Kotyal B Mahendrappa; N Rashmi; S. Mamatha
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 S...
Kotyal B Mahendrappa
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.
Diniz, Lucia Martins; Medeiros, Karina Bittencourt; Landeiro, Luana Gomes; Lucas, Elton Almeida
Bacillary angiomatosis is an infection determined by Bartonella henselae and B. quintana, rare and prevalent in patients with acquired immunodeficiency syndrome. We describe a case of a patient with AIDS and TCD4+ cells equal to 9/mm3, showing reddish-violet papular and nodular lesions, disseminated over the skin, most on the back of the right hand and third finger, with osteolysis of the distal phalanx observed by radiography. The findings of vascular proliferation with presence of bacilli, on the histopathological examination of the skin and bone lesions, led to the diagnosis of bacillary angiomatosis. Corroborating the literature, in the present case the infection affected a young man (29 years old) with advanced immunosuppression and clinical and histological lesions compatible with the diagnosis. PMID:28099606
Pak, Min Gyoung; Choi, Phil Jo; Choi, Won Suk; Kang, Eun Ju; Roh, Mee Sook
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...
Dinesh Singh Chauhan
Full Text Available Angiomatosis is a complex vascular malformation of infancy and childhood consisting of proliferating blood vessels with accompanying mature fat, fibrous tissue, lymphatic′s and nerves, which may involve skin, subcutaneous tissue, skeletal muscle and occasionally bone. It is extremely rare and benign, but a clinically extensive vascular lesion of soft-tissue, which usually becomes symptomatic during childhood or adolescence. We report a rare case of massive juvenile angiomatosis of maxilla and mandible in a 15-year-old male patient.
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)
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.)
Renata Félix da Justa
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.
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.
Díaz-Delgado, Josué; Arbelo, Manuel; Sacchini, Simona; Quesada-Canales, Óscar; Andrada, Marisa; Rivero, Miguel; Fernández, Antonio
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.
Full Text Available We present a 63-year-old man treated with alemtuzumab for chronic lymphocytic leukemia who developed multiple angiomatous papules and fever. Real-time polymerase chain reaction (RT-PCR from a skin lesion and blood sample revealed Bartonella quintana as causative agent confirming the diagnosis of bacillary angiomatosis with bacteremia. Treatment with doxycycline, initially in combination with gentamicin, led to complete resolution of the lesions. This case shows the importance of considering bacillary angiomatosis as a rare differential diagnosis of angiomatous lesions in the immunocompromised patient, particularly in chronic lymphocytic leukemia and following lymphocyte depleting treatments as alemtuzumab.
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
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.
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.
Niiro, Masaki; Mihara, Tadahiro; Maeda, Yoshiki; Awa, Hiroshi; Kadota, Koki; Asakura, Tetsuhiko (Kagoshima Univ. (Japan). Faculty of Medicine)
A case of left occipital leptomeningeal angiomatosis was reported. The patient was a 12-year-old boy who had episodes of severe vascular type headache accompanied by transient right homonymous hemianopsia. CT scan showed localized superficial high density area in the left occipital pole. Remarkable enhancement of the lower and inner surface of the left occipital lobe was demonstrated. Angiography showed poor filling of the distal portion of the left posterior cerebral artery. Skull tomograms showed linear calcifications in the left occipital region. Brain scan showed increased RI uptake in the left occipital region. During operation, the surface of the left occipital lobe was covered by excessive, fine, vascular networks which extended over the arachnoid membrane. The abnormal vessels were cauterized by a CO/sub 2/ laser as throughly as possible. The occipital pole felt gritty. Histologically, the abnormal vessels had spread into the subarachnoid space and were predominantly veins with thin and enlarged walls. The abnormal vessels followed the leptomeninges in the sulci of the cerebral cortex. Underneath the abnormal vessels, in the external layers of the cerebral cortex, calcium deposits were scattered and gliosis and degeneration of the ganglion cells were observed. The lesion was comparable with leptomeningeal angiomatosis. Though the pathological findings of the specimen, CT findings, and brain scan findings were extremely similar to those of Sturge-Weber disease, in this case, the typical clinical and roentgenographic findings of Sturge-Weber disease were all absent.
Farrah J Mateen
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.
张立英; 朱红艳; 皋岚湘
目的 探讨颅面血管瘤病的临床病理特征、诊断与鉴别诊断、治疗及预后.方法 回顾性分析1例颅面血管瘤病的临床资料、组织病理学形态.结果 光镜下大脑组织灰质萎缩,神经细胞变性、缺失,脑实质内散在较多大小不等的钙化灶,钙化小体呈圆形、同心环状或不规则形,或融合,部分区域见弥漫的钙化小球.局部软脑膜表面可见异常增生的毛细血管网,毛细血管扩张、腔内充满红细胞,呈海绵状血管瘤改变,局部见少量厚壁肌型静脉样血管,间质有出血.结论 颅面血管瘤病是一种罕见的先天性神经皮肤综合征,先天性脑部和三叉神经分布区同时有血管瘤存在.临床以颜面血管瘤、癫痫发作为主要症状.%Objective To investigate the clinical and pathological features,diagnosis and differential diagnosis,treatment and prognosis of craniofacial angiomatosis.Methods Retrospective analysis of the clinical data and the histopathological morphology was conducted in one case of craniofacial angiomatosis.Results Under light microscope,the gray matter of brain tissue atrophied and nerve cells degenerated and decreased,there were many calcification scattered or diffused in the brain parenchyma with varying sizes,and the calcification bodies were concentric-like,irregular or merged.Some dysplasia capillary network was visible on the surface of the leptomeninge,the capillaries dilated and filled with red blood cells.Some areas were similiar to cavernous hemangioma,a small amount of thick-walled muscular vein-like vessels were also seen in those areas.Hemorrhage could be seen in the interstitial tissue.Conclusions Craniofacial angiomatosis is a rare congenital neurocutaneous syndrome.It is named as congenital emangiomas distributing in the brain and trigeminal nerve area,and its incidence is very low,and therefore,grasping the clinical and pathological features of the disease will play an important role in the
Paulo Eduardo Neves Ferreira Velho
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.
ZHOU Jing; LI Nan-yun; ZHOU Xiao-jun; WANG Jian-dong; MA Heng-hui; ZHANG Ru-song
@@ 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.
LIU Qiong; CHEN Yi-peng; LI You-ming
@@ 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.
Full Text Available Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis or meningofacial angiomatosis, is characterised in its classical form by a congenital, usually unilateral, ‘portwine stain’ (capillary naevus on the face, convulsions, typical intracranial calcification and some degree of mental retardation and hemiparesis. The clinical correlation of intractable seizures with the presence of bilateral intracranial disease has management and prognostic implications, thus making the presence of bilateral disease an important factor to all those involved in the management of the child with Sturge-Weber syndrome.
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.
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 (TLRs
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)
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.).
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.
Limotai, Chusak; Go, Cristina Y; Baba, Shiro; Okanari, Kazuo; Ochi, Ayako; Rutka, James T; Snead, O Carter; Otsubo, Hiroshi
Infants with Sturge-Weber syndrome (SWS) are considered for surgery if they develop seizures and the seizures prove medically refractory. The authors report on 2 infants (15 and 19 months old) with SWS who underwent scalp video electroencephalography (EEG) and subsequent functional hemispherotomy for intractable partial motor seizures due to extensive left hemispheric angiomatosis. They presented with similar interictal and ictal EEG findings. Ictal EEG showed abrupt high-amplitude delta slow waves, without evolution on the contralateral hemisphere before the build-up of ictal EEG changes on the lesional hemisphere. The patients became seizure free after hemispherotomy. The ictal contralateral slow waves were not a sign of an ictal hemisphere and may indicate prominent ischemic changes resulting from a steal phenomenon of hemispheric angiomatosis during seizure.
Full Text Available The Sturge-Weber Syndrome, also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. The main clinical features of this syndrome are facial vascular cutaneous naevus, usually unilateral, which often follows the outline distribution of trigeminal nerve. We hereby are reporting a clinical case of Sturge-Weber Syndrome in a 16 year old female patient who presented with oral, cutaneous and ocular manifestations related to the syndrome.
WANG Guang-bin; XU Lei; ZHAO Bin; CAI Shi-feng; SHI Hao; LI Hui-hua; QU Lei
@@ 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.
Kozlowski, K.; Beluffi, G.; Cohen, D.H.; Padovani, J.; Tamaela, L.; Azouz, M.; Bale, P.; Martin, H.C.; Nayanar, V.V.; Arico, M.
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.
Berrich, Moez; Kieda, Claudine; Grillon, Catherine; Monteil, Martine; Lamerant, Nathalie; Gavard, Julie; Haddad, Nadia
Bartonella henselae, a zoonotic agent, induces tumors of endothelial cells (ECs), namely bacillary angiomatosis and peliosis in immunosuppressed humans but not in cats. In vitro studies on ECs represent to date the only way to explore the interactions between Bartonella henselae and vascular endothelium. However, no comparative study of the interactions between Bartonella henselae and human (incidental host) ECs vs feline (reservoir host) ECs has been carried out because of the absence of ...
Moez Berrich; Claudine Kieda; Catherine Grillon; Martine Monteil; Nathalie Lamerant; Julie Gavard; Henri Jean Boulouis; Nadia Haddad
Bartonella henselae, a zoonotic agent, induces tumors of endothelial cells (ECs), namely bacillary angiomatosis and peliosis in immunosuppressed humans but not in cats. In vitro studies on ECs represent to date the only way to explore the interactions between Bartonella henselae and vascular endothelium. However, no comparative study of the interactions between Bartonella henselae and human (incidental host) ECs vs feline (reservoir host) ECs has been carried out because of the absence of any...
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.
Luiz Felipe G. dos Santos; Joanna G. da Conceição; Thaís Pimentel de Sá Bahia; Vanessa de A. S. Silva; Maria Eliza Barbosa Ramos; Mônica Israel
Introduction: The Sturge-Weber Syndrome, also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. The main clinical features of this syndrome are facial vascular cutaneous naevus, usually unilateral, which often follows the outline distribution of trigeminal nerve. Objective: To report a clinical case of Sturge-Weber Syndrome in a 29-yeral-old male patient who presented oral manifestations related to the syndrome. Case report and conclusion: The patient re...
Berrich, Moez; Kieda, Claudine; Grillon, Catherine; Monteil, Martine; Lamerant, Nathalie; Gavard, Julie; Boulouis, Henri Jean; Haddad, Nadia
Bartonella henselae, a zoonotic agent, induces tumors of endothelial cells (ECs), namely bacillary angiomatosis and peliosis in immunosuppressed humans but not in cats. In vitro studies on ECs represent to date the only way to explore the interactions between Bartonella henselae and vascular endothelium. However, no comparative study of the interactions between Bartonella henselae and human (incidental host) ECs vs feline (reservoir host) ECs has been carried out because of the absence of any available feline endothelial cell lines.To this purpose, we have developed nine feline EC lines which allowed comparing the effects of Bartonella strains on human and feline micro-vascular ECs representative of the infection development sites such as skin, versus macro-vascular ECs, such as umbilical vein.Our model revealed intrinsic differences between human (Human Skin Microvascular ECs -HSkMEC and Human Umbilical Vein ECs - iHUVEC) and feline ECs susceptibility to Bartonella henselae infection.While no effect was observed on the feline ECs upon Bartonella henselae infection, the human ones displayed accelerated angiogenesis and wound healing.Noticeable differences were demonstrated between human micro- and macro-vasculature derived ECs both in terms of pseudo-tube formation and healing. Interestingly, Bartonella henselae effects on human ECs were also elicited by soluble factors.Neither Bartonella henselae-infected Human Skin Microvascular ECs clinically involved in bacillary angiomatosis, nor feline ECs increased cAMP production, as opposed to HUVEC.Bartonella henselae could stimulate the activation of Vascular Endothelial Growth Factor Receptor-2 (VEGFR-2) in homologous cellular systems and trigger VEGF production by HSkMECs only, but not iHUVEC or any feline ECs tested.These results may explain the decreased pathogenic potential of Bartonella henselae infection for cats as compared to humans and strongly suggest that an autocrine secretion of VEGF by human skin
Full Text Available Bartonella henselae, a zoonotic agent, induces tumors of endothelial cells (ECs, namely bacillary angiomatosis and peliosis in immunosuppressed humans but not in cats. In vitro studies on ECs represent to date the only way to explore the interactions between Bartonella henselae and vascular endothelium. However, no comparative study of the interactions between Bartonella henselae and human (incidental host ECs vs feline (reservoir host ECs has been carried out because of the absence of any available feline endothelial cell lines.To this purpose, we have developed nine feline EC lines which allowed comparing the effects of Bartonella strains on human and feline micro-vascular ECs representative of the infection development sites such as skin, versus macro-vascular ECs, such as umbilical vein.Our model revealed intrinsic differences between human (Human Skin Microvascular ECs -HSkMEC and Human Umbilical Vein ECs - iHUVEC and feline ECs susceptibility to Bartonella henselae infection.While no effect was observed on the feline ECs upon Bartonella henselae infection, the human ones displayed accelerated angiogenesis and wound healing.Noticeable differences were demonstrated between human micro- and macro-vasculature derived ECs both in terms of pseudo-tube formation and healing. Interestingly, Bartonella henselae effects on human ECs were also elicited by soluble factors.Neither Bartonella henselae-infected Human Skin Microvascular ECs clinically involved in bacillary angiomatosis, nor feline ECs increased cAMP production, as opposed to HUVEC.Bartonella henselae could stimulate the activation of Vascular Endothelial Growth Factor Receptor-2 (VEGFR-2 in homologous cellular systems and trigger VEGF production by HSkMECs only, but not iHUVEC or any feline ECs tested.These results may explain the decreased pathogenic potential of Bartonella henselae infection for cats as compared to humans and strongly suggest that an autocrine secretion of VEGF by human
Sujit A Jagtap
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.
Full Text Available Kaposi′s sarcoma is described as cutaneous and extracutaneous neoplasm predominantly affecting older individuals. Though earlier uncommon and endemic to certain African areas, its incidence is on a rise due to infections with human immunodeficiency virus and also due to transplant-associated immunosuppression. Further, certain benign conditions like Pseudo Kaposi′s sarcoma, certain infective conditions like bacillary angiomatosis of acquired immunodeficiency syndrome can mimic Kaposi′s sarcoma both clinically and histologically leading to a diagnostic dilemma. We report such a case here.
Bersano, Anna; Morbin, Michela; Ciceri, Elisa; Bedini, Gloria; Berlit, Peter; Herold, Michele; Saccucci, Stefania; Fugnanesi, Valeria; Nordmeyer, Hannes; Faragò, Giuseppe; Savoiardo, Mario; Taroni, Franco; Carriero, MariaRita; Boncoraglio Giorgio, Battista; Perucca, Laura; Caputi, Luigi; Parati Eugenio, Agostino; Kraemer, Markus
Divry van Bogaert Syndrome (DBS) is a familial juvenile-onset disorder characterized by livedo racemosa, white matter disease, dementia, epilepsy and angiographic finding of "cerebral angiomatosis". A similar syndrome including livedo racemosa and cerebrovascular disease, often associated with anticardiolipin antibodies, has been described as Sneddon Syndrome (SS) highlighting the question whether these two conditions have to be considered different entities or indeed different features of a unique syndrome. Herein, we report the clinical, neuroradiological, histopathological findings and follow up of three cases diagnosed as Divry-van Bogaert Syndrome, including an updated review of literature of both DBS and SS cases. Our findings support the assumption that DBS and SS are different disease entities. DBS is characterized by the typical angiographic feature of angiomatosis, a hereditary trait and a juvenile onset of cognitive impairment and leukoaraiosis, whereas SS has less severe manifestations of cerebrovascular disease associated with livedo racemosa but without the characteristic cerebral angiography. The report of our cases and the literature review underline the necessity of a detailed work-up and the collection of larger series to better clarify the DBS and SS phenotype and course.
@@ 杆菌性血管瘤病(bacillary angiomatosis)和杆菌性紫癜(bacillary peliosis)作为新的机会性感染出现在AIDS流行的初期,它们都是由横塞巴尔通体(bartonella henselae,过去认为是rochalimaea henselae)和五日热巴尔通体(bartonella quintana,或rochalimaea quintana)感染引起.这两种致病菌为革兰氏阴性杆菌,在实验室中培养非常困难.巴尔通体属中有4种对人类致病,分别为杆菌状巴尔通体、横塞巴尔通体(B.henselae)、五日热巴尔通体(B.quintana)和伊丽莎白巴尔通体.
Velho Paulo Eduardo Neves Ferreira
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.
Cutler, Sally; Abdissa, Alemseged; Adamu, Haileeysus; Tolosa, Tadele; Gashaw, Abebaw
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.
Full Text Available ABSTRACT: Sturge Weber syndrome (SWS also called encephalo-t rigeminal-angiomatosis, is an uncommon entity in India. The characteristic featur e of SWS is the presence of “port-wine stain” varying from light pink to deep purple, covering tr igeminal nerve distribution. Patients have associated ocular involvement, mental retardation, and seizures due to the involvement of the vasculature of eye and the CNS. Neuro-ophthalmological monitoring of patients with SWS may be useful for early detection of ocular involvement before the appearance of seri ous visual complications. We have reported an unusual case of SWS with port wine stain on half of the body, retinal detachment with raised IOP and cerebellar calcifications with no neurological symptomatic manifestations.
Palheta Neto, Francisco Xavier
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.
Okudaira, Yojiro; Bandoh, Kuniaki; Ito, Masanori; Sato, Kiyoshi; Tsuji, Masahiro (Juntendo Univ., Tokyo (Japan). School of Medicine)
In 4 Sturge-Weber-syndrome child patients with calcified lesions mainly in the occipital lobe, regional cerebral blood flow (rCBF) was determined with stable Xe-CT in the resting state and after iv injection of acetazolamide (AA) and megimide (M), with the purpose of examining factors affecting deterioration of neurologic manifestations and the influence of lobectomy. In the resting state, rCBF of the temporal and occipital areas was significantly lower on the affected than unaffected sides. The side-to-side asymmetry of rCBF in the affected and unaffected sides decreased from the frontal to temporal and occipital areas. It was indistinct after AA injection because cerebral vasoreactivity became higher on the affected side. A low rCBF in association with a high cerebral vasoreactivity on the lesion side suggested that the low rCBF matched the low cerebral metabolism of the brain area affected by leptomeningeal angiomatosis. However, cerebral vasoreactivity to AA depended on clinical presentations. Two patients presented with progressive mental retardation. The other two patients were in clinically stable condition. Cerebral vasoreactivity to AA in the former two cases was poorer on both the affected and unaffected sides than that in the latter two cases. M administration was associated with a significantly decreased rCBF in the area of leptomeningeal angiomatosis and cerebral calcification and with a significantly increased rCBF of the 'pericalcified' area. In one patient undergoing extended occipital lobectomy on the affected side, no decrease in rCBF was noted in the resting state in either affected or unaffected side, and cerebral vasoreactivity to AM on the unaffected side was apparently increased. Both circulatory disturbance and seizure appear to play a role in clinical presentations, as well as their deterioration in Sturge-Weber-Syndrome patients. (N.K.).
Roux, V; Raoult, D
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.
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.
Lima, Amorce; Cha, Byeong J; Amin, Jahanshah; Smith, Lisa K; Anderson, Burt
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.
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.
Anstead, Gregory M
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.
Mancuso, Matthew; Jiang, Li; Cesarman, Ethel; Erickson, David
Kaposi's sarcoma (KS) is an infectious cancer occurring most commonly in human immunodeficiency virus (HIV) positive patients and in endemic regions, such as Sub-Saharan Africa, where KS is among the top four most prevalent cancers. The cause of KS is the Kaposi's sarcoma-associated herpesvirus (KSHV, also called HHV-8), an oncogenic herpesvirus that while routinely diagnosed in developed nations, provides challenges to developing world medical providers and point-of-care detection. A major challenge in the diagnosis of KS is the existence of a number of other diseases with similar clinical presentation and histopathological features, requiring the detection of KSHV in a biopsy sample. In this work we develop an answer to this challenge by creating a multiplexed one-pot detection system for KSHV DNA and DNA from a frequently confounding disease, bacillary angiomatosis. Gold and silver nanoparticle aggregation reactions are tuned for each target and a multi-color change system is developed capable of detecting both targets down to levels between 1 nM and 2 nM. The system developed here could later be integrated with microfluidic sample processing to create a final device capable of solving the two major challenges in point-of-care KS detection.
Kempf Volkhard AJ
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.
Full Text Available Abstract Background Although the first clinical descriptions of Bartonella infection were associated with immunocompromised patient with bacillary angiomatosis, we currently know that this organism is directly involved in diseases affecting a large number of patients, regardless of their immune status. Cat scratch disease, hepatic peliosis, and some cases of bacteraemia and endocarditis, are directly caused by some species of the genus Bartonella. The purpose of this study was to determinate the prevalence of IgG antibodies against Bartonella henselae and B. quintana in HIV patients and to identify the epidemiological factors involved. Methods Serum samples were collected from HIV patients treated at Hospital de Sabadell. Antibodies to B. henselae and B. quintana from 340 patients were examined by indirect immunofluorescence assay (IFA. Significance levels for univariate statistical test were determined by the Mann-Whitney U test and χ2 test. Results Of 340 patients, 82 were women and 258 men, with a median age of 42.21 ± 10.35 years (range 16–86 years. Seventy-six (22.3% patients reacted with one or more Bartonella antigens. Of all the factors concerning the seroprevalence rate being studied (age, sex, intravenous drugs use, alcohol consumption, CD4 levels, AIDS, HCV, HBV, residential area, only age was statistically significant. Conclusion A high percentage of HIV patients presents antibodies to Bartonella and is increasing with age.
Angelakis, Emmanouil; Raoult, Didier
Bartonella spp. are responsible for emerging and re-emerging diseases around the world. The majority of human infections are caused by Bartonella henselae, Bartonella quintana and Bartonella bacilliformis, although other Bartonella spp. have also been associated with clinical manifestations in humans. The severity of Bartonella infection correlates with the patient's immune status. Clinical manifestations can range from benign and self-limited to severe and life-threatening disease. Clinical conditions associated with Bartonella spp. include local lymphadenopathy, bacteraemia, endocarditis, and tissue colonisation resulting in bacillary angiomatosis and peliosis hepatis. Without treatment, Bartonella infection can cause high mortality. To date, no single treatment is effective for all Bartonella-associated diseases. In the absence of systematic reviews, treatment decisions for Bartonella infections are based on case reports that test a limited number of patients. Antibiotics do not significantly affect the cure rate in patients with Bartonella lymphadenopathy. Patients with Bartonella spp. bacteraemia should be treated with gentamicin and doxycycline, but chloramphenicol has been proposed for the treatment of B. bacilliformis bacteraemia. Gentamicin in combination with doxycycline is considered the best treatment regimen for endocarditis, and erythromycin is the first-line antibiotic therapy for the treatment of angioproliferative lesions. Rifampicin or streptomycin can be used to treat verruga peruana. In this review, we present recent data and recommendations related to the treatment of Bartonella infections based on the pathogenicity of Bartonella spp.
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.
Scheidegger, F; Ellner, Y; Guye, P; Rhomberg, T A; Weber, H; Augustin, H G; Dehio, C
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.
Franz, Bettina; Kempf, Volkhard A J
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.
O'Rourke, Fiona; Schmidgen, Thomas; Kaiser, Patrick O; Linke, Dirk; Kempf, Volkhard A J
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.
Luís Rafael Moscote Salazar
Full Text Available Resumen La cervicobraquialgia es el dolor cervical irradiado a una de las extremidades superiores. Es un síndrome clínico que puede estar causado por diversas patologías. El síndrome de Coob consiste en la asociación de una malformación vascular que compromete la piel y malformación vascular de la medula espinal. Presentamos el caso de un paciente con cervicobraquialgia y síndrome de Coob manejado por el Servicio de Neurocirugía del Hospital Universitario CARI en Barranquilla, Colombia. (DUAZARY 2010, 88 - 90AbstractThe cervicobraquialgia is neck pain radiating to the upper extremities. it is a clinical syndrome that can be caused by various pathologies. Cobb syndrome is the association of a vascular malformation that involves the skin and vascular malformation of the spinal cord. We present a patient with cobb syndrome cervicobraquialgia and managed by the department of neurosurgery, university hospital Cari in Barranquilla, Colombia.Keywords: cobb syndrome; cervicobraquialgia; spinal vascular malformation; angiomatosis meningocutaneoespinal.
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.
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)
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.)
Diniz, Pedro Paulo Vissotto de Paiva; Velho, Paulo Eduardo Neves Ferreira; Pitassi, Luiza Helena Urso; Drummond, Marina Rovani; Lania, Bruno Grosselli; Barjas-Castro, Maria Lourdes; Sowy, Stanley; Breitschwerdt, Edward B; Scorpio, Diana Gerardi
Bacteria from the genus Bartonella are emerging blood-borne bacteria, capable of causing long-lasting infection in marine and terrestrial mammals, including humans. Bartonella are generally well adapted to their main host, causing persistent infection without clinical manifestation. However, these organisms may cause severe disease in natural or accidental hosts. In humans, Bartonella species have been detected from sick patients presented with diverse disease manifestations, including cat scratch disease, trench fever, bacillary angiomatosis, endocarditis, polyarthritis, or granulomatous inflammatory disease. However, with the advances in diagnostic methods, subclinical bloodstream infection in humans has been reported, with the potential for transmission through blood transfusion been recently investigated by our group. The objective of this study was to determine the risk factors associated with Bartonella species infection in asymptomatic blood donors presented at a major blood bank in Southeastern Brazil. Five hundred blood donors were randomly enrolled and tested for Bartonella species infection by specialized blood cultured coupled with high-sensitive PCR assays. Epidemiological questionnaires were designed to cover major potential risk factors, such as age, gender, ethnicity, contact with companion animals, livestock, or wild animals, bites from insects or animal, economical status, among other factors. Based on multivariate logistic regression, bloodstream infection with B. henselae or B. clarridgeiae was associated with cat contact (adjusted OR: 3.4, 95% CI: 1.1-9.6) or history of tick bite (adjusted OR: 3.7, 95% CI: 1.3-13.4). These risk factors should be considered during donor screening, as bacteremia by these Bartonella species may not be detected by traditional laboratory screening methods, and it may be transmitted by blood transfusion.
Pedro Paulo Vissotto de Paiva Diniz
Full Text Available Bacteria from the genus Bartonella are emerging blood-borne bacteria, capable of causing long-lasting infection in marine and terrestrial mammals, including humans. Bartonella are generally well adapted to their main host, causing persistent infection without clinical manifestation. However, these organisms may cause severe disease in natural or accidental hosts. In humans, Bartonella species have been detected from sick patients presented with diverse disease manifestations, including cat scratch disease, trench fever, bacillary angiomatosis, endocarditis, polyarthritis, or granulomatous inflammatory disease. However, with the advances in diagnostic methods, subclinical bloodstream infection in humans has been reported, with the potential for transmission through blood transfusion been recently investigated by our group. The objective of this study was to determine the risk factors associated with Bartonella species infection in asymptomatic blood donors presented at a major blood bank in Southeastern Brazil. Five hundred blood donors were randomly enrolled and tested for Bartonella species infection by specialized blood cultured coupled with high-sensitive PCR assays. Epidemiological questionnaires were designed to cover major potential risk factors, such as age, gender, ethnicity, contact with companion animals, livestock, or wild animals, bites from insects or animal, economical status, among other factors. Based on multivariate logistic regression, bloodstream infection with B. henselae or B. clarridgeiae was associated with cat contact (adjusted OR: 3.4, 95% CI: 1.1-9.6 or history of tick bite (adjusted OR: 3.7, 95% CI: 1.3-13.4. These risk factors should be considered during donor screening, as bacteremia by these Bartonella species may not be detected by traditional laboratory screening methods, and it may be transmitted by blood transfusion.
Lu, Yun-Yueh; Franz, Bettina; Truttmann, Matthias C; Riess, Tanja; Gay-Fraret, Jérémie; Faustmann, Marco; Kempf, Volkhard A J; Dehio, Christoph
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
diagnosis is wide, and include bacillary angiomatosis, cancers or metastasis. Chemotherapy and antiretroviral treatment did not affect bone lesions using CT scan despite a good response on other KS-affected sites. Prognostic factors are well established in AIDS-associated KS ; however disseminated bone disease does not seem to have an impact on disease evolution. A larger sample size is needed to confirm this hypothesis.
--- Various Authors
. 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