scholarly journals A Case Report of Vogt's Limbal Girdle and Retinitis Pigmentosa in a Thirteen-Year-Old Boy: A Rare and Unusual Association

2015 ◽  
Vol 6 (3) ◽  
pp. 311-316
Author(s):  
A.P. Vignesh ◽  
Renuka Srinivasan ◽  
Swathi Karanth ◽  
Sai Vijitha

Aim: To describe a rare case of Vogt's limbal girdle in a boy with retinitis pigmentosa. Methods: A 13-year-old boy from India presented to us with progressive diminution of vision and nyctalopia for 5 years. On examination, he had the characteristic features of retinitis pigmentosa with the fundus showing disc pallor, bony spicules and arteriolar attenuation. His anterior segment examination showed Vogt's limbal girdle in both eyes. Results: Vogt's limbal girdle is a corneal degeneration usually seen in elderly individuals. This is the first time it is seen in association with retinitis pigmentosa. It has also never been reported at such a young age. Conclusion: We report a rare case where Vogt's limbal girdle was observed in a 13-year-old boy with retinitis pigmentosa. This gives further insight into the pathogenesis of the disease.

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Aisha Khoja ◽  
Mubassar Fida ◽  
Attiya Shaikh

Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone disorders. Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients. This will guide them in planning realistic treatment goals. This paper reports the clinical and radiographic features of pycnodysostosis with the great emphasis on its dentofacial characteristics. The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients.


Author(s):  
kazem ghaemi ◽  
mahdieh rajabi-moghaddam ◽  
Hamid Abbaszadeh

Primary intracranial primitive neuroectodermal tumors (PNETs) are extremely rare malignancies, affects children and adolescents with only 10 cases has been reported over 33 years old. we present a case of PNET in a 36 years old female patient with the chief complaint of aphasia for the first time


Author(s):  
В. Л. Мыц ◽  
С. Л. Соловьев ◽  
А. Ф. Покровская ◽  
Н. Ф. Соловьева

В 2018-2019 гг. основным объектом исследований Севастопольской экспедиции ИИМК РАН стали оборонительные сооружения, расположенные на Историческом бульваре г. Севастополя. Археологические раскопки Четвертого бастиона позволили проследить историю его строительства. Впервые были получены достоверные данные о местоположении, характере и конструкции основных элементов фортификации правого фланга 4-го бастиона: валганга, горжи, эскарпа и контрэскарпа. Открыты орудийные дворики, части бруствера с орудийными амбразурами, вспомогательные площадки для размещения боеприпасов, погреба для хранения пороха и бомб, блиндажи для личного состава. Во рву обнаружены вырубленные в скале навесы-убежища и 14 входов в контрминные галереи, потайной ход для скрытного перемещения войск. В ходе раскопок собрана представительная археологическая коллекция предметов войны и быта защитников бастиона и их противников, достойная пополнить экспозиции музеев Севастополя, посвященных его обороне в годы Крымской войны. В 2019 г. специалистами ИИМК РАН и Эрмитажа разработана и представлена в Министерство культуры РФ концепция музеефикации и приспособления для современного использования выявленных сооружений в районе правого фаса 4-го бастиона на Мемориальном комплексе памятников обороны города в 1854-1855 гг. «Исторический бульвар» (г. Севастополь). In 2018-2019 defensive fortifications located in the Istoricheskiy (Historical) Boulevard of Sevastopol (Fig. 1) became the main object of excavations carried out by the Sevastopol expedition of the Institute for the History of Material Culture (RAS). The archaeological excavations of the fourth bastion provide an insight into the history of its construction. Reliable data on location, characteristic features and technical design of the key fortification elements of the bastion right flank such as terreplein, neck line, escarp and counterscarp were obtained for the first time. The excavations revealed gun positions, parts of breastwork with gun ports, auxiliary areas for ammunition storage, a magazin for gun powder and bombs, dugout shelters for manpower. Shelter awnings cut out in rock and 14 entries into countermine galleries as well as a secret passage for surreptitious movements of troops (Fig. 3; 4) were identified. A representative archaeological assembly of war items and paraphernalia of bastion defenders and their enemy was collected during the excavations. It will replenish expositions of the Sevastopol museums dedicated to its defense in the Crimean war (Figs. 5-8). In 2019 specialists of the Institute for the History of Material Culture prepared a concept note for museification and adaptive use of constructions discovered in the right side of the fourth bastion at the 1854-1855 Memorial Ensemble of the City Defense Sites known as ‘the Historical Boulevard' (Sevastopol). The concept note was forwarded to the Russian Ministry of Culture.


2013 ◽  
Vol 5 (2) ◽  
pp. 83-84
Author(s):  
Pooja Singhania ◽  
Deepti Shrivastava ◽  
Anuradha Kakani ◽  
Juhee Papalkar

ABSTRACT Prune belly syndrome is a congenital abnormality of unknown etiology with characteristic features: Deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune, cryptorchidism, abnormalities of the urinary tract. It is associated with high incidence of fetal and neonatal mortality. It can be diagnosed at 16 weeks during routine ultrasound and can be managed by fetal therapy. In rural setup women are neither visiting for routine anomaly scan nor affording for fetal therapy, hence termination of pregnancy remains the only option. How to cite this article Papalkar J, Shrivastava D, Kakani A, Singhania P. A Rare Case Report of Prune Belly Syndrome. J South Asian Feder Obst Gynae 2013;5(2):83-84.


2017 ◽  
Vol 145 (5-6) ◽  
pp. 294-295
Author(s):  
Marija Bozic ◽  
Vujica Markovic ◽  
Ivan Marjanovic ◽  
Vesna Maric

Introduction. This is an observational case report presenting retinitis pigmentosa associated with pseudoexfoliative glaucoma. Case outline. A 69-year-old man presented with retinitis pigmentosa. On examination, pseudoexfoliative material was detected on anterior segment structures, and intraocular pressure was 26 mmHg in the right and 24 mmHg in the left eye. The patient was commenced on topical antiglaucomatous therapy (timolol + dorzolamide twice daily, latanoprost once in the evening) to both eyes. Conclusion. To the best of our knowledge, this is the first reported case of retinitis pigmentosa associated with pseudoexfoliative glaucoma. Although rare, retinitis pigmentosa and glaucoma can occur in the same eye.


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