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Sample records for calcinosis

  1. Recurrent hyperphosphatemic tumoural calcinosis

    Science.gov (United States)

    Amit, Sonal; Agarwal, Asha; Nigam, Anand; Rao, Yashwant Kumar

    2012-01-01

    Tumoural calcinosis (TC) is a benign gradually developing disorder that can occur in a variety of clinical settings, characterised by subcutaneous deposition of calcium phosphate with or without giant cell reaction. We describe a case of 11-year-old girl presenting with recurrent hard swellings in the vicinity of shoulder and hip joints associated with elevated serum phosphate and normal serum calcium levels. TC has been mainly reported from Africa, with very few cases reported from India. After the diagnosis of hyperphosphatemic TC was established, the patient was treated with oral sevelamer and is under constant follow-up to detect recurrence, if any. The present case highlights the fact that although an uncommon lesion, TC must be considered in the differential diagnosis of subcutaneous hard lump in the vicinity of a joint. PMID:23010461

  2. Calcinosis tumoral de rodilla Tumoral calcinosis of the knee

    OpenAIRE

    Juan Pablo Restrepo; María del Pilar Molina

    2010-01-01

    La calcinosis tumoral o síndrome de Teutschlander es una condición benigna y rara caracterizada por la presencia de masas calcificadas de ubicación periarticular en la cadera, hombro, codo y menor frecuencia en pies, tobillos y rodillas. Se cree que hay una alteración genética en el GALNT3 y FGF23 que regulan la absorción renal de fosfato. Usualmente esta lesión es asintomática y rara vez puede dar síntomas producto de compresión de estructuras vecinas. La imaginología permite habitualmente r...

  3. Calcinosis tumoral de rodilla Tumoral calcinosis of the knee

    Directory of Open Access Journals (Sweden)

    Juan Pablo Restrepo

    2010-12-01

    Full Text Available La calcinosis tumoral o síndrome de Teutschlander es una condición benigna y rara caracterizada por la presencia de masas calcificadas de ubicación periarticular en la cadera, hombro, codo y menor frecuencia en pies, tobillos y rodillas. Se cree que hay una alteración genética en el GALNT3 y FGF23 que regulan la absorción renal de fosfato. Usualmente esta lesión es asintomática y rara vez puede dar síntomas producto de compresión de estructuras vecinas. La imaginología permite habitualmente realizar el diagnóstico. Hasta el momento no hay tratamiento médico exitoso y se sigue recomendando la escisión quirúrgica aunque hay recurrencias (Acta Med Colomb 2010; 35: 185-186.Tumoral calcinosis or Teutschlander syndrome is a rare and benign condition characterized by the presence of calcified masses around the hip, elbow, and less frequently feet, ankles and knees. It is believed that there is a genetic alteration in GALNT3 and FGF23 which regulate the renal absorption of phosphate. Usually it is an asymptomatic lesion and occasionally it produces symptoms of compression to peripheral tissues. Imaging permits the diagnosis. There is no successful medical treatment and surgical excision is recommended although recurrences are frequent (Acta Med Colomb 2010; 35: 185-186.

  4. A rare disorder: tumoral calcinosis and cirrhosis

    OpenAIRE

    Allameh, Seyyed Farshad; Anari, Akram Ghadiri; Gharabaghi, Mehrnaz Asadi; Nakhjavani, Manouchehr

    2011-01-01

    Tumoral calcinosis is a rare disease characterised by deposition of calcified mass near the joints. The pathogenesis of this disease is not exactly defined. A disorder of calcium and inorganic phosphate metabolism may play a role. Here, we report a case of 19-year-old girl who had both cryptogenic cirrhosis and idiopathic tumoral calcinosis. To our knowledge, there is few report of such concurrence.

  5. MDCT imaging of calcinosis in systemic sclerosis

    International Nuclear Information System (INIS)

    Calcinosis is a typical feature of systemic sclerosis (SSc) and can be found in many different tissues including the superficial soft tissues, periarticular structures, muscles, and tendons. It can also provoke erosive changes on bones. Investigation is conducted most often with plain radiographs. However, when a more detailed assessment is necessary, multidetector computed tomography (MDCT) is helpful owing to its multiplanar reformat (MPR) ability. The purpose of this review is to provide an overview of the various appearances of calcinosis in SSc patients as visualized at MDCT

  6. Tumoral calcinosis with vitamin D deficiency

    Directory of Open Access Journals (Sweden)

    Kannan Subramanian

    2008-01-01

    Full Text Available A 50-year-old woman presented with recurrent calcified mass in the left gluteal region. The clinical, radiological, and biochemical profile confirmed the diagnosis of tumoral calcinosis. She also had associated vitamin D deficiency. The patient underwent surgical removal of the mass to relieve the sciatic nerve compression and was managed with acetazolamide, calcium carbonate, and aluminium hydroxide gel with which she showed significant improve-ment. The management implications and effect of vitamin D deficiency on phosphate metabolism in the setting of tumoral calcinosis is discussed.

  7. Severe calcinosis cutis in an infant

    International Nuclear Information System (INIS)

    We report on an infant with severe asphyxia and persistent pulmonary hypertension as a newborn. The baby received prolonged intravenous calcium gluconate therapy for hypocalcemia. At 5 weeks of age, multiple firm, indurated areas (armor-like lesions) were palpable in the subcutaneous tissues of the trunk, arms, legs, and face, particularly in skin folds. Roentgenographic study showed generalized soft-tissue calcifications throughout the body, extremities, and face. Calcinosis cutis occurs through a variety of pathogenetic mechanisms. Case reports on calcinosis cutis in infants are uncommon, and the calcifications are mostly localized. In our patient, they are generalized. (orig.)

  8. Severe calcinosis cutis in an infant

    Energy Technology Data Exchange (ETDEWEB)

    Puvabanditsin, Surasak; Patel, Jigneshkumar B. [University of Medicine and Dentistry of New Jersey, Department of Pediatrics, Newark, NJ (United States); University of Medicine and Dentistry of New Jersey, Department of Surgery, Newark, NJ (United States); Garrow, Eugene; Titapiwatanakun, Ruetima; Getachew, Rahel [University of Medicine and Dentistry of New Jersey, Department of Pediatrics, Newark, NJ (United States)

    2005-05-01

    We report on an infant with severe asphyxia and persistent pulmonary hypertension as a newborn. The baby received prolonged intravenous calcium gluconate therapy for hypocalcemia. At 5 weeks of age, multiple firm, indurated areas (armor-like lesions) were palpable in the subcutaneous tissues of the trunk, arms, legs, and face, particularly in skin folds. Roentgenographic study showed generalized soft-tissue calcifications throughout the body, extremities, and face. Calcinosis cutis occurs through a variety of pathogenetic mechanisms. Case reports on calcinosis cutis in infants are uncommon, and the calcifications are mostly localized. In our patient, they are generalized. (orig.)

  9. A case of adult dermatomyositis with calcinosis universalis

    International Nuclear Information System (INIS)

    Calcinosis, although frequent in juvenile dermatomyositis is a rare finding in adults. It is more common in later phases of the disease, involving sites under chronic stress and trauma. We present a 52-year-old female patient of dermatomyositis who on single-photon emission computed tomography/computed tomography hybrid images showed exclusive subcutaneous fat calcinosis, also known as calcinosis universalis - a phenomenon that is only rarely reported in adult-onset dermatomyositis

  10. Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline

    OpenAIRE

    Robertson, L.; Marshall, R.; Hickling, P.

    2003-01-01

    Methods: Patients with lcSSc who had cutaneous calcinosis causing pain or ulceration, or both, were prescribed minocycline 50 or 100 mg daily regularly in an open label manner between November 1994 and April 2000. At routine clinical follow up the appearance of the calcinosis deposits was assessed clinically and radiographically, and the patients' assessment of the degree of discomfort, size, and frequency of ulceration was recorded. Demographic data, including disease duration, clinical feat...

  11. Pulmonary dystrophic Calcinosis associated to systemic sclerosis: Report of the first case in Colombia

    International Nuclear Information System (INIS)

    The association of pulmonary calcinosis with systemic sclerosis has not been described in the medical literature. There are two type of lung calcification: dystrophic and metastatic; in the collagen vascular diseases the most frequent is the dystrophic calcinosis, seen mainly in dermatomyositis and scleroderma. We describe the first case of dystrophic calcinosis associated with systemic sclerosis

  12. Imaging of tumoral calcinosis: New observations

    International Nuclear Information System (INIS)

    Tumoral calcinosis (TC) is a hereditary metabolic bone disease characterized by hyperphosphatemia, elevated 1-25 dihydorxy vitamin D, and the presence of particular soft-tissue masses. Less well-known manifestations include a unique dental lesion, a bone marrow process, and an association with angioid streaks. Four members of two different kindreds with a total of 11 particular masses, two dental lesions, and three bone marrow processes were evaluated with radiography, bone scintigraphy, CT, and MR imaging. The radiologic manifestations of TC depend on pathologic calcification of particular, marrow, and dental tissue. The appearance of these calcifications on different radiologic modalities is characteristic, and the combination of these findings is unique for TC

  13. Calcinosis circumscripta in a captive African cheetah (Acinonyx jubatus)

    Institute of Scientific and Technical Information of China (English)

    Chisoni Mumba; David Squarre; Maxwel Mwase; John Yabe; Tomoyuki Shibahara

    2014-01-01

    This article reports a first case of calcinosis circumscripta in a captive African cheetah (Acinonyx jubatus). Histopathology demonstrated well defined multiple cystic structures containing granular, dark basophilic materials with peripheral granulomatous reaction, characterized by presence of multinucleated giant cells surrounded by a varying amounts of fibrous connective tissues. Special staining with von Kossa revealed black stained deposits confirming the presence of calcium salts.

  14. Differential response of idiopathic sporadic tumoral calcinosis to bisphosphonates

    Directory of Open Access Journals (Sweden)

    Karthik Balachandran

    2014-01-01

    Full Text Available Context: Tumoral calcinosis is a disorder of phosphate metabolism characterized by ectopic calcification around major joints. Surgery is the current treatment of choice, but a suboptimal choice in recurrent and multicentric lesions. Aims: To evaluate the efficacy of bisphosphonates for the management of tumoral calcinosis on optimized medical treatment. Settings and Design: The study was done in the endocrine department of a tertiary care hospital in South India. We prospectively studied two patients with recurrent tumoral calcinosis who had failed therapy with phosphate lowering measures. Materials and Methods: After informed consent, we treated both patients with standard age adjusted doses of bisphosphonates for 18 months. The response was assessed by X ray and whole body 99mTc-methylene diphosphonate bone scan at the beginning of therapy and at the end of 1 year. We also estimated serum phosphate levels and urinary phosphate to document serial changes. Results: Two patients (aged 19 and 5 years with recurrent idiopathic hyperphosphatemic tumoral calcinosis, following surgery were studied. Both patients had failed therapy with conventional medical management − low phosphate diet and phosphate binders. They had restriction of joint mobility. Both were given standard doses of oral alendronate and parenteral pamidronate respectively for more than a year, along with phosphate lowering measures. At the end of 1 year, one of the patients had more than 95% and 90% reduction in the size of the lesions in right and left shoulder joints respectively with total improvement in range of motion. In contrast, the other patient (5-year-old had shown no improvement, despite continuing to maintain normophosphatemia following treatment. Conclusions: Bisphosphonate therapy in tumoral calcinosis is associated with lesion resolution and may be used as a viable alternative to surgery, especially in cases with multicentric recurrence or treatment failure to other

  15. Scrotal calcinosis due to resorption of cyst walls: a case report

    OpenAIRE

    Parlakgumus Alper; Canpolat Emine T; Calıskan Kenan; Colakoglu Tamer; Yıldırım Sedat; Ezer Ali; Noyan Turgut

    2008-01-01

    Abstract Introduction Scrotal calcinosis is a rare benign entity defined as the presence of multiple calcified nodules within the scrotal skin. There are controversies about the origin of this entity. In fact, it is still debatable whether scrotal calcinosis is an idiopathic growth or dystrophic calcification of dartoic muscles. It is also unclear whether scrotal calcinosis originates from inflammation of epidermal cysts affected by mild to moderate inflammation of mononuclear cells, from for...

  16. Gluteal Calcinosis in a Patient With Undifferentiated Connective Tissue Disease: A Bulky Lesion Resected Surgically

    OpenAIRE

    Metin Işık; Güneş Güner; Yakup Yeşilkaya; Meral Çalgüneri

    2011-01-01

    Calcinosis frequently accompanies rheumatologic diseases and mostly occurs after trauma, due to structural damage, hypovascularity, and tissue hypoxia. Calcinosis may be seen in a localized area or it may be widespread, causing muscle atrophy, joint contractures, and skin ulceration. Herein, we report a patient with localized form of calcinosis that occured without history of trauma and the patient also has a diagnosis of undifferentiated connective tissue disease. Turk J Phys Med Rehab 2011;...

  17. Gluteal Calcinosis in a Patient With Undifferentiated Connective Tissue Disease: A Bulky Lesion Resected Surgically

    Directory of Open Access Journals (Sweden)

    Metin Işık

    2011-10-01

    Full Text Available Calcinosis frequently accompanies rheumatologic diseases and mostly occurs after trauma, due to structural damage, hypovascularity, and tissue hypoxia. Calcinosis may be seen in a localized area or it may be widespread, causing muscle atrophy, joint contractures, and skin ulceration. Herein, we report a patient with localized form of calcinosis that occured without history of trauma and the patient also has a diagnosis of undifferentiated connective tissue disease. Turk J Phys Med Rehab 2011;57 Suppl 2: 358-60.

  18. Tumoral calcinosis presenting as an extradural mass: MR findings and pathological correlation

    Energy Technology Data Exchange (ETDEWEB)

    Iglesias, A.; Arias, M.; Brasa, J. [Unidad de Resonancia Magnetica (MEDTEC), Hospital Xeral-Cies, 36204 Vigo (Spain); Gonzalez, A. [Servicio de Anatomia Patologica, Hospital Xeral-Cies, 36204 Vigo (Spain); Conde, C. [Servicio de Neurocirugia, Hospital Xeral-Cies, 36204 Vigo (Spain)

    2002-09-01

    Two cases of idiopathic tumoral calcinosis presenting as an extradural mass are reported. There are few reports in the literature of this pathological process presenting as extradural masses, so both cases represent very unusual locations for tumoral calcinosis. Magnetic resonance imaging features and pathological correlation of these two cases are presented. Tumoral calcinosis might be considered as a rare but possible cause of extradural mass. (orig.)

  19. Tc-MDP bone scintigraphy in a case with sporodical tumoral calcinosis

    International Nuclear Information System (INIS)

    Tumoral calcinosis is an uncommon and benign condition characterized by the presence of large calcific soft tissue deposits occurring predominantly in a periarticular location. It generally occurs as a complication of renal dialysis or trauma, and is rarely seen in familial and sporadic cases. Bone scintigraphy is a sensitive method for diagnosing tumoral calcinosis. A 28-year-old female patient with a history of operation due to tumoral calcinosis located bilateral hips, referred to our department. She had a tender palpable mass in the right knee and a fistulized incisional scar overlying the bilateral hip joints. A sporadic case of tumoral calcinosis with relapses was presented. (author)

  20. Radiological features of progressive tumoral calcinosis in chronic renal failure.

    LENUS (Irish Health Repository)

    Hodnett, P

    2012-02-03

    We present the case of a young adult patient with chronic renal failure who developed painful subcutaneous nodules after failed renal transplant and recommencing dialysis. These nodules were juxta-articular in location and initially located over both shoulders. Radiological evaluation suggested tumoral calcinosis. The patient was placed on a strict dialysis and dietary regimen but was suboptimally compliant with same. The patient developed progressive disease with an increase in size and number of juxta-articular calcified soft-tissue masses. However, 6 months following a second renal transplant clinical and radiological follow up demonstrated marked resolution both in symptomatology and radiographic findings. We present the plain radiographic, CT and MRI findings which demonstrate the typical radiological features of tumoral calcinosis. We correlate these findings with clinical course and histological findings following surgical excision of one of these masses.

  1. Calcinosis circumscripta in a captive African cheetah(Acinonyx jubatus)

    Institute of Scientific and Technical Information of China (English)

    Chisoni; Mumba; David; Squarre; Maxwel; Mwase; John; Yabe; Tomoyuki; Shibahara

    2014-01-01

    This article reports a first case of calcinosis circumscripta in a captive African cheetah(Acinonyx jubatus).Histopathology demonstrated well defined multiple cystic structures containing granular,dark basophilic materials with peripheral granulomatous reaction,characterized by presence of multinucleated giant cells surrounded by a varying amounts of fibrous connective tissues.Special staining with von Kossa revealed black stained deposits confirming the presence of calcium salts.

  2. Uremic tumoral calcinosis in the cervical spine: case report.

    Science.gov (United States)

    Fatehi, Mostafa; Ahuja, Christopher S; Wang, Shelly; Ginsberg, Howard J

    2016-07-01

    Tumoral calcinosis is an uncommon condition characterized by the calcification of periarticular soft tissue. In uremic patients the disease is secondary to metabolic disturbances in predisposed patients. The authors report the case of a 73-year-old woman who presented with a new painful cervical mass while undergoing continuous ambulatory peritoneal dialysis for long-standing end-stage renal disease (ESRD). A CT scan of the neck showed a lobulated, calcified mass in the left paraspinal soft tissue at C2-3. This mass affected the facet joint and also extended into the neural foramen but did not cause any neurological compromise. Due to the patient's significant medical comorbidities, resection was deferred and the patient was followed in the clinic. Subsequent repeat imaging has shown a significant decrease in the size of the mass. In the context of ESRD, a diagnosis of uremic tumoral calcinosis (UTC) was made. The authors conducted a search of the PubMed and EMBASE databases and identified 7 previously reported cases of UTC of the cervical spine. They present a summary of these cases and discuss the etiology, diagnosis, and management of the condition. Although the metabolic disturbances seen in patients undergoing dialysis can lead to tumoral calcinosis, most reported cases involve large joints such as the shoulder or the hip; however, the spine can also be affected and should be considered in the differential diagnosis of patients with uremia as it can mimic aggressive bone-forming neoplasms. PMID:26943247

  3. Abatacept and Sodium Thiosulfate for Treatment of Recalcitrant Juvenile Dermatomyositis Complicated by Ulceration and Calcinosis

    OpenAIRE

    Arabshahi, Bita; Silverman, Robert A.; Jones, Olcay Y.; Rider, Lisa G

    2012-01-01

    We report the successful use of abatacept and sodium thiosulfate in a patient with severe recalcitrant juvenile dermatomyositis complicated by ulcerative skin disease and progressive calcinosis. This combination therapy resulted in significant reduction in muscle and skin inflammation, decreased corticosteroid dependence, and halted the progression of calcinosis.

  4. [Improvement of tumoral calcinosis of the right hand after parathyroidectomy in a patient on chronic hemodialysis].

    Science.gov (United States)

    El Maghraoui, Jaouad; Hammou, Mohamed; Kabbali, Nadia; Arrayhani, Mohamed; Houssaini, Tariq Sqalli

    2016-01-01

    Periarticular tissue calcifications are common in patients with chronic renal failure undergoing hemodialysis. We report the case of a patient on chronic hemodialysis for 10 years with significant improvement of isolated pseudotumoral calcinosis of the right hand after parathyroidectomy The aim of this study was to show the impact of parathyroidectomy on pseudotumoral calcinosis. PMID:27583094

  5. Use of dual-energy computed tomography for the evaluation of calcinosis in patients with systemic sclerosis

    OpenAIRE

    Hsu, Vivien; Bramwit, Mark; Schlesinger, Naomi

    2015-01-01

    We examined the usefulness of dual-energy computed tomography (DECT) in the evaluation of symptomatic systemic sclerosis (SSc)-related calcinosis of the hands. We performed DECT scan of the hands in 16 patients with symptomatic SSc-calcinosis to better characterize the calcinosis, their locations within the soft tissues, and exclude monosodium urate (MSU) crystal deposition. We also compared their computed tomography (CT) images to plain radiographs of one hand. Pertinent clinical information...

  6. Peritendinous calcinosis of calcaneus tendon associated with dermatomyositis: correlation between conventional radiograph, ultrasound, magnetic resonance imaging and gross surgical pathology

    International Nuclear Information System (INIS)

    Interstitial calcinosis is an uncommon condition in which there is either localized or widely disseminated deposition of calcium in the skin, subcutaneous tissues, muscles, and tendons. Calcinosis is often associated with collagen diseases, scleroderma and dermatomyositis. The authors report a case of interstitial calcinosis associated with dermatomyositis studied with conventional radiograph, ultrasound and magnetic resonance imaging, and correlate the imaging findings with the results of surgical pathology gross examination. (author)

  7. Scrotal calcinosis due to resorption of cyst walls: a case report

    Directory of Open Access Journals (Sweden)

    Parlakgumus Alper

    2008-12-01

    Full Text Available Abstract Introduction Scrotal calcinosis is a rare benign entity defined as the presence of multiple calcified nodules within the scrotal skin. There are controversies about the origin of this entity. In fact, it is still debatable whether scrotal calcinosis is an idiopathic growth or dystrophic calcification of dartoic muscles. It is also unclear whether scrotal calcinosis originates from inflammation of epidermal cysts affected by mild to moderate inflammation of mononuclear cells, from foreign body granuloma formation followed by resorption of cyst walls or from eccrine epithelial cysts. Case presentation We report a 41-year-old male Turkish patient presenting with a 10-year history of scrotal tumours increasing slowly in size and number. Histopathologically, there was no epithelial lining around the calcified nodules, but there was fibrosis adjacent to atrophic stratified squamous epithelium. Conclusion Results of histopathological examinations suggested that scrotal calcinosis might have been due to resorption of cyst walls. Surgery remains the key for this problem. In cases of non-massive scrotal calcinosis, like the case presented here, excision of the nodules from the affected part of the scrotal wall and repairing the defect with horizontal stitches offer good cosmetic results without relapse.

  8. Efficacy of thalidomide in a girl with inflammatory calcinosis, a severe complication of juvenile dermatomyositis

    Directory of Open Access Journals (Sweden)

    Inayama Yoshiaki

    2010-02-01

    Full Text Available Abstract We report a 14-year-old girl with juvenile dermatomyositis (JDM complicated by severe inflammatory calcinosis successfully treated with thalidomide. She was diagnosed as JDM when she was 4 years old after a few months of increasing lethargy, muscle pain, muscle weakness, and rash. During three months, clinical manifestations and abnormal laboratory findings were effectively treated with oral prednisolone. However, calcinosis was recognized 18 months after disease onset. Generalized calcinosis rapidly progressed with high fever, multiple skin/subcutaneous inflammatory lesions, and increased level of CRP. Fifty mg/day (1.3 mg/kg day of oral thalidomide was given for the first four weeks, and then the dose was increased to 75 mg/day. Clinical manifestations subsided, and inflammatory markers had clearly improved. Frequent high fever and local severe pain with calcinosis were suppressed. The levels of FDP-E, IgG, and tryglyceride, which were all elevated before the thalidomide treatment, were gradually returned to the normal range. Over the 18 months of observation up to the present, she has had no inflammatory calcinosis, or needed any hospitalization, although established calcium deposits still remain. Her condition became painless, less extensive and less inflammatory with the CRP level below 3.08 mg/dL. Recent examination by whole-body 18F-FDG-PET-CT over the 15 months of thalidomide treatment demonstrated fewer hot spots around the subcutaneous calcified lesions.

  9. Tumoral calcinosis in the neck region involving an unusual site in a hemodialysis patient.

    Science.gov (United States)

    Pan, Chien-Wei; Chen, Rong-Fong

    2016-05-01

    Tumoral calcinosis is a rare disorder characterized by calcium salt deposition in the periarticular soft tissue region. It often occurs in the hip, elbow, shoulder, foot, and wrist. Soft tissue calcification associated with tumoral calcinosis is rare in the head and neck. The present case describes a 65- year-old male who had been on hemodialysis for 3 years. A bulky mass, containing chalky amorphous calcified material, occurred at the lower neck and superior mediastinum. Chronic renal failure is a common condition associated with secondary tumoral calcinosis. The pathogenesis has been progressively unraveled, which indicates that secondary hyperparathyroidism and tissue injury play a part in multifactorial calcification. Laryngoscope, 126:E196-E198, 2016. PMID:26608820

  10. Disproportionately severe calcinosis cutis in an 88-year-old patient with CREST syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Buchowski, J.M.; Ahn, N.U.; Ahn, U.M. [Dept. of Orthopaedic Surgery, Johns Hopkins Univ. School of Medicine, Baltimore, MD (United States); McCarthy, E.F. [Dept. of Orthopaedic Surgery, Johns Hopkins Univ. School of Medicine, Baltimore, MD (United States); Dept. of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD (United States); Mehta, M.B. [Clinical Associates, Good Samaritan Hospital, Baltimore, MD (United States)

    2001-08-01

    An 88-year-old woman with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) presented with hyperglycemia, intravascular depletion, and atrial fibrillation. The patient was found to have unusually severe calcinosis cutis in both legs extending from the knees to the ankles bilaterally, as well as Raynaud's phenomenon, sclerodactyly, and telangiectasias. The patient was normocalcemic and normophosphatemic. Although subcutaneous calcification is often seen with CREST syndrome, this case is unusual in that the area of involvement was much larger than previously described. Furthermore, the amount of calcinosis was disproportionately severe and was the major cause of symptoms and disability compared with the other components of the syndrome. (orig.)

  11. Tumoral calcinosis presenting as a deformity of the thoracic spine.

    Science.gov (United States)

    Kalani, M Yashar S; Martirosyan, Nikolay L; Little, Andrew S; Kakarla, Udaya K; Theodore, Nicholas

    2011-12-01

    The authors describe a rare case of tumoral calcinosis (TC) of the thoracic spine in a 13-year-old boy with thoracic scoliosis. The patient presented with a 2-year history of back pain. He had no personal or family history of bone disease, deformity, or malignancy. Magnetic resonance imaging revealed a heterogeneously enhancing mass involving the T-7 vertebral body and the left pedicle. Computed tomography findings suggested that the mass was calcified and that this had resulted in scalloping of the vertebral body. The lesion was resected completely by using a left T-7 costotransversectomy and corpectomy. The deformity was corrected with placement of a vertebral body cage and pedicle screw fixation from T-5 to T-9. Pathological analysis of the mass demonstrated dystrophic calcification with marked hypercellularity and immunostaining consistent with TC. This represents the third reported case of vertebral TC in the pediatric population. Pediatric neurosurgeons should be familiar with lesions such as TC, which may be encountered in the elderly and in hemodialysis-dependent populations, and may not always require aggressive resection. PMID:22132916

  12. Tumoral calcinosis peri articular in infants: study by conventional radiology and CT

    International Nuclear Information System (INIS)

    Tumoral calcinosis is an uncommon disease of poorly defined origin that effects the peri articular soft tissue. It is very rarely located in the region of the knee except in children. We present a case occurring in the knee in which the course of both the clinical and radiological signs was favorable. (Author)

  13. Calcinosis of joints and periarticular tissues associated with vitamin D intoxication.

    Science.gov (United States)

    Butler, R C; Dieppe, P A; Keat, A C

    1985-01-01

    We describe a patient with rheumatoid arthritis and widespread joint and periarticular calcinosis related to self-medication with vitamin D, which was aggravated by oral phosphate therapy prescribed for her hypercalcaemia. Hydroxyapatite was shown in the synovial fluid from affected joints. The role played by tissue injury in the pathogenesis of soft tissue calcification is discussed. Images PMID:4026410

  14. Calcinosis of joints and periarticular tissues associated with vitamin D intoxication.

    OpenAIRE

    Butler, R C; Dieppe, P A; Keat, A C

    1985-01-01

    We describe a patient with rheumatoid arthritis and widespread joint and periarticular calcinosis related to self-medication with vitamin D, which was aggravated by oral phosphate therapy prescribed for her hypercalcaemia. Hydroxyapatite was shown in the synovial fluid from affected joints. The role played by tissue injury in the pathogenesis of soft tissue calcification is discussed.

  15. Tumoral calcinosis: seasonal biochemical studies and chemical studies of eyelid lesion.

    Science.gov (United States)

    Bruns, D E; Boskey, A L; Lieb, W; Conway, B P; Savory, J; Wills, M R

    1989-01-01

    We recently described (Arch Ophthalmol 1988; 106:725-6) the presence of unique calcific lesions in the eyelids of a young woman with a history of hyperphosphatemic tumoral calcinosis. Here we document that no immediate family members showed similar lesions and that none was hyperphosphatemic. Dental roentgenography revealed characteristic abnormalities in the patient that confirmed the clinical diagnosis of tumoral calcinosis. Seasonal biochemical studies demonstrated persistently increased concentrations of phosphorus and 1,25-dihydroxyvitamin D in her serum. A calcific eyelid excrescence removed from the patient, studied by x-ray diffraction, was found to consist of crystals of hydroxyapatite. Microprobe analysis indicated the major elements in the deposit to be Ca, P, S, and Cl, just as in the periarticular deposits found in tumoral calcinosis. The Ca concentration in the patient's tear fluid, measured by atomic absorption spectrometry, was within the range found in tears of healthy volunteers. Phosphorus was undetectable (less than 30 mumol/L) in tears of the patient and the volunteers. These findings suggest that the eyelid lesions represent a new manifestation of the pathological process that produces the characteristic periarticular calcific masses of tumoral calcinosis. PMID:2535974

  16. Subcutis calcinosis caused by injection of calcium-containing heparin in a chronic kidney injury patient

    Directory of Open Access Journals (Sweden)

    Lilia Ben Fatma

    2014-01-01

    Full Text Available Subcutis calcinosis, characterized by abnormal calcium deposits in the skin, is a rare complication of using calcium-containing heparin occurring in patients with advanced renal failure. We report the case of an 83-year-old female, a known case of chronic kidney disease (CKD for four years with recent worsening of renal failure requiring hospitalization and hemodialysis. She developed subcutis calcinosis following injection of calcium-containing heparin. Biochemical tests showed serum parathormone level at 400 pg/dL, hypercalcemia, elevated calcium-phosphate product and monoclonal gammopathy related to multiple myeloma. She developed firm subcu-taneous nodules in the abdomen and the thighs, the injection sites of Calciparin ® (calcium nadroparin that was given as a preventive measure against deep vein thrombosis. The diagnosis of subcutis calcinosis was confirmed by the histological examination showing calcium deposit in the dermis and hypodermis. These lesions completely disappeared after discontinuing calcium nadro-parin injections. Subcutis calcinosis caused by injections of calcium-containing heparin is rare, and, to the best our knowledge, not more than 12 cases have been reported in the literature. Pathogenesis is not well established but is attributed to the calcium disorders usually seen in advanced renal failure. Diagnosis is confirmed by histological tests. Outcome is mostly favorable. The main differential diagnosis is calciphylaxis, which has a poor prognosis. Even though rarely reported, we should be aware that CKD patients with elevated calcium-phosphorus product can develop subcutis calcinosis induced by calcium-containing heparin. When it occurs, fortunately and unlike calci-phylaxis, outcome is favorable.

  17. Tumoral calcinosis in a dog with chronic renal failure : clinical communication

    Directory of Open Access Journals (Sweden)

    T.C. Spotswood

    2003-06-01

    Full Text Available A 2-year-old male German shepherd dog in poor bodily condition was evaluated for thoracic limb lameness due to a large, firm mass medial to the left cranial scapula. Radiography revealed several large cauliflower-like mineralized masses in the craniomedial left scapula musculature, pectoral region and bilaterally in the biceps tendon sheaths. Urinalysis, haematology and serum biochemistry showed that the dog was severely anaemic, hyperphosphataemic and in chronic renal failure. The dog was euthanased and a full post mortem performed. A diagnosis of chronic renal failure with secondary hyperparathyroidism was confirmed. The mineralized masses were grossly and histopathologically consistent with a diagnosis of tumoral calcinosis. Tumoral calcinosis associated with chronic renal failure that does not involve the foot pads is rarely seen.

  18. Tumoral calcinosis of the foot: An unusual differential diagnosis of calcaneal mass

    OpenAIRE

    Amir Sobhani Eraghi; Babak Athari; Parnian Kheirkhah Rahimabad

    2015-01-01

    Introduction: Tumoral calcinosis (TC) is a rare disorder characterized by the development of calcified masses within the periarticular soft tissues of large joints. It commonly involves the hip, shoulders, and elbows. TC rarely involves the feet. Case presentation: In this report, we describe an unusual case of primary TC of the foot in a 76-year-old female and discuss the pathophysiology, diagnosis, and therapeutic interventions of the condition. Discussion: Due to the wide range of co...

  19. Milia-like Idiopathic Calcinosis Cutis Occurring in a Toddler Born as a Premature Baby

    OpenAIRE

    Jang, Eun Joo; Lee, Ji Yeoun; Yoon, Tae Young

    2011-01-01

    Milia-like idiopathic calcinosis cutis (MICC) is characterized by smooth, firm, whitish papules resembling milia. Histologically, it appears as a well-defined, round, basophilic nodule within the upper dermis. Although the etiology and treatment remain unclear, it may resolve spontaneously. Some cases have been associated with Down syndrome, and the mean age of MICC patients was 9.9 years old. Herein, we report a rare case of MICC that was not associated with Down syndrome. Noticeably, the pa...

  20. Tumoral Calcinosis as an Initial Complaint of Juvenile-Onset Amyopathic Dermatomyositis.

    Science.gov (United States)

    Doh, Eun Jin; Moon, Jungyoon; Shin, Sue; Seo, Soo Hyun; Park, Hyun Sun; Yoon, Hyun-Sun; Cho, Soyun

    2016-06-01

    Calcinosis is rarely observed in juvenile-onset amyopathic dermatomyositis in contrast to juvenile-onset dermatomyositis. A 6-year-old female presented with several 0.5 to 2 cm-sized painless grouped masses on both knees for 3 years. The patient also presented with multiple erythematous scaly patches and plaques on both elbows, knuckles, buttock, ankles and cheeks. Her mother had similar skin lesions which were erythematous scaly patches on the knuckles and elbows, since her childhood. When skin biopsy was performed from a left knee nodule, liquid chalky discharge was observed. The biopsy results were consistent with calcinosis cutis. Other biopsies from erythematous patch of the patient and erythematous patch of her mother showed vacuolization of basal cell layer with inflammatory cell infiltrations. Laboratory findings showed normal range of serum phosphorus (4.5 mg/dl), calcium (9.3 mg/dl), 1,25-dihydroxy-vitamin D (10.8 ng/ml) and parathyroid hormone levels (11 pg/ml). Both patient and her mother had no history of muscle weakness and showed normal levels of muscle-specific enzyme. Both patients were diagnosed with juvenile-onset amyopathic dermatomyositis based on histopathology and cutaneous manifestations with no evidence of muscle weakness and no serum muscle enzyme abnormalities. Tumoral calcium deposits observed in daughter was diagnosed as dystrophic calcinosis which can be rarely seen in juvenile-onset amyopathic dermatomyositis. The patient is being treated with oral acetazolamide (40 mg/kg/d) for calcinosis. PMID:27274638

  1. Dystrophic calcinosis with both a huge calcified mass in the cervical spine and calcification in the chest wall in a patient with rheumatoid overlap syndrome.

    Science.gov (United States)

    Nakamura, Tadashi; Hirakawa, Kei; Takaoka, Hirokazu; Iyama, Ken-Ichi

    2016-05-01

    Dystrophic calcinosis in soft tissue occurs in damaged or devitalized tissues in the presence of normal calcium and phosphorous metabolism. It is often noted in subcutaneous tissues in patients with collagen vascular diseases and may involve a relatively localized area or be widespread. A 74-year-old Japanese woman with an overlap of rheumatoid arthritis, Sjögren's syndrome, and systemic sclerosis developed a huge tumor-like mass at the atlanto-axial vertebral joint region that caused severe cervical pain and difficulty in activities of daily living. She also had subcutaneous dystrophic calcification in the soft tissue of the chest wall. Calcinosis associated with systemic sclerosis is a well-recognized phenomenon, but a destructive paraspinal tumor in the cervical spine associated with overlap syndrome is extremely unique. Because calcinosis in spinal locations can be complicated by neurological involvement, patients with progressive symptoms may require surgical intervention. Surgical resection and biological therapy improved this patient's life and activities of daily living. Calcinosis is common in the conditions reviewed here, and different agents have been used for treatment. However, calcinosis management is poorly organized and lacks an accepted classification, systematic studies, and clinical therapeutic trials. The association of calcinosis and collagen vascular diseases is clinically and etiologically important. Although a combination of calcinosis and rheumatoid overlap syndrome is rare, various collagen vascular diseases may occur simultaneously. A perceptive diagnostic approach toward these diseases is critical, and early diagnosis and treatment are needed to prevent dystrophic calcinosis. PMID:24894107

  2. Greater trochanteric pain syndrome due to tumoral calcinosis in a patient with chronic kidney disease.

    Science.gov (United States)

    Baek, Dongjin; Lee, Sang Eun; Kim, Woo-Jin; Jeon, Sanghoon; Lee, Kihwa; Jung, Jaewook; Joo, Hyunchul; Park, Jaehong; Kim, Yonghan; Choi, Young-gyun

    2014-01-01

    Tumoral calcinosis is a rare syndrome characterized by massive subcutaneous soft tissue deposits of calcium phosphate near the large joints. It is more prevalent in patients with chronic kidney disease undergoing dialysis. A 57-year-old woman was referred to our pain clinic with the complaint of severe pain in the left buttock and lateral hip. The patient had been suffering from chronic kidney disease for 10 years and had been undergoing peritoneal dialysis over the past 5 years. The patient's symptom was initially suspected to be of lumbar origin at the L5 level and a left L5 transforaminal epidural block was performed, but without success. Re-evaluation of the physical examination revealed severe tenderness over the left greater trochanter and piriformis muscle. On ultrasonographic evaluation, multiple mass-like lesions in the left buttock were observed. About 30 mL of fluid was aspirated from the cystic lesions, followed by 30 mL mixture of 0.08% levobupivacaine and triamcinolone 40 mg injected into the bursa under ultrasound guidance, which brought pain relief. Trochanteric bursitis was thought of as the cause of the symptoms. The patient was diagnosed with tumoral calcinosis based on the past medical history, simple plain radiographs, and hip magnetic resonance imaging (MRI). We diagnosed a case of greater trochanteric pain syndrome due to tumoral calcinosis related to chronic kidney disease in a patient whose symptoms had initially been considered to be radiating leg pain caused by lumbar spinal disease. We report our experience of symptomatic improvement following the repeated ultrasound-guided aspiration of calcific fluid and the injection of a mixture of local anesthetic and steroid. PMID:25415793

  3. Peritendinous calcinosis of calcaneus tendon associated with dermatomyositis: correlation between conventional radiograph, ultrasound, magnetic resonance imaging and gross surgical pathology; Calcinose peritendinea do tendao calcaneo associada a dermatomiosite: correlacao entre radiografia convencional, ultra-sonografia, ressonancia magnetica e macroscopia cirurgica

    Energy Technology Data Exchange (ETDEWEB)

    Rosa, Ana Claudia Ferreira; Gomide, Lidyane Marques de Paula; Lemes, Marcella Stival [Universidade Federal de Goias (UFG), Goiana, GO (Brazil). Faculdade de Medicina. Hospital das Clinicas; Costa, Edegmar Nunes; Rocha, Valney Luiz da [Universidade Federal de Goias (UFG), Goiania, GO (Brazil). Faculdade de Medicina. Dept. de Ortopedia; Machado, Marcio Martins; Santos Junior, Rubens Carneiro dos; Barros, Nestor de; Cerri, Giovanni Guido [Universidade Federal de Goias (UFG), Goiania, GO (Brazil). Faculdade de Medicina. Dept. de Radiologia; Sernik, Renato Antonio [Sao Paulo Univ., SP (Brazil). Hospital das Clinicas. Inst. de Radiologia; Nunes, Rodrigo Alvarenga [Universidade do Vale do Sapucai (UNIVAS), Pouso Alegre, MG (Brazil). Faculdade de Ciencias Medicas; Albieri, Alexandre Daher [Hospital de Acidentados de Goiania, GO (Brazil)

    2006-01-15

    Interstitial calcinosis is an uncommon condition in which there is either localized or widely disseminated deposition of calcium in the skin, subcutaneous tissues, muscles, and tendons. Calcinosis is often associated with collagen diseases, scleroderma and dermatomyositis. The authors report a case of interstitial calcinosis associated with dermatomyositis studied with conventional radiograph, ultrasound and magnetic resonance imaging, and correlate the imaging findings with the results of surgical pathology gross examination. (author)

  4. Calcinose na infância, um desafio terapêutico Calcinosis in juvenile dermatomyositis, a therapeutic challenge

    Directory of Open Access Journals (Sweden)

    Tânia Caroline Monteiro de Castro

    2007-02-01

    Full Text Available A calcinose é uma complicação comum e incapacitante da dermatomiosite. Ela constitui uma característica marcante da doença, ocorrendo principalmente em pacientes pediátricos. Pouco se sabe a respeito de sua fisiopatologia e não existe um tratamento reconhecido universalmente. Este trabalho relata dois casos de dermatomiosite juvenil e calcinose grave com melhora clínica com o uso do alendronato e do diltiazen em um paciente e da imunoglobulina endovenosa em outro.Calcinosis is a common and debilitating complication of dermatomyositis. It is a hallmark of the disease, occurring mainly in pediatric patients. Little is known about its pathophysiology, and there is no universally recognized treatment. This is a report of two children with juvenile dermatomyositis and severe calcinosis who showed improvement with the use of alendronate and diltiazen in one patient, and intravenous immunoglobulin in the other.

  5. Autoantibodies to a 140-kd protein in juvenile dermatomyositis are associated with calcinosis.

    LENUS (Irish Health Repository)

    Gunawardena, H

    2009-06-01

    OBJECTIVE: The identification of novel autoantibodies in juvenile dermatomyositis (DM) may have etiologic and clinical implications. The aim of this study was to describe autoantibodies to a 140-kd protein in children recruited to the Juvenile DM National Registry and Repository for UK and Ireland. METHODS: Clinical data and sera were collected from children with juvenile myositis. Sera that recognized a 140-kd protein by immunoprecipitation were identified. The identity of the p140 autoantigen was investigated by immunoprecipitation\\/immunodepletion, using commercial monoclonal antibodies to NXP-2, reference anti-p140, and anti-p155\\/140, the other autoantibody recently described in juvenile DM. DNA samples from 100 Caucasian children with myositis were genotyped for HLA class II haplotype associations and compared with those from 864 randomly selected UK Caucasian control subjects. RESULTS: Sera from 37 (23%) of 162 patients with juvenile myositis were positive for anti-p140 autoantibodies, which were detected exclusively in patients with juvenile DM and not in patients with juvenile DM-overlap syndrome or control subjects. No anti-p140 antibody-positive patients were positive for other recognized autoantibodies. Immunodepletion suggested that the identity of p140 was consistent with NXP-2 (the previously identified MJ autoantigen). In children with anti-p140 antibodies, the association with calcinosis was significant compared with the rest of the cohort (corrected P < 0.005, odds ratio 7.0, 95% confidence interval 3.0-16.1). The clinical features of patients with anti-p140 autoantibodies were different from those of children with anti-p155\\/140 autoantibodies. The presence of HLA-DRB1*08 was a possible risk factor for anti-p140 autoantibody positivity. CONCLUSION: This study has established that anti-p140 autoantibodies represent a major autoantibody subset in juvenile DM. This specificity may identify a further immunogenetic and clinical phenotype within the

  6. Familial tumoral calcinosis and hyperostosis-hyperphosphataemia syndrome are different manifestations of the same disease: novel missense mutations in GALNT3

    Energy Technology Data Exchange (ETDEWEB)

    Joseph, Leo; Joseph, Selvanayagam [Vinodhagan Memorial Hospital and Dr. Joseph' s Ortho Clinic, Department of Orthopaedic Surgery, Thanjavur (India); Hing, Sandra N.; Idowu, Bernadine D.; Delaney, David [Royal National Orthopaedic Hospital NHS Trust, Department of Histopathology, Stanmore, Middlesex (United Kingdom); Presneau, Nadege [University College London (UCL), Cancer Institute, London (United Kingdom); O' Donnell, Paul [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, Stanmore, Middlesex (United Kingdom); University College London (UCL), Institute of Orthopaedics and Musculoskeletal Science, Stanmore (United Kingdom); University College London (UCL), The Institute of Orthopaedics and Musculoskeletal Science, London (United Kingdom); Diss, Tim [University College London Hospital (UCLH) NHS Trust, Rockefeller Building, Department of Histopathology, London (United Kingdom); Flanagan, Adrienne Margaret [Royal National Orthopaedic Hospital NHS Trust, Department of Histopathology, Stanmore, Middlesex (United Kingdom); University College London (UCL), Cancer Institute, London (United Kingdom); University College London Hospital (UCLH) NHS Trust, Rockefeller Building, Department of Histopathology, London (United Kingdom); University College London (UCL), Institute of Orthopaedics and Musculoskeletal Science, Stanmore (United Kingdom); Institute of Orthopaedics and Musculoskeletal Science, Stanmore, Middlesex (United Kingdom)

    2010-01-15

    To report on the biochemistry and clinical and genetic findings of two siblings, the younger sister presenting with recurrent bone pain of the radius and ulna, and medullary sclerosis, and the older brother with soft tissue calcific deposits (tumoral calcinosis) but who later developed bone pain. Both were found to be hyperphosphaturic. The index family comprised four individuals (father, mother, brother, sister). The affected siblings were the offspring of a non-consanguineous Indian family of Tamil origin. Bidirectional sequencing was performed on the DNA from the index family and on 160 alleles from a population of 80 unrelated unaffected control individuals of Tamil extraction and 72 alleles from individuals of non-Tamil origin. Two symptomatic siblings were found to harbour previously unreported compound heterozygous missense UDP-N-acetyl-D-galactosamine: polypeptide N-acetylgalactosaminyltransferase 3 (GalNAc-transferase; GALNT3) mutations in exon 4 c.842A>G and exon 5 c.1097T>G. This sequence variation was not detected in the control DNA. This is the first report of siblings exhibiting stigmata of familial tumoral calcinosis and hyperostosis-hyperphosphataemia syndrome with documented evidence of autosomal recessive missense GALNT3 mutations. The findings from this family add further evidence to the literature that familial tumoral calcinosis and hyperostosis-hyperphosphataemia syndrome are manifestations of the same disease and highlight the importance of appropriate metabolic and genetic investigations. (orig.)

  7. Dystrophic calcification in muscles of legs in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome: Accurate evaluation of the extent with 99mTc-methylene diphosphonate single photon emission computed tomography/computed tomography

    International Nuclear Information System (INIS)

    We present the case of a 35-year-old man with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia variant scleroderma who presented with dysphagia, Raynaud's phenomenon and calf pain. 99mTc-methylene diphosphonate bone scintigraphy was performed to identify the extent of the calcification. It revealed extensive dystrophic calcification in the left thigh and bilateral legs which was involving the muscles and was well-delineated on single photon emission computed tomography/computed tomography. Calcinosis in scleroderma usually involves the skin but can be found in deeper periarticular tissues. Myopathy is associated with a poor prognosis

  8. Dystrophic calcification in muscles of legs in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia syndrome: Accurate evaluation of the extent with 99mTc-methylene diphosphonate single photon emission computed tomography/computed tomography

    OpenAIRE

    Chakraborty, Partha Sarathi; Karunanithi, Sellam; Dhull, Varun Singh; Kumar, Kunal; Tripathi, Madhavi

    2015-01-01

    We present the case of a 35-year-old man with calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia variant scleroderma who presented with dysphagia, Raynaud's phenomenon and calf pain. 99mTc-methylene diphosphonate bone scintigraphy was performed to identify the extent of the calcification. It revealed extensive dystrophic calcification in the left thigh and bilateral legs which was involving the muscles and was well-delineated on single photon emission c...

  9. Arterial diffuse intimal thickening associated with enzootic calcinosis of sheep Espessamento intimal difuso das artérias na calcinose enzoótica dos ovinos

    Directory of Open Access Journals (Sweden)

    Rosemeri Oliveira Vasconcelos

    1998-01-01

    Full Text Available Morphometric, immunohistochemical and ultrastructural studies were carried out on the diffuse intimal thickening (DIT in arteries of 7 sheep with clinical signs of naturally occurring enzootic calcinosis due to ingestion of the plant Nierembergia veitchii. Arterial lesions consisted of medial deposition of calcium salts and DIT. Calcification of the intima was rare, mild and located near the elastic lamina. By immunohistochemistry a-actin was detected in cells of the media and in cells forming the intimal thickening. Receptors for 1,25(OH2 vitamin D3 were detected in nuclei of intimal, medial and endothelial cells. DIT was irregularly distributed and was neither proportionally related to the intensity of the underlying mineralization area nor to the thickening of the remaining media. Ultrastructural morphometry in smooth muscle cells (SMCs of the media and thickened intima revealed, in the latter, an increase of 318% in the volumetric fraction of those organelles involved in synthesis and a proportional decrease in contractile elements when compared to normal values of media cells. There were histological and ultrastructural evidences of modification of SMCs and their migration to the intima, where they proliferated causing DIT. It was concluded that DIT is a consistent component of arteriosclerotic lesions in N. veitchii induced calcinosis of sheep and that the predominant cell in this process is the SMCs originated from its predecessors of the media. It is suggested that the inducing factor for the arterial changes is 1,25(OH2 D3 present in N. veitchii.Foram feitos estudos morfométrico, imunoistoquímico e ultra-estrutural do espessamento intimal difuso (DIT das artérias de 7 ovinos com sinais clínicos de calcinose enzoótica espontânea causada pela ingestão da planta Nierembergia veitchii. As lesões caracterizavam-se por deposição de sais de cálcio na média como placas e estrias que, com frequência faziam saliência para a luz

  10. Pseudoxanthoma elasticum and calcinosis cutis

    OpenAIRE

    Pala, E E; Bayol, Ü.; Usturalı, Keskin E; Küçük, Ü.; Türe, G; Bıçakçı, C

    2013-01-01

    Pseudoxanthoma elasticum (PXE) is an inherited multisystem disorder with ectopic mineralization of connective tissues and characteristic clinical manifestations in the skin, eyes and cardiovascular system. A 46-year-old woman was admitted to dermatology clinic with whitish/yellow papules that coalesce into cobblestone patterned plaques at the lateral, posterior aspect of the neck 6 previously. Two of the lesions were excised by plastic surgery. Histologically fragmented calcified elastic fib...

  11. Ocular involvement in tumoral calcinosis

    Directory of Open Access Journals (Sweden)

    Harsha Bhattacharjee

    2014-01-01

    Full Text Available We report a 32-year-old male who presented with blurring of vision in the right eye since 1.5 years. He had history of swelling over the extensor surfaces of large joints which were migratory in nature. Few of them spontaneously subsided following suppuration of chalky white discharges except over the gluteal region. Ophthalmological examination revealed visual acuity of counting fingers (CF at 1 m in the right eye and perilimbal conjunctival calcific deposits and retinal angiod streaks in both eyes. There was choroidal neovascular membrane with subretinal hemorrhage in right eye, confirmed by fundus fluorescein angiography (FFA and optical coherence tomography (OCT. B scan ultrasonography and simultaneous vector A scan detected calcification of the subretinal neovascular membrane and the adjoining sclera.

  12. Uso do clodronato endovenoso na calcinose difusa em uma criança com síndrome de superposição esclerose sistêmica e dermatomiosite Use of clodronate in extensive calcinosis in a child with systemic sclerosis and dermatomyositis overlap

    Directory of Open Access Journals (Sweden)

    Gláucio R. Werner de Castro

    2004-02-01

    Full Text Available Os autores descrevem o caso de uma garota negra, com diagnóstico de síndrome de superposição dermatomiosite e esclerose sistêmica, que desenvolveu calcinose difusa, complicada por infecções secundárias e significativa limitação funcional de membros. Tratamento com colchicina, diltiazem e alendronato sódico não se mostrou eficaz no controle da calcinose, requerendo uso endovenoso bimestral de clodronato, que contribuiu para significativa melhora na cicatrização das úlceras cutâneas e na qualidade de vida.The authors report an Afro-Brazilian girl with systemic sclerosis and dermatomyositis overlap who evolved with extensive calcinosis, complicated by secondary infections and important disability. Treatment with colchicine, diltiazem and sodium alendronate was not effective in the control of calcinosis, requiring the use of bimonthly intravenous doses of clodronate that resulted in significant improvement in the healing of cutaneous ulcers and in the quality of life of the patient.

  13. TUMOR CALCINOSIS : A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Kammar

    2015-04-01

    Full Text Available A 15 year old male Arun Suresh K ammar patient presented with the chief complaints of right elbow swelling with discharging sinus since 5 years . Left elbow swelling and ulcer over left gluteal region since 3 years . Patient noticed gradually progressive, painless swelling over right elbow 5 years back and left gluteal region 3 years back for which he underwent surgery 4 and 3 years back respectively. One year latter patient developed gradually progressive diffuse swelling at left elbow with painful left elbow joint movement. Blood investigations were normal except raised phosph orus - 6.8mg/dl(N - 2.5 - 4.5mg/dl and Alkaline phosphatase - 52IU/L(N - 20 - 40IU/L.Total excision was done for the left elbow and left gluteal region (periarticular swelling and right elbow swelling subsided by itself.

  14. Spontaneous Cardiac Calcinosis in BALB/cByJ Mice

    OpenAIRE

    Glass, Aaron M; Coombs, Wanda; Taffet, Steven M

    2013-01-01

    BALB/c mice are predisposed to dystrophic cardiac calcinosis—the mineralization of cardiac tissues, especially the right ventricular epicardium. In previous reports, the disease appeared in aged animals and had an unknown etiology. In the current study, we report a substrain of BALB/c mice (BALB/cByJ) that develops disease early and with high frequency. Here we analyzed hearts grossly to identify the presence and measure the severity of disease and to compare BALB/c substrains. Histologic ana...

  15. Dystrophic calcinosis in a child with a thumb sucking habit: case report

    Directory of Open Access Journals (Sweden)

    Giovannini Cesar Abrantes Lima de Figueiredo

    2000-10-01

    Full Text Available We present an uncommon case of a 3-year-old boy with a finger sucking habit who developed dystrophic calcification in his left thumb. Two years after excision, there was no recurrence, and the thumb retained full range of motion. We also discuss its probable pathogenesis and present a brief review of the literature about orthopedic complications in the hand due to this habit.Os autores apresentam caso incomum de uma criança de três anos de idade com o hábito de chupar o dedo que desenvolveu calcinose distrófica no polegar esquerdo. Dois anos após a ressecção cirúrgica, não ocorreu recidiva e o polegar mantém todos os movimentos. Discutem, ainda, sua provável patogênese e fazem breve revisão da literatura a respeito das complicações ortopédicas na mão devido a este hábito.

  16. CALCINOSIS CUTIS METASTÁSICA: CALCIFILAXIS (ARTERIOLOPATÍA URÉMICA CALCIFICADA. A PROPÓSITO DE UN CASO. [METASTATIC CALCINOSIS CUTIS: CALCIPHYLAXIS (CALCIFIED UREMIC ARTERIOLOPATHY. A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Lourdes Bolla de Lezcano

    2013-07-01

    Full Text Available Resumen La calcifilaxis es un síndrome clínico caracterizado por una calcificación vascular progresiva que ocasiona la aparición de lesiones violáceas, frecuentemente dolorosas, en la piel de pacientes con insuficiencia renal crónica, diálisis o trasplante renal, asociado usualmente a niveles elevados de hormona paratiroidea. Se presenta el caso clínico de una mujer de 44 años, diabética con insuficiencia renal crónica, en hemodiálisis desde hace 2 años, que fue diagnosticada de calcifilaxis tras sospecha clínica y biopsia de lesiones cutáneas. Abstract Calciphylaxis is a clinical syndrome characterized by progressive vascular calcification that causes the appearance of purplish lesions, often painful, in the skin of patients with chronic renal failure, dialysis or kidney transplantation, usually associated with elevated levels of parathyroid hormone. We report a case of a 44-year-old diabetic woman with chronic renal failure on hemodialysis for 2 years. She was diagnosed with calciphylaxis after clinical suspicion and biopsy of skin lesions.

  17. Correlation between quantitative ultrasound parameters of the calcinosis and bone density of the spine and femur in Arabian women: Relation to menopausal status

    International Nuclear Information System (INIS)

    Aims: Quantitative ultrasound (QUS) of the calcaneus is being frequently used to screen for osteoporosis. This technique correlates very well with dual-energy X-ray absorptiometry (DXA) of the spine and femur, and predicts fracture risk in postmenopausal women. The correlation between QUS and DXA in premenopausal women with prevalent vitamin D deficiency however is not known. We assessed the correlation between both techniques in 55 pre- and postmenopausal Arabian women, a population with high prevalence of vitamin D deficiency. Methods: BMD of the right calcaneus was estimated by Sahara ultrasound (Hologic, Waltham, MA). Spine and right total femur BMD was determined by DXA scan (Lunar Expert XL, Madison, WI). Results: Overall, the correlation between calcaneal BMD estimated by QUS and spine and femur BMD measured by DXA in pre- and postmenopausal United Arab Emirates women was statistically significant (r=0.41; p=0.002 for spine and r=0.44; p=0.001 for femur, respectively). In postmenopausal women, all correlations between DXA and QUS parameters were statistically significant. In contrast, only BUA and SOS correlated significantly with spine BMD in premenopausal women. Conclusion: The correlation between DXA and QUS is statistically significant in postmenopausal United Arab Emirates women. However, this correlation is much weaker in premenopausal UAE women suggesting that other factors such as vitamin D deficiency may influence this correlation

  18. Watermelon-stomach og sklerodermi

    DEFF Research Database (Denmark)

    Diederichsen, Axel Cosmus Pyndt; Rasmussen, Jens Møller

    2002-01-01

    We report here a 72-year-old man with severe, persistent, iron deficiency anaemia. Upper gastrointestinal endoscopy revealed red stripes radiating to the pylorus, characteristic of watermelon stomach (gastric antral vascular ectasia). The patient was found to have scleroderma with calcinosis, scl...

  19. CREST Syndrome - a Limited Form of Systemic Scleroderma: a Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Paravina Mirjana

    2015-09-01

    Full Text Available Systemic scleroderma (SSc is a multisystem disease with microvascular abnormalities, autoimmune disorders, excessive collagen production and deposition, and fibrosis of the skin and internal organs. According to the simplest, though incomplete classification, there are two forms of SSc: diffuse and limited (formerly acrosclerosis. CREST syndrome is a subtype of limited SSc, characterized by: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. We present a patient with all the features of the CREST syndrome, which appeared at the age of 43 and lasted for 23 years. The patient presented with a gradual development of symptoms during the first ten years, from Raynaud’s phenomenon, skin sclerosis, calcinosis, telangiectasia, and esophageal dysmotility. The diagnosis was based on clinical findings and relevant diagnostic procedures. The article presents a literature review on the epidemiology, etiology, pathophysiology, clinical manifestations, various attempts at classification, diagnostic criteria, and therapeutic modalities.

  20. Idiopathic Hypoparathyroidism associated Fahrs Disease: Two Case Reports and Review of the Literature

    OpenAIRE

    Mehtap Evran; Gamze Akkus; Murat Sert; Tamer Tetiker

    2015-01-01

    Bilateral Striopallidodentat Calcinosis also called Fahrs disease (FH); basal ganglia, thalamus, and cerebellar dentate nucleus is a disease characterized by symmetric calcifications in the cerebral white matter. Calcifications are often drawn attention in brain imaging for other reasons. The disease can progress with clinically variable neurological or neuro-psychiatric symptoms. Fahrs disease are believed to be associated with anoxia, radiation, systemic diseases, toxins calcium metabolism ...

  1. Idiopathic Hypoparathyroidism associated Fahr's Disease: Two Case Reports and Review of the Literature

    OpenAIRE

    Evran, Mehtap; Akkuş, Gamze; Sert, Murat; Tetiker, Tamer

    2015-01-01

    Bilateral Striopallidodentat Calcinosis also called Fahr's disease (FH); basal ganglia, thalamus, and cerebellar dentate nucleus is a disease characterized by symmetric calcifications in the cerebral white matter. Calcifications are often drawn attention in brain imaging for other reasons. The disease can progress with clinically variable neurological or neuro-psychiatric symptoms. Fahr's disease are believed to be associated with anoxia, radiation, systemic diseases, toxins calcium metabolis...

  2. Nodular regenerative hyperplasia of the liver, CREST syndrome and primary biliary cirrhosis: an overlap syndrome?

    OpenAIRE

    McMahon, R F; Babbs, C.; Warnes, T W

    1989-01-01

    Nodular regenerative hyperplasia of the liver (NRHL) has been found in association with collagen vascular diseases, after drug therapy, with autoimmune disease, and with a variety of haematological disorders. The association of NRHL with the syndrome of Calcinosis cutis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia (CREST syndrome) has only been reported on two previous occasions. The liver disease usually associated with CREST syndrome is primary biliary ci...

  3. Thymic atrophy in cattle poisoned with Solanum glaucophyllum Atrofia do timo em bovinos intoxicados por Solanum glaucophyllum

    OpenAIRE

    Paula A. Fontana; Carolina N. Zanuzzi; Barbeito, Claudio G; Gimeno, Eduardo J; Portiansky, Enrique L.

    2009-01-01

    Solanum glaucophyllum (Sg) [= S. malacoxylon] is a calcinogenic plant inducing "Enzootic Calcinosis" in cattle. The 1,25-dihydroxyvitamin D3, its main toxic principle, regulates bone and calcium metabolism and also exerts immunomodulatory effects. Thymocyte precursors from bone marrow-derived progenitor cells differentiate into mature T-cells. Differentiation of most T lymphocytes is characterized not only by the variable expression of CD4/CD8 receptor molecules and increased surface density ...

  4. Arthroscopic treatment of chronically painful calcific tendinitis of the rectus femoris

    OpenAIRE

    Peng, Xu; Feng, Yong; Chen, Guangxing; Yang, Liu

    2013-01-01

    Background Relatively large calcific tendinitis with persistent symptoms after extended periods of conservative treatment is an indication for operative therapy. Arthroscopy, as a treatment for calcific tendinitis of the hip abductors and calcinosis circumscripta, has been described previously; however, to our knowledge, the clinical and radiological response to arthroscopic removal of calcific tendinitis of the rectus femoris tendon has not. Methods We present arthroscopic treatment of unusu...

  5. Tinea Incognita in a Patient with Crest Syndrome: Case Report.

    Science.gov (United States)

    Gorgievska-Sukarovska, Biljana; Skerlev, Mihael; Žele-Starčević, Lidija

    2015-01-01

    Tinea incognita is a dermatophytic infection that is difficult to diagnose, usually modified by inappropriate topical or systemic corticosteroid therapy. We report an extensive case of tinea incognita caused by the zoophilic dermatophyte Trichophyton mentagrophytes (var. granulosa) in a 49-year-old female patient with CREST (Calcinosis; Raynaud phenomenon; Esophageal involvement; Sclerodactyly; Teleangiectasia) syndrome. Immunocompromised patients, as well as patients with keratinization disorders, seem to be especially susceptible to dermatophytic infections with atypical clinical presentation that is sometimes bizarre and difficult to recognize. Therefore, close monitoring and mycological skin examination is recommended in order to avoid misdiagnosis and to give the patient the best chance of recovery. PMID:26476904

  6. Idiopathic Hypoparathyroidism associated Fahrs Disease: Two Case Reports and Review of the Literature

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    Mehtap Evran

    2015-12-01

    Full Text Available Bilateral Striopallidodentat Calcinosis also called Fahrs disease (FH; basal ganglia, thalamus, and cerebellar dentate nucleus is a disease characterized by symmetric calcifications in the cerebral white matter. Calcifications are often drawn attention in brain imaging for other reasons. The disease can progress with clinically variable neurological or neuro-psychiatric symptoms. Fahrs disease are believed to be associated with anoxia, radiation, systemic diseases, toxins calcium metabolism disorders, and encephalitis. We presented two cases with primary hypoparathyroidism were diagnosed and detected of neurological problems, cerebral and cerebellar calcifications on imaging. [Cukurova Med J 2015; 40(4.000: 836-840

  7. Identification and determination of D3 vitamine, D3 vitamine 25 (OH) and D3 vitamine 1,25(OH)2 in plasma of animals treated with solanum glaucophyllum (Sg)

    International Nuclear Information System (INIS)

    The enzootic calcinosis is a disease produced in the bovines by the ingestion of the toxic plant Sg, which contains vitamine D3 glycosides and its active metabolites. This disease is characterized by the loss of weight and physical condition, motor disorders and alteration of phosphocalcic metabolism with deposition of calcium compounds in soft tissues. To contribute to the advanced diagnostic of the disease, analytic techniques to determine D vitamine, D vitamine 25 (OH) and D vitamine 1,25 (OH)2 in plasma, by high resolution liquid chromatography and radio receptor essay are used

  8. Canine multiple cartilaginous exostoses: unusual manifestations and a review of the literature

    International Nuclear Information System (INIS)

    Multiple cartilaginous exostoses were diagnosed in a two-year-old Great Dane and a four-month-old border collie. Clinically, the Great Dane showed only mild discomfort, while the border collie exhibited tetraparesis due to cervicothoracic compression. Unusual features in the Great Dane were exostoses that bridged physes, with progression after skeletal maturity. The border collie puppy's exostoses resembled tumoral calcinosis radiographically. Limb exostoses in this puppy often were para-articular, and most were not attached to the underlying bone. These features resembled metachondromatosis in humans. Analysis of previously reported cases of multiple cartilaginous exostoses indicated that the prognosis is guarded to poor

  9. Pulsed vs. CW low level light therapy on osteoarticular signs and symptoms in limited scleroderma (CREST syndrome)

    Science.gov (United States)

    Barolet, Daniel

    2012-03-01

    Limited cutaneous systemic sclerosis (lcSSc) was formerly known as CREST syndrome in reference to the associated clinical features: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias. The transforming growth factor beta (TGF-β) has been identified has a major player in the pathogenic process, while low level light therapy (LLLT) has been shown to modulate this cytokine superfamily. This case study was conducted to assess the efficacy of 940nm using microsecond domain pulsing and continuous wave mode (CW) on osteoarticular signs and symptoms associated with lcSSc. The patient was treated two to three times a week for 13 weeks, using a sequential pulsing mode on one elbow, and a CW mode on the other. Efficacy assessments included inflammation, symptoms, pain, and health scales, patient satisfaction, clinical global impression, and adverse effects monitoring. Significant functional and morphologic improvements were observed after LLLT, with best results seen with the pulsing mode. No significant adverse effects were noted. Two mechanisms of action may be at play. The 940nm wavelength provides inside-out heating possibly vasodilating capillaries which in turn increases catabolic processes leading to a reduction of in situ calcinosis. LLLT may also improve symptoms by triggering a cascade of cellular reactions, including the modulation of inflammatory mediators.

  10. [Articular chondrocalcinosis after 80 years of age].

    Science.gov (United States)

    Memin, Y; Monville, C; Ryckewaert, A

    1978-02-01

    In 108 women over 80 (mean age 88,4 years, extremes 80 and 99 years) hospitalized in a geriatric service for various reasons, radiograms were made of both knees in the frontal aspect on standard film to detect calcinosis of the meniscus and chondrocalcinosis of the joint. In 25 women (23.1%) the radiographs revealed calcinosis of the meniscus with or without chondrocalcinosis. In these 25 cases a lateral X-ray was also made of the two knees, frontal X-rays were made of the pelvis, thumbs and shoulders. In 22 cases (88%) these revealed calcification of the fibrocartilages or articular cartilages in joints other than the knee. Seven of the 25 women had at least one attack of articular inflammation (especially of the knee) resembling a pseudo-gout crisis. The frequency of chronic arthropathies resembling arthroses was high in the 25 patients with chondrocalcinosis: 8 (32%) had an internal or external femoro-tibial arthrosis, as against 11 of the 83 patients (13%) of the same age without chondrocalcinosis, a significant difference. Eleven of the 25 women had signs of femororotular arthrosis on the lateral X-rays of the knees, 5 had coxarthrosis (with in 3 cases a radiological image of fibrocartilaginous or coxofemoral cartilaginous calcification). One women had chronic radiocarpal arthropathy evocative or chondrocalcinosis. Ten had a scaphotrapezoidal arthrosis, 5 arthrosis of the shoulder, 3 with radiological aspect of glenohumeral chondrocalcinosis. PMID:644241

  11. Idiopathic infantile arterial calcification in a 12-year-old girl presenting as chronic mesenteric ischemia: imaging findings and angioplasty results

    International Nuclear Information System (INIS)

    We report an unusual case of chronic mesenteric ischemia presenting in a 12-year-old girl with idiopathic infantile arterial calcinosis (IIAC). This is the first reported case in the literature of chronic mesenteric ischemia in the setting of IIAC. The girl presented with a classical history of postprandial abdominal pain. Imaging demonstrated significant stenoses of the celiac axis, superior mesenteric artery (SMA) and inferior mesenteric artery (IMA). Angioplasty of the celiac axis and SMA was attempted, with successful dilation of the SMA only. At 3-, 6- and 12-month follow-ups, the child's symptoms had almost resolved. This case report has three important ramifications: chronic mesenteric ischemia is a possible clinical presentation in children with IACC, pre-angioplasty imaging is important in guiding treatment approach, and angioplasty was effective in this case of chronic mesenteric ischemia and offers hope for other similarly affected children. (orig.)

  12. FIELD STUDY OF THE PREVALENCE OF LAMENESS IN HORSES IN THREE PROVINCES OF IRAN

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    A.T. Naeini and B. T. Niak

    2005-07-01

    Full Text Available A population of 364 show jumper horses was examined for signs of lameness in three province of Iran, i.e., Tehran, Isfahan and Fars. The basis of the survey was the inability of the horse to take part in jumping exercise. The diagnosis of lameness was performed using appropriate methods of visual and physical examination, aided by nerve blocking and radiographic examination. In 364 horses, included cases of hygroma, very short heel, tendonitis, fissure in the hoof, navicular syndrome, laminitis, white line disease, splints, sore shine, side bone, ring bone, tumoral calcinosis, sheard heel, incorrect shoeing and wind puff. This suggests that the diagnosis, therapy and prophylaxis of lameness merit a high priority in research efforts to reduce wastage among valubale show jumper horses.

  13. The Use of Calcium Channel Blockers in Skin Diseases

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    Özge Uzun

    2013-05-01

    Full Text Available Calcium channel blockers are a group of drugs often used to treat cardiovascular diseases, such as hypertension, angina, peripheral vascular disorders and some arrhythmias. These drugs may suppress the growth and proliferation of vascular smooth muscle cells and fibroblasts, and inhibit the synthesis of extracellular-matrix proteins,such as collagen, fibronectin, proteoglycans. Some calcium channel blockers also have immunomodulatory or dysregulatory effects on lymphocytes and can suppress superoxide generation and phagocytic activity of neutrophils. Moreover, mast cell degranulation and platelet aggregation may also be impaired. On account of these properties, calcium channel blockers have also been used for the prevention and treatment of various dermatologic diseases. In this review, we evaluated the use of calcium channel blockers in various dermatologic diseases, such as Raynaud’s phenomenon, chilblains, chronic anal fissures, vulvodynia, keloids and burn scars, calcinosis cutis, and leiomyoma.

  14. Medical image of the week: CREST plus ILD

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    Oliva I

    2013-06-01

    Full Text Available A 60 year old female with a history of fibromyalgia presented with dyspnea and skin changes, predominantly on the hands. Physical exam and imaging showed classic findings of limited cutaneous systemic sclerosis (scleroderma CREST syndrome. Calcinosis cutis (Figure 1A, Raynaud’s (not shown but endorsed by the patient, Esophageal dysmotility (Figure 1B, dilated esophagus, Sclerodactyly (Figure 1C, and Teleganectasias (Figure 1D were all present. Ground glass opacities were seen predominantly in the bilateral lower lung zones, associated with increased reticular markings (Figure 2A, and traction bronchiectasis (Figure 2B. Pulmonary involvement is noted in the majority of scleroderma patients. Interstitial lung disease (ILD is common and often portends a poor prognosis.

  15. Radiodiagnosis of hemophiliac bone pseudotumors

    International Nuclear Information System (INIS)

    Of 259 hemophiliacs bone pseudotumors were diagnosed in 11 (4.3 %); they were localised in the femur (6 cases), calcaneus (4) and in the iliac bone (3). Two cases of combined fermoral and calcaneal lesions and 4 cases of bone fracture were observed. As a rule, pseudotumors developed in hemophiliacs with severe disease. An x-ray picture of a pseudotumor depended on its site and was characterized by a large soft tissue tumor shadow, often with calcinosis, and serious destructive changes in bones in the form or round foci of 7 cm in diameter with clear-cut contours. An adge defect of the cortical layer was defined in the diaphysis of the femoral bone (15 cm long). Destructive changes were often accompanied by osteosclerosis and periostitis

  16. Imaging of connective tissue diseases of the head and neck.

    Science.gov (United States)

    Abdel Razek, Ahmed Abdel Khalek

    2016-06-01

    We review the imaging appearance of connective tissue diseases of the head and neck. Bilateral sialadenitis and dacryoadenitis are seen in Sjögren's syndrome; ankylosis of the temporo-mandibular joint with sclerosis of the crico-arytenoid joint are reported in rheumatoid arthritis and lupus panniculitis with atypical infection are reported in patients with systemic lupus erythematosus. Relapsing polychondritis shows subglottic stenosis, prominent ear and saddle nose; progressive systemic sclerosis shows osteolysis of the mandible, fibrosis of the masseter muscle with calcinosis of the subcutaneous tissue and dermatomyositis/polymyositis shows condylar erosions and autoimmune thyroiditis. Vascular thrombosis is reported in antiphospholipid antibodies syndrome; cervical lymphadenopathy is seen in adult-onset Still's disease, and neuropathy with thyroiditis reported in mixed connective tissue disorder. Imaging is important to detect associated malignancy with connective tissue disorders. Correlation of the imaging findings with demographic data and clinical findings are important for the diagnosis of connective tissue disorders. PMID:26988082

  17. Pituitary-dependent hyperadrenocorticism in a terrier dog:A case report

    Institute of Scientific and Technical Information of China (English)

    Mahdieh Rezaei; Sara Rostami; Mehdi Saberi; Dariush Vosugh

    2016-01-01

    We report a case of pituitary-dependent hyperadrenocorticism in a 10-year-old, female, terrier dog. The animal was admitted due to polyphagia, weight gain, polyuria, polydipsia, hair loss, exercise intolerance and panting at rest. On physical examination, abdominal distention, truncal and bilaterally symmetric alopecia, thin hypotonic skin, comedones, bruising, hyperpigmentation and calcinosis cutis on the dorsal midline were observed. Hematologic investigations showed stress leukogram, high serum alkaline phosphatase activity, mild to moderate alanine aminotransferase activity, hypercholesterolemia, hypertriglyceridemia and hyperglycemia. Mild generalized interstitial lung patterns and hepatomegaly were detected in the radiographs. Bilaterally symmetric normal-sized adrenal glands were also diagnosed in ultrasonography. Diagnosis of pituitary-dependent hyperadrenocorticism was confirmed with low-dose dexamethasone suppression test. The dog was successfully treated with mitotane.

  18. [Anatomic incidence of meniscochondrocalcinosis of the knee].

    Science.gov (United States)

    Mitrovic, D; Stankovic, A; Morin, J; Borda-Iriarte, O; Uzan, M; Quintero, M; Memin, Y; Bard, M; de Sèze, S; Richewaert, A

    1982-06-01

    The authors have studied the incidence of menisco-calcinosis (MC) and that of menisco-chondrocalcinosis (MCC) of knee joints of 108 non selected cadavera. The mean age of the subjects was 71.8 +/- 13.8 years. The study was performed by radiographic examination of the menisci and cartilagineous fragments of femoral condyles using high contrast films. The incidence of MC or MCC was found to be 18.5 per cent. It was slightly higher in females (21.5 p. cent) than in males (15.8 p. cent) subjects but this difference failed to reach the level of statistical significance. No positive case was detected before the age of 60 years. For the age groups of: 60-69, 70-79, 80-89 and over 90 years, its incidence was: 11.7; 26.9; 21.2 and 50 (4 subjects out of 8) per cent respectively. Approximately 40 per cent of all positive cases had meniscocalcinosis without associated chondrocalcinosis. No single case of chondrocalcinosis without meniscocalcinosis was observed. Six out of 8 cases with MC calcinosis and 2 out of 12 cases with MCC were unilateral. The external menisci were more frequently and more heavily affected than internal ones. Eighty per cent of the knees affected by either MC or MCC were at the same time associated to an osteoarthrotic lesion compared to 35 p. cent of the knees without MC or MCC matched for age: a result which appeared to be highly significant difference. PMID:6896928

  19. Non-Venereal Dermatoses In Male Genital Region-Prevalence And Patterns In A Referral Centre In South India

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    Karthikeyan K

    2001-01-01

    Full Text Available A series of 100 male patients with non-venereal dermatoses of external genitalia were screened amongst patients visiting Dermatology OPD of JIPMER, Pondicherry from Aug ’97 to March ’99. The overall prevalence was found to be 14.1 per 10,000. Non-venereal dermatoses were common in the 21-40 years age group. Most of the patients (74% belonged to labourer class. A total of 25 different non-venereal dermatoses were studied. Genital vitiligo was the most common disorder accounting for 16 cases. Sebaceous cyst of the scrotum was present 13 patients. Among infections and infestations, scabies was observed in 9 patients. Ariboflavinosis was seen in 9 cases. Other disorders encountered were calcinosis scrotum. Iymphangiectasia of the scrotum. Lichen simplex chronicus. Fixed drug eruption, angiokeratoma of Fordyce, lichen sclerosus et atrophicus etc. The study has been quite useful in understanding the clinical and aetiological characteristics of various types of non-veneral dermatoses in males in this subcontinen of Asia.

  20. Paneth cells: histochemical and morphometric study in control and Solanum glaucophyllum intoxicated rabbits

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    CN Zanuzzi

    2009-08-01

    Full Text Available The intestinal epithelium has a critical roll in host defence. One specialised cell type involved in this function is the Paneth cell, which secretes many substances with antimicrobial properties in response to different stimuli. Under pathological conditions, changes in the Paneth cell number, morphology and location as well as in granule number, morphology and composition have been reported. In the normal animal, 1,25-dihydroxyvitamin D3 participates in the maintenance of mineral homeostasis, immunomodulation and cell proliferation and differentiation. Solanum glaucophyllum, a calcinogenic plant containing high levels of 1,25-dihydroxyvitamin D3, is responsible for a condition known as enzootic calcinosis in ruminants, characterised by loss of body condition and mineralization of soft tissues. Using and established rabbit model, this study analyses the changes that rabbit Paneth cells undergo during intoxication with S. glaucophyllum. Male New Zealand white rabbits were experimentally intoxicated with S. glaucophyllum for 15 or 30 days. Lectin, immunohistochemical and morphometric studies were carried out on Paneth cells from samples of jejunum. SBA, DBA and WGA lectins bound to Paneth cellsgranules in both normal and intoxicated rabbits, with more heterogenity in the labelling of granules from intoxicated rabbits. Paneth cells in both groups were immunonegative for lysosyme. A time and dose-dependent increase in the size and number of Paneth cells was found in both intoxicated groups.We suggest that the changes described in these cells may be directly or indirectly induced by S. glaucophyllum intoxication.

  1. Role of radionuclide scintigraphy in the detection of parathyroid adenoma

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    Singh N

    2007-01-01

    Full Text Available Background: Preoperative detection of parathyroid adenoma is a diagnostic challenge. The sonography and computerized tomography (CT scan demonstrate high sensitivity but low specificity. The advent of radionuclide scanning technique has enhanced the specificity in this context. Aim: We undertook a study to assess the role of radionuclide scanning in suspected cases of parathyroid adenomas. Materials And Methods: Totally 28 cases were incorporated in the study. The suspicion was raised either due to raised PTH levels or recurrent calcinosis. Most of these patients had estimation of calcium done as a routine or specific investigation. The parathyroid scan was performed using either of the two techniques - Dual isotope subtraction or Sestamibi washout technique. We also used the recent approach of fusion imaging (CT + tomographic nuclear images in selected cases. Results: There were 16 true positive, 10 true negative, 1 false negative and 1 equivocal scan findings. The findings were compared with sonography, CT Scan and PTH values. The true positive yield in our study was 57%, true negative 35% and the overall sensitivity and specificity was found to be 94% and 100% respectively. Conclusion: We conclude that parathyroid scintigraphy is a reliable and sensitive technique in the preoperative detection of parathyroid adenomas and should be the first choice of imaging modality in suspicion of parathyroid adenoma.

  2. CREST Syndrome

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    Tuğçe Köksüz

    2014-06-01

    Full Text Available We report a case of CREST syndrome (calsinosis cutis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasia with all of the five major symptoms. A 46-year-old woman was admitted to our clinic with the complaint of erythema, rigidity and pain on the plantar surface of the feet. She had had Raynaud’s phenomenon for 20 years and oesophageal reflux for five years. Her face had become masklike and there was prominent telangiectasies on her face and hands. Sclerosis were confined to the fingers (sclerodactyly. Direct X-ray graphy demonstrated calcinosis cutis on the left hand and suprapatellar region. She was treated with nifedipine 30 mg/day, acetylsalicylic acid 100 mg/day for Raynaud’s phenomenon and famotidine 40 mg/day, metoclopramide HCL 30 mg/day for oesophageal dysmotility. Her complaints were partially relieved after the treatment. This case had all of the five major symptoms of CREST syndrome, and we aimed to emphasize the major symptoms and complications of CREST syndrome. (Turk J Dermatol 2012; 6: 48-50

  3. Pulsed versus continuous wave low-level light therapy on osteoarticular signs and symptoms in limited scleroderma (CREST syndrome): a case report

    Science.gov (United States)

    Barolet, Daniel

    2014-11-01

    Limited cutaneous systemic sclerosis (lcSSc) was formerly known as CREST syndrome in reference to the associated clinical features: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. The transforming growth factor beta has been identified as a major player in the pathogenic process, where low-level light therapy (LLLT) has been shown to modulate this cytokine superfamily. This case study was conducted to assess the efficacy of 940 nm using millisecond pulsing and continuous wave (CW) modes on osteoarticular signs and symptoms associated with lcSSc. The patient was treated two to three times a week for 13 weeks using a sequential pulsing mode on one elbow and a CW mode on the other. Efficacy assessments included inflammation, symptoms, pain, health scales, patient satisfaction, clinical global impression, and adverse effects monitoring. Considerable functional and morphologic improvements were observed after LLLT, with the best results seen with the pulsing mode. No adverse effects were noted. Pulsed LLLT represents a treatment alternative for osteoarticular signs and symptoms in limited scleroderma (CREST syndrome).

  4. Radiographic manifestations of Mönckeberg arteriosclerosis in the head and neck region

    Energy Technology Data Exchange (ETDEWEB)

    Tahmasbi-Arashlow, Mehrnaz; Barghan, Sevin; Kashtwari, Beeba; Nair, Madhu K. [Oral and Maxillofacial Radiology, Colleges of Dentistry and Medicine, University of Florida, Gainesville (United States)

    2016-03-15

    Mönckeberg sclerosis is a disease of unknown etiology, characterized by dystrophic calcification within the arterial tunica media of the lower extremities leading to reduced arterial compliance. Medial calcinosis does not obstruct the lumina of the arteries, and therefore does not lead to symptoms or signs of limb or organ ischemia. Mönckeberg sclerosis most commonly occurs in aged and diabetic individuals and in patients on dialysis. Mönckeberg arteriosclerosis is frequently observed in the visceral arteries, and it can occur in the head and neck region as well. This report describes a remarkable case of Mönckeberg arteriosclerosis in the head and neck region as detected on dental imaging studies. To the best of our knowledge, this is the first case that has been reported in which this condition presented in the facial vasculature. The aim of this report was to define the radiographic characteristics of Mönckeberg arteriosclerosis in an effort to assist health care providers in diagnosing and managing this condition.

  5. [Yellow oat grass intoxication in horses: Pitfalls by producing hay from extensive landscapes? A case report].

    Science.gov (United States)

    Bockisch, F; Aboling, S; Coenen, M; Vervuert, I

    2015-01-01

    Vitamin-D intoxication by yellow oat grass is often reported under the syndrome of enzootic calcinosis in ruminants in the upper regions of the Alps. The intake of Trisetum flavescens in ruminants and horses induces calcification of soft tissue, including vessels, tendons and ligaments, lung, heart and kidneys. Clinical symptoms, including a reluctance to move, inappetence, body-weight loss and impaired organ function, are frequently observed. To date, there are only a very few case reports about yellow-oat-grass intoxication in horses. The present case report describes Vitamin-D intoxication by yellow oat grass in a riding stable in Thuringia, Germany. The horses, which were fed hay with a 50% contamination of Trisetum flavescens, displayed symptoms, including inappetence, body-weight loss, colic, polydipsia and polyuria. The hay, contaminated with Trisetum flavescens, was harvested from an extensively cultivated landscape according to the European Fauna-Flora-Habitat (FFH)-directive. The present case report demonstrates the pitfalls in producing hay from extensively used landscapes and indicates some peculiarities of Vitamin-D metabolism in horses. PMID:26346225

  6. MIXED CONNECTIVE TISSUE DISORDER WITH PREGNANCY

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    Narmadha

    2015-12-01

    Full Text Available Mixed connective tissue disease (MCTD is a term involving the features of lupus systemic sclerosis, polymyositis, rheumatoid arthritis and high titre of anti ribonucleoprotein (RNP antibodies, exact etiology is not known. It is characterized by microvascular damage, immune system activation leading to inflammation and excessive deposition of collagen in the skin, lungs, heart, gastrointestinal tract and kidneys. The females are being more affected especially after childbirth attributed to the hypothesis of microchimerism, the pathogenesis being a two way migration of fetal cells through the placenta. It cannot be cured completely but treatment with corticosteroids is helpful. ACE inhibitors are useful in renal involvement and hypertension. We had a case of mixed connective tissue disorder in a patient aged 28 years with 12 weeks of gestation for medical termination of pregnancy (MTP and permanent sterilization. The complications are preeclampsia, preterm labor, fetal growth restriction, eclampsia, thrombocytopenia and infections like pneumonia, sepsis like syndrome and the maternal mortality rate is 325/100000. This is a unique case of MCTD wherein we had limited cutaneous disease like CREST-calcinosis, Raynaud’s phenomenon, esophageal involvement, sclerodactyly and telangiectasia of a lesser degree. So early diagnosis and timely intervention is advocated to prevent complications

  7. Computed tomography angiography - noninvasive tool for diagnostics of peripheral arterial occlusive disease

    International Nuclear Information System (INIS)

    Important health issue problem in the European community is the disease of the abdominal aorta and the arteries of the lower limbs. The majority (>80%) is due to the Atherosclerotic Peripheral Arterial Occlusive Disease (APAOD). In the period August 2009- February 2012, 530 patients with PAOB were diagnosed with peripheral CTA in the department of Radiology, University Hospital of Pleven, Bulgaria. The results showed that men: women ratio is approximately 3:1, the age group of 61-70 years old is of great danger for the development of the disease and the most often location of the lesions is in the femoro-popliteal segment. The most benefit advantages of the CTA compared to the other image diagnostics modalities are: non-invasiveness, short time of the examination, 2D and 3D imaging, imaging of the patency of the arterial tree and calculation of the stenosis of the lumen, visualization of the arterial wall pathologies like calcinosis and other. Key words: APAOD. Peripheral CTA. Arterial stenosis and occlusion. CTA advantages and disadvantages

  8. Adenocarcinoma of the third portion of the duodenum in a man with CREST syndrome

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    Fragulidis Georgios

    2008-10-01

    Full Text Available Abstract Background CREST (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasias syndrome has been rarely associated with other malignancies (lung, esophagus.This is the first report of a primary adenocarcinoma of the third portion of the duodenum in a patient with CREST syndrome. Case presentation A 54-year-old male patient with CREST syndrome presented with colicky postprandial pain of the upper abdomen, diminished food uptake and a 6-Kg-body weight loss during the previous 2 months. An ulcerative lesion in the third portion of the duodenum was revealed during duodenoscopy, with a diagnosis of adenocarcinoma on biopsy specimen histology. The patient underwent a partial pancreatoduodenectomy. No adjuvant therapy was instituted and follow-up is negative for local recurrence or metastases 21 months postoperatively. Conclusion CREST syndrome has been associated with colon cancer, gastric polyps, familial adenomatous polyposis (FAP syndrome and Crohn's disease; however, this is the first report of a primary adenocarcinoma of the duodenum in a patient with CREST syndrome. However, any etiologic relationship remains to be further investigated.

  9. Sterile α Motif Domain Containing 9 Is a Novel Cellular Interacting Partner to Low-Risk Type Human Papillomavirus E6 Proteins

    Science.gov (United States)

    Wang, Jia; Dupuis, Crystal; Tyring, Stephen K.; Underbrink, Michael P.

    2016-01-01

    Low-risk type human papillomavirus (HPV) 6 and 11 infection causes recurrent respiratory papillomatosis (RRP) and genital warts. RRP is the most common benign tumor of the larynx in children with frequent relapses. Repeated surgeries are often needed to improve vocal function and prevent life-threatening respiratory obstruction. Currently, there are no effective treatments available to completely eliminate these diseases, largely due to limited knowledge regarding their viral molecular pathogenesis. HPV E6 proteins contribute to cell immortalization by interacting with a variety of cellular proteins, which have been well studied for the high-risk type HPVs related to cancer progression. However, the functions of low-risk HPV E6 proteins are largely unknown. In this study, we report GST-pulldown coupled mass spectrometry analysis with low-risk HPV E6 proteins that identified sterile alpha motif domain containing 9 (SAMD9) as a novel interacting partner. We then confirmed the interaction between HPV-E6 and SAMD9 using co-immunoprecipitation, proximity ligation assay, and confocal immunofluorescence staining. The SAMD9 gene is down-regulated in a variety of neoplasms and deleteriously mutated in normophosphatemic familial tumoral calcinosis. Interestingly, SAMD9 also has antiviral functions against poxvirus. Our study adds to the limited knowledge of the molecular properties of low-risk HPVs and describes new potential functions for the low-risk HPV E6 protein. PMID:26901061

  10. Skin Findings in Renal Transplantation Patients

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    Demet Kartal

    2013-03-01

    Full Text Available Objective: It was aimed to identify skin findings those were seen in patients who undergone renal transplantation. Methods: Patients who have been followed in Erciyes University Nephrology Hospital renal transplantation outpatient clinic were included in the study. They were evaluated for dermatologic findings during routine controls. Age, gender, transplantation date, identity of organ donor, history of medications, dermatological history and dermatological findings during examination were recorded. Biopsy was performed when needed. Results: In total 94 patients, 25 female (26.6% and 69 male (73.4%, were recruited to the study. Mean age was 36±10 years. The most frequent skin finding was drug-related acne (n=20. Most common infectious disease was verruca (n=17. There were viral disease other than verruca such as herpes zoster (n=3, superficial mycosis such as onychomycosis (n=5, tinea versicolor, tinea pedis and bacterial skin disease (n=2, and paronychia (n=1 and pre-malign lesions such as actinic cheilitis and bowenoid papulosis. Besides these, stria (n=3, kserosis (n=2, cornu cutaneum, café-au-lait spots, sebaceous hyperplasia and seborrheic dermatitis, skin tag, hypertrichosis, unguis incarinatus and calcinosis were other skin findings those were seen. No malign skin lesion was observed in any of patients. Conclusion: Miscellaneous skin lesions should develop in patients those undergone renal transplantation due to long-term utilization of various immunosuppressive drugs.

  11. Systemic sclerosis: Current concepts in pathogenesis and therapeutic aspects of dermatological manifestations

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    Vishalakshi Viswanath

    2013-01-01

    Full Text Available Systemic sclerosis (SSc is a chronic, multisystem connective tissue disease with protean clinical manifestations. Recent advances in understanding the pathogenic mechanisms have led to development of target-oriented and vasomodulatory drugs which play a pivotal role in treating various dermatological manifestations. An exhaustive literature search was done using Medline, Embase, and Cochrane library to review the recent concepts regarding pathogenesis and evidence-based treatment of salient dermatological manifestations. The concept of shared genetic risk factors for the development of autoimmune diseases is seen in SSc. It is divided into fibroproliferative and inflammatory groups based on genome-wide molecular profiling. Genetic, infectious, and environmental factors play a key role; vascular injury, fibrosis, and immune activation are the chief pathogenic factors. Vitamin D deficiency has been documented in SSc and correlates with the severity of skin involvement. Skin sclerosis, Raynaud′s phenomenon (RP with digital vasculopathies, pigmentation, calcinosis, and leg ulcers affect the patient′s quality of life. Immunosuppressives, biologicals, and hematopoietic stem cell transplantation are efficacious in skin sclerosis. Endothelin A receptor antagonists, calcium-channel blockers, angiotensin receptor inhibitors, prostacyclin analogs, and phosphodiesterase type 5 (PDE-5 inhibitors are the mainstay in RP and digital vasculopathies. Pigmentation in SSc has been attributed to melanogenic potential of endothelin-1 (ET-1; the role of ET 1 antagonists and vitamin D analogs needs to be investigated. Sexual dysfunction in both male and female patients has been attributed to vasculopathy and fibrosis, wherein PDE-5 inhibitors are found to be useful. The future concepts of treating SSc may be based on the gene expression signature.

  12. Systemic sclerosis: current concepts in pathogenesis and therapeutic aspects of dermatological manifestations.

    Science.gov (United States)

    Viswanath, Vishalakshi; Phiske, Meghana M; Gopalani, Vinay V

    2013-07-01

    Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disease with protean clinical manifestations. Recent advances in understanding the pathogenic mechanisms have led to development of target-oriented and vasomodulatory drugs which play a pivotal role in treating various dermatological manifestations. An exhaustive literature search was done using Medline, Embase, and Cochrane library to review the recent concepts regarding pathogenesis and evidence-based treatment of salient dermatological manifestations. The concept of shared genetic risk factors for the development of autoimmune diseases is seen in SSc. It is divided into fibroproliferative and inflammatory groups based on genome-wide molecular profiling. Genetic, infectious, and environmental factors play a key role; vascular injury, fibrosis, and immune activation are the chief pathogenic factors. Vitamin D deficiency has been documented in SSc and correlates with the severity of skin involvement. Skin sclerosis, Raynaud's phenomenon (RP) with digital vasculopathies, pigmentation, calcinosis, and leg ulcers affect the patient's quality of life. Immunosuppressives, biologicals, and hematopoietic stem cell transplantation are efficacious in skin sclerosis. Endothelin A receptor antagonists, calcium-channel blockers, angiotensin receptor inhibitors, prostacyclin analogs, and phosphodiesterase type 5 (PDE-5) inhibitors are the mainstay in RP and digital vasculopathies. Pigmentation in SSc has been attributed to melanogenic potential of endothelin-1 (ET-1); the role of ET 1 antagonists and vitamin D analogs needs to be investigated. Sexual dysfunction in both male and female patients has been attributed to vasculopathy and fibrosis, wherein PDE-5 inhibitors are found to be useful. The future concepts of treating SSc may be based on the gene expression signature. PMID:23918994

  13. Effects of x-ray irradiation on mast cells and mastocalcergy in the connective tissue

    International Nuclear Information System (INIS)

    Experiments were performed to observe the influence of x-ray irradiation on mast cells and mastocalcergy in rats. Animals were irradiated single dose of x-ray. X-ray irradiation was applied to the whole body in doses either 100 rads or 150 rads (Cobalt-60 Teletherapy Unit). One day after irradiation the rats were injected lead acetate intravenously, followed by injection of compound 48/80 in the back subcutaneously. Animals were killed by decapitation at intervals, 1 hour, 5 hours, 1 day and 6 day after subcutaneous injection. Specimens of the abdominal and back skin were fixed in alcohol formol solution and stained with the following methods; H-E for observation of pathological changes of tissues, toluidine blue for demonstration of mast cells, von Kossa-azure A for demonstration of carbonate and phosphate, and chloranilic acid for demonstration of calcium. The following conclusions were obtained. Calciphylatic wheals are large size in the control group, medium size in 100 rads irradiation group and small size in 150 rads irradiation group. In x-ray irradiation groups the number of mast cells decreases more in the 150 rads than in the 100 rads irradiation. In the 100 rads x-ray irradiation group, histochemical study of the injection sites showed that calcium impregnated to mast cell granules and connective tissue fibers in 1 days after subcutaneous injection. The morphogenesis of this calcinosis was the same in the rat of 5 hour after subcutaneous injection of the control group. Whereas, 1 day after subcutaneous injection in 150 rads x-ray irradiation group calcium deposited more slightly than other groups

  14. Extracellular Cl(-) regulates human SO4 (2-)/anion exchanger SLC26A1 by altering pH sensitivity of anion transport.

    Science.gov (United States)

    Wu, Meng; Heneghan, John F; Vandorpe, David H; Escobar, Laura I; Wu, Bai-Lin; Alper, Seth L

    2016-08-01

    Genetic deficiency of the SLC26A1 anion exchanger in mice is known to be associated with hyposulfatemia and hyperoxaluria with nephrolithiasis, but many aspects of human SLC26A1 function remain to be explored. We report here the functional characterization of human SLC26A1, a 4,4'-diisothiocyanato-2,2'-stilbenedisulfonic acid (DIDS)-sensitive, electroneutral sodium-independent anion exchanger transporting sulfate, oxalate, bicarbonate, thiosulfate, and (with divergent properties) chloride. Human SLC26A1-mediated anion exchange differs from that of its rodent orthologs in its stimulation by alkaline pHo and inhibition by acidic pHo but not pHi and in its failure to transport glyoxylate. SLC26A1-mediated transport of sulfate and oxalate is highly dependent on allosteric activation by extracellular chloride or non-substrate anions. Extracellular chloride stimulates apparent V max of human SLC26A1-mediated sulfate uptake by conferring a 2-log decrease in sensitivity to inhibition by extracellular protons, without changing transporter affinity for extracellular sulfate. In contrast to SLC26A1-mediated sulfate transport, SLC26A1-associated chloride transport is activated by acid pHo, shows reduced sensitivity to DIDS, and exhibits cation dependence of its DIDS-insensitive component. Human SLC26A1 resembles SLC26 paralogs in its inhibition by phorbol ester activation of protein kinase C (PKC), which differs in its undiminished polypeptide abundance at or near the oocyte surface. Mutation of SLC26A1 residues corresponding to candidate anion binding site-associated residues in avian SLC26A5/prestin altered anion transport in patterns resembling those of prestin. However, rare SLC26A1 polymorphic variants from a patient with renal Fanconi Syndrome and from a patient with nephrolithiasis/calcinosis exhibited no loss-of-function phenotypes consistent with disease pathogenesis. PMID:27125215

  15. 系统性硬化病诊断及治疗指南

    Institute of Scientific and Technical Information of China (English)

    中华医学会风湿病学分会

    2011-01-01

    @@ 1概述 系统性硬化病(systemic sclerosis,SSc)是一种以皮肤变硬和增厚为主要特征的结缔组织病,女性多见,多数发病年龄在30~50岁.根据患者皮肤受累的情况将SSc分为5种亚型:①局限性皮肤型SSc(limited cutaneous SSc):皮肤增厚限于肘(膝)的远端,但可累及面部、颈部.②CREST综合征(CREST syndrome):局限性皮肤型SSc的一个亚型,表现为钙质沉着(calcinosis,C),雷诺现象(Raynaud's phenomenon,R),食管功能障碍(esophageal dysmotility,E),指端硬化(sclerodactylv,S)和毛细血管扩张(telangiectasia,T).③弥漫性皮肤型SSc (diffuse cutaneous SSc):除面部、肢体远端外,皮肤增厚还累及肢体近端和躯干.④无皮肤硬化的SSc(SSc sine scleroderma):无皮肤增厚的表现,但有雷诺现象、SSc特征性的内脏表现和血清学异常.⑤重叠综合征(overlap syndrome):弥漫或局限性皮肤型SSc与其他诊断明确的结缔组织病同时出现,包括系统性红斑狼疮、多发性肌炎/皮肌炎或类风湿关节炎.

  16. Survival and causes of death in systemic sclerosis patients: a single center registry report from Iran.

    Science.gov (United States)

    Poormoghim, Hadi; Andalib, Elham; Jalali, Arash; Ghaderi, Afshin; Ghorbannia, Ali; Mojtabavi, Nazanin

    2016-07-01

    The aims of the study were to determine prognostic factors for survival and causes of death in a cohort of patients with systemic sclerosis (SSc). This was a cohort study of SSc patients in single rheumatologic center from January 1998 to August 2012. They fulfilled the American College of Rheumatology classification criteria for SSc or had calcinosis Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia or sine sclerosis. Causes of death were classified as SSc related and non-SSc related. Kaplan-Meier and Cox proportional hazard regression models were used in univariate and multivariate analysis to analyse survival in subgroups and determine prognostic factors of survival. The study includes 220 patients (192 female, 28 male). Out of thirty-two (14.5 %) who died, seventeen (53.1 %) deaths were SSc related and in nine (28.1 %) non-SSc-related causes, and in six (18.8 %) of patients causes of death were not defined. Overall survival rate was 92.6 % (95 % CI 87.5-95.7 %) after 5 years and 82.3 % (95 % CI 73.4-88.4 %) after 10 years. Pulmonary involvement was a major SSc-related cause of death, occurred in seven (41.1 %) patients. Cardiovascular events were leading cause of in overall death (11) 34.3 % and 6 in non-SSc-related death. Independent risk factors for mortality were age >50 at diagnosis (HR 5.10) advance pulmonary fibrosis (HR 11.5), tendon friction rub at entry (HR 6.39), arthritis (HR 3.56). In this first Middle Eastern series of SSc registry, pulmonary and cardiac involvements were the leading cause of SSc-related death. PMID:27061806

  17. Radioimmunoassay (RIA), radioreceptorassay (RRA) and atomic absorption spectroscopy (AAS) applied to studies on animal nutrition and health

    International Nuclear Information System (INIS)

    In 1990, our group began working in the development of a sensitive method to measure the active principle (1,25 dihydroxy-vitamin D3-glycoside) of Solanum glaucophyllum, a plant that grows wild in our country causing calcinosis of breeding cattle. RIA and RRA have been applied to determine this glycoside in the aqueous extracts of the plant leaves and the free vitamin D metabolite in animal plasma samples, respectively. AAS was also used to determine calcium, together with phosphorus determined by colorimetric methods, in blood and tissues of experimental animals in order to study the relationship between the active principle kinetics and its effects. More recently, this plant has been proposed as a source of vitamin D activity (VDA) that might contribute with environment care improving calcium and phosphorus utilization by animals. Our group is by now, as a first step, studying the effects of different diet levels of calcium (Ca) and phosphorus (P) [covering the range between commercial recommendations and half of NRC requirements (1994)], as well as different sources of those minerals, upon productive, nutritional, skeletal and biochemical parameters, in a series of experiments covering either a part or the entire breeding cycle of broilers. We think that the high levels of vitamin D3 employed in commercial farms (4 times NRC recommendations) could enable birds fed on basal diets to enhance the synthesis of the active metabolite of the vitamin in order to overcome partially these minerals deficiency. These methods of analysis have been applied successfully in our research projects contributing to the improvement of animal health and production and our approach has been considered adequate for the study of this additive and therefore has been required by the private industry of foreign countries. (author)

  18. Vitamin D Metabolism in Experimental Animals: Kinetics of Solanum glaucophyllum Active Principle in Cows and Assessment of Calcium, Phosphorus and Vitamin D3 Requirements in Broilers

    International Nuclear Information System (INIS)

    In 1990 our group began working on the development of a sensitive method to measure the active principle (1,25 dihydroxy-vitamin D3-glycoside) of Solanum glaucophyllum, a plant which grows wild in Argentina and causes calcinosis in breeding cattle. A radioreceptor assay (RRA) was applied to measure the free vitamin D metabolite in the plasma of experimental cows that were fed the plant in order to study the kinetics of the active principle. The 1,25 dihydroxyvitamin D concentration in plasma showed a 33-fold increase four h post treatment. Peak levels were recorded 12 h after dosing, decreased by half between 24-36 h and continued declining until 48 h. More recently, this plant has been proposed as a source of vitamin D activity (VDA) and thereby may contribute to improving Ca and P utilisation by animals and environmental care. The effects of different dietary levels of calcium (Ca) and phosphorus (P) over the range between commercial recommendations (control) and two thirds of NRC requirements (basal) as well as different sources of those minerals were therefore studied in experiments covering either a part or the entire breeding cycle of broilers through measurements of productive, nutritional, skeletal and biochemical parameters. Results indicated that birds fed diets deficient in these minerals exhibited skeletal responses but nevertheless showed better productive responses than those fed control diets. The high levels of vitamin D3 employed in commercial farms (25 times NRC recommendations) could enable birds fed on deficient diets to increase synthesis of the active metabolite of the vitamin in order to partially overcome deficiencies in these minerals. On the other hand, such high levels of vitamin D3 might have been unbalanced for optimal efficiency, at least under the experimental farm conditions of the present work. (author)

  19. Biochemical and histopathological studies on the influence of aqueous extract of fenugreek seed (Trigonella foenum graecum on alloxan diabetic male rats

    Directory of Open Access Journals (Sweden)

    Enas A. M. Khalil

    2004-06-01

    increase in serum total cholesterol and triglycerides accompanied by degenerative changes in aorta and formation of medial calcinosis in some cases, significant decrease in serum testosterone levels associated by germ cells depletion as well as sloughing and degeneration of sperms. In conclusion, fenugreek seeds exhibited antioxidant property could ameliorate the alternations induced in diabetes. This investigation recommended that higher concentrations of debitterized fenugreek seeds may double the regeneration of beta ­cells in pancreas, further studies would be done in mammals. Fenugreek seed aqueous extract exhibited antioxidant property which ameliorated the biochemical and histopathological alternation induced by alloxan. In conclusion, higher concentrations of debitterized fenugreek seeds can regenerate beta cells in pancreas; further studies would be done in mammals.

  20. Mucocutaneous and demographic features of systemic sclerosis: A profile of 46 patients from Eastern India

    Directory of Open Access Journals (Sweden)

    Sudip Kumar Ghosh

    2012-01-01

    Full Text Available Background: Systemic sclerosis (SSc is a multisystem connective tissue disorder of uncertain etiology. The clinical picture is frequently dominated by prominent cutaneous manifestations that have diagnostic and prognostic significance. The objective of the present study was to find out the demographic profile and the relative frequencies and characteristics of different mucocutaneous features of SSc in a group of patients from eastern India. In addition, we sought to compare the frequency and pattern of the findings in the limited versus the diffuse variety of the disease. Materials and Methods:This was a cross-sectional, clinical observational study. Consecutive patients of SSc attending the dermatology O.P.D. of a tertiary care hospital of eastern India over 3 years were enrolled to the present study. Results:A total of 46 patients (41 females and 5 males; mean age 29.6±12.3 years of SSc were evaluated. Among mucocutaneous manifestations Raynaud′s phenomenon was present in 39 (84.8% patients. Other cutaneous features included dyspigmentation (40, 86.9%, sclerodactyly (38, 82.6%, inability to open the mouth (38,82.6%, mat-like telangiectasia (11,23.1%, fingertip ulceration and scarring (29,63%, cutaneous calcinosis (1,2.2%, digital gangrene in (2,4.3%, generalized pruritus (4,8.7%, cutaneous small vessel vasculitis (2,4.3%, chronic urticaria (2,4.3%, flexion contractures of the fingers (13,28.3%, and amputation of the digits (3,6.5%. Mucosal changes were observed in 10 (21.7% patients and nail changes were seen in 13 (28.2% patients. Diffuse cutaneous SSc was noted in 27 (58.7% patients and limited cutaneous SSc was seen in the remainder. Thirty-six (78.2% patients tested positive for ANA. Conclusion: The present study provides a snapshot of the spectrum of the demographic and mucocutaneous manifestations of SSc in the eastern Indian population. We have not observed any statistically significant differences between dcSSc and lcSSc in terms

  1. Linhas escleróticas metafisárias em crianças e adolescentes em uso de alendronato Sclerotic metaphyseal lines in children and adolescents treated with alendronate

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    Érika C.C. Silva

    2010-06-01

    Full Text Available INTRODUÇÃO: Os bisfosfonatos inibem a reabsorção óssea pela interferência na ação dos osteoclastos. Dentre os efeitos adversos, as linhas escleróticas em metáfise de ossos longos são descritas como principal alteração radiográfica na faixa etária pediátrica. OBJETIVO: Avaliar a frequência de alterações radiográficas causadas pelo alendronato utilizado em crianças e adolescentes com baixa densidade óssea ou calcinose. PACIENTES E MÉTODOS: Foi realizado um estudo do tipo coorte retrospectiva analisando-se prontuários de 21 pacientes que fizeram uso de alendronato semanal por no mínimo 10 meses. Os pacientes realizaram radiografias de ossos longos antes do início do alendronato e aproximadamente um ano após o seu uso. RESULTADOS: Onze pacientes (52,3% apresentaram linhas escleróticas em metáfise dos ossos longos. A localização mais frequente foi em tíbia (8/11 pacientes, seguida de fêmur (7/11, úmero (6/11, rádio (4/11, ulna (3/11 e fíbula (2/11. Nenhum paciente apresentou regress��o das alterações radiográficas durante o tempo de evolução (até 1,1 ano após a suspensão do alendronato. CONCLUSÃO: Se usado com critério, o alendronato é seguro e as alterações radiográficas não mostraram ter um significado mais importante.INTRODUCTION: Bisphosphonates inhibit bone resorption by interfering with the action of osteoclasts. Among the adverse effects, sclerotic lines observed in the metaphysis of long bones have been described as the main imaging finding in pediatric patients. OBJECTIVE: To evaluate the frequency of radiographic changes caused by alendronate in children and adolescents with low bone density or calcinosis. PATIENTS AND METHODS: We conducted a cross-sectional study with 21 patients who were treated with once-weekly alendronate for at least 10 months. Patients underwent x-rays of long bones before the start of alendronate and approximately one year after its use. RESULTS: Eleven patients