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    28 results match your criteria Haberland Syndrome

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    Encephalocraniocutaneous lipomatosis: A case report with review of literature.
    Neuroradiol J 2017 Jan 1:1971400917693638. Epub 2017 Jan 1.
    1 Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Aligarh Muslim University, India.
    Encephalocraniocutaneous lipomatosis (ECCL) or Haberland syndrome is an uncommon sporadic neurocutaneous syndrome of unknown origin. The rarity and common ignorance of the condition often makes diagnosis difficult. The hallmark of this syndrome is the triad of skin, ocular and central nervous system (CNS) involvement and includes a long list of combination of conditions. Read More

    Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report.
    Indian Dermatol Online J 2016 Nov-Dec;7(6):523-525
    Department of Dermatology, B.J. Medical College, Civil Hospital, Ahmedabad, Gujarat, India.
    Haberland syndrome or Fishman syndrome also known as encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital neurocutaneous disorder. It is characterized by unilateral involvement of skin, eyes and central nervous system. We report the case of a 28-year-old woman who presented with soft lipomatous swelling over right temporal area with nonscarring alopecia of part of frontal and parietal region. Read More

    Low prevalence of patients with mitochondrial disease in the German/Austrian DPV diabetes registry.
    Eur J Pediatr 2016 May 15;175(5):613-22. Epub 2015 Dec 15.
    Institute of Epidemiology and Medical Biometry, ZIBMT, University of Ulm, German Center for Diabetes Research (DZD), 89081, Ulm, Germany.
    Unlabelled: The aim of this study was to characterize the phenotype and treatment of young patients (manifestation <30 years) with diabetes of mitochondrial origin (DMO), based on the German/Austrian DPV (Diabetes Patienten Verlaufsdokumentation) registry. Only 13 (0.02 %) of all patients with diabetes in this cohort were identified with DMO, mainly due to the Kearns-Sayre (n = 5), Pearson (n = 3), or mitochondrial myopathy, encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome (n = 2). Read More

    Osteonecrosis of the Jaw in Association With Chemotherapy in the Setting of Cutaneous T-Cell Lymphoma.
    J Oral Maxillofac Surg 2016 Feb 3;74(2):292-301. Epub 2015 Aug 3.
    Residency Program Director, Division of Oral and Maxillofacial Surgery, Yale-New Haven Hospital, New Haven, CT.
    T-cell lymphomas (TCLs) account for approximately 15 to 20% of all non-Hodgkin lymphomas in the United States. The most common form of TCL is cutaneous TCL (CTCL), with Sézary syndrome and mycosis fungoides being the most prevalent subtypes. Sézary syndrome is the more aggressive form and often is referred to as a late-stage variant of mycosis fungoides. Read More

    Encephalocraniocutaneous lipomatosis, a rare neurocutaneous disorder: report of additional three cases.
    Childs Nerv Syst 2016 Mar 1;32(3):559-62. Epub 2015 Aug 1.
    Department of Pediatric Neurology, Eskisehir Osmangazi University Hospital, Meselik, Eskisehir, Turkey.
    Purpose: Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital neurocutaneous disorder. It was described by Haberland in 1970 and is also called Haberland syndrome. It is characterized by unilateral skin lesions such as lipomas, connective tissue nevi, and alopecia with ipsilateral ophthalmological and cerebral malformations with or without psychomotor and mental retardation and early-onset seizure. Read More

    Encephalocraniocutaneous Lipomatosis: A Rare Association With Tethered Spinal Cord Syndrome With Review of Literature.
    Child Neurol Open 2015 Jan-Mar;2(1):2329048X14553297. Epub 2015 Feb 13.
    Department of Pediatrics, Makassed General Hospital, Beirut, Lebanon.
    Encephalocraniocutaneous lipomatosis or Haberland syndrome is a rare, congenital neurocutaneous syndrome. It is characterized by unilateral lipomatous hamartomata of the scalp, eyelid, and outer globe of the eye and ipsilateral neurologic malformations. We describe the first case from Lebanon, an infant with classical encephalocraniocutaneous lipomatosis characterized by nevus psiloliparus, unilateral right facial and frontal-temporal subcutaneous lipomas, alopecia, ocular coloboma, aniridia and eyelid nodular tags, ventriculomegaly with intracranial and intraspinal lipomas, and tethered spinal cord. Read More

    Encephalocraniocutaneous lipomatosis with calvarial exostosis - Case report and review of literature.
    Indian J Radiol Imaging 2013 Oct;23(4):333-6
    Department of Radiodiagnosis, Indira Gandhi Medical College and Hospital, Shimla, Himachal Pradesh, India.
    Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland syndrome, is a rare syndrome with unknown etiology. The syndrome is characterized by a triad of unique cutaneous, ocular, and central nervous system (CNS) manifestations. The cutaneous hallmark, nevus psiloliparus (NP), along with overlying alopecia is a constant feature. Read More

    Persistent multifocal atrial tachycardia in infant with encephalocraniocutaneous lipomatosis: a case report.
    Eur J Pediatr 2014 Dec 14;173(12):1577-80. Epub 2013 Aug 14.
    Department of Cardiology, The Children's Memorial Health Institute, Dzieci Polskich 20, 04-730, Warsaw, Poland,
    Unlabelled: Encephalocraniocutaneous lipomatosis (ECCL, Haberland syndrome, Fishman syndrome) is a very rare congenital disorder, involving skin, eye, bone and central nervous system malformations. In this paper we present a case of a 2-month-old boy with encephalocraniocutaneous lipomatosis diagnosed on the basis of characteristic clinical manifestations and neuroimaging findings. Neurologically, the child presented only with mild physical and mental retardation. Read More

    Are Agonistic Autoantibodies against G-Protein Coupled Receptors Involved in the Development of Long-Term Side Effects of Tumor Chemotherapy?
    Case Rep Oncol 2013 Jan 21;6(1):104-8. Epub 2013 Feb 21.
    Charité - Universitätsmedizin Berlin, Berlin, Germany.
    Metabolic syndrome and cardiomyopathies are long-term consequences of chemo- and radiotherapy and develop long after completing the initial tumor treatment. The slow progression of such late effects might be an indication of the involvement of autoimmune processes in the development of such follow-up consequences. Functionally active autoantibodies, which permanently stimulate relevant cell receptors, might be a crucial component. Read More

    Encephalocraniocutaneous lipomatosis (ECCL): neuroradiological findings in three patients and a new association with fibrous dysplasia.
    Am J Med Genet A 2011 Jul 27;155A(7):1690-6. Epub 2011 May 27.
    Department of Imaging, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
    Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome characterized by involvement of tissues of ectodermal and mesodermal origin such as skin, eye, adipose tissue, and brain. Since 1970, when Haberland and Perou had described the first patient, 54 cases of ECCL have been reported in literature. We report on three new boys with ECCL. Read More

    A case of encephalocraniocutaneous lipomatosis syndrome with epilepsy (Haberland syndrome).
    Folia Med (Plovdiv) 2009 Oct-Dec;51(4):46-8
    Department of Neurology, Medical University, Plovdiv, Bulgaria.
    Encephalocraniocutaneous lipomatosis is a rare congenital neurocutaneous syndrome characterized by scalp, facial, and ocular lesions and multiple intracranial malformations. Approximately 50 cases have been described in the literature. We report a 34-year-old woman with a 6-year history of epilepsy, without mental retardation, with predominantly ipsilateral skin lesions evident at birth, with limbal lipodermoid of the left eye and multiple non-progressive, ipsilateral intracranial structures of soft, cystic components. Read More

    Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis.
    J Neurooncol 2010 Feb 4;96(3):437-41. Epub 2009 Aug 4.
    Division of Pediatric Oncology, Department of Pediatrics, University of São Paulo, São Paulo, Brazil.
    Encephalocutaneous lipomatosis (ECCL), or Haberland syndrome, is an uncommon congenital disorder with unique cutaneous, ocular and neurological features. In the present article, we describe a 3-year-old boy with ECCL who developed an extensive and recurring intraventricular low-grade glioma with atypical pathological features and elevated mitotic index. Cytogenetic analysis from tumor sample was also performed. Read More

    [Encephalocraniocutaneous lipomatosis (Haberland syndrome) with bilateral ocular damage].
    An Pediatr (Barc) 2007 Jun;66(6):619-21
    Unidad de Neonatología, Departamento de Pediatría, Hospital Clínico Universitario, Santiago de Compostela, España.
    Encephalocraniocutaneous lipomatosis, or Haberland syndrome, is an unusual form of neurocutaneous syndrome. Clinically, this syndrome is characterized by unilateral lipomatous hamartomas on the scalp and face, ocular damage and ipsilateral malformations of the central nervous system. First described by Haberland and Perou in 1970, only about 40 cases have been reported to date. Read More

    Mandibular osteoma in the encephalocraniocutaneous lipomatosis.
    J Craniomaxillofac Surg 2005 Aug;33(4):286-9
    Department of Cranio-Maxillofacial Surgery, Barlicki University Hospital, Medical University, Lodz, Poland.
    Introduction: Encephalocraniocutaneous lipomatosis is a rare congenital disorder characterized by an abnormal development of adipose tissue, and is of unknown pathogenesis. Catherine Haberland and Maurice Perou first diagnosed this disorder in 1970. To our knowledge, approximately 25 patients have been reported with this diagnosis but so far, only 1 patient has been reported in Poland by Roszkowski and Dabrowski in 1997. Read More

    Systematic review: surveillance systems for early detection of bioterrorism-related diseases.
    Ann Intern Med 2004 Jun;140(11):910-22
    University of California, San Francisco-Stanford Evidence-based Practice Center, Stanford University School of Medicine, Stanford, California, USA.
    Background: Given the threat of bioterrorism and the increasing availability of electronic data for surveillance, surveillance systems for the early detection of illnesses and syndromes potentially related to bioterrorism have proliferated.

    Purpose: To critically evaluate the potential utility of existing surveillance systems for illnesses and syndromes related to bioterrorism.

    Data Sources: Databases of peer-reviewed articles (for example, MEDLINE for articles published from January 1985 to April 2002) and Web sites of relevant government and nongovernment agencies. Read More

    Encephalocraniocutaneous lipomatosis (Haberland syndrome) with bilateral cutaneous and visceral involvement.
    Clin Exp Dermatol 2003 Jul;28(4):387-90
    Department of Dermatology, University of Siena, Siena, Italy.
    Encephalocraniocutaneous lipomatosis, or Haberland syndrome, is a rare congenital neurocutaneous disease. It is characterized clinically by unilateral lipomatous hamartomata of the scalp, eyelid, and outer globe of the eye, ipsilateral porencephalic cysts with cortical atrophy, cranial asymmetry, marked developmental delay and mental retardation. This syndrome should be distinguished from other mosaic neurocutaneous phenotypes such as as Delleman syndrome, Schimmelpenning syndrome, Goltz syndrome, Goldenhar syndrome and Proteus syndrome. Read More

    Referral patterns, lesion prevalence, and patient care parameters in a clinical oral pathology practice.
    Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999 May;87(5):583-8
    Oral and Maxillofacial Pathology, College of Dentistry, The Ohio State University, Columbus 43210, USA.
    Objective: The purpose of this study was to determine the following parameters in a referral-based private practice oral and maxillofacial pathology clinic: (1) sources of clinical referrals; (2) types of problems referred; and (3) clinical effectiveness of treatment.

    Study Design: Clinical charts were reviewed for a cohort of 362 patients seen over a 2 1/2-year period (1993-1995). From these charts, we determined the source of referral and the final diagnosis for each patient. Read More

    [Encephalo-cranio-cutaneous lipomatosis (ECCL) -- Haberland syndrome. A case report with review of the literature].
    Neurol Neurochir Pol 1997 May-Jun;31(3):607-13
    Oddziału Neurochirurgii Instytutu Pomnika Centrum Zdrowia Dziecka w Warszawie.
    Encephalocraniocutaneous lipomatosis (ECCL) is a congenital neurocutaneous disorder of the adipose tissue. The dominant features of the syndrome, include: unilateral cerebral malformation, ipsilateral scalp, face and eye lesions and convulsions beginning in infancy. The authors report a case of 13-year old girl with signs of ECCL syndrome treated surgically for to intractable epilepsy. Read More

    [The significance of AIDS for mortality in Berlin (West)].
    Gesundheitswesen 1994 Nov;56(11):611-6
    Robert Koch-Institut Berlin.
    Following a long-term decline, death rates due to infectious diseases in West-Berlin men have dramatically increased since 1983. The Berlin mortality statistics introduced AIDS as a specific cause of death only in 1989. However, opportunistic infections, a negligible quantity until 1983, and pointing to AIDS as the underlying cause, show a steep increase in the mid and late eighties. Read More

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