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[PMID]: 27472202
[Au] Autor:Pasyanthi B; Mendonca T; Sachdeva V; Kekunnaya R
[Ad] Address:Jasti V Ramanamma Children's Eye Care Centre, Pediatric Ophthalmology, Strabismus and Neuro-ophthalmology Services, L V Prasad Eye Institute, KAR Campus, Hyderabad, India.
[Ti] Title:Ophthalmologic manifestations of Hallermann-Streiff-Francois syndrome: report of four cases.
[So] Source:Eye (Lond);30(9):1268-71, 2016 Sep.
[Is] ISSN:1476-5454
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:PurposeHallermann-Streiff-Francois syndrome (HSS) is a rare genetic disorder characterised by ocular and craniofacial anomalies. The purpose of this report is to highlight the ophthalmological features in four such patients and outcomes of cataract surgery.Patients and methodsRetrospective review of medical records of patients with cataract and/or microcornea due to HSS was done. Presenting features, ocular findings, ocular motility and visual outcomes were noted.ResultsWe identified four children with microcornea/cataract who had associated clinical features suggestive of HSS. Mean age at presentation was 25.5±27.8 months. Three children presented with poor vision in both eyes and one with strabismus. All patients had a microcornea and microphthalmos. Three patients had a membranous cataract. Horizontal corneal diameter ranged from 5.5 to 10.5 mm and axial length ranged from 12 to 18 mm. Three patients had associated strabismus. Three patients underwent lens extraction and two underwent strabismus surgery. Best corrected visual acuity (BCVA) improved from fixing, following light to a median post-operative BCVA of 20/380. One eye developed retinal detachment.ConclusionChildren with HSS present with membranous cataracts, microcornea and microphthalmos and present surgical challenges. Though the patients were mostly left aphakic, all showed moderate visual improvement.
[Mh] MeSH terms primary: Cataract/diagnosis
Corneal Diseases/diagnosis
Hallermann´s Syndrome/diagnosis
Microphthalmos/diagnosis
[Mh] MeSH terms secundary: Cataract Extraction
Child, Preschool
Corneal Diseases/surgery
Female
Humans
Infant
Male
Retrospective Studies
Strabismus/diagnosis
Vision Disorders/diagnosis
Visual Acuity/physiology
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1703
[Cu] Class update date: 170902
[Lr] Last revision date:170902
[Js] Journal subset:IM
[Da] Date of entry for processing:160730
[St] Status:MEDLINE
[do] DOI:10.1038/eye.2016.161

  2 / 186 MEDLINE  
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[PMID]: 26865807
[Au] Autor:Mohebbi M; Shadravan M; Pour EK; Ameli K; Badiei S
[Ad] Address:Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
[Ti] Title:Brown-McLean Syndrome in a Patient with Hallermann-Streiff Syndrome.
[So] Source:Korean J Ophthalmol;30(1):76-7, 2016 Feb.
[Is] ISSN:2092-9382
[Cp] Country of publication:Korea (South)
[La] Language:eng
[Mh] MeSH terms primary: Cataract Extraction/adverse effects
Corneal Edema/etiology
Corneal Stroma/pathology
Epithelium, Corneal/pathology
Hallermann´s Syndrome/complications
[Mh] MeSH terms secundary: Adult
Corneal Edema/diagnosis
Female
Hallermann's Syndrome/diagnosis
Humans
Lens Implantation, Intraocular
[Pt] Publication type:CASE REPORTS; LETTER
[Em] Entry month:1612
[Cu] Class update date: 170220
[Lr] Last revision date:170220
[Js] Journal subset:IM
[Da] Date of entry for processing:160212
[St] Status:MEDLINE
[do] DOI:10.3341/kjo.2016.30.1.76

  3 / 186 MEDLINE  
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[PMID]: 26194770
[Au] Autor:Bénateau H; Rocha CS; Rocha Fde S; Veyssiere A
[Ad] Address:Department of Oral and Maxillofacial Surgery, Caen University Hospital, Caen, France; Laboratory EA 4652 Microenvironnement Cellulaire et Pathologies, University of Caen Basse-Normandie, Caen, France.
[Ti] Title:Treatment of the nasal abnormalities of Hallermann-Streiff syndrome by lipofilling.
[So] Source:Int J Oral Maxillofac Surg;44(10):1246-9, 2015 Oct.
[Is] ISSN:1399-0020
[Cp] Country of publication:Denmark
[La] Language:eng
[Ab] Abstract:Hallermann-Streiff syndrome (HSS) is a rare congenital abnormality affecting mostly the head and face area. Craniofacial deformities, which are present in 98-99% of cases, are the principal abnormalities detected. We focus here on a particular subset of these deformities: atrophy of the skin of the centre of the face and nose. Two patients diagnosed with HSS were treated in our department. Both patients underwent nasal lipofilling to treat the atrophy of the nasal skin, as described by Nguyen et al. In both cases, a satisfactory improvement in nasal skin colour and texture was observed. A functional gain was also reported by the parents and observed during follow-up consultations. Lipofilling thus appears to be an excellent option for treating craniofacial deformities in children.
[Mh] MeSH terms primary: Adipose Tissue/transplantation
Hallermann´s Syndrome/surgery
Nose/abnormalities
Nose/surgery
[Mh] MeSH terms secundary: Child
Child, Preschool
Humans
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1609
[Cu] Class update date: 150911
[Lr] Last revision date:150911
[Js] Journal subset:D; IM
[Da] Date of entry for processing:150722
[St] Status:MEDLINE

  4 / 186 MEDLINE  
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[PMID]: 25966733
[Au] Autor:Gungor OE; Nur BG; Yalcin H; Karayilmaz H; Mihci E
[Ti] Title:Comprehensive dental management in a Hallermann-Streiff syndrome patient with unusual radiographic appearance of teeth.
[So] Source:Niger J Clin Pract;18(4):559-62, 2015 Jul-Aug.
[Is] ISSN:1119-3077
[Cp] Country of publication:India
[La] Language:eng
[Ab] Abstract:Hallermann-Streiff syndrome (HSS) is a genetic disorder characterized by proportionate dwarfism, birdlike facies, hypotrichosis, skin atrophy, dyscephaly, bilateral microphthalmia, congenital cataracts, a narrow, weak, beaked nose, a hypoplastic mandible, and orodental anomalies. Occurrence is sporadic and distinct patterns of inheritance have not been found. This case report describes the dental management of a 3-year-old girl patient with HSS, who had unusual radiographic appearance of teeth. Furthermore, dental treatments and a 30-month follow-up period of the patient with this rare tooth structure malformation have been presented.
[Mh] MeSH terms primary: Denture, Partial, Removable
Hallermann´s Syndrome/complications
Malocclusion/therapy
[Mh] MeSH terms secundary: Child, Preschool
Female
Hallermann's Syndrome/diagnostic imaging
Hallermann's Syndrome/therapy
Humans
Malocclusion/diagnostic imaging
Malocclusion/etiology
Radiography
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1603
[Cu] Class update date: 161125
[Lr] Last revision date:161125
[Js] Journal subset:IM
[Da] Date of entry for processing:150514
[St] Status:MEDLINE
[do] DOI:10.4103/1119-3077.156910

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[PMID]: 24168356
[Au] Autor:Singh AL; Chandak M; Jain D; Mogre R
[Ad] Address:Department of Dermatology, Venereology and Leprosy, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (DMIMS), Sawangi (Meghe) Wardha, Maharastra, India.
[Ti] Title:Hallermann-Streiff syndrome with cutaneous manifestations.
[So] Source:Int J Dermatol;54(9):1068-70, 2015 Sep.
[Is] ISSN:1365-4632
[Cp] Country of publication:England
[La] Language:eng
[Mh] MeSH terms primary: Alopecia/etiology
Cafe-au-Lait Spots/etiology
Hallermann´s Syndrome/complications
[Mh] MeSH terms secundary: Alopecia/physiopathology
Cafe-au-Lait Spots/physiopathology
Child
Hallermann's Syndrome/diagnosis
Humans
Male
Rare Diseases
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1605
[Cu] Class update date: 150819
[Lr] Last revision date:150819
[Js] Journal subset:IM
[Da] Date of entry for processing:131031
[St] Status:MEDLINE
[do] DOI:10.1111/ijd.12172

  6 / 186 MEDLINE  
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[PMID]: 25047934
[Au] Autor:Damasceno JX; Couto JL; Alves KS; Chaves CM; Costa FW; Pimenta Ade M; Fonteles CS
[Ad] Address:PhD student, Postgraduate Program in Dentistry, Federal University of Ceará, Fortaleza, Ceará, Brazil.
[Ti] Title:Generalized odontodysplasia in a 5-year-old patient with Hallermann-Streiff syndrome: clinical aspects, cone beam computed tomography findings, and conservative clinical approach.
[So] Source:Oral Surg Oral Med Oral Pathol Oral Radiol;118(2):e58-64, 2014 Aug.
[Is] ISSN:2212-4411
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:This article aims to report the main clinical aspects, cone beam computed tomography (CBCT) findings, and conservative oral rehabilitation in a child born from a consanguineous marriage who presented with Hallermann-Streiff syndrome (HSS) and generalized odontodysplasia. A 5-year-old girl presented with a diagnosis of HSS for oral evaluation. Radiographically, all teeth showed wide pulp chambers and roots with thin dentinal walls and open apices, resembling ghost teeth and indicating a diagnosis of odontodysplasia. Oral rehabilitation consisted of partial dentures that were regularly adjusted to conform the device with the pattern of growth and development of the child. CBCT scan provided great insight into HSS, allowing a detailed view of the morphologic aspects and associated trabecular bone pattern. Treatment of these 2 rare conditions in young children must consider the stage of growth and development. Although extremely rare in HSS, odontodysplasia should be investigated and conservatively managed in young children.
[Mh] MeSH terms primary: Cone-Beam Computed Tomography
Hallermann´s Syndrome/complications
Hallermann´s Syndrome/diagnostic imaging
Odontodysplasia/diagnostic imaging
Odontodysplasia/etiology
[Mh] MeSH terms secundary: Child, Preschool
Female
Hallermann's Syndrome/therapy
Humans
Odontodysplasia/therapy
Radiography, Panoramic
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1504
[Cu] Class update date: 161125
[Lr] Last revision date:161125
[Js] Journal subset:D; IM
[Da] Date of entry for processing:140723
[St] Status:MEDLINE

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[PMID]: 24601178
[Au] Autor:Daka Q; Miftari A; Vuciterrna A; Shiroka L; Bucinca S; Capuni-Brestovci M
[Ad] Address:University Clinical Centre-Eye Clinic "Mother Theresa" Prishtina, Republic of Kosovo. qendrese@hotmail.com
[Ti] Title:Hallermann-Streiff syndrome without cataract: case report from Kosova.
[So] Source:Med Arch;67(5):378-80, 2013.
[Is] ISSN:0350-199X
[Cp] Country of publication:Bosnia and Herzegovina
[La] Language:eng
[Ab] Abstract:Hallermann-Streiff syndrome is a rare congenital disorder characterized as a complex association of developmental anomalies involving structures of ectodermal origin (the face, the scull, the hair, the skin, the eyes, the teeth) and overall growth and development. Ophthalmic abnormalities included bilateral congenital cataract. Glaucoma is uncommon. We report a case with Hallermann-Streiffsyndrome having all the main features of the syndrome, however associated with juvenile glaucoma and without congenital cataract. An 16 year-old-boy was admitted in our hospital for surgical treatment of juvenile glaucoma. His ophthalmic features included microphthalmos, enophthalmos, short upper lid, and thin conjunctiva, edematous corneas with fine epithelial bulla, atrophic-colobomatous iris in the left eye, slightly dilated pupils and clear lenses. The right optic disc was pale and cupped (0.9-1). The left optic disc was pale. The IOP was 70 mmHg in the right eye and 62 mmHg in the left eye. There was no light perception on the right eye and 1/60 in the left improving to 0.1 with +7.0 Dsph. Antiglaucomatous therapy failed to reduce the pressure and a left trabeculecthomy was carried out. Protective eye shields during sleep were recommended to protect the corneas from dryness because of the short upper lids. In the left eye the IOP has fallen to within normal limits and the vision has improved 0.3-0.4 with +7.0 Dsph. The medical therapy was continued in the right eye but the pressure still remains high (40 mmHg).
[Mh] MeSH terms primary: Hallermann´s Syndrome/diagnosis
[Mh] MeSH terms secundary: Adolescent
Glaucoma/etiology
Hallermann's Syndrome/complications
Humans
Kosovo
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1404
[Cu] Class update date: 150901
[Lr] Last revision date:150901
[Js] Journal subset:IM
[Da] Date of entry for processing:140308
[St] Status:MEDLINE

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[PMID]: 23728903
[Au] Autor:Muthugaduru DJ; Sahu C; Ali MJ; Dalal A; Jalali S
[Ad] Address:Srimati Kannuri Santhamma Centre for Vitreoretinal diseases, L V Prasad Eye Institute, Kallam Anji Reddy campus Banjara Hills, Hyderabad, 500034, India.
[Ti] Title:Report on ocular biometry of microphthalmos, retinal dystrophy, flash electroretinography, ocular coherence tomography, genetic analysis and the surgical challenge of entropion correction in a rare case of Hallermann-Streiff-Francois syndrome.
[So] Source:Doc Ophthalmol;127(2):147-53, 2013 Oct.
[Is] ISSN:1573-2622
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:PURPOSE: To report new aspects of the phenotype including Retinal dystrophy and surgical challenges in Hallermann-Streiff Francois syndrome (HSFS). METHODS: Detailed phenotype of a female with HSFS was evaluated including skeletal changes, comprehensive eye examination, detailed ocular biometry, electroretinography and macular Ocular coherence tomography. Surgical notes of lid surgery for entropion were reviewed. Genetic screening was also done. RESULTS: Unique Ocular biometry with electroretinography changes, macular folds and fundus changes suggestive of an unreported Retinal dystrophy in a typical patient with HSFS were noted. Surgery was challenging both due to difficulty in endotracheal intubation anaesthesia because of the dento-facial abnormalities and the skin fragility. CONCLUSION: This report provides additional information especially pigmentary retinal dystrophy, macular folds and electroretinography in HSFS. The microphthalmos had overlapping posterior segment findings usually reported with Nanophthalmos and Posterior microphthalmos. The surgical difficulties and outcomes of the rarely encountered adnexal abnormalities emphasize the need for a multi disciplinary approach for appropriate management.
[Mh] MeSH terms primary: Electroretinography
Entropion/etiology
Hallermann´s Syndrome/complications
Microphthalmos/etiology
Retinal Dystrophies/etiology
[Mh] MeSH terms secundary: Biometry
Connexin 43/genetics
Entropion/surgery
Female
Hallermann's Syndrome/genetics
Hallermann's Syndrome/physiopathology
Humans
Microphthalmos/genetics
Microphthalmos/physiopathology
Ophthalmologic Surgical Procedures
Phenotype
Photic Stimulation
Retina/physiopathology
Retinal Dystrophies/genetics
Retinal Dystrophies/physiopathology
Tomography, Optical Coherence
Tomography, X-Ray Computed
Visual Acuity/physiology
Young Adult
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Name of substance:0 (Connexin 43); 0 (GJA1 protein, human)
[Em] Entry month:1403
[Cu] Class update date: 171019
[Lr] Last revision date:171019
[Js] Journal subset:IM
[Da] Date of entry for processing:130604
[St] Status:MEDLINE
[do] DOI:10.1007/s10633-013-9389-7

  9 / 186 MEDLINE  
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[PMID]: 22996707
[Au] Autor:Goddard JC; Oliver ER; Meyer TA
[Ad] Address:House Ear Clinic, Los Angeles, CA 90057, USA. jgoddard@hei.org
[Ti] Title:Bilateral posterior semicircular canal dehiscence in the setting of Hallermann-Streiff syndrome.
[So] Source:Ear Nose Throat J;91(9):360, 362-3, 2012 Sep.
[Is] ISSN:1942-7522
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Hallermann-Streiff syndrome, also known as oculomandibulofacial syndrome, is a rare congenital disorder affecting growth and cranial, dental, ocular, pilocutaneous, and mental development. In addition to routine audiologic testing in patients with this syndrome, high-resolution computed tomography of the temporal bones should be performed in those with documented or suspected sensorineural hearing loss. Cochlear implantation may be considered, as in other children with syndromic hearing loss and certain inner ear abnormalities, if the appropriate audiologic, psychosocial, and medical criteria are met. The current case report radiographically and clinically characterizes inner ear dysplasia in an 8-year-old patient with Hallermann-Streiff syndrome. High-resolution computed tomography of the temporal bones revealed a hypoplastic bony island between the vestibule and horizontal semicircular canals, as well as incomplete bony coverage of the posterior semicircular canal crura bilaterally. To our knowledge, this is the first report of a pediatric patient demonstrating bilateral posterior semicircular canal dehiscence.
[Mh] MeSH terms primary: Ear, Inner/abnormalities
Hallermann´s Syndrome/diagnostic imaging
Semicircular Canals/abnormalities
[Mh] MeSH terms secundary: Audiometry, Pure-Tone
Bone Diseases, Developmental/complications
Bone Diseases, Developmental/diagnostic imaging
Child
Ear, Inner/diagnostic imaging
Hallermann's Syndrome/complications
Hearing Loss, Sensorineural/complications
Humans
Male
Semicircular Canals/diagnostic imaging
Tomography, X-Ray Computed
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1303
[Cu] Class update date: 161125
[Lr] Last revision date:161125
[Js] Journal subset:IM
[Da] Date of entry for processing:120922
[St] Status:MEDLINE

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[PMID]: 22842271
[Au] Autor:Parikh S; Gupta S
[Ad] Address:Department of Oral Medicine and Radiology, Government Dental College and Hospital, Ahmedabad, Gujarat, India.
[Ti] Title:Orodental findings in Hallermann-Streiff syndrome.
[So] Source:Indian J Dent Res;23(1):124, 2012 Jan-Feb.
[Is] ISSN:1998-3603
[Cp] Country of publication:India
[La] Language:eng
[Ab] Abstract:Hallermann-Streiff syndrome-also called occulomandibulofacial syndrome, Francois syndrome, oculomandibulodyscephaly with hypotrichosis, Aubry syndrome I, and Ullrich-Fremery-Dohna syndrome-is a rare genetic disorder, which comprisesmultiple congenital abnormalities affecting chiefly the head and face. It is characterized by bird-like facies, dental abnormalities, hypotrichosis, atrophy of skin, congenital cataracts, bilateral microphthalmia, and proportionate nanism. An interesting case of Hallermann-Streiff syndrome in a 23-year-old female patient is reported here, with the emphasis on the orodental findings.
[Mh] MeSH terms primary: Hallermann´s Syndrome/pathology
Tooth Abnormalities/pathology
[Mh] MeSH terms secundary: Dental Caries/pathology
Dental Enamel Hypoplasia/pathology
Facies
Female
Humans
Mandible/abnormalities
Syndactyly/pathology
Temporal Bone/abnormalities
Temporomandibular Joint/abnormalities
Young Adult
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1303
[Cu] Class update date: 120730
[Lr] Last revision date:120730
[Js] Journal subset:D
[Da] Date of entry for processing:120731
[St] Status:MEDLINE
[do] DOI:10.4103/0970-9290.99063


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