and histologically. We reviewed the cases of 4 patients with primary SS presenting with myositis and a common histologic pattern on muscular biopsy with germinal centre-like structures resembling that which occurs in salivary glands. We analysed the data files of patients with SS who had muscular manifestations and underwent a muscular biopsy. Among 23 patients with SS who had muscle biopsies, 13 had non-specific myositis and 10 (4 primary and 6 secondary SS) had a common histologic pattern consisting of germinal centre-like structures. We analysed the data files of the 4 patients with primary SS presenting with myositis with muscular germinal-centre like structures. The 4 patients had an unspecific clinical presentation, with myalgias, muscular weakness and normal or elevated values of CPK. In the four patients, SS-associated myositis had common histologic characteristics, with endomysial and perimysial inflammatory infiltrate. The cellular infiltrate was composed predominantly of CD4+ T lymphocytes and B lymphocytes. The B and T CD4+ cells infiltrates may gather into masses, even forming lymphoid follicles. Three patients were treated with corticosteroids and/or hydroxychloroquine with improvement of myositis and 1 patient was lost to follow-up. We describe four patients with a common histologic appearance of myositis with lymphoid follicles associated with primary SS. The clinical presentation was non-specific and non-severe, with favorable outcome with corticosteroids and/or hydroxycholoroquine. The discovery of this particular histologic appearance in a muscle biopsy independent of the final diagnosis should indicate the possibility of SS. Copyright © 2016 Elsevier B.V. All rights reserved.

Myositis with endomysial cell invasion indicates inclusion body myositis even if other criteria are not fulfilled

NARCIS (Netherlands)

Van de Vlekkert, J.; Hoogendijk, J. E.; de Visser, M.

2015-01-01

The objective of this study was to investigate if patients with endomysial mononuclear cell infiltrates invading non-necrotic fibers have a disease course consistent with inclusion body myositis (IBM), irrespective of other histopathological and clinical characteristics. All patients with a muscle

An Autoimmune Myositis-Overlap Syndrome Associated With Autoantibodies to Nuclear Pore Complexes

Science.gov (United States)

Senécal, Jean-Luc; Isabelle, Catherine; Fritzler, Marvin J.; Targoff, Ira N.; Goldstein, Rose; Gagné, Michel; Raynauld, Jean-Pierre; Joyal, France; Troyanov, Yves; Dabauvalle, Marie-Christine

2014-01-01

Abstract Autoimmune myositis encompasses various myositis-overlap syndromes, each being identified by the presence of serum marker autoantibodies. We describe a novel myositis-overlap syndrome in 4 patients characterized by the presence of a unique immunologic marker, autoantibodies to nuclear pore complexes. The clinical phenotype was characterized by prominent myositis in association with erosive, anti-CCP, and rheumatoid factor-positive arthritis, trigeminal neuralgia, mild interstitial lung disease, Raynaud phenomenon, and weight loss. The myositis was typically chronic, relapsing, and refractory to corticosteroids alone, but remitted with the addition of a second immunomodulating drug. There was no clinical or laboratory evidence for liver disease. The prognosis was good with 100% long-term survival (mean follow-up 19.5 yr). By indirect immunofluorescence on HEp-2 cells, sera from all 4 patients displayed a high titer of antinuclear autoantibodies (ANA) with a distinct punctate peripheral (rim) fluorescent pattern of the nuclear envelope characteristic of nuclear pore complexes. Reactivity with nuclear pore complexes was confirmed by immunoelectron microscopy. In a cohort of 100 French Canadian patients with autoimmune myositis, the nuclear pore complex fluorescent ANA pattern was restricted to these 4 patients (4%). It was not observed in sera from 393 adult patients with systemic sclerosis (n = 112), mixed connective tissue disease (n = 35), systemic lupus (n = 94), rheumatoid arthritis (n = 45), or other rheumatic diseases (n = 107), nor was it observed in 62 normal adults. Autoantibodies to nuclear pore complexes were predominantly of IgG isotype. No other IgG autoantibody markers for defined connective tissue diseases or overlap syndromes were present, indicating a selective and highly focused immune response. In 3 patients, anti-nuclear pore complex autoantibody titers varied in parallel with myositis activity, suggesting a pathogenic

Idiopathic Inflammatory Myopathies; Association with Overlap Myositis and Syndromes: Classification, Clinical Characteristics, and Associated Autoantibodies

Directory of Open Access Journals (Sweden)

Pari Basharat

2016-07-01

Full Text Available Idiopathic inflammatory myopathies (IIM are traditionally identified as a group of disorders that target skeletal muscle due to autoimmune dysfunction. The IIM can be divided into subtypes based on certain clinical characteristics, and several classification schemes have been proposed. The predominant diagnostic criteria for IIM is the Bohan and Peter criteria, which subdivides IIM into primary polymyositis (PM, primary dermatomyositis (DM, myositis with another connective tissue disease, and myositis associated with cancer. However, this measure has been criticised for several reasons including lack of specific criteria to help distinguish between muscle biopsy findings of PM, DM, and immune-mediated necrotising myopathy, as well as the lack of identification of cases of overlap myositis (OM. Because of this issue, other classification criteria for IIM have been proposed, which include utilising myositis-associated antibodies and myositis-specific antibodies, as well as overlap features such as Raynaud’s phenomenon, polyarthritis, oesophageal abnormalities, interstitial lung disease, small bowel abnormalities such as hypomotility and malabsorption, and renal crises, amongst others. Indeed, the identification of autoantibodies associated with certain clinical phenotypes of myositis, in particular connective tissue disease-myositis overlap, has further helped divide IIM into distinct clinical subsets, which include OM and overlap syndromes (OS. This paper reviews the concepts of OM and OS as they pertain to IIM, including definitions in the literature, clinical characteristics, and overlap autoantibodies.

Increase in hospital admissions for acute childhood asthma in Cape ...

African Journals Online (AJOL)

To determine whether hospital admissions for acute childhood asthma were rising in Cape Town in line with the experience of other countries, Red Cross War Memorial Children's Hospital's records for the period 1978 - 1990 were analysed. These were compared with total admissions for non-surgical causes and lower ...

Epigenetic analysis of childhood acute lymphoblastic leukemia.

Science.gov (United States)

Dunwell, Thomas L; Hesson, Luke B; Pavlova, Tatiana; Zabarovska, Veronika; Kashuba, Vladimir; Catchpoole, Daniel; Chiaramonte, Raffaella; Brini, Anna T; Griffiths, Mike; Maher, Eamonn R; Zabarovsky, Eugene; Latif, Farida

2009-04-01

We used a chromosome 3 wide NotI microarray for identification of epigenetically inactivated genes in childhood acute lymphoblastic leukemia (ALL). Three novel genes demonstrated frequent methylation in childhood ALL. PPP2R3A (protein phosphatase 2, regulatory subunit B", alpha) was frequently methylated in T (69%) and B (82%)-ALL. Whilst FBLN2 (fibulin 2) and THRB (thyroid hormone receptor, beta) showed frequent methylation in B-ALL (58%; 56% respectively), but were less frequently methylated in T-ALL (17% for both genes). Recently it was demonstrated that BNC1 (Basonuclin 1) and MSX1 (msh homeobox 1) were frequently methylated across common epithelial cancers. In our series of childhood ALL BNC1 was frequently methylated in both T (77%) and B-ALL (79%), whilst MSX1 showed T-ALL (25%) specific methylation. The methylation of the above five genes was cancer specific and expression of the genes could be restored in methylated leukemia cell lines treated with 5-aza-2'-deoxycytidine. This is the first report demonstrating frequent epigenetic inactivation of PPP2R3A, FBLN2, THRB, BNC1 and MSX1 in leukemia. The identification of frequently methylated genes showing cancer specific methylation will be useful in developing early cancer detection screens and for targeted epigenetic therapies.

Myositis registries and biorepositories: powerful tools to advance clinical, epidemiologic and pathogenic research.

Science.gov (United States)

Rider, Lisa G; Dankó, Katalin; Miller, Frederick W

2014-11-01

Clinical registries and biorepositories have proven extremely useful in many studies of diseases, especially rare diseases. Given their rarity and diversity, the idiopathic inflammatory myopathies, or myositis syndromes, have benefited from individual researchers' collections of cohorts of patients. Major efforts are being made to establish large registries and biorepositories that will allow many additional studies to be performed that were not possible before. Here, we describe the registries developed by investigators and patient support groups that are currently available for collaborative research purposes. We have identified 46 myositis research registries, including many with biorepositories, which have been developed for a wide variety of purposes and have resulted in great advances in understanding the range of phenotypes, clinical presentations, risk factors, pathogenic mechanisms, outcome assessment, therapeutic responses, and prognoses. These are now available for collaborative use to undertake additional studies. Two myositis patient registries have been developed for research, and myositis patient support groups maintain demographic registries with large numbers of patients available to be contacted for potential research participation. Investigator-initiated myositis research registries and biorepositories have proven extremely useful in understanding many aspects of these rare and diverse autoimmune diseases. These registries and biorepositories, in addition to those developed by myositis patient support groups, deserve continued support to maintain the momentum in this field as they offer major opportunities to improve understanding of the pathogenesis and treatment of these diseases in cost-effective ways.

Core biopsy as a simple and effective diagnostic tool in head and neck focal myositis.

Science.gov (United States)

Tan, Chun Yee; Chong, Sheldon; Shaw, Chi-Kee Leslie

2015-12-01

Most unilateral head and neck masses are benign, although malignancy is a possibility in some cases. However, there are other rare causes of unilateral neck masses, such as focal myositis, which is a rare, benign condition belonging to the family of inflammatory pseudotumors of the skeletal muscles, with rare presentations in the head and neck region. Focal myositis presents as a rapidly enlarging neck mass that can be misdiagnosed by fine-needle aspiration biopsy and/or radiologic imaging as either an infective or a neoplastic process. To date, there are only 5 reported cases of adult focal myositis of the sternocleidomastoid muscle in the medical literature. In this article, the authors present 2 cases involving patients with focal myositis of the sternocleidomastoid muscle that were successfully diagnosed with core-needle biopsy and managed conservatively. The pros and cons of fine-needle aspiration biopsy and core-needle biopsy are discussed. Based on the authors' results, fine-needle aspiration biopsy universally fails to provide the diagnosis of focal myositis. In contrast, core-needle biopsy successfully diagnosed focal myositis in both of our patients. Both of them had complete resolution with conservative management.

Childhood acute lymphoblastic leukemia: from genome to patient

International Nuclear Information System (INIS)

Kolenova, A.

2016-01-01

Acute lymphoblastic leukemia is the most common malignant disease in childhood. During recent decades prognosis for children with acute leukemia has greatly improved, including the patients treated in the Slovak Republic. The prognosis for these patients has improved as a result of the systematic and well-organized international research efforts and clinical trials. The advent of new genomic technologies has provided new insights into leukemogenesis, identified many novel subtypes of leukemia, and triggered development of new therapeutic formulations. The success of treatment depends on stratifying patients into risk group and incorporating novel treatment strategies.The Slovak pediatric leukemia group is actively incorporated into these international clinical trials and the outcome for our patients is comparable to the results published in Western Europe. (author)

Aetiology of childhood acute leukaemias: Current status of knowledge

International Nuclear Information System (INIS)

Rossig, C.; Juergens, H.

2008-01-01

Acute leukaemia is a consequence of malignant transformation of a haematopoietic progenitor cell. Molecular studies have revealed a prenatal origin of many childhood leukaemias. According to current models, a pre-leukaemic stem cell clone is generated by a first mutation in utero which, in a minority of children, progresses to leukaemia after receiving further postnatal genetic hits. The nature of pre- and postnatal events involved in leukemogenesis in children is not well understood. Although genetic predisposition and specific environmental exposures may account for individual cases, the bulk of childhood leukaemia cannot be explained by any of these factors. The higher incidence of the most common leukaemia subtype in affluent societies, as well as the age peak between 2-5 y, suggest a contributory role of socioeconomic factors. An abnormal immune response during delayed exposure to common infections provides a plausible mechanism for malignant progression of pre-leukaemic clones in a subgroup of children. As highlighted in this review, a common cause for all types and subtypes of childhood leukaemia is highly unlikely. Deeper insights into the pathogenesis of childhood leukaemia will rely on large-scale and combined epidemiological and bio-molecular studies. (authors)

Management of Myositis-Related Interstitial Lung Disease.

Science.gov (United States)

Morisset, Julie; Johnson, Cheilonda; Rich, Eric; Collard, Harold R; Lee, Joyce S

2016-11-01

Interstitial lung disease (ILD) is a frequent pulmonary manifestation and an important cause of morbidity and mortality in patients with idiopathic inflammatory myopathy. Myositis-related ILD presents a therapeutic challenge for clinicians, as there are no available guidelines to help with management decisions. This review covers the existing evidence on the pharmacologic and nonpharmacologic management of myositis-related ILD, highlighting the lack of randomized controlled data to guide treatment. Given the absence of existing guidelines to inform treatment decisions, we provide a comprehensive summary, including dosing, side effects, and suggested monitoring of the commonly used immunosuppressive agents and a proposed treatment algorithm based on the existing literature. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

Brief Report: Association of Myositis Autoantibodies, Clinical Features, and Environmental Exposures at Illness Onset With Disease Course in Juvenile Myositis.

Science.gov (United States)

Habers, G Esther A; Huber, Adam M; Mamyrova, Gulnara; Targoff, Ira N; O'Hanlon, Terrance P; Adams, Sharon; Pandey, Janardan P; Boonacker, Chantal; van Brussel, Marco; Miller, Frederick W; van Royen-Kerkhof, Annet; Rider, Lisa G

2016-03-01

To identify early factors associated with disease course in patients with juvenile idiopathic inflammatory myopathies (IIMs). Univariable and multivariable multinomial logistic regression analyses were performed in a large juvenile IIM registry (n = 365) and included demographic characteristics, early clinical features, serum muscle enzyme levels, myositis autoantibodies, environmental exposures, and immunogenetic polymorphisms. Multivariable associations with chronic or polycyclic courses compared to a monocyclic course included myositis-specific autoantibodies (multinomial odds ratio [OR] 4.2 and 2.8, respectively), myositis-associated autoantibodies (multinomial OR 4.8 and 3.5), and a documented infection within 6 months of illness onset (multinomial OR 2.5 and 4.7). A higher overall clinical symptom score at diagnosis was associated with chronic or monocyclic courses compared to a polycyclic course. Furthermore, severe illness onset was associated with a chronic course compared to monocyclic or polycyclic courses (multinomial OR 2.1 and 2.6, respectively), while anti-p155/140 autoantibodies were associated with chronic or polycyclic courses compared to a monocyclic course (multinomial OR 3.9 and 2.3, respectively). Additional univariable associations of a chronic course compared to a monocyclic course included photosensitivity, V-sign or shawl sign rashes, and cuticular overgrowth (OR 2.2-3.2). The mean ultraviolet index and highest ultraviolet index in the month before diagnosis were associated with a chronic course compared to a polycyclic course in boys (OR 1.5 and 1.3), while residing in the Northwest was less frequently associated with a chronic course (OR 0.2). Our findings indicate that myositis autoantibodies, in particular anti-p155/140, and a number of early clinical features and environmental exposures are associated with a chronic course in patients with juvenile IIM. These findings suggest that early factors, which are associated with poorer

Myositis is meer dan een spierziekte

NARCIS (Netherlands)

Lim, J.; van Royen-Kerkhof, A.; Jonkers, R. E.; Starink, M. V.; Voskuyl, A. E.; van der Kooi, A. J.

2018-01-01

Idiopathic inflammatory myopathy (IIM), commonly referred to as "myositis", is a rare but treatable auto-immune disease that is often misdiagnosed or diagnosed after significant delay. Using three clinical case reports as introductory examples, an overview is given - and pitfalls are discussed - of

International consensus on preliminary definitions of improvement in adult and juvenile myositis.

Science.gov (United States)

Rider, Lisa G; Giannini, Edward H; Brunner, Hermine I; Ruperto, Nicola; James-Newton, Laura; Reed, Ann M; Lachenbruch, Peter A; Miller, Frederick W

2004-07-01

To use a core set of outcome measures to develop preliminary definitions of improvement for adult and juvenile myositis as composite end points for therapeutic trials. Twenty-nine experts in the assessment of myositis achieved consensus on 102 adult and 102 juvenile paper patient profiles as clinically improved or not improved. Two hundred twenty-seven candidate definitions of improvement were developed using the experts' consensus ratings as a gold standard and their judgment of clinically meaningful change in the core set of measures. Seventeen additional candidate definitions of improvement were developed from classification and regression tree analysis, a data-mining decision tree tool analysis. Six candidate definitions specifying percentage change or raw change in the core set of measures were developed using logistic regression analysis. Adult and pediatric working groups ranked the 13 top-performing candidate definitions for face validity, clinical sensibility, and ease of use, in which the sensitivity and specificity were >/=75% in adult, pediatric, and combined data sets. Nominal group technique was used to facilitate consensus formation. The definition of improvement (common to the adult and pediatric working groups) that ranked highest was 3 of any 6 of the core set measures improved by >/=20%, with no more than 2 worse by >/=25% (which could not include manual muscle testing to assess strength). Five and 4 additional preliminary definitions of improvement for adult and juvenile myositis, respectively, were also developed, with several definitions common to both groups. Participants also agreed to prospectively test 6 logistic regression definitions of improvement in clinical trials. Consensus preliminary definitions of improvement were developed for adult and juvenile myositis, and these incorporate clinically meaningful change in all myositis core set measures in a composite end point. These definitions require prospective validation, but they are now

Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies.

Science.gov (United States)

Huber, Adam M; Feldman, Brian M; Rennebohm, Robert M; Hicks, Jeanne E; Lindsley, Carol B; Perez, Maria D; Zemel, Lawrence S; Wallace, Carol A; Ballinger, Susan H; Passo, Murray H; Reed, Ann M; Summers, Ronald M; White, Patience H; Katona, Ildy M; Miller, Frederick W; Lachenbruch, Peter A; Rider, Lisa G

2004-05-01

To examine the measurement characteristics of the Childhood Myositis Assessment Scale (CMAS) in children with juvenile idiopathic inflammatory myopathy (juvenile IIM), and to obtain preliminary data on the clinical significance of CMAS scores. One hundred eight children with juvenile IIM were evaluated on 2 occasions, 7-9 months apart, using various measures of physical function, strength, and disease activity. Interrater reliability, construct validity, and responsiveness of the CMAS were examined. The minimum clinically important difference (MID) and CMAS scores corresponding to various degrees of physical disability were estimated. The intraclass correlation coefficient for 26 patients assessed by 2 examiners was 0.89, indicating very good interrater reliability. The CMAS score correlated highly with the Childhood Health Assessment Questionnaire (C-HAQ) score and with findings on manual muscle testing (MMT) (r(s) = -0.73 and 0.73, respectively) and moderately with physician-assessed global disease activity and skin activity, parent-assessed global disease severity, and muscle magnetic resonance imaging (r(s) = -0.44 to -0.61), thereby demonstrating good construct validity. The standardized response mean was 0.81 (95% confidence interval 0.53, 1.09) in patients with at least 0.8 cm improvement on a 10-cm visual analog scale for physician-assessed global disease activity, indicating strong responsiveness. In bivariate regression models predicting physician-assessed global disease activity, MMT remained significant in models containing the CMAS (P = 0.03) while the C-HAQ did not (P = 0.4). Estimates of the MID ranged from 1.5 to 3.0 points on a 0-52-point scale. CMAS scores corresponding to no, mild, mild-to-moderate, and moderate physical disability, respectively, were 48, 45, 39, and 30. The CMAS exhibits good reliability, construct validity, and responsiveness, and is therefore a valid instrument for the assessment of physical function, muscle strength, and

MR findings of infectious myositis caused by vibrio vulnificus: case report

Energy Technology Data Exchange (ETDEWEB)

Lee, Joon Ho; Na, Jae Boem [Gyeongsang National University College of Medicine, Jinju (Korea, Republic of)

2003-03-01

Vibrio vulnificus infection is a fatal disease occurring after the consumption of seafood in patients with underlying liver disease. Inflammation of the skin, subcutanous fat and fascia disseminates from the lower extremity to the trunk and upper extremity. Infection myositis caused by vibrio vulnificus is rare, and its MR findings have not been reported. We report these in a case of infectious myositis caused by vibrio vulnificus involving both lower extremities.

MR findings of infectious myositis caused by vibrio vulnificus: case report

International Nuclear Information System (INIS)

Lee, Joon Ho; Na, Jae Boem

2003-01-01

Vibrio vulnificus infection is a fatal disease occurring after the consumption of seafood in patients with underlying liver disease. Inflammation of the skin, subcutanous fat and fascia disseminates from the lower extremity to the trunk and upper extremity. Infection myositis caused by vibrio vulnificus is rare, and its MR findings have not been reported. We report these in a case of infectious myositis caused by vibrio vulnificus involving both lower extremities

Atypical proliferative myositis: original MR description with pathologic correlation: Case report

Energy Technology Data Exchange (ETDEWEB)

Jarraya, Mohamed; Guermazi, Ali [Boston University School of Medicine, Department of Radiology, Musculoskeletal Section, Boston, MA (United States); Parva, Pedram [VA Boston Healthcare System, Boston, MA (United States); Stone, Michael [Stamford Hospital, Department of Surgery, Stamford, CT (United States); Klein, Michael J. [Hospital for Special Surgery, Department of Pathology and Laboratory Medicine, New York, NY (United States)

2014-08-15

Proliferative myositis (PM) along with proliferative fasciitis and nodular fasciitis are a group of pseudosarcomatous myofibroblastic proliferations. Although the histologic presentation of each is almost identical, the magnetic resonance imaging (MRI) appearance of proliferative myositis is closer to that of inflammatory myopathies. We report a case of PM in which the imaging and histologic features combine typical findings of PM with unusual imaging features, suggesting of reactive (or nodular) fasciitis. (orig.)

Atypical proliferative myositis: original MR description with pathologic correlation: Case report

International Nuclear Information System (INIS)

Jarraya, Mohamed; Guermazi, Ali; Parva, Pedram; Stone, Michael; Klein, Michael J.

2014-01-01

Proliferative myositis (PM) along with proliferative fasciitis and nodular fasciitis are a group of pseudosarcomatous myofibroblastic proliferations. Although the histologic presentation of each is almost identical, the magnetic resonance imaging (MRI) appearance of proliferative myositis is closer to that of inflammatory myopathies. We report a case of PM in which the imaging and histologic features combine typical findings of PM with unusual imaging features, suggesting of reactive (or nodular) fasciitis. (orig.)

Testing for myositis specific autoantibodies: Comparison between line blot and immunoprecipitation assays in 57 myositis sera.

Science.gov (United States)

Cavazzana, Ilaria; Fredi, Micaela; Ceribelli, Angela; Mordenti, Cristina; Ferrari, Fabio; Carabellese, Nice; Tincani, Angela; Satoh, Minoru; Franceschini, Franco

2016-06-01

To analyze the performance of a line blot assay for the identification of autoantibodies in sera of patients affected by myositis, compared with immunoprecipitation (IP) as gold standard. 66 sera of patients with myositis (23 polymyositis, 8 anti-synthetase syndromes, 29 dermatomyositis and 6 overlap syndromes) were tested by commercial LB (Euroimmun, Lubeck, Germany); 57 sera were analyzed also by IP of K562 cell extract radiolabeled with (35)S-methionine. Inter-rater agreement was calculated with Cohen's k coefficient. Myositis-specific antibodies (MSA) were detected in 36/57 sera (63%) by IP and in 39/66 sera (59%) by LB. The most frequent MSA found by LB were anti-Jo1 and anti-Mi2 found in 15% (10/66) of sera, followed by anti-NXP2 and anti-SRP detected in 106% (7/66) of sera. Anti-TIF1gamma and anti-MDA5 were found in 6 (9%) and 5 sera (7.6%), respectively. A good agreement between methods was found only for anti-TIF1γ, anti-MDA5 and anti-NXP-2 antibodies, while a moderate agreement was estimated for anti-Mi2 and anti-EJ. By contrast, a high discordance rate for the detection of anti-Jo1 antibodies was evident (k: 0.3). Multiple positivity for MSA were found in 11/66 (17%) by LB and 0/57 by IP (p: 0001). Comparing the clinical features of these 11 sera, we found total discrepancies between assays in 3 sera (27.3%), a relative discrepancy due to the occurrence of one discordant autoantibody (not confirmed by IP) in 5 cases (45.5%) and a total discrepancy between LB and IP results, but with a relative concordance with clinical features were found in other 3 sera (27.3%). The semiquantitative results do not support the interpretation of the data. The use of LB assay allowed the detection of new MSA, such as anti-MDA5, anti-MJ and anti-TIF1gamma antibodies, previously not found with routine methods. However, the high prevalence of multiple positivities and the high discondant rate of anti-Jo1 antibodies could create some misinterpretation of the results from the

Sodium and chloride channelopathies with myositis: coincidence or connection?

Science.gov (United States)

Matthews, E.; Miller, J.A.L.; Macleod, M.R.; Ironside, J.; Ambler, G.; Labrum, R.; Sud, R.; Holton, J.L.; Hanna, M.G.

2011-01-01

Introduction A proximal myopathy develops in some patients with muscle channelopathies, but the causative molecular mechanisms are unknown. Methods We reviewed retrospectively all clinical and muscle biopsy findings of three patients with channelopathy and additional myositis. Direct DNA sequencing was performed. Results Pathogenic mutations were identified in each case. Biopsies illustrated inflammatory infiltrates. Conclusions Clinicians should consider muscle biopsy in channelopathy patients with severe myalgia and/or subacute weakness and accompanying elevated CK. Chance association of myositis and channelopathy is statistically unlikely. An alternative hypothesis suggests that inflammatory insults could contribute to myopathy in some patients. PMID:21698652

[Acute scrotal pain in childhood: legal pitfalls].

Science.gov (United States)

Bader, Pia; Hugemann, Christoph; Frohneberg, Detlef

2017-12-01

Acute scrotal pain in childhood is an emergency.Sudden scrotal pain may be caused by a variety of diseases. Therefore, it is important to carefully consider the specific medical history and possible differential diagnoses in each case for fast and decisive action (e. g. in case of testicular torsion). As minors lack the capacity for consent, it is absolutely necessary to obtain consent from their legal guardian. However, obtaining consent in the available time frame can cause organisational challenges in an acute emergency, which may lead to situations in the daily routine where a therapeutic decision needs to be taken (including surgery) without legal security based on consent by the guardian. In some cases, the child's consent also needs to be taken into account, depending on its age and development.For the physician and surgeon in charge, the legal evaluation of the case at hand and therewith the obtainment of legal security are of great significance. © Georg Thieme Verlag KG Stuttgart · New York.

Nanoparticle targeted therapy against childhood acute lymphoblastic leukemia

Science.gov (United States)

Satake, Noriko; Lee, Joyce; Xiao, Kai; Luo, Juntao; Sarangi, Susmita; Chang, Astra; McLaughlin, Bridget; Zhou, Ping; Kenney, Elaina; Kraynov, Liliya; Arnott, Sarah; McGee, Jeannine; Nolta, Jan; Lam, Kit

2011-06-01

The goal of our project is to develop a unique ligand-conjugated nanoparticle (NP) therapy against childhood acute lymphoblastic leukemia (ALL). LLP2A, discovered by Dr. Kit Lam, is a high-affinity and high-specificity peptidomimetic ligand against an activated α4β1 integrin. Our study using 11 fresh primary ALL samples (10 precursor B ALL and 1 T ALL) showed that childhood ALL cells expressed activated α4β1 integrin and bound to LLP2A. Normal hematopoietic cells such as activated lymphocytes and monocytes expressed activated α4β1 integrin; however, normal hematopoietic stem cells showed low expression of α4β1 integrin. Therefore, we believe that LLP2A can be used as a targeted therapy for childhood ALL. The Lam lab has developed novel telodendrimer-based nanoparticles (NPs) which can carry drugs efficiently. We have also developed a human leukemia mouse model using immunodeficient NOD/SCID/IL2Rγ null mice engrafted with primary childhood ALL cells from our patients. LLP2A-conjugated NPs will be evaluated both in vitro and in vivo using primary leukemia cells and this mouse model. NPs will be loaded first with DiD near infra-red dye, and then with the chemotherapeutic agents daunorubicin or vincristine. Both drugs are mainstays of current chemotherapy for childhood ALL. Targeting properties of LLP2A-conjugated NPs will be evaluated by fluorescent microscopy, flow cytometry, MTS assay, and mouse survival after treatment. We expect that LLP2A-conjugated NPs will be preferentially delivered and endocytosed to leukemia cells as an effective targeted therapy.

Extraskeletal osteosarcoma arising in myositis ossificans

Energy Technology Data Exchange (ETDEWEB)

Konishi, Eiichi [Div. of Anatomic Pathology, Kyoto Prefectural University of Medicine (Japan); Kusuzaki, Katsuyuki; Murata, Hiroaki [Dept. of Orthopedic Surgery, Kyoto Prefectural University of Medicine (Japan); Tsuchihashi, Yasunari [Hospital Department of Pathology, Kyoto Prefectural University of Medicine (Japan); Beabout, J.W. [Dept. of Diagnostic Radiology, Mayo Clinic and Mayo Foundation, Rochester, MN (United States); Unni, K.K. [Division of Anatomic Pathology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905 (United States); Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (United States)

2001-01-01

A 53-year-old woman had extraskeletal osteosarcoma that developed from a soft tissue bony mass present on the volar aspect of the left wrist for 4 years. Initially, the bony mass was soft and movable, but during the first year it became hard and fixed. The patient had no history of trauma. Because the lesion did not grow or cause any symptoms, the patient did not come to the hospital until 4 years after she first noticed the lesion. Radiologically, the bony mass had features characteristic of mature myositis ossificans, showing ''eggshell'' ossification. A nonmineralized soft tissue mass occurred between the surface of the radius and the bony shell. Histologically, a high-grade osteosarcoma was present between the surface of the radius and the well-differentiated bone tissue, which included fatty and hematopoietic marrow. All the findings indicated that our patient had an extremely rare case of malignant transformation of myositis ossificans. (orig.)

Extraskeletal osteosarcoma arising in myositis ossificans

International Nuclear Information System (INIS)

Konishi, Eiichi; Kusuzaki, Katsuyuki; Murata, Hiroaki; Tsuchihashi, Yasunari; Beabout, J.W.; Unni, K.K.

2001-01-01

A 53-year-old woman had extraskeletal osteosarcoma that developed from a soft tissue bony mass present on the volar aspect of the left wrist for 4 years. Initially, the bony mass was soft and movable, but during the first year it became hard and fixed. The patient had no history of trauma. Because the lesion did not grow or cause any symptoms, the patient did not come to the hospital until 4 years after she first noticed the lesion. Radiologically, the bony mass had features characteristic of mature myositis ossificans, showing ''eggshell'' ossification. A nonmineralized soft tissue mass occurred between the surface of the radius and the bony shell. Histologically, a high-grade osteosarcoma was present between the surface of the radius and the well-differentiated bone tissue, which included fatty and hematopoietic marrow. All the findings indicated that our patient had an extremely rare case of malignant transformation of myositis ossificans. (orig.)

Myositis Ossificans of the Psoas Muscle After Compression Fracture of Lumbar Spine: CT and MR Imaging Findings

International Nuclear Information System (INIS)

Choi, Mi Nyong; Lee, Gyung Kyu; Suh, Kyung Jin

2010-01-01

Myositis ossificans is a benign, self-limiting and non-neoplastic development of heterotopic bone in skeletal muscle following trauma. Although myositis ossificans can occur anywhere in the body, psoas muscle involvement is very rare. To the best of our knowledge, CT and MR imaging findings of myositis ossificans in the psoas muscle secondary to lumbar spine fracture have not been reported in the radiological literature. In this article, we describe the CT and MR imaging findings of myositis ossificans of the psoas muscle after lumbar spine fracture in a 64-year-old man, and conduct a review of the relevant literature

Myositis Ossificans of the Psoas Muscle After Compression Fracture of Lumbar Spine: CT and MR Imaging Findings

Energy Technology Data Exchange (ETDEWEB)

Choi, Mi Nyong; Lee, Gyung Kyu [Hallym University College of Medicine, Hangang Sacred Heart Hospital, Seoul (Korea, Republic of); Suh, Kyung Jin [Dongguk University College of Medicine, Gyungju Hospital, Gyeongju (Korea, Republic of)

2010-02-15

Myositis ossificans is a benign, self-limiting and non-neoplastic development of heterotopic bone in skeletal muscle following trauma. Although myositis ossificans can occur anywhere in the body, psoas muscle involvement is very rare. To the best of our knowledge, CT and MR imaging findings of myositis ossificans in the psoas muscle secondary to lumbar spine fracture have not been reported in the radiological literature. In this article, we describe the CT and MR imaging findings of myositis ossificans of the psoas muscle after lumbar spine fracture in a 64-year-old man, and conduct a review of the relevant literature

Fungal myositis in children: serial ultrasonographic findings

Energy Technology Data Exchange (ETDEWEB)

Kwon, Jung Hwa; Lee, Hee Jung; Choi, Jin Soo [Keimyung University School of Medicine, Daegu (Korea, Republic of)

2003-08-01

To evaluate serial ultrasonographic findings of fungal myositis in children. Eleven lesions caused by fungal myositis and occurring in six children were included in this study. Eight lesions in five children were histopathologically proven and the other three were clinically diagnosed. Serial ultrasonographic findings were retrospectively evaluated in terms of size, location, margin, internal echotexture and adjacent cortical change occurring during the follow-up period ranging from five days to two months. Three patients (50%) had multiple lesions. The sites of involvment were the thigh (n=4), calf (n=3), chest wall (n=2), abdominal wall (n=1) and forearm (n=1). Initially, diffuse muscular swelling was revealed, with ill-defined hypoechoic lesions confined to the muscle layer (n=8). Follow-up examination of eight lesions over a period of 5-10 days showed that round central echogenic lesions were surrounded by previous slightly echogenic lesions (n=6, 75%). Long-term follow-up of five lesions over a two-month period revealed periosteal thickening in one case (20%), and the peristence of echogenic solid nodules in four (80%). Pathologic examination showed that the central lesions correlated with a fungus ball and the peripheral slightly echogenic lesions corresponded to hematoma and necrosis. Serial ultrasonographic findings of fungal myositis in children revealed relatively constant features in each case. In particular, the findings of muscular necrosis and a fungus ball over a period of 5-14 days were thought to be characteristic.

Fungal myositis in children: serial ultrasonographic findings

International Nuclear Information System (INIS)

Kwon, Jung Hwa; Lee, Hee Jung; Choi, Jin Soo

2003-01-01

To evaluate serial ultrasonographic findings of fungal myositis in children. Eleven lesions caused by fungal myositis and occurring in six children were included in this study. Eight lesions in five children were histopathologically proven and the other three were clinically diagnosed. Serial ultrasonographic findings were retrospectively evaluated in terms of size, location, margin, internal echotexture and adjacent cortical change occurring during the follow-up period ranging from five days to two months. Three patients (50%) had multiple lesions. The sites of involvment were the thigh (n=4), calf (n=3), chest wall (n=2), abdominal wall (n=1) and forearm (n=1). Initially, diffuse muscular swelling was revealed, with ill-defined hypoechoic lesions confined to the muscle layer (n=8). Follow-up examination of eight lesions over a period of 5-10 days showed that round central echogenic lesions were surrounded by previous slightly echogenic lesions (n=6, 75%). Long-term follow-up of five lesions over a two-month period revealed periosteal thickening in one case (20%), and the peristence of echogenic solid nodules in four (80%). Pathologic examination showed that the central lesions correlated with a fungus ball and the peripheral slightly echogenic lesions corresponded to hematoma and necrosis. Serial ultrasonographic findings of fungal myositis in children revealed relatively constant features in each case. In particular, the findings of muscular necrosis and a fungus ball over a period of 5-14 days were thought to be characteristic

Recovery from severe dysphagia in systemic sclerosis - myositis overlap: a case report.

Science.gov (United States)

Chinniah, Keith J; Mody, Girish M

2017-06-01

Dysphagia is common in inflammatory myopathies and usually responds to corticosteroids. Severe dysphagia requiring feeding by percutaneous endoscopic gastrostomy is associated with significant morbidity and high mortality. A 56-year old African Black woman initially presented with systemic sclerosis (SSC) - myositis overlap and interstitial lung disease. She responded to high dose corticosteroids and cyclophosphamide followed by azathioprine, with improvement in her lung function and regression of the skin changes. Six years later she had a myositis flare with severe dysphagia. Her myositis improved after high doses of corticosteroids, azathioprine and two doses of intravenous immunoglobulin (IVIG). As her dysphagia persisted, she was fed via a percutaneous endoscopic gastrostomy (PEG) tube and given a course of rituximab. Her dysphagia gradually resolved and the PEG tube was removed within two months. She received another dose of rituximab six months later and continued low dose prednisone and azathioprine. Her muscle power improved, weight returned to normal and she remained well 20 months after hospital discharge. Our patient with SSC-myositis overlap and severe dysphagia requiring PEG feeding, improved with high dose corticosteroids, azathioprine, two courses of IVIG and rituximab, and remained in remission 20 months after hospital discharge.

The Clinical Features of Myositis-Associated Autoantibodies: a Review.

Science.gov (United States)

Gunawardena, Harsha

2017-02-01

The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

Cost-analysis of treatment of childhood acute lymphoblastic leukemia with asparaginase preparations: The impact of expensive chemotherapy

NARCIS (Netherlands)

W.H. Tong (Wing); I.M. van der Sluis (Inge); C.J.M. Alleman (Cathelijne); R.R. van Litsenburg (Raphaële ); G.J. Kaspers (Gertjan); R. Pieters (Rob); C.A. Uyl-de Groot (Carin)

2013-01-01

markdownabstract__Abstract__ Asparaginase is an expensive drug, but important in childhood acute lymphoblastic leukemia. In order to compare costs of PEGasparaginase, Erwinia asparaginase and native E. coli asparaginase, we performed a cost-analysis in the Dutch Childhood Oncology Group ALL-10

A case of late-onset, thymoma-associated myasthenia gravis with ryanodine receptor and titin antibodies and concomitant granulomatous myositis.

Science.gov (United States)

Stefanou, M I; Komorowski, L; Kade, S; Bornemann, A; Ziemann, U; Synofzik, M

2016-09-13

Myasthenia gravis is an autoimmune neuromuscular disorder, which has only rarely been reported to co-manifest with myositis. The diagnosis of concomitant myositis in patients with myasthenia gravis is clinically challenging, and requires targeted investigations for the differential diagnosis, including EMG, autoantibody assays, muscle biopsy and, importantly, imaging of the mediastinum for thymoma screening. This report presents a case-vignette of a 72-year-old woman with progressive proximal muscle weakness and myalgias, diagnosed with thymoma-associated myasthenia and bioptically verified granulomatous myositis, with positive autoantibody status for ryanodine receptor and titin antibodies. The diagnosis of concurrent myositis and myasthenia gravis, especially in the presence of ryanodine receptor and titin antibodies, should lead neurologists to adopt different treatment strategies compared to those applied in myasthenia or myositis alone. Moreover, further evidence is warranted that titin and, particularly, ryanodine receptor antibodies may co-occur or be pathophysiologically involved in myasthenia-myositis cases.

Non-infectious chemotherapy-associated acute toxicities during childhood acute lymphoblastic leukemia therapy [version 1; referees: 3 approved

Directory of Open Access Journals (Sweden)

Kjeld Schmiegelow

2017-04-01

Full Text Available During chemotherapy for childhood acute lymphoblastic leukemia, all organs can be affected by severe acute side effects, the most common being opportunistic infections, mucositis, central or peripheral neuropathy (or both, bone toxicities (including osteonecrosis, thromboembolism, sinusoidal obstruction syndrome, endocrinopathies (especially steroid-induced adrenal insufficiency and hyperglycemia, high-dose methotrexate-induced nephrotoxicity, asparaginase-associated hypersensitivity, pancreatitis, and hyperlipidemia. Few of the non-infectious acute toxicities are associated with clinically useful risk factors, and across study groups there has been wide diversity in toxicity definitions, capture strategies, and reporting, thus hampering meaningful comparisons of toxicity incidences for different leukemia protocols. Since treatment of acute lymphoblastic leukemia now yields 5-year overall survival rates above 90%, there is a need for strategies for assessing the burden of toxicities in the overall evaluation of anti-leukemic therapy programs.

Gallium uptake in myositis ossificans. Potential pitfalls in diagnosis

International Nuclear Information System (INIS)

Salzman, L.; Lee, V.W.; Grant, P.

1987-01-01

Seven cases of gallium uptake in myositis ossificans are described. Gallium scans are done frequently in paraplegics, quadriplegics, and comatose patients to look for occult infection. It is important to be aware of possible gallium uptake in myositis ossificans, particularly in the extremities, which is frequent in these patients. Gallium uptake may be present prior to any abnormalities seen on plain films or CT scans. It is important to correlate roentgenograms with abnormal gallium scans, particularly in the extremities, to avoid potential pitfalls in diagnosis and prevent unnecessary antibiotic treatment. A bone scan should be obtained whenever possible, particularly when roentgenograms are negative, to confirm the diagnosis

Association of inclusion body myositis with T cell large granular lymphocytic leukaemia

DEFF Research Database (Denmark)

Greenberg, Steven A; Pinkus, Jack L; Amato, Anthony A

2016-01-01

SEE HOHLFELD AND SCHULZE-KOOPS DOI101093/BRAIN/AWW053 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Inclusion body myositis and T cell large granular lymphocytic leukaemia are rare diseases involving pathogenic cytotoxic CD8+ T cells. After encountering four patients with both disorders, we...... prospectively screened 38 patients with inclusion body myositis for the presence of expanded large granular lymphocyte populations by standard clinical laboratory methods (flow cytometry, examination of blood smears, and T cell receptor gene rearrangements), and performed muscle immunohistochemistry for CD8, CD......57, and TIA1. Most (22/38; 58%) patients with inclusion body myositis had aberrant populations of large granular lymphocytes in their blood meeting standard diagnostic criteria for T cell large granular lymphocytic leukaemia. These T cell populations were clonal in 20/20 patients and stably present...

Inclusion body myositis.

Science.gov (United States)

Dimachkie, Mazen M; Barohn, Richard J

2014-08-01

The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of rare disorders that share many similarities. In addition to sporadic inclusion body myositis (IBM), these include dermatomyositis, polymyositis, and autoimmune necrotizing myopathy. IBM is the most common IIM after age 50 years. Muscle histopathology shows endomysial inflammatory exudates surrounding and invading nonnecrotic muscle fibers often accompanied by rimmed vacuoles and protein deposits. It is likely that IBM is has a prominent degenerative component. This article reviews the evolution of knowledge in IBM, with emphasis on recent developments in the field, and discusses ongoing clinical trials. Copyright © 2014 Elsevier Inc. All rights reserved.

Mutational analysis of Bax and Bcl-2 in childhood acute lymphoblastic leukaemia

NARCIS (Netherlands)

Salomons, G. S.; Buitenhuis, C. K.; Martínez Muñoz, C.; Verwijs-Jassen, M.; Behrendt, H.; Zsiros, J.; Smets, L. A.

1998-01-01

In childhood acute lymphoblastic leukaemia there are large interpatient variations in levels of the apoptosis-regulating proteins Bax and Bcl-2, but the molecular basis for this variation is unknown. Point-mutations in bax have been reported in cell lines derived from haematological malignancies.

Autoantibody levels in myositis patients correlate with clinical response during B cell depletion with rituximab.

Science.gov (United States)

Aggarwal, Rohit; Oddis, Chester V; Goudeau, Danielle; Koontz, Diane; Qi, Zengbiao; Reed, Ann M; Ascherman, Dana P; Levesque, Marc C

2016-06-01

To determine the longitudinal trends in serum levels of four myositis-associated autoantibodies: anti-Jo-1, -transcription intermediary factor 1 γ (TIF1-γ), -signal recognition particle (SRP) and -Mi-2, after B cell depletion with rituximab, and to determine the longitudinal association of these autoantibody levels with disease activity as measured by myositis core-set measures (CSMs). Treatment-resistant adult and pediatric myositis subjects (n = 200) received rituximab in the 44-week Rituximab in Myositis Trial. CSMs [muscle enzymes, manual muscle testing (MMT), physician and patient global disease activity, HAQ, and extramuscular disease activity] were evaluated monthly and anti-Jo-1 (n = 28), -TIF1-γ (n = 23), -SRP (n = 25) and -Mi-2 (n = 26) serum levels were measured using validated quantitative ELISAs. Temporal trends and the longitudinal relationship between myositis-associated autoantibodies levels and CSM were estimated using linear mixed models. Following rituximab, anti-Jo-1 levels decreased over time (P myositis subjects decreased after B cell depletion and were correlated with changes in disease activity, whereas anti-SRP levels were only associated with longitudinal muscle enzyme levels. The strong association of anti-Jo-1 levels with clinical outcomes suggests that anti-Jo-1 autoantibodies may be a good biomarker for disease activity. © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain.

Science.gov (United States)

Nuño-Nuño, Laura; Joven, Beatriz Esther; Carreira, Patricia E; Maldonado-Romero, Valentina; Larena-Grijalba, Carmen; Cubas, Irene Llorente; Tomero, Eva Gloria; Barbadillo-Mateos, María Carmen; De la Peña Lefebvre, Paloma García; Ruiz-Gutiérrez, Lucía; López-Robledillo, Juan Carlos; Moruno-Cruz, Henry; Pérez, Ana; Cobo-Ibáñez, Tatiana; Almodóvar González, Raquel; Lojo, Leticia; García De Yébenes, María Jesús; López-Longo, Francisco Javier

2017-11-01

The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9.7 years. In the overall cohort, the 2-, 5-, and 10-year survival probabilities were 91.9, 86.7, and 77%, respectively. Main causes of death were infections and cancer (24% each). Multivariate model revealed that CAM (HR = 24.06), OM (HR = 12.00), DM (HR = 7.26), higher age at diagnosis (HR = 1.02), severe infections (HR = 3.66), interstitial lung disease (HR = 1.61), and baseline elevation of acute phase reactants (HR = 3.03) were associated with a worse prognosis, while edema of the hands (HR = 0.39), female gender (HR = 0.39), and longer disease duration (HR = 0.73) were associated with a better prognosis. The standardized mortality ratio was 1.56 (95% CI 1.28-1.87) compared to the Spanish general population. Our findings indicate that IIM has a high long-term mortality, with an excess of mortality compared to the Spanish population. A more aggressive therapy may be required in IIM patients presenting with poor predictive factors.

Sarcoidosis presenting as granulomatous myositis in a 16-year-old adolescent.

Science.gov (United States)

Orandi, Amir B; Eutsler, Eric; Ferguson, Cole; White, Andrew J; Kitcharoensakkul, Maleewan

2016-11-10

Sarcoidosis is a multi-system disease characterized by the presence of non-caseating epithelioid granulomas in affected tissues, including skeletal muscle. These organized collections of immune cells have important pathophysiologic action including cytokine production leading to inflammation as well as enzymatic conversion of cholecalciferol to calcitriol via 1-α hydroxylase. There are limited reports of isolated granulomatous myositis causing hypercalcemia in pediatric patients. Our patient uniquely presented with symptoms from hypercalcemia and renal insufficiency caused by an overwhelming burden of granulomatous myositis in her lower extremities, but was otherwise asymptomatic. A 16 year old Caucasian female presented with protracted symptoms of fatigue, nausea and prominent weight loss with laboratory evidence of hypercalcemia and renal insufficiency. She lacked clinical and physical findings of arthritis, weakness, rash, uveitis, fever, lymphadenopathy or respiratory symptoms. After extensive negative investigations, re-examination yielded subtle soft tissue changes in her lower extremities, with striking MRI findings of extensive myositis without correlative weakness or serum enzyme elevation. Biopsy showed the presence of non-caseating epithelioid granulomas and calcium oxalate crystals. The patient responded well to prednisone and methotrexate but relapsed with weaning of steroids. She reachieved remission with addition of adalimumab. Sarcoidosis should be considered in patients presenting with symptomatic hypercalcemia with no apparent causes and negative routine workup. The absences of decreased muscle strength or elevated muscle enzymes do not preclude the diagnosis of granulomatous myositis.

Cure rates of childhood acute lymphoblastic leukemia in Lithuania and the benefit of joining international treatment protocol

DEFF Research Database (Denmark)

Vaitkevičienė, Goda; Matuzevičienė, Rėda; Stoškus, Mindaugas

2014-01-01

BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) represents the largest group of pediatric malignancies with long-term survival rates of more than 80% achieved in developed countries. Epidemiological data and survival rates of childhood ALL in Lithuania were lacking. Therefore, the aim of...

Cardiac function in survivors of childhood acute myeloid leukemia treated with chemotherapy only

DEFF Research Database (Denmark)

Jarfelt, Marianne; Andersen, Niels Holmark; Glosli, Heidi

2015-01-01

OBJECTIVES: We report cardiac function of patients treated for Childhood acute myeloid leukemia with chemotherapy only according to three consecutive Nordic protocols. METHODS: Ninety-eight of 138 eligible patients accepted examination with standardized echocardiography. Results were compared...

Eosinophilic myositis as first manifestation in a patient with type 2 myotonic dystrophy CCTG expansion mutation and rheumatoid arthritis.

Science.gov (United States)

Meyer, Alain; Lannes, Béatrice; Carapito, Raphaël; Bahram, Seiamak; Echaniz-Laguna, Andoni; Geny, Bernard; Sibilia, Jean; Gottenberg, Jacques Eric

2015-02-01

Eosinophilic myositis is characterized by eosinophilic infiltration of skeletal muscles. In the absence of an identifiable causative factor or source (including parasitic infection, intake of drugs or L-tryptophan, certain systemic disorders as well as malignant diseases), the diagnosis of idiopathic eosinophilic myositis is usually retained. However, some muscular dystrophies have been recently identified in this subset of eosinophilic myositis. Here, we report a patient with an 8 kb CCTG expansion in intron 1 of the CNBP gene, a mutation characteristic of myotonic dystrophy type 2 (DM2), whose first manifestation was "idiopathic" eosinophilic myositis. This report suggests that in "idiopathic" eosinophilic myositis, clinicians should consider muscular dystrophies, including DM2. Copyright © 2014 Elsevier B.V. All rights reserved.

Myositis complicating benzathine penicillin-G injection in a case of rheumatic heart disease

Directory of Open Access Journals (Sweden)

Joshua R. Francis

2016-01-01

Full Text Available A 7-year old boy developed myositis secondary to intramuscular injection of benzathine penicillin-G in the context of secondary prophylaxis for rheumatic heart disease. Side effects of intramuscular delivery of benzathine penicillin-G are well described and include injection site pain and inflammation, but myositis, as depicted on magnetic resonance imaging in this case, has not previously been described.

Myositis ossificans circumscripta of the psoas muscle due to overuse in an adolescent gymnast.

Science.gov (United States)

Masquijo, Julio Javier; Sartori, Federico

2014-11-01

Myositis ossificans is a pseudoinflammatory tumour that originates from skeletal muscle and corresponds to a heterotopic, metaplastic, nonmalignant bone tumour. The purpose of this article is to report the case of myositis ossificans circumscripta (MOC) of the psoas muscle due to overuse in an adolescent gymnast. A 16-year-old female athlete presented at our outpatient orthopaedic clinic for evaluation of a 1-month history of low back pain. Initial plain radiographs were initially interpreted as negative, and laboratory values were normal. MRI imaging demonstrated a circumscribed mass with associated oedema in the psoas muscle. Computed tomography-guided percutaneous biopsy was performed and histology confirmed the diagnosis of MOC. Conservative treatment was initiated with rest and anti-inflammatory drugs (indomethacin). The patient had a resolution of pain and function after 3 months of conservative treatment. At 6 months' follow-up, MRI demonstrated complete resolution of the lesion and she gradually returned to her sports activity. At last follow-up she was asymptomatic. MOC is a rare lesion in the paediatric-adolescent population. To our knowledge, this is the first report of MOC in the psoas muscle produced by overuse. MRI is very sensitive in detecting oedema during the acute phase of the lesion. Conservative treatment should be considered, especially at the early stage of the disease. Spontaneous resolution can be expected in most cases.

Myositis ossificans traumatica causing ankylosis of the elbow.

Science.gov (United States)

Kanthimathi, B; Udhaya Shankar, S; Arun Kumar, K; Narayanan, V L

2014-12-01

Myositis ossificans traumatica is an unusual complication following a muscle contusion injury. A significantly large myositic mass causing ankylosis of the elbow is even rarer. We report a 13-year-old boy who presented with a 14-month history of a fixed elbow with no movement and a palpable bony mass in the anterior aspect of the elbow. He had sustained significant trauma to the affected limb 1 month prior to onset of symptoms, which was managed by native massage and bandaging for 4 weeks. The clinicoradiological diagnosis was suggestive of myositis ossificans, and the myositic mass was completely excised. Histopathology revealed lamellar bone. The 2-year follow-up showed full function of the affected limb and no signs of recurrence. We report this case of clinical interest due to the unusually large myositic mass.

Oval pulsed high-dose dexamethasone for myositis

NARCIS (Netherlands)

Hoogendijk, JE; Wokke, JHJ; de Visser, M

To study the short-term effect of oral pulsed high-dose dexamethasone for myositis we treated eight newly diagnosed patients with three 28-day cycles of oral dexamethasone. Primary outcome measures were muscle strength, pain, and serum creatine kinase activity. Sis patients responded. Side effects

Oral pulsed high-dose dexamethasone for myositis

NARCIS (Netherlands)

van der Meulen, M. F.; Hoogendijk, J. E.; Wokke, J. H.; de Visser, M.

2000-01-01

To study the short-term effect of oral pulsed high-dose dexamethasone for myositis we treated eight newly diagnosed patients with three 28-day cycles of oral dexamethasone. Primary outcome measures were muscle strength, pain, and serum creatine kinase activity. Six patients responded. Side effects

The Eleventh International Childhood Acute Lymphoblastic Leukemia Workshop Report: Ponte di Legno, Italy, 6-7 May 2009

DEFF Research Database (Denmark)

Biondi, A; Baruchel, A; Hunger, S

2009-01-01

An international childhood acute lymphoblastic leukemia (ALL)working group was formed during the 27th annual meeting of the International Society of Pediatric Oncology in 1995. Since then, 10 workshops have been held to address many issues that help advance treatment outcome of childhood ALL but ...

Association of Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme A Reductase Autoantibodies With DRB1*07:01 and Severe Myositis in Juvenile Myositis Patients.

Science.gov (United States)

Kishi, Takayuki; Rider, Lisa G; Pak, Katherine; Barillas-Arias, Lilliana; Henrickson, Michael; McCarthy, Paul L; Shaham, Bracha; Weiss, Pamela F; Horkayne-Szakaly, Iren; Targoff, Ira N; Miller, Frederick W; Mammen, Andrew L

2017-07-01

Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of juvenile anti-HMGCR-positive myositis patients. The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti-HMGCR-positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis-specific autoantibodies (MSAs). Five of 440 patients (1.1%) were anti-HMGCR-positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and 2 patients had immune-mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti-HMGCR-positive subjects was 17,000 IU/liter. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR-positive subjects compared to MSA-negative patients or those with other MSAs. Anti-HMGCR-positive patients had only partial responses to multiple immunosuppressive medications, and their disease often took a chronic course. The DRB1*07:01 allele was present in all 5 patients, compared to 26.25% of healthy controls (corrected P = 0.01); none of the 5 juvenile patients had DRB1*11:01. Compared to children with other MSAs, muscle disease appears to be more severe in those with anti-HMGCR autoantibodies. Like adults, children with anti-HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, in anti-HMGCR-positive children, there is a strong association with HLA-DRB1*07:01. © 2017, American College of Rheumatology.

Nontraumatic myositis ossificans in the breast

International Nuclear Information System (INIS)

Salomonowitz, Erich; Youssefzadeh, Soraya; Reiner, Angelika; Heilbron, E.A.; Zollikofer, C.L.

1991-01-01

The authors report the 1st case of a healthy female who developed a rapidly growing mass in her left breast which proved to be a non-progressive form of myositis ossificans originating in fat tissue. This rare entitity may be important to radiologists because the clinical symptoms are suspicious of a malignancy. (author). 5 refs.; 3 figs

Genome‐wide analysis of cytogenetic aberrations in ETV6/RUNX1‐positive childhood acute lymphoblastic leukaemia

DEFF Research Database (Denmark)

Borst, Louise; Wesolowska, Agata; Joshi, Tejal

2012-01-01

The chromosomal translocation t(12;21) resulting in the ETV6/RUNX1 fusion gene is the most frequent structural cytogenetic abnormality among patients with childhood acute lymphoblastic leukaemia (ALL). We investigated 62 ETV6/RUNX1‐positive childhood ALL patients by single nucleotide polymorphism...... childhood ALL, which may be important for understanding poor responses among this otherwise highly curable subset of ALL and lead to novel targeted treatment strategies....

Virus detection and cytokine profile in relation to age among acute exacerbations of childhood asthma.

Science.gov (United States)

Kato, Masahiko; Suzuki, Kazuo; Yamada, Yoshiyuki; Maruyama, Kenichi; Hayashi, Yasuhide; Mochizuki, Hiroyuki

2015-09-01

Little information is available regarding eosinophil activation and cytokine profiles in relation to age in virus-induced bronchial asthma. We therefore explored the association between age, respiratory viruses, serum eosinophil cationic protein (ECP), and cytokines/chemokines in acute exacerbations of childhood asthma. We investigated viruses in nasal secretions from 88 patients with acute exacerbation of childhood asthma by using antigen detection kits and/or RT-PCR, followed by direct DNA sequencing analysis. We also measured peripheral eosinophil counts, and the serum levels of ECP and 27 types of cytokines/chemokines in 71 virus-induced acute asthma cases and 13 controls. Viruses were detected in 71(80.7%) of the 88 samples. The three major viruses detected were rhinoviruses, RS viruses, and enteroviruses; enteroviruses were found to be dominant in patients aged ≥3 years. There was no change in the levels of rhinoviruses and RS viruses between the two age groups, defined as children aged asthma cases compared with controls. Serum ECP values were significantly higher in patients with virus-induced asthma at age ≥3 years compared with those aged asthma in patients childhood asthma. Copyright © 2015 The Authors. Production and hosting by Elsevier B.V. All rights reserved.

Cytosolic 5'-nucleotidase 1A autoantibody profile and clinical characteristics in inclusion body myositis.

Science.gov (United States)

Lilleker, J B; Rietveld, A; Pye, S R; Mariampillai, K; Benveniste, O; Peeters, M T J; Miller, J A L; Hanna, M G; Machado, P M; Parton, M J; Gheorghe, K R; Badrising, U A; Lundberg, I E; Sacconi, S; Herbert, M K; McHugh, N J; Lecky, B R F; Brierley, C; Hilton-Jones, D; Lamb, J A; Roberts, M E; Cooper, R G; Saris, C G J; Pruijn, G J M; Chinoy, H; van Engelen, B G M

2017-05-01

Autoantibodies directed against cytosolic 5'-nucleotidase 1A have been identified in many patients with inclusion body myositis. This retrospective study investigated the association between anticytosolic 5'-nucleotidase 1A antibody status and clinical, serological and histopathological features to explore the utility of this antibody to identify inclusion body myositis subgroups and to predict prognosis. Data from various European inclusion body myositis registries were pooled. Anticytosolic 5'-nucleotidase 1A status was determined by an established ELISA technique. Cases were stratified according to antibody status and comparisons made. Survival and mobility aid requirement analyses were performed using Kaplan-Meier curves and Cox proportional hazards regression. Data from 311 patients were available for analysis; 102 (33%) had anticytosolic 5'-nucleotidase 1A antibodies. Antibody-positive patients had a higher adjusted mortality risk (HR 1.89, 95% CI 1.11 to 3.21, p=0.019), lower frequency of proximal upper limb weakness at disease onset (8% vs 23%, adjusted OR 0.29, 95% CI 0.12 to 0.68, p=0.005) and an increased prevalence of excess of cytochrome oxidase deficient fibres on muscle biopsy analysis (87% vs 72%, adjusted OR 2.80, 95% CI 1.17 to 6.66, p=0.020), compared with antibody-negative patients. Differences were observed in clinical and histopathological features between anticytosolic 5'-nucleotidase 1A antibody positive and negative patients with inclusion body myositis, and antibody-positive patients had a higher adjusted mortality risk. Stratification of inclusion body myositis by anticytosolic 5'-nucleotidase 1A antibody status may be useful, potentially highlighting a distinct inclusion body myositis subtype with a more severe phenotype. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Differences in disease features between childhood-onset and adult-onset systemic lupus erythematosus patients presenting with acute abdominal pain.

Science.gov (United States)

Tu, Yu-Ling; Yeh, Kuo-Wei; Chen, Li-Chen; Yao, Tsung-Chieh; Ou, Liang-Shiou; Lee, Wen-I; Huang, Jing-Long

2011-04-01

Abdominal pain in systemic lupus erythematosus (SLE) patients has rarely been analyzed in pediatric populations. We planned to investigate the potential differences between childhood-onset and adult-onset SLE patients who were hospitalized because of acute abdominal pain. A retrospective study including 23 childhood-onset SLE patients with 38 admissions and 88 adult-onset SLE patients with 108 admissions from 1999 to 2008 were conducted in our hospital. All of them had the chief complaint of diffuse abdominal pain. The etiologies of acute abdominal pain in adult-onset SLE patients were more diverse than childhood-onset SLE patients. The most common cause of acute abdominal pain in SLE patients was lupus mesenteric vasculitis (LMV) (18.5%), followed by acute gastroenteritis (14.4%), pancreatitis (10.3%), appendicitis (7.5%), and cholecystitis (6.2%). Compared with adults, children were admitted more often due to LMV (31.6% versus 13.9%; P = 0.016), had more frequently recurrent episodes (39.1% versus 14.8%; P = 0.009), and were more often treated with immunosuppressive agents (31.6% versus 7.4%; P abdominal pain should be considered in SLE patients. LMV is the most common cause of acute abdomen in childhood-onset SLE patients with low mortality and morbidity provided by prompt diagnosis and timely administration of high-dose intravenous corticosteroids after excluding real surgical abdomen. Crown Copyright © 2011. Published by Elsevier Inc. All rights reserved.

Tumour TIF1 mutations and loss of heterozygosity related to cancer-associated myositis.

Science.gov (United States)

Pinal-Fernandez, Iago; Ferrer-Fabregas, Berta; Trallero-Araguas, Ernesto; Balada, Eva; Martínez, Maria Angeles; Milisenda, Jose César; Aparicio-Español, Gloria; Labrador-Horrillo, Moises; Garcia-Patos, Vicente; Grau-Junyent, Josep M; Selva-O'Callaghan, Albert

2018-02-01

To analyse the influence of genetic alterations and differential expression of transcription intermediary factor 1 (TIF1) genes in the pathophysiology of cancer-associated myositis (CAM). Paired blood and tumour DNA samples from patients with anti-TIF1γ-positive CAM and from controls were analysed by whole-exome sequencing for the presence of somatic mutations and loss of heterozygosity (LOH) in their TIF1 genes. The genesis and maintenance of the autoimmune process were investigated immunohistochemically by studying TIF1γ expression in the different tissues involved in CAM (skin, muscle and tumour) based on the immunohistochemical H-score. From seven patients with anti-TIF1γ-positive CAM, we detected one somatic mutation and five cases of LOH in one or more of the four TIF1 genes compared with just one case of LOH in tumours from TIF1γ-negative myositis patients (86% vs 17%; P = 0.03). Compared with type-matched control tumours from non-myositis patients, TIF1γ staining was more intense in tumours from anti-TIF1γ-positive patients (H-score 255 vs 196; P = 0.01). Also, TIF1γ staining in muscle was slightly more intense in anti-TIF1γ-positive than in anti-TIF1γ-negative myositis (H-score 22 vs 5; P = 0.03). In contrast, intense TIF1γ staining was detected in the skin of both myositis and control patients. Tumours from paraneoplastic anti-TIF1γ-positive patients showed an increased number of genetic alterations, such as mutations and LOH, in TIF1 genes. These genetic alterations, in the context of a high expression of TIF1γ in the tumour, muscle and skin of these patients may be key to understanding the genesis of paraneoplastic myositis. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com

Myalgia Cruris Epidemica: an unusual presentation of dengue fever.

Science.gov (United States)

Ahmad, Rashidi; Abdul Latiff, Abdul Kursi; Abdul Razak, Salmi

2007-11-01

We describe a 5-year-old girl who had sudden onset difficulty in walking after 3 days of febrile illness. In the emergency department her creatine kinase level was elevated but urine myoglobin was normal. She was diagnosed as having benign acute childhood myositis. Because of poor oral intake and dehydration, she was admitted to the pediatric ward. The next day she had a petechial rash over the antecubital fossa, and dengue IgM back was positive. She was treated conservatively and recovered uneventfully. Despite dengue fever being endemic in Malaysia, this is the first case report of myositis following dengue infection in Malaysia.

Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

Science.gov (United States)

Berko, Netanel S; Hay, Arielle; Sterba, Yonit; Wahezi, Dawn; Levin, Terry L

2015-09-01

Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P myositis were 75% and 31%, respectively, with a sensitivity of 40% and specificity of 67%. Compression-strain US

Proliferative myositis: a case report

International Nuclear Information System (INIS)

Kim, Young Sook; Jeon, Ho Jong

2002-01-01

We report a case of proliferative myositis arising in the pectoralis major muscle of a 59-year-old man who presented with palpable mass. The initial clinical impression was a malignant tumor. Ultrasonography revealed the lesion as a spindle-shaped hypoechoic mass, and MR imaging of the left pectoralis major muscle showed hypointensity at T1-weighted imaging, hyperintensity at T2-weighted imaging, and strong enhancement at contrast-enhanced T1-weighted imaging

Proliferative myositis: a case report

Energy Technology Data Exchange (ETDEWEB)

Kim, Young Sook; Jeon, Ho Jong [Chosun University College of Medicine, Gwangju (Korea, Republic of)

2002-09-01

We report a case of proliferative myositis arising in the pectoralis major muscle of a 59-year-old man who presented with palpable mass. The initial clinical impression was a malignant tumor. Ultrasonography revealed the lesion as a spindle-shaped hypoechoic mass, and MR imaging of the left pectoralis major muscle showed hypointensity at T1-weighted imaging, hyperintensity at T2-weighted imaging, and strong enhancement at contrast-enhanced T1-weighted imaging.

[Evaluation of association between an acute attack of childhood bronchial asthma and Chlamydia pneumoniae infection].

Science.gov (United States)

Jiang, Yi; Liu, Xing-Lian; Xing, Fu-Qiang; Yang, Ju-Sheng; Tu, Hong

2006-04-01

To identify whether there is an association between an acute attack of childhood bronchial asthma and Chlamydia pneumoniae (CP) infection. Serum specific antibodies IgM and IgG to CP were detected by ELISA in 120 asthmatic children with an acute attack and 82 healthy children. Anti-CP IgM was demonstrated in 22 cases (18.3%) and anti-CP IgG was demonstrated in 32 cases (26.7%) out of the 120 asthmatic patients. The incidence of CP infection in asthmatic children was significantly higher than that in healthy controls (3.7%) (P attack of asthma in 15 cases out of the 32 cases with CP infection, but 17 cases required glucocorticoid inhalation treatment together with anti-CP infection treatment (macrolide antibiotics, eg. azithromycin) for remission of asthma attack. There may be a link between an acute attack of childhood asthma and CP infection. It is thus necessary to detect the CP-specific antibodies in asthmatic children for proper treatment.

Myositis Ossificans in a 4-year-old Child

Directory of Open Access Journals (Sweden)

HW Boon

2010-11-01

Full Text Available Sprengel’s shoulder and myositis ossificans (MO are rarely seen concomitantly. This report is about a rare case in a 4 year-old girl who presented with right shoulder deformity and pain associated with right proximal arm swelling.

Richtlijn 'dermatomyositis, polymyositis en sporadische "inclusion body"-myositis'

NARCIS (Netherlands)

Hoogendijk, J. E.; Bijlsma, J. W. J.; van Engelen, B. G. M.; Lindeman, E.; van Royen-Kerkhof, A.; de Rie, M. A.; de Visser, M.; Jennekens, F. G. I.

2005-01-01

This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a

Myositis ossificans circumscripta, secondary to high-velocity gunshot and fragment wound that causes sciatica.

Science.gov (United States)

Gokkus, Kemal; Sagtas, Ergin; Suslu, Feride Ekimler; Aydin, Ahmet Turan

2013-10-17

This report concerns an unusual cause of sciatica. The case presented is of a young man with myositis ossificans that resulted in sciatica and was treated with en bloc excision and low-dose radiotherapy and indomethacine. The aim of this study was to explain the different diagnostic properties of myositis ossificans around the hip and non-classic causes of sciatica.

The role of radiation therapy in childhood acute leukemia. A review from the viewpoint of basic and clinical radiation oncology

International Nuclear Information System (INIS)

Nozaki, Miwako

2003-01-01

Radiation therapy has been playing important roles in the treatment of childhood acute leukemia since the 1970s. The first is the preventive cranial irradiation for central nervous system therapy in acute lymphoblastic leukemia. The second is the total body irradiation as conditioning before bone marrow transplantation for children with acute myeloid leukemia in first remission and with acute lymphoblastic leukemia in second remission. Although some late effects have been reported, a part of them could be overcome by technical improvement in radiation and salvage therapy. Radiation therapy for children might have a successful outcome on a delicate balance between efficiencies and potential late toxicities. The role of radiation therapy for childhood acute leukemia was reviewed from the standpoint of basic and clinical radiation oncology in this paper. (author)

Decreased PARP and procaspase-2 protein levels are associated with cellular drug resistance in childhood acute lymphoblastic leukemia

NARCIS (Netherlands)

A. Holleman (Amy); M.L. den Boer (Monique); K.M. Kazemier (Karin); H.B. Beverloo (Berna); A.R.M. von Bergh (Anne); G.E. Janka-Schaub (Gritta); R. Pieters (Rob)

2005-01-01

textabstractDrug resistance in childhood acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) is associated with impaired ability to induce apoptosis. To elucidate causes of apoptotic defects, we studied the protein expression of Apaf-1, procaspases-2, -3, -6, -7,

Proliferating Myositis: An Inflammatory Lesion often Misdiagnosed as A Malignant Tumor.

Science.gov (United States)

Binesh, Fariba; Sobhanardekani, Mohammad; Zabihi, Somayeh; Behniafard, Nasim

2016-12-01

Proliferative myositis (PM) is a rare inflammatory disease. Most commonly, the lesion occurs in the extremities. Regarding its fast growth and bizarre shape of the cellular components this entity commonly misdiagnosed and the patients undergo improper therapeutic approaches. In other words, it is often misdiagnosed as sarcoma. The diagnosis can only be made by the microscopic examination, so biopsy is mandatory. Here the authors report a patient with PM who was initially misdiagnosed as pleomorphic sarcoma of the lower extremity and explain this rare entity. Proliferative myositis should be taken into account if a fast growing, intramuscular mass occurs in the extremities.

Myositis ossificans with Ga-67 citrate positivity

International Nuclear Information System (INIS)

Moreno, A.J.; Yedinak, M.A.; Spicer, M.J.; Turnbull, G.L.; Byrd, B.F.; Brown, T.J.

1985-01-01

A 16 year-old boy presented with a firm mass within the left thigh. Ga-67 citrate and bone scintigraphy revealed soft tissue radiotracer accumulation within the mass lesion. Radiographs and xerography of the left thigh revealed calcification within the soft tissue mass typical of myositis ossificans. The patient's clinical course was uneventful

Inflammatory focal myositis of the sternomastoid muscle: is there an absolute indication for biopsy? A case report and review of the literature

NARCIS (Netherlands)

Georgalas, Christos; Kapoor, Lekha; Chau, Ha; Bhattacharyya, Abir

2006-01-01

Focal myositis is a localised inflammatory process affecting skeletal muscles belonging to the pathological group of inflammatory pseudo tumours of soft tissue that includes myositis ossificans, proliferative myositis and nodular pseudosarcomatous fasciitis. Very rarely, it may affect one of the

Cytosolic 5′-nucleotidase 1A autoantibody profile and clinical characteristics in inclusion body myositis

Science.gov (United States)

Rietveld, A; Pye, S R; Mariampillai, K; Benveniste, O; Peeters, M T J; Miller, J A L; Hanna, M G; Machado, P M; Parton, M J; Gheorghe, K R; Badrising, U A; Lundberg, I E; Sacconi, S; Herbert, M K; McHugh, N J; Lecky, B R F; Brierley, C; Hilton-Jones, D; Lamb, J A; Roberts, M E; Cooper, R G; Saris, C G J; Pruijn, G J M; Chinoy, H; van Engelen, B G M

2017-01-01

Objectives Autoantibodies directed against cytosolic 5′-nucleotidase 1A have been identified in many patients with inclusion body myositis. This retrospective study investigated the association between anticytosolic 5′-nucleotidase 1A antibody status and clinical, serological and histopathological features to explore the utility of this antibody to identify inclusion body myositis subgroups and to predict prognosis. Materials and methods Data from various European inclusion body myositis registries were pooled. Anticytosolic 5′-nucleotidase 1A status was determined by an established ELISA technique. Cases were stratified according to antibody status and comparisons made. Survival and mobility aid requirement analyses were performed using Kaplan-Meier curves and Cox proportional hazards regression. Results Data from 311 patients were available for analysis; 102 (33%) had anticytosolic 5′-nucleotidase 1A antibodies. Antibody-positive patients had a higher adjusted mortality risk (HR 1.89, 95% CI 1.11 to 3.21, p=0.019), lower frequency of proximal upper limb weakness at disease onset (8% vs 23%, adjusted OR 0.29, 95% CI 0.12 to 0.68, p=0.005) and an increased prevalence of excess of cytochrome oxidase deficient fibres on muscle biopsy analysis (87% vs 72%, adjusted OR 2.80, 95% CI 1.17 to 6.66, p=0.020), compared with antibody-negative patients. Interpretation Differences were observed in clinical and histopathological features between anticytosolic 5′-nucleotidase 1A antibody positive and negative patients with inclusion body myositis, and antibody-positive patients had a higher adjusted mortality risk. Stratification of inclusion body myositis by anticytosolic 5′-nucleotidase 1A antibody status may be useful, potentially highlighting a distinct inclusion body myositis subtype with a more severe phenotype. PMID:28122761

Deregulated WNT signaling in childhood T-cell acute lymphoblastic leukemia

International Nuclear Information System (INIS)

Ng, O H; Erbilgin, Y; Firtina, S; Celkan, T; Karakas, Z; Aydogan, G; Turkkan, E; Yildirmak, Y; Timur, C; Zengin, E; Dongen, J J M van; Staal, F J T; Ozbek, U; Sayitoglu, M

2014-01-01

WNT signaling has been implicated in the regulation of hematopoietic stem cells and plays an important role during T-cell development in thymus. Here we investigated WNT pathway activation in childhood T-cell acute lymphoblastic leukemia (T-ALL) patients. To evaluate the potential role of WNT signaling in T-cell leukomogenesis, we performed expression analysis of key components of WNT pathway. More than 85% of the childhood T-ALL patients showed upregulated β-catenin expression at the protein level compared with normal human thymocytes. The impact of this upregulation was reflected in high expression of known target genes (AXIN2, c-MYC, TCF1 and LEF). Especially AXIN2, the universal target gene of WNT pathway, was upregulated at both mRNA and protein levels in ∼40% of the patients. When β-CATENIN gene was silenced by small interfering RNA, the cancer cells showed higher rates of apoptosis. These results demonstrate that abnormal WNT signaling activation occurs in a significant fraction of human T-ALL cases independent of known T-ALL risk factors. We conclude that deregulated WNT signaling is a novel oncogenic event in childhood T-ALL

Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

Energy Technology Data Exchange (ETDEWEB)

Berko, Netanel S.; Levin, Terry L. [Montefiore Medical Center, Department of Radiology, Bronx, NY (United States); Hay, Arielle [Montefiore Medical Center, Department of Pediatrics, Division of Pediatric Rheumatology, Bronx, NY (United States); Miami Children' s Hospital, Department of Pediatrics, Miami, FL (United States); Sterba, Yonit; Wahezi, Dawn [Montefiore Medical Center, Department of Pediatrics, Division of Pediatric Rheumatology, Bronx, NY (United States)

2015-09-15

Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P < 0.001). The positive and negative predictive values for elastography in the determination of active myositis were 75% and 31

Efficacy of ultrasound elastography in detecting active myositis in children: can it replace MRI?

International Nuclear Information System (INIS)

Berko, Netanel S.; Levin, Terry L.; Hay, Arielle; Sterba, Yonit; Wahezi, Dawn

2015-01-01

Juvenile idiopathic inflammatory myopathy is a rare yet potentially debilitating condition. MRI is used both for diagnosis and to assess response to treatment. No study has evaluated the performance of US elastography in the diagnosis of this condition in children. To assess the performance of compression-strain US elastography in detecting active myositis in children with clinically confirmed juvenile idiopathic inflammatory myopathy and to compare its efficacy to MRI. Children with juvenile idiopathic inflammatory myopathy underwent non-contrast MR imaging as well as compression-strain US elastography of the quadriceps muscles. Imaging findings from both modalities were compared to each other as well as to the clinical determination of active disease based on physical examination and laboratory data. Active myositis on MR was defined as increased muscle signal on T2-weighted images. Elastography images were defined as normal or abnormal based on a previously published numerical scale of muscle elastography in normal children. Muscle echogenicity was graded as normal or abnormal based on gray-scale sonographic images. Twenty-one studies were conducted in 18 pediatric patients (15 female, 3 male; age range 3-19 years). Active myositis was present on MRI in ten cases. There was a significant association between abnormal MRI and clinically active disease (P = 0.012). US elastography was abnormal in 4 of 10 cases with abnormal MRI and in 4 of 11 cases with normal MRI. There was no association between abnormal elastography and either MRI (P > 0.999) or clinically active disease (P > 0.999). Muscle echogenicity was normal in 11 patients; all 11 had normal elastography. Of the ten patients with increased muscle echogenicity, eight had abnormal elastography. There was a significant association between muscle echogenicity and US elastography (P < 0.001). The positive and negative predictive values for elastography in the determination of active myositis were 75% and 31

Altered brain function in new onset childhood acute lymphoblastic leukemia before chemotherapy: A resting-state fMRI study.

Science.gov (United States)

Hu, Zhanqi; Zou, Dongfang; Mai, Huirong; Yuan, Xiuli; Wang, Lihong; Li, Yue; Liao, Jianxiang; Liu, Liwei; Liu, Guosheng; Zeng, Hongwu; Wen, Feiqiu

2017-10-01

Cognitive impairments had been reported in childhood acute lymphoblastic leukemia, what caused the impairments needed to be demonstrated, chemotherapy-related or the disease itself. The primary aim of this exploratory investigation was to determine if there were changes in brain function of children with acute lymphoblastic leukemia before chemotherapy. In this study, we advanced a measure named regional homogeneity to evaluate the resting-state brain activities, intelligence quotient test was performed at same time. Using regional homogeneity, we first investigated the resting state brain function in patients with new onset childhood acute lymphoblastic leukemia before chemotherapy, healthy children as control. The decreased ReHo values were mainly founded in the default mode network and left frontal lobe, bilateral inferior parietal lobule, bilateral temporal lobe, bilateral occipital lobe, precentral gyrus, bilateral cerebellum in the newly diagnosed acute lymphoblastic leukemia patients compared with the healthy control. While in contrast, increased ReHo values were mainly shown in the right frontal lobe (language area), superior frontal gyrus-R, middle frontal gyrus-R and inferior parietal lobule-R for acute lymphoblastic leukemia patients group. There were no significant differences for intelligence quotient measurements between the acute lymphoblastic leukemia patient group and the healthy control in performance intelligence quotient, verbal intelligence quotient, total intelligence quotient. The altered brain functions are associated with cognitive change and language, it is suggested that there may be cognition impairment before the chemotherapy. Regional homogeneity by functional magnetic resonance image is a sensitive way for early detection on brain damage in childhood acute lymphoblastic leukemia. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Associations between apparent diffusion coefficient and electromyography parameters in myositis-A preliminary study.

Science.gov (United States)

Meyer, Hans-Jonas; Emmer, Alexander; Kornhuber, Malte; Surov, Alexey

2018-05-01

MRI is widely used in several muscle disorders. Diffusion-weighted imaging (DWI) is an emergent imaging modality sensitive to microstructural alterations in tissue. The apparent diffusion coefficient (ADC) is used to quantify the random motion of water molecules. Electromyography (EMG) is a clinically used diagnostic tool in myositis. The aim of this study was to elucidate possible associations between ADC values and EMG findings in myositis patients. Seven patients (eight investigated muscles) with myositis (mean age 51.43 ± 19 years) were included in this study. The diagnosis was confirmed by histopathology in every case. DWI was obtained with a 1.5-T scanner using two b-values 0 and 1000 s/mm². In all patients, a needle electromyography (EMG) was performed within 3 days to the MRI. The following EMG parameters were studied: motor unit action potential (MUAP) amplitudes and durations, as well as pathological spontaneous activity. Spearman's correlation coefficient was used to analyze associations between investigated parameters. The estimated mean ADC mean value was 1.51 ± 0.29 × 10 -3  mm²/s, mean ADC min was 1.28 ± 0.27 × 10 -3  mm²/s, and mean ADC max was 1.73 ± 0.28 × 10 -3  mm²/s. Correlation analysis identified significant associations between ADC mean and duration of the MUAP (p   = .78 P = .0279) and between ADC min and duration of the MUAP (p = .85, P = .01). There were no significant differences according to pathological spontaneous activity. ADC mean and ADC min showed strong positive correlations with the duration of the MUAP in myositis patients. Both modalities might similarly reflect muscle fiber loss in myositis patients.

Adult Niemann-Pick disease type B with myositis ossificans: a case report

Directory of Open Access Journals (Sweden)

Russka Shumnalieva

2016-07-01

Full Text Available Niemann-Pick Disease (NPD is a rare autosomal recessive lysosomal lipid storage disorder. The disease is caused by gene mutations that affect the metabolism of sphingolipids. The dysfunctions cause sphingomyelin to accumulate in different organs. NPD includes forms with low and high levels of sphingomyelin. We report a case of a 34 year-old man with a family history of NPD type B who presented with hepatosplenomegaly, neurological deficiency, bone abnormalities, and myositis ossificans. The clinical, biochemical, and imaging data confirmed the combined diagnosis of NPD type B with myositis ossificans.

Blood-flow restricted resistance training in patients with sporadic inclusion body myositis

DEFF Research Database (Denmark)

Jørgensen, A.; Aagaard, P.; Frandsen, U.

2018-01-01

Objectives: To investigate the effect of 12 weeks of low-load blood-flow restricted resistance (BFR) training on self-reported and objective physical function, and maximal muscle strength in patients with sporadic inclusion body myositis (sIBM). Method: Twenty-two patients with sIBM were randomized......), which was used to measure self-reported physical function. All patients performed physical function tests (2-Minute Walk Test, Timed Up and Go, and 30-Second Chair Stand), completed the Inclusion Body Myositis Functional Rating Scale (IBMFRS), and were tested for isolated knee extensor muscle strength...

The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

Science.gov (United States)

Rider, Lisa G; Shah, Mona; Mamyrova, Gulnara; Huber, Adam M; Rice, Madeline Murguia; Targoff, Ira N; Miller, Frederick W

2013-07-01

The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. In follow-up to our study defining the major clinical subgroup phenotypes of JIIM, we compared demographics, clinical features, laboratory measures, and outcomes among myositis-specific autoantibody (MSA) subgroups, as well as with published data on adult idiopathic inflammatory myopathy patients enrolled in a separate natural history study. In the present study, of 430 patients enrolled in a nationwide registry study who had serum tested for myositis autoantibodies, 374 had either a single specific MSA (n = 253) or no identified MSA (n = 121) and were the subject of the present report. Following univariate analysis, we used random forest classification and exact logistic regression modeling to compare autoantibody subgroups. Anti-p155/140 autoantibodies were the most frequent subgroup, present in 32% of patients with juvenile dermatomyositis (JDM) or overlap myositis with JDM, followed by anti-MJ autoantibodies, which were seen in 20% of JIIM patients, primarily in JDM. Other MSAs, including anti-synthetase, anti-signal recognition particle (SRP), and anti-Mi-2, were present in only 10% of JIIM patients. Features that characterized the anti-p155/140 autoantibody subgroup included Gottron papules, malar rash, "shawl-sign" rash, photosensitivity, cuticular overgrowth, lowest creatine kinase (CK) levels, and a predominantly chronic illness course. The features that differed for patients with anti-MJ antibodies included muscle cramps, dysphonia, intermediate CK levels, a high frequency of hospitalization, and a monocyclic disease course. Patients with anti-synthetase antibodies had higher frequencies of interstitial lung disease, arthralgia, and "mechanic's hands," and had an older age at diagnosis. The anti-SRP group, which had exclusively juvenile polymyositis, was characterized by high

Non-traumatic myositis ossificans mimicking a malignant neoplasm in an 83-year-old woman: a case report

Directory of Open Access Journals (Sweden)

Nishio Jun

2010-08-01

Full Text Available Abstract Introduction Myositis ossificans is a benign, self-limiting condition that usually affects young, athletically active men. To the best of our knowledge, this case report describes the oldest recorded patient with myositis ossificans. Case presentation Our patient was an 83-year-old Japanese woman who presented with a one week history of a palpable mass in the left thigh. She had a history of surgery for transverse colon cancer and lung cancer at the ages of 73 and 80, respectively. Clinical and radiological examinations suggested a malignant neoplasm such as metastatic carcinoma or extraskeletal osteosarcoma. A diagnosis of myositis ossificans was made by core needle biopsy. Our patient was asymptomatic and had no recurrence at one year follow-up. Conclusion Clinicians should consider myositis ossificans as a possible diagnosis for a soft tissue mass in the limb of an older patient, thereby avoiding unnecessarily aggressive therapy.

Host genome variations and risk of infections during induction treatment for childhood acute lymphoblastic leukaemia

DEFF Research Database (Denmark)

Lund, Bendik; Wesolowska-Andersen, Agata; Lausen, Birgitte

2014-01-01

Objectives: To investigate association of host genomic variation and risk of infections during treatment for childhood acute lymphoblastic leukaemia (ALL). Methods: We explored association of 34 000 singlenucleotide polymorphisms (SNPs) related primarily to pharmacogenomics and immune function...

New developments in genetics of myositis.

Science.gov (United States)

Rothwell, Simon; Lamb, Janine A; Chinoy, Hector

2016-11-01

This article reviews the advances that have been made in our understanding of the genetics of the idiopathic inflammatory myopathies (IIM) in the past 2 years, with a particular focus on polymyositis, dermatomyositis and inclusion body myositis. Two large human leukocyte antigen (HLA) imputation studies have confirmed a strong association with the 8.1 ancestral haplotype in clinical subgroups of myositis and suggest multiple independent associations on this haplotype. Risk in these genes may be due to specific amino acid positions within the peptide-binding grooves of HLA molecules. A large genetic study in 2566 IIM patients revealed associations such as PTPN22, STAT4, UBE2L3 and BLK, which overlap with risk variants reported in other seropositive autoimmune diseases. There is also evidence of different genetic architectures in clinical subgroups of IIM. Candidate gene studies in the Japanese and Chinese populations have replicated previous IIM associations which suggest common aetiology between ethnicities. International collaborations have facilitated large genetic studies in IIM that have revealed much about the genetics of this rare complex disease both within the HLA region and genome-wide. Future approaches, such as sequencing and trans-ethnic meta-analyses, will advance our knowledge of IIM genetics.

Acute neuromuscular weakness associated with dengue infection

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Harmanjit Singh Hira

2012-01-01

Full Text Available Background: Dengue infections may present with neurological complications. Whether these are due to neuromuscular disease or electrolyte imbalance is unclear. Materials and Methods: Eighty-eight patients of dengue fever required hospitalization during epidemic in year 2010. Twelve of them presented with acute neuromuscular weakness. We enrolled them for study. Diagnosis of dengue infection based on clinical profile of patients, positive serum IgM ELISA, NS1 antigen, and sero-typing. Complete hemogram, kidney and liver functions, serum electrolytes, and creatine phosphokinase (CPK were tested. In addition, two patients underwent nerve conduction velocity (NCV test and electromyography. Results: Twelve patients were included in the present study. Their age was between 18 and 34 years. Fever, myalgia, and motor weakness of limbs were most common presenting symptoms. Motor weakness developed on 2 nd to 4 th day of illness in 11 of 12 patients. In one patient, it developed on 10 th day of illness. Ten of 12 showed hypokalemia. One was of Guillain-Barré syndrome and other suffered from myositis; they underwent NCV and electromyography. Serum CPK and SGOT raised in 8 out of 12 patients. CPK of patient of myositis was 5098 IU. All of 12 patients had thrombocytopenia. WBC was in normal range. Dengue virus was isolated in three patients, and it was of serotype 1. CSF was normal in all. Within 24 hours, those with hypokalemia recovered by potassium correction. Conclusions: It was concluded that the dengue virus infection led to acute neuromuscular weakness because of hypokalemia, myositis, and Guillain-Barré syndrome. It was suggested to look for presence of hypokalemia in such patients.

Challenges in implementing individualized medicine illustrated by antimetabolite therapy of childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Nersting, Jacob; Borst, Louise; Schmiegelow, Kjeld

2011-01-01

illustrated by studies involving childhood acute lymphoblastic leukemia (ALL), where each patient may receive up to 13 different anticancer agents over a period of 2-3 years. The challenges include i) addressing important, but low-frequency outcomes, ii) difficulties in interpreting the impact of single drug...

Implications of infectious diseases and the adrenal hypothesis for the etiology of childhood acute lymphoblastic leukemia

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F. Azevedo-Silva

2010-03-01

Full Text Available Acute leukemia is the most frequent cancer in children. Recently, a new hypothesis was proposed for the pathogenesis of childhood acute lymphoblastic leukemia (ALL. The so-called "adrenal hypothesis" emphasized the role of endogenous cortisol in the etiology of B-cell precursor ALL. The incidence peak of ALL in children between 3 to 5 years of age has been well documented and is consistent with this view. The adrenal hypothesis proposes that the risk of childhood B-cell precursor ALL is reduced when early childhood infections induce qualitative and quantitative changes in the hypothalamus-pituitary-adrenal axis. It suggests that the increased plasma cortisol levels would be sufficient to eliminate all clonal leukemic cells originating during fetal life. Because Brazil is a continental and tropical country, the exposure to infections is diversified with endemic viral and regionally non-viral infections, with some characteristics that support the recent adrenal hypothesis. Here we discuss this new hypothesis in terms of data from epidemiological studies and the possible implications of the diversity of infections occurring in Brazilian children.

Dermatomyositis Sine Myositis with Membranoproliferative Glomerulonephritis

Directory of Open Access Journals (Sweden)

Mohammad Bagher Owlia

2012-01-01

Full Text Available Dermatomyositis (DM is an autoimmune disease that is characterized by involvement of proximal musculature and skin. We report a 52-year-old woman with a 6-year history of dermatomyositis sine myositis, who developed lower extremity edema and proteinuria. Pathological examination of renal biopsy showed membranoproliferative glomerulonephritis. She received steroid, cyclophosphamide, and mycophenolate mofetil. Over the 9 to 10 months after the beginning of treatment, the proteinuria was improved.

Plasma Exchange for Refractory MDA5 Myositis and ILD

Science.gov (United States)

2017-04-26

plasma exchange, the patient had improvement of hypocarbic respiratory alkalosis and reversal of myositis with resolution of dysphagia/odynophagia and...symptoms with respiratory distress. A five day course of plasmapheresis was initiated on hospital day eight. Subsequently his respiratory acid-base

Self-resolving focal non-ossifying myositis: a poorly known clinical and imaging entity diagnosed with MRI

International Nuclear Information System (INIS)

Perlepe, Vasiliki; Dallaudière, Benjamin; Omoumi, Patrick; Hristova, Lora; Rezzazadeh, Afshin; Vande Berg, Bruno; Malghem, Jacques; Lecouvet, Frederic

2015-01-01

Focal myositis is a rare benign inflammatory pseudotumor, presenting as a painful nodular mass within a muscle, and characterized by spontaneous resolution within weeks. To assess the clinical and imaging findings of focal nodular myositis simulating a neoplasm at clinical examination, with no history of trauma. This study describes the locations and appearance at ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) of this condition in a series of five patients. MRI and US displayed a solid intramuscular “tumor” and suggested a continuum between the proximal and distal muscle fibers that appeared thickened within the nodular lesion, a sign that has been reported in myositis ossificans. MRI showed edema in adjacent muscles and soft tissues, as well as intense enhancement of the mass. Intense vascular flows were seen at Doppler analysis. CT did not reveal the appearance of peripheral ossifications, ruling out the diagnosis of myositis ossificans. In some patients, the diagnosis of sarcoma had been suggested as possible by the radiologist. Imaging follow-up with MRI showed complete resolution of the masses over several weeks, thus avoiding a biopsy; no recurrence was observed at long-term follow-up (more than 24 months). This paper highlights MRI and US findings in focal non-ossifying myositis, and emphasizes the role of MRI in suggesting this diagnosis, leading to the careful follow-up of the lesion until its resolution, and ruling out more aggressive lesions

Childhood central nervous system leukemia: historical perspectives, current therapy, and acute neurological sequelae

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Laningham, Fred H. [St. Jude Children' s Research Hospital, Division of Diagnostic Imaging, Department of Radiological Sciences, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States); Kun, Larry E. [St. Jude Children' s Research Hospital, Division of Radiation Oncology, Department of Radiological Sciences, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States); Reddick, Wilburn E.; Ogg, Robert J. [St. Jude Children' s Research Hospital, Division of Translational Imaging Research, Department of Radiological Sciences, Memphis, TN (United States); Morris, E.B. [St. Jude Children' s Research Hospital, Department of Oncology, Memphis, TN (United States); Pui, Ching-Hon [St. Jude Children' s Research Hospital, Department of Oncology, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States)

2007-11-15

During the past three decades, improvements in the treatment of childhood leukemia have resulted in high cure rates, particularly for acute lymphoblastic leukemia (ALL). Unfortunately, successful therapy has come with a price, as significant morbidity can result from neurological affects which harm the brain and spinal cord. The expectation and hope is that chemotherapy, as a primary means of CNS therapy, will result in acceptable disease control with less CNS morbidity than has been observed with combinations of chemotherapy and radiotherapy over the past several decades. In this review we discuss the poignant, historical aspects of CNS leukemia therapy, outline current methods of systemic and CNS leukemia therapy, and present imaging findings we have encountered in childhood leukemia patients with a variety of acute neurological conditions. A major objective of our research is to understand the neuroimaging correlates of acute and chronic effects of cancer and therapy. Specific features related to CNS leukemia and associated short-term toxicities, both disease- and therapy-related, are emphasized in this review with the specific neuroimaging findings. Specific CNS findings are similarly important when treating acute myelogenous leukemia (AML), and details of leukemic involvement and toxicities are also presented in this entity. Despite contemporary treatment approaches which favor the use of chemotherapy (including intrathecal therapy) over radiotherapy in the treatment of CNS leukemia, children still occasionally experience morbid neurotoxicity. Standard neuroimaging is sufficient to identify a variety of neurotoxic sequelae in children, and often suggest specific etiologies. Specific neuroimaging findings frequently indicate a need to alter antileukemia therapy. It is important to appreciate that intrathecal and high doses of systemic chemotherapy are not innocuous and are associated with acute, specific, recognizable, and often serious neurological

Childhood central nervous system leukemia: historical perspectives, current therapy, and acute neurological sequelae

International Nuclear Information System (INIS)

Laningham, Fred H.; Kun, Larry E.; Reddick, Wilburn E.; Ogg, Robert J.; Morris, E.B.; Pui, Ching-Hon

2007-01-01

During the past three decades, improvements in the treatment of childhood leukemia have resulted in high cure rates, particularly for acute lymphoblastic leukemia (ALL). Unfortunately, successful therapy has come with a price, as significant morbidity can result from neurological affects which harm the brain and spinal cord. The expectation and hope is that chemotherapy, as a primary means of CNS therapy, will result in acceptable disease control with less CNS morbidity than has been observed with combinations of chemotherapy and radiotherapy over the past several decades. In this review we discuss the poignant, historical aspects of CNS leukemia therapy, outline current methods of systemic and CNS leukemia therapy, and present imaging findings we have encountered in childhood leukemia patients with a variety of acute neurological conditions. A major objective of our research is to understand the neuroimaging correlates of acute and chronic effects of cancer and therapy. Specific features related to CNS leukemia and associated short-term toxicities, both disease- and therapy-related, are emphasized in this review with the specific neuroimaging findings. Specific CNS findings are similarly important when treating acute myelogenous leukemia (AML), and details of leukemic involvement and toxicities are also presented in this entity. Despite contemporary treatment approaches which favor the use of chemotherapy (including intrathecal therapy) over radiotherapy in the treatment of CNS leukemia, children still occasionally experience morbid neurotoxicity. Standard neuroimaging is sufficient to identify a variety of neurotoxic sequelae in children, and often suggest specific etiologies. Specific neuroimaging findings frequently indicate a need to alter antileukemia therapy. It is important to appreciate that intrathecal and high doses of systemic chemotherapy are not innocuous and are associated with acute, specific, recognizable, and often serious neurological

Treatment of relapsed or refractory acute leukemia in childhood with bisantrene combined with high dose aracytine.

Science.gov (United States)

Leblanc, T; Deméocq, F; Leverger, G; Baruchel, A; Lemerle, S; Vannier, J P; Nelken, B; Guillot, T; Schaison, G

1994-01-01

Bisantrene is an anthracene derivative which has demonstrated activity in acute myeloblastic leukemia (AML) and in lymphoma. The present study was designed to assess the reinduction rate and toxicity of bisantrene (250 mg/m2/d x 5) associated with aracytine (100 mg/m2 twice a day x 5) in refractory and relapsed acute childhood leukemia. Patients who relapsed after bone marrow transplantation were eligible. Twenty-six children were included. Diagnoses were as follows: 13 AML, 9 acute lymphoblastic leukemia (ALL), and 4 undifferentiated leukemia (AUL). All patients had been very highly pretreated, especially with anthracyclines, and most of them were of poor prognosis. The overall response rate was 46% with a 95% confidence interval ranging from 27-65%. According to diagnosis, complete remission (CR) rates are: AML: 5/13, ALL: 5/9, and AUL: 2/4. Four children died, three from infection and one from acute lysis syndrome. The major toxicity was infection with grade 3 and 4 episodes occurring in 42% of patients. No significant cardiac toxicity was noted. Hepatic and renal toxicity was noted. Hepatic and renal toxicity were limited and transient. Bisantrene in association with aracytine is effective in both AML and ALL of childhood. Bisantrene should be evaluated with a five-day schedule in other pediatric malignancies. In children with acute leukemia previously treated with high dose aracytine, new combination regimen is warranted.

[Childhood acute lymphoblastic leukemia in Norway 1992-2000].

Science.gov (United States)

Kolmannskog, Svein; Flaegstad, Trond; Helgestad, Jon; Hellebostad, Marit; Zeller, Bernward; Glomstein, Anders

2007-05-31

Acute lymphoblastic leukemia is the most common malignancy in childhood. The survival rate has increased steadily over the last 40 years. All children aged 0-15 years and diagnosed in Norway in the period 1992-2000, were included in the study (n = 301). The patients were followed up until 1.1. 2005. The diagnosis was made in 301 children, 33 new cases per year (range 24 to 40) on average. The peak incidence was between 2 and 5 years. Four of 6 infants with acute lymphoblastic leukemia and all 4 with mature B-cell leukemia are alive. Two of the remaining 291 children died before treatment was started. 289 were all treated according to the common Nordic NOPHO-ALL 1992 protocol. All children achieved remission (99.7%), except for one who died before remission was achieved. 55 children (19%) relapsed. Radiation to the brain as part of central nervous system prophylaxis was given to just 10% of the children. The 10-year event-free survival (p-EFS) was 76%, and 244 of 289 (84%) were alive 4-13 years after the diagnosis was made. The data are comparable with the best international results.

Myositis in primary Sjögren's syndrome: data from a multicentre cohort.

Science.gov (United States)

Colafrancesco, Serena; Priori, Roberta; Gattamelata, Angelica; Picarelli, Giovanna; Minniti, Antonina; Brancatisano, Filippo; D'Amati, Giulia; Giordano, Carla; Cerbelli, Bruna; Maset, Marta; Quartuccio, Luca; Bartoloni, Elena; Carubbi, Francesco; Cipriani, Paola; Baldini, Chiara; Luciano, Nicoletta; De Vita, Salvatore; Gerli, Roberto; Giacomelli, Roberto; Bombardieri, Stefano; Valesini, Guido

2015-01-01

In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was performed in 13/17 (76.4%) cases with histologically confirmed myositis in 6/13 (46.1%) (1"IBM-like"-5"PM-like"). In two "PM-like" cases, several fibres showed a decreased histochemical cytochrome C oxidase (COX) stain. Two biopsies tested "negative", four showed "non-specific" findings. All patients were treated with corticosteroids followed by different DMARDs. Our retrospective analysis shows a prevalence of myositis in pSS lower than previously reported, mainly appearing as an overlapping syndrome. Histological findings confirm the possible presence of an IBM or of a myopathy more similar to PM with a decreased COX activity. Classical immunosuppressants are effective although in most difficult cases IVIg or RTX may be used with benefit.

Clinicopathologic features of myositis patients with CD8-MHC-1 complex pathology.

Science.gov (United States)

Ikenaga, Chiseko; Kubota, Akatsuki; Kadoya, Masato; Taira, Kenichiro; Uchio, Naohiro; Hida, Ayumi; Maeda, Meiko Hashimoto; Nagashima, Yu; Ishiura, Hiroyuki; Kaida, Kenichi; Goto, Jun; Tsuji, Shoji; Shimizu, Jun

2017-09-05

To determine the clinical features of myositis patients with the histopathologic finding of CD8-positive T cells invading non-necrotic muscle fibers expressing major histocompatibility complex class 1 (CD8-MHC-1 complex), which is shared by polymyositis (PM) and inclusion body myositis (IBM), in relation to the p62 immunostaining pattern of muscle fibers. All 93 myositis patients with CD8-MHC-1 complex who were referred to our hospital from 1993 to 2015 were classified on the basis of the European Neuromuscular Center (ENMC) diagnostic criteria for IBM (Rose, 2013) or PM (Hoogendijk, 2004) and analyzed. The 93 patients included were 17 patients with PM, 70 patients with IBM, and 6 patients who neither met the criteria for PM nor IBM in terms of muscle weakness distribution (unclassifiable group). For these PM, IBM, and unclassifiable patients, their mean ages at diagnosis were 63, 70, and 64 years; autoimmune disease was present in 7 (41%), 13 (19%), and 4 (67%); hepatitis C virus infection was detected in 0%, 13 (20%), and 2 (33%); and p62 was immunopositive in 0%, 66 (94%), and 2 (33%), respectively. Of the treated patients, 11 of 16 PM patients and 4 of 6 p62-immunonegative patients in the unclassifiable group showed responses to immunotherapy, whereas all 44 patients with IBM and 2 p62-immunopositive patients in the unclassifiable group were unresponsive to immunotherapy. CD8-MHC-1 complex is present in patients with PM, IBM, or unclassifiable group. The data may serve as an argument for a trial of immunosuppressive treatment in p62-immunonegative patients with unclassifiable myositis. © 2017 American Academy of Neurology.

Acute necrotising encephalopathy of childhood after exanthema subitum outside Japan or Taiwan

International Nuclear Information System (INIS)

Porto, L.; Lanferman, H.; Moeller-Hartmann, W.; Jacobi, G.; Zanella, F.

1999-01-01

Acute necrotising encephalopathy of childhood (ANE) is an uncommon disease which predominantly affects infants and young children living in Japan and Taiwan. A multifocal encephalopathy with symmetrical lesions in the thalamus, tegmentum of the brain stem, cerebral periventricular white matter and cerebellar medulla is characteristic. We present the imaging features in a 4-year-old Japanese boy who had been living in Germany for 2 1 / 2 years before presentation. (orig.)

Tubuloreticular structures in different types of myositis: implications for pathogenesis

NARCIS (Netherlands)

Bronner, Irene M.; Hoogendijk, Jessica E.; Veldman, Henk; Ramkema, Marja; van den Bergh Weerman, Marius A.; Rozemuller, Annemieke J. M.; de Visser, Marianne

2008-01-01

In dermatomyositis (DM) there is strong histopathological evidence of a microvascular pathogenesis, including endothelial microtubular inclusions. In nonspecific myositis, perimysial and perivascular infiltrates in the muscle biopsy similar to DM are found. Microtubular inclusions in endothelial

Tubuloreticular structures in different types of myositis: Implications for pathogenesis

NARCIS (Netherlands)

Bronner, I.M.; Hoogendijk, J.E.; Veldman, H.; Ramkema, M; Weerman, M.A.V.; Rozemuller, A.J.M.; Visser, M.

2008-01-01

In dermatomyositis (DM) there is strong histopathological evidence of a microvascular pathogenesis, including endothelial microtubular inclusions. In nonspecific myositis, perimysial and perivascular infiltrates in the muscle biopsy similar to DM are found. Microtubular inclusions in endothelial

Surgical Treatment of Traumatic Myositis Ossificans of the Extensor Carpi Radialis Muscle in a Dog.

Science.gov (United States)

Morton, Bridget A; Hettlich, Bianca F; Pool, Roy R

2015-07-01

To report clinical signs, diagnostic imaging findings, and outcome in a dog with traumatic myositis ossificans of the origin of the extensor carpi radialis muscle. Clinical report. An 8-month-old intact female Irish Setter Dog. After radiographic and computed tomographic evaluation of an osseous proliferation arising from the cranial cortex of the right distal humeral diaphysis, the protruding bone was surgically removed and evaluated by histopathology. Traumatic myositis ossificans was successfully treated with surgical removal of the osseous proliferation resulting in improved postoperative range of motion of the right elbow joint. There was no evidence of lameness or abnormal bone regrowth associated with the surgical site radiographically at follow up. Surgical removal of a traumatic myositis ossificans lesion resulted in full return to function in a young, competitive show dog. © Copyright 2014 by The American College of Veterinary Surgeons.

[Risk factors for cancer in patients with myositis. Clinical, immunological characteristics and the role of the anti-p155/140 antibody].

Science.gov (United States)

Szankai, Zsuzsanna; Nagy-Vincze, Melinda; Bodoki, Levente; Jakab, András; Betteridge, Zoe; Dankó, Katalin

2014-09-07

Idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by progressive proximal muscle weakness. Cancer-associated myositis represents the worst prognostic group within this heterogeneous disease. The aim of this study was to reveal factors which increase the risk factors for association of cancerous disease in patients with myositis. Furthermore, the authors explored the most common types of associated malignancies in their patients with myositis and characterize the clinical findings in a sub-group of anti-p155/140 positive patients. In this retrospective study, myositis patients with and without associated cancer were analysed (32 and 64 patients, respectively). In addition, anti-p155/140 positive and negative groups were compared, irrespective to the presence of associated malignancies. The risk for associated malignancy was higher in patients with severe muscle and skin symptoms and those with dermatomyositis. Furthermore, increased risk for malignancy was noted in the presence of particular skin symptoms and the absence of systemic symptoms. The anti-p155/140 antibody was proved to be a feasible marker of an independent clinical sub-group which overlapped clinical characteristics with cancer-associated myositis. These results may help the identification of patients with myositis with a higher risk for associated malignancy.

Myositis ossificans in hemophilia

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Vas, W.; Cockshott, W.P.; Martin, R.F.; Pai, M.K.; Walker, I.

1981-10-01

A review of the radiographs of 60 hemophilia patients showed nine (15%) with ectopic new bone formation. Three of these patients had multiple sites of involvement. The high frequency discovered in this series contrasts with the paucity of descriptions to be found in the literature. This process of myositis ossificans affects the lower half of the body and probably represents dysplastic metaplasia developing at the site of an intramuscular hematoma when remote from bone, as well as ossification of hemorrhagic lesions related to the periosteum. In conventional radiographs anatomic localization of bone foci is difficult, but use of computed tomography permits precise identification of the affected muscle. There is negligible disability associated with this condition.

Myositis ossificans in hemophilia

International Nuclear Information System (INIS)

Vas, W.; Cockshott, W.P.; Martin, R.F.; Pai, M.K.; Walker, I.

1981-01-01

A review of the radiographs of 60 hemophilia patients showed nine (15%) with ectopic new bone formation. Three of these patients had multiple sites of involvement. The high frequency discovered in this series contrasts with the paucity of descriptions to be found in the literature. This process of myositis ossificans affects the lower half of the body and probably represents dysplastic metaplasia developing at the site of an intramuscular hematoma when remote from bone, as well as ossification of hemorrhagic lesions related to the periosteum. In conventional radiographs anatomic localization of bone foci is difficult, but use of computed tomography permits precise identification of the affected muscle. There is negligible disability associated with this condition. (orig.)

Improved outcome of childhood acute myeloid leukemia in an Eastern European country: Lithuanian experience.

Science.gov (United States)

Kairiene, Igne; Pasauliene, Ramune; Lipunova, Nadezda; Vaitkeviciene, Goda; Rageliene, Lina; Rascon, Jelena

2017-10-01

The reported treatment outcomes of children treated for cancer in Eastern European countries are inferior to those in Northern/Western Europe. We hypothesized that recent survival rates could be comparable to the current standards and performed a population-based analysis of treatment outcome of childhood acute myeloid leukemia (AML) in Lithuania, a small Eastern European country. Children  80% in high-income countries. The difference in survival rates between Northern/Western and Eastern European countries as well as between high- and middle-/low-income countries is as much as 20%. Recently, the 5-year event-free survival rate of acute myeloid leukemia (AML) has reached > 60% in high-income countries. The survival rates for myeloproliferative diseases were the lowest in Eastern European countries. • The reported inferior survival rates were calculated based on outcome data of patients treated until 2007. The recent survival rates in Eastern European countries are unknown. What is New: • Being a small Eastern European country, Lithuania has experienced good economic growth during the last decade. We hypothesized that economic growth and gain of experience could result in better survival rates of children treated for cancer in our country in recent years. • A population-based analysis of treatment outcome of childhood AML treated in Lithuania in the recent years was performed for the first time. The survival rates of childhood AML in Lithuania are comparable to those of other high-income countries. Current survival rates of children treated for cancer in Eastern European countries could be comparable to the best current standards contributing to better European survival rates of childhood cancer in general.

Efficacy and Toxicity of Asparaginases During Prospective Drug Monitoring in Patients With Childhood Acute Lymphoblastic Leukemia

NARCIS (Netherlands)

W.H. Tong (Wing)

2014-01-01

markdownabstract__Abstract__ Intensified and effective asparaginase therapy is very important in modern treatment of childhood acute lymphoblastic leukemia. The use of native E.coli asparaginase in induction leads to a high rate of hypersensitivity reactions to PEGasparaginase in the

A case of non-specific interstitial pneumonia with recurrent gastric carcinoma and anti-Jo-1 antibody positive myositis.

Science.gov (United States)

Ebisutani, Chikara; Ito, Isao; Kitaichi, Masanori; Tanabe, Naoya; Mishima, Michiaki; Kadowaki, Seizo

2016-07-01

We report the first case of non-specific interstitial pneumonia (NSIP) in a patient with cancer-associated myositis (CAM) that emerged along with the recurrence of the cancer. A 60-year-old woman, with a history of partial gastrectomy for gastric cancer 11 years ago, presented with exertional dyspnea with anti-Jo-1 antibody-positive myositis. Surgical lung biopsy showed NSIP with metastatic gastric cancer. Accordingly, her condition was diagnosed as CAM with cancer recurrence. In patients with a history of cancer, development of myositis may indicate cancer recurrence; therefore, careful observation would be necessary. Copyright © 2016 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

Biological Therapy in Treating Patients With Advanced Myelodysplastic Syndrome, Acute or Chronic Myeloid Leukemia, or Acute Lymphoblastic Leukemia Who Are Undergoing Stem Cell Transplantation

Science.gov (United States)

2017-03-27

Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; Essential Thrombocythemia; Polycythemia Vera; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; T-cell Adult Acute Lymphoblastic Leukemia; T-cell Childhood Acute Lymphoblastic Leukemia

Acute necrotising encephalopathy of childhood after exanthema subitum outside Japan or Taiwan

Energy Technology Data Exchange (ETDEWEB)

Porto, L.; Lanferman, H.; Moeller-Hartmann, W.; Jacobi, G.; Zanella, F. [Inst. fuer Neuroradiologie, Klinikum der Johann Wolfgang Goethe-Univ., Frankfurt am Main (Germany)

1999-10-01

Acute necrotising encephalopathy of childhood (ANE) is an uncommon disease which predominantly affects infants and young children living in Japan and Taiwan. A multifocal encephalopathy with symmetrical lesions in the thalamus, tegmentum of the brain stem, cerebral periventricular white matter and cerebellar medulla is characteristic. We present the imaging features in a 4-year-old Japanese boy who had been living in Germany for 2{sup 1}/{sub 2} years before presentation. (orig.)

Secreted histidyl-tRNA synthetase splice variants elaborate major epitopes for autoantibodies in inflammatory myositis.

Science.gov (United States)

Zhou, Jie J; Wang, Feng; Xu, Zhiwen; Lo, Wing-Sze; Lau, Ching-Fun; Chiang, Kyle P; Nangle, Leslie A; Ashlock, Melissa A; Mendlein, John D; Yang, Xiang-Lei; Zhang, Mingjie; Schimmel, Paul

2014-07-11

Inflammatory and debilitating myositis and interstitial lung disease are commonly associated with autoantibodies (anti-Jo-1 antibodies) to cytoplasmic histidyl-tRNA synthetase (HisRS). Anti-Jo-1 antibodies from different disease-afflicted patients react mostly with spatially separated epitopes in the three-dimensional structure of human HisRS. We noted that two HisRS splice variants (SVs) include these spatially separated regions, but each SV lacks the HisRS catalytic domain. Despite the large deletions, the two SVs cross-react with a substantial population of anti-Jo-l antibodies from myositis patients. Moreover, expression of at least one of the SVs is up-regulated in dermatomyositis patients, and cell-based experiments show that both SVs and HisRS can be secreted. We suggest that, in patients with inflammatory myositis, anti-Jo-1 antibodies may have extracellular activity. © 2014 by The American Society for Biochemistry and Molecular Biology, Inc.

[The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis'

NARCIS (Netherlands)

Hoogendijk, J.E.; Bijlsma, J.W.J.; Engelen, B.G.M. van; Lindeman, E.J.M.; Royen-Kerkhof, A. van; Rie, M.A. de; Visser, M. de; Jennekens, F.G.I.

2005-01-01

This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a

Anti-TIF1-γ antibody and cancer-associated myositis: A clinicohistopathologic study.

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Hida, Ayumi; Yamashita, Takenari; Hosono, Yuji; Inoue, Manami; Kaida, Kenichi; Kadoya, Masato; Miwa, Yusuke; Yajima, Nobuyuki; Maezawa, Reika; Arai, Satoko; Kurasawa, Kazuhiro; Ito, Kazuhiro; Shimada, Hiroyuki; Iwanami, Tomoko; Sonoo, Masahiro; Hatanaka, Yuki; Murayama, Shigeo; Uchibori, Ayumi; Chiba, Atsuro; Aizawa, Hitoshi; Momoo, Takayuki; Nakae, Yoshiharu; Sakurai, Yasuhisa; Shiio, Yasushi; Hashida, Hideji; Yoshizawa, Toshihiro; Sakiyama, Yoshio; Oda, Aya; Inoue, Kiyoharu; Takeuchi, Sousuke; Iwata, Nobue K; Date, Hidetoshi; Masuda, Naoki; Mikata, Takashi; Motoyoshi, Yasufumi; Uesaka, Yoshikazu; Maeda, Meiko Hashimoto; Nakashima, Ran; Tsuji, Shoji; Kwak, Shin; Mimori, Tsuneyo; Shimizu, Jun

2016-07-19

We aimed to analyze the clinical and histopathologic features of cancer-associated myositis (CAM) in relation to anti-transcriptional intermediary factor 1 γ antibody (anti-TIF1-γ-Ab), a marker of cancer association. We retrospectively studied 349 patients with idiopathic inflammatory myopathies (IIMs), including 284 patients with pretreatment biopsy samples available. For the classification of IIMs, the European Neuromuscular Center criteria were applied. Patients with CAM with (anti-TIF1-γ-Ab[+] CAM) and without anti-TIF1-γ-Ab (anti-TIF1-γ-Ab[-] CAM) were compared with patients with IIM without cancers within and beyond 3 years of myositis diagnosis. Cancer was detected in 75 patients, of whom 36 (48%) were positive for anti-TIF1-γ-Ab. In anti-TIF1-γ-Ab(+) patients with CAM, cancers were detected within 1 year of myositis diagnosis in 35 (97%) and before 1 year of myositis diagnosis in 1. All the anti-TIF1-γ-Ab(+) patients with CAM satisfied the dermatomyositis (DM) criteria, including 2 possible DM sine dermatitis cases, and were characterized histologically by the presence of perifascicular atrophy, vacuolated fibers (VFs), and dense C5b-9 deposits on capillaries (dC5b-9). In contrast, 39 anti-TIF1-γ-Ab(-) patients with CAM were classified into various subgroups, and characterized by a higher frequency of necrotizing autoimmune myopathy (NAM). Notably, all 7 patients with CAM classified into the NAM subgroup were anti-TIF1-γ-Ab(-) and exhibited no dC5b-9 or VFs. CAM includes clinicohistopathologically heterogeneous disease entities. Among CAM entities, anti-TIF1-γ-Ab(+) CAM has characteristically shown a close temporal association with cancer detection and the histopathologic findings of dC5b-9 and VFs, and CAM with NAM is a subset of anti-TIF1-γ-Ab(-) CAM. © 2016 American Academy of Neurology.

Ophthalmic evaluation of long-term survivors of childhood acute lymphoblastic leukemia

International Nuclear Information System (INIS)

Weaver, R.G. Jr.; Chauvenet, A.R.; Smith, T.J.; Schwartz, A.C.

1986-01-01

Thirty-four long-term survivors of childhood acute lymphoblastic leukemia (ALL) underwent comprehensive ophthalmic examinations to detect retinopathy or other ocular sequelae. Sixteen of the 34 patients received whole brain radiation (greater than or equal to 2400 rad). All 18 patients in the non-radiated group had normal eye examinations, while 4 of 16 in the radiated group had ocular abnormalities. None of the ocular abnormalities could be definitely attributed to radiation and all patients had normal visual acuity. No radiation retinopathy was found in either group

Acute muscular weakness in children

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Ricardo Pablo Javier Erazo Torricelli

Full Text Available ABSTRACT Acute muscle weakness in children is a pediatric emergency. During the diagnostic approach, it is crucial to obtain a detailed case history, including: onset of weakness, history of associated febrile states, ingestion of toxic substances/toxins, immunizations, and family history. Neurological examination must be meticulous as well. In this review, we describe the most common diseases related to acute muscle weakness, grouped into the site of origin (from the upper motor neuron to the motor unit. Early detection of hyperCKemia may lead to a myositis diagnosis, and hypokalemia points to the diagnosis of periodic paralysis. Ophthalmoparesis, ptosis and bulbar signs are suggestive of myasthenia gravis or botulism. Distal weakness and hyporeflexia are clinical features of Guillain-Barré syndrome, the most frequent cause of acute muscle weakness. If all studies are normal, a psychogenic cause should be considered. Finding the etiology of acute muscle weakness is essential to execute treatment in a timely manner, improving the prognosis of affected children.

[Ocular myositis as a rare cause of vision loss].

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Rollnik, J D; Requadt, H

2017-04-01

Ocular myositis is a rare disease characterized by painful diplopia but loss of vision rarely occurs. The article reviews the literature focusing on the differential diagnostics. We report the case of an 80-year-old women suffering from slowly progressive loss of vision in the left eye. Diplopia was only present at the beginning and there was only moderate pain. Computed tomography and magnetic resonance imaging revealed a swelling of the left medial, lateral and inferior rectus muscles of the orbit leading to compression of the optic nerve in the orbital cone. An intravenous prednisolone stoss therapy (1000 mg per day for 3 consecutive days) was initiated, followed by oral medication of 100 mg per day then tapering over 10 weeks. Vision improved and no relapses were observed. Physicians should be aware of this rare disease to ensure quick diagnosis and treatment of ocular myositis.

Acute intrascrotal pathology in childhood: color Doppler study

International Nuclear Information System (INIS)

Cinta Sanguesa, C.; Muro, D.; Cortina, H.; Moreno, A.

1997-01-01

To asses the utility of color Doppler ultrasound in the study of acute intrascrotal pathology in childhood. Seventy-five boys with evidence of an acute intrascrotal abnormality were studied by means of color Doppler ultrasound. A 7.5 MHz linear transducer was used. The most common findings was inflammatory disease (60%) which was indicated by the presence of hyperemia in color Doppler. Of the nine boys with testicular torsion (12%) two presented extravaginal torsion, with a complete absence of intratesticular vascularization. Intravaginal torsion was observed in seven patients, six of whom presented reduced vascularization in the abnormal testicle with respect to the healthy testicle. There was one false negative in which testicular vascularization was normal but 360 degree centigree torsion was found at surgery. In addition, there were seven cases of hydatid torsion (9.33%), four of idiopathic scrotal swelling (5.3%). two cases of intermittent torsion (2.6%) and one case of Shcolein-Henoch purpura (1.3%). The ultrasound findings were completely nornal in seven boys. Color Doppler is a rapid, noninvasive method for assessing testicular blood flow. The elevated sensitivity and specificity (85.7 and 100%, respectively, in our series) for testicular torsion make Doppler ultrasound a highly reliable method for the study of this disorder. (Author) 18 refs

Fatal Tuberculous Myositis in an Immunocompromised Adult With Primary Sjögren's Syndrome

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Chi-Chang Huang

2010-09-01

Full Text Available Tuberculous myositis, which mimics rheumatic symptoms, is an extremely rare disease. Clinical ambiguity easily leads to misdiagnosis and delayed initial treatment. We present the case of a 55-year-old man who had primary Sjögren's syndrome and active cutaneous vasculitis treated with steroid and immunosuppressive drugs. He presented with a swollen, painful, hot left thigh. Although anti-tuberculosis medications were administered soon after a positive acid-fast stain of incisional muscular tissue, he died of rapidly progressive tuberculous myositis and multiorgan failure following 18 days of hospitalization. This case is presented to increase the awareness of this rare entity in clinical practice.

Childhood acute non-traumatic coma: aetiology and challenges in management in resource-poor countries of Africa and Asia.

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Gwer, Samson; Chacha, Clifford; Newton, Charles R; Idro, Richard

2013-08-01

This review examines the best available evidence on the aetiology of childhood acute non-traumatic coma in resource-poor countries (RPCs), discusses the challenges associated with management, and explores strategies to address them. Publications in English and French which reported on studies on the aetiology of childhood non-traumatic coma in RPCs are reviewed. Primarily, the MEDLINE database was searched using the keywords coma, unconsciousness, causality, aetiology, child, malaria cerebral, meningitis, encephalitis, Africa, Asia, and developing countries. 14 records were identified for inclusion in the review. Cerebral malaria (CM) was the commonest cause of childhood coma in most of the studies conducted in Africa. Acute bacterial meningitis (ABM) was the second most common known cause of coma in seven of the African studies. Of the studies in Asia, encephalitides were the commonest cause of coma in two studies in India, and ABM was the commonest cause of coma in Pakistan. Streptococcus pneumoniae was the most commonly isolated organism in ABM. Japanese encephalitis, dengue fever and enteroviruses were the viral agents most commonly isolated. Accurate diagnosis of the aetiology of childhood coma in RPCs is complicated by overlap in clinical presentation, limited diagnostic resources, disease endemicity and co-morbidity. For improved outcomes, studies are needed to further elucidate the aetiology of childhood coma in RPCs, explore simple and practical diagnostic tools, and investigate the most appropriate specific and supportive interventions to manage and prevent infectious encephalopathies.

Corticosteroids in Myositis and Scleroderma.

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Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

2016-02-01

Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients. Although CS can be effective first-line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. Published by Elsevier Inc.

Experimental myositis inducible with transfer of dendritic cells presenting a skeletal muscle C protein-derived CD8 epitope peptide.

Science.gov (United States)

Okiyama, Naoko; Hasegawa, Hisanori; Oida, Takatoku; Hirata, Shinya; Yokozeki, Hiroo; Fujimoto, Manabu; Miyasaka, Nobuyuki; Kohsaka, Hitoshi

2015-07-01

It is suggested that polymyositis, an autoimmune inflammatory myopathy, is mediated by autoaggressive CD8 T cells. Skeletal muscle C protein is a self-antigen that induces C protein-induced myositis, a murine model of polymyositis. To establish a new murine model of myositis inducible with a single CD8 T-cell epitope peptide that derives from the C protein, three internet-based prediction systems were employed to identify 24 candidate peptides of the immunogenic fragment of the C protein and bind theoretically to major histocompatibility complex class I molecules of C57BL/6 (B6) mice. RMA-S cell assay revealed that a HILIYSDV peptide, amino acid position 399-406 of the C protein, had the highest affinity to the H2-K(b) molecules. Transfer of mature bone marrow-derived dendritic cells pulsed with HILIYSDV induced myositis in naive B6 mice. This myositis was suppressed by anti-CD8-depleting antibodies but not by anti-CD4-depleting antibodies. Because this myositis model is mediated by CD8 T cells independently of CD4 T cells, it should be a useful tool to investigate pathology of polymyositis and develop therapies targeting CD8 T cells. © The Japanese Society for Immunology. 2015. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Constitutional sequence variation in the Fanconi anaemia group C (FANCC) gene in childhood acute myeloid leukaemia.

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Barber, Lisa M; McGrath, Helen E N; Meyer, Stefan; Will, Andrew M; Birch, Jillian M; Eden, Osborn B; Taylor, G Malcolm

2003-04-01

The extent to which genetic susceptibility contributes to the causation of childhood acute myeloid leukaemia (AML) is not known. The inherited bone marrow failure disorder Fanconi anaemia (FA) carries a substantially increased risk of AML, raising the possibility that constitutional variation in the FA (FANC) genes is involved in the aetiology of childhood AML. We have screened genomic DNA extracted from remission blood samples of 97 children with sporadic AML and 91 children with sporadic acute lymphoblastic leukaemia (ALL), together with 104 cord blood DNA samples from newborn children, for variations in the Fanconi anaemia group C (FANCC) gene. We found no evidence of known FANCC pathogenic mutations in children with AML, ALL or in the cord blood samples. However, we detected 12 different FANCC sequence variants, of which five were novel to this study. Among six FANCC variants leading to amino-acid substitutions, one (S26F) was present at a fourfold greater frequency in children with AML than in the cord blood samples (odds ratio: 4.09, P = 0.047; 95% confidence interval 1.08-15.54). Our results thus do not exclude the possibility that this polymorphic variant contributes to the risk of a small proportion of childhood AML.

Primary segmental omental infarction as a rare cause of acute abdominal pain in childhood

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N.F. Tepeneu

2018-01-01

Full Text Available Introduction: Primary omental infarction (POI has a low incidence worldwide, with most cases occurring in adults. This condition is rarely considered in the differential diagnosis of acute abdominal pain in childhood. Material and methods: We present 2 cases of omental infarction in an obese 8-year-old boy and a 5-year-old boy who presented with acute abdominal pain in the right abdomen. Both patients were initially treated with intravenous fluids and analgesics with no improvement. Abdominal ultrasound of the first patient showed free intraperitoneal fluid, meteorism and distended bowel loops. The appendix was not visualized. With a presumptive clinical diagnosis of appendicitis the child underwent laparotomy.On entering the peritoneal cavity an omental infarction was seen and a portion of the omentum was resected. Appendectomy was performed.The second patient presented with acute abdominal pain in the right upper quadrant, which started 2 days before. There was a history of possible abdominal trauma about 3 weeks earlier. The patient had repeated ultrasound examinations and a CT scan of the abdomen which showed a omental infarction. He underwent laparoscopy and resection of the omental infarction, as well as incidental appendectomy. Results: The postoperative period was uneventful. The first patient was discharged on day 3, the second patient on day 4 after surgery. Histology showed a normal vermiform appendix and an omental infarction in both cases. Conclusion and discussion: Since the omental infarction as etiology of acute abdominal pain is uncommon in children, we emphasize the importance of accurate diagnosis and appropriate treatment of omental infarction. Keywords: Primary segmental omental infarction (POI, Appendicitis, Childhood

Identification of de Novo Fanconi Anemia in Younger Patients With Newly Diagnosed Acute Myeloid Leukemia

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2016-05-13

Childhood Acute Erythroleukemia (M6); Childhood Acute Megakaryocytic Leukemia (M7); Childhood Acute Minimally Differentiated Myeloid Leukemia (M0); Childhood Acute Monoblastic Leukemia (M5a); Childhood Acute Monocytic Leukemia (M5b); Childhood Acute Myeloblastic Leukemia With Maturation (M2); Childhood Acute Myeloblastic Leukemia Without Maturation (M1); Childhood Acute Myelomonocytic Leukemia (M4); Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Fanconi Anemia; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Anemia With Ringed Sideroblasts; Secondary Myelodysplastic Syndromes; Untreated Childhood Acute Myeloid Leukemia and Other Myeloid Malignancies

MRI features of myositis ossificans with X-ray and CT findings

International Nuclear Information System (INIS)

Gu Xiang; Bai Rongjie; Qu Hui; Cheng Xiaoguang; Li Yuang

2009-01-01

Objective: To investigate the MRI features of myositis ossificans, and to address the correlation with X-ray radiography and CT findings. Methods: X-ray films, CT and MRI of 36 documented cases of myositis ossificans were retrospectively analyzed, and the literatures were reviewed. Results: Of the 36 cases, 4 cases occurred in the elbow joint, 4 in the shoulder joint, 15 in the hip joint, 6 in the tibiofibula, 5 in the femur, 1 in the metatarsal bones, and 1 in the ilium, respectively. Irregular patchy or lamellar high density calcification or ossification could be seen in the soft tissue parenchym on X-ray films and CT scan. Cortical bone integrity was preserved in diaphysis. CT enhanced scan showed that the swollen parenchyma was not enhanced and there was no parenchyma mass. On the early and middle stages, MR T 1 WI and T 2 WI showed slice-shaped low signal in the peripheral parenchyma, but patchy high signal was found around the low signal on T 2 WI. STIR showed mixed high and low signals in the swollen parenchyma with unclear demarcation. The lesions showed low signal on MR T 1 WI and T 2 WI in the late stage, and there was no edema in peripheral parenehyma. MRI enhanced scan found that the swollen parenchyma showed no enhancement in all stages. Conclusions: The imaging features of myositis ossificans have some characteristics. Misdiagnosis could be avoided when the disease was evaluated with the course. (authors)

Fetal Growth and Childhood Acute Lymphoblastic Leukemia: Findings from the Childhood Leukemia International Consortium (CLIC)

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Milne, Elizabeth; Greenop, Kathryn R.; Metayer, Catherine; Schüz, Joachim; Petridou, Eleni; Pombo-de-Oliveira, Maria S.; Infante-Rivard, Claire; Roman, Eve; Dockerty, John D.; Spector, Logan G.; Koifman, Sérgio; Orsi, Laurent; Rudant, Jérémie; Dessypris, Nick; Simpson, Jill; Lightfoot, Tracy; Kaatsch, Peter; Baka, Margarita; Faro, Alessandra; Armstrong, Bruce K.; Clavel, Jacqueline; Buffler, Patricia A.

2013-01-01

Positive associations have been reported between measures of accelerated fetal growth and risk of childhood acute lymphoblastic leukemia (ALL). We investigated this association by pooling individual-level data from 12 case-control studies participating in the Childhood Leukemia International Consortium. Two measures of fetal growth – weight-for-gestational-age and proportion of optimal birth weight (POBW) – were analysed. Study-specific odds ratios (ORs) and 95% confidence intervals (CIs) were estimated using multivariable logistic regression, and combined in fixed effects meta-analyses. Pooled analyses of all data were also undertaken using multivariable logistic regression. Subgroup analyses were undertaken when possible. Data on weight for gestational age were available for 7,348 cases and 12,489 controls from all 12 studies and POBW data were available for 1,680 cases and 3,139 controls from three studies. The summary ORs from the meta-analyses were 1.24 (95% CI 1.13, 1.36) for children who were large for gestational age relative to appropriate for gestational age, and 1.16 (95% CI: 1.09, 1.24) for a one standard deviation increase in POBW. The pooled analyses produced similar results. The summary and pooled ORs for small-for-gestational-age children were 0.83 (95% CI: 0.75, 0.92) and 0.86 (95% CI 0.77, 0.95) respectively. Results were consistent across subgroups defined by sex, ethnicity and immunophenotype, and when the analysis was restricted to children who did not have high birth weight. The evidence that accelerated fetal growth is associated with a modest increased risk of childhood ALL is strong and consistent with known biological mechanisms involving insulin like growth factors. PMID:23754574

Quantitative assessments of indoor air pollution and the risk of childhood acute leukemia in Shanghai

International Nuclear Information System (INIS)

Gao, Yu; Zhang, Yan; Kamijima, Michihiro; Sakai, Kiyoshi; Khalequzzaman, Md; Nakajima, Tamie; Shi, Rong; Wang, Xiaojin; Chen, Didi; Ji, Xiaofan; Han, Kaiyi; Tian, Ying

2014-01-01

We investigated the association between indoor air pollutants and childhood acute leukemia (AL). A total of 105 newly diagnosed cases and 105 1:1 gender-, age-, and hospital-matched controls were included. Measurements of indoor pollutants (including nitrogen dioxide (NO 2 ) and 17 types of volatile organic compounds (VOCs)) were taken with diffusive samplers for 64 pairs of cases and controls. Higher concentrations of NO 2 and almost half of VOCs were observed in the cases than in the controls and were associated with the increased risk of childhood AL. The use of synthetic materials for wall decoration and furniture in bedroom was related to the risk of childhood AL. Renovating the house in the last 5 years, changing furniture in the last 5 years, closing the doors and windows overnight in the winter and/or summer, paternal smoking history and outdoor pollutants affected VOC concentrations. Our results support the association between childhood AL and indoor air pollution. - Highlights: • We firstly assessed the effects of indoor air pollution on childhood AL in China. • Indoor air pollutants were assessed by questionnaire and quantitative measurements. • NO 2 and 17 types of VOCs were measured in bedrooms of both cases and controls. • Higher concentrations of indoor air pollutants increased the risk of childhood AL. • Indoor behavioral factors and outdoor pollution might affect indoor air pollution. - Higher concentrations of indoor air pollutants were related to an elevated risk of childhood AL

MINIMAL REQUIREMENTS FOR THE DIAGNOSIS, CLASSIFICATION, AND EVALUATION OF THE TREATMENT OF CHILDHOOD ACUTE LYMPHOBLASTIC-LEUKEMIA (ALL) IN THE BFM FAMILY COOPERATIVE GROUP

NARCIS (Netherlands)

VANDERDOESVANDENBERG, A; BARTRAM, CR; BASSO, G; BENOIT, YCM; BIONDI, A; DEBATIN, KM; HAAS, OA; HARBOTT, J; KAMPS, WA; KOLLER, U; LAMPERT, F; LUDWIG, WD; NIEMEYER, CM; VANWERING, ER

1992-01-01

Minimal requirements and their rationale for the diagnosis and the response to treatment in childhood acute lymphoblastic leukemia (ALL) were defined in the recently instituted "BFM-Family"-Group, in which the German, Austrian, Dutch, Italian, Belgian, French and Hungarian childhood leukemia study

Management of a femoral fracture complicated by clostridial myositis

International Nuclear Information System (INIS)

Thomson, M.J.; Eger, C.E.

1997-01-01

A clinical case of clostridial myositis secondary to a comminuted femoral fracture is described. This case is unusual because, despite the severe degree of obvious muscle necrosis and gas production, the dog had minimal signs of systemic toxicity. Union of the fracture was achieved but six months postoperatively muscular contracture had resulted in permanent stifle extension

Risk-Based Classification System of Patients With Newly Diagnosed Acute Lymphoblastic Leukemia

Science.gov (United States)

2018-02-22

Adult B Acute Lymphoblastic Leukemia; Adult T Acute Lymphoblastic Leukemia; Childhood B Acute Lymphoblastic Leukemia; Childhood T Acute Lymphoblastic Leukemia; Untreated Adult Acute Lymphoblastic Leukemia; Untreated Childhood Acute Lymphoblastic Leukemia

Visual Attention and Math Performance in Survivors of Childhood Acute Lymphoblastic Leukemia.

Science.gov (United States)

Richard, Annette E; Hodges, Elise K; Heinrich, Kimberley P

2018-01-24

Attentional and academic difficulties, particularly in math, are common in survivors of childhood acute lymphoblastic leukemia (ALL). Of cognitive deficits experienced by survivors of childhood ALL, attention deficits may be particularly responsive to intervention. However, it is unknown whether deficits in particular aspects of attention are associated with deficits in math skills. The current study investigated relationships between math calculation skills, performance on an objective measure of sustained attention, and parent- and teacher-reported attention difficulties. Twenty-four survivors of childhood ALL (Mage = 13.5 years, SD= 2.8 years) completed a computerized measure of sustained attention and response control and a written measure of math calculation skills in the context of a comprehensive clinical neuropsychological evaluation. Parent and teacher ratings of inattention and impulsivity were obtained. Visual response control and visual attention accounted for 26.4% of the variance observed among math performance scores after controlling for IQ (p < .05). Teacher-rated, but not parent-rated, inattention was significantly negatively correlated with math calculation scores. Consistency of responses to visual stimuli on a computerized measure of attention is a unique predictor of variance in math performance among survivors of childhood ALL. Objective testing of visual response control, rather than parent-rated attentional problems, may have clinical utility in identifying ALL survivors at risk for math difficulties. © The Author(s) 2018. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Adult bacterial myositis: report of a single-center series of 26 cases

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Fernando Gallucci

2016-09-01

Full Text Available Bacterial infections involving muscle are quite uncommon and generally require specific predisposing factors. Bacterial myositis is more rarely described in the typical kind of patients observed in Internal Medicine (presence of multiple co-morbidities, partial/limited immune-deficiency, advanced age. Twenty-six patients suffering from bacterial myositis (8 women and 18 men; mean age 58.5 years, range 27-82 observed in a single Internal Medicine Unit were reported. Muscles involved were ileopsoas, thigh, paravertebral, gluteus, calf, forearm and rectus abdomen. Simultaneous presence of arthritis was registered in 17 patients and all patients presented relevant comorbidity. Main cultured bacteria were Staphylococcus aureus, Escherichia coli, other Gram-negative bacteria, Streptococcus spp. Multi-drug-resistance was observed in 14 out 26 (53.8%. Computed tomography, ultrasound and magnetic resonance imaging were utilized for diagnostic purposes. Antibiotic treatment was administered to all patients. Surgical debridement and drainage were performed in 12 patients; 7 patients were treated with percutaneous aspiration and drainage. At discharge, relevant functional impairment was present in 17 patients (65.3%. Four patients died (in-hospital mortality 7.6%, global mortality at three months 15.3%. Management of bacterial myositis is difficult and its prognosis is poor. In the near future, this demanding infection will be more frequently observed in Internal Medicine setting as comorbidity, which is very often the main characteristic of these patients.

INCLUSION BODY MYOSITIS

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Luh Yeni Laksmini

2014-09-01

Full Text Available Inclusion body myositis (IBM merupakan penyakit inflamasi pada otot yang bersifat progresif dengan penyebab yang tidak diketahui dan tidak menunjukkan respon yang baik terhadap berbagai terapi. Gambaran histopatologi IBM ditandai dengan infiltrat sel-sel limfosit diantara ruangan endomisial, di dalam otot dan di sekitar otot dengan fokus-fokus inklusi di dalam miosit (rimmed vacuole serta beberapa serat otot terlihat atrofi dan nekrosis. Dilaporkan wanita, usia 46 tahun dengan IBM. Keluhan utama pasien berupa kelemahan pada kedua tangan, kaki kanan terasa berat jika diangkat sehingga susah berjalan. Pemeriksaan saraf sensorik ekstremitas dekstra dan sinistra dalam batas normal. Pemeriksaan enzim cretinine kinase meningkat secara dramatik. Pemeriksaan histopatologi dari biospi otot gastrocnemius menunjukkan gambaran yang sesuai untuk IBM dan telah dilakukan penanganan dengan pemberian oral methilprednisolon 3x32 mg dan mecobalmin 1x500ìg intravena, namun tidak menunjukkan respon yang baik terhadap terapi dan akhirnya pasien meninggal. [MEDICINA 2013;44:118-123].

Physical function and muscle strength in sporadic inclusion body myositis

DEFF Research Database (Denmark)

Jørgensen, Anders N; Aagaard, Per; Nielsen, Jakob L

2017-01-01

INTRODUCTION: In this study, self-reported physical function, functional capacity, and isolated muscle function were investigated in sporadic inclusion body myositis (sIBM) patients. METHODS: The 36-item Short Form (SF-36) Health Survey and 2-min walk test (2MWT), timed up & go test (TUG), and 30-s...

Tubercular myositis of infraspinatus: a rare clinical entity

OpenAIRE

Vikas Verma; Yogesh Kumar Yadav; Anuj Rastogi; Farid Mohammed

2016-01-01

Tuberculosis of the musculoskeletal system is generally confined to bones and joints. The surrounding soft tissue is secondarily infected. Tuberculous bursitis, tenosynovitis and primary pyomyositis are rarer manifestations of the disease. Of these, primary tuberculouspyomyositis is probably the rarest entity. We report a case of tubercular myositis of infraspinatus in an 8 year-old female who presented with pain, low grade fever, weight loss, anorexia, progressively increasing pain in the sc...

Proliferative myositis in a patient with AIDS

International Nuclear Information System (INIS)

Wlachovska, B.; Deux, J.F.; Marsault, C.; Le Breton, C.; Abraham, B.; Sibony, M.

2004-01-01

We report a case of proliferative myositis in the right biceps of a 56-year-old man with acquired immune deficiency syndrome (AIDS). Imaging methods included sonography, computed tomography and magnetic resonance imaging. The diagnosis was made by a core-cut biopsy and fine needle aspiration biopsy with immunohistochemical analysis. The lesion disappeared after 2 months without treatment. It is particularly important to determine whether intramuscular masses arising in patients with AIDS are due to an infectious or malignant process. (orig.)

Myositis ossificans imaging: keys to successful diagnosis

International Nuclear Information System (INIS)

Lacout, Alexis; Jarraya, Mohamed; Marcy, Pierre-Yves; Thariat, Juliette; Carlier, Robert Yves

2012-01-01

Myositis ossificans (MO) is an inflammatory pseudotumor of the muscle that may be mistaken clinically and even histologically for a malignant soft tissue tumor. The aim of this article is to report the imaging characteristics of MO, the emphasis being on the early diagnostic clues. USG can be used at an early stage to reveal the ‘zone phenomenon,’ which is highly suggestive of MO. A short course of nonsteroidal anti-inflammatory drug therapy may be an efficient treatment for early MO

Proliferative myositis in a patient with AIDS

Energy Technology Data Exchange (ETDEWEB)

Wlachovska, B.; Deux, J.F.; Marsault, C.; Le Breton, C. [Department of Radiology, Hopital Tenon, 4 rue de la Chine, 75020, Paris (France); Abraham, B. [Department of Tropical and Infectious Diseases, Hopital Tenon, Paris (France); Sibony, M. [Department of Anatomy, Hopital Tenon, Paris (France)

2004-04-01

We report a case of proliferative myositis in the right biceps of a 56-year-old man with acquired immune deficiency syndrome (AIDS). Imaging methods included sonography, computed tomography and magnetic resonance imaging. The diagnosis was made by a core-cut biopsy and fine needle aspiration biopsy with immunohistochemical analysis. The lesion disappeared after 2 months without treatment. It is particularly important to determine whether intramuscular masses arising in patients with AIDS are due to an infectious or malignant process. (orig.)

The Immune Response and the Pathogenesis of Idiopathic Inflammatory Myositis: a Critical Review.

Science.gov (United States)

Ceribelli, Angela; De Santis, Maria; Isailovic, Natasa; Gershwin, M Eric; Selmi, Carlo

2017-02-01

The pathogenesis of idiopathic inflammatory myositis (IIMs, including polymyositis and dermatomyositis) remains largely enigmatic, despite advances in the study of the role played by innate immunity, adaptive immunity, genetic predisposition, and environmental factors in an orchestrated response. Several factors are involved in the inflammatory state that characterizes the different forms of IIMs which share features and mechanisms but are clearly different with respect to the involved sites and characteristics of the inflammation. Cellular and non-cellular mechanisms of both the immune and non-immune systems have been identified as key regulators of inflammation in polymyositis/dermatomyositis, particularly at different stages of disease, leading to the fibrotic state that characterizes the end stage. Among these, a special role is played by an interferon signature and complement cascade with different mechanisms in polymyositis and dermatomyositis; these differences can be identified also histologically in muscle biopsies. Numerous cellular components of the adaptive and innate immune response are present in the site of tissue inflammation, and the complexity of idiopathic inflammatory myositis is further supported by the involvement of non-immune mechanisms such as hypoxia and autophagy. The aim of this comprehensive review is to describe the major pathogenic mechanisms involved in the onset of idiopathic inflammatory myositis and to report on the major working hypothesis with therapeutic implications.

New Myositis Classification Criteria-What We Have Learned Since Bohan and Peter.

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Leclair, Valérie; Lundberg, Ingrid E

2018-03-17

Idiopathic inflammatory myopathy (IIM) classification criteria have been a subject of debate for many decades. Despite several limitations, the Bohan and Peter criteria are still widely used. The aim of this review is to discuss the evolution of IIM classification criteria. New IIM classification criteria are periodically proposed. The discovery of myositis-specific and myositis-associated autoantibodies led to the development of clinico-serological criteria, while in-depth description of IIM morphological features improved histopathology-based criteria. The long-awaited European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) IIM classification criteria were recently published. The Bohan and Peter criteria are outdated and validated classification criteria are necessary to improve research in IIM. The new EULAR/ACR IIM classification criteria are thus a definite improvement and an important step forward in the field.

MR imaging and ultrasonography findings of early myositis ossificans: a case report

International Nuclear Information System (INIS)

Lee, Kyung Ryeol; Park, So Young; Jin, Wook; Won, Kyu Yeoun

2016-01-01

Myositis ossificans (MO) is a benign soft tissue lesion with non-neoplastic heterotopic bone formation. MO in the intermediate and mature stages can be easily diagnosed if characteristic imaging findings such as a peripheral zonal pattern of ossification with variable thickness is observed. However, it is difficult to correctly diagnose early MO because it can mimic malignancy clinically, radiologically, and histopathologically. We report a case of early pseudosarcomatous phase of non-traumatic MO with atypical imaging findings. A 59-year-old woman presented with pain followed by a mass in the left thigh within a week. MR imaging and ultrasonography showed an intramuscular lesion with preserved muscle fascicles in the vastus lateralis muscle. Intralesional ossification or calcification was not seen on ultrasonography. A diagnosis of myositis ossificans was made by ultrasonographically guided biopsy. (orig.)

MR imaging and ultrasonography findings of early myositis ossificans: a case report

Energy Technology Data Exchange (ETDEWEB)

Lee, Kyung Ryeol [Jeju National University Hospital, Department of Radiology, Jeju-si, Jeju Special Self-Governing Province (Korea, Republic of); Park, So Young; Jin, Wook [Kyung Hee University Hospital at Gangdong, Department of Radiology, Seoul (Korea, Republic of); Won, Kyu Yeoun [Kyung Hee University Hospital at Gangdong, Department of Pathology, Seoul (Korea, Republic of)

2016-10-15

Myositis ossificans (MO) is a benign soft tissue lesion with non-neoplastic heterotopic bone formation. MO in the intermediate and mature stages can be easily diagnosed if characteristic imaging findings such as a peripheral zonal pattern of ossification with variable thickness is observed. However, it is difficult to correctly diagnose early MO because it can mimic malignancy clinically, radiologically, and histopathologically. We report a case of early pseudosarcomatous phase of non-traumatic MO with atypical imaging findings. A 59-year-old woman presented with pain followed by a mass in the left thigh within a week. MR imaging and ultrasonography showed an intramuscular lesion with preserved muscle fascicles in the vastus lateralis muscle. Intralesional ossification or calcification was not seen on ultrasonography. A diagnosis of myositis ossificans was made by ultrasonographically guided biopsy. (orig.)

Epidemiology of inclusion body myositis in the Netherlands : A nationwide study

NARCIS (Netherlands)

Badrising, UA; Maat-Schieman, M; van Duinen, SG; Breedveld, F; van Doorn, P; van Engelen, B; van den Hoogen, F; Hoogendijk, J; Howeler, C; de Jager, A; Jennekens, F; Koehler, P; van der Leeuw, H; de Visser, M; Verschuuren, JJ; Wintzen, AR

2000-01-01

Epidemiologic data on inclusion body myositis (IBM) are scarce, and possibly biased, because they are derived from larger neuromuscular centers. The present nationwide collaborative cross-sectional study, which culminated on July 1, 1999, resulted in identification of 76 patients with IBM and the

The EuroMyositis registry: an international collaborative tool to facilitate myositis research.

Science.gov (United States)

Lilleker, James B; Vencovsky, Jiri; Wang, Guochun; Wedderburn, Lucy R; Diederichsen, Louise Pyndt; Schmidt, Jens; Oakley, Paula; Benveniste, Olivier; Danieli, Maria Giovanna; Danko, Katalin; Thuy, Nguyen Thi Phuong; Vazquez-Del Mercado, Monica; Andersson, Helena; De Paepe, Boel; deBleecker, Jan L; Maurer, Britta; McCann, Liza J; Pipitone, Nicolo; McHugh, Neil; Betteridge, Zoe E; New, Paul; Cooper, Robert G; Ollier, William E; Lamb, Janine A; Krogh, Niels Steen; Lundberg, Ingrid E; Chinoy, Hector

2018-01-01

The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%). Smoking was more frequent in connective tissue disease overlap cases (45%, OR 1.44, 95% CI 1.09 to 1.90, p=0.012). Smoking was associated with interstitial lung disease (ILD) (OR 1.32, 95% CI 1.06 to 1.65, p=0.013), dysphagia (OR 1.43, 95% CI 1.16 to 1.77, p=0.001), malignancy ever (OR 1.78, 95% CI 1.36 to 2.33, p<0.001) and cardiac involvement (OR 2.40, 95% CI 1.60 to 3.60, p<0.001).Dysphagia occurred in 39% and cardiac involvement in 9%; either occurrence was associated with higher Health Assessment Questionnaire (HAQ) scores (adjusted OR 1.79, 95% CI 1.43 to 2.23, p<0.001). HAQ scores were also higher in inclusion body myositis cases (adjusted OR 3.85, 95% CI 2.52 to 5.90, p<0.001). Malignancy (ever) occurred in 13%, most commonly in DM (20%, OR 2.06, 95% CI 1.65 to 2.57, p<0.001).ILD occurred in 30%, most frequently in antisynthetase syndrome (71%, OR 10.7, 95% CI 8.6 to 13.4, p<0.001). Rash characteristics differed between adult-onset and juvenile-onset DM cases ('V' sign: 56% DM vs 16% juvenile-DM, OR 0.16, 95% CI 0.07 to 0.36, p<0.001). Glucocorticoids were used in 98% of cases, methotrexate in 71% and azathioprine in 51%. This large multicentre cohort demonstrates the importance of extramuscular involvement in patients with IIM, its association with smoking and its influence on disease severity. Our findings emphasise that IIM is a multisystem inflammatory disease and will help inform prognosis and clinical management of patients. © Article author(s) (or their employer(s) unless otherwise stated

Polymorphisms in the ABCB1 gene and effect on outcome and toxicity in childhood acute lymphoblastic leukemia

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Gregers, J; Gréen, H; Christensen, I J

2015-01-01

The membrane transporter P-glycoprotein, encoded by the ABCB1 gene, influences the pharmacokinetics of anti-cancer drugs. We hypothesized that variants of ABCB1 affect outcome and toxicity in childhood acute lymphoblastic leukemia (ALL). We studied 522 Danish children with ALL, 93% of all those e...

Anti-synthetase syndrome associated with anti PL-12 and anti-Signal recognition particle antibodies and a necrotizing auto-immune myositis.

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Malkan, Ashish; Cappelen-Smith, Cecilia; Beran, Roy; Griffith, Neil; Toong, Catherine; Wang, Min-Xia; Cordato, Dennis

2015-02-01

We report a 37-year-old woman with a 2 month history of proximal muscle weakness and extremely high creatine kinase (21,808 U/L) due to necrotizing auto-immune myositis (NAM) in association with anti-synthetase syndrome. Myositis-specific auto-immune antibody panel was positive for anti-Signal recognition particle and anti-PL-12. CT scan of the chest confirmed interstitial lung disease. Prednisolone, intravenous immunoglobulin and cyclophosphamide therapy was given with gradual improvement. This patient is notable for the unusual combination of NAM and anti-synthetase syndrome with the rare finding of two myositis-specific autoantibodies, which directed testing for associated extramuscular features and management with more aggressive immunotherapy. Copyright © 2014 Elsevier Ltd. All rights reserved.

The role of tumor necrosis factor-α-related apoptosis-inducing ligand (TRAIL) in mediating autophagy in myositis skeletal muscle: A potential non-immune mechanism of muscle damage

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Alger, Heather M.; Raben, Nina; Pistilli, Emidio; Francia, Dwight; Rawat, Rashmi; Getnet, Derese; Ghimbovschi, Svetlana; Chen, Yi-Wen; Lundberg, Ingrid E.; Nagaraju, Kanneboyina

2011-01-01

Objective Multinucleated cells are relatively resistant to classical apoptosis, and the factors initiating cell-death and damage in myositis are not well defined. We hypothesized that non-immune autophagic cell death may play a role in muscle fiber damage. Recent literature indicates that tumor necrosis factor-alpha-related apoptosis inducing ligand (TRAIL) may induce both NFκB (nuclear factor kappa-light chain enhancer of activated B cells) activation and autophagic cell death in other systems. Here, we have investigated its role in cell death and pathogenesis in vitro and in vivo using myositis (human and mouse) muscle tissues. Methods Gene expression profiling indicated that expression of TRAIL and several autophagy markers was specifically upregulated in myositis muscle tissue; these results were confirmed by immunohistochemistry and immunoblotting. We also analyzed TRAIL-induced cell death (apoptosis and autophagy) and NFκB activation in vitro in cultured cells. Results TRAIL was expressed predominantly in muscle fibers of myositis, but not in biopsies from normal or other dystrophic-diseased muscle. Autophagy markers were upregulated in human and mouse models of myositis. TRAIL expression was restricted to regenerating/atrophic areas of muscle fascicles, blood vessels, and infiltrating lymphocytes. TRAIL induced NFκB activation and IκB degradation in cultured cells that are resistant to TRAIL-induced apoptosis but undergo autophagic cell death. Conclusion Our data demonstrate that TRAIL is expressed in myositis muscle and may mediate both activation of NFκB and autophagic cell death in myositis. Thus, this non-immune pathway may be an attractive target for therapeutic intervention in myositis. PMID:21769834

Patient-reported outcomes and adult patients' disease experience in the idiopathic inflammatory myopathies. report from the OMERACT 11 Myositis Special Interest Group.

Science.gov (United States)

Alexanderson, Helene; Del Grande, Maria; Bingham, Clifton O; Orbai, Ana-Maria; Sarver, Catherine; Clegg-Smith, Katherine; Lundberg, Ingrid E; Song, Yeong Wook; Christopher-Stine, Lisa

2014-03-01

The newly formed Outcome Measures in Rheumatology (OMERACT) Myositis Special Interest Group (SIG) was established to examine patient-reported outcome measures (PROM) in myositis. At OMERACT 11, a literature review of PROM used in the idiopathic inflammatory myopathies (IIM) and other neuromuscular conditions was presented. The group examined in more detail 2 PROM more extensively evaluated in patients with IIM, the Myositis Activities Profile, and the McMaster-Toronto Arthritis Patient Preference Disability Questionnaire, through the OMERACT filter of truth, discrimination, and feasibility. Preliminary results from a qualitative study of patients with myositis regarding their symptoms were discussed that emphasized the range of symptoms experienced: pain, physical tightness/stiffness, fatigue, disease effect on emotional life and relationships, and treatment-related side effects. Following discussion of these results and following additional discussions since OMERACT 11, a research agenda was developed. The next step in evaluating PROM in IIM will require additional focus groups with a spectrum of patients with different myositis disease phenotypes and manifestations across a range of disease activity, and from multiple international settings. The group will initially focus on dermatomyositis and polymyositis in adults. Qualitative analysis will facilitate the identification of commonalities and divergent patient-relevant aspects of disease, insights that are critical given the heterogeneous manifestations of these diseases. Based on these qualitative studies, existing myositis PROM can be examined to more thoroughly assess content validity, and will be important to identify gaps in domain measurement that will be required to develop a preliminary core set of patient-relevant domains for IIM.

The impact of CYP3A5*3 on risk and prognosis in childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Borst, Louise; Wallerek, Sandra; Dalhoff, Kim

2011-01-01

Objectives: Acute lymphoblastic leukemia (ALL) is the most common cancer in childhood; however, little is known of the molecular etiology and environmental exposures causing the disease. Cytochrome P450 3A5 (CYP3A5) plays a crucial role in the catalytic oxidation of endogenous metabolites and toxic...

The impact of CYP3A5*3 on risk and prognosis in childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Borst, Louise; Wallerek, Sandra; Dalhoff, Kim Peder

2011-01-01

Objectives:  Acute lymphoblastic leukemia (ALL) is the most common cancer in childhood; however, little is known of the molecular etiology and environmental exposures causing the disease. Cytochrome P450 3A5 (CYP3A5) plays a crucial role in the catalytic oxidation of endogenous metabolites...

Late effects of treatment in survivors of childhood acute lymphoblastic leukaemia

International Nuclear Information System (INIS)

Roux, P.

1987-01-01

The overall aim of this study was a comprehensive assessment of the nature and severity of the late effects of treatment in a group of children surviving acute lymphoblastic leukaemia. In the absence of damage preceding treatment, late effects could be ascribed to treatment. Cranial irradiation, methotrexate, L-asparaginase and cytosine arabinoside are therapeutic modalities most likely to cause injury to the central nervous system. Survivors of childhood leukaemia also showed an increase in weight-for-height during and after therapy which appeared to be the consequence of a loss in statural growth as well as increasing weight-for-age. Assessment of endocrine function in leukaemia survivors indicated abnormalities in the regulation of growth hormone and thyroid stimulating hormone in some patients. Survivors of childhood leukaemia were shown to have an intellectual deficit compared with their siblings and a high incidence of visual-perceptual defects. The intellectual effects of lower doses of cranial irradiation are as yet unknown. A variety of minor neurological abnormalities were detected among leukaemia survivors and thought to be related to preceding central nervous system 'prophylactic' chemotherapy and irradiation. A new instrument, the functional deficit score, was derived to reflect overall outcome in survivors of childhood leukaemia. With few exceptions, leukaemia survivors in this study had received 2400 rads of deep x-ray therapy as cranial irradiation. This dosage has since been reduced world-wide. Current cranial irradiation 'prophylaxis' consists of 1800 rad of megavoltage radiotherapy

Late effects of treatment in survivors of childhood acute lymphoblastic leukaemia

Energy Technology Data Exchange (ETDEWEB)

Roux, P

1987-01-01

The overall aim of this study was a comprehensive assessment of the nature and severity of the late effects of treatment in a group of children surviving acute lymphoblastic leukaemia. In the absence of damage preceding treatment, late effects could be ascribed to treatment. Cranial irradiation, methotrexate, L-asparaginase and cytosine arabinoside are therapeutic modalities most likely to cause injury to the central nervous system. Survivors of childhood leukaemia also showed an increase in weight-for-height during and after therapy which appeared to be the consequence of a loss in statural growth as well as increasing weight-for-age. Assessment of endocrine function in leukaemia survivors indicated abnormalities in the regulation of growth hormone and thyroid stimulating hormone in some patients. Survivors of childhood leukaemia were shown to have an intellectual deficit compared with their siblings and a high incidence of visual-perceptual defects. The intellectual effects of lower doses of cranial irradiation are as yet unknown. A variety of minor neurological abnormalities were detected among leukaemia survivors and thought to be related to preceding central nervous system 'prophylactic' chemotherapy and irradiation. A new instrument, the functional deficit score, was derived to reflect overall outcome in survivors of childhood leukaemia. With few exceptions, leukaemia survivors in this study had received 2400 rads of deep x-ray therapy as cranial irradiation. This dosage has since been reduced world-wide. Current cranial irradiation 'prophylaxis' consists of 1800 rad of megavoltage radiotherapy.

Cytogenetic Profile and Gene Mutations of Childhood Acute Lymphoblastic Leukemia

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Nawaf Alkhayat

2017-07-01

Full Text Available Background: Childhood acute lymphoblastic leukemia (ALL is characterized by recurrent genetic aberrations. The identification of those abnormalities is clinically important because they are considered significant risk-stratifying markers. Aims: There are insufficient data of cytogenetic profiles in Saudi Arabian patients with childhood ALL leukemia. We have examined a cohort of 110 cases of ALL to determine the cytogenetic profiles and prevalence of FLT3 mutations and analysis of the more frequently observed abnormalities and its correlations to other biologic factors and patient outcomes and to compare our results with previously published results. Materials and methods: Patients —We reviewed all cases from 2007 to 2016 with an established diagnosis of childhood ALL. Of the 110 patients, 98 were B-lineage ALL and 12 T-cell ALL. All the patients were treated by UKALL 2003 protocol and risk stratified according previously published criteria. Cytogenetic analysis —Chromosome banding analysis and fluorescence in situ hybridization were used to detect genetic aberrations. Analysis of FLT3 mutations —Bone marrow or blood samples were screened for FLT3 mutations (internal tandem duplications, and point mutations, D835 using polymerase chain reaction methods. Result: Cytogenetic analysis showed chromosomal anomalies in 68 out of 102 cases with an overall incidence 66.7%. The most frequent chromosomal anomalies in ALL were hyperdiploidy, t(9;22, t(12;21, and MLL gene rearrangements. Our data are in accordance with those published previously and showed that FLT3 mutations are not common in patients with ALL (4.7% and have no prognostic relevance in pediatric patients with ALL. On the contrary, t(9;22, MLL gene rearrangements and hypodiploidy were signs of a bad prognosis in childhood ALL with high rate of relapse and shorter overall survival compared with the standard-risk group ( P  = .031.The event-free survival was also found to be worse ( P

CT in idiopathic pyogenic myositis of the iliopsoas muscle

International Nuclear Information System (INIS)

Kvernebo, K.; Stiris, G.; Haaland, M.; Aker Sykehus, Oslo; Buskerud Country Hospital

1983-01-01

Pyogenic myositis of the iliopsoas muscle may occur as a primary clinical entity of an idiopathie nature, or more commonly secondarily to an adjacent disease process. We report 2 cases of idiopathic pyogenic infection caused by Staphylococcus aureus. This disease entity is rare in temperate climates. CT combined with clinical and biochemical information enabled the correct diagnosis, and appropriate treatment could thus be started. (orig.)

The clinical phenotype associated with myositis-specific and associated autoantibodies: a meta-analysis revisiting the so-called antisynthetase syndrome.

Science.gov (United States)

Lega, Jean-Christophe; Fabien, Nicole; Reynaud, Quitterie; Durieu, Isabelle; Durupt, Stéphane; Dutertre, Marine; Cordier, Jean-François; Cottin, Vincent

2014-09-01

To describe the clinical spectrum associated with aminoacyl-transfer RNA synthetase (ARS) autoantibodies in patients with idiopathic inflammatory myositis defined according to Peter and Bohan's criteria. Cohort studies were selected from MEDLINE and Embase up to August 2013. Two investigators independently extracted data on study design, patient characteristics, and clinical features (interstitial lung disease [ILD], fever, mechanic's hands [MH], Raynaud's phenomenon [RPh], arthralgia, sclerodactyly, cancer and dermatomyositis-specific rash) according to the presence of myositis-specific (anti-aminoacyl-transfer RNA synthetase [ARS], anti-signal recognition particle [anti-SRP] and anti-Mi2) and myositis-associated (anti-PM/Scl, anti-U1-RNP and anti-Ku) autoantibodies. 27 studies (3487 patients) were included in the meta-analysis. Arthralgia (75%, CI 67-81) and ILD (69%, CI 63-74) were the most prevalent clinical signs associated with anti-ARS autoantibodies. Anti-Mi2 and anti-SRP autoantibodies were associated with few extramuscular signs. ARS autoantibodies were identified in 13% of patients with cancer-associated myositis (5-25). Patients with non-anti-Jo1 ARS had greater odds of presenting fever (RR 0.63, CI 0.52-0.90) and ILD (RR 0.87, CI 0.81-0.93) compared to those with anti-Jo1 autoantibodies. The frequencies of myositis (RR 1.60, CI 1.38-1.85), arthralgia (RR 1.52, CI 1.32-1.76) and MH (RR 1.47, CI 1.11-1.94) were almost 50% higher in patients with anti-Jo1 compared to non-anti-Jo1 ARS autoantibodies. Patients with anti-PM/Scl differed from those with anti-ARS autoantibodies by a greater prevalence of RPh (RR 0.70, CI 0.53-0.94) and sclerodactyly (RR 0.47, CI 0.25-0.89). ILD was less frequent in patients with anti-U1-RNP autoantibodies (RR 3.35, CI 1.07-10.43). No difference was observed between anti-ARS and myositis-associated autoantibodies for other outcomes. The presence of anti-ARS autoantibodies delimits a heterogeneous subset of patients with a high

Statin-induced autoimmune necrotizing myositis

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Katarzyna Ząber

2016-02-01

Full Text Available Myositides comprise a large group of disorders involving limb muscle weakness. In differential diagnosis we have to consider idiopathic myositides, myositides associated with other diseases, and those induced by external factors, e.g. drug-induced. Statins are commonly used drugs, but many patients experience a broad spectrum of adverse effects including symptoms from skeletal muscle. Physicians should pay special attention to patients reporting muscle weakness lasting longer than 12 weeks, despite statin withdrawal, as well as other symptoms: dysphagia, disturbed grip function, elevated creatinine kinase (CK levels and abnormal electromyography. The reported case deals with the problem of differential diagnosis of drug-induced muscle injury, polymyositis with a recently reported myopathy – statin-induced autoimmune necrotizing myositis, related to anti-HMGCR antibodies.

Acute necrotizing encephalopathy of childhood: a fatal complication of swine flu

International Nuclear Information System (INIS)

Khan, M.R.; Maheshwari, P.K.; Haque, A.

2010-01-01

Acute necrotizing encephalopathy of childhood (ANEC) is a rare condition characterized by the presence of multifocal symmetrical brain lesions involving mainly thalami, brainstem, cerebellum and white matter. ANEC is a serious and life threatening complication of simple viral infections. We present a case of a young child who developed this condition with classical clinical and radiological findings consistent with ANEC, secondary to swine flu (H1N1). He needed ventilatory support and had profound motor and intellectual deficit on discharge. We report this case with aim of raising awareness about this fatal complication of swine flu which has become a global health care issue these days. (author)

Risk factors for treatment related mortality in childhood acute lymphoblastic leukaemia

DEFF Research Database (Denmark)

Lund, Bendik; Åsberg, Ann; Heyman, Mats

2011-01-01

-cell disease (HR: 1.9, 95% CI: 1.01-3.7), Down syndrome (HR: 7.3, 95% CI: 3.6-14.9) and haematopoietic stem cell transplantation in CR1 (HR: 8.0, 95% CI: 3.3-19.5) were identified as independent risk factors for TRD. CONCLUSION: Several TRDs were potentially preventable and future efforts should be directed......BACKGROUND: In spite of major improvements in the cure rate of childhood acute lymphoblastic leukaemia (ALL), 2-4% of patients still die from treatment related complications. PROCEDURE: We investigated the pattern of treatment related deaths (TRDs) and possible risk factors in the NOPHO ALL-92...

DNA incorporation of 6-thioguanine nucleotides during maintenance therapy of childhood acute lymphoblastic leukaemia and non-Hodgkin lymphoma

DEFF Research Database (Denmark)

Hedeland, Rikke L; Hvidt, Kristian; Nersting, Jacob

2010-01-01

To explore the DNA incorporation of 6-thioguanine nucleotide levels (DNA-6TGN) during 6-mercaptopurine (6MP) therapy of childhood acute lymphoblastic leukaemia (ALL) and non-Hodgkin lymphoma (NHL) and its relation to erythrocyte levels of their metabolites: 6-thioguanine-nucleotides (E-6TGN...

Cancer-associated myositis associated with oesophageal adenocarcinoma arising in Barrett's oesophagus without serum myogenic enzymes elevation: an example suggesting the importance of MRI.

Science.gov (United States)

Sasaki, Yosuke; Shimizu, Hiroshige; Nemoto, Tetsuo; Urita, Yoshihisa

2016-04-21

The strong association between myositis and malignancy has been well recognised. Cancer-associated myositis (CAM) is thought to be a cross-reaction to regenerating muscle tissue similar to tumour antigen. We report a case of CAM due to oesophageal adenocarcinoma arising in Barrett's oesophagus without elevation of myogenic enzymes, diagnosed by MRI and repeated endoscopy. Elderly onset, prominent symptoms, lack of interstitial pneumonia, poorer response to immunosuppressive therapies, and the combination of negative conventional myositis-related antibodies and positive anti-p155/140 antibody may help to distinguish CAM from idiopathic inflammatory myopathy. As the prognosis of patients with CAM depends on the malignancy, aggressive diagnosis of CAM and the causative malignancy is required. Our experience underscores the importance of avoiding the over-reliance on serum myogenic enzymes for excluding CAM and recognising MRI as a useful diagnostic tool of myositis. 2016 BMJ Publishing Group Ltd.

Side effects of treatment in childhood acute leukemia, 2

International Nuclear Information System (INIS)

Fujinami, Akira; Murakami, Mako; Sako, Masahiro; Takubo, Yoshiyuki; Nakagawa, Kimiko; Konishi, Shouzaburo; Tsujino, Giiti; Hata, Shinn; Koizumi, Yoshiko

1989-01-01

We evaluated delayed neurotoxicities in treatment of childhood acute leukemia. Of 28 patients treated over 2 years who were examined on computed tomography of brain scans, 7 patients had abnormal findings. These abnormalities included two cases of leukoencephalopathy, three cases of intracranial calcifications, and two of ventricular dilatation. These patients were under 6 years old at the onset of disease, especially under 3 years old. Also, delayed neurotoxicities developed after relapse of leukemia, especially CNS relapse. It was considered that these were caused by cranial irradiation, intravenous methotrexate injection, intrathecal methotrexate, and sometimes high-dose Ara-C therapy, etc. Most of the cases of leukoencephalopathy were associated with treatment of intermediate-dose or high-dose methotrexate after relapse. These abnormalities must be carefully considered in the treatment of younger children with leukemia and patients with relapse. (author)

Gene Dose Effects of GSTM1, GSTT1 and GSTP1 Polymorphisms on Outcome in Childhood Acute Lymphoblastic Leukemia

DEFF Research Database (Denmark)

Borst, Louise; Buchard, Anders; Rosthoj, Susanne

2012-01-01

Children with acute lymphoblastic leukemia (ALL) react very differently to chemotherapy. One explanation for this is inherited genetic variation. The glutathione S-transferase (GST) enzymes inactivate a number of chemotherapeutic drugs administered in childhood ALL therapy. Two multiplexing methods...

Developing standardised treatment for adults with myositis and different phenotypes: an international survey of current prescribing preferences.

Science.gov (United States)

Tansley, Sarah; Shaddick, Gavin; Christopher-Stine, Lisa; Sharp, Charlotte; Dourmishev, Lyubomir; Maurer, Britta; Chinoy, Hector; McHugh, Neil

2016-01-01

The evidence base for treatment of the idiopathic inflammatory myopathies is extremely limited. The rarity and heterogeneity of these diseases has hampered the development of good quality clinical trials and while a range of immunomodulatory treatments are commonly used in clinical practice, as yet there are no clear guidelines directing their use. We aimed to establish current prescribing regimens used to treat adults with myositis internationally. An electronic survey based on different clinical scenarios was distributed internationally to clinicians involved in the treatment of patients with myositis. Participants were asked to select their first-line treatment preferences in each situation. A multinomial regression analysis was used to assess the influence of clinical scenario, respondent expertise and country of origin on first-line treatment choice. 107 survey responses were received. 57% of respondents considered themselves an expert in myositis and the majority of respondents were rheumatologists although responses from other specialities were also received. Pharmacological treatment with steroids and additional immunotherapy was the preference in most scenarios. First-line immunosuppressant choice was significantly influenced by the clinical scenario, the expertise of the treating physician and country of practice. Azathioprine, methotrexate and mycophenolate mofetil were the most commonly chosen agents. In the absence of available evidence, clinical experience and expert consensus often forms the basis of treatment guidelines. These results suggest that an international consensus approach would be possible in myositis and would overcome an urgent, yet unmet need for patients suffering with this difficult disease.

Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes.

Science.gov (United States)

Miller, F W; Chen, W; O'Hanlon, T P; Cooper, R G; Vencovsky, J; Rider, L G; Danko, K; Wedderburn, L R; Lundberg, I E; Pachman, L M; Reed, A M; Ytterberg, S R; Padyukov, L; Selva-O'Callaghan, A; Radstake, T R; Isenberg, D A; Chinoy, H; Ollier, W E R; Scheet, P; Peng, B; Lee, A; Byun, J; Lamb, J A; Gregersen, P K; Amos, C I

2015-10-01

Autoimmune muscle diseases (myositis) comprise a group of complex phenotypes influenced by genetic and environmental factors. To identify genetic risk factors in patients of European ancestry, we conducted a genome-wide association study (GWAS) of the major myositis phenotypes in a total of 1710 cases, which included 705 adult dermatomyositis, 473 juvenile dermatomyositis, 532 polymyositis and 202 adult dermatomyositis, juvenile dermatomyositis or polymyositis patients with anti-histidyl-tRNA synthetase (anti-Jo-1) autoantibodies, and compared them with 4724 controls. Single-nucleotide polymorphisms showing strong associations (Pmyositis phenotypes together, as well as for the four clinical and autoantibody phenotypes studied separately. Imputation and regression analyses found that alleles comprising the human leukocyte antigen (HLA) 8.1 ancestral haplotype (AH8.1) defined essentially all the genetic risk in the phenotypes studied. Although the HLA DRB1*03:01 allele showed slightly stronger associations with adult and juvenile dermatomyositis, and HLA B*08:01 with polymyositis and anti-Jo-1 autoantibody-positive myositis, multiple alleles of AH8.1 were required for the full risk effects. Our findings establish that alleles of the AH8.1 comprise the primary genetic risk factors associated with the major myositis phenotypes in geographically diverse Caucasian populations.

An autoimmune myositis-overlap syndrome associated with autoantibodies to nuclear pore complexes: description and long-term follow-up of the anti-Nup syndrome.

Science.gov (United States)

Senécal, Jean-Luc; Isabelle, Catherine; Fritzler, Marvin J; Targoff, Ira N; Goldstein, Rose; Gagné, Michel; Raynauld, Jean-Pierre; Joyal, France; Troyanov, Yves; Dabauvalle, Marie-Christine

2014-11-01

Autoimmune myositis encompasses various myositis-overlap syndromes, each being identified by the presence of serum marker autoantibodies. We describe a novel myositis-overlap syndrome in 4 patients characterized by the presence of a unique immunologic marker, autoantibodies to nuclear pore complexes. The clinical phenotype was characterized by prominent myositis in association with erosive, anti-CCP, and rheumatoid factor-positive arthritis, trigeminal neuralgia, mild interstitial lung disease, Raynaud phenomenon, and weight loss. The myositis was typically chronic, relapsing, and refractory to corticosteroids alone, but remitted with the addition of a second immunomodulating drug. There was no clinical or laboratory evidence for liver disease. The prognosis was good with 100% long-term survival (mean follow-up 19.5 yr).By indirect immunofluorescence on HEp-2 cells, sera from all 4 patients displayed a high titer of antinuclear autoantibodies (ANA) with a distinct punctate peripheral (rim) fluorescent pattern of the nuclear envelope characteristic of nuclear pore complexes. Reactivity with nuclear pore complexes was confirmed by immunoelectron microscopy. In a cohort of 100 French Canadian patients with autoimmune myositis, the nuclear pore complex fluorescent ANA pattern was restricted to these 4 patients (4%). It was not observed in sera from 393 adult patients with systemic sclerosis (n = 112), mixed connective tissue disease (n = 35), systemic lupus (n = 94), rheumatoid arthritis (n = 45), or other rheumatic diseases (n = 107), nor was it observed in 62 normal adults.Autoantibodies to nuclear pore complexes were predominantly of IgG isotype. No other IgG autoantibody markers for defined connective tissue diseases or overlap syndromes were present, indicating a selective and highly focused immune response. In 3 patients, anti-nuclear pore complex autoantibody titers varied in parallel with myositis activity, suggesting a pathogenic link to

Extremely low-frequency magnetic fields and survival from childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Schüz, J; Grell, K; Kinsey, S

2012-01-01

A previous US study reported poorer survival in children with acute lymphoblastic leukemia (ALL) exposed to extremely low-frequency magnetic fields (ELF-MF) above 0.3 μT, but based on small numbers. Data from 3073 cases of childhood ALL were pooled from prospective studies conducted in Canada......, Denmark, Germany, Japan, UK and US to determine death or relapse up to 10 years from diagnosis. Adjusting for known prognostic factors, we calculated hazard ratios (HRs) and 95% confidence intervals (CI) for overall survival and event-free survival for ELF-MF exposure categories and by 0.1 μT increases...

Integration of childhood TB into guidelines for the management of acute malnutrition in high burden countries.

Science.gov (United States)

Patel, L N; Detjen, A K

2017-06-21

Introduction: Childhood tuberculosis (TB) and undernutrition are major global public health challenges. In 2015, although an estimated 1 million children aged malnutrition from 17 high TB burden countries were reviewed to gather information on TB symptom screening, exposure history, and treatment. Results: Seven (41%) countries recommend routine TB screening among children with acute malnutrition, and six (35%) recommend obtaining a TB exposure history. Conclusion: TB screening is not consistently included in guidelines for acute malnutrition in high TB burden countries. Routine TB risk assessment, especially history of TB exposure, among acutely malnourished children, combined with improved linkages with TB services, would help increase TB case finding and could impact outcomes. Operational research on how best to integrate services at different levels of the health care system is needed.

Childhood Acute Lymphoblastic Leukemia and Indicators of Early Immune Stimulation: A Childhood Leukemia International Consortium Study

Science.gov (United States)

Rudant, Jérémie; Lightfoot, Tracy; Urayama, Kevin Y.; Petridou, Eleni; Dockerty, John D.; Magnani, Corrado; Milne, Elizabeth; Spector, Logan G.; Ashton, Lesley J.; Dessypris, Nikolaos; Kang, Alice Y.; Miller, Margaret; Rondelli, Roberto; Simpson, Jill; Stiakaki, Eftichia; Orsi, Laurent; Roman, Eve; Metayer, Catherine; Infante-Rivard, Claire; Clavel, Jacqueline

2015-01-01

The associations between childhood acute lymphoblastic leukemia (ALL) and several proxies of early stimulation of the immune system, that is, day-care center attendance, birth order, maternally reported common infections in infancy, and breastfeeding, were investigated by using data from 11 case-control studies participating in the Childhood Leukemia International Consortium (enrollment period: 1980–2010). The sample included 7,399 ALL cases and 11,181 controls aged 2–14 years. The data were collected by questionnaires administered to the parents. Pooled odds ratios and 95% confidence intervals were estimated by unconditional logistic regression adjusted for age, sex, study, maternal education, and maternal age. Day-care center attendance in the first year of life was associated with a reduced risk of ALL (odds ratio = 0.77, 95% confidence interval: 0.71, 0.84), with a marked inverse trend with earlier age at start (P < 0.0001). An inverse association was also observed with breastfeeding duration of 6 months or more (odds ratio = 0.86, 95% confidence interval: 0.79, 0.94). No significant relationship with a history of common infections in infancy was observed even though the odds ratio was less than 1 for more than 3 infections. The findings of this large pooled analysis reinforce the hypothesis that day-care center attendance in infancy and prolonged breastfeeding are associated with a decreased risk of ALL. PMID:25731888

Brain Function in Young Patients Receiving Methotrexate for Acute Lymphoblastic Leukemia

Science.gov (United States)

2017-07-19

Childhood B Acute Lymphoblastic Leukemia; Childhood T Acute Lymphoblastic Leukemia; Cognitive Side Effects of Cancer Therapy; Long-Term Effects Secondary to Cancer Therapy in Children; Neurotoxicity Syndrome; Psychological Impact of Cancer; Untreated Childhood Acute Lymphoblastic Leukemia

Targeting FLT3 Signaling in Childhood Acute Myeloid Leukemia

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Amy N. Sexauer

2017-11-01

Full Text Available Acute myeloid leukemia (AML is the second most common leukemia of childhood and is associated with high rates of chemotherapy resistance and relapse. Clinical outcomes for children with AML treated with maximally intensive multi-agent chemotherapy lag far behind those of children with the more common acute lymphoblastic leukemia, demonstrating continued need for new therapeutic approaches to decrease relapse risk and improve long-term survival. Mutations in the FMS-like tyrosine kinase-3 receptor gene (FLT3 occur in approximately 25% of children and adults with AML and are associated with particularly poor prognoses. Identification and development of targeted FLT3 inhibitors represents a major precision medicine paradigm shift in the treatment of patients with AML. While further development of many first-generation FLT3 inhibitors was hampered by limited potency and significant toxicity due to effects upon other kinases, the more selective second- and third-generation FLT3 inhibitors have demonstrated excellent tolerability and remarkable efficacy in the relapsed/refractory and now de novo FLT3-mutated AML settings. While these newest and most promising inhibitors have largely been studied in the adult population, pediatric investigation of FLT3 inhibitors with chemotherapy is relatively recently ongoing or planned. Successful development of FLT3 inhibitor-based therapies will be essential to improve outcomes in children with this high-risk subtype of AML.

Mercaptopurine metabolite levels are predictors of bone marrow toxicity following high-dose methotrexate therapy of childhood acute lymphoblastic leukaemia

DEFF Research Database (Denmark)

Vang, Sophia Ingeborg; Schmiegelow, Kjeld; Frandsen, Thomas

2015-01-01

High-dose methotrexate (HD-MTX) courses with concurrent oral low-dose MTX/6-mercaptopurine (6MP) for childhood acute lymphoblastic leukaemia (ALL) are often followed by neutro- and thrombocytopenia necessitating treatment interruptions. Plasma MTX during HD-MTX therapy guides folinic acid rescue ...

Apparent diffusion coefficient (ADC) does not correlate with different serological parameters in myositis and myopathy.

Science.gov (United States)

Meyer, Hans-Jonas; Ziemann, Oliver; Kornhuber, Malte; Emmer, Alexander; Quäschling, Ulf; Schob, Stefan; Surov, Alexey

2018-06-01

Background Magnetic resonance imaging (MRI) is widely used in several muscle disorders. Diffusion-weighted imaging (DWI) is an imaging modality, which can reflect microstructural tissue composition. The apparent diffusion coefficient (ADC) is used to quantify the random motion of water molecules in tissue. Purpose To investigate ADC values in patients with myositis and non-inflammatory myopathy and to analyze possible associations between ADC and laboratory parameters in these patients. Material and Methods Overall, 17 patients with several myositis entities, eight patients with non-inflammatory myopathies, and nine patients without muscle disorder as a control group were included in the study (mean age = 55.3 ± 14.3 years). The diagnosis was confirmed by histopathology in every case. DWI was obtained in a 1.5-T scanner using two b-values: 0 and 1000 s/mm 2 . In all patients, the blood sample was acquired within three days to the MRI. The following serological parameters were estimated: C-reactive protein, lactate dehydrogenase, alanine aminotransferase, aspartate aminotransferase, creatine kinase, and myoglobine. Results The estimated mean ADC value for the myositis group was 1.89 ± 0.37 × 10 -3  mm 2 /s and for the non-inflammatory myopathy group was 1.79 ± 0.33 × 10 -3  mm 2 /s, respectively. The mean ADC values (1.15 ± 0.37 × 10 -3  mm 2 /s) were significantly higher to unaffected muscles (vs. myositis P = 0.0002 and vs. myopathy P = 0.0021). There were no significant correlations between serological parameters and ADC values. Conclusion Affected muscles showed statistically significantly higher ADC values than normal muscles. No linear correlations between ADC and serological parameters were identified.

Dynamic exophthalmos and lateral strabismus in a dog caused by masticatory muscle myositis.

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Czerwinski, Sarah L; Plummer, Caryn E; Greenberg, Shari M; Craft, William F; Conway, Julia A; Perez, Mayrim L; Cooke, Kirsten L; Winter, Matthew D

2015-11-01

A 5.5-year-old neutered male mixed-breed dog was presented for evaluation of a 5-month history of deviation of the right globe upon opening the mouth and a 1-month history of conjunctivitis in the same eye. Clinical findings, diagnostic imaging results, treatment and follow-up are described. When the mouth was opened, the right globe deviated rostrally and laterally. There was no pain or resistance to opening the mouth, or resistance to retropulsion of the globe. No other abnormalities were observed. Computed tomography was performed, revealing a contrast-enhancing region associated with the right masseter muscle impinging into the right orbit; this was more pronounced with the mouth open. Cytology revealed a small number of mildly to moderately atypical mesenchymal cells. Histopathology was consistent with polyphasic myositis, with a predominance of lymphocytes and plasma cells. No infectious agents were identified. Serum antibody titers for Toxoplasma gondii and Neospora caninum were negative. Serum titers for 2 M antibody were positive at 1:500, consistent with a diagnosis of masticatory muscle myositis. Therapy with prednisone was initiated. During a follow-up period of 5 months, there was no recurrence of clinical signs, and the dose of prednisone was reduced by 25%. To the authors' knowledge, this is the first reported case of masticatory muscle myositis causing dynamic exophthalmos and strabismus in a dog. © 2015 American College of Veterinary Ophthalmologists.

Pediatric nontraumatic myositis ossificans of the neck

International Nuclear Information System (INIS)

Kokkosis, Angela A.; Balsam, Dvorah; Lee, Thomas K.; Schreiber, Z.J.

2009-01-01

Nontraumatic myositis ossificans circumscripta (MOC) is a rarely reported benign heterotopic ossification characterized by the aberrant formation of bone in extraskeletal soft tissues. Although a history of trauma can be elicited in 75% of MOC patients, the etiology is unclear in patients without inciting injury. MOC is associated with young male athletes, and is most often localized to the muscle groups of the extremities. Rare cases have been reported in children and adolescents of nontraumatic MOC in the neck. We present a 15-year-old adolescent with a rapidly growing, painful neck mass without traumatic stimulus. (orig.)

Pediatric nontraumatic myositis ossificans of the neck

Energy Technology Data Exchange (ETDEWEB)

Kokkosis, Angela A. [Stony Brook University Medical Center, Stony Brook University School of Medicine, Stony Brook, NY (United States); Balsam, Dvorah [Stony Brook University Medical Center, Department of Radiology, Stony Brook, NY (United States); Lee, Thomas K. [Stony Brook University Medical Center, Department of Pediatric Surgery, Stony Brook, NY (United States); Schreiber, Z.J. [Stony Brook University Medical Center, Department of Pathology, Stony Brook, NY (United States)

2009-04-15

Nontraumatic myositis ossificans circumscripta (MOC) is a rarely reported benign heterotopic ossification characterized by the aberrant formation of bone in extraskeletal soft tissues. Although a history of trauma can be elicited in 75% of MOC patients, the etiology is unclear in patients without inciting injury. MOC is associated with young male athletes, and is most often localized to the muscle groups of the extremities. Rare cases have been reported in children and adolescents of nontraumatic MOC in the neck. We present a 15-year-old adolescent with a rapidly growing, painful neck mass without traumatic stimulus. (orig.)

Genes commonly deleted in childhood B-cell precursor acute lymphoblastic leukemia: association with cytogenetics and clinical features

Science.gov (United States)

Schwab, Claire J.; Chilton, Lucy; Morrison, Heather; Jones, Lisa; Al-Shehhi, Halima; Erhorn, Amy; Russell, Lisa J.; Moorman, Anthony V.; Harrison, Christine J.

2013-01-01

In childhood B-cell precursor acute lymphoblastic leukemia, cytogenetics is important in diagnosis and as an indicator of response to therapy, thus playing a key role in risk stratification of patients for treatment. Little is known of the relationship between different cytogenetic subtypes in B-cell precursor acute lymphoblastic leukemia and the recently reported copy number abnormalities affecting significant leukemia associated genes. In a consecutive series of 1427 childhood B-cell precursor acute lymphoblastic leukemia patients, we have determined the incidence and type of copy number abnormalities using multiplex ligation-dependent probe amplification. We have shown strong links between certain deletions and cytogenetic subtypes, including the novel association between RB1 deletions and intrachromosomal amplification of chromosome 21. In this study, we characterized the different copy number abnormalities and show heterogeneity of PAX5 and IKZF1 deletions and the recurrent nature of RB1 deletions. Whole gene losses are often indicative of larger deletions, visible by conventional cytogenetics. An increased number of copy number abnormalities is associated with NCI high risk, specifically deletions of IKZF1 and CDKN2A/B, which occur more frequently among these patients. IKZF1 deletions and rearrangements of CRLF2 among patients with undefined karyotypes may point to the poor risk BCR-ABL1-like group. In conclusion, this study has demonstrated in a large representative cohort of children with B-cell precursor acute lymphoblastic leukemia that the pattern of copy number abnormalities is highly variable according to the primary genetic abnormality. PMID:23508010

A Rare Case Of Non Traumatic Myositis Ossificans Circumscripta

OpenAIRE

Mahale, Yashwant J.; Vyawahare, Chaitanya S.; Dravid, Nandkishore V.; Upase, Aditya; Rathi, Romil

2015-01-01

Introduction: Myositis ossificans circumscripta is a benign non neoplastic ossifying tumor presenting with bone like osteoid tissue extraskelletaly amidst the muscle planes. This condition when not associated with trauma is very trivial and considering the way it mimics certain characteristics, it may be misunderstood as a malignant neoplasm, abscess or antibioma. The aetiology of this atraumatic condition is still indistinct and remains a question unsolved. We would like to report such a cas...

Extraocular myositis in a female puppy

Directory of Open Access Journals (Sweden)

O. Adegboye

2015-04-01

Full Text Available Extraocular myositis (EOM is not commonly encountered in dogs. It is generally diagnosed based on clinical features of exophthalmos without third eyelid protrusion, pain or vision loss. The traditional treatment of choice is prednisolone. This report describes a case of a mixed-breed puppy with clinical signs consistent with EOM, the use of ascorbic acid as an adjuvant to traditional corticosteroid therapy and rapid resolution of the condition without recurrence. It also shows that prolapse of the third eyelid and ptosis of the lower eyelids are possible signs of EOM during recovery. This is the first report of this sort from Africa and therefore the report is of epidemiological significance.

Myositis Ossificans Traumatica Causing Ankylosis of the Elbow

OpenAIRE

Kanthimathi, B.; Udhaya Shankar, S.; Arun Kumar, K.; Narayanan, V. L.

2014-01-01

Myositis ossificans traumatica is an unusual complication following a muscle contusion injury. A significantly large myositic mass causing ankylosis of the elbow is even rarer. We report a 13-year-old boy who presented with a 14-month history of a fixed elbow with no movement and a palpable bony mass in the anterior aspect of the elbow. He had sustained significant trauma to the affected limb 1 month prior to onset of symptoms, which was managed by native massage and bandaging for 4 weeks. Th...

Myositis in the head and neck: challenges in diagnosis and management.

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Ratansi, R; Fabbroni, G; Kanatas, A

2017-09-01

Myositis in the head and neck may present with non-specific symptoms, and radiographically may mimic malignancy. Multidisciplinary management is often essential, and we describe the challenges in an effort to raise awareness of the condition. Copyright © 2017 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Daily supplementation of D-ribose shows no therapeutic benefits in the MHC-I transgenic mouse model of inflammatory myositis.

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William Coley

Full Text Available BACKGROUND: Current treatments for idiopathic inflammatory myopathies (collectively called myositis focus on the suppression of an autoimmune inflammatory response within the skeletal muscle. However, it has been observed that there is a poor correlation between the successful suppression of muscle inflammation and an improvement in muscle function. Some evidence in the literature suggests that metabolic abnormalities in the skeletal muscle underlie the weakness that continues despite successful immunosuppression. We have previously shown that decreased expression of a purine nucleotide cycle enzyme, adenosine monophosphate deaminase (AMPD1, leads to muscle weakness in a mouse model of myositis and may provide a mechanistic basis for muscle weakness. One of the downstream metabolites of this pathway, D-ribose, has been reported to alleviate symptoms of myalgia in patients with a congenital loss of AMPD1. Therefore, we hypothesized that supplementing exogenous D-ribose would improve muscle function in the mouse model of myositis. We treated normal and myositis mice with daily doses of D-ribose (4 mg/kg over a 6-week time period and assessed its effects using a battery of behavioral, functional, histological and molecular measures. RESULTS: Treatment with D-ribose was found to have no statistically significant effects on body weight, grip strength, open field behavioral activity, maximal and specific forces of EDL, soleus muscles, or histological features. Histological and gene expression analysis indicated that muscle tissues remained inflamed despite treatment. Gene expression analysis also suggested that low levels of the ribokinase enzyme in the skeletal muscle might prevent skeletal muscle tissue from effectively utilizing D-ribose. CONCLUSIONS: Treatment with daily oral doses of D-ribose showed no significant effect on either disease progression or muscle function in the mouse model of myositis.

Daily Supplementation of D-ribose Shows No Therapeutic Benefits in the MHC-I Transgenic Mouse Model of Inflammatory Myositis

Science.gov (United States)

Coley, William; Rayavarapu, Sree; van der Meulen, Jack H.; Duba, Ayyappa S.; Nagaraju, Kanneboyina

2013-01-01

Background Current treatments for idiopathic inflammatory myopathies (collectively called myositis) focus on the suppression of an autoimmune inflammatory response within the skeletal muscle. However, it has been observed that there is a poor correlation between the successful suppression of muscle inflammation and an improvement in muscle function. Some evidence in the literature suggests that metabolic abnormalities in the skeletal muscle underlie the weakness that continues despite successful immunosuppression. We have previously shown that decreased expression of a purine nucleotide cycle enzyme, adenosine monophosphate deaminase (AMPD1), leads to muscle weakness in a mouse model of myositis and may provide a mechanistic basis for muscle weakness. One of the downstream metabolites of this pathway, D-ribose, has been reported to alleviate symptoms of myalgia in patients with a congenital loss of AMPD1. Therefore, we hypothesized that supplementing exogenous D-ribose would improve muscle function in the mouse model of myositis. We treated normal and myositis mice with daily doses of D-ribose (4 mg/kg) over a 6-week time period and assessed its effects using a battery of behavioral, functional, histological and molecular measures. Results Treatment with D-ribose was found to have no statistically significant effects on body weight, grip strength, open field behavioral activity, maximal and specific forces of EDL, soleus muscles, or histological features. Histological and gene expression analysis indicated that muscle tissues remained inflamed despite treatment. Gene expression analysis also suggested that low levels of the ribokinase enzyme in the skeletal muscle might prevent skeletal muscle tissue from effectively utilizing D-ribose. Conclusions Treatment with daily oral doses of D-ribose showed no significant effect on either disease progression or muscle function in the mouse model of myositis. PMID:23785461

Long-term hearing outcomes after recurrent acute otitis media during early childhood.

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Krakau, Mattias; Dagöö, Britta Rynnel; Hellström, Sten; Granath, Anna

2017-12-01

To survey long-term hearing outcomes and middle ear pathology in a 30-year follow-up in individuals with onset of recurrent acute otitis media (rAOM) before three years of age. 28 adults, aged 30.1-31.8 years, who originally - at the age of 12-32 months - participated in a study on rAOM between 1979 and 1983, were re-examined regarding self-reported ear problems, current tympanic membrane changes and audiology. Thirteen subjects had suffered from rAOM during early childhood and 15 subjects served as a control group. Recurrent acute otitis media subjects reported hearing problems comparable to those of the controls. Pure tone audiometry, at 125-8000 Hz, did not differ between groups. The rAOM group had a trend for impaired high-frequency (9000-14,000 Hz) threshold levels (9000-14,000 Hz); implying that their cochlear function seemed to have deteriorated. Adults, who suffered from recurrent acute otitis media as infants, did not show any clinically significant hearing loss for pure tone audiometry when compared to controls, but there was a trend for impaired results regarding extended high frequency audiometry (9-14 kHz). Children suffering from rAOM will be at low risk of developing hearing loss and severe middle ear disease.

Outcome and prognostic factors in a French cohort of patients with myositis-associated interstitial lung disease.

Science.gov (United States)

Obert, Julie; Freynet, Olivia; Nunes, Hilario; Brillet, Pierre-Yves; Miyara, Makoto; Dhote, Robin; Valeyre, Dominique; Naccache, Jean-Marc

2016-12-01

Interstitial lung disease (ILD) is a common form of extramuscular involvement in patients with polymyositis/dermatomyositis and is associated with poor prognosis. This study was designed to describe the long-term outcome of myositis-associated ILD. This retrospective observational study was conducted in 48 consecutive patients. Two groups defined according to outcome were compared to determine prognostic factors: a "severe" group (vital capacity [VC] myositis-associated ILD had severe initial PFT results but a low mortality rate. Independent prognostic factors at presentation were initial VC and myopathic changes on electromyography. This study highlights the need for studies focusing on the correlation between muscle and lung pathogenic mechanisms.

Cost-Effectiveness of Treatment of Childhood Acute Lymphoblastic Leukemia With Chemotherapy Only: The Influence of New Medication and Diagnostic Technology

NARCIS (Netherlands)

van Litsenburg, R.R.L.; Uyl-de Groot, C.A.; Raat, H.; Kaspers, G.J.L.; Gemke, R.J.B.J.

2011-01-01

Background: Survival for childhood acute lymphoblastic leukemia (ALL) has reached 80-90%. Future improvement in treatment success will involve new technologies and medication, adding to the pressure on limited financial resources. Therefore a retrospective cost-effectiveness analysis of ALL

Contextual factors associated with health care service utilization for children with acute childhood illnesses in Nigeria.

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Sulaimon T Adedokun

Full Text Available To examine the independent contribution of individual, community and state-level factors to health care service utilization for children with acute childhood illnesses in Nigeria.The study was based on secondary analyses of cross-sectional population-based data from the 2013 Nigeria Demographic and Health Survey (DHS. Multilevel logistic regression models were applied to the data on 6,427 under-five children who used or did not use health care service when they were sick (level 1, nested within 896 communities (level 2 from 37 states (level 3.About one-quarter of the mothers were between 15 and 24 years old and almost half of them did not have formal education (47%. While only 30% of the children utilized health service when they were sick, close to 67% lived in the rural area. In the fully adjusted model, mothers with higher education attainment (Adjusted odds ratio [aOR] = 1.63; 95% credible interval [CrI] = 1.31-2.03, from rich households (aOR = 1.76; 95% CrI = 1.35-2.25, with access to media (radio, television or magazine (aOR = 1.18; 95% CrI = 1.08-1.29, and engaging in employment (aOR = 1.18; 95% CrI = 1.02-1.37 were significantly more likely to have used healthcare services for acute childhood illnesses. On the other hand, women who experienced difficulty getting to health facilities (aOR = 0.87; 95% CrI = 0.75-0.99 were less likely to have used health service for their children.Our findings highlight that utilization of healthcare service for acute childhood illnesses was influenced by not only maternal factors but also community-level factors, suggesting that public health strategies should recognise this complex web of individual composition and contextual composition factors to guide provision of healthcare services. Such interventions could include: increase in female school enrolment, provision of interest-free loans for small and medium scale enterprises, introduction of mobile clinics and establishment of more primary health care

Radiation-recall myositis presenting as low-back pain (2010: 4b)

International Nuclear Information System (INIS)

Heirwegh, Geert; Bruyeer, Eveline; Demaerel, Philippe; Renard, Marleen; Uyttebroeck, Anne

2010-01-01

We report on a patient with a history of Ewing sarcoma who underwent surgery and subsequent adjuvant chemotherapy and radiotherapy. He developed low-back pain 6 months after the end of the radiotherapy and during consolidation chemotherapy. Magnetic resonance imaging showed evidence of myositis corresponding to the 'radiation-recall phenomenon', an inflammatory reaction of irradiated tissue. (orig.)

Radiation-recall myositis presenting as low-back pain (2010: 4b)

Energy Technology Data Exchange (ETDEWEB)

Heirwegh, Geert [AZ Damiaanziekenhuis, Department of Radiology, Oostende (Belgium); University Hospital K.U. Leuven, Department of Radiology, Leuven (Belgium); Bruyeer, Eveline; Demaerel, Philippe [University Hospital K.U. Leuven, Department of Radiology, Leuven (Belgium); Renard, Marleen; Uyttebroeck, Anne [University Hospital K.U. Leuven, Department of Paediatrics, Leuven (Belgium)

2010-07-15

We report on a patient with a history of Ewing sarcoma who underwent surgery and subsequent adjuvant chemotherapy and radiotherapy. He developed low-back pain 6 months after the end of the radiotherapy and during consolidation chemotherapy. Magnetic resonance imaging showed evidence of myositis corresponding to the 'radiation-recall phenomenon', an inflammatory reaction of irradiated tissue. (orig.)

''Dropped-head'' syndrome due to isolated myositis of neck extensor muscles: MRI findings

International Nuclear Information System (INIS)

Gaeta, Michele; Mazziotti, Silvio; Blandino, Alfredo; Toscano, Antonio; Rodolico, Carmelo; Mazzeo, Anna

2006-01-01

MRI findings of a patient with dropped-head syndrome due to focal myositis of the neck extensor muscles are presented. MRI showed oedematous changes and marked enhancement of the neck extensor muscles. After therapy MRI demonstrated disappearance of the abnormal findings. (orig.)

High Throughput Drug Sensitivity Assay and Genomics- Guided Treatment of Patients With Relapsed or Refractory Acute Leukemia

Science.gov (United States)

2018-02-28

Acute Leukemia of Ambiguous Lineage; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Acute Myeloid Leukemia; Refractory Adult Acute Lymphoblastic Leukemia; Refractory Childhood Acute Lymphoblastic Leukemia

Living on a farm, contact with farm animals and pets, and childhood acute lymphoblastic leukemia: pooled and meta-analyses from the Childhood Leukemia International Consortium.

Science.gov (United States)

Orsi, Laurent; Magnani, Corrado; Petridou, Eleni T; Dockerty, John D; Metayer, Catherine; Milne, Elizabeth; Bailey, Helen D; Dessypris, Nick; Kang, Alice Y; Wesseling, Catharina; Infante-Rivard, Claire; Wünsch-Filho, Victor; Mora, Ana M; Spector, Logan G; Clavel, Jacqueline

2018-04-16

The associations between childhood acute lymphoblastic leukemia (ALL) and several factors related to early stimulation of the immune system, that is, farm residence and regular contacts with farm animals (livestock, poultry) or pets in early childhood, were investigated using data from 13 case-control studies participating in the Childhood Leukemia International Consortium. The sample included 7847 ALL cases and 11,667 controls aged 1-14 years. In all studies, the data were obtained from case and control parents using standardized questionnaires. Pooled odds ratios (ORs) and 95% confidence intervals (CIs) were estimated by unconditional logistic regression adjusted for age, sex, study, maternal education, and maternal age. Contact with livestock in the first year of life was inversely associated with ALL (OR = 0.65, 95% CI: 0.50, 0.85). Inverse associations were also observed for contact with dogs (OR = 0.92, 95% CI: 0.86, 0.99) and cats (OR = 0.87, 95% CI: 0.80, 0.94) in the first year of life. There was no evidence of a significant association with farm residence in the first year of life. The findings of these large pooled and meta-analyses add additional evidence to the hypothesis that regular contact with animals in early childhood is inversely associated with childhood ALL occurrence which is consistent with Greaves' delayed infection hypothesis. © 2018 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.

Childhood Acute Lymphoblastic Leukemia: Progress Through Collaboration

Science.gov (United States)

Yang, Jun J.; Hunger, Stephen P.; Pieters, Rob; Schrappe, Martin; Biondi, Andrea; Vora, Ajay; Baruchel, André; Silverman, Lewis B.; Schmiegelow, Kjeld; Escherich, Gabriele; Horibe, Keizo; Benoit, Yves C.M.; Izraeli, Shai; Yeoh, Allen Eng Juh; Liang, Der-Cherng; Downing, James R.; Evans, William E.; Relling, Mary V.; Mullighan, Charles G.

2015-01-01

Purpose To review the impact of collaborative studies on advances in the biology and treatment of acute lymphoblastic leukemia (ALL) in children and adolescents. Methods A review of English literature on childhood ALL focusing on collaborative studies was performed. The resulting article was reviewed and revised by the committee chairs of the major ALL study groups. Results With long-term survival rates for ALL approaching 90% and the advent of high-resolution genome-wide analyses, several international study groups or consortia were established to conduct collaborative research to further improve outcome. As a result, treatment strategies have been improved for several subtypes of ALL, such as infant, MLL-rearranged, Philadelphia chromosome–positive, and Philadelphia chromosome–like ALL. Many recurrent genetic abnormalities that respond to tyrosine kinase inhibitors and multiple genetic determinants of drug resistance and toxicities have been identified to help develop targeted therapy. Several genetic polymorphisms have been recognized that show susceptibility to developing ALL and that help explain the racial/ethnic differences in the incidence of ALL. Conclusion The information gained from collaborative studies has helped decipher the heterogeneity of ALL to help improve personalized treatment, which will further advance the current high cure rate and the quality of life for children and adolescents with ALL. PMID:26304874

Renal, gastrointestinal, and hepatic late effects in survivors of childhood acute myeloid leukemia treated with chemotherapy only--a NOPHO-AML study

DEFF Research Database (Denmark)

Skou, Anne-Sofie; Glosli, Heidi; Jahnukainen, Kirsi

2014-01-01

BACKGROUND: We investigated the spectrum, frequency, and risk factors for renal, gastrointestinal, and hepatic late adverse effects in survivors of childhood acute myeloid leukemia (AML) without relapse treated with chemotherapy alone according to three consecutive AML trials by the Nordic Society...

The use of intraosseous anesthesia in a patient with myositis ossificans progressiva.

Science.gov (United States)

Webb, M D; Wilson, C

1996-01-01

The case of a pediatric patient with myositis ossificans progressiva in whom it became increasingly difficult to obtain local anesthesia is presented. Intraosseous anesthesia was successful in allowing pain-free dental treatment to be completed. This approach should be considered in other patients who have limited mouth-opening ability due to injury or disease.

INTERSTITIAL LUNG-DISEASE AND MYOSITIS IN A PATIENT WITH SIMULTANEOUSLY OCCURRING SARCOIDOSIS AND SCLERODERMA

NARCIS (Netherlands)

GROEN, H; POSTMA, DS; KALLENBERG, CGM

1993-01-01

A patient initially presented with sarcoidosis in combination with myositis of sarcoid origin and Raynaud's phenomenon. During the course of his disease, he additionally developed scleroderma. Bronchoalveolar lavage, performed because of increase of interstitial markings in the presence of enlarged

Clinical features and early treatment response of central nervous system involvement in childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Levinsen, Mette; Taskinen, Mervi; Abrahamsson, Jonas

2014-01-01

BACKGROUND: Central nervous system (CNS) involvement in childhood acute lymphoblastic leukemia (ALL) remains a therapeutic challenge. PROCEDURE: To explore leukemia characteristics of patients with CNS involvement at ALL diagnosis, we analyzed clinical features and early treatment response of 744...... leukemia and patients without such characteristics (0.50 vs. 0.61; P = 0.2). CONCLUSION: CNS involvement at diagnosis is associated with adverse prognostic features but does not indicate a less chemosensitive leukemia....

Case report 488: Post-traumatic myositis ossificans mimicking a soft tissue neoplasm

International Nuclear Information System (INIS)

Ackerman, L.; Ramamurthy, S.; Jablokow, V.; Van Drunen, M.; Kaplan, E.

1988-01-01

A case of post-traumatic myosotis ossificans (MO) in a young man with only a vague history of preceding physical trauma to the area of the lesion was presented. Imaging modalities, including plain film radiography, CT, contrast angiography and bone scintigraphy, could not exclude a malignancy with a reliable degree of certainty. The biopsy specimen was consistent with MO but could easily be misinterpreted as a sarcomatous lesion. A diagnosis of MO was only established by a scrupulous analysis of all clinical, radiological, scintigraphic and microscopic findings and unnecessary extensive surgery for a malignancy was avoided. The correlative radiological and pathological features in distinguishing post-traumatic myositis ossificans from such lesions as soft tissue osteosarcoma or parosteal osteosarcoma were described and the meaning of the term zoning phenomenon was elucidated. It was emphasized that utilizing the zonal phenomenon (both pathologically and radiologically) ensures a correct diagnosis most of the time. However, it must be emphasized that this zoning phenomenon is not established in the early stages of post-traumatic myositis ossificans. (oerig./MG)

HMGCR-associated myositis: a New Zealand case series and estimate of incidence.

Science.gov (United States)

Kennedy, N; Keating, P; O'Donnell, J

2016-05-01

Statins are one of the most commonly prescribed drugs in New Zealand, with 525 772 or 16.5% of the adult New Zealand population prescribed a statin between June 2013 and July 2014. While generally well-tolerated, statins are known to cause a range of muscle-related side effects, ranging from myalgia to life-threatening rhabdomyolysis. Recently, it has been recognised that in rare instances, statins can induce an immune-mediated necrotising myositis with antibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR), the enzymatic target of statins. In 2014, anti-HMGCR antibody testing was introduced to Canterbury Health Laboratories (CHL), with this being the only laboratory in New Zealand performing this test during the period of this case series. This article describes an index case and characterises the clinical features of a subsequent 12-month series. From this series, we estimated the yearly incidence of HMGCR-associated myositis at 1.7/million/year or ~1/90 000 New Zealand statin users. © 2016 Royal Australasian College of Physicians.

Cranial radiotherapy predisposes to abdominal adiposity in survivors of childhood acute lymphocytic leukemia

International Nuclear Information System (INIS)

Siviero-Miachon, Adriana Aparecida; Spinola-Castro, Angela Maria; Lee, Maria Lúcia de Martino; Andreoni, Solange; Geloneze, Bruno; Lederman, Henrique; Guerra-Junior, Gil

2013-01-01

Advances in treatment of acute lymphocytic leukemia increased the likelihood of developing late treatment-associated effects, such as abdominal adiposity, increasing the risk of cardiovascular disease in this population. Cranial radiotherapy is one of the factors that might be involved in this process. The aim of this study was to determine the effect of cranial radiotherapy on adiposity indexes in survivors of acute lymphocytic leukemia. A comparative cross-sectional study of 56 acute lymphocytic leukemia survivors, chronological age between 15 and 24 years, assigned into two groups according to the exposure to cranial radiotherapy (25 irradiated and 31 non-irradiated), assessed according to body fat (dual energy X-ray absorptiometry), computed tomography scan-derived abdominal adipose tissue, lipid profile, and insulin resistance. Cranial radiotherapy increased body fat and abdominal adipose tissue and altered lipid panel. Yet, lipids showed no clinical relevance so far. There were significantly more obese patients among those who received cranial radiotherapy (52% irradiated versus 22.6% non-irradiated), based on dual energy X-ray absorptiometry body fat measurements. Nonetheless, no association was observed between cranial radiotherapy and body mass index, waist circumference, waist-to-height ratio or insulin resistance. Adolescent and young adult survivors of childhood acute lymphocytic leukemia showed an increase in body fat and an alteration of fat distribution, which were related to cranial radiotherapy. Fat compartment modifications possibly indicate a disease of adipose tissue, and cranial radiotherapy imports in this process

Consequenses of childhood adversity on health concerns in adulthood

African Journals Online (AJOL)

Consequenses of childhood adversity on health concerns in adulthood. ... childhood adversity have similar levels of depression, acute and chronic health. ... to explain the pathways linking childhood adversity to physical health in adulthood.

Three-dimensional CT diagnosis of myositis ossificans of the sacrospinous ligament

International Nuclear Information System (INIS)

Agrons, G.A.; Markowitz, R.I.; Bronson, W.E.

1993-01-01

We present the case of a 4-year-old female with a complex fracture of the left hemipelvis who, on follow-up CT imaging, developed new ossific densities within the peripelvic soft tissues of the contralateral side. Three-dimensional surface reformations of the pelvis demonstrated myositis ossificans along the course of the right sacrospinous ligament, thus elucidating unsuspected ligamentous injury and implying prior instability. (orig.)

Three-dimensional CT diagnosis of myositis ossificans of the sacrospinous ligament

Energy Technology Data Exchange (ETDEWEB)

Agrons, G.A. (Children' s Hospital of Philadelphia, PA (United States)); Markowitz, R.I. (Children' s Hospital of Philadelphia, PA (United States)); Bronson, W.E. (Children' s Hospital of Philadelphia, PA (United States))

1993-04-01

We present the case of a 4-year-old female with a complex fracture of the left hemipelvis who, on follow-up CT imaging, developed new ossific densities within the peripelvic soft tissues of the contralateral side. Three-dimensional surface reformations of the pelvis demonstrated myositis ossificans along the course of the right sacrospinous ligament, thus elucidating unsuspected ligamentous injury and implying prior instability. (orig.)

Nivolumab-induced synchronous occurrence of myositis and hypothyroidism in a patient with squamous cell lung cancer.

Science.gov (United States)

Badovinac, Sonja; Korsic, Marta; Zarkovic, Kamelija; Mursic, Davorka; Roglic, Mihovil; Jakopovic, Marko; Samarzija, Miroslav

2018-03-01

Alongside the proven efficacy, immunotherapy in treatment of malignant diseases can cause immune-related adverse events different from commonly known chemotherapy-related toxicities. During nivolumab treatment of metastatic squamous cell lung cancer, the patient developed a symptomatic inflammatory myositis confirmed with muscle biopsy and primary hypothyroidism. After initiation of corticosteroids and thyroid hormone replacement, the clinical and laboratory improvement occurred. To the best of our knowledge, this is the first description of a case of nivolumab-induced synchronous manifestation of immune-related myositis and hypothyroidism. Immunotherapy can trigger a wide spectrum of immune-related adverse events that could occur simultaneously. If not detected and treated, these events could become severe or even fatal and require clinicians' awareness and routine check-ups.

Aberrant Muscle Antigen Exposure in Mice Is Sufficient to Cause Myositis in a Treg Cell–Deficient Milieu

Science.gov (United States)

Young, Nicholas A; Sharma, Rahul; Friedman, Alexandra K; Kaffenberger, Benjamin H; Bolon, Brad; Jarjour, Wael N

2013-01-01

Objective Myositis is associated with muscle-targeted inflammation and is observed in some Treg cell–deficient mouse models. Because an autoimmune pathogenesis has been strongly implicated, the aim of this study was to investigate the hypothesis that abnormal exposure to muscle antigens, as observed in muscle injury, can induce autoimmune-mediated myositis in susceptible hosts. Methods FoxP3 mutant (scurfy) mice were mated to synaptotagmin VII (Syt VII) mutant mice, which resulted in a new mouse strain that combines impaired membrane resealing with Treg cell deficiency. Lymphocyte preparations from double-mutant mice were adoptively transferred intraperitoneally, with or without purified Treg cells, into recombination-activating gene 1 (RAG-1)–null recipients. Lymph node cells from mice with the FoxP3 mutation were transferred into RAG-1–null mice either 1) intraperitoneally in conjunction with muscle homogenate or purified myosin protein or 2) intramuscularly with or without cotransfer of purified Treg cells. Results FoxP3-deficient mouse lymph node cells transferred in conjunction with myosin protein or muscle homogenate induced robust skeletal muscle inflammation. The infiltrates consisted predominantly of CD4+ and CD8+ T cells, a limited number of macrophages, and no B cells. Significant inflammation was also seen in similar experiments using lymph node cells from FoxP3/Syt VII double-mutant mice but was absent in experiments using adoptive transfer of FoxP3 mutant mouse cells alone. The cotransfer of Treg cells completely suppressed myositis. Conclusion These data, derived from a new, reproducible model, demonstrate the critical roles of Treg cell deficiency and aberrant muscle antigen exposure in the priming of autoreactive cells to induce myositis. This mouse system has multifaceted potential for examining the interplay in vivo between tissue injury and autoimmunity. PMID:24022275

Vitamin and mineral supplements in pregnancy and the risk of childhood acute lymphoblastic leukaemia: a case-control study

Directory of Open Access Journals (Sweden)

Skegg David CG

2007-07-01

Full Text Available Abstract Background An earlier case-control study from Western Australia reported a protective effect of maternal folic acid supplementation during pregnancy on the risk of childhood acute lymphoblastic leukaemia (ALL. The present study tested that association. Methods A national case-control study was conducted in New Zealand. The mothers of 97 children with ALL and of 303 controls were asked about vitamin and mineral supplements taken during pregnancy. Results There was no association between reported folate intake during pregnancy and childhood ALL (adjusted odds ratio (OR 1.1, 95% confidence interval (CI 0.5–2.7. Combining our results with the study from Western Australia and another study from Québec in a meta-analysis gave a summary OR of 0.9 (95% CI 0.8–1.1. Conclusion Our own study, of similar size to the Australian study, does not support the hypothesis of a protective effect of folate on childhood ALL. Neither do the findings of the meta-analysis.

Childhood Leukemia

Science.gov (United States)

... acute types. Symptoms include Infections Fever Loss of appetite Tiredness Easy bruising or bleeding Swollen lymph nodes Night sweats Shortness of breath Pain in the bones or joints Risk factors for childhood leukemia include having a brother ...

Heterogeneous clinical spectrum of anti-SRP myositis and importance of the methods of detection of anti-SRP autoantibodies: a multicentric study.

Science.gov (United States)

Picard, Cécile; Vincent, Thierry; Lega, Jean-Christophe; Hue, Sophie; Fortenfant, Françoise; Lakomy, Daniela; Humbel, René-Louis; Goetz, Joelle; Molinari, Nicolas; Bardin, Nathalie; Bertin, Daniel; Johanet, Catherine; Chretien, Pascale; Dubucquoi, Sylvain; Streichenberger, Nathalie; Desplat-Jégo, Sophie; Bossuyt, Xavier; Sibilia, Jean; Abreu, Isabelle; Chevailler, Alain; Fabien, Nicole

2016-06-01

Anti-signal recognition particle (SRP) antibodies are important serological markers for the diagnosis and the prognosis of idiopathic inflammatory myopathy (IIM), especially to distinguish immune-mediated necrotizing myopathy (IMNM). This study was set up to investigate the phenotype associated with anti-SRP antibodies and to evaluate the methods for detecting these antibodies. Clinical and biological data were retrospectively obtained from 60 adult patients with anti-SRP antibodies detected by a dot immunoassay from 12 centers. Thirty-six (60 %) out of these 60 patients suffered from an IIM, and among them, 21 patients were diagnosed as IMNM. Among patients with a definite IIM, proximal weakness and myalgia were prominent symptoms at the time of diagnosis. Only few patients displayed severe extra-muscular symptoms such as cardiac involvement or severe myositis. Mean creatine kinase levels were high for all patients except for two of them. When testing by indirect immunofluorescence (IIF) on HEp2 cells, the fraction of patients displaying the typical anti-SRP fine speckled staining of the cytoplasm was higher in patients with IIM (30/36) (83 %) than in patients with non-IIM (3/24) (12.5 %) (p myositis, whereas only 6 (22 %) out of 27 patients with a positive immunodot but a negative cytoplasmic pattern suffered from a myositis (p myositis.

Late effects of childhood leukemia therapy.

Science.gov (United States)

Fulbright, Joy M; Raman, Sripriya; McClellan, Wendy S; August, Keith J

2011-09-01

As survival rates for children treated for childhood cancers become significantly better, the focus is increasingly on determining the late effects of treatments and the best ways to monitor for them and prevent their occurrence. This review focuses on recent literature discussing the late effects of treatment in patients treated for acute myeloid leukemia and acute lymphoblastic leukemia during childhood. The late effects of therapy for childhood leukemia include secondary malignancy, cardiotoxicity, obesity, endocrine abnormalities, reproductive changes, neurocognitive deficits, and psychosocial effects. As clinicians have become more aware of the late effects of therapy, treatment regimens have been changed to decrease late effects, but patients still require long-term follow-up for their prevention and treatment.

Acute kidney injury in symptomatic primary Epstein-Barr virus infectious mononucleosis: Systematic review.

Science.gov (United States)

Moretti, Milena; Lava, Sebastiano A G; Zgraggen, Lorenzo; Simonetti, Giacomo D; Kottanattu, Lisa; Bianchetti, Mario G; Milani, Gregorio P

2017-06-01

Textbooks and reviews do not mention the association of symptomatic primary Epstein-Barr virus infectious mononucleosis with acute kidney injury in subjects without immunodeficiency or autoimmunity. Stimulated by our experience with two cases, we performed a review of the literature. The literature documents 38 cases (26 male and 12 female individuals ranging in age from 0.3 to 51, median 18 years) of symptomatic primary Epstein-Barr virus infectious mononucleosis complicated by acute kidney injury: 27 acute interstitial nephritides, 1 jaundice-associated nephropathy, 7 myositides and 3 hemolytic uremic syndromes. Acute kidney injury requiring renal replacement therapy was observed in 18 (47%) cases. Acute kidney injury did not resolve in one patient with acute interstitial nephritis. Two patients died because of systemic complications. The remaining 35 cases fully recovered. In individuals with acute symptomatic Epstein-Barr virus infectious mononucleosis, a relevant kidney injury is rare but the outcome potentially fatal. It results from interstitial nephritis, myositis-associated acute kidney injury, hemolytic uremic syndrome or jaundice-associated nephropathy. Copyright © 2017 Elsevier B.V. All rights reserved.

Eosinophilic Fasciitis Associated with Myositis

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Yuko Adachi

2015-04-01

Full Text Available Eosinophilic fasciitis is clinically characterized by symmetrical scleroderma-like indurations of the skin with pain. The histological features are fascial inflammation with lymphocytes and eosinophils as well as thickened and fibrotic fascia. Lymphocytic infiltration and degeneration of the underlying muscle are rarely observed. We report a 69-year-old Japanese woman who presented with multiple areas of glossy induration and painful peau d'orange-like lesions on the chest and four extremities. T2-weighted magnetic resonance imaging showed significant hyperintense thickening of the fascia of the lower extremities. Histopathological examination of a biopsy specimen from the induration showed marked fibrinoid degeneration of the fascia and the neighboring muscle with mixed cellular infiltration of lymphocytes and eosinophils. The predominant CD8+ lymphocytic infiltrates were observed by immunohistological study. A diagnosis of eosinophilic fasciitis with myositis was made. Oral administration of prednisolone and discontinuation of exercise significantly improved the lesions and pain.

Corticosteroids in Myositis and Scleroderma

Science.gov (United States)

Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

2017-01-01

Synopsis Idiopathic inflammatory myopathies (IIM) involve inflammation of the muscles and are classified based on the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into four categories: dermatomyositis, polymyositis, inclusion body myositis, and immune mediated necrotizing myopathy. The term “scleroderma” refers to fibrosis of the skin. Localized scleroderma (morphea) is skin-limited, while systemic sclerosis (SSc) is associated with vascular and internal organ involvement. Although there is a paucity of randomized clinical trials, treatment with systemic corticosteroids (CS) is the standard of care for IIM with muscle and organ involvement. The extra-cutaneous features of systemic sclerosis are frequently treated with CS, however high doses have been associated with scleroderma renal crisis in high-risk patients. CS monotherapy is neither recommended for the cutaneous manifestations of dermatomyositis nor scleroderma. While CS can be effective first line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. PMID:26611554

[Focal myositis: An unknown disease].

Science.gov (United States)

Gallay, L; Streichenberger, N; Benveniste, O; Allenbach, Y

2017-10-01

Focal myositis are inflammatory muscle diseases of unknown origin. At the opposite from the other idiopathic inflammatory myopathies, they are restricted to a single muscle or to a muscle group. They are not associated with extramuscular manifestations, and they have a good prognosis without any treatment. They are characterized by a localized swelling affecting mostly lower limbs. The pseudo-tumor can be painful, but is not associated with a muscle weakness. Creatine kinase level is normal. Muscle MRI shows an inflammation restricted to a muscle or a muscle group. Muscle biopsy and pathological analysis remain necessary for the diagnosis, showing inflammatory infiltrates composed by macrophages and lymphocytes without any specific distribution within the muscle. Focal overexpression of HLA-1 by the muscle fibers is frequently observed. The muscle biopsy permits to rule out differential diagnosis such a malignancy (sarcoma). Spontaneous remission occurs within weeks or months after the first symptoms, relapse is unusual. Copyright © 2017. Published by Elsevier SAS.

Relapsed childhood high hyperdiploid acute lymphoblastic leukemia: presence of preleukemic ancestral clones and the secondary nature of microdeletions and RTK-RAS mutations

DEFF Research Database (Denmark)

Davidsson, J; Paulsson, K; Lindgren, D

2010-01-01

Although childhood high hyperdiploid acute lymphoblastic leukemia is associated with a favorable outcome, 20% of patients still relapse. It is important to identify these patients already at diagnosis to ensure proper risk stratification. We have investigated 11 paired diagnostic and relapse...

Parental Perceptions of Obesity and Obesity Risk Associated With Childhood Acute Lymphoblastic Leukemia.

Science.gov (United States)

Jones, Gary L; McClellan, Wendy; Raman, Sripriya; Sherman, Ashley; Guest, Erin; August, Keith

2017-07-01

The prevalence of obesity and related comorbidities in survivors of childhood acute lymphoblastic leukemia (ALL) is well established and ranges anywhere from 29% to 69% depending on the study. We sought to explore the awareness of parents of survivors of childhood ALL regarding the increased risk of obesity and their perceptions regarding the overall health of their child. One hundred twenty-one parents of 99 survivors of pediatric ALL completed surveys regarding perceptions of obesity risk in survivors. Eighty percent of parents of overweight and obese survivors correctly identified their child as "a little overweight" or "overweight." Few parents recalled discussing weight gain (21%) or obesity risk (36%) with their practitioner. Parents that did recall having these discussions and/or reported a decreased level of posttherapy activity in their child were more likely to be concerned about their child's weight status. Improved awareness and education regarding the risk of obesity and associated comorbid conditions may provide an avenue for future prevention of obesity in survivors of pediatric ALL. Discussion and education regarding a healthy lifestyle, including proper diet and exercise, should be incorporated early in routine patient visits.

Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placebo

NARCIS (Netherlands)

Badrising, UA; Maat-Schieman, MLC; Ferrari, MD; Zwinderman, AH; Wessels, JAM; Breedveld, FC; van Doorn, PA; van Engelen, BGM; Hoogendijk, JE; Howeler, CJ; de Jager, AE; Jennekens, FGI; Koehler, PJ; de Visser, M; Viddeleer, A; Verschuuren, JJ; Wintzen, AR

We investigated whether 5 to 20mg per week oral methotrexate could slow down disease progression in 44 patients with inclusion body myositis in a randomized double-blind placebo-controlled study over 48 weeks. Mean change of quantitative muscle strength testing sum scores was the primary study

ATF5 polymorphisms influence ATF function and response to treatment in children with childhood acute lymphoblastic leukemia

OpenAIRE

Rousseau, Julie; Gagné, Vincent; Labuda, Malgorzata; Beaubois, Cyrielle; Sinnett, Daniel; Laverdière, Caroline; Moghrabi, Albert; Sallan, Stephen E.; Silverman, Lewis B.; Neuberg, Donna; Kutok, Jeffery L.; Krajinovic, Maja

2011-01-01

Asparaginase is a standard and critical component in the therapy of childhood acute lymphoblastic leukemia. Asparagine synthetase (ASNS) and the basic region leucine zipper activating transcription factor 5 (ATF5) and arginosuccinate synthase 1 (ASS1) have been shown to mediate the antileukemic effect of asparaginase and to display variable expression between leukemia cells that are resistant and sensitive to treatment. Fourteen polymorphisms in the regulatory and coding regions of these gene...

Myositis Ossificans Progressiva in the Whole Spine: A Case Report

Directory of Open Access Journals (Sweden)

Ebrahim Ghayem Hasankhani

2018-02-01

Full Text Available Myositis ossificans progressiva is a rare inherited disease characterized by progressive ectopic ossifications associated with thumb and big toe anomalies. Ossification usually progresses from central to the peripheral, proximal to distal, cranial to caudal, and from dorsal to ventral directions and leading to activity limitation, significant eating disability, recurrent pulmonary infection, and atelectasis. In this report, we present a 7-year-old boy with a total spine stiffness (wooden spine seriously limited his activity of daily living.

Relapsed childhood high hyperdiploid acute lymphoblastic leukemia: presence of preleukemic ancestral clones and the secondary nature of microdeletions and RTK-RAS mutations

DEFF Research Database (Denmark)

Davidsson, J; Paulsson, K; Lindgren, D

2010-01-01

Although childhood high hyperdiploid acute lymphoblastic leukemia is associated with a favorable outcome, 20% of patients still relapse. It is important to identify these patients already at diagnosis to ensure proper risk stratification. We have investigated 11 paired diagnostic and relapse samp...

[Clinical features and therapeutic response of our anti-SRP positive patients with myositis].

Science.gov (United States)

Botos, Balázs; Nagy-Vincze, Melinda; Dankó, Katalin

2017-09-01

Idiopathic inflammatory myopathies are a group of clinically heterogeneous diseases, which have been classified by myositis specific antibodies recently. The anti-SRP positive subset of this group is characterized by more severe clinical prognosis than other myositis specific antibody positive types. Our goal was to compare 16 anti-SRP positive patients in the Division of Clinical Immunology, Department of Internal Medicine, University of Debrecen with 16 antibody negative ones. Muscle strength validated in both groups by the manual muscle test proved to be significantly decreased both before and after therapy (χ 2 = 0.006 and 0.019) in the anti-SRP positive group. Muscle-specific inflammatory laboratory parameters showed significant difference only in case of LDH-levels after therapy. Both groups showed good clinical response to first line steroid treatment, yet the significantly higher rate of second line administration suggests worse therapeutic response of the antibody positive group. Based on these facts we determined poor clinical prognosis and therapeutic response of the anti-SRP positive group. Orv Hetil. 2017; 158(35): 1382-1389.

Impact of Childhood Nutritional Status on Pathogen Prevalence and Severity of Acute Diarrhea.

Science.gov (United States)

Tickell, Kirkby D; Pavlinac, Patricia B; John-Stewart, Grace C; Denno, Donna M; Richardson, Barbra A; Naulikha, Jaqueline M; Kirera, Ronald K; Swierczewski, Brett E; Singa, Benson O; Walson, Judd L

2017-11-01

Children with acute and chronic malnutrition are at increased risk of morbidity and mortality following a diarrheal episode. To compare diarrheal disease severity and pathogen prevalence among children with and without acute and chronic malnutrition, we conducted a cross-sectional study of human immunodeficiency virus-uninfected Kenyan children aged 6-59 months, who presented with acute diarrhea. Children underwent clinical and anthropometric assessments and provided stool for bacterial and protozoal pathogen detection. Clinical and microbiological features were compared using log binomial regression among children with and without wasting (mid-upper arm circumference ≤ 125 mm) or stunting (height-for-age z score ≤ -2). Among 1,363 children, 7.0% were wasted and 16.9% were stunted. After adjustment for potential confounders, children with wasting were more likely than nonwasted children to present with at least one Integrated Management of Childhood Illness danger sign (adjusted prevalence ratio [aPR]: 1.3, 95% confidence interval [CI]: 1.0 to 1.5, P = 0.05), severe dehydration (aPR: 2.4, 95% CI: 1.5 to 3.8, P malnutrition which may be explained by a delay in care-seeking or diminished immune response to infection. Combating social determinants and host risk factors associated with severe disease, rather than specific pathogens, may reduce the disparities in poor diarrhea-associated outcomes experienced by malnourished children.

Early diagnosis of myositis ossificans with Tc-99m diphosphonate imaging

International Nuclear Information System (INIS)

Tyler, J.L.; Derbekyan, V.; Lisbona, R.

1984-01-01

Myositis ossificans is primarily a disorder of young adults, whereby an area of muscle mass undergoes progressive ossification. The authors review a case in which the patient's presentation was somewhat atypical, and where the course of disease was unusually prolonged. Examination of the soft tissue lesion using Tc-99m diphosphonate bone scans was helpful in establishing the diagnosis and in determining the full extent of the process early in its evolution

Leukemic blasts are present at low levels in spinal fluid in one-third of childhood acute lymphoblastic leukemia cases

DEFF Research Database (Denmark)

Levinsen, Mette; Marquart, Hanne V; Groth-Pedersen, Line

2016-01-01

BACKGROUND: Central nervous system (CNS) involvement is associated with relapse in childhood acute lymphoblastic leukemia (ALL) and is a diagnostic challenge. PROCEDURE: In a Nordic/Baltic prospective study, we assessed centralized flow cytometry (FCM) of locally fixed cerebrospinal fluid (CSF......: 45 × 10(9) /l vs. 10 × 10(9) /l, P diagnosis remained so despite at least two doses...

A fatal case of metastatic squamous cell carcinoma in a patient with myositis ossificans traumatica.

NARCIS (Netherlands)

Vlasveld, I N; Scheper, H; Stalenhoef, J; Baas, J M; van Dissel, J

Myositis ossificans traumatica is a rare disease associated with chronic wounds and fistulae. Chronic ulcers, fistulae and wounds can transform into squamous cell carcinoma, the so-called Marjolin's ulcer. We describe a rapid, progressive and fulminant course of a metastatic squamous cell carcinoma

[Cytopathologic features of childhood acute leukemia at the Hospital de Especialidades Pediátricas, Chiapas, Mexico].

Science.gov (United States)

Lepe-Zúñiga, José Luis; Jerónimo-López, Francisco Javier; Hernández-Orantes, Jorge Gregorio

Childhood acute leukemia cytological features are unknown in Chiapas, Mexico. Defining these features is important because this is a relatively isolated population with high consanguinity index, and these aspects could determine differences in responses to treatment and outcome. Eighty-one childhood acute leukemia cases treated at the Hospital de Especialidades Pediátricas in Chiapas were characterized by morphology, immunophenotype, genotype, initial risk assignment and status at the time of the study. The proportion of leukemic cell types found in this study was B cell, 75.3%; myeloid, 16%; T cell, 3.7% and NK 1.2%. In B cell leukemia, genetic alterations were present in 40.6% of cases and had a specific outcome regardless of initial risk assessment. Cases with MLL gene alteration died within a month from diagnosis. Translocations were present in 17.5% B cases; t(1;19) was present in those with a favorable outcome. The t(12;21) translocation was related to initial remission and midterm relapse and dead. Hyperdiploidy was present in 20% of B cell cases with good outcome. In 38.5%of myeloid cases were translocations and karyotypic abnormalities. Short-term outcome in this group has been poor; 69% have died or abandoned treatment in relapse from 15 days to 37 months after diagnosis. Relative frequency of different types of acute leukemia in patients treated at a tertiary level pediatric hospital in Chiapas, Mexico, was similar to the one found in other parts of the country. Patients' outcome, under a standardized treatment, differs according to the group, the subgroup and the presence and type of genetic alterations. Copyright © 2017 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

The importance of monitoring minimal residual disease in childhood acute lymphoblastic leukemia

International Nuclear Information System (INIS)

Kolenova, A.; Subova, Z.; Cizmar, A.; Sejnova, D.; Kaiserova, E.; Hikkel, I.; Hikkelova, M.; Bubanska, E.; Oravkinova, I.

2012-01-01

Since the strong correlation between minimal residual disease (MRD) levels and risk of relapse in childhood acute lymphoblastic leukemia, monitoring of MRD provides unique information regarding treatment response. Because the significance of MRD monitoring has been strongly supported by several studies and because it has been implemented in the latest protocols, there has been a significant effort to develop MRD monitoring in the Slovak Republic. Between 1. 10. 2006 and 31. 12. 2009, 50 children with ALL who were treated at three Slovak centers were included in the RQ PCR MRD pilot project. Based on MRD stratification, we identified 26 patients who were stratified into the HRG (high risk group) 3 patients (11,5 %), IRG (intermediate risk group), 14 p. 54 % and SRG (standard risk group), 9 p. (34,5 %). (author)

The Circadian Schedule for Childhood Acute Lymphoblastic Leukemia Maintenance Therapy does not Influence Event-Free Survival in the NOPHO ALL92 Protocol

DEFF Research Database (Denmark)

Clemmensen, Kim K. B.; Christensen, Regitse H.; Shabaneh, Diana N.

2014-01-01

BACKGROUND: The event-free survival of childhood acute lymphoblastic leukemia (ALL) has been reported to be superior when oral methotrexate (MTX) and 6-mercaptopurine (6MP) maintenance therapy (MT) is administered in the evening compared to the morning. PROCEDURE: In the ALL92 MT study we prospec...

Health-related quality of life assessment in Indonesian childhood acute lymphoblastic leukemia

Directory of Open Access Journals (Sweden)

Sutaryo

2008-11-01

Full Text Available Abstract Background Most studies on Health-related Quality of Life (HRQOL in children with cancer were conducted in developed countries. The aims of this study were to assess the HRQOL in childhood acute lymphoblastic leukemia (ALL patients in Indonesia and to assess the influence of demographic and medical characteristics on HRQOL. Methods After cultural linguistic validation, a cross-sectional study of HRQOL was conducted with childhood ALL patients and their guardians in various phases of treatment using the Pediatric Quality of Life Inventory™ (PedsQL™ 4.0 Generic Core Scale and the Pediatric Quality of Life Inventory™ (PedsQL™ 3.0 Cancer Module. Results Ninety-eight guardians and 55 patients participated. The internal consistency of both scales ranged from 0.57 to 0.92. HRQOL of Indonesian patients was comparable with those in developed countries. There were moderate to good correlations between self-reports and proxy-reports, however guardians tended to report worse HRQOL than patients. Children of the 2–5 year-group significantly had more problems in procedural anxiety, treatment anxiety and communication subscales than in older groups (p Conclusion Younger children had more problems in procedural anxiety, treatment anxiety and communication subscales. Therefore, special care during intervention procedures is needed to promote their normal development. Psychosocial support should be provided to children and their parents to facilitate their coping with disease and its treatment.

Increased μ-Calpain Activity in Blasts of Common B-Precursor Childhood Acute Lymphoblastic Leukemia Correlates with Their Lower Susceptibility to Apoptosis.

Directory of Open Access Journals (Sweden)

Anna Mikosik

Full Text Available Childhood acute lymphoblastic leukemia (ALL blasts are characterized by inhibited apoptosis promoting fast disease progress. It is known that in chronic lymphocytic and acute myeloid leukemias the reduced apoptosis is strongly related with the activity of calpain-calpastatin system (CCS composed of cytoplasmic proteases--calpains--performing the modulatory proteolysis of key proteins involved in cell proliferation and apoptosis, and of their endogenous inhibitor--calpastatin. Here, the CCS protein abundance and activity was for the first time studied in childhood ALL blasts and in control bone marrow CD19+ B cells by semi-quantitative flow cytometry and western blotting of calpastatin fragments resulting from endogenous calpain activity. Significantly higher μ-calpain (CAPN1 gene transcription, protein amounts and activity (but not those of m-calpain, with calpastatin amount and transcription of its gene (CAST greatly varying were observed in CD19(+ ALL blasts compared to control cells. Significant inverse relation between the amount/activity of calpain and spontaneous apoptosis was noted. Patients older than 10 years (considered at higher risk displayed increased amounts and activities of blast calpain. Finally, treatment of blasts with the tripeptide calpain inhibitors II and IV significantly and in dose-dependent fashion increased the percentage of blasts entering apoptosis. Together, these findings make the CCS a potential new predictive tool and therapeutic target in childhood ALL.

Using adaptive model predictive control to customize maintenance therapy chemotherapeutic dosing for childhood acute lymphoblastic leukemia.

Science.gov (United States)

Noble, Sarah L; Sherer, Eric; Hannemann, Robert E; Ramkrishna, Doraiswami; Vik, Terry; Rundell, Ann E

2010-06-07

Acute lymphoblastic leukemia (ALL) is a common childhood cancer in which nearly one-quarter of patients experience a disease relapse. However, it has been shown that individualizing therapy for childhood ALL patients by adjusting doses based on the blood concentration of active drug metabolite could significantly improve treatment outcome. An adaptive model predictive control (MPC) strategy is presented in which maintenance therapy for childhood ALL is personalized using routine patient measurements of red blood cell mean corpuscular volume as a surrogate for the active drug metabolite concentration. A clinically relevant mathematical model is developed and used to describe the patient response to the chemotherapeutic drug 6-mercaptopurine, with some model parameters being patient-specific. During the course of treatment, the patient-specific parameters are adaptively identified using recurrent complete blood count measurements, which sufficiently constrain the patient parameter uncertainty to support customized adjustments of the drug dose. While this work represents only a first step toward a quantitative tool for clinical use, the simulated treatment results indicate that the proposed mathematical model and adaptive MPC approach could serve as valuable resources to the oncologist toward creating a personalized treatment strategy that is both safe and effective. Copyright (c) 2010 Elsevier Ltd. All rights reserved.

A rare case of Enterococcus faecalis-induced orbital cellulitis and myositis

Directory of Open Access Journals (Sweden)

Piyush Kohli

2016-01-01

Full Text Available Orbital cellulitis is an infection of soft tissue behind the orbital septum. Common pathogens isolated include Staphylococcus aureus, Staphylococcus epidermidis, and Streptococcus pneumoniae. It is a straightforward diagnosis and usually responds to empirical treatment without any sequela. We report a case of orbital cellulitis caused by Enterococcus faecalis, which was complicated by myositis of levator palpebrae superioris. To the best of our knowledge, only one case report exists dating way back to 1986.

A Unique Case of Muscle Invasive Metastatic Breast Cancer Mimicking Myositis

Science.gov (United States)

2017-06-28

TYPE 08/ 03/20 17 Publ ication/Journal 4. TITLE AND SUBTITLE A unique case of muscle-invasive metastatic breast cancer mimicking myositis 6...Rev. 8/98) Prescnbed by ANSI Std Z39. 18 Adobe Profes11on11 7.0 Title: A Unique Case of M uscle-Invasive Metastatic Breast Cancer M imicking...an 84-year-old female who presented with neck swelling and upper airway obstruction due to metastatic breast cancer invading the sternocleidomastoid

Novel assessment tools to evaluate clinical and laboratory responses in a subset of patients enrolled in the Rituximab in Myositis trial.

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Rider, Lisa G; Yip, Adrienne L; Horkayne-Szakaly, Iren; Volochayev, Rita; Shrader, Joseph A; Turner, Maria L; Kong, Heidi H; Jain, Minal S; Jansen, Anna V; Oddis, Chester V; Fleisher, Thomas A; Miller, Frederick W

2014-01-01

We aimed to assess changes in myositis core set measures and ancillary clinical and laboratory data from the National Institutes of Health's subset of patients enrolled in the Rituximab in Myositis trial. Eighteen patients (5 dermatomyositis, 8 polymyositis, 5 juvenile dermatomyositis) completed more in-depth testing of muscle strength and cutaneous assessments, patient-reported outcomes, and laboratory tests before and after administration of rituximab. Percentage change in individual measures and in the definitions of improvement (DOIs) and standardized response means were examined over 44 weeks. Core set activity measures improved by 18-70% from weeks 0-44 and were sensitive to change. Fifteen patients met the DOI at week 44, 9 patients met a DOI 50% response, and 4 met a DOI 70% response. Muscle strength and function measures were more sensitive to change than cutaneous assessments. Constitutional, gastrointestinal, and pulmonary systems improved 44-70%. Patient-reported outcomes improved up to 28%. CD20+ B cells were depleted in the periphery, but B cell depletion was not associated with clinical improvement at week 16. This subset of patients had high rates of clinical response to rituximab, similar to patients in the overall trial. Most measures were responsive, and muscle strength had a greater degree of change than cutaneous assessments. Several novel assessment tools, including measures of strength and function, extra-muscular organ activity, fatigue, and health-related quality of life, are promising for use in future myositis trials. Further study of B cell-depleting therapies in myositis, particularly in treatment-naïve patients, is warranted.

Acute traumatic subdural hematoma in infancy and childhood classification and treatment from CT findings

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Mochimatsu, Yasuhiko

1988-11-01

Acute traumatic subdural hematoma in infancy and childhood has much difference from that in adult on their symptom, clinical course, and indication of surgical treatment. The aim of this study is to examine their clinical course and CT findings just after the injury and to evaluate the treatment modality for types of complex this disease. As the result of these examination, SDH patients are divided into five categories in account of their CT findings, especially according to the relationship between the hemispheric swelling and the amount of SDH. Simple SDH type shows classical clinical course and surgical treatment are frequently essential in rapidly progressive cases. Isodensity hemispheric swelling (IHS) type is more frequently seen in CT findings which shows thin SDH and prominent brain swelling. This IHS (a subtype of diffuse brain injury) type should be recognized for their favourable outcome under conservative treatment. Other three types are; contusion with SDH, acute excerbation of chronic SDH, and battered child. Indication of surgical treatment will be decided considering to the volume of hematomas. (author).

Acute traumatic subdural hematoma in infancy and childhood classification and treatment from CT findings

International Nuclear Information System (INIS)

Mochimatsu, Yasuhiko

1988-01-01

Acute traumatic subdural hematoma in infancy and childhood has much difference from that in adult on their symptom, clinical course, and indication of surgical treatment. The aim of this study is to examine their clinical course and CT findings just after the injury and to evaluate the treatment modality for types of complex this disease. As the result of these examination, SDH patients are divided into five categories in account of their CT findings, especially according to the relationship between the hemispheric swelling and the amount of SDH. Simple SDH type shows classical clinical course and surgical treatment are frequently essential in rapidly progressive cases. Isodensity hemispheric swelling (IHS) type is more frequently seen in CT findings which shows thin SDH and prominent brain swelling. This IHS (a subtype of diffuse brain injury) type should be recognized for their favourable outcome under conservative treatment. Other three types are; contusion with SDH, acute excerbation of chronic SDH, and battered child. Indication of surgical treatment will be decided considering to the volume of hematomas. (author)

Family characteristics as risk factors for childhood acute lymphoblastic leukemia: a population-based case-control study.

Directory of Open Access Journals (Sweden)

Martin Feller

Full Text Available BACKGROUND: To date, few risk factors for childhood acute lymphoblastic leukemia (ALL have been confirmed and the scientific literature is full of controversial "evidence." We examined if family characteristics, particularly maternal and paternal age and number of older siblings, were risk factors for childhood acute lymphoblastic leukemia (ALL. METHODOLOGY/PRINCIPAL FINDINGS: In this population-based nationwide matched case-control study, patients 0-14 years of age with ALL diagnosed 1991-2006 and registered in the Swiss Childhood Cancer Registry were linked with their census records of 1990 and 2000. Eight controls per case were selected from the census. The association between family characteristics and ALL was analyzed by conditional logistic regressions. We found that increasing maternal age was associated with incidence of ALL in the offspring (OR per 5-year increase in maternal age 1.18, 95% CI 1.05-1.31; p = 0.004, remaining stable (trend OR 1.14, 95% CI 0.99-1.31; p = 0.060 after adjustment for other risk factors. The association with paternal age was weaker (OR per 5-year increase 1.14, 95% CI 1.01-1.28, p = 0.032 and disappeared after adjustments. Number of older siblings was not associated with risk of ALL in the overall group of children aged 0-14 years at diagnosis. However, we found a negative trend between number of older siblings and ALL diagnosed at age 0-4 years (OR per sibling 0.85, 95% CI 0.68-1.06; p = 0.141 and a positive trend for ALL diagnosed at age 5-9 (OR 1.34, 95% CI 1.05-1.72; p = 0.019, with some evidence for an effect modification (p-value for interaction  = 0.040. CONCLUSIONS: As in other studies, increasing maternal, but not paternal age was associated with risk of ALL. We found only a weak association with the number of older siblings, suggesting a delay in disease manifestation rather than a decrease in incidence.

Methotrexate resistance in relation to treatment outcome in childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Wojtuszkiewicz, Anna; Peters, Godefridus J; van Woerden, Nicole L

2015-01-01

BACKGROUND: Methotrexate (MTX) eradicates leukemic cells by disrupting de novo nucleotide biosynthesis and DNA replication, resulting in cell death. Since its introduction in 1947, MTX-containing chemotherapeutic regimens have proven instrumental in achieving curative effects in acute lymphoblast...... resistant to MTX at diagnosis may allow for tailoring novel treatment strategies to individual leukemia patients....... leukemia (ALL). However, drug resistance phenomena pose major obstacles to efficacious ALL chemotherapy. Moreover, clinically relevant molecular mechanisms underlying chemoresistance remain largely obscure. Several alterations in MTX metabolism, leading to impaired accumulation of this cytotoxic agent...... in tumor cells, have been classified as determinants of MTX resistance. However, the relation between MTX resistance and long-term clinical outcome of ALL has not been shown previously. METHODS: We have collected clinical data for 235 childhood ALL patients, for whom samples taken at the time of diagnosis...

HA-1 T TCR T Cell Immunotherapy for the Treating of Patients With Relapsed or Refractory Acute Leukemia After Donor Stem Cell Transplant

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2018-04-30

HLA-A*0201 HA-1 Positive Cells Present; Minimal Residual Disease; Recurrent Acute Biphenotypic Leukemia; Recurrent Acute Undifferentiated Leukemia; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Acute Myeloid Leukemia; Refractory Adult Acute Lymphoblastic Leukemia; Refractory Childhood Acute Lymphoblastic Leukemia

Statin-induced focal myositis of the upper extremity. A report of two cases

International Nuclear Information System (INIS)

Wagner, M.; Muehldorfer-Fodor, M.; Prommersberger, K.J.; Schmitt, R.

2011-01-01

Statins are widely used to lower increased cholesterol levels with the aim to prevent major cardiovascular events. However, they bare the risk of myotoxic side effects. We report on two patients with focal weakness and pain in the upper extremities. In both patients, abnormal MRI signal heights in the muscle groups involved were indicative of the final diagnosis of focal myositis during statin therapy.

Statin-induced focal myositis of the upper extremity. A report of two cases

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Wagner, M., E-mail: wagner.radiologie@herzchirurgie.de [Department of Radiology, Herz- und Gefaessklinik GmbH, Salzburger Leite 1, D-97616 Bad Neustadt an der Saale (Germany); Muehldorfer-Fodor, M.; Prommersberger, K.J. [Department of Handsurgery, Herz- und Gefaessklinik GmbH, Bad Neustadt an der Saale (Germany); Schmitt, R. [Department of Radiology, Herz- und Gefaessklinik GmbH, Salzburger Leite 1, D-97616 Bad Neustadt an der Saale (Germany)

2011-02-15

Statins are widely used to lower increased cholesterol levels with the aim to prevent major cardiovascular events. However, they bare the risk of myotoxic side effects. We report on two patients with focal weakness and pain in the upper extremities. In both patients, abnormal MRI signal heights in the muscle groups involved were indicative of the final diagnosis of focal myositis during statin therapy.

Inflammatory muscle diseases (myositis); Entzuendliche Muskelerkrankungen (Myositiden)

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Vahlensieck, M. [Praxisnetz Radiologie und Nuklearmedizin Bonn Bad Godesberg Rhein Sieg, Bonn (Germany)

2017-12-15

Inflammatory muscle diseases are quite heterogeneous and frequently a specific diagnosis is not easy. Magnetic resonance imaging (MRI) is best suited to demonstrate pattern and size of inflamed muscles. Sensitivity of MRI in showing inflamed muscles is nearly 100%. Specificity, however, is much lower. In cases of suspected bacterial myositis, sonography may be used to exclude abscess formation prior to MRI for more precise information about inflamed muscles. (orig.) [German] Entzuendliche Muskelerkrankungen stellen eine heterogene Gruppe dar und sind mitunter nur schwer exakt zu diagnostizieren. Die Magnetresonanztomographie (MRT) stellt Ausdehnung und Befallsmuster am besten dar. Der Nachweis entzuendlicher Oedeme oder von Einschmelzungen gelingt mit der MRT in annaehernd 100 % der Faelle. Die Spezifitaet der Methode ist allerdings deutlich geringer. Bei Verdacht auf bakterielle Myositiden kommt erst die Sonographie zum Ausschluss von Einschmelzungen und dann die MRT zum Einsatz. (orig.)

Redefining dermatomyositis: a description of new diagnostic criteria that differentiate pure dermatomyositis from overlap myositis with dermatomyositis features.

Science.gov (United States)

Troyanov, Yves; Targoff, Ira N; Payette, Marie-Pier; Raynauld, Jean-Pierre; Chartier, Suzanne; Goulet, Jean-Richard; Bourré-Tessier, Josiane; Rich, Eric; Grodzicky, Tamara; Fritzler, Marvin J; Joyal, France; Koenig, Martial; Senécal, Jean-Luc

2014-11-01

Dermatomyositis (DM) is a major clinical subset of autoimmune myositis (AIM). The characteristic DM rash (Gottron papules, heliotrope rash) and perifascicular atrophy at skeletal muscle biopsy are regarded as specific features for this diagnosis. However, new concepts are challenging the current definition of DM. A modified Bohan and Peter classification of AIM was proposed in which the core concept was the inclusion of the diagnostic significance of overlap connective tissue disease features. In this clinical classification, a DM rash in association with myositis in the absence of overlap features indicates a diagnosis of pure DM. However, overlap features in association with myositis allow a diagnosis of overlap myositis (OM), irrespective of the presence or absence of the DM rash. Perifascicular atrophy may be present in both pure DM and OM. Recently, the presence of perifascicular atrophy in myositis without a DM rash was proposed as diagnostic of a novel entity, adermatopathic DM. We conducted the present study to evaluate these new concepts to further differentiate pure DM from OM.Using the modified Bohan and Peter classification, we performed a follow-up study of a longitudinal cohort of 100 consecutive adult French Canadian patients with AIM, including 44 patients with a DM phenotype, defined as a DM rash, and/or DM-type calcinosis, and/or the presence of perifascicular atrophy on muscle biopsy. A detailed evaluation was performed for overlap features, the extent and natural history of the DM rash, adermatopathic DM, DM-specific and overlap autoantibodies by protein A immunoprecipitation on coded serum samples, and associations with cancer and survival.Two distinct subsets were identified in patients with a DM phenotype: pure DM (n = 24) and OM with DM features, or OMDM (n = 20). In pure DM, the DM rash was a dominant finding. It was the first disease manifestation, was always present at the time of myositis diagnosis, and was associated with a high

Expression of BAFF receptors in muscle tissue of myositis patients with anti-Jo-1 or anti-Ro52/anti-Ro60 autoantibodies.

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Kryštůfková, Olga; Barbasso Helmers, Sevim; Venalis, Paulius; Malmström, Vivianne; Lindroos, Eva; Vencovský, Jiří; Lundberg, Ingrid E

2014-10-10

Anti-Jo-1 and anti-Ro52 autoantibodies are common in patients with myositis, but the mechanisms behind their production are not known. Survival of autoantibody-producing cells is dependent on B-cell-activating factor of the tumour necrosis factor family (BAFF). BAFF levels are elevated in serum of anti-Jo-1-positive myositis patients and are influenced by type-I interferon (IFN). IFN-producing cells and BAFF mRNA expression are present in myositis muscle. We investigated expression of the receptors for BAFF in muscle tissue in relation to anti-Jo-1 and anti-Ro52/anti-Ro60 autoantibodies and type-I IFN markers. Muscle biopsies from 23 patients with myositis selected based on autoantibody profile and 7 healthy controls were investigated for expression of BAFF receptor (BAFF-R), B-cell maturation antigen (BCMA) and transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI). Nineteen samples were assessed for plasma (CD138) and B-cell (CD19) markers. The numbers of positive cells per area were compared with the expression of plasmacytoid dendritic cell (pDC) marker blood dendritic cell antigen-2 (BDCA-2) and IFNα/β-inducible myxovirus resistance-1 protein (MX-1). BAFF-R, BCMA and TACI were expressed in five, seven and seven patients, respectively, and more frequently in anti-Jo-1-positive and/or anti-Ro52/anti-Ro60-positive patients compared to controls and to patients without these autoantibodies (P = BAFF-R: 0.007, BCMA: 0.03 and TACI: 0.07). A local association of receptors with B and plasma cells was confirmed by confocal microscopy. The numbers of CD138-positive and BCMA-positive cells were correlated (r = 0.79; P = 0.001). Expression of BDCA-2 correlated with numbers of CD138-positive cells and marginally with BCMA-positive cells (r = 0.54 and 0.42, respectively; P = 0.04 and 0.06, respectively). There was a borderline correlation between the numbers of positively stained TACI cells and MX-1 areas (r = 0.38, P = 0.08). The expression

Childhood acute bacterial meningitis: clinical spectrum, bacteriological profile and outcome

International Nuclear Information System (INIS)

Bari, A.; Zeeshan, S.; Rathore, A. W.

2016-01-01

Objective: To determine the disease pattern, etiological agents and outcome of childhood acute bacterial meningitis. Study Design: A descriptive study. Place and Duration of Study: Department of Paediatric Medicine, The Children's Hospital, Lahore, from January to December 2012. Methodology: A total of 199 children between the ages of 1 month and 5 years, admitted with the diagnosis of meningitis on the basis of clinical findings and positive cerebrospinal fluid (CSF), were included. In all patients, complete blood count (CBC), CSF culture sensitivity, and blood culture sensitivity were performed. Data was analysed using SPSS version 20. Results: Out of 199 children, 127 (63.8%) were males with M:F ratio of 1.7:1. Mean age was 11.33 ± 12 months. Maximum numbers of children were < 1 year of age, 136 (68.3%). Only 90 (45.2%) children were fully vaccinated according to Expanded Program of Immunisation (EPI) schedule. Presentations with refusal to take feed (p=0.008) and with impaired conscious state were independent predictors of death (p=0.002). Complications were noted in 34 (17%) and were significantly associated with severe malnutrition (p=0.006) and altered conscious level at presentation (p < 0.001). The common pathogens identified on CSF culture were coagulase negative staphylococci (CoNS) in 11 (5.5%) and streptococcus pneumoniae in 5 (2.5%). Overall mortality was 10.1%. The commonest pathogen isolated from children who died was streptococcus pneumoniae (p=0.039). Conclusion: Acute bacterial meningitis mostly affected children under the age of 1 year. CSF culture revealed both Gram-positive and Gram-negative bacteria. The most common pathogen in children who died was streptococcus pneumoniae. (author)

Vaccine-induced myositis with intramuscular sterile abscess formation: MRI and ultrasound findings

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Polat, Ahmet Veysel; Bekci, Tumay; Selcuk, Mustafa Bekir [Ondokuz Mayis University, Department of Radiology, Faculty of Medicine, Samsun (Turkey); Dabak, Nevzat [Ondokuz Mayis University, Department of Orthopaedics and Traumatology, Faculty of Medicine, Samsun (Turkey); Ulu, Esra Meltem Kayahan [Samsun Medical Park Hospital, Department of Radiology, Samsun (Turkey)

2015-12-15

Although limb swelling is a well-known complication of vaccination, its rarity and wide band of differential diagnosis of limb swelling make it a diagnostic challenge. In this case report, we describe three cases of vaccine-induced myositis with intramuscular sterile abscess formation in patients with limb swelling and their magnetic resonance imaging and ultrasonography findings. Both radiologists and clinicians should be familiar with this rare entity, its clinical and imaging spectrum, and follow-up strategies. (orig.)

Vaccine-induced myositis with intramuscular sterile abscess formation: MRI and ultrasound findings

International Nuclear Information System (INIS)

Polat, Ahmet Veysel; Bekci, Tumay; Selcuk, Mustafa Bekir; Dabak, Nevzat; Ulu, Esra Meltem Kayahan

2015-01-01

Although limb swelling is a well-known complication of vaccination, its rarity and wide band of differential diagnosis of limb swelling make it a diagnostic challenge. In this case report, we describe three cases of vaccine-induced myositis with intramuscular sterile abscess formation in patients with limb swelling and their magnetic resonance imaging and ultrasonography findings. Both radiologists and clinicians should be familiar with this rare entity, its clinical and imaging spectrum, and follow-up strategies. (orig.)

Heterotopic ossification (myositis ossificans) in acquired immune deficiency syndrome. Detection by gallium scintigraphy

International Nuclear Information System (INIS)

Drane, W.E.; Tipler, B.M.

1987-01-01

A case of heterotopic ossification (myositis ossificans) secondary to the central nervous system complications of acquired immune deficiency syndrome (AIDS) is reported. Because of the overwhelming suspicion of infection in this patient, this diagnosis was not considered until a gallium scan revealed the typical findings of heterotopic ossification. Because of the increasing utilization of gallium imaging in the AIDS population, every imaging specialist should be aware of this potential disorder

Myositis-specific autoantibodies and their association with malignancy in Italian patients with polymyositis and dermatomyositis.

Science.gov (United States)

Ceribelli, Angela; Isailovic, Natasa; De Santis, Maria; Generali, Elena; Fredi, Micaela; Cavazzana, Ilaria; Franceschini, Franco; Cantarini, Luca; Satoh, Minoru; Selmi, Carlo

2017-02-01

This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis. In six dermatomyositis cases, we found five solid forms of cancer and one Hodgkin's lymphoma in long-term remission. Among patients with cancer-associated dermatomyositis, three were positive for anti-TIF1γ/α, two for anti-Mi-2, and one for anti-MJ/NXP-2. The strongest positivity of anti-TIF1γ was seen in two active forms of cancer, and this antibody was either negative or positive at low titers in the absence of cancer or in the 7-year remission Hodgkin's lymphoma. Four out of twenty (20 %) patients with polymyositis had solid cancer, but no specific association with autoantibodies was identified; further, none of the four cases of antisynthetase syndrome had a history of cancer. No serum myositis-associated autoantibody was observed in control sera, resulting in positive predictive value 75 %, negative predictive value 78.5 %, sensitivity 50 %, specificity 92 %, and area under the ROC curve 0.7083 for the risk of paraneoplastic DM in anti-TIF1γ/α (+) patients. Myositis-specific autoantibodies can be identified thanks to the use of immunoprecipitation, and their association with cancer is particularly clear for anti-TIF1γ/α in dermatomyositis. This association should be evaluated in a prospective study by immunoprecipitation in clinical practice.

''Dropped-head'' syndrome due to isolated myositis of neck extensor muscles: MRI findings

Energy Technology Data Exchange (ETDEWEB)

Gaeta, Michele; Mazziotti, Silvio; Blandino, Alfredo [University of Messina, Department of Radiological Sciences, Messina (Italy); Toscano, Antonio; Rodolico, Carmelo; Mazzeo, Anna [University of Messina, Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

2006-02-15

MRI findings of a patient with dropped-head syndrome due to focal myositis of the neck extensor muscles are presented. MRI showed oedematous changes and marked enhancement of the neck extensor muscles. After therapy MRI demonstrated disappearance of the abnormal findings. (orig.)

[Spontaneous bile duct perforation: a rare cause of acute abdominal pain during childhood].

Science.gov (United States)

Ozdemir, Tunç; Akgül, Ahsen Karagözlü; Arpaz, Yağmur; Arikan, Ahmet

2008-07-01

Spontaneous perforation of the bile duct (SPBD) is a rare cause of acute abdominal pain during childhood. Pancreatico-biliary malfunction has been postulated to contribute to its etiology. Factors related to diagnosis and treatment and difference from the other common causes of acute abdominal pain are emphasized. Five patients (3 boys, 2 girls, mean age 4.6) were admitted with peritonitis and operated with initial diagnosis of perforated appendicitis. During laparotomy, SPBD was detected. Presentation, laboratory findings and operative technique of the patients were evaluated retrospectively. Common complaints were abdominal pain and bilious vomiting. Abdominal distention was present in all patients. Leukocytosis and mild hyperbilirubinemia were detected in 5, elevated serum transaminase levels in 4, hyperglycemia in 1 and constipation in 1 patient(s). Abdominal ultrasonography showed a large amount of free fluid. During laparotomy, sterile bile peritonitis was detected initially. After exploration, SPBD was seen. T-tube drainage of the bile duct was carried out. Patients were discharged after removal of the T-tubes. Pancreatico-biliary malfunction was detected in 4 of 5 patients. In patients with generalized peritonitis, elevated transaminase levels and hyperbilirubinemia, SPBD must be considered. Even though the T-tube drainage is the treatment of choice, Roux-en-Y hepatico-portoenterostomy may be mandatory in certain patients.

Exposure to professional pest control treatments and the risk of childhood acute lymphoblastic leukemia.

Science.gov (United States)

Bailey, Helen D; Armstrong, Bruce K; de Klerk, Nicholas H; Fritschi, Lin; Attia, John; Scott, Rodney J; Smibert, Elizabeth; Milne, Elizabeth

2011-10-01

Previous studies suggest that exposure to pesticides increases the risk of childhood acute lymphoblastic leukemia (ALL). The aim of this analysis was to investigate whether professional pest treatments in or around the home before birth or during childhood increased the risk of childhood ALL. Data from 388 cases and 870 frequency-matched controls were analyzed using unconditional logistic regression, adjusting for study matching variables and potential confounders, to calculate odds ratios (ORs). A meta-analysis of our findings with the published findings of previous studies was also conducted. The ORs for any professional pest control treatments were 1.19 (95% CI 0.83, 1.69) in the year before pregnancy, 1.30 (95% CI 0.86, 1.97) during pregnancy and 1.24 (95% CI 0.93, 1.65) for those done after the child's birth. The ORs for exposure after birth were highest when it occurred between the ages of two and three years. ORs were elevated for termite treatments before birth. ORs were higher for pre-B than T cell ALL and for t(12;21) (ETV6-Runx-1) than other cytogenetic sub-types. The pooled OR from a meta-analysis of our study with three previous studies of professional pest control treatments during pregnancy was 1.37 (95% CI 1.00, 1.88). Our results, and those of our meta-analysis, provide some evidence of a modestly increased risk of ALL for professional pest control treatments done during the index pregnancy and possibly in the child's early years. The analysis of pooled data from international collaborations may provide more certainty regarding these potentially important associations. Copyright © 2011 UICC.

Injury and subsequent regeneration of muscles for activation of local innate immunity to facilitate the development and relapse of autoimmune myositis in C57BL/6 mice.

Science.gov (United States)

Kimura, Naoki; Hirata, Shinya; Miyasaka, Nobuyuki; Kawahata, Kimito; Kohsaka, Hitoshi

2015-04-01

To determine whether injury and regeneration of the skeletal muscles induce an inflammatory milieu that facilitates the development and relapse of autoimmune myositis. The quadriceps of C57BL/6 mice were injured with bupivacaine hydrochloride (BPVC) and evaluated histologically. Macrophages and regenerating myofibers in the treated muscles and differentiating C2C12 myotubes were examined for cytokine expression. Mice were immunized with C protein fragments at the base of the tail and in the right hind footpads (day 0) to evoke systemic anti-C protein immunity and to induce local myositis in the right hind limbs. The contralateral quadriceps muscles were injured with BPVC or phosphate buffered saline (PBS) on day 7 or after spontaneous regression of myositis (day 42). The quadriceps muscle in nonimmunized mice was injured with BPVC on day 7. The muscles were examined histologically 14 days after treatment. The BPVC-injured muscles had macrophage infiltration most abundantly at 3 days after the injection, with emergence of regenerating fibers from day 5. The macrophages expressed inflammatory cytokines, including tumor necrosis factor α, interleukin-1β, and CCL2. Regenerating myofibers and C2C12 myotubes also expressed the cytokines. The BPVC-injected muscles from nonimmunized mice had regenerating myofibers with resolved cell infiltration 14 days after treatment. In mice preimmunized with C protein fragments, the muscles injected with BPVC on day 7 as well as on day 42, but not those injected with PBS, had myositis accompanied by CD8+ T cell infiltration. Injury and regeneration could set up an inflammatory milieu in the muscles and facilitate the development and relapse of autoimmune myositis. Copyright © 2015 by the American College of Rheumatology.

Association of eosinophilic myositis with an unusual species of Sarcocystis in a beef cow.

Science.gov (United States)

Gajadhar, A A; Yates, W D; Allen, J R

1987-01-01

The carcass of a mature cow had numerous, disseminated lesions typical of eosinophilic myositis. To elucidate the nature and possible cause of the lesions, histological sections were examined by light microscopy and selected areas were removed and processed for electron microscopy. The lesions were granulomatous in nature. Each granuloma contained at its centre an intact or ruptured sarcocyst associated with degenerate muscle fibers. Surrounding this was a layer of epithelioid cells and an intense accumulation of inflammatory cells, most of which were eosinophils. The primary cyst wall of the sarcocysts in these granulomas consisted of hair-like protrusions that featured many unusual electron-dense bodies. Sarcocysts with ultrastructures characteristic of Sarcocystis cruzi and Sarcocystis hirsuta were also present in muscle from the same animal, but these sarcocysts lacked any associated cellular responses. The eosinophilic myositis in this case appeared to be associated with sarcocystosis of an unknown species. Possibly, the inflammatory reaction was due to the host-parasite interaction in an unusual host. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. Fig. 5. Fig. 6. Fig. 7. Fig. 8. Fig. 9. Fig. 10. PMID:3115553

A case of myositis ossificans in the upper cervical spine of a young child.

Science.gov (United States)

Findlay, Iain; Lakkireddi, Prabhat Reddy; Gangone, Ravinder; Marsh, Gavin

2010-12-01

Case report. We present a case of myositis ossificans (MO) of the upper cervical spine in a young child. The literature is reviewed with the classification, etiology, and treatment of MO discussed. Calcification of joint capsule, muscle, cartilage, and ligaments is a well-known phenomenon and is known as myositis ossificans. It is very rarely seen in the head and neck, with no reports of MO of the soft tissues surrounding the first 2 cervical vertebrae. An 8-year-old boy presented with severe neck pain after a fall. He had had a similar neck injury 4 years before, but made a full recovery. Radiographs showed a large ossified lesion between the posterior elements of C1 and C2. After further imaging, a diagnosis of MO was made. The child was treated with simple analgesia and observation. With no evidence of neurologic compromise and minimal symptoms, there was no indication for surgical intervention. Although rare, MO should be suspected as one of the possible causes of persistent pain following cervical spine injury in children. We would advise a low threshold for cervical spine imaging in the child presenting with persistent neck pain and stiffness, even years after injury.

Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome

Directory of Open Access Journals (Sweden)

Stéphane Mathis

2016-01-01

Full Text Available Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy, myositis, and myasthenia gravis (with positive acetylcholine receptor antibodies. For each patient, we carried out a pathological analysis (nerve and muscle and an electrophysiological study (and follow-up. To our knowledge, this is the first description of such a triple immune-mediated neuromuscular syndrome. We compared our observations with a few other cases of simultaneous diagnosis of two inflammatory neuromuscular disorders.

MR imaging findings of focal myositis: a pseudotumour that may mimic muscle neoplasm

International Nuclear Information System (INIS)

Gaeta, Michele; Mazziotti, Silvio; Minutoli, Fabio; Genitori, Antonino; Blandino, Alfredo; Toscano, Antonio; Rodolico, Carmelo

2009-01-01

The authors describe magnetic resonance (MR) findings in eight patients with histologically confirmed focal myositis. In each patient, axial TSE T1-weighted and fast short-tau inversion recovery (STIR) images were obtained using a 1.5-T MR scanner. Three patients also underwent dynamic contrast-enhanced MR examination using a GE T1-weighted sequence. The following features were evaluated: anatomical distribution, extent of the involvement, signal intensity characteristics, dynamic enhancement pattern and outcome at follow-up examinations. Seven of eight lesions were located in the lower extremities, one of eight in the arm; four of eight involved part of a muscle, two of eight diffusely involved a muscle and two of eight showed multifocal involvement of two or more muscles. All lesions were hyperintense on fast-STIR images: the hyperintensity was homogeneous in six of eight and inhomogeneous in two of eight. On T1-weighted unenhanced images, all lesions but two appeared isointense or slightly hypointense in comparison to normal muscles; two lesions showed a slight hyperintensity. Dynamic enhancement pattern corresponded to the type usually seen in benign soft tissue lesions. All lesions disappeared. Focal myositis is an uncommon pseudotumour which should be considered in the differential diagnosis of muscular masses and myopathies. (orig.)

MR imaging findings of focal myositis: a pseudotumour that may mimic muscle neoplasm

Energy Technology Data Exchange (ETDEWEB)

Gaeta, Michele; Mazziotti, Silvio; Minutoli, Fabio; Genitori, Antonino; Blandino, Alfredo [University of Messina, A.O.U. ' ' Policlinico G. Martino' ' , Department of Radiological Sciences, Messina (Italy); Toscano, Antonio; Rodolico, Carmelo [University of Messina, A.O.U. ' ' Policlinico G. Martino' ' , Department of Neurosciences, Psychiatry and Anaesthesiology, Messina (Italy)

2009-06-15

The authors describe magnetic resonance (MR) findings in eight patients with histologically confirmed focal myositis. In each patient, axial TSE T1-weighted and fast short-tau inversion recovery (STIR) images were obtained using a 1.5-T MR scanner. Three patients also underwent dynamic contrast-enhanced MR examination using a GE T1-weighted sequence. The following features were evaluated: anatomical distribution, extent of the involvement, signal intensity characteristics, dynamic enhancement pattern and outcome at follow-up examinations. Seven of eight lesions were located in the lower extremities, one of eight in the arm; four of eight involved part of a muscle, two of eight diffusely involved a muscle and two of eight showed multifocal involvement of two or more muscles. All lesions were hyperintense on fast-STIR images: the hyperintensity was homogeneous in six of eight and inhomogeneous in two of eight. On T1-weighted unenhanced images, all lesions but two appeared isointense or slightly hypointense in comparison to normal muscles; two lesions showed a slight hyperintensity. Dynamic enhancement pattern corresponded to the type usually seen in benign soft tissue lesions. All lesions disappeared. Focal myositis is an uncommon pseudotumour which should be considered in the differential diagnosis of muscular masses and myopathies. (orig.)

Influence of socioeconomic status on childhood acute lymphoblastic leukemia treatment in Indonesia.

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Mostert, Saskia; Sitaresmi, Mei N; Gundy, Chad M; Sutaryo; Veerman, Anjo J P

2006-12-01

A major reason for poor survival of childhood acute lymphoblastic leukemia in developing countries is treatment refusal or abandonment. This can be associated with parental socioeconomic status and attitudes of health care providers. Our study examined the influence of 2 socioeconomic status determinants, parental income and education, on treatment in an Indonesian academic hospital. Medical charts of 164 patients who received a diagnosis of acute lymphoblastic leukemia between 1997 and 2002 were abstracted retrospectively. Data on treatment results and parental financial and educational background were collected. Open interviews were conducted with parents and health care providers. Of all patients, 35% refused or abandoned treatment, 23% experienced treatment-related death, 22% had progressive or relapsed leukemia, and 20% had an overall event-free survival. Treatment results differed significantly between patients with different socioeconomic status; 47% of poor and 2% of prosperous patients refused or abandoned treatment. Although poor and prosperous patients used the same protocol, the provided treatment differed. Poor patients received less individualized attention from oncologists and less structured parental education. Strong social hierarchical structures hindered communication with doctors, resulting in a lack of parental understanding of the necessity to continue treatment. Most poor patients could not afford treatment. Access to donated chemotherapy also was inadequate. Treatment refusal or abandonment frequently resulted. There was no follow-up system to detect and contact dropouts. Health care providers were not fully aware that their own attitude and communication skills were important for ensuring compliance of patients and parents. Children's survival of acute lymphoblastic leukemia in developing countries could improve if problems that are associated with parental financial and educational background and medical teams' attitudes to treatment and

Histogram analysis derived from apparent diffusion coefficient (ADC) is more sensitive to reflect serological parameters in myositis than conventional ADC analysis.

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Meyer, Hans Jonas; Emmer, Alexander; Kornhuber, Malte; Surov, Alexey

2018-05-01

Diffusion-weighted imaging (DWI) has the potential of being able to reflect histopathology architecture. A novel imaging approach, namely histogram analysis, is used to further characterize tissues on MRI. The aim of this study was to correlate histogram parameters derived from apparent diffusion coefficient (ADC) maps with serological parameters in myositis. 16 patients with autoimmune myositis were included in this retrospective study. DWI was obtained on a 1.5 T scanner by using the b-values of 0 and 1000 s mm - 2 . Histogram analysis was performed as a whole muscle measurement by using a custom-made Matlab-based application. The following ADC histogram parameters were estimated: ADCmean, ADCmax, ADCmin, ADCmedian, ADCmode, and the following percentiles ADCp10, ADCp25, ADCp75, ADCp90, as well histogram parameters kurtosis, skewness, and entropy. In all patients, the blood sample was acquired within 3 days to the MRI. The following serological parameters were estimated: alanine aminotransferase, aspartate aminotransferase, creatine kinase, lactate dehydrogenase, C-reactive protein (CRP) and myoglobin. All patients were screened for Jo1-autobodies. Kurtosis correlated inversely with CRP (p = -0.55 and 0.03). Furthermore, ADCp10 and ADCp90 values tended to correlate with creatine kinase (p = -0.43, 0.11, and p = -0.42, = 0.12 respectively). In addition, ADCmean, p10, p25, median, mode, and entropy were different between Jo1-positive and Jo1-negative patients. ADC histogram parameters are sensitive for detection of muscle alterations in myositis patients. Advances in knowledge: This study identified that kurtosis derived from ADC maps is associated with CRP in myositis patients. Furthermore, several ADC histogram parameters are statistically different between Jo1-positive and Jo1-negative patients.

Molecular signatures in childhood acute leukemia and their correlations to expression patterns in normal hematopoietic subpopulations.

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Andersson, Anna; Olofsson, Tor; Lindgren, David; Nilsson, Björn; Ritz, Cecilia; Edén, Patrik; Lassen, Carin; Råde, Johan; Fontes, Magnus; Mörse, Helena; Heldrup, Jesper; Behrendtz, Mikael; Mitelman, Felix; Höglund, Mattias; Johansson, Bertil; Fioretos, Thoas

2005-12-27

Global expression profiles of a consecutive series of 121 childhood acute leukemias (87 B lineage acute lymphoblastic leukemias, 11 T cell acute lymphoblastic leukemias, and 23 acute myeloid leukemias), six normal bone marrows, and 10 normal hematopoietic subpopulations of different lineages and maturations were ascertained by using 27K cDNA microarrays. Unsupervised analyses revealed segregation according to lineages and primary genetic changes, i.e., TCF3(E2A)/PBX1, IGH@/MYC, ETV6(TEL)/RUNX1(AML1), 11q23/MLL, and hyperdiploidy (>50 chromosomes). Supervised discriminatory analyses were used to identify differentially expressed genes correlating with lineage and primary genetic change. The gene-expression profiles of normal hematopoietic cells were also studied. By using principal component analyses (PCA), a differentiation axis was exposed, reflecting lineages and maturation stages of normal hematopoietic cells. By applying the three principal components obtained from PCA of the normal cells on the leukemic samples, similarities between malignant and normal cell lineages and maturations were investigated. Apart from showing that leukemias segregate according to lineage and genetic subtype, we provide an extensive study of the genes correlating with primary genetic changes. We also investigated the expression pattern of these genes in normal hematopoietic cells of different lineages and maturations, identifying genes preferentially expressed by the leukemic cells, suggesting an ectopic activation of a large number of genes, likely to reflect regulatory networks of pathogenetic importance that also may provide attractive targets for future directed therapies.

Brain sarcoma of meningeal origin after cranial irradiation in childhood acute lymphocytic leukemia. Case report

International Nuclear Information System (INIS)

Tiberin, P.; Maor, E.; Zaizov, R.; Cohen, I.J.; Hirsch, M.; Yosefovich, T.; Ronen, J.; Goldstein, J.

1984-01-01

The authors report their experience with an unusual case of intracerebral sarcoma of meningeal cell origin in an 8 1/2-year-old girl. This tumor occurred 6 1/2 years after cranial irradiation at relatively low dosage (2200 rads) had been delivered to the head in the course of a multimodality treatment for acute lymphocytic leukemia. The tumor recurred approximately 10 months after the first surgical intervention. Macroscopic total excision of the recurrent growth followed by whole-brain irradiation (4500 rads) failed to eradicate it completely and local recurrence prompted reoperation 18 months later. This complication of treatment in long-term childhood leukemia survivors is briefly discussed, as well as the pathology of meningeal sarcomas

Severe childhood malnutrition

DEFF Research Database (Denmark)

Bhutta, Zulfiqar A; Berkley, James A; Bandsma, Robert H J

2017-01-01

The main forms of childhood malnutrition occur predominantly in children malnutrition. Here, we use...... the term 'severe malnutrition' to describe these conditions to better reflect the contributions of chronic poverty, poor living conditions with pervasive deficits in sanitation and hygiene, a high prevalence of infectious diseases and environmental insults, food insecurity, poor maternal and fetal...... nutritional status and suboptimal nutritional intake in infancy and early childhood. Children with severe malnutrition have an increased risk of serious illness and death, primarily from acute infectious diseases. International growth standards are used for the diagnosis of severe malnutrition and provide...

Acute severe childhood asthma

African Journals Online (AJOL)

Attending school regularly. • Sleeping well at night ... and children must know exactly what to do when an acute attack occurs, and when to ... peak flow reading that is 30% below the expected level, are ... oxygen saturations < 94%, should receive high-flow oxygen ... usual of the metered dose inhaler are required to achieve.

Childhood acute lymphoblastic leukemia presenting as ''cold'' lesions on bone scan: a report of two cases

International Nuclear Information System (INIS)

Caudle, R.J.; Crawford, A.H.; Gelfand, M.J.; Gruppo, R.A.

1987-01-01

''Cold'' lesions on bone scan have been reported in a variety of disease processes, including infection, avascular necrosis, and cysts. We present two cases of children who presented with large ''cold'' areas on technetium bone scans and were treated initially for septic processes. Acute childhood leukemia frequently presents with bone or joint pain, fever, and elevation of the erythrocyte sedimentation rate. Although the diagnosis may be difficult if the characteristic clinical signs and laboratory findings are absent, the presence of anemia should alert the physician to the possibility of malignancy. Bone scanning provides a sensitive method of localizing pathology, but diagnosis requires biopsy or marrow aspiration

Therapy-Related Myelodysplastic Syndrome Following Treatment for Childhood Acute Lymphoblastic Leukemia: Outcome of Patients Registered in the EWOG-MDS 98/06 Studies

DEFF Research Database (Denmark)

Strahm, Birgitte; Amann, Roland; De Moerloose, Barbara

Objective: Therapy-related myelodysplastic syndrome (tMDS) following treatment of childhood acute lymphoblastic leukemia (ALL) is one of the most frequently observed secondary malignancies in survivors of childhood cancer. Allogeneic stem cell transplantation (SCT) is the only curative treatment....... This analysis was performed to asses the outcome of patients with tMDS following treatment for childhood ALL reported to the EWOG-MDS study group. Patients and Transplant Procedure: Forty-three patients (19 male/24 female) were diagnosed with tMDS between August 1989 and August 2009. The median age at diagnosis...... was 8.9 yrs (3.4–20.5). The median interval from diagnosis of ALL to the diagnosis of tMDS was 3.3 yrs (1.7–7.0). Five patients did not receive SCT and died due to progressive disease at a median of 5.6 mo after diagnosis. Thirty-eight patients were transplanted. One patient was excluded from...

Ossificans myositis: inflammatory changes and contrast enhancement of adjacent bone shown by MR imaging

International Nuclear Information System (INIS)

David, H.; Jolles, E.; Le Friant, G.; Silvestre, A.; Sarrazin, J.L.; Gordoliani, Y.S.

1995-01-01

The authors report a case of ossificans myositis, in which magnetic resonance imaging (MRI) showed inflammatory changes of the adjacent bone. T 1 weighted fat saturation sequence with gadolinium injection showed enhancement of medullary and cortical bone. This potentially mistaking pattern must be known, to avoid mis diagnosing with malignant osseous tumor, specially before achievement of the characteristic pattern of zonal maturation and its calcified rim. (authors). 15 refs., 6 figs

Anti-Jo-1 antibody-positive patients show a characteristic necrotizing perifascicular myositis.

Science.gov (United States)

Mescam-Mancini, Lénaig; Allenbach, Yves; Hervier, Baptiste; Devilliers, Hervé; Mariampillay, Kuberaka; Dubourg, Odile; Maisonobe, Thierry; Gherardi, Romain; Mezin, Paulette; Preusse, Corinna; Stenzel, Werner; Benveniste, Olivier

2015-09-01

Idiopathic inflammatory myopathies can be classified as polymyositis, dermatomyositis, immune-mediated necrotizing myopathy, sporadic inclusion body myositis or non-specific myositis. Anti-Jo-1 antibody-positive patients are assigned to either polymyositis or dermatomyositis suggesting overlapping pathological features. We aimed to determine if anti-Jo-1 antibody-positive myopathy has a specific morphological phenotype. In a series of 53 muscle biopsies of anti-Jo-1 antibody-positive patients, relevant descriptive criteria defining a characteristic morphological pattern were identified. They were tested in a second series of anti-Jo-1 antibody-positive patients and compared to 63 biopsies from patients suffering from other idiopathic inflammatory myopathies. In anti-Jo-1 antibody-positive patients, necrotic fibres, which strongly clustered in perifascicular regions, were frequently observed. Sarcolemmal complement deposition was detected specifically in perifascicular areas. Inflammation was mainly located in the perimysium and around vessels in 90.6%. Perimysial fragmentation was observed in 90% of cases. Major histocompatibility complex class I staining was diffusely positive, with a perifascicular reinforcement. Multivariate analysis showed that criteria defining perifascicular pathology: perifascicular necrosis, atrophy, and perimysial fragmentation allow the distinction of anti-Jo-1 antibody-positive patients, among patients suffering from other idiopathic inflammatory myopathies. Anti-Jo-1 antibody-positive patients displayed perifascicular necrosis, whereas dermatomyositis patients exhibited perifascicular atrophy. © The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Necrotising Myositis, the Deadly Impersonator

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A. Rahman

2014-01-01

Full Text Available We report two cases of patients with necrotising myositis who presented initially with limb pain and swelling on a background of respiratory complaints. Patient 1, a previously well 38-year-old female, underwent various investigations in the emergency department for excessive lower limb pain and a skin rash. Patient 2, a 61-year-old female with a background of rheumatoid arthritis and hypertension, presented to accident and emergency feeling generally unwell and was treated for presumed respiratory sepsis. Both deteriorated rapidly and were referred to the plastic surgery team with soft tissue necrosis, impending multiorgan failure and toxaemia. Large areas of necrotic muscle and skin were debrided, which grew group A streptococci, Streptococcus pyogenes. Patient 1 had a high above knee amputation of the left leg with extensive debridement of the right. Despite aggressive surgical intervention and microbiological input with intensive care support, patient 2 died. These two cases highlight the importance of early diagnosis and prompt surgical and pharmacological intervention in managing this life-threatening disease. Pain is the primary symptom with skin changes being a late and subtle sign in a septic patient. The Laboratory Risk Indicator for Necrotising Fasciitis (LRINEC may be of use if there is concern to aid diagnosis of this life-threatening disease.

A Comprehensive Overview on Myositis-Specific Antibodies: New and Old Biomarkers in Idiopathic Inflammatory Myopathy

Science.gov (United States)

Satoh, Minoru; Tanaka, Shin; Ceribelli, Angela; Calise, S. John; Chan, Edward K. L.

2018-01-01

Autoantibodies specific for idiopathic inflammatory myopathy (myositis-specific autoantibodies (MSAs)) are clinically useful biomarkers to help the diagnosis of polymyositis/dermatomyositis (PM/DM). Many of these are also associated with a unique clinical subset of PM/DM, making them useful in predicting and monitoring certain clinical manifestations. Classic MSAs known for over 30 years include antibodies to Jo-1 (histidyl transfer RNA (tRNA) synthetase) and other aminoacyl tRNA synthetases (ARS), anti-Mi-2, and anti-signal recognition particle (SRP). Anti-Jo-1 is the first autoantibodies to ARS detected in 15–25 % of patients. In addition to anti-Jo-1, antibodies to seven other aminoacyl tRNA synthetases (ARS) have been reported with prevalence, usually 1–5 % or lower. Patients with any antiARS antibodies are associated with anti-synthetase syndrome characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud’s phenomenon, and others. Several recent studies suggested heterogeneity in clinical features among different anti-ARS antibody-positive patients and anti-ARS may also be found in idiopathic ILD without myositis. Anti-Mi-2 is a classic marker for DM and associated with good response to steroid treatment and good prognosis. Anti-SRP is specific for PM and associated with treatment-resistant myopathy histologically characterized as necrotizing myopathy. In addition to classic MSAs, several new autoantibodies with strong clinical significance have been described in DM. Antibodies to transcription intermediary factor 1γ/α (TIF1γ/α, p155/140) are frequently found in DM associated with malignancy while anti-melanoma differentiation-associated gene 5 (MDA5; CADM140) are associated with clinically amyopathic DM (CADM) complicated by rapidly progressive ILD. Also, anti-MJ/nuclear matrix protein 2 (NXP-2) and anti-small ubiquitin-like modifier-1 (SUMO-1) activating enzyme (SAE) are recognized as new DM-specific autoantibodies. Addition of

Group G streptococcal myositis in a patient with myeloproliferative neoplasm

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Monica Midha, MD MBS

2016-01-01

Full Text Available While many cases of streptococcal infection are due to Lancefield groups A and B, there has been a rise in reported cases of infections due to group G streptococcus. We present a case of an individual with a hematologic malignancy who developed myositis secondary to group G streptococcus, with no clearly identifiable source of infection. The patient was managed with antibiotic therapy rather than surgical intervention due to high surgical risk related to severe thrombocytopenia. Targeted antibiotics initiated early in the course of disease may prevent the need for surgical intervention. Early diagnosis and treatment are critical to avoid the high morbidity and mortality of life-threatening infections caused by group G streptococcus.

Beneficial role of rapamycin in experimental autoimmune myositis.

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Nicolas Prevel

Full Text Available We developed an experimental autoimmune myositis (EAM mouse model of polymyositis where we outlined the role of regulatory T (Treg cells. Rapamycin, this immunosuppressant drug used to prevent rejection in organ transplantation, is known to spare Treg. Our aim was to test the efficacy of rapamycin in vivo in this EAM model and to investigate the effects of the drug on different immune cell sub-populations.EAM is induced by 3 injections of myosin emulsified in CFA. Mice received rapamycin during 25 days starting one day before myosin immunization (preventive treatment, or during 10 days following the last myosin immunization (curative treatment.Under preventive or curative treatment, an increase of muscle strength was observed with a parallel decrease of muscle inflammation, both being well correlated (R(2 = -0.645, p<0.0001. Rapamycin induced a general decrease in muscle of CD4 and CD8 T cells in lymphoid tissues, but spared B cells. Among T cells, the frequency of Treg was increased in rapamycin treated mice in draining lymph nodes (16.9 ± 2.2% vs. 9.3 ± 1.4%, p<0.001, which were mostly activated regulatory T cells (CD62L(lowCD44(high: 58.1 ± 5.78% vs. 33.1 ± 7%, treated vs. untreated, p<0.001. In rapamycin treated mice, inhibition of proliferation (Ki-67(+ is more important in effector T cells compared to Tregs cells (p<0.05. Furthermore, during preventive treatment, rapamycin increased the levels of KLF2 transcript in CD44(low CD62L(high naive T cell and in CD62L(low CD44(high activated T cell.Rapamycin showed efficacy both as curative and preventive treatment in our murine model of experimental myositis, in which it induced an increase of muscle strength with a parallel decrease in muscle inflammation. Rapamycin administration was also associated with a decrease in the frequency of effector T cells, an increase in Tregs, and, when administered as preventive treatment, an upregulation of KFL2 in naive and activated T cells.

Automated diagnosis of myositis from muscle ultrasound: Exploring the use of machine learning and deep learning methods.

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Philippe Burlina

Full Text Available To evaluate the use of ultrasound coupled with machine learning (ML and deep learning (DL techniques for automated or semi-automated classification of myositis.Eighty subjects comprised of 19 with inclusion body myositis (IBM, 14 with polymyositis (PM, 14 with dermatomyositis (DM, and 33 normal (N subjects were included in this study, where 3214 muscle ultrasound images of 7 muscles (observed bilaterally were acquired. We considered three problems of classification including (A normal vs. affected (DM, PM, IBM; (B normal vs. IBM patients; and (C IBM vs. other types of myositis (DM or PM. We studied the use of an automated DL method using deep convolutional neural networks (DL-DCNNs for diagnostic classification and compared it with a semi-automated conventional ML method based on random forests (ML-RF and "engineered" features. We used the known clinical diagnosis as the gold standard for evaluating performance of muscle classification.The performance of the DL-DCNN method resulted in accuracies ± standard deviation of 76.2% ± 3.1% for problem (A, 86.6% ± 2.4% for (B and 74.8% ± 3.9% for (C, while the ML-RF method led to accuracies of 72.3% ± 3.3% for problem (A, 84.3% ± 2.3% for (B and 68.9% ± 2.5% for (C.This study demonstrates the application of machine learning methods for automatically or semi-automatically classifying inflammatory muscle disease using muscle ultrasound. Compared to the conventional random forest machine learning method used here, which has the drawback of requiring manual delineation of muscle/fat boundaries, DCNN-based classification by and large improved the accuracies in all classification problems while providing a fully automated approach to classification.

Automated diagnosis of myositis from muscle ultrasound: Exploring the use of machine learning and deep learning methods.

Science.gov (United States)

Burlina, Philippe; Billings, Seth; Joshi, Neil; Albayda, Jemima

2017-01-01

To evaluate the use of ultrasound coupled with machine learning (ML) and deep learning (DL) techniques for automated or semi-automated classification of myositis. Eighty subjects comprised of 19 with inclusion body myositis (IBM), 14 with polymyositis (PM), 14 with dermatomyositis (DM), and 33 normal (N) subjects were included in this study, where 3214 muscle ultrasound images of 7 muscles (observed bilaterally) were acquired. We considered three problems of classification including (A) normal vs. affected (DM, PM, IBM); (B) normal vs. IBM patients; and (C) IBM vs. other types of myositis (DM or PM). We studied the use of an automated DL method using deep convolutional neural networks (DL-DCNNs) for diagnostic classification and compared it with a semi-automated conventional ML method based on random forests (ML-RF) and "engineered" features. We used the known clinical diagnosis as the gold standard for evaluating performance of muscle classification. The performance of the DL-DCNN method resulted in accuracies ± standard deviation of 76.2% ± 3.1% for problem (A), 86.6% ± 2.4% for (B) and 74.8% ± 3.9% for (C), while the ML-RF method led to accuracies of 72.3% ± 3.3% for problem (A), 84.3% ± 2.3% for (B) and 68.9% ± 2.5% for (C). This study demonstrates the application of machine learning methods for automatically or semi-automatically classifying inflammatory muscle disease using muscle ultrasound. Compared to the conventional random forest machine learning method used here, which has the drawback of requiring manual delineation of muscle/fat boundaries, DCNN-based classification by and large improved the accuracies in all classification problems while providing a fully automated approach to classification.

Premature atherosclerosis after treatment for acute lymphoblastic leukemia in childhood

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Elżbieta Sadurska

2018-03-01

Survivors of childhood ALL in the examined group demonstrated elevated concentrations of selected new biomarkers and increased IMT values, compared to controls, which may confirm the occurrence of endothelial injuries in blood vessels. This study indicates that subjects treated for childhood malignancy are at a higher risk of prematurely developing atherosclerosis.

OUTPATIENT PHYSICAL THERAPY EVALUATION AND TREATMENT OF A PATIENT DIAGNOSED WITH SPORADIC INCLUSION BODY MYOSITIS: A CASE STUDY

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Tyler Harrigfeld

2017-08-01

Full Text Available Background: Sporadic inclusion body myositis is an autoimmune and degenerative disorder of skeletal muscle that affects people at random. It most commonly begins as progressive weakness and atrophy of lower extremity musculature, beginning with the proximal leg. These impairments in body structure adversely affect the performance of functional activities and mobility, resulting in a progressive decrease in independence and participation both at home and in the community. Physical therapy attempts to minimize these effects through educational and procedural interventions focused on treating impairments and limitations. The purpose of this case study was to provide a description of the physical therapy management of a patient diagnosed with sporadic inclusion body myositis. Case Summary: The patient was a 66-year-old male who was diagnosed with sporadic inclusion body myositis with a chief complaint of weakness and fall risk. He presented with generalized lower extremity weakness and atrophy of bilateral quadriceps, as well as impaired balance and increasing fatigue with activity. Therapeutic exercise, home exercise program, balance, gait, and stair training were delivered to address these impairments. Patient outcomes showed improvement in balance and safety with functional activities. Discussion: The patient was seen for seven visits that were 45 – 60 minutes in length, over a five-week period. The patient made subjective reports of improvement in functional activities and balance; however many objective outcome measures could not be reassessed. There is a need for further research on this population to determine the effectiveness and parameters of physical therapy interventions. Conclusion: Physical therapy may have helped improve balance as well as subjective reports from the patient of increased feeling of confidence while navigating stairs.

Causes of childhood leukaemia and lymphoma

International Nuclear Information System (INIS)

Lightfoot, Tracy J.; Roman, Eve

2004-01-01

Childhood cancer is rare comprising less than 1% of all malignancies diagnosed each year in developed countries. Leukaemia is the commonest form of cancer in children accounting for around a third of all childhood cancer, with acute lymphoblastic leukaemia (ALL) being the most prevalent. Biologically specific subtypes of ALL and acute myeloblastic leukaemia (AML), the other major morphological type of childhood leukaemia, are characterised by chromosomal changes. Whilst over 200 genes have been associated with chromosomal translocations, to date, only MLL, TEL, and AML1 have been linked with childhood leukaemia. Interestingly, there is increasing evidence to support the theory that gene rearrangements such as these may originate in utero. As with many other human diseases, both genetic and environmental factors have been implicated in the aetiology of the disease. Although much has been documented with regard to diet, smoking, alcohol consumption and recreational and prescription drug use during pregnancy, there is no consistent evidence to support a link with any of these factors and childhood leukaemia. However, findings from studies investigating prenatal and early life exposures are often based on small numbers of cases as both the type of cancer and exposure are rare. Furthermore, accurate information relating to past exposures can be difficult to obtain and is often reliant on self-reporting. To further our understanding of the aetiology of childhood leukaemia and lymphoma, there are areas which clearly warrant investigation. These include collection of parental dietary folate data combined with genetic analysis of the folate related genes, in utero exposure to DNA topoisomerase II inhibitors, and the possible effects of assisted reproduction technology on disease susceptibility

Allergy and acute leukaemia in children with Down syndrome: a population study. Report from the Mexican inter-institutional group for the identification of the causes of childhood leukaemia

OpenAIRE

Núñez-Enríquez, J C; Fajardo-Gutiérrez, A; Buchán-Durán, E P; Bernáldez-Ríos, R; Medina-Sansón, A; Jiménez-Hernández, E; Amador-Sanchez, R; Peñaloza-Gonzalez, J G; Paredes-Aguilera, R; Alvarez-Rodriguez, F J; Bolea-Murga, V; de Diego Flores-Chapa, J; Flores-Lujano, J; Bekker-Mendez, V C; Rivera-Luna, R

2013-01-01

Background: Allergies have been described as protective factors against the development of childhood acute leukaemia (AL). Our objective was to investigate the associations between allergy history and the development of AL and acute lymphoblastic leukaemia (ALL) in children with Down syndrome (DS). Methods: A case–control study was performed in Mexico City. The cases (n=97) were diagnosed at nine public hospitals, and the controls (n=222) were recruited at institutions for children with DS. O...

Osteogenic toxicity in childhood acute lymphoblastic leukemia

NARCIS (Netherlands)

M.L. te Winkel (Mariël Lizet)

2013-01-01

textabstractBone mineral density (BMD) Our multi-center study in children treated according to the Dutch Childhood Oncology Group (DCOG)-ALL9 protocol showed a three-years cumulative incidence of fractures of 18%. BMD of ALL patients was lower than of healthy peers. The year after treatment

Identification of germline susceptibility loci in ETV6-RUNX1-rearranged childhood acute lymphoblastic leukemia

Science.gov (United States)

Ellinghaus, E; Stanulla, M; Richter, G; Ellinghaus, D; te Kronnie, G; Cario, G; Cazzaniga, G; Horstmann, M; Panzer Grümayer, R; Cavé, H; Trka, J; Cinek, O; Teigler-Schlegel, A; ElSharawy, A; Häsler, R; Nebel, A; Meissner, B; Bartram, T; Lescai, F; Franceschi, C; Giordan, M; Nürnberg, P; Heinzow, B; Zimmermann, M; Schreiber, S; Schrappe, M; Franke, A

2012-01-01

Acute lymphoblastic leukemia (ALL) is a malignant disease of the white blood cells. The etiology of ALL is believed to be multifactorial and likely to involve an interplay of environmental and genetic variables. We performed a genome-wide association study of 355 750 single-nucleotide polymorphisms (SNPs) in 474 controls and 419 childhood ALL cases characterized by a t(12;21)(p13;q22) — the most common chromosomal translocation observed in childhood ALL — which leads to an ETV6–RUNX1 gene fusion. The eight most strongly associated SNPs were followed-up in 951 ETV6-RUNX1-positive cases and 3061 controls from Germany/Austria and Italy, respectively. We identified a novel, genome-wide significant risk locus at 3q28 (TP63, rs17505102, PCMH=8.94 × 10−9, OR=0.65). The separate analysis of the combined German/Austrian sample only, revealed additional genome-wide significant associations at 11q11 (OR8U8, rs1945213, P=9.14 × 10−11, OR=0.69) and 8p21.3 (near INTS10, rs920590, P=6.12 × 10−9, OR=1.36). These associations and another association at 11p11.2 (PTPRJ, rs3942852, P=4.95 × 10−7, OR=0.72) remained significant in the German/Austrian replication panel after correction for multiple testing. Our findings demonstrate that germline genetic variation can specifically contribute to the risk of ETV6–RUNX1-positive childhood ALL. The identification of TP63 and PTPRJ as susceptibility genes emphasize the role of the TP53 gene family and the importance of proteins regulating cellular processes in connection with tumorigenesis. PMID:22076464

Heterotopic bone formation (myositis ossificans) and lower-extremity swelling mimicking deep-venous disease

International Nuclear Information System (INIS)

Orzel, J.A.; Rudd, T.G.; Nelp, W.B.

1984-01-01

A quadriplegic patient with a swollen leg was suspected of having deep-venous thrombosis, and was studied with radionuclide venography (RNV) and contrast venography. Focal narrowing of the femoral vein, seen on RNV, was due to extrinsic compression. Although soft-tissue radiographs were normal, Tc-99m diphosphonate imaging established the diagnosis of early heterotopic bone formation (myositis ossificans), which was responsible for the venous compression. Clinically this inflammatory process can mimic deep-venous thrombosis, and should be considered in evaluating patients at risk for both heterotopic bone formation and deep-venous thrombosis

Infection and childhood leukemia: review of evidence

Directory of Open Access Journals (Sweden)

Raquel da Rocha Paiva Maia

2013-12-01

Full Text Available OBJECTIVE : To analyze studies that evaluated the role of infections as well as indirect measures of exposure to infection in the risk of childhood leukemia, particularly acute lymphoblastic leukemia. METHODS : A search in Medline, Lilacs, and SciELO scientific publication databases initially using the descriptors “childhood leukemia” and “infection” and later searching for the words “childhood leukemia” and “maternal infection or disease” or “breastfeeding” or “daycare attendance” or “vaccination” resulted in 62 publications that met the following inclusion criteria: subject aged ≤ 15 years; specific analysis of cases diagnosed with acute lymphoblastic leukemia or total leukemia; exposure assessment of mothers’ or infants’ to infections (or proxy of infection, and risk of leukemia. RESULTS : Overall, 23 studies that assessed infections in children support the hypothesis that occurrence of infection during early childhood reduces the risk of leukemia, but there are disagreements within and between studies. The evaluation of exposure to infection by indirect measures showed evidence of reduced risk of leukemia associated mainly with daycare attendance. More than 50.0% of the 16 studies that assessed maternal exposure to infection observed increased risk of leukemia associated with episodes of influenza, pneumonia, chickenpox, herpes zoster, lower genital tract infection, skin disease, sexually transmitted diseases, Epstein-Barr virus, and Helicobacter pylori . CONCLUSIONS : Although no specific infectious agent has been identified, scientific evidence suggests that exposure to infections has some effect on childhood leukemia etiology.

Acute Pancreatitis in Children

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... a feeding tube or an IV to prevent malnutrition and improve healing. Does my child have to ... Acute Pancreatitis in Children Chronic Pancreatitis in Children Childhood Inherited Disorders Pancreatic Cancer Pancreatic Cancer Risks and ...

Upregulation of microRNA-21 is a poor prognostic marker in patients with childhood B cell acute lymphoblastic leukemia.

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Labib, Hany Abedelmalik; Elantouny, Neveen G; Ibrahim, Nevin F; Alnagar, Ahmed A

2017-08-01

Many studies have demonstrated that microRNA-21 (miR-21) is an oncogene and is upregulated in tumor tissue. However, its association with B-cell acute lymphoblastic leukemia (B-ALL) remains poorly understood. The expression of miR-21 was detected by real-time quantitative PCR in 75 children with de novo B-ALL as well as in 50 healthy controls. This study was conducted to evaluate the miR-21 as a biomarker for risk assessment, diagnosis and prognosis. Compared with normal controls, miR-21 expression was significantly upregulated in childhood B-ALL patients. Using the receiver operating characteristic curve 3.23 was selected as the cut-off value of miR-21 expression in distinguishing patients from controls. Patients group with High miR-21 expression was significantly associated with those aged 10 years, lower platelets count, more incidence of CNS infiltration and poorer treatment outcome also, they showed a significantly poorer disease-free survival (DFS) and overall survival (OS) compared to those with low miR-21 expression group. Its expression was an independent prognostic marker according to multivariate analysis. This is the first report demonstrating the upregulation of miR-21 in childhood B-ALL, and its association with poor response to induction therapy, shorter DFS and OS. These results suggest that miR-21 upregulation represent an unfavorable prognostic marker in Childhood B-ALL.

Psychosocial function in the first year after childhood stroke.

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Greenham, Mardee; Anderson, Vicki; Hearps, Stephen; Ditchfield, Michael; Coleman, Lee; Mackay, Mark T; Monagle, Paul; Gordon, Anne L

2017-10-01

Childhood stroke disrupts brain development and emerging neural networks. Motor, cognitive, and language deficits are well recognized, yet little is known about psychosocial function after childhood stroke. This study aims to describe psychosocial function within the first year after childhood stroke, and to identify factors associated with outcome. Thirty-seven children were involved in a prospective, longitudinal study investigating recovery over the first year after childhood stroke. Children's social functioning was assessed at 6-months and 12-months poststroke and psychological function at 12-months poststroke, using standardized measures. Mean social function was poorer at both 6-months and 12-months poststroke, compared to prestroke. Psychological problems were more common than expected, with emotional difficulties and hyperactivity-inattention most significantly affected. Poorer social function was associated with older age at onset, acute neurological impairment, and prestroke social impairment. Social and psychological problems were associated with parent mental health. While not all children are affected, psychosocial impairment affects a significant minority after childhood stroke. Older age at onset, acute neurological impairment, prestroke social problems, and poorer parent mental health were associated with deficits. Identifying early predictors of poorer outcome will facilitate early intervention. Of particular importance is parent mental health, suggesting support for families may improve child outcome. © 2017 Mac Keith Press.

Impact of sleep, fatigue, and systemic inflammation on neurocognitive and behavioral outcomes in long-term survivors of childhood acute lymphoblastic leukemia.

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Cheung, Yin Ting; Brinkman, Tara M; Mulrooney, Daniel A; Mzayek, Yasmin; Liu, Wei; Banerjee, Pia; Panoskaltsis-Mortari, Angela; Srivastava, Deokumar; Pui, Ching-Hon; Robison, Leslie L; Hudson, Melissa M; Krull, Kevin R

2017-09-01

Long-term survivors of childhood acute lymphoblastic leukemia (ALL) are at risk for neurocognitive impairment, which may be associated with fatigue, sleep problems, systemic inflammation, and oxidative stress. We examined these associations among survivors of childhood ALL treated with chemotherapy only. Survivors of childhood ALL (male, n = 35 and female, n = 35; mean age, 14.3 years [standard deviation, 4.7 years] and mean years from diagnosis, 7.4 years [standard deviation, 1.9 years]) completed neurocognitive testing, behavioral ratings, and reported sleep quality and fatigue symptoms 5 years after diagnosis. Serum was collected concurrently and assayed for interleukin (IL)-1β and IL-6, tumor necrosis factor α (TNF-α), high-sensitivity C-reactive protein (hsCRP), malondialdehyde, myeloperoxidase, and oxidized low-density lipoprotein. General linear modeling was used to assess associations among biomarkers and functional outcomes, adjusting for age and stratified by sex. Survivors performed worse than population norms on executive function and processing speed and reported more behavioral problems (P fatigue was associated with poor executive function (r = 0.41; P = .02), processing speed (r = 0.56; P fatigue measures were observed. Neurocognitive function in female survivors of childhood ALL appears more susceptible to the effects of sleep disturbance and fatigue. Systemic inflammation may play a role in neurocognitive impairment and behavioral symptoms. Cancer 2017;123:3410-9. © 2017 American Cancer Society. © 2017 American Cancer Society.

Prenatal origin of childhood AML occurs less frequently than in childhood ALL

International Nuclear Information System (INIS)

Burjanivova, Tatiana; Zuna, Jan; Madzo, Jozef; Muzikova, Katerina; Meyer, Claus; Schneider, Bjoern; Votava, Felix; Marschalek, Rolf; Stary, Jan; Trka, Jan

2006-01-01

While there is enough convincing evidence in childhood acute lymphoblastic leukemia (ALL), the data on the pre-natal origin in childhood acute myeloid leukemia (AML) are less comprehensive. Our study aimed to screen Guthrie cards (neonatal blood spots) of non-infant childhood AML and ALL patients for the presence of their respective leukemic markers. We analysed Guthrie cards of 12 ALL patients aged 2–6 years using immunoglobulin (Ig) and T-cell receptor (TCR) gene rearrangements (n = 15) and/or intronic breakpoints of TEL/AML1 fusion gene (n = 3). In AML patients (n = 13, age 1–14 years) PML/RARalpha (n = 4), CBFbeta/MYH11 (n = 3), AML1/ETO (n = 2), MLL/AF6 (n = 1), MLL/AF9 (n = 1) and MLL/AF10 (n = 1) fusion genes and/or internal tandem duplication of FLT3 gene (FLT3/ITD) (n = 2) were used as clonotypic markers. Assay sensitivity determined using serial dilutions of patient DNA into the DNA of a healthy donor allowed us to detect the pre-leukemic clone in Guthrie card providing 1–3 positive cells were present in the neonatal blood spot. In 3 patients with ALL (25%) we reproducibly detected their leukemic markers (Ig/TCR n = 2; TEL/AML1 n = 1) in the Guthrie card. We did not find patient-specific molecular markers in any patient with AML. In the largest cohort examined so far we used identical approach for the backtracking of non-infant childhood ALL and AML. Our data suggest that either the prenatal origin of AML is less frequent or the load of pre-leukemic cells is significantly lower at birth in AML compared to ALL cases

Brain Activity Associated With Attention Deficits Following Chemotherapy for Childhood Acute Lymphoblastic Leukemia.

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Fellah, Slim; Cheung, Yin T; Scoggins, Matthew A; Zou, Ping; Sabin, Noah D; Pui, Ching-Hon; Robison, Leslie L; Hudson, Melissa M; Ogg, Robert J; Krull, Kevin R

2018-05-21

The impact of contemporary chemotherapy treatment for childhood acute lymphoblastic leukemia on central nervous system activity is not fully appreciated. Neurocognitive testing and functional magnetic resonance imaging (fMRI) were obtained in 165 survivors five or more years postdiagnosis (average age = 14.4 years, 7.7 years from diagnosis, 51.5% males). Chemotherapy exposure was measured as serum concentration of methotrexate following high-dose intravenous injection. Neurocognitive testing included measures of attention and executive function. fMRI was obtained during completion of two tasks, the continuous performance task (CPT) and the attention network task (ANT). Image analysis was performed using Statistical Parametric Mapping software, with contrasts targeting sustained attention, alerting, orienting, and conflict. All statistical tests were two-sided. Compared with population norms, survivors demonstrated impairment on number-letter switching (P < .001, a measure of cognitive flexibility), which was associated with treatment intensity (P = .048). Task performance during fMRI was associated with neurocognitive dysfunction across multiple tasks. Regional brain activation was lower in survivors diagnosed at younger ages for the CPT (bilateral parietal and temporal lobes) and the ANT (left parietal and right hippocampus). With higher serum methotrexate exposure, CPT activation decreased in the right temporal and bilateral frontal and parietal lobes, but ANT alerting activation increased in the ventral frontal, insula, caudate, and anterior cingulate. Brain activation during attention and executive function tasks was associated with serum methotrexate exposure and age at diagnosis. These findings provide evidence for compromised and compensatory changes in regional brain function that may help clarify the neural substrates of cognitive deficits in acute lymphoblastic leukemia survivors.

Effects of blood-flow-restricted resistance training on muscle function in a 74-year-old male with sporadic inclusion body myositis

DEFF Research Database (Denmark)

Jørgensen, Anders Nørkær; Aagaard, P; Nielsen, J L

2016-01-01

Sporadic inclusion body myositis (sIBM) is a systemic disease that is characterized by substantial skeletal muscle weakness and muscle inflammation, leading to impaired physical function. The objective was to investigate the effect of low-load resistance exercise with concurrent partial blood flow...

Child symptoms, parent behaviors, and family strain in long-term survivors of childhood acute lymphoblastic leukemia.

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Huang, I-Chan; Brinkman, Tara M; Mullins, Larry; Pui, Ching-Hon; Robison, Leslie L; Hudson, Melissa M; Krull, Kevin R

2018-05-17

How family environment and parental factors affect health status and symptoms in childhood cancer survivors is understudied. We examined the influence of family cohesion, parent distress, and overprotection on child symptom burden and health-related quality of life (HRQOL) and family strain in survivors of childhood acute lymphoblastic leukemia (ALL). Parents of 213 children treated with chemotherapy-only completed a survey when survivors were at least five-years post-diagnosis. Family Environment Scale, Brief Symptom Inventory-18, Parent Protection Scale, PedsQL, and Impact on Family were used to assess family cohesion, parental distress, overprotection, child symptom burden and HRQOL, and family strain, respectively. Path analysis was conducted to quantify effects of family cohesion on family strain through parental distress, overprotection, child symptoms, and HRQOL. Lower family cohesion (β=0.06, 95% CI=0.01 to 0.13), higher parental distress (β=0.35, 95% CI=0.20 to 0.45), and overprotection (β=0.17, 95% CI=0.01 to 0.32) were associated with more child symptom burden. More symptom burden were associated with poorer child HRQOL (β=0.66, 95% CI=0.57 to 0.75), which in turn was associated with more family strain (β=0.11, 95% CI=0.01 to 0.22). Lower maternal education was associated with overprotection (β=-0.23, 95% CI=-0.33 to -0.12), more child symptoms (β=-0.30, 95% CI=-0.41 to -0.16), poorer child HRQOL (β=-0.36, 95% CI=-0.46 to -0.21), and more family strain (β=-0.15, 95% CI=-0.23 to -0.08). Family and parental factors contributed to health outcomes of childhood ALL survivors. Interventions to enhance family cohesion, decrease parental distress and overprotection, and ameliorate child symptoms may improve family functioning. This article is protected by copyright. All rights reserved.

Dislipidemia em pacientes com dermatomiosite juvenil

OpenAIRE

Katia Tomie Kozu

2012-01-01

OBJETIVO: Avaliar a presença de dislipidemia em pacientes com dermatomiosite juvenil (DMJ) e seus possíveis fatores de risco. MÉTODO: 25 pacientes com DMJ foram comparados a 25 controles de acordo com dados demográficos, composição corporal, perfil lipídico, glicêmico, autoanticorpos e enzimas musculares. Foram avaliados os instrumentos de atividade da DMJ: Disease Activity Score (DAS), Childhood Myositis Assessment Scale (CMAS), Manual Muscle Testing (MMT), Myositis Disease Activity Assessme...

Educational late effects in long-term survivors of childhood acute lymphocytic leukemia.

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Peckham, V C; Meadows, A T; Bartel, N; Marrero, O

1988-01-01

Records of levels of school achievement in long-term survivors of childhood acute lymphocytic leukemia were obtained for 23 children who had received 2,400-rad cranial irradiation and intrathecal methotrexate and standard chemotherapeutic agents 8 to 10 years previously. The children had been evaluated with standardized tests of intelligence at the time of diagnosis and periodically thereafter. Declines in IQ and cognitive dysfunctions have been previously described. School placements, educational histories, attendance records, learning strengths and weaknesses, social/emotional adjustments, and grade level achievements in reading and mathematics as measured by standardized achievement tests are reported here. Children achieved less than the expected levels in both reading and mathematics given both pretreatment and most recent IQ scores. Neither sex nor initial IQ were related to achievement scores. Children experienced difficulty with attention/concentration, memory, sequencing, and comprehension when performing school tasks. Individual children showed different degrees of dysfunction, but results of this study suggest that there are patterns of specific learning disabilities rather than global retardation. A small number of children achieved greater than expected levels, indicating that individualized instruction, tutoring, and parental support may reduce some learning deficits. Early educational intervention is recommended for similarly treated patients.

Childhood acute disseminated encephalomyelitis: the role of brain and spinal cord MRI

International Nuclear Information System (INIS)

Khong, Pek-Lan; Cheng, Pui-Wai; Chan, Fu-Luk; Ho, Hok-Kung; Wong, Virginia C.N.; Goh, Winnie

2002-01-01

Background. It is recognised that the clinical and radiological spectrum of childhood acute disseminated encephalomyelitis (ADEM) is wide. Objective. To determine whether initial MRI features are predictive of clinical outcome and to determine the role of MRI in the management of ADEM. Materials and methods. The MRI scans of ten consecutive children (eight boys, two girls), clinically and radiologically diagnosed to have ADEM, were retrospectively reviewed. Follow-up MRI was available for eight patients. Results. Lesions ranged from small and punctate (<1 cm) to moderate sized and confluent (4-5 cm) to diffuse and extensive. Spinal cord lesions, seen in five of seven children, were contiguous or segmental. Seven children (70%) made good clinical recovery while three children (30%) remained severely handicapped. There was no correlation between the site, extent and pattern of involvement and clinical outcome. However, the evolution of MRI findings on follow-up correlated well with the subsequent clinical course and outcome. Conclusions. Although the extent and site of lesions on initial MRI scans are not predictive of clinical outcome, early MRI of the brain and spine is useful in aiding clinical diagnosis, and subsequent follow-up MRI is helpful in monitoring disease progression. (orig.)

Functional impairment in patients with sporadic Inclusion Body Myositis.

Science.gov (United States)

Dunlap, Heather V; Macneil, Lauren G; Tarnopolsky, Mark A

2014-03-01

We conducted a retrospective chart review of 53 patients diagnosed with sporadic Inclusion Body Myositis (sIBM) who have been followed at the McMaster Neuromuscular Clinic since 1996. We reviewed patient medical histories in order to compare our findings with similar cohorts, and analyzed quantitative strength data to determine functionality in guiding decisions related to gait assistive devices. Patient information was acquired through retrospective clinic chart review. Our study found knee extension strength decreased significantly as patients transitioned to using more supportive gait assistive devices (P cane)(P Falls and fear of falling poses a significant threat to patient physical well-being. The prevalence of dysphagia increased as patients required more supportive gait devices, and finally a significant negative correlation was found between time after onset and creatine kinase (CK) levels (P falling would be beneficial in preventing future falls and improving long-term patient outcomes.

Interferon-regulated chemokine score associated with improvement in disease activity in refractory myositis patients treated with rituximab.

Science.gov (United States)

López De Padilla, Consuelo M; Crowson, Cynthia S; Hein, Molly S; Strausbauch, Michael A; Aggarwal, Rohit; Levesque, Marc C; Ascherman, Dana P; Oddis, Chester V; Reed, Ann M

2015-01-01

The purpose of this study was to investigate whether serum interferon (IFN)-regulated chemokine and distinct cytokine response profiles are associated with clinical improvement in patients with refractory inflammatory myopathy treated with rituximab. In a randomised, placebo-phase trial Rituximab in Myositis Trial (RIM), 200 refractory adult and paediatric myositis subjects received rituximab. Following rituximab, clinical response and disease activity were assessed. Serum samples and clinical data were collected at baseline and several time-points after rituximab treatment. Multiplexed sandwich immunoassays quantified serum levels of IFN-regulated chemokines and other pro-inflammatory cytokines. Composite IFN-regulated chemokine and Th1, Th2, Th17 and regulatory cytokine scores were computed. Baseline IFN-regulated chemokine, Th1, Th2, Th17 and regulatory cytokine scores correlated with baseline physician global VAS, whereas the baseline Th1, Th2 and Th17 cytokine scores correlated with baseline muscle VAS. We also found baseline IFN-regulated chemokine scores correlated with specific non-muscular targets such as baseline cutaneous (r=0.29; p=0.002) and pulmonary (r=0.18; p=0.02) VAS scores. Among all cytokine/chemokines examined, the baseline score of IFN-regulated chemokines demonstrated the best correlation with changes in muscle VAS at 8 (r=-0.19; p=0.01) and 16 weeks (r=-0.17; p=0.03) following rituximab and physician global VAS at 16 weeks (r=-0.16; p=0.04). In vitro experiments showed increased levels of IL-8 (p=0.04), MCP-1 (p=0.04), IL-6 (p=0.03), IL-1β (p=0.04), IL-13 (p=0.04), IL-10 (p=0.02), IL-2 (p=0.04) and IFN-γ (p=0.02) in supernatants of TLR-3 stimulated PBMCs from non-responder compared to patients responders to rituximab. IFN-regulated chemokines before treatment is associated with improvement in disease activity measures in refractory myositis patients treated with rituximab.

Family-based exome-wide association study of childhood acute lymphoblastic leukemia among Hispanics confirms role of ARID5B in susceptibility.

Directory of Open Access Journals (Sweden)

Natalie P Archer

Full Text Available We conducted an exome-wide association study of childhood acute lymphoblastic leukemia (ALL among Hispanics to confirm and identify novel variants associated with disease risk in this population. We used a case-parent trio study design; unlike more commonly used case-control studies, this study design is ideal for avoiding issues with population stratification bias among this at-risk ethnic group. Using 710 individuals from 323 Guatemalan and US Hispanic families, two inherited SNPs in ARID5B reached genome-wide level significance: rs10821936, RR = 2.31, 95% CI = 1.70-3.14, p = 1.7×10-8 and rs7089424, RR = 2.22, 95% CI = 1.64-3.01, p = 5.2×10-8. Similar results were observed when restricting our analyses to those with the B-ALL subtype: ARID5B rs10821936 RR = 2.22, 95% CI = 1.63-3.02, p = 9.63×10-8 and ARID5B rs7089424 RR = 2.13, 95% CI = 1.57-2.88, p = 2.81×10-7. Notably, effect sizes observed for rs7089424 and rs10821936 in our study were >20% higher than those reported among non-Hispanic white populations in previous genetic association studies. Our results confirmed the role of ARID5B in childhood ALL susceptibility among Hispanics; however, our assessment did not reveal any strong novel inherited genetic risks for acute lymphoblastic leukemia among this ethnic group.

Translational profiling in childhood acute lymphoblastic leukemia: no evidence for glucocorticoid regulation of mRNA translation.

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Aneichyk, Tatsiana; Bindreither, Daniel; Mantinger, Christine; Grazio, Daniela; Goetsch, Katrin; Kofler, Reinhard; Rainer, Johannes

2013-12-01

Glucocorticoids (GCs) are natural stress induced steroid hormones causing cell cycle arrest and cell death in lymphoid tissues. Therefore they are the central component in the treatment of lymphoid malignancies, in particular childhood acute lymphoblastic leukemia (chALL). GCs act mainly via regulating gene transcription, which has been intensively studied by us and others. GC control of mRNA translation has also been reported but has never been assessed systematically. In this study we investigate the effect of GCs on mRNA translation on a genome-wide scale. Childhood T- (CCRF-CEM) and precursor B-ALL (NALM6) cells were exposed to GCs and subjected to "translational profiling", a technique combining sucrose-gradient fractionation followed by Affymetrix Exon microarray analysis of mRNA from different fractions, to assess the translational efficiency of the expressed genes. Analysis of GC regulation in ribosome-bound fractions versus transcriptional regulation revealed no significant differences, i.e., GC did not entail a significant shift between ribosomal bound and unbound mRNAs. In the present study we analyzed for the first time possible effects of GC on the translational efficiency of expressed genes in two chALL model systems employing whole genome polysome profiling. Our results did not reveal significant differences in translational efficiency of expressed genes thereby arguing against a potential widespread regulatory effect of GCs on translation at least in the investigated in vitro systems.

Regional blood flow in experimental myositis ossificans

International Nuclear Information System (INIS)

Hierton, C.

1983-01-01

In a recent model for heterotopic bone formation, muscular oedema, swelling and necrosis is seen in the quadriceps muscle of rabbit hind limbs immobilized for at least 2 weeks when, from the second week, the immobilized limb is subjected to dayly forcible mobilization lasting about 5 min. According to this model, heterotopic calcification develops gradually from the second week of forcible mobilization and is located in the vastus intermedius region. Between the fourth and fifth week of immobilization and forcible mobilization, heterotopic bone formation is seen in virtually all cases. The histological findings are similar to those in human ectopic bone formation. In the present investigation the labelled microsphere technique was used to study the regional blood flow effects in the early development of myositis ossificans with this model. The results are quite different from those reported by other investigators on immobilization alone and point to a causal relation between regional blood flow and forcible mobilization of the immobilized rabbit hind limp. Prostaglandins as mediators between the traumatic inflammation, a part of the circulatory effects observed and the induction of a new bone is suggested. (author)

Sodium phenylbutyrate reverses lysosomal dysfunction and decreases amyloid-β42 in an in vitro-model of inclusion-body myositis.

Science.gov (United States)

Nogalska, Anna; D'Agostino, Carla; Engel, W King; Askanas, Valerie

2014-05-01

Sporadic inclusion-body myositis (s-IBM) is a severe, progressive muscle disease for which there is no enduring treatment. Pathologically characteristic are vacuolated muscle fibers having: accumulations of multi-protein aggregates, including amyloid-β(Aβ) 42 and its toxic oligomers; increased γ-secretase activity; and impaired autophagy. Cultured human muscle fibers with experimentally-impaired autophagy recapitulate some of the s-IBM muscle abnormalities, including vacuolization and decreased activity of lysosomal enzymes, accompanied by increased Aβ42, Aβ42 oligomers, and increased γ-secretase activity. Sodium phenylbutyrate (NaPB) is an orally bioavailable small molecule approved by the FDA for treatment of urea-cycle disorders. Here we describe that NaPB treatment reverses lysosomal dysfunction in an in vitro model of inclusion-body myositis, involving cultured human muscle fibers. NaPB treatment improved lysosomal activity, decreased Aβ42 and its oligomers, decreased γ-secretase activity, and virtually prevented muscle-fiber vacuolization. Accordingly, NaPB might be considered a potential treatment of s-IBM patients. Copyright © 2014 Elsevier Inc. All rights reserved.

Confirmation of childhood acute lymphoblastic leukemia variants, ARID5B and IKZF1, and interaction with parental environmental exposures.

Directory of Open Access Journals (Sweden)

Tiffany-Jane Evans

Full Text Available Genome wide association studies (GWAS have established association of ARID5B and IKZF1 variants with childhood acute lymphoblastic leukemia (ALL. Epidemiological studies suggest that environmental factors alone appear to make a relatively minor contribution to disease risk. The polygenic nature of childhood ALL predisposition together with the timing of environmental triggers may hold vital clues for disease etiology. This study presents results from an Australian GWAS of childhood ALL cases (n = 358 and population controls (n = 1192. Furthermore, we utilised family trio (n = 204 genotypes to extend our investigation to gene-environment interaction of significant loci with parental exposures before conception, and child's sex and age. Thirteen SNPs achieved genome wide significance in the population based case/control analysis; ten annotated to ARID5B and three to IKZF1. The most significant SNPs in these regions were ARID5B rs4245595 (OR 1.63, CI 1.38-1.93, P = 2.13×10(-9, and IKZF1 rs1110701 (OR 1.69, CI 1.42-2.02, p = 7.26×10(-9. There was evidence of gene-environment interaction for risk genotype at IKZF1, whereby an apparently stronger genetic effect was observed if the mother took folic acid or if the father did not smoke prior to pregnancy (respective interaction P-values: 0.04, 0.05. There were no interactions of risk genotypes with age or sex (P-values >0.2. Our results evidence that interaction of genetic variants and environmental exposures may further alter risk of childhood ALL however, investigation in a larger population is required. If interaction of folic acid supplementation and IKZF1 variants holds, it may be useful to quantify folate levels prior to initiating use of folic acid supplements.

Maintenance therapy of childhood acute lymphoblastic leukemia revisited—Should drug doses be adjusted by white blood cell, neutrophil, or lymphocyte counts?

DEFF Research Database (Denmark)

Schmiegelow, Kjeld; Nersting, Jacob; Nielsen, Stine Nygaard

2016-01-01

BACKGROUND: 6-Mercaptopurine (6MP) and methotrexate (MTX) based maintenance therapy is a critical phase of childhood acute lymphoblastic leukemia treatment. Wide interindividual variations in drug disposition warrant frequent doses adjustments, but there is a lack of international consensus on dose...... levels of 6-thioguanine nucleotides or MTX (including its polyglutamates) to be significant relapse predictors. The parameters significantly associated with risk of relapse (N = 83) were male sex (hazard ratio [HR] 2.0 [1.3-3.1], P = 0.003), WBC at diagnosis (HR = 1.04 per 10 × 10(9) /l rise [1...

Sporadic inclusion body myositis: the genetic contributions to the pathogenesis

Science.gov (United States)

2014-01-01

Sporadic inclusion body myositis (sIBM) is the commonest idiopathic inflammatory muscle disease in people over 50 years old. It is characterized by slowly progressive muscle weakness and atrophy, with typical pathological changes of inflammation, degeneration and mitochondrial abnormality in affected muscle fibres. The cause(s) of sIBM are still unknown, but are considered complex, with the contribution of multiple factors such as environmental triggers, ageing and genetic susceptibility. This review summarizes the current understanding of the genetic contributions to sIBM and provides some insights for future research in this mysterious disease with the advantage of the rapid development of advanced genetic technology. An international sIBM genetic study is ongoing and whole-exome sequencing will be applied in a large cohort of sIBM patients with the aim of unravelling important genetic risk factors for sIBM. PMID:24948216

Indoor radon and childhood leukaemia

International Nuclear Information System (INIS)

Raaschou-Nielsen, O.

2008-01-01

This paper summarises the epidemiological literature on domestic exposure to radon and risk for childhood leukaemia. The results of 12 ecological studies show a consistent pattern of higher incidence and mortality rates for childhood leukaemia in areas with higher average indoor radon concentrations. Although the results of such studies are useful to generate hypotheses, they must be interpreted with caution, as the data were aggregated and analysed for geographical areas and not for individuals. The seven available case - control studies of childhood leukaemia with measurement of radon concentrations in the residences of cases and controls gave mixed results, however, with some indication of a weak (relative risk < 2) association with acute lymphoblastic leukaemia. The epidemiological evidence to date suggests that an association between indoor exposure to radon and childhood leukaemia might exist, but is weak. More case - control studies are needed, with sufficient statistical power to detect weak associations and based on designs and methods that minimise misclassification of exposure and provide a high participation rate and low potential selection bias. (authors)

Are repeated single-limb heel raises and manual muscle testing associated with peak plantar-flexor force in people with inclusion body myositis?

Science.gov (United States)

Harris-Love, Michael O; Shrader, Joseph A; Davenport, Todd E; Joe, Galen; Rakocevic, Goran; McElroy, Beverly; Dalakas, Marinos

2014-04-01

Repeated heel raises have been proposed as a method of ankle plantar-flexor strength testing that circumvents the limitations of manual muscle testing (MMT). The study objective was to examine the relationships among ankle plantar-flexion isometric maximum voluntary contraction (MVC), repeated single-limb heel raises (SLHRs), and MMT in people with myositis. This was a cross-sectional study with a between-group design. The ability to complete 1 SLHR determined group assignment (SLHR group, n=24; no-SLHR group, n=19). Forty-three participants with myositis (13 women; median age=64.9 years) participated. Outcome measures included MVC, predicted MVC, Kendall MMT, and Daniels-Worthingham MMT. The Kendall MMT was unable to detect significant ankle plantar-flexor weakness established by quantitative methods and was unable to discriminate between participants who could and those who could not perform the SLHR task. Ankle plantar-flexion MVC was not associated with the number of heel-raise repetitions in the SLHR group (pseudo R(2)=.13). No significant relationship was observed between MVC values and MMT grades in the SLHR and no-SLHR groups. However, a moderate relationship between MVC values and MMT grades was evident in a combined-group analysis (ρ=.50-.67). The lower half of both MMT grading scales was not represented in the study despite the profound weakness of the participants. Both Kendall MMT and Daniels-Worthingham MMT had limited utility in the assessment of ankle plantar-flexor strength. Repeated SLHRs should not be used as a proxy measure of ankle plantar-flexion MVC in people with myositis.

Differential diagnosis of the acute abdomen. Pt. 2

International Nuclear Information System (INIS)

Wiesner, Walter; Kirchhoff, Timm D.; Opherk, Jan Patrick

2009-01-01

The imaging of the acute abdomen has undergone fundamental changes in the recent past. The diagnostic strategy requires an up-date since ultrasound and computed-tomography have replaced classic radiography in cases of acute lower abdominal pain as well. The present contribution is the second of four reviews on this issue and focuses on the acute lower quadrant pain while part three will depict diffuse acute abdominal pain and, finally, the diagnostic strategy of the acute abdomen in childhood will be described in part four. (orig.)

Intercostal myositis ossificans misdiagnosed as osteosarcoma in a 10-year-old child

International Nuclear Information System (INIS)

Koob, Meriam; Durckel, Jean; Dosch, Jean-Claude; Dietemann, Jean-Louis; Entz-Werle, Natacha

2010-01-01

Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation within soft tissue. It most commonly affects adolescents and young adults, typically in the limbs and following trauma. Very few cases have been reported in children. We report here a case of nontraumatic MO occurring in a 10-year-old girl with an uncommon location in the 5th right intercostal space; it was initially misdiagnosed and treated as osteosarcoma. Imaging findings including plain radiographs, CT, MRI, bone scintigraphy and PET-CT are described. This case highlights the central role played by imaging in diagnosis, thus avoiding biopsy that can erroneously suggest osteosarcoma as the diagnosis, as occurred in this case. (orig.)

Asymptomatic hyper-creatine-kinase-emia as sole manifestation of inclusion body myositis

Directory of Open Access Journals (Sweden)

Josef Finsterer

2013-06-01

Full Text Available Sporadic inclusion body myositis (sIBM usually manifests with painless weakness of the hand, finger and hip flexors. Absence of symptoms or signs, but mild hyper-CK-emia as the sole manifestation of IBM, has not been reported. We report the case of a 73-year-old male who presented with asymptomatic recurrent hyper-CK-emia ranging from 200 to 1324 U/L (n<171 U/L, since 10 years. Clinical neurologic investigation, nerve conduction studies and EMG were non-informative. Muscle biopsy surprisingly revealed sIBM. sIBM may be asymptomatic and may manifest with hyper-CK-emia exclusively. So, it has to be included in the differential diagnoses of asymptomatic hyper-CK-emia.

Intercostal myositis ossificans misdiagnosed as osteosarcoma in a 10-year-old child

Energy Technology Data Exchange (ETDEWEB)

Koob, Meriam; Durckel, Jean; Dosch, Jean-Claude; Dietemann, Jean-Louis [Hopital de Hautepierre, Service de Radiologie II, Hopitaux Universitaires, Strasbourg Cedex (France); Entz-Werle, Natacha [Hopitaux Universitaires, Hopital de Hautepierre, Service d' Onco-hematologie pediatrique, Strasbourg Cedex (France)

2010-12-15

Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation within soft tissue. It most commonly affects adolescents and young adults, typically in the limbs and following trauma. Very few cases have been reported in children. We report here a case of nontraumatic MO occurring in a 10-year-old girl with an uncommon location in the 5th right intercostal space; it was initially misdiagnosed and treated as osteosarcoma. Imaging findings including plain radiographs, CT, MRI, bone scintigraphy and PET-CT are described. This case highlights the central role played by imaging in diagnosis, thus avoiding biopsy that can erroneously suggest osteosarcoma as the diagnosis, as occurred in this case. (orig.)

Anti-Erwinia asparaginase antibodies during treatment of childhood acute lymphoblastic leukemia and their relationship to outcome

DEFF Research Database (Denmark)

Albertsen, BK; Schmiegelow, Kjeld; Schrøder, Henrik

2002-01-01

PURPOSE: A case-control study was performed to determine whether patients who had been treated with Erwinia asparaginase as part of their treatment for childhood acute lymphoblastic leukemia (ALL) and who showed relapsed of their disease more often developed anti-asparaginase antibodies than...... (median follow-up 70 months). Anti- Erwinia asparaginase antibodies were measured (ELISA method) during maintenance therapy after asparaginase treatment (30,000 IU/m(2) daily for 10 days in all patients plus twice weekly for 2 weeks in intermediate-risk and high-risk ALL patients). RESULTS: The overall...... incidence of anti- Erwinia asparaginase antibodies was 8% (3 of 39 patients). There was no statistically significant difference in the incidence of antibody formation between patients who had suffered relapse (1 of 13) and those who had not (2 of 26). In two of the three patients who developed antibodies...

Pediatric intracerebral hemorrhage: acute symptomatic seizures and epilepsy.

Science.gov (United States)

Beslow, Lauren A; Abend, Nicholas S; Gindville, Melissa C; Bastian, Rachel A; Licht, Daniel J; Smith, Sabrina E; Hillis, Argye E; Ichord, Rebecca N; Jordan, Lori C

2013-04-01

Seizures are believed to be common presenting symptoms in neonates and children with spontaneous intracerebral hemorrhage (ICH). However, few data are available on the epidemiology of acute symptomatic seizures or the risk for later epilepsy. To define the incidence of and explore risk factors for seizures and epilepsy in children with spontaneous ICH. Our a priori hypotheses were that younger age at presentation, cortical involvement of ICH, acute symptomatic seizures after presentation, ICH due to vascular malformation, and elevated intracranial pressure requiring urgent intervention would predict remote symptomatic seizures and epilepsy. Prospective cohort study conducted between March 1, 2007, and January 1, 2012. Three tertiary care pediatric hospitals. Seventy-three pediatric subjects with spontaneous ICH including 20 perinatal (≥37 weeks' gestation to 28 days) and 53 childhood subjects (>28 days to Acute symptomatic seizures (clinically evident and electrographic-only seizures within 7 days), remote symptomatic seizures, and epilepsy. Acute symptomatic seizures occurred in 35 subjects (48%). Acute symptomatic seizures as a presenting symptom of ICH occurred in 12 perinatal (60%) and 19 childhood (36%) subjects (P = .07). Acute symptomatic seizures after presentation occurred in 7 children. Electrographic-only seizures were present in 9 of 32 subjects (28%) with continuous electroencephalogram monitoring. One-year and 2-year remote symptomatic seizure-free survival rates were 82% (95% CI, 68-90) and 67% (95% CI, 46-82), respectively. One-year and 2-year epilepsy-free survival rates were 96% (95% CI, 83-99) and 87% (95% CI, 65-95), respectively. Elevated intracranial pressure requiring acute intervention was a risk factor for seizures after presentation (P = .01; Fisher exact test), remote symptomatic seizures, and epilepsy (P = .03, and P = .04, respectively; log-rank test). Presenting seizures are common in perinatal and childhood ICH. Continuous

Contribution of dot-blot assay to the diagnosis and management of myositis: a three-year practice at a university hospital centre.

Science.gov (United States)

Martel, Clothilde; Vignaud, Guillaume; Liozon, Eric; Magy, Laurent; Gallouedec, Gael; Ly, Kim; Bezanahary, Holly; Cypierre, Anne; Lapébie, François-Xavier; Palat, Sylvain; Gondran, Guillaume; Jauberteau, Marie-Odile; Fauchais, Anne-Laure

2016-01-01

Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune diseases with wide clinical spectrum that may lead to delayed diagnosis. The aim of this study was to examine the impact of IIM-specific dot-blot assay on diagnostic process of patients presenting with muscular or systemic symptoms evocating of IIM. We collected all the prescriptions of an IIM specific dot-blot assay (8 autoantigens including Jo-1, PL-7, PL-12, SRP, Mi-2, Ku, PM/Scl and Scl-70) over a 38-month period. 316 myositis dot-blot assays (MSD) were performed in 274 patients (156 women, mean age 53±10.6 years) referring for muscular and/or systemic symptoms suggesting IIM. The timing of dot prescription through the diagnostic process was highly variable: without (35%), concomitantly (16%) or after electromyographic studies (35%). Fifty-nine patients (22%) had IIM according to Bohan and Peter's criteria. Among them, 29 (49%) had positive dot (8 Jo-1, 6 PM-Scl, 5 PL-12, 5 SRP, 2 Mi-2, 2 PL-7 and 1 Ku). Various other diagnoses were performed including 35 autoimmune disease or granulomatosis (12%), 19 inflammatory rheumatic disease (7%), 16 non inflammatory muscular disorders (6%), 10 drug-induced myalgia (4%), 11 infectious myositis (4%). Except 11 borderline SRP results and one transient PM-Scl, MSD was positive only in one case of IIM. Dot allowed clinicians to correct diagnosis in 4 cases and improved the diagnosis of IIM subtypes in 4 cases. This study reflects the interest of myositis dot in the rapid diagnosis process of patients with non-specific muscular symptoms leading to various diagnoses including IIM.

Human rotavirus genotypes causing acute watery diarrhea among ...

African Journals Online (AJOL)

Background: Diarrhea is a major cause of childhood morbidity and mortality in the developing countries. Rotavirus is a major cause of acute watery diarrhea. Aim: This study aims at characterizing the prevalent rotavirus G-genotypes among under.five children presenting with acute watery diarrhea in Benin City, Nigeria.

Childhood Leukemia--A Look at the Past, the Present and the Future.

Science.gov (United States)

Findeisen, Regina; Barber, William H.

1997-01-01

Provides an overview of childhood leukemia. The causes, the survival period, different types (acute lymphocytic leukemia, acute myeloid leukemia, chronic lymphocytic leukemia, chronic myeloid leukemia, and hairy cell leukemia), symptoms, treatment, side effects of treatment (including learning problems), and the expected future direction of…

Radionuclide bone scintigraphy in pediatric orthopedics

International Nuclear Information System (INIS)

Conway, J.J.

1986-01-01

Radionuclide bone scintigraphy is highly sensitive and specific for diagnosing the musculoskeletal disorders of childhood. Conditions such as neonatal osteomyelitis, septic arthritis, diskitis of childhood, Legg-Calve-Perthes disease, the osteochondroses, the toddler's fracture, sports injuries, spondylolysis, myositis ossificians, and reflex sympathetic dystrophy are readily defined. High-quality state-of-the-art scintigraphy is essential in infants and young children. 64 references

AR-42 and Decitabine in Treating Patients With Acute Myeloid Leukemia

Science.gov (United States)

2018-03-12

Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Secondary Acute Myeloid Leukemia; Untreated Adult Acute Myeloid Leukemia

Cured meat, vegetables, and bean-curd foods in relation to childhood acute leukemia risk: a population based case-control study.

Science.gov (United States)

Liu, Chen-Yu; Hsu, Yi-Hsiang; Wu, Ming-Tsang; Pan, Pi-Chen; Ho, Chi-Kung; Su, Li; Xu, Xin; Li, Yi; Christiani, David C

2009-01-13

Consumption of cured/smoked meat and fish leads to the formation of carcinogenic N-nitroso compounds in the acidic stomach. This study investigated whether consumed cured/smoked meat and fish, the major dietary resource for exposure to nitrites and nitrosamines, is associated with childhood acute leukemia. A population-based case-control study of Han Chinese between 2 and 20 years old was conducted in southern Taiwan. 145 acute leukemia cases and 370 age- and sex-matched controls were recruited between 1997 and 2005. Dietary data were obtained from a questionnaire. Multiple logistic regression models were used in data analyses. Consumption of cured/smoked meat and fish more than once a week was associated with an increased risk of acute leukemia (OR = 1.74; 95% CI: 1.15-2.64). Conversely, higher intake of vegetables (OR = 0.55; 95% CI: 0.37-0.83) and bean-curd (OR = 0.55; 95% CI: 0.34-0.89) was associated with a reduced risk. No statistically significant association was observed between leukemia risk and the consumption of pickled vegetables, fruits, and tea. Dietary exposure to cured/smoked meat and fish may be associated with leukemia risk through their contents of nitrites and nitrosamines among children and adolescents, and intake of vegetables and soy-bean curd may be protective.

DNA methylation for subtype classification and prediction of treatment outcome in patients with childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Milani, Lili; Lundmark, Anders; Kiialainen, Anna

2010-01-01

CpG sites in regulatory regions of 416 genes in cells from 401 children diagnosed with ALL. Hierarchical clustering of 300 CpG sites distinguished between T-lineage ALL and B-cell precursor (BCP) ALL and between the main cytogenetic subtypes of BCP ALL. It also stratified patients with high......Despite improvements in the prognosis of childhood acute lymphoblastic leukemia (ALL), subgroups of patients would benefit from alternative treatment approaches. Our aim was to identify genes with DNA methylation profiles that could identify such groups. We determined the methylation levels of 1320...... hyperdiploidy and t(12;21) ALL into 2 subgroups with different probability of relapse. By using supervised learning, we constructed multivariate classifiers by external cross-validation procedures. We identified 40 genes that consistently contributed to accurate discrimination between the main subtypes of BCP...

Family-based exome-wide association study of childhood acute lymphoblastic leukemia among Hispanics confirms role of ARID5B in susceptibility

Science.gov (United States)

Stoltze, Ulrik; Scheurer, Michael E.; Wilkinson, Anna V.; Lin, Ting-Nien; Qian, Maoxiang; Goodings, Charnise; Swartz, Michael D.; Ranjit, Nalini; Rabin, Karen R.; Peckham-Gregory, Erin C.; Plon, Sharon E.; de Alarcon, Pedro A.; Zabriskie, Ryan C.; Antillon-Klussmann, Federico; Najera, Cesar R.; Yang, Jun J.

2017-01-01

We conducted an exome-wide association study of childhood acute lymphoblastic leukemia (ALL) among Hispanics to confirm and identify novel variants associated with disease risk in this population. We used a case-parent trio study design; unlike more commonly used case-control studies, this study design is ideal for avoiding issues with population stratification bias among this at-risk ethnic group. Using 710 individuals from 323 Guatemalan and US Hispanic families, two inherited SNPs in ARID5B reached genome-wide level significance: rs10821936, RR = 2.31, 95% CI = 1.70–3.14, p = 1.7×10−8 and rs7089424, RR = 2.22, 95% CI = 1.64–3.01, p = 5.2×10−8. Similar results were observed when restricting our analyses to those with the B-ALL subtype: ARID5B rs10821936 RR = 2.22, 95% CI = 1.63–3.02, p = 9.63×10−8 and ARID5B rs7089424 RR = 2.13, 95% CI = 1.57–2.88, p = 2.81×10−7. Notably, effect sizes observed for rs7089424 and rs10821936 in our study were >20% higher than those reported among non-Hispanic white populations in previous genetic association studies. Our results confirmed the role of ARID5B in childhood ALL susceptibility among Hispanics; however, our assessment did not reveal any strong novel inherited genetic risks for acute lymphoblastic leukemia among this ethnic group. PMID:28817678

The association of folate pathway and DNA repair polymorphisms with susceptibility to childhood acute lymphoblastic leukemia.

Science.gov (United States)

Goričar, Katja; Erčulj, Nina; Faganel Kotnik, Barbara; Debeljak, Maruša; Hovnik, Tinka; Jazbec, Janez; Dolžan, Vita

2015-05-15

Genetic factors may play an important role in susceptibility to childhood acute lymphoblastic leukemia (ALL). The aim of our study was to evaluate the associations of genetic polymorphisms in folate pathway and DNA repair genes with susceptibility to ALL. In total, 121 children with ALL and 184 unrelated healthy controls of Slovenian origin were genotyped for 14 polymorphisms in seven genes of folate pathway, base excision repair and homologous recombination repair (TYMS, MTHFR, OGG1, XRCC1, NBN, RAD51, and XRCC3). In addition, the exon 6 of NBN was screened for the presence of mutations using denaturing high performance liquid chromatography. Twelve polymorphisms were in Hardy-Weinberg equilibrium in controls and their genotype frequencies were in agreement with those reported in other Caucasian populations. Among the investigated polymorphisms and mutations, NBN Glu185Gln significantly decreased susceptibility to B-cell ALL (p=0.037), while TYMS 3R allele decreased susceptibility to T-cell ALL (p=0.011). Moreover, significantly decreased susceptibility to ALL was observed for MTHFR TA (p=0.030) and RAD51 GTT haplotypes (p=0.016). Susceptibility to ALL increased with the increasing number of risk alleles (ptrend=0.007). We also observed significant influence of hOGG-RAD51 and NBN-RAD51 interactions on susceptibility to ALL. Our results suggest that combination of several polymorphisms in DNA repair and folate pathways may significantly affect susceptibility to childhood ALL. Copyright © 2015 Elsevier B.V. All rights reserved.

FDG PET/CT findings in a case of myositis ossificans circumscripta of the forearm.

Science.gov (United States)

Clarençon, Frédéric; Larousserie, Frédérique; Babinet, Antoine; Zylbersztein, Christophe; Talbot, Jean-Noël; Kerrou, Khaldoun

2011-01-01

Myositis ossificans circumscripta (MOC) is a rare benign neoplasm located in soft tissues that, most of the time, appears after a local trauma. The positive diagnosis of MOC may be challenging on CT or MRI findings. We report on an atypical case of a spontaneous nontraumatic MOC in a 54-year-old man, located in the longus supinatus muscle diagnosed with MRI and F-18 FDG PET/CT findings. Rarely described F-18 FDG PET/CT features in MOC are presented. Pattern of avid FDG focus on PET/CT, that may wrongly suggest osteosarcoma, is presented.

Molecular biomarkers for the study of childhood leukemia

International Nuclear Information System (INIS)

Smith, Martyn T.; McHale, Cliona M.; Wiemels, Joseph L.; Zhang, Luoping; Wiencke, John K.; Zheng, Shichun; Gunn, Laura; Skibola, Christine F.; Ma, Xiaomei; Buffler, Patricia A.

2005-01-01

Various specific chromosome rearrangements, including t(8;21), t(15;17), and inv(16), are found in acute myeloid leukemia (AML) and in childhood acute lymphocytic leukemia (ALL), t(12;21) and t(1;19) are common. We sequenced the translocation breakpoints of 56 patients with childhood ALL or AML harboring t(12;21), t(8;21), t(15;17), inv(16), and t(1;19), and demonstrated, with the notable exception of t(1;19), that these rearrangements are commonly detected in the neonatal blood spots (Guthrie cards) of the cases. These findings show that most childhood leukemias begin before birth and that maternal and perinatal exposures such as chemical and infectious agents are likely to be critical. Indeed, we have reported that exposure to indoor pesticides during pregnancy and the first year of life raises leukemia risk, but that later exposures do not. We have also examined aberrant gene methylation in different cytogenetic subgroups and have found striking differences between them, suggesting that epigenetic events are also important in the development of some forms of childhood leukemia. Further, at least two studies now show that the inactivating NAD(P)H:quinone acceptor oxidoreductase (NQO1) C609T polymorphism is positively associated with leukemias arising in the first 1-2 years of life and polymorphisms in the 5,10-methylenetetrahydrofolate reductase (MTHFR) gene have been associated with adult and childhood ALL. Thus, low folate intake and compounds that are detoxified by NQO1 may be important in elevating leukemia risk in children. Finally, we are exploring the use of proteomics to subclassify leukemia, because cytogenetic analysis is costly and time-consuming. Several proteins have been identified that may serve as useful biomarkers for rapidly identifying different forms of childhood leukemia

Neuropsychological sequelae of central nervous system prophylaxis in survivors of childhood acute lymphoblastic leukemia

International Nuclear Information System (INIS)

Said, J.A.; Waters, B.G.; Cousens, P.; Stevens, M.M.

1989-01-01

We assessed neuropsychologically 106 children with acute lymphoblastic leukemia (ALL) who had all received cranial irradiation for the prevention of central nervous system (CNS) leukemia 1-13 years previously. Children were assessed for adverse late effects of their therapy, using age-appropriate Wechsler measures of overall intellectual ability and supplementary tests. Forty-five siblings near in age to the patients were tested as controls. The patients who had had the most intensive central nervous system (CNS) prophylaxis were found to have a WISC-R Full Scale IQ 17 points lower than the sibling control group. Performance IQ was more affected than verbal IQ. The patients were more easily distracted and less able to concentrate. The severity of the aftereffects was related to younger age at the time of CNS prophylaxis and to a higher dose of cranial irradiation but not to time since CNS prophylaxis. CNS prophylaxis using a combination of cranial irradiation and intrathecal methotrexate has lowered the incidence of CNS relapse in childhood ALL but is associated with considerable long-term morbidity in survivors

Acute external otitis as debut of acute myeloid leukemia - A case and review of the literature.

Science.gov (United States)

Slengerik-Hansen, Joachim; Ovesen, Therese

2018-03-01

Acute leukemia is a well known childhood cancer. The relation between leukemia and otological symptoms has long been established but is highly rare as a debut symptom of leukemia. External otitis is a common condition affecting many children, and most cases are successively treated with topical medicine. Here we present a child with acute external otitis later shown to be the debut symptom of acute myeloid leukemia, to our knowledge the first specific case described. We have reviewed the literature to find red flags for suspicion of severe disease in case of acute external otitis. Copyright © 2018 Elsevier B.V. All rights reserved.

Common childhood kidney diseases in Uganda and their prevention.

African Journals Online (AJOL)

2016-03-10

Mar 10, 2016 ... to households, health education on personal and food hygiene, childhood ... treatment of mal- nutrition will contribute to primary prevention of AKI ... maintaining steady state haemoglobin, and treating acute infections are ...

Childhood acute lower respiratory tract infections in Northern Nigeria

African Journals Online (AJOL)

2015-03-02

Mar 2, 2015 ... cause of childhood morbidity and deaths worldwide1, 2. Of the estimated 7.6 ... years of age with community-acquired pneumonia. (CAP) would require ..... mon respiratory pathogens, such as Haemophilus influ- enzae and ...

Doença pulmonar intersticial relacionada a miosite e a síndrome antissintetase Myositis-related interstitial lung disease and antisynthetase syndrome

Directory of Open Access Journals (Sweden)

Joshua Solomon

2011-02-01

Full Text Available Em pacientes com miosite, é comum o comprometimento pulmonar, e a presença de anticorpos anti-aminoacil-RNAt sintetase (anti-ARS é preditora da presença ou do desenvolvimento de doença pulmonar intersticial (DPI. Uma entidade clínica distinta - a síndrome antissintetase - é caracterizada pela presença de anticorpos anti-ARS, miosite, DPI, artrite, fenômeno de Raynaud e "mãos de mecânico". O mais comum anticorpo anti-ARS é o anti-Jo-1. Anticorpos anti-ARS mais recentemente descritos podem conferir um fenótipo que é distinto daquele de pacientes com positividade para anti-Jo-1, sendo caracterizado por uma menor incidência de miosite e uma maior incidência de DPI. Nos pacientes com DPI relacionada à síndrome antissintetase, a resposta a medicações imunossupressoras é em geral favorável.In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS antibodies marks the presence or predicts the development of interstitial lung disease (ILD. A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

Application of nuclear medicine to heart diseases in childhood

International Nuclear Information System (INIS)

Tsuji, Atsutoshi; Osano, Mitsuru.

1981-01-01

Various procedures to obtain radioisotopic images of the cardiovascular system were described, and representative cases in childhood were presented. Apparatuses, radiopharmaceuticals such as sup(99m)Tc pertechnatate, 201 Thallium, and 133 Xenon gas, and their dosage in childhood were briefly reviewed. Several cases of persistent fetal circulation, common A-V valve, tetralogy of Fallot, pulmonary atresia, etc. were presented with figures with special emphasis on acute febrile mucocutaneous lymph node syndrome and 201 Thallium myocardial imaging. (Kondo, M.)

Influence of IL15 gene variations on the clinical features, treatment response and risk of developing childhood acute lymphoblastic leukemia in Latvian population.

Science.gov (United States)

Rots, Dmitrijs; Kreile, Madara; Nikulshin, Sergejs; Kovalova, Zhanna; Gailite, Linda

2018-02-01

Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy. Modern treatment protocols allow achievement of long-term event-free survival rates in up to 85% of cases, although the treatment response varies among different patient groups. It is hypothesized that treatment response is influenced by the IL15 gene variations, although research results are conflicting. To analyze IL15 gene variations influence treatment response, clinical course and the risk of developing ALL we performed a case-control and family-based study. The study included 81 patients with childhood ALL. DNA samples of both or one biological parent were available for 62 of ALL patients and 130 age and gender adjusted healthy samples were used as a control group. Analyzed IL15 gene variations: rs10519612, rs10519613 and rs17007695 were genotyped using PCR-RFLP assay. Our results shows that IL15 gene variations haplotypes are associated with the risk of developing childhood ALL (p variations separately. The variations rs10519612 and rs1059613 in a recessive pattern of inheritance were associated with hyperdiploidy (p = 0.048). Analyzed genetic variations had no impact on other clinical features and treatment response (assessed by the minimal residual disease) in our study.

Myositis ossificans: a false positive of malignancy in PET - CT F.D.G. and in osseous scintigraphy with HMDP 99Tc

International Nuclear Information System (INIS)

Hassler, S.; Bourahla, K.

2010-01-01

Myositis ossificans is a difficult differential diagnosis and a false positive for malignancy in both 99m Tc H.M.D.P. (hydroxy-methylene diphosphonate) scintigraphy and in exploration PET-F.D.G. This hypothesis should be mentioned in case of young patients, despite the unusual scintigraphic appearance in a benign process. (N.C.)

Calcium dysregulation, functional calpainopathy, and endoplasmic reticulum stress in sporadic inclusion body myositis.

Science.gov (United States)

Amici, David R; Pinal-Fernandez, Iago; Mázala, Davi A G; Lloyd, Thomas E; Corse, Andrea M; Christopher-Stine, Lisa; Mammen, Andrew L; Chin, Eva R

2017-03-22

Sporadic inclusion body myositis (IBM) is the most common primary myopathy in the elderly, but its pathoetiology is still unclear. Perturbed myocellular calcium (Ca 2+ ) homeostasis can exacerbate many of the factors proposed to mediate muscle degeneration in IBM, such as mitochondrial dysfunction, protein aggregation, and endoplasmic reticulum stress. Ca 2+ dysregulation may plausibly be initiated in IBM by immune-mediated membrane damage and/or abnormally accumulating proteins, but no studies to date have investigated Ca 2+ regulation in IBM patients. We first investigated protein expression via immunoblot in muscle biopsies from IBM, dermatomyositis, and non-myositis control patients, identifying several differentially expressed Ca 2+ -regulatory proteins in IBM. Next, we investigated the Ca 2+ -signaling transcriptome by RNA-seq, finding 54 of 183 (29.5%) genes from an unbiased list differentially expressed in IBM vs. controls. Using an established statistical approach to relate genes with causal transcription networks, Ca 2+ abundance was considered a significant upstream regulator of observed whole-transcriptome changes. Post-hoc analyses of Ca 2+ -regulatory mRNA and protein data indicated a lower protein to transcript ratio in IBM vs. controls, which we hypothesized may relate to increased Ca 2+ -dependent proteolysis and decreased protein translation. Supporting this hypothesis, we observed robust (4-fold) elevation in the autolytic activation of a Ca 2+ -activated protease, calpain-1, as well as increased signaling for translational attenuation (eIF2a phosphorylation) downstream of the unfolded protein response. Finally, in IBM samples we observed mRNA and protein under-expression of calpain-3, the skeletal muscle-specific calpain, which broadly supports proper Ca 2+ homeostasis. Together, these data provide novel insight into mechanisms by which intracellular Ca 2+ regulation is perturbed in IBM and offer evidence of pathological downstream effects.

Guillain-Barre Syndrome Presenting as Acute Abdomen

Directory of Open Access Journals (Sweden)

Faruk incecik

2015-09-01

Full Text Available Guillain-Barr and eacute; syndrome (GBS is the most common cause of acute flaccid paralysis in childhood. Symmetric weakness, headache, respiratory symptom, neuropathic pain, muscle pain, paresthesia, and facial palsy were the most common clinical presentations. We report 13-year-old boy with GBS who presented with acute abdominal pain. This is the first report, to our knowledge, first presented of acute abdomen of a pediatric patient with GBS. [Cukurova Med J 2015; 40(3.000: 601-603

Sporadic inclusion body myositis: pilot study on the effects of a home exercise program on muscle function, histopathology and inflammatory reaction.

Science.gov (United States)

Arnardottir, Snjolaug; Alexanderson, Helene; Lundberg, Ingrid E; Borg, Kristian

2003-01-01

To evaluate the safety and effect of a home training program on muscle function in 7 patients with sporadic inclusion body myositis. The patients performed exercise 5 days a week over a 12-week period. Safety was assessed by clinical examination, repeated muscle biopsies and serum levels of creatine kinase. Muscle strength was evaluated by clinical examination, dynamic dynamometer and by a functional index in myositis. Strength was not significantly improved after the exercise, however none of the patients deteriorated concerning muscle function. The histopathology was unchanged and there were no signs of increased muscle inflammation or of expression of cytokines and adhesion molecules in the muscle biopsies. Creatine kinase levels were unchanged. A significant decrease was found in the areas that were positively stained for EN-4 (a marker for endothelial cells) in the muscle biopsies after training. The home exercise program was considered as not harmful to the muscles regarding muscle inflammation and function. Exercise may prevent loss of muscle strength due to disease and/or inactivity.

Myositis ossificans around shoulder following military training programme

Directory of Open Access Journals (Sweden)

Mustafa C Kir

2011-01-01

Full Text Available The myositis ossificans around shoulder in military recruits are not reported yet. Three young male soldiers presented with complaints of palpable mass at the anterior aspect of shoulder; tenderness around the superior part of deltopectoral groove close to acromioclavicular joint; and restriction of shoulder motion. They also noticed ecchymosis and pain around the coracoid process and anterior shoulder region during regular firing exercises. Plain X-rays and computerized tomography showed extra-capsular, dense, irregular structure in the space between pectoralis and deltoid muscles which correlated with heterotopic bone. One patient refused surgical intervention because of the completion of his military serving period. Surgical excision was performed for the other two patients. During surgical exploration, both ossified masses were found in deltopectoral region and mostly in fibers of clavicular and acromial parts of deltoid muscle. Pathological reports confirmed the structure of masses as mature trabecular bone. Postoperatively indomethacin treatment and active shoulder exercises were started until the full range of motion was regained. Mini soft bag was used on the rifle contact area of the shoulder. No complications or recurrences were observed during the 24 months of followup period.

Radionuclide bone scintigraphy in pediatric orthopedics

Energy Technology Data Exchange (ETDEWEB)

Conway, J.J.

1986-12-01

Radionuclide bone scintigraphy is highly sensitive and specific for diagnosing the musculoskeletal disorders of childhood. Conditions such as neonatal osteomyelitis, septic arthritis, diskitis of childhood, Legg-Calve-Perthes disease, the osteochondroses, the toddler's fracture, sports injuries, spondylolysis, myositis ossificians, and reflex sympathetic dystrophy are readily defined. High-quality state-of-the-art scintigraphy is essential in infants and young children. 64 references.

Acute lymphoblastic leukemia mimicking Wilms tumor at presentation.

Science.gov (United States)

Singh, Amitabh; Mandal, Anirban; Guru, Vijay; Seth, Rachna

2016-09-01

Acute lymphoblastic leukemia (ALL), the commonest malignancy of childhood, is known to manifest with a myriad of atypical presentations. Nephromegaly is a rare presentation of childhood ALL with hepatic mass being even rarer. We present a 3 year-old child with unilateral renal mass and hepatic mass lesion with normal blood counts, initially suspected to have metastatic Wilms tumor based on clinical, radiological and WT1 positivity on immunocytochemistry of renal mass. He was later diagnosed as ALL with peripheral blood flowcytometry and bone marrow examination. Renomegaly at presentation of acute leukemia is not necessarily due to leukemic infiltration and rarely leads to renal impairment. The radiological differential of such a renal mass includes both benign and malignant entities including metastasis. Over-expression of WT1 mRNA has been found in a number of solid tumors and hematological malignancies and is far from being diagnostic of Wilms tumor. Again, a small number of children with acute leukemia may have a deceptively normal complete blood count at presentation. Though, initial all (clinical, radiological, hematological, and immunocytological) parameters pointed towards a diagnosis of Wilms tumor in our case, the subsequent development of thrombocytopenia and lymphocytic leukocytosis prompted further investigation and final diagnosis of ALL. WT1 positivity is a known phenomenon in childhood ALL and undifferentiated lymphoblasts may be positive for WT1 and negative for Leucocyte common antigen. Acute leukemia with renal and hepatic mass with normal blood counts at presentation is a diagnostic challenge.

DNA methylation for subtype classification and prediction of treatment outcome in patients with childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Milani, Lili; Lundmark, Anders; Kiialainen, Anna

2010-01-01

Despite improvements in the prognosis of childhood acute lymphoblastic leukemia (ALL), subgroups of patients would benefit from alternative treatment approaches. Our aim was to identify genes with DNA methylation profiles that could identify such groups. We determined the methylation levels of 1320...... CpG sites in regulatory regions of 416 genes in cells from 401 children diagnosed with ALL. Hierarchical clustering of 300 CpG sites distinguished between T-lineage ALL and B-cell precursor (BCP) ALL and between the main cytogenetic subtypes of BCP ALL. It also stratified patients with high...... ALL and gene sets that discriminated between subtypes of ALL and between ALL and controls in pairwise classification analyses. We also identified 20 individual genes with DNA methylation levels that predicted relapse of leukemia. Thus, methylation analysis should be explored as a method to improve...

TLR4-HMGB1 signaling pathway affects the inflammatory reaction of autoimmune myositis by regulating MHC-I.

Science.gov (United States)

Wan, Zemin; Zhang, Xiujuan; Peng, Anping; He, Min; Lei, Zhenhua; Wang, Yunxiu

2016-12-01

To analyze the effects of TLR4 on the expression of the HMGB1, MHC-I and downstream cytokines IL-6 and TNF-α, and to investigate the biological role of the TLR4-HMGB1 signaling pathway in the development of the autoimmune myositis. We built mice models with experimental autoimmune myositis (EAM) and used the inverted screen experiment to measure their muscle endurance; we also examined inflammatory infiltration of muscle tissues after HE staining; and we assessed the expression of MHC-I using immunohistochemistry. In addition, peripheral blood mononuclear cells (PBMC) were extracted and flow cytometry was utilized to detect the effect of IFN-γ on the expression of MHC-I. Furthermore, PBMCs were treated with IFN-γ, anti-TLR4, anti-HMGB1 and anti-MHC-I. Real-time PCR and western blotting were employed to examine the expressions of TLR4, HMGB1 and MHC-I in different groups. The ELISA method was also utilized to detect the expression of the downstream cytokines TNF-α and IL-6. The expressions of TLR4, HMGB1 and MHC-I in muscle tissues from mice with EAM were significantly higher than those in the control group (all Pmyositis inflammation by regulating the expression of MHC-I and other pro-inflammatory cytokines. Copyright © 2016 Elsevier B.V. All rights reserved.

Current status of total body irradiation in conditioning regimen for childhood acute lymphoblastic leukemia. Survey in the Japan Association of Childhood Leukemia Study (JACLS) Group

International Nuclear Information System (INIS)

Suzuki, Nobuhiro; Hara, Jun-ichi; Chayama, Kohsuke; Akiyama, Yuichi; Nakahata, Tatsutoshi

2005-01-01

We surveyed methods of total body irradiation (TB I) in conditioning regimens of stem cell transplantation (SCT) for children with acute lymphoblastic leukemia (ALL) at participating institutions of the Japan Association of Childhood Leukemia Study (JACLS) ALL-97 protocol. We obtained information about TBI from 25 institutions. Total dose of 12 Gy fractionated by four to six in two to three days for TBI was conducted in 22 of 25 institutions. High-risk patients, such as patients with Philadelphia positive ALL, received over 12 Gy in five institutions. Beam direction and patient's positioning were horizontal and lateral respectively in 15 institutions. Shielding of lung and/or eyes and boost irradiation to central nervous system and/or testis were done in 24 and 11 institutions respectively, but in various ways. We have to keep in mind that a great variety of TBI have been undergone in each institution when we intend to interpret multi-institutional trials of treatment including SCT for patients with ALL. (author)

The metabolic syndrome in survivors of childhood acute lymphoblastic leukemia in Isfahan, Iran

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Nahid Reisi

2009-04-01

Full Text Available

• BACKGROUND: To determine the prevalence of metabolic syndrome in survivors of childhood leukemia in Isfahan, Iran.
• METHODS: During a 4-year period (2003 to 2007, 55 children (33 male and 22 female diagnosed with ALL at Unit of Hematology/ Oncology, Department of Pediatrics, Isfahan University of Medical Science, were enrolled in this crosssectional study. Metabolic syndrome was defined using the modified version of Adult Treatment Panel (ATP III criteria. Insulin resistance was defined based on the homeostasis model assessment index (HOMA-IR.
• RESULTS: The mean age of participates was 10.4 years (range 6-19 years and the mean interval since completion of chemotherapy was 35 months. Twenty percent (11/55 of survivors (10 male, 1 female met criteria for diagnosis of metabolic syndrome. Obesity was observed in one forth of patients and nearly 3/4 of obese patients had metabolic syndrome. High serum insulin levels were found in 16% of participants and in 63% of obese survivors. The mean insulin levels in survivors with metabolic syndrome was three-times more than those without (28.3 mu/l vs. 9.57 mu/l, p = 0.004. Insulin resistance was detected in 72.7% of survivors with metabolic syndrome and it was  ositively correlated with serum triglycerides (0.543, p < 0.001, systolic and diastolic BP (0.348, p = 0.01 and 0.368, p = 006 respectively, insulin levels (0.914, p < 0.001 and blood sugar (0.398, p = 003.
• CONCLUSIONS: The prevalence of metabolic syndrome in survivors of childhood leukemia in Iran is higher than developed countries. Nearly all of the obese patients had metabolic syndrome. Weight control and regular physical exercise are recommended to the survivors.
• KEYWORDS: Acute lymphoblastic leukemia, metabolic syndrome, obesity, children.

Diagnosis and treatment of Sarcocystis neurona-induced myositis in a free-ranging California sea lion.

Science.gov (United States)

Carlson-Bremer, Daphne P; Gulland, Frances M D; Johnson, Christine K; Colegrove, Kathleen M; Van Bonn, William G

2012-02-01

An underweight, lethargic adult female California sea lion (Zalophus californianus) became stranded along the California shore and was captured and transported to a rehabilitation hospital for assessment and care. Initial physical assessment revealed the sea lion was lethargic and in poor body condition. Active myositis was diagnosed on the basis of concurrent elevations in activities of alanine aminotransferase and creatine kinase detected during serum biochemical analysis. Infection with Sarcocystis neurona was diagnosed after serologic titers increased 4-fold over a 3-week period. Diagnosis was confirmed on the basis of histopathologic findings, positive results on immunohistochemical staining, and results of quantitative PCR assay on biopsy specimens obtained from the diaphragm and muscles of the dorsal cervical region. Anticoccidial treatment was instituted with ponazuril (10 mg/kg [4.5 mg/lb], PO, q 24 h) and continued for 28 days. Prednisone (0.2 mg/kg [0.09 mg/lb], PO, q 12 h) was administered for 2 days and then every 24 hours for 5 days to treat associated inflammation. At the end of treatment, the sea lion was clinically normal, alanine aminotransferase and creatine kinase values were within reference limits, and antibody titers against S neurona had decreased 6-fold. The sea lion was released approximately 3 months after becoming stranded. S neurona-induced myositis was diagnosed in a free-ranging California sea lion. On the basis of the successful treatment and release of this sea lion, anticoccidial treatment should be considered for marine mammals in which protozoal disease is diagnosed.

Asparaginase-associated pancreatitis in childhood acute lymphoblastic leukaemia: an observational Ponte di Legno Toxicity Working Group study.

Science.gov (United States)

Wolthers, Benjamin O; Frandsen, Thomas L; Baruchel, André; Attarbaschi, Andishe; Barzilai, Shlomit; Colombini, Antonella; Escherich, Gabriele; Grell, Kathrine; Inaba, Hiroto; Kovacs, Gábor; Liang, Der-Cherng; Mateos, Marion; Mondelaers, Veerle; Möricke, Anja; Ociepa, Tomasz; Samarasinghe, Sujith; Silverman, Lewis B; van der Sluis, Inge M; Stanulla, Martin; Vrooman, Lynda M; Yano, Michihiro; Zapotocka, Ester; Schmiegelow, Kjeld

2017-09-01

Survival for childhood acute lymphoblastic leukaemia surpasses 90% with contemporary therapy; however, patients remain burdened by the severe toxic effects of treatment, including asparaginase-associated pancreatitis. To investigate the risk of complications and risk of re-exposing patients with asparaginase-associated pancreatitis to asparaginase, 18 acute lymphoblastic leukaemia trial groups merged data for this observational study. Patient files from 26 trials run by 18 trial groups were reviewed on children (aged 1·0-17·9 years) diagnosed with t(9;22)-negative acute lymphoblastic leukaemia between June 1, 1996, and Jan 1, 2016, who within 50 days of asparaginase exposure developed asparaginase-associated pancreatitis. Asparaginase-associated pancreatitis was defined by at least two criteria: abdominal pain, pancreatic enzymes at least three times the upper limit of normal (ULN), and imaging compatible with pancreatitis. Patients without sufficient data for diagnostic criteria were excluded. Primary outcomes were defined as acute and persisting complications of asparaginase-associated pancreatitis and risk of re-exposing patients who suffered an episode of asparaginase-associated pancreatitis to asparaginase. Data were collected from Feb 2, 2015, to June 30, 2016, and analysed and stored in a common database at Rigshospitalet, Copenhagen, Denmark. Of 465 patients with asparaginase-associated pancreatitis, 33 (8%) of 424 with available data needed mechanical ventilation, 109 (26%) of 422 developed pseudocysts, acute insulin therapy was needed in 81 (21%) of 393, and seven (2%) of 458 patients died. Risk of assisted mechanical ventilation, need for insulin, pseudocysts, or death was associated with older age (median age for patients with complications 10·5 years [IQR 6·4-13·8] vs without complications 6·1 years [IQR 3·6-12·2], ppancreatitis, 31 (11%) of 275 patients still needed insulin or had recurrent abdominal pain or both. Both the risk of persisting

Proliferative myositis of the latissimus dorsi presenting in a 20-year-old male athlete

LENUS (Irish Health Repository)

Mc Hugh, N

2017-08-01

We describe the case of a 20-year-old rower presenting with an uncommon condition of Proliferative Myositis (PM) affecting the Latissimus Dorsi (LD). PM is a rare, benign tumour infrequently developing in the upper back. Its rapid growth and firm consistency may mistake it for sarcoma at presentation. Therefore, careful multidisciplinary work-up is crucial, and should involve appropriate radiological and histopathological investigations. Here, we propose the aetiology of LD PM to be persistent myotrauma induced by repetitive rowing motions. Symptoms and rate of progression ultimately determine the management which includes surveillance and\\/or conservative resection. There have been no documented cases of recurrence or malignant transformation.

Application of nuclear medicine to heart diseases in childhood

Energy Technology Data Exchange (ETDEWEB)

Tsuji, A. (Yamanashi Medical Coll., Tamaho, Kofu (Japan)); Osano, M.

1981-06-01

Various procedures to obtain radioisotopic images of the cardiovascular system were described, and representative cases in childhood were presented. Apparatuses, radiopharmaceuticals such as sup(99m)Tc pertechnatate, /sup 201/Thallium, and /sup 133/Xenon gas, and their dosage in childhood were briefly reviewed. Several cases of persistent fetal circulation, common A-V valve, tetralogy of Fallot, pulmonary atresia, etc. were presented with figures with special emphasis on acute febrile mucocutaneous lymph node syndrome and /sup 201/Thallium myocardial imaging.

Long-Term Effect of Cranial Radiotherapy on Pituitary-Hypothalamus Area in Childhood Acute Lymphoblastic Leukemia Survivors.

Science.gov (United States)

Follin, Cecilia; Erfurth, Eva Marie

2016-09-01

Survival rates of childhood cancer have improved markedly, and today more than 80 % of those diagnosed with a pediatric malignancy will become 5-year survivors. Nevertheless, survivors exposed to cranial radiotherapy (CRT) are at particularly high risk for long-term morbidity, such as endocrine insufficiencies, metabolic complications, and cardiovascular morbidity. Deficiencies of one or more anterior pituitary hormones have been described following therapeutic CRT for primary brain tumors, nasopharyngeal tumors, and following prophylactic CRT for childhood acute lymphoblastic leukemia (ALL). Studies have consistently shown a strong correlation between the total radiation dose and the development of pituitary deficits. Further, age at treatment and also time since treatment has strong implications on pituitary hormone deficiencies. There is evidence that the hypothalamus is more radiosensitive than the pituitary and is damaged by lower doses of CRT. With doses of CRT hypothalamus and this usually causes isolated GH deficiency (GHD). Higher doses (>50 Gy) may produce direct anterior pituitary damage, which contributes to multiple pituitary deficiencies. The large group of ALL survivors treated with CRT in the 70-80-ties has now reached adulthood, and these survivors were treated mainly with 24 Gy, and the vast majority of these patients suffer from GHD. Further, after long-term follow-up, insufficiencies in prolactin (PRL) and thyroid stimulating hormone (TSH) have also been reported and a proportion of these patients were also adrenocoticotrophic hormone (ACTH) deficient. CRT to the hypothalamus causes neuroendocrine dysfunction, which means that the choice of GH test is crucial for the diagnosis of GHD.

Hepatic sinusoidal obstruction syndrome during maintenance therapy of childhood acute lymphoblastic leukemia is associated with continuous asparaginase therapy and mercaptopurine metabolites

DEFF Research Database (Denmark)

Toksvang, Linea Natalie; De Pietri, Silvia; Nielsen, Stine N.

2017-01-01

BACKGROUND: Hepatic sinusoidal obstruction syndrome (SOS) during treatment of childhood acute lymphoblastic leukemia (ALL) has mainly been associated with 6-thioguanine. The occurrence of several SOS cases after the introduction of extended pegylated asparaginase (PEG-asparaginase) therapy...... in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL2008 protocol led us to hypothesize that PEG-asparaginase, combined with other drugs, may trigger SOS during 6-thioguanine-free maintenance therapy. PROCEDURE: In children with ALL treated in Denmark according to the NOPHO ALL2008 protocol...... children receiving PEG-asparaginase biweekly, 29 developed SOS (≥2 criteria: hyperbilirubinemia, hepatomegaly, ascites, weight gain ≥2.5%, unexplained thrombocytopenia

High-resolution Antibody Array Analysis of Childhood Acute Leukemia Cells

Czech Academy of Sciences Publication Activity Database

Kanderová, V.; Kuzilkova, D.; Stuchlý, J.; Vašková, M.; Brdička, Tomáš; Fišer, K.; Hrušák, O.; Lund-Johansen, F.; Kalina, T.

2016-01-01

Roč. 15, č. 4 (2016), s. 1246-1261 ISSN 1535-9476 R&D Projects: GA MŠk 2B06064 Institutional support: RVO:68378050 Keywords : acute lymphoblastic-leukemia * acute promyelocytic leukemia * cytometric immunobead assay * caspase-dependent cleavage * acute myeloid-leukemia * gene-expression * fusion proteins * flow-cytometry * pcr data * b-cells Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 6.540, year: 2016

Acute Renal Failure In Infancy and Childhood

African Journals Online (AJOL)

1974-10-19

Oct 19, 1974 ... Single kidney + calculus in ureter ... Calculus in one ureter + acute glomeru- lonephritis ... Ureteric ... A careful history and physical examination will often provide clues to ..... variation in response and tolerance occurs; frequent.

Mean Platelet volume in diagnosis of acute appendicitis in children ...

African Journals Online (AJOL)

Background: The clinical diagnosis of acute appendicitis (AA) in children is still problematic in status. Objectives: To investigate the diagnostic value of mean platelet volume (MPV) in acute AA at childhood. Methods: One hundred patients diagnosed as AA patients and 100 healthy individuals. Laboratory tests were studied ...

Pharmacogenetics in Acute Lymphoblastic Leukemia

Science.gov (United States)

Cheok, Meyling H.; Pottier, Nicolas; Kager, Leo

2009-01-01

Progress in the treatment of acute leukemia in children has been remarkable, from a disease being lethal four decades ago to current cure rates exceeding 80%. This exemplary progress is largely due to the optimization of existing treatment modalities rather than the discovery of new antileukemic agents. However, despite these high cure rates, the annual number of children whose leukemia relapses after their initial therapy remains greater than that of new cases of most types of childhood cancers. The aim of pharmacogenetics is to develop strategies to personalize treatment and tailor therapy to individual patients, with the goal of optimizing efficacy and safety through better understanding of human genome variability and its influence on drug response. In this review, we summarize recent pharmacogenomic studies related to the treatment of pediatric acute lymphoblastic leukemia. These studies illustrate the promise of pharmacogenomics to further advance the treatment of human cancers, with childhood leukemia serving as a paradigm. PMID:19100367

Danish Childhood Cancer Registry

DEFF Research Database (Denmark)

Schrøder, Henrik; Rechnitzer, Catherine; Wehner, Peder Skov

2016-01-01

AIM OF DATABASE: The overall aim is to monitor the quality of childhood cancer care in Denmark; to register late effects of treatment; to analyze complications of permanent central venous catheters (CVCs); to study blood stream infections in children with cancer; and to study acute toxicity of high......-dose methotrexate infusions in children with leukemia. STUDY POPULATION: All children below 15 years of age at diagnosis living in Denmark diagnosed after January 1, 1985 according to the International Classification of Diseases 10, including diagnoses DC00-DD48. MAIN VARIABLES: Cancer type, extent of disease......, and outcome of antimicrobial chemotherapy. DESCRIPTIVE DATA: Since 1985, 4,944 children below 15 years of age have been registered in the database. There has been no significant change in the incidence of childhood cancer in Denmark since 1985. The 5-year survival has increased significantly since 1985...

Acute Transverse Myelitis in Children, Literature Review.

Science.gov (United States)

Tavasoli, Azita; Tabrizi, Aidin

2018-01-01

Acute transverse myelitis (ATM) is a rare inflammatory demyelinating disorder characterized by relatively acute onset of motor, sensory, and autonomic dysfunction. Children comprise 20% of total cases of ATM. In this review, we described the current literature on childhood ATM, focusing on the epidemiology, pathogenesis, clinical presentation, approach to diagnosis, differential diagnosis, treatment and outcome in the pediatric population. We searched the related articles in electronic databases such as Scopus, EMBASE, Google Scholar, and PubMed. All study designs were included and the essential key words for searching were myelitis, acute transverse myelitis, childhood transverse myelitis, and acquired demyelinating syndromes. The related data focusing on the epidemiology, pathogenesis, clinical presentation, diagnostic approach and differential diagnosis, treatment and outcome of pediatric ATM were gathered and described. ATM is a heterogeneous disorder in children with a broad spectrum of clinical presentation, etiology, and outcome. It may be the first presentation of relapsing acquired demyelinating syndromes and also must be distinguished from compressive and noninflamatory myelopathies. Correct diagnosis is crucial for treatment and prognosis.

[Epidemiological survey of childhood asthma in Hefei City, China].

Science.gov (United States)

Xiong, Mei; Ni, Chen; Pan, Jia-Hua; Wang, Qiang; Zheng, Li-Lin

2013-02-01

To investigate the cumulative prevalence rate, distribution characteristics, epidemic seasons, predisposing factors and current treatment situation of childhood asthma in Hefei City, China. In the investigation, stratified cluster random sampling as well as centralized access and separate home visits were applied, and primary screening forms were filled out. Further confirmation was sought in the primary positive cases, according to the diagnostic criteria for asthma. Statistical analysis was performed to determine the cumulative prevalence rate, current treatment situation and predisposing factors for childhood asthma as well as the distribution characteristics of asthma in children of different ages and sexes. The cumulative prevalence rate of childhood asthma was 5.92%, and there was no significant difference between males and females (6.33% vs 5.42%; P>0.05). The cumulative prevalence rate was highest (8.25%) in children aged 3-6 years. Of the children with acute asthma attack, 42.0% suffered attacks during periods of seasonal transition, and 34.4% suffered attacks at midnight. Among the 552 children with a confirmed diagnosis of asthma, 533 (96.6%) developed asthma due to respiratory tract infection and 312 (56.5%) due to weather change. Most asthmatic children (513/552, 92.9%) received treatment with antibiotics, and 492 asthmatic children (89.1%) were treated with systemic hormones. The cumulative prevalence rate of childhood asthma is 5.92% in Hefei, and the peak age of onset is 3-6 years. Acute asthma attack occurs mostly during periods of seasonal transition and at midnight. Respiratory tract infection and weather change are the main predisposing factors for childhood asthma. Antibiotics and systemic hormones are still widely used in the treatment of asthma.

Acute acquired comitant esotropia of childhood

DEFF Research Database (Denmark)

Hesgaard, Helena; Vinding, Troels

2015-01-01

acute onset of comitant esotropia, available data on ophthalmologic, orthoptic and neurologic examinations. Children with neurological signs, AACE recurrence or hyperopia followed. RESULTS...... no obvious neurological signs at onset. Four significant risk factors for intracranial disease were identified as follows: larger esodeviation at distance, recurrence of AACE, neuro signs (papilledema) and older age at onset (>6 years). CONCLUSION: In a large case series of children with AACE and by review...

Cognitive, behaviour, and academic functioning in adolescent and young adult survivors of childhood acute lymphoblastic leukaemia: a report from the Childhood Cancer Survivor Study.

Science.gov (United States)

Jacola, Lisa M; Edelstein, Kim; Liu, Wei; Pui, Ching-Hon; Hayashi, Robert; Kadan-Lottick, Nina S; Srivastava, Deokumar; Henderson, Tara; Leisenring, Wendy; Robison, Leslie L; Armstrong, Gregory T; Krull, Kevin R

2016-10-01

Survivors of childhood acute lymphoblastic leukaemia (ALL) are at risk for neurocognitive deficits that affect development in adolescence and young adulthood, and influence educational attainment and future independence. We examined a large and diverse cohort of survivors to identify risk predictors and modifiers of these outcomes. In this cohort study, cognitive and behaviour symptoms were assessed via a standardised parent questionnaire for 1560 adolescent survivors of ALL diagnosed between 1970 and 1999. Clinically significant symptoms (≥90th percentile) and learning problems were compared between survivors and a sibling cohort. Multivariable regression models were used to examine associations with demographic and treatment characteristics. Models were adjusted for inverse probability of sampling weights to reflect undersampling of ALL survivors in the expansion cohort. In a subset of survivors with longitudinal data (n=925), we examined associations between adolescent symptoms or problems and adult educational attainment. Compared with siblings, survivors treated with chemotherapy only were more likely to demonstrate headstrong behaviour (155 [19%] of 752 survivors vs 88 [14%] of 610 siblings, p=0·010), inattention-hyperactivity (15 [19%] vs 86 [14%], p4·3 g/m 2 ) conferred increased risk of inattention-hyperactivity (relative risk [RR] 1·53, 95% CI 1·13-2·08). Adolescent survivors with cognitive or behaviour problems and those with learning problems were less likely to graduate from college as young adults than adolescent survivors without cognitive or behaviour problems. Although modern therapy for childhood ALL has eliminated the use of cranial radiation therapy, adolescent survivors treated with chemotherapy only remain at increased risk for cognitive, behaviour, and academic problems that adversely affect adult education outcomes. National Cancer Institute, American Lebanese-Syrian Associated Charities. Copyright © 2016 Elsevier Ltd. All rights

Eosinophilic myositis resulted from Sarcocystis infection in prime marbled beef of Japanese black cattle

Directory of Open Access Journals (Sweden)

Tohru Kimura

Full Text Available Partial changes of color (greenish to brownish were found in prime marbled beef of Japanese black cattle. The disseminated lesions of the skeletal muscles were histopathologically examined in relation to Sarcocystis infection. The lesions in the muscles showed granulomas with inflammatory cell infiltration. The sarcocysts had a distinct wall, which was radically striated by palisading villar protrusions. The sarcocyst wall was surrounded by degenerative eosinophils and necrotic muscle fibers. In conclusion, eosinophilic myositis in prime marbled beef of Japanese black cattle resulted from Sarcocystis spp. infection. The muscular lesions were characterized by the presence of granulomas and capsulated sarcocysts surrounded by numerous eosinophils. [Vet. World 2011; 4(11.000: 500-502

Non-infectious chemotherapy-associated acute toxicities during childhood acute lymphoblastic leukemia therapy

DEFF Research Database (Denmark)

Schmiegelow, Kjeld; Müller, Klaus Gottlob; Mogensen, Signe Sloth

2017-01-01

obstruction syndrome, endocrinopathies (especially steroid-induced adrenal insufficiency and hyperglycemia), high-dose methotrexate-induced nephrotoxicity, asparaginase-associated hypersensitivity, pancreatitis, and hyperlipidemia. Few of the non-infectious acute toxicities are associated with clinically...

QUANTITATIVE MUSCLE ULTRASONOGRAPHY IN THE FOLLOW-UP OF JUVENILE DERMATOMYOSITIS

NARCIS (Netherlands)

Habers, G. Esther A.; van Brussel, Marco; Bhansing, Kavish J.; Hoppenreijs, Esther P.; Janssen, Anjo J. W. M.; van Royen-Kerkhof, Annet; Pillen, Sigrid

2015-01-01

Introduction: We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM). Methods: Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT)

Tumors of the soft tissues of the knee in childhood; Osteo-articulaire masse des tissus mous de genou. Imagerie des masses des tissus mous du genou chez l'enfant

Energy Technology Data Exchange (ETDEWEB)

Ludig, T.; Mainard, L.; Galloy, M.A.; Arnould, V.; Gerber, R.; Hoeffel, J.C. [Centre Hospitalier Universitaire Nancy-Brabois, 54 - Vandoeuvre-les-Nancy (France)

1998-04-01

Soft tissue tumors of the knee are rare in childhood. Modern imaging modalities are very useful to evaluate these tumors. Their diagnosis has to be considered in view of the clinical content, their precise location and imaging features. Succinct review of the normal anatomy of the knee is made. We offer a diagnostic approach to these tumors in children including conventional radiology, ultrasound and MRI. We discuss the role of the different imaging techniques available for the diagnosis of these lesions, including pseudo-tumors such as popliteal cysts, hematoma, ossifying myositis, extra-articular benign and malignant tumors, as well as intra-articular lesions, synovial lipoma, osteochondromatosis and pigmented villo-nodular synovitis. MRI is particularly useful to evaluate the extent of the malignant tumors. (authors)

Upregulation of immunoproteasome subunits in myositis indicates active inflammation with involvement of antigen presenting cells, CD8 T-cells and IFNΓ.

Directory of Open Access Journals (Sweden)

Khetam Ghannam

Full Text Available OBJECTIVE: In idiopathic inflammatory myopathies (IIM infiltration of immune cells into muscle and upregulation of MHC-I expression implies increased antigen presentation and involvement of the proteasome system. To decipher the role of immunoproteasomes in myositis, we investigated individual cell types and muscle tissues and focused on possible immune triggers. METHODS: Expression of constitutive (PSMB5, -6, -7 and corresponding immunoproteasomal subunits (PSMB8, -9, -10 was analyzed by real-time RT-PCR in muscle biopsies and sorted peripheral blood cells of patients with IIM, non-inflammatory myopathies (NIM and healthy donors (HD. Protein analysis in muscle biopsies was performed by western blot. Affymetrix HG-U133 platform derived transcriptome data from biopsies of different muscle diseases and from immune cell types as well as monocyte stimulation experiments were used for validation, coregulation and coexpression analyses. RESULTS: Real-time RT-PCR revealed significantly increased expression of immunoproteasomal subunits (PSMB8/-9/-10 in DC, monocytes and CD8+ T-cells in IIM. In muscle biopsies, the immunosubunits were elevated in IIM compared to NIM and exceeded levels of matched blood samples. Proteins of PSMB8 and -9 were found only in IIM but not NIM muscle biopsies. Reanalysis of 78 myositis and 20 healthy muscle transcriptomes confirmed these results and revealed involvement of the antigen processing and presentation pathway. Comparison with reference profiles of sorted immune cells and healthy muscle confirmed upregulation of PSMB8 and -9 in myositis biopsies beyond infiltration related changes. This upregulation correlated highest with STAT1, IRF1 and IFNγ expression. Elevation of T-cell specific transcripts in active IIM muscles was accompanied by increased expression of DC and monocyte marker genes and thus reflects the cell type specific involvement observed in peripheral blood. CONCLUSIONS: Immunoproteasomes seem to indicate

Upregulation of Immunoproteasome Subunits in Myositis Indicates Active Inflammation with Involvement of Antigen Presenting Cells, CD8 T-Cells and IFNγ

Science.gov (United States)

Ghannam, Khetam; Martinez-Gamboa, Lorena; Spengler, Lydia; Krause, Sabine; Smiljanovic, Biljana; Bonin, Marc; Bhattarai, Salyan; Grützkau, Andreas; Burmester, Gerd-R.

2014-01-01

Objective In idiopathic inflammatory myopathies (IIM) infiltration of immune cells into muscle and upregulation of MHC-I expression implies increased antigen presentation and involvement of the proteasome system. To decipher the role of immunoproteasomes in myositis, we investigated individual cell types and muscle tissues and focused on possible immune triggers. Methods Expression of constitutive (PSMB5, -6, -7) and corresponding immunoproteasomal subunits (PSMB8, -9, -10) was analyzed by real-time RT-PCR in muscle biopsies and sorted peripheral blood cells of patients with IIM, non-inflammatory myopathies (NIM) and healthy donors (HD). Protein analysis in muscle biopsies was performed by western blot. Affymetrix HG-U133 platform derived transcriptome data from biopsies of different muscle diseases and from immune cell types as well as monocyte stimulation experiments were used for validation, coregulation and coexpression analyses. Results Real-time RT-PCR revealed significantly increased expression of immunoproteasomal subunits (PSMB8/-9/-10) in DC, monocytes and CD8+ T-cells in IIM. In muscle biopsies, the immunosubunits were elevated in IIM compared to NIM and exceeded levels of matched blood samples. Proteins of PSMB8 and -9 were found only in IIM but not NIM muscle biopsies. Reanalysis of 78 myositis and 20 healthy muscle transcriptomes confirmed these results and revealed involvement of the antigen processing and presentation pathway. Comparison with reference profiles of sorted immune cells and healthy muscle confirmed upregulation of PSMB8 and -9 in myositis biopsies beyond infiltration related changes. This upregulation correlated highest with STAT1, IRF1 and IFNγ expression. Elevation of T-cell specific transcripts in active IIM muscles was accompanied by increased expression of DC and monocyte marker genes and thus reflects the cell type specific involvement observed in peripheral blood. Conclusions Immunoproteasomes seem to indicate IIM activity and

High resolution melting curve analysis, a rapid and affordable method for mutation analysis in childhood acute myeloid leukemia

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Yin eLiu

2014-09-01

Full Text Available Background: Molecular genetic alterations with prognostic significance have been described in childhood acute myeloid leukemia (AML. The aim of this study was to establish cost-effective techniques to detect mutations of FMS-like tyrosine kinase 3 (FLT3, Nucleophosmin 1 (NPM1, and a partial tandem duplication within the mixed lineage leukemia (MLL-PTD genes in childhood AML. Procedure: Ninety-nine children with newly diagnosed AML were included in this study. We developed a fluoresent dye SYTO-82 based high resolution melting curve (HRM anaylsis to detect FLT3 internal tandem duplication (FLT3-ITD, FLT3 tyrosine kinase domain (FLT3-TKD and NPM1 mutations. MLL-PTD was screened by real-time quantitative PCR. Results: The HRM methodology correlated well with gold standard Sanger sequencing with less cost. Among the 99 patients studied, the FLT3-ITD mutation was associated with significantly worse event free survival (EFS. Patients with the NPM1 mutation had significantly better EFS and overall survival. However, HRM was not sensitive enough for minimal residual disease monitoring. Conclusions: HRM was a rapid and efficient method for screening of FLT3 and NPM1 gene mutations. It was both affordable and accurate, especially in resource underprivileged regions. Our results indicated that HRM could be a useful clinical tool for rapid and cost effective screening of the FLT3 and NPM1 mutations in AML patients.

Mutations in LPIN1 cause recurrent acute myoglobinuria in childhood

NARCIS (Netherlands)

Zeharia, Avraham; Shaag, Avraham; Houtkooper, Riekelt H.; Hindi, Tareq; de Lonlay, Pascale; Erez, Gilli; Hubert, Laurence; Saada, Ann; de Keyzer, Yves; Eshel, Gideon; Vaz, Frédéric M.; Pines, Ophry; Elpeleg, Orly

2008-01-01

Recurrent episodes of life-threatening myoglobinuria in childhood are caused by inborn errors of glycogenolysis, mitochondrial fatty acid beta-oxidation, and oxidative phosphorylation. Nonetheless, approximately half of the patients do not suffer from a defect in any of these pathways. Using

Acute meningococcal disease in children and adolescents

DEFF Research Database (Denmark)

Nygaard, Ulrikka; Vissing, Nadja Hawwa; Steensen, Morten

2017-01-01

Meningococcal disease is a rapidly progressing infection, which continues to cause deaths among children and adolescents. In this review, clinical signs and initial treatment of acute childhood meningococcal disease is described. Operational flow charts have been developed for assessment of non...

Management of necrotizing myositis in a field hospital: a case report

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Bharathi Ramanathan

2009-04-01

Full Text Available Abstract Necrotizing myositis is a rare and fatal disease of skeletal muscles caused by group A beta hemolytic streptococci (GABHS. Its early detection by advanced imaging forms the basis of current management strategy. Paucity of advanced imaging in field/rural hospitals necessitates adoption of management strategy excluding imaging as its basis. Such a protocol, based on our experience and literature, constitutes: i. Prompt recognition of the clinical triad: disproportionate pain; precipitous course; and early loss of power- in a swollen limb with/without preceding trauma. ii. Support of clinical suspicion by 2 ubiquitous laboratory tests: gram staining- of exudates from bullae/muscles to indicate GABHS infection; and CPK estimation- to indicate myonecrosis. iii. Replacement of empirical antibiotics with high intravenous doses of sodium penicillin and clindamycin iv. Exploratory fasciotomy: to confirm myonecrosis without suppuration- its hallmark v. Emergent radical debridement vi. Primary closure with viable flaps – unconventional, if need be.