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Sample records for angiomatosis

  1. Cystic angiomatosis: case report and review of the literature

    International Nuclear Information System (INIS)

    Levey, D.S.; MacCormack, L.M.; Sartoris, D.J.; Haghighi, P.; Resnick, D.; Thorne, R.

    1996-01-01

    The objective of this article was to offer a better characterization of the typical clinical presentation, radiologic findings, histology, treatment approaches, and differential diagnosis of cystic angiomatosis, a rare condition of which previous reports have been confusing because of unclear diagnostic criteria, different classifications, and variations in terminology. A case report using the improved imaging techniques of computed tomography scanning is presented in additin to an analysis and review of the previous literature, which relied heavily on plain film radiography, biopsy, and necropsy for diagnosis. A case report of a 26-year-old man initially symtomatic at age 12 is presented. Although a rare condition, cystic angiomatosis must be considered in pediatric and young adult patients presenting with diffuse, multifocal, cystic skeletal lesions, with or without visceral involvement. (orig.)

  2. Cystic angiomatosis with splenic involvement: unusual MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Vanhoenacker, F.M. [Dept. of Radiology, Univ. Hospital Antwerp, Edegem (Belgium); Dept. of Radiology, AZ St-Maarten, Campus Duffel, Duffel (Belgium); Schepper, A.M. [Dept. of Radiology, Univ. Hospital Antwerp, Edegem (Belgium); Raeve, H. [Dept. of Pathology, Univ. Hospital Antwerp, Edegem (Belgium); Berneman, Z. [Dept. of Hematology, Univ. Hospital Antwerp, Edegem (Belgium)

    2003-12-01

    Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with longstanding bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient. (orig.)

  3. Cystic angiomatosis with splenic involvement: unusual MRI findings

    International Nuclear Information System (INIS)

    Vanhoenacker, F.M.; Schepper, A.M.; Raeve, H.; Berneman, Z.

    2003-01-01

    Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with longstanding bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient. (orig.)

  4. Bacillary angiomatosis in HIV-positive patient from Northeastern Brazil: a case report

    Directory of Open Access Journals (Sweden)

    Renata Félix da Justa

    2011-10-01

    Full Text Available It is a report of disseminated bacillary angiomatosis (BA in a 23-year-old female patient, who is HIV-positive and with fever, weight loss, hepatomegaly, ascites, and papular-nodular skin lesions. The clinical and diagnostic aspects involved in the case were discussed. Bacillary angiomatosis must always be considered in the diagnosis of febrile cutaneous manifestations in AIDS.

  5. Angiomatosis cutanea: a próposito de un caso en un gato

    OpenAIRE

    Ortuñez, A.

    2010-01-01

    La angiomatosis cutánea es una lesión progresiva y proliferativa del tejido vascular que afecta a la dermis y al tejido subcutáneo. Se conocen muy pocos casos de angiomatosis cutánea en animales y en esta comunicación se describe el primer caso observado en España en un gato adulto.

  6. A case of bacillary angiomatosis developed at a burn site

    Directory of Open Access Journals (Sweden)

    Ayse Albayrak

    2012-01-01

    Full Text Available Bacillary Angiomatosis (BA is frequently seen in patients with human immunodeficiency virus (HIV-induced immunodeficiency. Our patient was a case that developed granuloma-like lesions in the area of a burn, 8 days after being burnt on the upper right arm by scalding water. No indication of immune deficiency was observed and no history of direct contact with cats was evident. By the sixth day of the patient′s admission to our clinic, some of the lesions had reached a diameter of 2.5 cm. An excision biopsy was carried out from the lesions present on the patient. Electron microscopy revealed solitary bacilli located close to the capillary wall. Oral erythromycin treatment was implemented at 250 mg, 4 times a day for 2.5 months. Within this period of treatment, the lesions regressed completely, and a complete cure was achieved. This case demonstrates that BA must be considered in the differential diagnosis of both HIV-infected and immunocompetent patients.

  7. Systemic Cystic Angiomatosis Mimicking Metastatic Cancer: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Vivek Kumar

    2017-01-01

    Full Text Available Systemic cystic angiomatosis is a rare benign disorder due to the maldeveloped vascular and lymphatic system with less than 50 cases reported in literature so far. We report here a case of systemic cystic angiomatosis (SCA with multisystem involvement affecting the neck, thyroid, thoracic cavity, and skeletal system. The patient initially presented in her 4th decade of life with isolated lymphangioma in the neck requiring surgery. However, she experienced full-blown manifestations of SCA in her 6th decade which closely mimicked metastatic cancer. The diagnosis of SCA could only be established after multiple biopsies. The radiological and histological features of SCA with its course over 31 years in this patient have been described.

  8. Bacillary angiomatosis: description of 13 cases reported in five reference centers for AIDS treatment in Rio de Janeiro, Brazil

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    GAZINEO Jorge L. D.

    2001-01-01

    Full Text Available The aim of this case series was to describe the clinical, laboratory and epidemiological characteristics and the presentation of bacillary angiomatosis cases (and/or parenchymal bacillary peliosis that were identified in five public hospitals of Rio de Janeiro state between 1990 and 1997; these cases were compared with those previously described in the medical literature. Thirteen case-patients were enrolled in the study; the median age was 39 years and all patients were male. All patients were human immunodeficiency virus type 1 (HIV-1 infected and they had previous or concomitant HIV-associated opportunistic infections or malignancies diagnosed at the time bacillary angiomatosis was diagnosed. Median T4 helper lymphocyte counts of patients was 96 cells per mm³. Cutaneous involvement was the most common clinical manifestation of bacillary angiomatosis in this study. Clinical remission following appropriate treatment was more common in our case series than that reported in the medical literature, while the incidence of relapse was similar. The frequency of bacillary angiomatosis in HIV patients calculated from two of the hospitals included in our study was 1.42 cases per 1000 patients, similar to the frequencies reported in the medical literature. Bacillary angiomatosis is an unusual opportunistic pathogen in our setting.

  9. Bacillary angiomatosis

    African Journals Online (AJOL)

    The patient was a 28~year~0Id white homosexual man who had been confirmed as HIV~positive by means of enzyme-linked immunosorbent assay (ELISA) and. Western blot in July 1991. He had never travelled over- seas. In August 1992 he complained of a cough produc- tive of yellow sputum, and pneumonia was ...

  10. An unusual case of bacillary angiomatosis in the oral cavity of an AIDS patient who had no concomitant tegumentary lesions – case report and review

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    Walter de Araujo Eyer-Silva

    2017-08-01

    Full Text Available ABSTRACT Bacillary angiomatosis (BA is an angioproliferative disease of immunocompromised patients that usually presents as vascular tumors in the skin and subcutaneous tissues. It is caused by chronic infections with either Bartonella henselae or B. quintana. Oral cavity BA is exceedingly rare and even rarer without simultaneous cutaneous disease. We report herein the case of a 51-year-old HIV-infected man who presented severe odynophagia and an eroded lesion on the hard palate that progressed to an oronasal fistula. No cutaneous lesions were recorded. Doxycycline led to complete resolution. To the best of our knowledge, only six previous cases of oral BA without tegumentary disease have been previously reported and none of them progressed to fistula.

  11. Angiomatose bacilar: revisão da literatura e documentação iconográfica Bacillary angiomatosis: literature review and iconographic documentation

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    Paulo Eduardo Neves Ferreira Velho

    2003-10-01

    Full Text Available A angiomatose bacilar é uma das bartoneloses humanas. É doença sistêmica de manifestação cutânea freqüente. Caracteriza-se por lesões angioproliferativas causadas pela Bartonella henselae e pela B. quintana, que comprometem especialmente indivíduos imunodeficientes. Deve ser bem conhecida pelos dermatologistas, pois é potencialmente fatal, mas responsiva à antibioterapia. Para estabelecer o diagnóstico nosológico eles deverão sugeri-lo ao anatomopatologista, facilitando a diferenciação histológica, sobretudo, com o granuloma piogênico e o sarcoma de Kaposi. O presente artigo apresenta revisão da literatura e fotografias clínicas, de microscopia de luz e eletrônica de transmissão.Bacillary angiomatosis is one of the human bartoneloses. It is a systemic disease usually affecting the skin. Its angioproliferative lesions are caused by B. henselae and by B. quintana. Such lesions are more frequent in immunodeficient hosts. Dermatologists must be well aware of this disease because it is potentially fatal, though it is treatable with antibiotic therapy. To establish a nosologic diagnosis, dermatologists should refer patients to a pathologist. In so doing, they will facilitate histologic differentiation especially from pyogenic granuloma and Kaposi's sarcoma. This paper presents a literature review of the disease, and includes clinical, light and transmission electron microscope photographs.

  12. Successful wireless capsule endoscopy for a 2.5-year-old child: obscure gastrointestinal bleeding from mixed, juvenile, capillary hemangioma-angiomatosis of the jejunum.

    Science.gov (United States)

    Kavin, Hymie; Berman, James; Martin, Thomas L; Feldman, Ansley; Forsey-Koukol, Kimberly

    2006-02-01

    The lesion responsible for obscure gastrointestinal bleeding in the pediatric population may not be determined with standard primary endoscopic methods. Wireless capsule endoscopy, now a first-line modality for evaluation of the small bowel in the adult population, is a tool that may be useful among children. We report a case of a 2.5-year-old girl who presented with melenic stools. Upper and lower endoscopy, Meckel scans, and mesenteric angiography yielded negative results. Wireless capsule endoscopy identified numerous abnormal, dilated, blood vessels in the proximal jejunum, with associated fresh blood. The patient underwent surgical exploration, with resection of the affected portion of the jejunum. Pathologically, the dilated blood vessels were consistent with mixed, juvenile, capillary hemangioma-angiomatosis of developmental or congenital origin. The patient fared well postoperatively, with no additional bleeding in 9 months of follow-up monitoring. This case report highlights the use of capsule endoscopy in the diagnosis and successful treatment of gastrointestinal bleeding in a young infant. This is the youngest reported patient treated with the use of wireless capsule endoscopy in the pediatric population.

  13. Encephalofacial angiomatosis (Sturge-Weber syndrome): report of three cases.

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    Simonini, Vincenzina Mazzeo; Lodi, Laura

    2012-12-01

    The purpose is to show and discuss the findings in three cases of Sturge-Weber syndrome. One adult and two children were examined in the last three years. The girl was scanned after diode laser treatment for her right eye glaucoma. Ultrasonographic examinations were performed with 20- and 25-MHz probes (nominal frequencies) for posterior pole and 35-MHz probe (closed system, HiScan Optikon 2000, Rome, Italy) for anterior segment. All cases were characterized by choroid thickening due to capillary hemangioma. The thickening was not regular and the use of 25-MHz probe proved superior in visualizing the two layers (choroid vs. sclera), showing a striking reflectivity difference between them. All cases also showed enlarged vortex veins and some anomalous vessels adjacent to the external surface of the sclera, i.e. posterior episcleral dilated vessels. In one case only, very anomalous tortuous vessels were found in the orbit. Disk cupping and/ or pseudo cupping was always present in all cases; in the oldest patient only, who had a thinner choroid, it was possible to display a concave bottom of the cup instead of the straight, small, highly reflective segment representing the cribrosa. Serous retinal and choroid detachments were present as temporary complications after glaucoma treatment. In conclusion, choroid, facial and encephalon involvement in Sturge-Weber syndrome is widely described in ocular oncology books, whereas by far less attention is paid to orbital involvement. Anterior dilated episcleral vessels are visible and till now the posterior vessel involvement has only been demonstrated in some pathology specimens.

  14. Meningio- angiomatosis — case report and subject review

    African Journals Online (AJOL)

    Enrique

    showed multiple areas of hyperinten- sity in the left parietal lobe extending in a linear radial fashion along a num- ber of gyni and sulci over the surface of the brain (Fig. 1a). Moderate con- trast enhancement was seen adjacent to some of these hyperintensities (Fig. 1b). The hyperintense areas appeared denser than blood.

  15. Bacillary angiomatosis: A rare finding in the setting of antiretroviral ...

    African Journals Online (AJOL)

    eases in humans.[1] The two most commonly associated with HIV are B. quintana and. B. henselae.[1,2,5] Transmission of Bartonella to humans occurs via a cat scratch that is contaminated with Bartonella-infected fleas.[1,2,5,6] The prevalence of Bartonella in. HIV-positive persons is reported to be very. CASE REPORT.

  16. Mediastinal nodal angiomatosis - an unusual mass lesion in the superior mediastinum

    International Nuclear Information System (INIS)

    Steinke, Karin; Sanadgol, Babak; Singh, Mahendra

    2011-01-01

    Full text: A 54-year-old male, lifelong non-smoker, presented to the authors' hospital with a 5-day history of fever and productive cough, not improving on oral amoxicillin. His past medical history was unremarkable. On examina tion, he was febrile, tachycardic and tachypneic, and had right basal crackles on auscultation. Initial blood tests revealed a leucocytosis with increased neutrophils and normal lymphocytes. C-reactive protein was elevated at 312 mg/L 5). The coagulation profile was unremarkable. The initial chest X-ray (CXR) showed areas of opacifi cation in the right lower lobe and a right paratracheal mediastinal bulging (Fig, 1). A previous CXR from 6 years before, unrelated to this recent presentation, was normal. Further evaluation by contrast-enhanced chest CT (SIEMENS Sensation 16 (Siemens, Erlangen, Germany); slice thickness 3 mm) showed a well-defined heteroge neous 4.0 x 4.8 cm mass lesion in the superior mediast num with densities of 60-70 HU, separate from vessels, the esophagus or the trachea (Fig. 2a) along with bilateral lower lobe patchy confluent pulmonary infiltrates (not shown), more conspicuous on the right. The patient's hospital stay was complicated by a non-ST elevation myocardial infarction, which precluded him from having a transbronchial biopsy of the mediastinal mass. The patient's serum tested positive for mycoplasma pneumo niae antibodies with a titre of 640 40); the pneumonia was successfully treated with a course of oral doxycycline administered over 14 days, the symptoms fully resolving. He was eventually discharged, having recovered from the pneumonia and from the myocardial infarction. A subs quent CT scan performed 3 months later showed persis tence of the mediastinal mass with no significant changes in size or density (Fig. 2b); the bilateral pneumonic infil trates have fully resolved. The ensuing endobronchial ultrasound (EBUS)-guided needle aspiration with biopsy of the mediastinal mass produced a sample of macrophages and erythrocytes, but no malignant cells. Still without a clear diagnosis, MRI (SIEMENS Avanto, 1.5 T, MAGNEVIST contrast, 10 mL) was performed. The mass appeared as a hyper intense lesion on T2 weighted imaging (Fig. 3a) and as a solid well-defined heterogenous enhancing tumour with prolonged pooling of contrast material on dynamic T1 acquisition (Fig, 3b-e). Finally, the lesion was thoracoscopically excised, given the presence of an almost 5-cm mediastinal mass with vascular supply. Histology showed florid.

  17. Osseous involvement in AIDS patients

    International Nuclear Information System (INIS)

    Marchiori, Edson; Pereira, Abercio Arantes

    1995-01-01

    The radiological findings of eight patients with the acquired immunodeficiency syndrome (AIDS) who developed bone lesions were analyzed in conjunction with twelve similar published cases. Our series included three patients with lymphoma, two with bacillary angiomatosis, two with tuberculosis and one with staphylococcal osteomyelitis. All the lesions were lithic regardless of the etiology. Both in our cases and in those previously published bone repair was only seen in cases of bacillary angiomatosis treated with erythromycin. No pathognomonic findings were observed. However, the association of skin and bone lesions in immuno deficient patients should always bring the consideration of bacillary angiomatosis in the differential diagnosis. This is particularly relevant since this a condition amenable to treatment once correctly identified. The radiological findings in the lymphoma and tuberculosis patients have not been described previously. (author). 9 refs., 7 figs., 3 tabs

  18. The Janus face of Bartonella quintana recognition by Toll-like receptors (TLRs): a review.

    NARCIS (Netherlands)

    Matera, G.; Liberto, M.C.; Joosten, L.A.B.; Vinci, M.; Quirino, A.; Pulicari, M.C.; Kullberg, B.J.; Meer, J.W.M. van der; Netea, M.G.; Foca, A.

    2008-01-01

    Bartonella quintana (B. quintana) is a facultative, intracellular bacterium, which causes trench fever, chronic bacteraemia and bacillary angiomatosis. Little is known about the recognition of B. quintana by the innate immune system. In this review, we address the impact of Toll-like receptors

  19. Case Report - Isolated splenic peliosis in an immunocompromised ...

    African Journals Online (AJOL)

    Isolated splenic peliosis is an even more unusual phenomenon. Patients with AIDS may develop peliosis in association with bacillary angiomatosis. This is due to secondary infection with Bartonella henselae or a similar organism, Rochalimaea henselae. Case presentation: A 45-year-old HIV-positive man on antiretroviral ...

  20. Oral and extraoral manifestationse of sturge-Weber syndrome: A rare finding

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    Amitandra K Tripathi

    2016-01-01

    Full Text Available Sturge-Weber syndrome (SWS is a rare congenital developmental disorder characterized by unilateral cutaneous vascular malformation (nevus flammeus or port-wine stains in association with ipsilateral leptomeningeal angiomatosis and glaucoma. This article presents a case of SWS associated with gingival hyperplasia and pyogenic granuloma.

  1. Oral and extraoral manifestationse of sturge-Weber syndrome: A rare finding

    OpenAIRE

    Amitandra K Tripathi; Mohammad Arif Khan; Krishna Deo; Ranjan Mani Tripathi

    2016-01-01

    Sturge-Weber syndrome (SWS) is a rare congenital developmental disorder characterized by unilateral cutaneous vascular malformation (nevus flammeus or port-wine stains) in association with ipsilateral leptomeningeal angiomatosis and glaucoma. This article presents a case of SWS associated with gingival hyperplasia and pyogenic granuloma.

  2. Sturge-Weber syndrome

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    Natarajan Manivannan

    2012-01-01

    Full Text Available Encephalotrigeminal angiomatosis (Sturge-Weber syndrome is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face, and sometimes the skull, jaws, and oral soft tissues. A case of portwine stain with intraoral gingival hemangioma is presented. There were no other systemic manifestations. Patient reported with a complaint of localized tumor-like swelling in gums. Based on the presence of sharply demarcated vascular lesion unilaterally on the face and with ipsilateral intraoral vascular hyperplasia in the lip and gingiva, a variant of encephalotrigeminal angiomatosis was diagnosed. Ultrasound Doppler flowmetry was used to determine the blood flow. Dental management included plaque control instructions, scaling, root planning, and excision of the lesion done under general anesthesia. Close follow-up and meticulous plaque control have kept the oral condition under fairly good control.

  3. Sturge-Weber disease: Diagnostic imaging and neuropathology. Die Sturge-Weber-Erkrankung: Bildgebende Diagnostik in bezug zur Neuropathologie

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    Henkes, H.; Huber, G.; Piepgras, U. (Universitaet des Saarlandes, Homburg/Saar (Germany, F.R.). Abt. fuer Neuroradiologie); Bittner, R. (Freie Univ. Berlin (Germany, F.R.). Strahlenklinik und Poliklinik); Sperner, J. (Freie Univ. Berlin (Germany, F.R.). Abt. fuer Neuropaediatrie); Heye, N. (Freie Univ. Berlin (Germany, F.R.). Inst. fuer Neuropathologie); Bassir, C. (Freie Univ. Berlin (Germany, F.R.). Abt. fuer Paediatrische Radiologie)

    1991-06-01

    Clinical presentation of a child with port-wine stain and seizures leads to the suspicion of Sturge-Weber disease (SWD). This diagnosis can be confirmed by the detection of a meningeal angiomatosis. In rare cases, early detection of meningeal pathology by ultrasound has been reported. Key findings are brain atrophy, gyriform cortical calcifications demonstrated by skull radiographs after the first year of life or earlier by cranial CT, and dys- or aplasia of the deep cerebral veins on angiography. Radionuclide imaging shows focal or diffuse tracer accumulation over the affected brain regions. MR demonstrates an abnormal appearance of the affected meninges, especially thickening and pathologically increased signal intensity after Gd-DTPA application. This, in association with the demonstration of abnormal enhancement in deep medullary veins, is the most characteristic finding. Contrast-enhanced MR allows early and non-invasive diagnosis of SWD, mainly by revealing leptomeningeal angiomatosis and abnormal venous vessels. (orig.).

  4. Sturge-Weber Syndrome: A Review.

    Science.gov (United States)

    Higueros, E; Roe, E; Granell, E; Baselga, E

    2017-06-01

    Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder caused by a somatic activating mutation in GNAQ; it affects 1 in every 20,000 to 50,000 newborns. It is characterized by a facial Port-wine stain, leptomeningeal angiomatosis, and glaucoma. Seizures are the most common neurological manifestation and typically present in the first months of life. Glaucoma may be present at birth or develop later. Neuroimaging studies show leptomeningeal angiomatosis, supporting diagnosis. Standard treatment for Sturge-Weber syndrome includes laser treatment for the Port-wine stain, anticonvulsants, and medical or surgical treatment for the glaucoma. Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma. Copyright © 2016 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  5. Report of a Rare Case of Gorham-Stout Disease of Both Shoulders: Bisphosphonate Treatment and Shoulder Replacement

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    Eike Garbers

    2011-01-01

    Full Text Available Massive osteolysis known as Gorham-Stout disease is a rare idiopathic disorder typically affecting long bones in a unifocal pattern. Angiomatosis is strongly connected to the osteolysis. Weather angiomatosis is the cause or the result of osteolysis is subject of intense discussion (Kawasaki et al. (2003, Möller et al. (1999, Radhakrishnan and Rockson (2008. There are about 200 cases described since 1955. Our patient is a 77-year-old female patient with osteolyses of both shoulders involving the proximal humerus, lateral clavicle, and the glenoid. Under bisphosphonate therapy, the progressive osteolysis stopped on the right side and showed progression on the left. With the patient complaining about severe rest pain and impaired function, we performed surgical reconstruction by implantation of total shoulder prosthesis three months after onset of symptoms. Our case shows a possibility of primary and early surgical reconstruction with good clinical outcome.

  6. Primary bone tumours in infants

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    Kozlowski, K.; Beluffi, G.; Cohen, D.H.; Padovani, J.; Tamaela, L.; Azouz, M.; Bale, P.; Martin, H.C.; Nayanar, V.V.; Arico, M.

    1985-09-01

    Ten cases of primary bone tumours in infants (1 osteosarcoma, 3 Ewing's sarcoma, 1 chondroblastoma and 5 angiomastosis) are reported. All cases of angiomatosis showed characteristic radiographic findings. In all the other tumours the X-ray appearances were different from those usually seen in older children and adolescents. In the auhtors' opinion the precise diagnosis of malignant bone tumours in infancy is very difficult as no characteristic X-ray features are present in this age period.

  7. STURGE WEBER SYNDROME: A CASE REPORT WITH CLINICAL AND RADIOLOGICAL FEATURES

    OpenAIRE

    Puneet; Shifa; Jai; Pawan; Hemant Kumar

    2014-01-01

    : Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma (cerebral malformations and tumors). It is characterized by abnormal blood vessels on the brain surface. Normally, only one side of the brain is affected. We report a case of 1...

  8. Pyogenic Granuloma in a Patient of Sturge-Weber Syndrome with Bilateral Port Wine Stain- A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Shantala Arunkumar

    2014-07-01

    Full Text Available Sturge-Weber syndrome (SWS also known as encephalotrigeminal angiomatosis. It is a neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. An ipsilateral or bilateral facial cutaneous vascular malformation Port Wine Stain (PWS usually affects the upper face. Other clinical manifestations are seizures, glaucoma, hemiparesis, mental retardation and delayed developmental milestones. The main objective of this case report is to unravel such a rarest syndrome with bilateral port-wine stain, which has intraoral manifestation of pyogenic granuloma involving gingiva in an 11 year old boy.

  9. A clinician′s dilemma: Sturge-Weber syndrome ′without facial nevus′!!

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    Sujit A Jagtap

    2013-01-01

    Full Text Available Sturge-Weber syndrome (SWS is a rare, sporadic neurocutaneous syndrome characterized by a classical triad of facial port wine nevus, ipsilateral leptomeningeal angiomatosis (LAM and glaucoma. The incidence of SWS is 1/50,000 live births, although it is more often underreported. The incidence of SWS without facial nevus is not known, although very few patients without facial nevus have been reported. In these patients, the diagnosis of SWS is made by the findings of computed tomography, magnetic resonance imaging, and histopathology. Here, we report three patients with SWS from our cohort of 28 patients with SWS without facial nevus and discuss their clinical profile and outcome.

  10. Unusual cause of lower extremity wounds: Cobb syndrome.

    Science.gov (United States)

    Abtahi-Naeini, Bahareh; Saffaei, Ali; Pourazizi, Mohsen

    2016-10-01

    Cobb syndrome (Cutaneomeningospinal Angiomatosis) is a rare segmental neurocutaneous syndrome associated with metameric cutaneous and spinal cord arteriovenous malformations (AVMs). In this syndrome, capillary malformation or angiokeratoma-like lesions are formed in a dermatomal distribution, with an AVM in the corresponding segment of the spinal cord. The spinal cord lesions can cause neurological disorder and paraplegia, which typically develop during young adulthood. We report a 32-year-old male with the Cobb syndrome associated with lower extremity painful wounds and acute-onset paraplegia due to metameric vascular malformations. © 2015 Medicalhelplines.com Inc and John Wiley & Sons Ltd.

  11. What do we (not know about the human bartonelloses?

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    Velho Paulo Eduardo Neves Ferreira

    2003-01-01

    Full Text Available The human bartonelloses are a group of diseases with a rapidly increasing clinical spectrum. Well known manifestations such as Carrion's disease, trench fever, cat-scratch disease, and bacillary angiomatosis are examples of Bartonella spp. infection. Along with these diseases, recurrent bacteremia, endocarditis, septicemia, erythema nodosum, erythema multiforme, trombocytopenic purpura and other syndromes have been reported having been caused by bacteria of this genus. The infectious process and the pathogenesis of these microorganisms are poorly understood. The bartonelloses may have a benign and self-limited evolution in a host, or a potentially fatal one. These bacteria can provoke a granulomatous or an angioproliferative histopathologic response. As these diseases are not yet well defined, we have reviewed the four main human bartonelloses and have examined unclear points about these emergent diseases.

  12. Sturge Weber Syndrome: review of literature with case illustration

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    Satyarthee Guru Dutta

    2017-03-01

    Full Text Available Sturge-Weber syndrome (SWS also called as encephalotrigeminal angiomatosis, is a sporadically occurring rare neuro-cutaneous syndrome, characterized by vascular malformation with capillary venous angiomas involving face, choroidal layer of eye globe and leptomeninges responsible for ophthamological as well as neurological signs and symptoms. Authors report an interesting case, a six year old girl, who presented with seizures, facial port wine stain and normal psychomotor development. CT scan showed left cerebral hemiatrophy, left frontal and parieto occipital calcification with cortical calcification in left high frontal convexity. Cranial MRI scan also confirmed finding of left cerebral hemiatrophy and also revealed presence of gyriform cortical calcification, prominent flow voids seen in left basal ganglia. Her seizure is well controlled with antiepileptic medication. The pertinent literature is reviewed and management of such cases is discussed briefly.

  13. Sturge -Weber Syndrome - Three Classic variants

    Directory of Open Access Journals (Sweden)

    R S Sathawane

    2006-01-01

    Full Text Available Sturge-Weber syndrome (SWS, also known as encephalotrigeminal angiomatosis, a sporadic, non-familial, congenital disorder consists of congenital hamartomatous malformations that may affect the eye, skin and central nervous system at different times. Sturge-Weber syndrome is classified as 1 Complete trisymptomatic: - when all three organ systems i.e. eye, skin and CNS are involved 2 Incomplete bisymptomatic:- when the involvement is either oculocutaneous or neurocutaneous, and 3 Incomplete monosymptomatic: when there is only neural or cutaneous involvement. Failure of proper vascular development is believed to be the most likely cause of this condition. The malformed blood vessels or hemangiomas may lead to port-wine stain, epilepsy and glaucoma depending on its location. Three classic variants with typical findings are discussed.

  14. Bartonella henselae: subversion of vascular endothelial cell functions by translocated bacterial effector proteins.

    Science.gov (United States)

    Pulliainen, Arto Tapio; Dehio, Christoph

    2009-03-01

    Bartonella henselae (Bh) is a worldwide distributed zoonotic pathogen. Depending on the immune status of the infected individual this bacterium can cause a wide spectrum of clinical manifestations, ranging from cat scratch disease (CSD) to bacillary angiomatosis (BA) and bacillary peliosis (BP). BA and BP are characterized by tumor-like lesions at the skin or in the inner organs, respectively. These structures display pathological sprouting of capillaries with enlarged and hyperproliferated vascular endothelial cells (ECs) that are frequently found in close association with bacteria. Here we review the cellular changes observed upon Bh infection of ECs in vitro and outline the role of the VirB type IV secretion system (T4SS) and its translocated effector proteins in the modulation of EC signalling cascades. The current model how this virulence system could contribute to the vasoproliferative activity of Bh is described.

  15. Síndrome de Sturge weber. Revisión

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    Otman Fernández Concepción

    1999-09-01

    Full Text Available La angiomatosis encefalotrigeminal (síndrome de Sturge-Weber es el más frecuente de los síndromes neurocutáneos con predominio de anomalías vasculares. Es una enfermedad congénita, que aparece de manera esporádica y en su forma completa consiste en la asociación de angiomas cerebral, cutáneo y ocular que se caracterizan clínicamente por una mancha color vino en la cara, epilepsia, retraso mental, déficits neurológicos (hemiparesia y hemianopsia y glaucoma. Para el diagnóstico de la enfermedad resultan de mucha utilidad los estudios de tomografía axial computadorizada y resonancia magnética nuclear craneales, así como para estudiar la relación del angioma leptomeníngeo u atrofia subyacente, importantes para el pronóstico y la conducta médica que se debe seguir. El control de las crisis epilépticas constituye un elemento capital para una evolución favorable de los pacientes. El tratamiento quirúrgico debe ser reservado para enfermos de corta edad, con afectación unilateral exclusiva, con epilepsia refractaria y sin retraso mental profundo; mediante la resección del área afectada, lobectomía o hemisferectomía, siempre que el otro hemisferio esté intacto. Se profundiza en los aspectos clínicos, diagnósticos, patogénicos y terapéuticos de esta enfermedadThe encephalotrigeminal angiomatosis (Sterge-Weber syndrome is the commonest of the neurocutaneous syndromes with predominance of vascular anomalies. Its complete form consists in the association of cerebral, cutaneous and ocular angiomas, which are characterized by a wine-clored stain, epilepsy, mental retardation, neurological deficits (hemiparesis and hemianopsia and glaucoma. CAT and cranial magnetic resonance imaging are very useful for diagnosing the disease and for studying the extension of the leptomeningeal angioma or underlying atrophy. These studies are also important for the prognosis and medical conduct to be followed. The control of the epileptic seizures

  16. Distinct activities of Bartonella henselae type IV secretion effector proteins modulate capillary-like sprout formation.

    Science.gov (United States)

    Scheidegger, F; Ellner, Y; Guye, P; Rhomberg, T A; Weber, H; Augustin, H G; Dehio, C

    2009-07-01

    The zoonotic pathogen Bartonella henselae (Bh) can lead to vasoproliferative tumour lesions in the skin and inner organs known as bacillary angiomatosis and bacillary peliosis. The knowledge on the molecular and cellular mechanisms involved in this pathogen-triggered angiogenic process is confined by the lack of a suitable animal model and a physiologically relevant cell culture model of angiogenesis. Here we employed a three-dimensional in vitro angiogenesis assay of collagen gel-embedded endothelial cell (EC) spheroids to study the angiogenic properties of Bh. Spheroids generated from Bh-infected ECs displayed a high capacity to form sprouts, which represent capillary-like projections into the collagen gel. The VirB/VirD4 type IV secretion system and a subset of its translocated Bartonella effector proteins (Beps) were found to profoundly modulate this Bh-induced sprouting activity. BepA, known to protect ECs from apoptosis, strongly promoted sprout formation. In contrast, BepG, triggering cytoskeletal rearrangements, potently inhibited sprouting. Hence, the here established in vitro model of Bartonella- induced angiogenesis revealed distinct and opposing activities of type IV secretion system effector proteins, which together with a VirB/VirD4-independent effect may control the angiogenic activity of Bh during chronic infection of the vasculature.

  17. Strategy for identification & characterization of Bartonella henselae with conventional & molecular methods

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    Kavita Diddi

    2013-01-01

    Full Text Available Background & objectives: Bartonella henselae is a fastidious gram-negative bacterium usually causing self limiting infections in immunocompetent individuals but often causes potentially life threatening infection, such as bacillary angiomatosis in immunocompromised patients. Both diagnosis of infections and research into molecular mechanisms of pathogenesis have been hindered by lack of appropriate and reliable diagnostic techniques. We undertook this study to standardize methods to characterize B. henselae in clinical samples to diagnose Bartonella infection correctly. Methods: B. henselae ATCC 49882 strain was procured from American type culture collection, USA. This strain was revived and maintained in the laboratory, and identification and characterization of this strain was done by conventional and molecular techniques, which included culture on various media, staining by different methods including electron microscopy, biochemical analysis by conventional methods and API, polymerase chain reaction (PCR for amplification of citrate synthase gene followed by restriction fragment length polymorphism (RFLP. Results: This organism was biochemically inert due to slow growth and generated unique identification code with API. The amplification of the citrate-synthase gene with primers yielded a 381 bp product followed by specific RFLP profile for B. henselae. Interpretation & conclusions: Bartonella is fastidious and fragile organism and should be handled carefully. Extra effort and careful observation are required to isolate and characterize this organism.

  18. The role of red blood cell scintigraphy in the multiple-modality imaging diagnosis of a rare case of diffuse hepatic hemangiomatosis in an adult

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    Ernesto Cason

    2013-03-01

    Full Text Available Introduction: Angiomas are one of the most common primary tumors of the liver. Diffuse hepatic angiomatosis, however, is quite rare and usually observed in pediatric patients. We report a rare case of diffuse hepatic hemangiomatosis in a 33-year-old woman. Case report: The patient presented with abdominal pain and a palpable upper abdominal mass. Abdominal CT and magnetic resonance imaging (MRI findings suggested diffuse hepatic hemangiomatosis, but this finding was not confirmed by subsequent contrast-enhanced abdominal ultrasonography (US. The patient then underwent single photon emission computed tomography (SPECT/CT scintigraphy with Tc-99m-labeling of red blood cells (RBC. This examination revealed increased uptake of labeled erythrocytes in several of the hepatic lesions corresponding to CT and RM findings, thereby confirming the clinical hypothesis of diffuse hepatic hemangiomatosis. Discussion: RBC scintigraphy with SPECT/CT can facilitate the comparison of other crosssectional imaging methods such as CT and MRI. This case highlights the importance of a multiple-modality approach in the imaging diagnosis of this condition.

  19. Musculoskeletal disorders associated with HIV infection and AIDS. Part I: Infectious musculoskeletal conditions

    International Nuclear Information System (INIS)

    Tehranzadeh, Jamshid; Ter-Oganesyan, Ramon R.; Steinbach, Lynne S.

    2004-01-01

    The musculoskeletal system can be affected by a variety of abnormalities in association with human immunodeficiency virus (HIV) infection. Although not as common as complications involving other organ systems, such as the pulmonary and the central nervous systems, HIV-associated musculoskeletal disorders are sometimes the initial presentation of the viral illness. Knowledge of the existence and the characteristic appearance of the conditions affecting bone, joint, and muscle in HIV-infected patients is valuable to radiologists for diagnosis and to clinicians for detection and appropriate treatment. We reviewed recent literature to provide a comprehensive assessment of the HIV-associated musculoskeletal disorders, and present radiologic examples from our own collection. This article is divided into two parts. In the first part we review the infectious musculoskeletal disorders associated with HIV illness and AIDS, including cellulitis, abscesses, pyomyositis, septic bursitis, septic arthritis, osteomyelitis, and bacillary angiomatosis. We also present a comprehensive spectrum of mycobacterial infections, consisting of tuberculous spondylitis and spondylodiskitis, arthritis, osteomyelitis, and tenosynovitis, as well as infections caused by atypical mycobacteria. Part II of this review will concentrate on non-infectious musculoskeletal conditions, including rheumatic disorders and neoplasms. (orig.)

  20. Musculoskeletal disorders associated with HIV infection and AIDS. Part I: Infectious musculoskeletal conditions

    Energy Technology Data Exchange (ETDEWEB)

    Tehranzadeh, Jamshid [Department of Radiological Sciences, University of California, Irvine, Irvine, California (United States); Department of Radiological Sciences, Rt. 140, 101 The City Drive ZC 5005, CA 92868-3298, Orange (United States); Ter-Oganesyan, Ramon R. [College of Medicine, University of California, Irvine, Irvine, California (United States); Steinbach, Lynne S. [Department of Radiological Sciences, University of California, San Francisco, San Francisco, California (United States)

    2004-05-01

    The musculoskeletal system can be affected by a variety of abnormalities in association with human immunodeficiency virus (HIV) infection. Although not as common as complications involving other organ systems, such as the pulmonary and the central nervous systems, HIV-associated musculoskeletal disorders are sometimes the initial presentation of the viral illness. Knowledge of the existence and the characteristic appearance of the conditions affecting bone, joint, and muscle in HIV-infected patients is valuable to radiologists for diagnosis and to clinicians for detection and appropriate treatment. We reviewed recent literature to provide a comprehensive assessment of the HIV-associated musculoskeletal disorders, and present radiologic examples from our own collection. This article is divided into two parts. In the first part we review the infectious musculoskeletal disorders associated with HIV illness and AIDS, including cellulitis, abscesses, pyomyositis, septic bursitis, septic arthritis, osteomyelitis, and bacillary angiomatosis. We also present a comprehensive spectrum of mycobacterial infections, consisting of tuberculous spondylitis and spondylodiskitis, arthritis, osteomyelitis, and tenosynovitis, as well as infections caused by atypical mycobacteria. Part II of this review will concentrate on non-infectious musculoskeletal conditions, including rheumatic disorders and neoplasms. (orig.)

  1. Orofacial manifestation of Sturge-Weber syndrome: A case report with review

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    Mandeep Kaur

    2012-01-01

    Full Text Available Sturge-Weber syndrome (SWS, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses which is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by proliferation of arteries of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. Normally, only one side of the head is affected and mainly results from the errors in both the mesodermal and ectodermal development. Here, we report a rare case of SWS having oral and facial manifestations, where an 8-year-old boy had actually come for treatment of swollen gums, and on examination, we found that he had typical signs and symptoms of this rare developmental anomaly. The main purpose of this publication is to make the clinician aware of the oral manifestations that are seen in the patients with SWS and also to lay stress on the importance of radiographs in the diagnosis of this syndrome.

  2. Diagnostic dilemma: Sturge-Weber syndrome, without facial nevus

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    Paresh Zanzmera

    2015-01-01

    Full Text Available Sturge-Weber syndrome (SWS, a rare sporadic neurocutaneous disease, is characterized by a congenital unilateral port-wine nevus affecting the area innervated by V1, ipsilateral leptomeningeal angiomatosis, and calcification in the occipital or frontoparietal region and glaucoma/vascular eye abnormality. Three types of SWS have been described in literature: Type I (classic demonstrates facial and leptomeningeal angioma, often with glaucoma; type II has facial angioma and glaucoma, with no evidence of intracranial lesions; and type III (rarest presents with only leptomeningeal angioma. Only a few cases of type III SWS have been reported. Here, we report a case of a seven-year-old boy with focal complex partial seizure, who was diagnosed with SWS without facial nevus. Recognition of this type of SWS is important, as our patient had been misdiagnosed and received inappropriate antiepileptic drugs for six years. We suggest that in the appropriate clinical scenario, the diagnosis of SWS without facial nevus should be considered before labelling idiopathic or cryptogenic localization-related epilepsy, and gadolinium-enhanced magnetic resonance imaging (MRI should be done in clinically suspicious cases of SWS, without facial nevus.

  3. Oral Kaposi's sarcoma: a 10-year retrospective histopathologic study.

    Science.gov (United States)

    Regezi, J A; MacPhail, L A; Daniels, T E; Greenspan, J S; Greenspan, D; Dodd, C L; Lozada-Nur, F; Heinic, G S; Chinn, H; Silverman, S

    1993-08-01

    Microscopic diagnosis of early Kaposi's sarcoma continues to be a challenge to the pathologist, as does the identification of bacillary angiomatosis (BA) which may have a similar appearance. 120 oral Kaposi's sarcoma (KS) biopsies submitted to the UCSF oral pathology service from 1981-1991 were reviewed in order to describe the clinical-pathologic spectrum of these lesions and to search for unrecognized cases of BA. Also, histopathologic features of oral KS were compared to 30 oral pyogenic granulomas, and immunohistochemical stains for endothelium-associated CD34 antigen were done. The diagnosis of KS was confirmed in all biopsies and no cases of BA were found. Histologically, the KS specimens exhibited numerous features that separated them from pyogenic granulomas, and could themselves be divided into two clinical-pathologic subtypes: small, well-delineated macular lesions (31), which were characterized by inconspicuous patches of spindle cells containing ill-defined vascular spaces; and larger, infiltrative nodular lesions (89), which were characterized by spindle cells lining vascular slits and bizarre-shaped vessels. Extravasated RBCs were evident in almost all KS lesions; hemosiderin deposits and hyaline globules were seen in half of each of the small and large lesions. Nuclear atypia was minimal and mitotic activity was slight. Lymphocytes in small lesions added to the difficulty of microscopic interpretation of these incipient lesions. CD34 was expressed on all spindle cells lining vascular spaces in larger lesions and on spindle cells of small, subtle lesions. We conclude that within the spectrum of lesions that are diagnosed as oral KS, two clinical-pathologic types can be identified: macular small spindle-cell lesions and nodular infiltrative vascular lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

  4. [Hereditary pheochromocytoma--a family affected by von Hippel-Lindau disease].

    Science.gov (United States)

    Mertl, J; Rovný, A; Krepelová, A; Prásek, J

    2003-07-01

    The authors present a case of a 37 year old male (proband) with a 13 year history of progressive sight impairment leading to blindness and a 4 year history of a mild hypertension. He was incidentally found to have large adrenal tumors after an ultrasound kidney examination. The tumors were confirmed with CT scan and magnetic resonance imaging. A bilateral pheochromocytoma was biochemically demonstrated and successfully removed. The eye diagnosis of angiomatosis retinae von Hippel-Lindau was ascertained after a search of the patient files in other medical departments, which led to a family screening. Proband's brother, having hypertension and a history of a cerebellar astrocytoma operation, was also diagnosed with CT scan to have a bilateral pheochromocytoma. Unfortunately, at the same time he was found to have a large irremovable neuroendocrine pancreatic carcinoma, which caused complications and his eventual death. Both proband and his brother were affected by the same CGG(Arg167)->CAG(Gln) mutation in the exon 3 of the VHL gene. Other living and examined family members were not affected, which was confirmed by negative genetic testing. One year after the pheochromocytoma operation, proband was diagnosed to have a retroperitoneal tumor left to the aorta, clinically silent, with slightly and non-constantly elevated urine norepinephrine and normetanephrine. Metaiodobenzylguanidine scintigraphy showed that it was a paraganglioma. The old CT and magnetic resonance picture review demonstrated that the tumor had already been present at the time of the operation. It was surgically removed and histologically verified. It is a pity that proband had not been sent by his ophthalmologist for an endocrine examination when the eye diagnosis was determined. Affection of the family would have been discovered earlier, and proband's brother might have possibly been saved.

  5. Kaposiform hemangioendothelioma causing intestinal obstruction

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    Bassel Salman

    2018-01-01

    Full Text Available A previously healthy toddler with bilious vomiting and erythematous gluteal rash over 2 weeks had intermittent pain, constipation and decreased appetite. All labs were negative with the exception of fecal occult blood. Abdominal x-ray and ultrasound revealed dilated air-filled loops of bowel and partial small bowel obstruction. After persistent worsening abdominal pain and vomiting a CT scan with IV contrast (Fig. 1 suggested small bowel obstruction. Emergent surgery was performed and diagnostic laparoscopy revealed about 61 cm of necrotic bowel causing stricture formation and mesenteric shortening in the distal small bowel. 56 cm of inflamed bowel was resected with end-to-end anastomosis. Final pathology report indicated diffuse intestinal angiomatosis with transmural involvement and focal erosion consistent with KHE (Fig. 2. Presentation is varied, consists of cutaneous lesion, retroperitoneal mass, intestinal obstruction, jaundice, intussusception, or multifocal neoplasms. Complete surgical resection with wide margins is the best therapeutic option and has achieved the best outcomes. If not treated in sufficient time, KHE has a relatively high mortality rate of 30%, with most deaths occurring due to its locally invasive effects [5]. There are limited reports of identifying features of KHE on imaging. Of 165 cases of KHE none were presented in the small bowel [5]. We report the unique case of KHE presenting as a hypervascular mass causing obstruction in the distal small bowel. Although extremely rare, KHE should be considered as a reason for severe GI stricture or obstruction in infants and children in obscure cases and included in the differential.

  6. Bartonella henselae trimeric autotransporter adhesin BadA expression interferes with effector translocation by the VirB/D4 type IV secretion system.

    Science.gov (United States)

    Lu, Yun-Yueh; Franz, Bettina; Truttmann, Matthias C; Riess, Tanja; Gay-Fraret, Jérémie; Faustmann, Marco; Kempf, Volkhard A J; Dehio, Christoph

    2013-05-01

    The Gram-negative, zoonotic pathogen Bartonella henselae is the aetiological agent of cat scratch disease, bacillary angiomatosis and peliosis hepatis in humans. Two pathogenicity factors of B. henselae - each displaying multiple functions in host cell interaction - have been characterized in greater detail: the trimeric autotransporter Bartonella adhesin A (BadA) and the type IV secretion system VirB/D4 (VirB/D4 T4SS). BadA mediates, e.g. binding to fibronectin (Fn), adherence to endothelial cells (ECs) and secretion of vascular endothelial growth factor (VEGF). VirB/D4 translocates several Bartonella effector proteins (Beps) into the cytoplasm of infected ECs, resulting, e.g. in uptake of bacterial aggregates via the invasome structure, inhibition of apoptosis and activation of a proangiogenic phenotype. Despite this knowledge of the individual activities of BadA or VirB/D4 it is unknown whether these major virulence factors affect each other in their specific activities. In this study, expression and function of BadA and VirB/D4 were analysed in a variety of clinical B. henselae isolates. Data revealed that most isolates have lost expression of either BadA or VirB/D4 during in vitro passages. However, the phenotypic effects of coexpression of both virulence factors was studied in one clinical isolate that was found to stably coexpress BadA and VirB/D4, as well as by ectopic expression of BadA in a strain expressing VirB/D4 but not BadA. BadA, which forms a dense layer on the bacterial surface, negatively affected VirB/D4-dependent Bep translocation and invasome formation by likely preventing close contact between the bacterial cell envelope and the host cell membrane. In contrast, BadA-dependent Fn binding, adhesion to ECs and VEGF secretion were not affected by a functional VirB/D4 T4SS. The obtained data imply that the essential virulence factors BadA and VirB/D4 are likely differentially expressed during different stages of the infection cycle of

  7. Bartonella henselae bacteremia in a mother and son potentially associated with tick exposure.

    Science.gov (United States)

    Maggi, Ricardo G; Ericson, Marna; Mascarelli, Patricia E; Bradley, Julie M; Breitschwerdt, Edward B

    2013-04-15

    Bartonella henselae is a zoonotic, alpha Proteobacterium, historically associated with cat scratch disease (CSD), but more recently associated with persistent bacteremia, fever of unknown origin, arthritic and neurological disorders, and bacillary angiomatosis, and peliosis hepatis in immunocompromised patients. A family from the Netherlands contacted our laboratory requesting to be included in a research study (NCSU-IRB#1960), designed to characterize Bartonella spp. bacteremia in people with extensive arthropod or animal exposure. All four family members had been exposed to tick bites in Zeeland, southwestern Netherlands. The mother and son were exhibiting symptoms including fatigue, headaches, memory loss, disorientation, peripheral neuropathic pain, striae (son only), and loss of coordination, whereas the father and daughter were healthy. Each family member was tested for serological evidence of Bartonella exposure using B. vinsonii subsp. berkhoffii genotypes I-III, B. henselae and B. koehlerae indirect fluorescent antibody assays and for bacteremia using the BAPGM enrichment blood culture platform. The mother was seroreactive to multiple Bartonella spp. antigens and bacteremia was confirmed by PCR amplification of B. henselae DNA from blood, and from a BAPGM blood agar plate subculture isolate. The son was not seroreactive to any Bartonella sp. antigen, but B. henselae DNA was amplified from several blood and serum samples, from BAPGM enrichment blood culture, and from a cutaneous striae biopsy. The father and daughter were seronegative to all Bartonella spp. antigens, and negative for Bartonella DNA amplification. Historically, persistent B. henselae bacteremia was not thought to occur in immunocompetent humans. To our knowledge, this study provides preliminary evidence supporting the possibility of persistent B. henselae bacteremia in immunocompetent persons from Europe. Cat or flea contact was considered an unlikely source of transmission and the mother, a

  8. La Pediatría 1989 – 1990

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    Alberto Hernández Saenz

    1990-12-01

    afirman que la circuncisión evita la IU, la Academia Americana de Pediatría vuelve a recomendarla, así vuelve a oscilar el péndulo, antes se negaba …

    Se demuestra que la cocaína consumida durante el embarazo produce: abruptio placenta, vasoconstricción y aborto espontáneo.

    El recién nacido tiene malformaciones urinarias y microcefalia.

    Los adictos a la cocaína pueden padecer convulsiones refractarias a los fármacos habituales, la droga de elección es el Midazolam (Versed o Dormicum.

    El síndrome CHARGE se encuentra familiarizado en la literatura médica actual, su “sigla” es como sigue: coloboma, “Heart” porque hay cardiopatías, atresia de coanas, retardo del crecimiento , genitales hipoplásicos, “ears” por malformaciones auriculares, todo acoI1 pañado de una severa dificultad para la deglución.

    El síndrome de Kasabach-Merritt, angiomatosis hepática, se trata con corticoides, dipiramidol o ciclofosfamida.

    En el grupo de las Carbapenems, el Imipenem asociado, con Cilistatin, parece ser el antibiótico actual de mayor cobertura, su uso en pacientes seleccionados …

    Las quinolonas se depositan en los núcleos de crecimiento, de ahí su uso restringido en pediatría …

    La Mupirocina tópica tiene un efecto igualo superior a la Eritromicina en el tratamiento del impétigo …

    El Parvovirus 8-19, agente etiológico del eritema infeccioso o quinta enfermedad, puede producir depranositosis, es contagiosa 7-10 días antes de la aparición del exantema, la infección contraída durante el embarazo puede producir hidrops fetalis…

    Los investigadores coinciden en afirmar que el herpes virus 6, es causante de la roséola infantum (exantema súbito…

  9. Selected Abstracts of the 6th International Congress of UENPS; Valencia (Spain; November 23rd-25th 2016; Session “Brain and development”

    Directory of Open Access Journals (Sweden)

    --- Various Authors

    2016-11-01

    . Piersigilli, I. Savarese, P. Giliberti, A. DottaABS 32. THERAPEUTIC HYPOTHERMIA IN ASPHYXIATED NEONATES: EXPERIENCE FROM NEONATAL INTENSIVE CARE UNIT OF UNIVERSITY HOSPITAL OF MARRAKESH • F.Z. Elalouani, N. Idrissi Slitine, M. Elbaz, S. Elfaiq, G. Boufrioua, M. Barkane, F.M.R. MaoulainineABS 33. FIRST EXPERIENCES IN THERAPEUTIC HYPOTHERMIA TREATMENT OF HIE IN THE PERIOD JANUARY 2011-DECEMBER 2011 IN NICU AT UNIVERSITY CLINICAL CENTER SARAJEVO • A. Cengic, R. Spahovic, I. Kalkan, S. Tanovic, A. KadicABS 34. THE EFFECT OF PHOTOTHERAPY ON THE ELECTROCORTICAL BRAIN ACTIVITY IN TERM NEWBORNS MEASURED BY AMPLITUDE-INTEGRATED EEG – PILOT STUDY • Z. Zimová, T. Jurko, K. Maťašová, M. ZibolenABS 35. FINLA DISEASE (FIBROSIS, NEURODEGENERATION AND LEPTOMENINGEAL ANGIOMATOSIS CAUSED BY DELETERIOUS MUTATIONS IN THE NHLRC2 GENE • J. Uusimaa, R. Kaarteenaho, T. Paakkola, H. Tuominen, M.K. Karjalainen, J. Nadaf, T. Varilo, M. Suo-Palosaari, I. Pietilä, A. Hiltunen, L. Ruddock, H. Alanen, E. Biterova, A. Salminen, I. Miinalainen, M. HallmanABS 36. HYDROCORTISONE SUPPLEMENTATION IN CIRCULATORY FAILURE OF COOLED ASPHYXIATED NEWBORNS • K. Kovacs, E. Szakmar, A. Cseko, U. Meder, M. Szabo, A. JermendyABS 37. INHALATIVE CO2 FOR PREVENTING HYPOCAPNIA IN NEWBORNS WITH HYPOXIC-ISCHEMIC ENCEPHALOPATHY • E. Szakmar, A. Jermendy, K. Kovac, U. Meder, C. Andorka, K. Kaila, M. SzaboABS 38. FLOPPY INFANTS: REPORT OF THREE CLINICAL CASES • M. Rodrigues, M. Costa, J. Teixeira, C. Sá, L. Pinheiro, E. Abreu, A. Silva, N. Silva, M. Marques, M. Rocha, A. PereiraABS 39. PRONE VERSUS SUPINE POSITION FOR CEREBRAL REGIONAL TISSUE OXYGENATION IN PRETERM NEONATES UNDERGOING NON-INVASIVE VENTILATION • T. Barsan Kaya, O. Aydemir, N. Tekin