WorldWideScience

Sample records for angiomatosis

  1. Encephalotrigeminal angiomatosis

    Directory of Open Access Journals (Sweden)

    Kotyal B Mahendrappa

    2013-01-01

    Full Text Available Encephalotrigeminal angiomatosis, more commonly known as Sturge-Weber syndrome (SWS, is a rare disorder occurring sporadically with a frequency of 1:50,000. It is characterized by facial nevus, seizures, hemiparesis, intracranial calcification, and mental retardation. We report here a young infant presenting with port-wine stain of one side of the face and transient hemiparesis (stroke-like episode of the opposite side, seizures, and intracranial calcification, suggesting the diagnosis of SWS. The child reported here presented with a stroke-like event at a relatively young age, which is unusual. The baby was given supportive therapy.

  2. Mediastinal angiomatosis: a rare case report

    OpenAIRE

    Pak, Min Gyoung; Choi, Phil Jo; Choi, Won Suk; Kang, Eun Ju; Roh, Mee Sook

    2015-01-01

    Angiomatosis refers to a rare condition of large hamartomatous vascular lesions that extensively affect a region of the body or several different tissue types in a contiguous way. Several cases have been reported in the mediastinum. We experienced a histologically proven case of mediastinal angiomatosis in a 56-year-old woman that was incidentally detected as multiple conglomerated masses mimicking metastatic lymph nodes on chest radiography. Despite its rareness, our case emphasizes that pat...

  3. Pulmonary angiomatosis and hemangioma in common dolphins (Delphinus delphis) stranded in Canary Islands.

    Science.gov (United States)

    Díaz-Delgado, Josué; Arbelo, Manuel; Sacchini, Simona; Quesada-Canales, Óscar; Andrada, Marisa; Rivero, Miguel; Fernández, Antonio

    2012-08-01

    Vascular tumors and disorders, like angiomatosis, are rarely described in cetacean species. A retrospective histological study was carried out on lung samples from 35 common dolphins (Delphinus delphis) stranded in the Canary Islands coasts looking for morphological vascular changes and likely related causes. Twenty-five out of thirty-five (71%) common dolphins showed focal or multifocal angiomatosis-like lesions. A high association between this type of vascular proliferation and parasitic infestation was observed. In addition, a single pulmonary cavernous hemangioma not previously reported in common dolphins is presented.

  4. 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.

  5. [Bacillary angiomatosis in an adult infected with HIV-1 at an early stage of immunodepression in Abidjan, Côte d'Ivoire].

    Science.gov (United States)

    Minga, K A; Gberi, I; Boka, M B; Gourvellec, G; Abo, Y; Dohoun, L; Abe, H; Ekra, D; Bonard, D; Danel, C; Huet, C; Salamon, R; Bondurand, A; N'Dri-Yoman, T; Anglaret, X

    2002-03-01

    Human immunodeficiency virus (HIV)-associated bacillary angiomatosis has rarely been described in Africa. We report here the first case in Côte d'Ivoire. Although in industrialised countries bacillary angiomatosis has been described in patients with low CD4 count, this episode occurred in the first year following HIV-seroconversion in an adult patient with more than 500 CD4 cells per cubic millimetre. Symptoms rapidly and totally disappeared under erythromycin treatment, although with a relapse two years after the end of the first episode. In Africa where people living with HIV often present chronic cutaneous lesions, bacillary angiomatosis may be under-diagnosed. Bacillary angiomatosis must be systematically considered in face of lesions similar to Kaposi's sarcoma. Improving knowledge on symptoms of bacillary angiomatosis in Africa should lead to better treatment and a better estimation of its true frequency which may be underestimated.

  6. Diffuse Dermal Angiomatosis: A Clue to the Diagnosis of Atherosclerotic Vascular Disease

    Directory of Open Access Journals (Sweden)

    Tueboon Sriphojanart

    2015-05-01

    Full Text Available Diffuse dermal angiomatosis (DDA is a benign, acquired, reactive vascular proliferation. DDA is clinically characterized by painful purpuric plaque with central ulceration. The histopathologic hallmark is diffuse proliferation of endothelial cells that are arranged interstitially between collagen bundles of the reticular dermis. DDA has been reported in association with peripheral atherosclerotic disease, arteriovenous fistula and heavy smoking. We report the case of a 49-year-old Asian male with DDA who presented with a painful stellate-shaped purpuric patch on the right thigh. Histopathologic examination showed proliferation of CD34-positive spindle cells in the dermis. Our patient underwent vascular bypass surgery along with tight control of cardiovascular risk factors, which yielded successful results.

  7. Bacillary Angiomatosis in an HIV-positive Man with Multiple Risk Factors: A Clinical and Epidemiological Puzzle

    Directory of Open Access Journals (Sweden)

    Farrah J Mateen

    2005-01-01

    Full Text Available A 36-year-old man with advanced AIDS presented with skin lesions, fever and weight loss. Physical examination revealed skin lesions suggestive of bacillary angiomatosis. Diagnostic imaging identified bone lesions, soft tissue masses and a single hepatic lesion. His clinical presentation was most consistent with Bartonella quintana; however, his risk for infection with B quintana was limited to a remote, brief period of homelessness. In contrast, his frequent contact with cats presented substantial potential exposure to Bartonella henselae. Diagnosing a Bartonella infection presents challenges. The detection of bacteremia is hindered by slow growth in BACTEC media (BD Diagnostic Systems, Canada. In the present case, recovery of the organism required blind subcultures after at least eight days of incubation. Speciation by 16s ribosomal RNA analysis confirmed B quintana as the pathogen. Because the patient presented with skin lesions, the diagnosis was straightforward; however, skin lesions are nondiagnostic in some cases. Microbiological confirmation of the diagnosis rests on sufficient clinical suspicion to apply labour-intensive laboratory protocols.

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

    International Nuclear Information System (INIS)

    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.

  9. Sturge-Weber syndrome: a case report and review of literatures

    Institute of Scientific and Technical Information of China (English)

    ZHOU Jing; LI Nan-yun; ZHOU Xiao-jun; WANG Jian-dong; MA Heng-hui; ZHANG Ru-song

    2010-01-01

    @@ Sturge-Weber syndrome (SWS), or encephalotrigeminal angiomatosis, is a rare, congenital neurocutaneous syndrome characterized by unilateral facial cutaneous vascular malformation (nevus flammeus or port-wine stain) in association with ipsilateral leptomeningeal angiomatosis.

  10. Blue rubber bleb nevus syndrome: a report of one case associated with recurrent epistaxis

    Institute of Scientific and Technical Information of China (English)

    LIU Qiong; CHEN Yi-peng; LI You-ming

    2007-01-01

    @@ Blue rubber bleb nevus syndrome (BRBNS), or Bean's syndrome, is a rare angiomatosis characterized by distinctive cutaneous and gastrointestinal venous malformations that usually cause massive or occult gastrointestinal hemorrhage and iron deficiency anemia secondary to the bleeding episodes.

  11. Bartonellosis

    OpenAIRE

    Guptill, Lynn

    2010-01-01

    International audience Bartonellosis is a constellation of clinical conditions affecting human beings and a variety of animals. Many infections are zoonotic, with some of the most commonly reported zoonotic manifestations of infection including cat scratch disease, bacillary angiomatosis, endocarditis, and neuroretinitis. Companion animals serve as reservoirs for several zoonotic species of , and may also serve as sentinels for zoonotic species harbored by wildlife.

  12. Periodontal manifestations and treatment of Sturge-Weber syndrome--report of two cases.

    Science.gov (United States)

    Huang, J S; Chen, C C; Wu, Y M; Ho, K Y; Wang, C C; Ho, Y P; Liu, C S; Wang, Y P

    1997-02-01

    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. Two patients came to the Kaohsiung Medical College Hospital's dental clinic with complaints of localized gingival enlargement or tumor-like swelling. Based on the presence of facial nevus flammeus, examinations of angiography, radiological evidence of calcific densities, and ipsilaterally intraoral vascular hyperplasia in the lip, cheek and gingiva, encephalotrigeminal angiomatosis was diagnosed. Dental management included plaque control instructions, scaling, root planing and periodontal surgery. Recurrence of gingival enlargement in both cases was noted, so periodontal surgery was performed a second time. Close follow up and complete plaque control have kept the periodontal condition fairly well under control in these two cases. We introduce the oral manifestations and the experience of treatments in these two cases.

  13. Sturge-Weber syndrome

    Directory of Open Access Journals (Sweden)

    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.

  14. Anaesthetic Management of Cataract Surgery in a Patient with Sturge-Weber Syndrome

    Directory of Open Access Journals (Sweden)

    Vasantha Kumar

    2015-05-01

    Full Text Available Sturge-Weber Syndrome (SWS, also called as encephalo trigeminal angiomatosis, is a rare congenital syndrome, characterized by lepto meningeal haemangioma, a facial port-wine stains distributed over the trigeminal nerve area, (usually involving one side and glaucoma. During cataract surgery, there may be rupture of choroid haemangioma, leading to excessive bleeding, or of haemangioma involving the airway, leading to difficult mask ventilation, laryngoscopy and intubation. We discuss the anaesthetic management of the patient with SWS for cataract surgery.

  15. Pestilence, persistence and pathogenicity: infection strategies of Bartonella

    OpenAIRE

    Minnick, Michael F.; Battisti, James M.

    2009-01-01

    It has been nearly two decades since the discovery of Bartonella as an agent of bacillary angiomatosis in AIDS patients and persistent bacteremia and ‘nonculturable’ endocarditis in homeless people. Since that time, the number of Bartonella species identified has increased from one to 24, and 10 of these bacteria are associated with human disease. Although Bartonella is the only genus that infects human erythrocytes and triggers pathological angiogenesis in the vascular bed, the group remains...

  16. Medical imaging findings in Cobb syndrome: two case reports

    Institute of Scientific and Technical Information of China (English)

    WANG Guang-bin; XU Lei; ZHAO Bin; CAI Shi-feng; SHI Hao; LI Hui-hua; QU Lei

    2005-01-01

    @@ Cobb syndrome, also known as cutaneomeningospinal angiomatosis, is a rare clinical entity characterized by the combination of a vascular skin nevus and an angioma in the spinal canal at the same metamere.1 It was first described by Berenbruch in 1890 and did not receive recognition until Cobb's description2 in 1915. Only few documents about this disease had been reported.1 We present two cases here and review the other reported cases.

  17. Divry-Van Bogaert syndrome in a female: relationship to Sneddon's syndrome and radiographic appearances

    International Nuclear Information System (INIS)

    A 28-year-old woman presented with generalised livedo reticularis, dementia, epilepsy, and pyramidal and extrapyramidal signs. Multiple focal infarcts were seen on MRI. Angiography demonstrated widespread cerebromeningeal angiomatosis with multiple small and medium size arterial occlusions. A lifelong personal and family history of mental handicap in the absence of anticardiolipin antibodies suggests Divry-Van Bogaert syndrome, not previously been reported in a female. Similarities to Sneddon's syndrome are discussed. (orig.)

  18. Primary bone tumours in infants

    Energy Technology Data Exchange (ETDEWEB)

    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.

  19. Sturge–Weber syndrome

    OpenAIRE

    Manivannan, Natarajan; Gokulanathan, Subramanium; Ahathya, Ramakrishnan Swamy; Gubernath,; Daniel, Rajkumar; Shanmugasundaram

    2012-01-01

    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. Ba...

  20. Sturge-Weber syndrome

    OpenAIRE

    Natarajan Manivannan; Subramanium Gokulanathan; Ramakrishnan Swamy Ahathya; Gubernath,; Rajkumar Daniel; Shanmugasundaram

    2012-01-01

    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. Ba...

  1. Prävalenz und molekulare Epidemiologie der Bartonella henselae-Infektion bei Katzen in Berlin

    OpenAIRE

    Klose, Alexander

    2010-01-01

    Bartonella henselae is a world-wide distributed bacterial pathogen, which was first described in the 1990s. Its best known manifestations are Cat Scratch disease and Bacillary angiomatosis. In this study we determined a prevalence of 9,8% in Berlin composed of two populations prevalences: 1% in the group of cats which lived mainly indoors and 18,7% in the group of stray cats. Riskfactors for Bacteremia in cats are flee-infestation and age. We could confirm the flee-infestation as a riskfactor...

  2. [Massive Gorham-Stout osteolysis of the temporal bone and the craniocervical transition].

    Science.gov (United States)

    Plontke, S; Koitschev, A; Ernemann, U; Pressler, H; Zimmermann, R; Plasswilm, L

    2002-04-01

    Massive osteolysis Gorham-Stout is a rare, benign but locally aggressive angiomatosis which results in destruction and resorption of bone. The etiology and pathogenesis are undefined. The occurrence of the disease in the skull base is uncommon. A 54-year-old female presented with isolated, one-sided surditas. Eight years before the patient underwent surgery and radiation therapy for treatment of hypopharyngeal cancer. A transtemporal biopsy was taken and a highly vascularized, cystic lesion with destruction of the right occipital and temporal bone and the atlas was found. Histopathology showed thin-walled capillaries with flattened endothelial lining cells. After exclusion of malignant and infectious components the diagnosis of Gorham's disease was established. Review of the literature suggests radiation therapy as the method of choice for stopping the disease's progress. The aim of this case report is to emphasize the Gorham-Stout-Syndrome as a rare differential diagnosis for skull base lesions. PMID:12063694

  3. Bartonella quintana in Ethiopian lice.

    Science.gov (United States)

    Cutler, Sally; Abdissa, Alemseged; Adamu, Haileeysus; Tolosa, Tadele; Gashaw, Abebaw

    2012-01-01

    Head and clothing lice from Jimma, Ethiopia were investigated for pathogenic bacteria. Genomic DNA from pools of lice was subjected to PCR analysis for Bartonella spp., Borrelia spp. Coxiella burnetii, Rickettsia spp. and Yersinia pestis. All 102 lice pools were negative for the afore mentioned pathogens, with the exception of Bartonella species found among 6 of 65 (9.2%) head lice pools and1 of 33 clothing lice pools. Identification was achieved by sequencing the ribosomal intragenic transcribed spacer region (ITS), revealing all to be Bartonella quintana. Although established as a clothing louse-borne infection, typically causing chronic bacteraemia, trench fever, bacillary angiomatosis and endocarditis, this has only been rarely reported among head lice. The higher numbers of infected head lice pools compared with clothing lice suggests their competence for maintaining this infection within Ethiopia.

  4. Inter- and intraspecies identification of Bartonella (Rochalimaea) species.

    Science.gov (United States)

    Roux, V; Raoult, D

    1995-06-01

    Species of the genus Rochalimaea, recently renamed Bartonella, are of a growing medical interest. Bartonella quintana was reported as the cause of trench fever, endocarditis, and bacillary angiomatosis. B. henselae has been implicated in symptoms and infections of human immunodeficiency virus-infected patients, such as fever, endocarditis, and bacillary angiomatosis, and is involved in the etiology of cat scratch disease. Such a wide spectrum of infections makes it necessary to obtain an intraspecies identification tool in order to perform epidemiological studies. B. vinsonii, B. elizabethae, seven isolates of B. quintana, and four isolates of B. henselae were studied by pulsed-field gel electrophoresis (PFGE) after restriction with the infrequently cutting endonucleases NotI, EagI, and SmaI. Specific profiles were obtained for each of the four Bartonella species. Comparison of genomic fingerprints of isolates of the same species showed polymorphism in DNA restriction patterns, and a specific profile was obtained for each isolate. A phylogenetic analysis of the B. quintana isolates was obtained by using the Dice coefficient, UPGMA (unweighted pair-group method of arithmetic averages), and Package Philip programming. Amplification by PCR and subsequent sequencing using an automated laser fluorescent DNA sequencer (Pharmacia) was performed on the intergenic spacer region (ITS) between the 16 and 23S rRNA genes. It was found that each B. henselae isolate had a specific sequence, while the B. quintana isolates fell into only two groups. When endonuclease restriction analysis of the ITS PCR product was done, three enzymes, TaqI, HindIII, and HaeIII, allowed species identification of Bartonella spp. Restriction fragment length polymorphism after PCR amplification of the 16S-23S rRNA gene ITS may be useful for rapid species identification, and PFGE could be an efficient method for isolate identification.

  5. Inter- and intraspecies identification of Bartonella (Rochalimaea) species.

    Science.gov (United States)

    Roux, V; Raoult, D

    1995-06-01

    Species of the genus Rochalimaea, recently renamed Bartonella, are of a growing medical interest. Bartonella quintana was reported as the cause of trench fever, endocarditis, and bacillary angiomatosis. B. henselae has been implicated in symptoms and infections of human immunodeficiency virus-infected patients, such as fever, endocarditis, and bacillary angiomatosis, and is involved in the etiology of cat scratch disease. Such a wide spectrum of infections makes it necessary to obtain an intraspecies identification tool in order to perform epidemiological studies. B. vinsonii, B. elizabethae, seven isolates of B. quintana, and four isolates of B. henselae were studied by pulsed-field gel electrophoresis (PFGE) after restriction with the infrequently cutting endonucleases NotI, EagI, and SmaI. Specific profiles were obtained for each of the four Bartonella species. Comparison of genomic fingerprints of isolates of the same species showed polymorphism in DNA restriction patterns, and a specific profile was obtained for each isolate. A phylogenetic analysis of the B. quintana isolates was obtained by using the Dice coefficient, UPGMA (unweighted pair-group method of arithmetic averages), and Package Philip programming. Amplification by PCR and subsequent sequencing using an automated laser fluorescent DNA sequencer (Pharmacia) was performed on the intergenic spacer region (ITS) between the 16 and 23S rRNA genes. It was found that each B. henselae isolate had a specific sequence, while the B. quintana isolates fell into only two groups. When endonuclease restriction analysis of the ITS PCR product was done, three enzymes, TaqI, HindIII, and HaeIII, allowed species identification of Bartonella spp. Restriction fragment length polymorphism after PCR amplification of the 16S-23S rRNA gene ITS may be useful for rapid species identification, and PFGE could be an efficient method for isolate identification. PMID:7650189

  6. Clinical features of Sturge-Weber syndrome

    Directory of Open Access Journals (Sweden)

    Palheta Neto, Francisco Xavier

    2008-12-01

    Full Text Available Introduction: The Syndrome of Sturge-Weber is a rare condition of congenital development, and is characterized by a neurocutaneous disorder with angiomas wrapping the leptomeninges and the face skin, mainly in the course of ophthalmic (V1 and maxillary (V2 branches of the trigeminal nerve. Objective: To review the literature about the Sturge-Weber Syndrome with emphasis on the current aspects. Method: The following databases were searched: EMedicine, Encyclopedia of Medicine, FindArticles, LILACS, MEDLINE, Merck Manuals On-Line Medical Library and Scielo, and the searches applied the terms: Sturge-Weber Syndrome, neurocutaneous syndromes, encephalo-trigeminal angiomatosis, nevus flammeus, in articles published between 1991 and 2007. Literature's Review: The most characteristic clinical statement is the presence, since the birth, of nevus flammeus, that generally reaches one half of the face and may stretch out up to the neck; in addition, other clinical manifestations may be present, like the corticocerebral angiomatosis, cerebral calcifications, epilepsy, ocular and buccal affections and mental retardation. The diagnosis is established by means of the inquiry of neurological and ophthalmic alterations in patients with a characteristic nevus flammeus, allied to the clinical data of complementary exams such as Computerized Tomography. The treatment consists basically of controlling the already confirmed clinical manifestations and preventing from the appearing of other alterations, mainly buccal and ocular. Conclusion: This syndrome is not much frequent, but it needs to be early diagnosed, since it brings a series of complications to its carriers when not treated, specially because of reaching the Nervous Central System. The health professionals have to be suitably able to recognize its characteristic signs and symptoms, and so improve the quality of life of these patients.

  7. Adhesion and host cell modulation: critical pathogenicity determinants of Bartonella henselae.

    Science.gov (United States)

    Franz, Bettina; Kempf, Volkhard A J

    2011-04-13

    Bartonella henselae, the agent of cat scratch disease and the vasculoproliferative disorders bacillary angiomatosis and peliosis hepatis, contains to date two groups of described pathogenicity factors: adhesins and type IV secretion systems. Bartonella adhesin A (BadA), the Trw system and possibly filamentous hemagglutinin act as promiscous or specific adhesins, whereas the virulence locus (Vir)B/VirD4 type IV secretion system modulates a variety of host cell functions. BadA mediates bacterial adherence to endothelial cells and extracellular matrix proteins and triggers the induction of angiogenic gene programming. The VirB/VirD4 type IV secretion system is responsible for, e.g., inhibition of host cell apoptosis, bacterial persistence in erythrocytes, and endothelial sprouting. The Trw-conjugation system of Bartonella spp. mediates host-specific adherence to erythrocytes. Filamentous hemagglutinins represent additional potential pathogenicity factors which are not yet characterized. The exact molecular functions of these pathogenicity factors and their contribution to an orchestral interplay need to be analyzed to understand B. henselae pathogenicity in detail.

  8. Adhesins of Bartonella spp.

    Science.gov (United States)

    O'Rourke, Fiona; Schmidgen, Thomas; Kaiser, Patrick O; Linke, Dirk; Kempf, Volkhard A J

    2011-01-01

    Adhesion to host cells represents the first step in the infection process and one of the decisive features in the pathogenicity of Bartonella spp. B. henselae and B. quintana are considered to be the most important human pathogenic species, responsible for cat scratch disease, bacillary angiomatosis, trench fever and other diseases. The ability to cause vasculoproliferative disorders and intraerythrocytic bacteraemia are unique features of the genus Bartonella. Consequently, the interaction with endothelial cells and erythrocytes is a focus in Bartonella research. The genus harbours a variety of trimeric autotransporter adhesins (TAAs) such as the Bartonella adhesin A (BadA) of B. henselae and the variably expressed outer-membrane proteins (Vomps) of B. quintana, which display remarkable variations in length and modular construction. These adhesins mediate many of the biologically-important properties of Bartonella spp. such as adherence to endothelial cells and extracellular matrix proteins and induction of angiogenic gene programming. There is also significant evidence that the laterally acquired Trw-conjugation systems of Bartonella spp. mediate host-specific adherence to erythrocytes. Other potential adhesins are the filamentous haemagglutinins and several outer membrane proteins. The exact molecular functions of these adhesins and their interplay with other pathogenicity factors (e.g., the VirB/D4 type 4 secretion system) need to be analysed in detail to understand how these pathogens adapt to their mammalian hosts.

  9. Adhesion and host cell modulation: critical pathogenicity determinants of Bartonella henselae

    Directory of Open Access Journals (Sweden)

    Kempf Volkhard AJ

    2011-04-01

    Full Text Available Abstract Bartonella henselae, the agent of cat scratch disease and the vasculoproliferative disorders bacillary angiomatosis and peliosis hepatis, contains to date two groups of described pathogenicity factors: adhesins and type IV secretion systems. Bartonella adhesin A (BadA, the Trw system and possibly filamentous hemagglutinin act as promiscous or specific adhesins, whereas the virulence locus (VirB/VirD4 type IV secretion system modulates a variety of host cell functions. BadA mediates bacterial adherence to endothelial cells and extracellular matrix proteins and triggers the induction of angiogenic gene programming. The VirB/VirD4 type IV secretion system is responsible for, e.g., inhibition of host cell apoptosis, bacterial persistence in erythrocytes, and endothelial sprouting. The Trw-conjugation system of Bartonella spp. mediates host-specific adherence to erythrocytes. Filamentous hemagglutinins represent additional potential pathogenicity factors which are not yet characterized. The exact molecular functions of these pathogenicity factors and their contribution to an orchestral interplay need to be analyzed to understand B. henselae pathogenicity in detail.

  10. Adhesins of Bartonella spp.

    Science.gov (United States)

    O'Rourke, Fiona; Schmidgen, Thomas; Kaiser, Patrick O; Linke, Dirk; Kempf, Volkhard A J

    2011-01-01

    Adhesion to host cells represents the first step in the infection process and one of the decisive features in the pathogenicity of Bartonella spp. B. henselae and B. quintana are considered to be the most important human pathogenic species, responsible for cat scratch disease, bacillary angiomatosis, trench fever and other diseases. The ability to cause vasculoproliferative disorders and intraerythrocytic bacteraemia are unique features of the genus Bartonella. Consequently, the interaction with endothelial cells and erythrocytes is a focus in Bartonella research. The genus harbours a variety of trimeric autotransporter adhesins (TAAs) such as the Bartonella adhesin A (BadA) of B. henselae and the variably expressed outer-membrane proteins (Vomps) of B. quintana, which display remarkable variations in length and modular construction. These adhesins mediate many of the biologically-important properties of Bartonella spp. such as adherence to endothelial cells and extracellular matrix proteins and induction of angiogenic gene programming. There is also significant evidence that the laterally acquired Trw-conjugation systems of Bartonella spp. mediate host-specific adherence to erythrocytes. Other potential adhesins are the filamentous haemagglutinins and several outer membrane proteins. The exact molecular functions of these adhesins and their interplay with other pathogenicity factors (e.g., the VirB/D4 type 4 secretion system) need to be analysed in detail to understand how these pathogens adapt to their mammalian hosts. PMID:21557057

  11. Adhesion and host cell modulation: critical pathogenicity determinants of Bartonella henselae.

    Science.gov (United States)

    Franz, Bettina; Kempf, Volkhard A J

    2011-01-01

    Bartonella henselae, the agent of cat scratch disease and the vasculoproliferative disorders bacillary angiomatosis and peliosis hepatis, contains to date two groups of described pathogenicity factors: adhesins and type IV secretion systems. Bartonella adhesin A (BadA), the Trw system and possibly filamentous hemagglutinin act as promiscous or specific adhesins, whereas the virulence locus (Vir)B/VirD4 type IV secretion system modulates a variety of host cell functions. BadA mediates bacterial adherence to endothelial cells and extracellular matrix proteins and triggers the induction of angiogenic gene programming. The VirB/VirD4 type IV secretion system is responsible for, e.g., inhibition of host cell apoptosis, bacterial persistence in erythrocytes, and endothelial sprouting. The Trw-conjugation system of Bartonella spp. mediates host-specific adherence to erythrocytes. Filamentous hemagglutinins represent additional potential pathogenicity factors which are not yet characterized. The exact molecular functions of these pathogenicity factors and their contribution to an orchestral interplay need to be analyzed to understand B. henselae pathogenicity in detail. PMID:21489243

  12. BASED TO CLINICAL CASE. VON HIPLEA-LINDAU SYNDROME

    Directory of Open Access Journals (Sweden)

    Brzeziński Piotr

    2011-01-01

    Full Text Available von Hippel-Lindau syndrome (VHL is a rare, genetic multi-system disorder characterized by the abnormal growth of tumors in certain parts of the body (angiomatosis. The tumors of the central nervous system (CNS are benign and are comprised of a nest of blood vessels and are called hemangioblastomas. Hemangioblastomas may develop in the brain, the retina of the eyes, and other areas of the nervous system. Other types of tumors develop in the adrenal glands, the kidneys, or the pancreas. Symptoms of VHL vary among patients and depend on the size and location of the tumors. Symptoms may include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Cysts (fluid-filled sacs and/or tumors (benign or cancerous may develop around the hemangioblastomas and cause the symptoms listed above. Individuals with VHL are also at a higher risk than normal for certain types of cancer, especially kidney cancer. Based on the case of 30-year old patient with characteristics of von Hippel-Lindau syndrome as phakomatosis.

  13. Whole-body magnetic resonance imaging: a useful additional sequence in paediatric imaging

    Energy Technology Data Exchange (ETDEWEB)

    Laffan, Eoghan E.; O' Connor, Rachael; Ryan, Stephanie P.; Donoghue, Veronica B. [Department of Radiology, Children' s University Hospital, Temple Street, 1, Dublin (Ireland)

    2004-06-01

    Background: Whole-body MR (WBMR) imaging allows the acquisition of images of the entire body in a matter of minutes. Its use has primarily been in the evaluation of possible metastases in the setting of a known primary tumour. Objective: To document the value of WBMR imaging in ten children in whom this was added as an additional sequence when the primary diagnosis had not yet been made. Materials and methods: Ten children, age range 4 months-15 years (mean 7 years 4 months) had WBMR imaging after initial MR showed an abnormality that raised the possibility of systemic disease. Initial scanning was of the brain (n=1), spine (n=2), retroperitoneum (n=4), hips (n=1), femur (n=1) and wrist (n=1). Results: Abnormalities were detected in eight patients. Two patients had acute lymphoblastic leukaemia, and another had an anaplastic lymphoma, unsuspected prior to the WBMR. Two patients had a previously undiagnosed neuroblastoma with bone marrow metastases. Two patients had Langerhans' cell histiocytosis. Another had multiple bone lesions due to cystic angiomatosis. Conclusions: WBMR imaging may be a useful additional sequence in children in whom a systemic and especially a bone marrow abnormality is suspected. (orig.)

  14. The centenary of the discovery of trench fever, an emerging infectious disease of World War 1.

    Science.gov (United States)

    Anstead, Gregory M

    2016-08-01

    In 1915, a British medical officer on the Western Front reported on a soldier with relapsing fever, headache, dizziness, lumbago, and shin pain. Within months, additional cases were described, mostly in frontline troops, and the new disease was called trench fever. More than 1 million troops were infected with trench fever during World War 1, with each affected soldier unfit for duty for more than 60 days. Diagnosis was challenging, because there were no pathognomonic signs and symptoms and the causative organism could not be cultured. For 3 years, the transmission and cause of trench fever were hotly debated. In 1918, two commissions identified that the disease was louse-borne. The bacterium Rickettsia quintana was consistently found in the gut and faeces of lice that had fed on patients with trench fever and its causative role was accepted in the 1920s. The organism was cultured in the 1960s and reclassified as Bartonella quintana; it was also found to cause endocarditis, peliosis hepatis, and bacillary angiomatosis. Subsequently, B quintana infection has been identified in new populations in the Andes, in homeless people in urban areas, and in individuals with HIV. The story of trench fever shows how war can lead to the recrudescence of an infectious disease and how medicine approached an emerging infection a century ago.

  15. The centenary of the discovery of trench fever, an emerging infectious disease of World War 1.

    Science.gov (United States)

    Anstead, Gregory M

    2016-08-01

    In 1915, a British medical officer on the Western Front reported on a soldier with relapsing fever, headache, dizziness, lumbago, and shin pain. Within months, additional cases were described, mostly in frontline troops, and the new disease was called trench fever. More than 1 million troops were infected with trench fever during World War 1, with each affected soldier unfit for duty for more than 60 days. Diagnosis was challenging, because there were no pathognomonic signs and symptoms and the causative organism could not be cultured. For 3 years, the transmission and cause of trench fever were hotly debated. In 1918, two commissions identified that the disease was louse-borne. The bacterium Rickettsia quintana was consistently found in the gut and faeces of lice that had fed on patients with trench fever and its causative role was accepted in the 1920s. The organism was cultured in the 1960s and reclassified as Bartonella quintana; it was also found to cause endocarditis, peliosis hepatis, and bacillary angiomatosis. Subsequently, B quintana infection has been identified in new populations in the Andes, in homeless people in urban areas, and in individuals with HIV. The story of trench fever shows how war can lead to the recrudescence of an infectious disease and how medicine approached an emerging infection a century ago. PMID:27375211

  16. Zebrafish embryo model of Bartonella henselae infection.

    Science.gov (United States)

    Lima, Amorce; Cha, Byeong J; Amin, Jahanshah; Smith, Lisa K; Anderson, Burt

    2014-10-01

    Bartonella henselae (Bh) is an emerging zoonotic pathogen that has been associated with a variety of human diseases, including bacillary angiomatosis that is characterized by vasoproliferative tumor-like lesions on the skin of some immunosuppressed individuals. The study of Bh pathogenesis has been limited to in vitro cell culture systems due to the lack of an animal model. Therefore, we wanted to investigate whether the zebrafish embryo could be used to model human infection with Bh. Our data showed that Tg(fli1:egfp)(y1) zebrafish embryos supported a sustained Bh infection for 7 days with >10-fold bacterial replication when inoculated in the yolk sac. We showed that Bh recruited phagocytes to the site of infection in the Tg(mpx:GFP)uwm1 embryos. Infected embryos showed evidence of a Bh-induced angiogenic phenotype and an increase in the expression of genes encoding pro-inflammatory factors and pro-angiogenic markers. However, infection of zebrafish embryos with a deletion mutant in the major adhesin (BadA) resulted in little or no bacterial replication and a diminished host response, providing the first evidence that BadA is critical for in vivo infection. Thus, the zebrafish embryo provides the first practical model of Bh infection that will facilitate efforts to identify virulence factors and define molecular mechanisms of Bh pathogenesis.

  17. 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.)

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

    International Nuclear Information System (INIS)

    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.)

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

    Directory of Open Access Journals (Sweden)

    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.

  20. [Medullary venous malformation with azygos anterior cerebral artery aneurysm: a case report].

    Science.gov (United States)

    Harada, K; Kobayashi, S; Sigemori, M; Watanabe, M; Kuramoto, S

    1987-03-01

    A case of medullary venous malformation with azygos anterior cerebral artery aneurysm is reported, which was associated with the leptomenigeal angiomatosis on the cortex of the right frontal lobe. A 62-year-old female was admitted to the Kurume University Hospital on June 21, 1985, because of a convulsive seizure. On admission, neurological and general examinations, except for slightly liver function disorder, was no abnormality. Plain CT scan showed the cortical atrophy and two small high-density areas in the right frontal lobe. Enhanced CT scan showed a high-density area in the genu of the corpus callosum. MRI (magnetic resonance imaging), on T2 weighted image, showed a high signal intensity area in the right paraventricular deep white matter and the right frontal region. A right internal carotid angiogram in the venous phase showed numerous enlarged medullary veins in the deep frontal region, converging into a single large draining vein that empties into the basal vein of Rosenthal. An azygos anterior cerebral artery was visualized on the right and left carotid angiograms. The aneurysm was situated at the distal end of the azygos artery. Twenty eight days after admission, a right frontal cranioplastic craniotomy was performed. During operation, the surface of the right frontal lobe was covered by fine, vascular networks, which was reddish brown. A right frontal lobectomy, including venous malformation and vascular networks, was performed. The aneurysm was clipped via an interhemispheric approach. Histologically, the malformation vessels had spread into the subarachnoid space.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3600991

  1. Sturge-Weber综合征临床分析及影像诊断%Clinical and imaging features of Sturge-Weber syndrome

    Institute of Scientific and Technical Information of China (English)

    黄晓星; 王志龙; 肖学红; 杨昂

    2015-01-01

    目的:探讨 Sturge-Weber 综合征的临床特点及 CT、MRI 表现及诊断。方法归纳8例 Sturge-Weber 综合征患者的临床表现、影像学特点,进行回顾性分析,并复习相关文献。结果8例均有脑内软脑膜血管瘤,其中3例伴有颜面部血管瘤,均位于三叉神经眼支分布区(均累及单侧);7例表现为癫痫发作,其中5例伴有头痛,1例伴有头晕及一侧肢体乏力;1例表现为单纯反复头痛;均无智力低下或青光眼。影像学检查可清晰显示脑萎缩、脑回样钙化、软脑膜广泛强化伴静脉畸形。结论 Sturge-Weber综合征是一种少见疾病,根据典型临床特点及CT、MRI等影像学检查有助于确诊。%Objective To determine the clinical and imaging features of Sturge-Weber syndrome (SWS). Methods The clinical and imaging features of 8 patients with Sturge-Weber syndrome were reviewed. Results Leptomeningeal angiomatosis was found in all patients,accompanied by unilateral facial hemangioma (3) in the distribution of the ophthalmic division of the trigeminal nerve. Of 7 patients with epilepsy , 5 were accompanied by headache , 1 by dizziness and unilateral muscle weakness , 1 by recurring simple headache. There was no mental retardation or glaucoma in any of the patients...CT and MRI clearly showed brain atrophy, gyriform calcification, diffuse enhancement of pia mater accompanied by cerebral venous malformation. Conclusion Sturge-Weber syndrome has typical clinical and imaging features on CT and MRI for diagnosis.

  2. [Intraspinal hemangioblastomas].

    Science.gov (United States)

    Hurth, M

    1975-06-01

    138 cases of spinal haemangioblastomas have been reviewed (30 original cases). The first part of this study is devoted to a general survey concerning history, pathological anatomy, genetics and nosology of haemangioblastomas of the central nervous system. Microscopic anatomy of these tumors has been a much debated question and is responsible for the numerous denominations proposed: angiomatosis, capillary angiomas, angioreticulomas, haemangiomas, haemangioendotheliomas, haemangiopericytomas, angioblastic meningiomas; haemangioblastoma seems the most correct. Recent studies in optical and mainly electron microscopy do not allow exact typing of tumoral cells and histogenesis remains uncertain. It is generally accepted that the tumor arises in vascular nesenchyme as a result of dygenetic abnormality. This theory provides a rational explanation for visceral localizations and classification of Lindau disease among phakomatoses. An hereditary factor is present in 25 percent of cases, mainly in multifocal localizations. In the second part, interest is focused on spinal haemangioblastomas, 17 extradural, 121 subdural haemangioblastomas are studied and listed. Extradural tumors are always solitary, mostly in the dorsal area. Subdural tumors may be out of the cord (10 tumors), on the posterior roots (28 tumors), or within the cord. The lesions are situated mainly in the cervicodorsal and dorsolumbar regions. Intramedullary haemangioblastomas are situated in nearly all cases in the dorsal part of the cord behind the central canal. They are close to the dorsal surface of the cord (60 percent) or partly out of the cord (6 percent) or totally intramedullary (30 percent). Cysts close to the tumor or at a distance are present in nearly 50 percent of cases and their origin is still questionnable. Clinical patterns have no special peculiarities, syrinomyelic symptomatology occurs in only 25 percent of cases. Neuroradiology and mainly spinal angiography are a very important step