scholarly journals Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition

2019 ◽  
Vol 17 (1) ◽  
pp. 69-72
Author(s):  
Kompal Agarwal ◽  
Sudha Agrawal ◽  
Anju Pradhan
Keyword(s):  
Early Childhood ◽  
Rare Disease ◽  
Autosomal Dominant ◽  
Rare Condition ◽  
Palmoplantar Keratoderma ◽  
The Family ◽  
The Face ◽  
History Of ◽  
Erythematous Plaques ◽  
Systemic Abnormality

Progressive symmetrical erythrokeratodermia (PSEK) is a rare autosomal dominant genodermatosis presenting in infancy or early childhood. An 11-year-old male presented with a history of pruritic, erythematous, scaly, hyperkeratotic plaques first noted at 5 years of age, with no history of similar lesions in the family. Cutaneous examination revealed multiple, irregularly shaped, erythematous plaques with fine, shiny white, adherent scaling distributed symmetrically over the face, trunk, inguinal area, bilateral axillae and extensor surfaces of limbs. Palmoplantar keratoderma was present with thickened, yellowish discolored nails. No systemic abnormality was found. The histopathological findings were consistent with PSEK. The case is being reported to increase the awareness about this rare disease.

The Catholic Recusancy of the Yorkshire Fairfaxes. Part I.

1956 ◽  
Vol 3 (02) ◽  
pp. 68-114
Author(s):  
Hugh Aveling
Keyword(s):  
Sixteenth Century ◽  
Fifteenth Century ◽  
Middle Ages ◽  
Economic History ◽  
Personal Choice ◽  
Family Ties ◽  
The Family ◽  
The Face ◽  
History Of ◽  
Landed Gentry

In the middle ages the Fairfaxes ranked amongst the minor landed gentry of Yorkshire. They seem to have risen to this status in the thirteenth century, partly by buying land out of the profits of trade in York, partly by successful marriages. But they remained of little importance until the later fifteenth century. They had, by then, produced no more than a series of bailiffs of York, a treasurer of York Minster and one knight of the shire. The head of the family was not normally a knight. The family property consisted of the two manors of Walton and Acaster Malbis and house property in York. But in the later fifteenth century and onwards the fortunes of the family were in the ascendant and they began a process of quite conscious social climbing. At the same time they began to increase considerably in numbers. The three main branches, with al1 their cadet lines, were fixed by the middle of the sixteenth century – the senior branch, Fairfax of Walton and Gilling, the second branch, Fairfax of Denton, Nunappleton, Bilhorough and Newton Kyme, the third branch, Fairfax of Steeton. It is very important for any attempt to assess the strength and nature of Catholicism in Yorkshire to try to understand the strong family – almost clan – unity of these pushing, rising families. While adherence to Catholicism could be primarily a personal choice in the face of family ties and property interests, the history of the Faith in Yorkshire was conditioned greatly at every point by the strength of those ties and interests. The minute genealogy and economic history of the gentry has therefore a very direct bearing on recusant history.

scholarly journals Branchio-oto-renal syndrome: a clinical case

2019 ◽  
Vol 25 (1) ◽  
pp. 5
Author(s):  
Xavier Lagarde ◽  
Mathilde Fenelon ◽  
Jean Christophe Fricain
Keyword(s):  
Medical History ◽  
Rare Disease ◽  
Genetic Study ◽  
Clinical Case ◽  
Autosomal Dominant ◽  
Clinical Criteria ◽  
Dermatology Clinic ◽  
Family Medical History ◽  
The Family ◽  
Renal Abnormalities

Observation: A 19-week-old patient seen in an oral dermatology clinic had small labial commissures pits associated with auricular abnormalities. Similar clinical elements were found in the family medical history. A diagnosis of branchio-oto-renal syndrome (BOR) was quickly evoked. Commentary: BOR syndrome is a rare disease. This autosomal dominant pathology is characterized by facial lesions and renal abnormalities. Diagnosis is based on many clinical criteria. An optional genetic study can sometimes assist in diagnosis.

scholarly journals Hyperphalangy in a new sinemydid turtle from the Early Cretaceous Jehol Biota

PeerJ ◽  
2018 ◽  
Vol 6 ◽  
pp. e5371 ◽  
Author(s):  
Shuai Shao ◽  
Lan Li ◽  
Yang Yang ◽  
Chang-Fu Zhou
Keyword(s):  
Early Cretaceous ◽  
Evolutionary History ◽  
Rare Condition ◽  
Freshwater Turtle ◽  
Aquatic Environments ◽  
Jehol Biota ◽  
The Family ◽  
History Of ◽  
Primitive Condition ◽  
Phalangeal Formula

Hyperphalangy is a rare condition in extant aquatic turtles, and mainly limited to soft-shelled turtles. Here we report a new freshwater turtle,Jeholochelys lingyuanensisgen. et sp. nov. from the Early Cretaceous Jehol Biota of western Liaoning, China. This new turtle is characterized by a hyperphalangy condition with one additional phalanx in pedal digit V, rather than the primitive condition (phalangeal formula: 2-3-3-3-3) of crown turtles.J. lingyuanensisis recovered with other coexisting turtles in the family Sinemydidae in the phylogenetic analysis. This discovery further confirms that hyperphalangy occurred multiple times in the early evolutionary history of the crown turtles. Hyperphalangy is possibly a homoplasy inJeholochelysand the soft-shelled turtles to adapt to the aquatic environments.

Henry Solomon Wellcome, 1853-1936

1938 ◽  
Vol 2 (6) ◽  
pp. 229-238 ◽  
Keyword(s):  
Early Childhood ◽  
Garden City ◽  
Fine Chemicals ◽  
Medicinal Products ◽  
Native Land ◽  
American Citizen ◽  
The Family ◽  
The World ◽  
History Of ◽  
Rifle Bullets

Sir Henry Wellcome, who died on 25 July, 1936, at the age of eighty-three, was by birth an American citizen but transferred his interests to this country after a brief business career in his native land. It was in 1880 that he entered into partnership with Mr. S. M. Burroughs to found the firm of Burroughs Wellcome and Company, which developed rapidly and soon became known throughout the world for its manufacture of fine chemicals, alkaloids, and other medicinal products. Wellcome’s connexion with England, the land of his ancestors, was more firmly sealed in 1910, when be became a naturalized British subject. Henry Solomon Wellcome was born in 1853 in a log cabin about 125 miles from Milwaukee and spent his early childhood amongst the Dakota Indians. His father, the Rev. S. C. Wellcome, was an itinerant missionary who with his wife, Mary Curtis Wellcome, travelled throughout Wisconsin and Minnesota in a covered wagon, preaching to the Indians. In 1859 the family trekked westwards to Garden City, Blue Earth County, Minn., where it established its home shortly before the outbreak of the civil war. When young Wellcome was eight years old the great Sioux Indian rebellion occurred and led to the massacre of more than a thousand whites. He assisted in casting rifle bullets for the defence of the settlement and actually helped his uncle, Dr. J. B. Wellcome, in caring for the wounded. Wellcome’s early contact with the Indians found expression in a life-long sympathy for the Red Man, a sympathy which in after years led him to spend considerable sums of money and energy in fighting for what he considered to be the rights of a certain Alaskan tribe. In support of this mission he published in 1887 a history of the tribe under the title of The Story of Metlakhatala .

scholarly journals De novo arteriovenous malformation in a patient with hereditary hemorrhagic telangiectasia

2016 ◽  
Vol 17 (3) ◽  
pp. 330-335 ◽  
Author(s):  
Yusuke Shimoda ◽  
Toshiya Osanai ◽  
Naoki Nakayama ◽  
Satoshi Ushikoshi ◽  
Masaaki Hokari ◽  
...  
Keyword(s):  
Family History ◽  
Arteriovenous Malformation ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Autosomal Dominant ◽  
De Novo ◽  
The Family ◽  
Systemic Disorder ◽  
History Of ◽  
Cerebral Avms

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant systemic disorder characterized by the enlargement of capillaries, recurrent nosebleeds, and multiple arteriovenous malformations (AVMs). Although cerebral AVMs are traditionally considered to be congenital lesions, some reports have described de novo AVMs, which suggests that the authors believed them to be dynamic conditions. In this article, the authors describe the case of a 5-year-old boy with HHT in whom a de novo cerebral AVM was detected after a negative MRI result at 5 months. To the authors’ knowledge, this is the first report of a de novo AVM in a patient with HHT. In patients with a family history of HHT, de novo AVMs are possible, even when no lesions are detected at the first screening. Therefore, regular screenings need to be performed, and the family should be informed that AVMs could still develop despite normal MRI results.

scholarly journals Family and School Relationship during COVID-19 Pandemic: A Systematic Review

2021 ◽  
Vol 18 (21) ◽  
pp. 11710
Author(s):  
José Juan Carrión-Martínez ◽  
Cristina Pinel-Martínez ◽  
María Dolores Pérez-Esteban ◽  
Isabel María Román-Sánchez
Keyword(s):  
Systematic Review ◽  
Distance Learning ◽  
History Of Education ◽  
Classroom Learning ◽  
Education Systems ◽  
The Family ◽  
The Face ◽  
History Of ◽  
The Relationship ◽  
Present Systematic Review

Education systems worldwide have been affected by a sudden interruption in classroom learning because the coronavirus pandemic forced both the closure of all schools in March 2020 and the beginning of distance learning from home, thus compelling families, schools, and students to work together in a more coordinated fashion. The present systematic review was carried out following PRISMA guidelines. The main objective was to present critical information on the relationship between the family and the school in the face of the imposed distance learning scenario caused by COVID-19. A total of 25 articles dealing with the relationships established during the pandemic of any of the three agents involved (family, students, and school) were analysed. The results showed that the relationships between the three groups involved must be improved to some extent to meet the needs that have arisen as a result of distance learning. In conclusion, the educational scenario during the pandemic has been one of the most significant challenges experienced in the recent history of education.

scholarly journals LUPUS VULGARIS DENGAN LESI DISEMINATA

2015 ◽  
Vol 7 (3) ◽  
Author(s):  
Nana N. Jayadi ◽  
Niken Ernaningtyas ◽  
Nurdjannah J. Niode ◽  
Marthen C. P. Wongkar
Keyword(s):  
Wound Care ◽  
Giant Cells ◽  
Lupus Vulgaris ◽  
Open Wound ◽  
Cutaneous Tuberculosis ◽  
Left Neck ◽  
The Face ◽  
Night Sweats ◽  
History Of ◽  
Erythematous Plaques

Abstract: Lupus vulgaris (LV) is a chronic progressive form of paucibacillary cutaneous tuberculosis. Lesion is usually solitary in the form of nodes or erythematous plaques with an apple-jelly sign on diascopy. Disseminated LV is a rare form of cutanoeus tuberculosis with multiple lesions in several body areas. We reported a male of 40 years old with a suppurative wound on the left neck and reddish nodules on the face, neck, trunk, and limbs along with fever, night sweats, weight loss, and history of previous TB infections. There were multiple erythematous nodules and painful suppurating ulcers with enlargement of several lymph nodes. Apple-jelly sign appeared on diascopy. The FNAB showed specific granulamatous inflammation for TB with lymphocytes, epitheloid macrophages, and multinucleated giant cells. The histopathological finding showed tubercles surrounded by macrophages and lymphocytes. Anti-tuberculosis drugs category I were given for 6 months, ofloxacin, and open wound care compressed with NaCl 0.9%. In the third month of observation, there was significant improvement. Conclusion: This case was diagnosed as lupus vulgaris based on the history of lymphadenitis TB and scrofuloderma, lesions in several body area with positive diascopy test, the FNAB as well as the histopathologic result supporting the diagnosis of tuberculosis, and there was significant improvement after treatment with antiTB drugs.Keywords: lupus vulgaris, diseminata, tuberculosis, ofloksasinAbstrak: Lupus vulgaris (LV) merupakan tuberkulosis (TB) kutis pausibasiler kronis dan progresif. Lesi biasanya soliter, berupa nodus atau plak eritematosa dengan gambaran apple-jelly pada diaskopi. Lupus vulgaris diseminata merupakan bentuk TB kutis yang jarang ditemukan dengan lesi multipel pada beberapa area tubuh secara bersamaan. Kami melaporkan seorang laki-laki, 40 tahun, dengan luka bernanah pada leher kiri dan benjolan-benjolan kemerahan pada wajah, leher, badan, dan tungkai disertai demam, keringat malam, penurunan berat badan dan riwayat infeksi tuberkulosis sebelumnya. Pada pemeriksaan fisik tampak nodus eritematosa multipel disertai ulkus bernanah dan pembesaran beberapa kelenjar getah bening. Gambaran apple-jelly tampak pada diaskopi. Pemeriksaan FNAB menunjukkan gambaran radang granulomatik spesifik TB dengan adanya sel-sel radang limfosit, kelompok makrofag epiteloid, dan sel-sel datia Langhans. Pemeriksaan histopatologis memberikan gambaran tuberkel yang dikelilingi oleh makrofag dan limfosit. Terapi diberikan berupa OAT kategori I selama 6 bulan, ofloksasin, dan kompres terbuka dengan NaCL 0,9%. Pada bulan ketiga tampak perbaikan signifikan. Simpulan: Pada kasus ini, diagnosis lupus vulgaris ditegakkan berdasarkan adanya riwayat limfadenitis TB dan skrofuloderma, lesi di beberapa area tubuh sekaligus dengan pemeriksaan diaskopi positif, gambaran FNAB dan histopatologis menunjang diagnosis TB, dan pengobatan dengan OAT memberikan perbaikan bermakna.Kata kunci: lupus vulgaris, diseminata, OAT, ofloksasin

scholarly journals Orestes bojownikiem ruchu oporu, czyli mit Atrydów w filmie "Podróż komediantów" Theo Angelopoulosa

Adeptus ◽  
2016 ◽  
pp. 18-28
Author(s):  
Iga Łomanowska
Keyword(s):  
Political Dimension ◽  
Modern Times ◽  
The Family ◽  
The World ◽  
The Face ◽  
History Of ◽  
Of Greece

Orestes as a resistance fighter, or the myth of the Atreides in Theo Angelopoulos’s film The travelling playersThe paper examines the use of the Atreides myth in Theo Angelopoulos’s film The travelling players (1975) in the context of the director’s interpretation of the phenomenon of myth. Angelopoulos treated myth as a set of archetypical situations and patterns of conduct constantly reproduced in the history of the world. He intertwined elements of classical stories with the history of Greece and the Byzantine tradition, thus showing their universal character. In The travelling players, Angelopoulos used the story of betrayed and murdered Agamemnon, who is avenged by his children: Orestes and Electra, but he moved it into modern times, setting the film in Greece of the 1940s and 1950s. The myth is reproduced with modulations: the most important events take place as a result of interventions of History, not fate or decisions of the gods. Moreover, the characters’ conflicts are enriched with a political dimension, as Angelopolous portrays the discord between their ideological stances. But the members of the acting company are as helpless in the face of events as the family of the king of Argos. Orestes bojownikiem ruchu oporu, czyli mit Atrydów w filmie Podróż komediantów Theo AngelopoulosaArtykuł jest analizą sposobu wykorzystania przez Theo Angelopoulosa mitu Atrydów w filmie Podróż komediantów (1975) w kontekście dokonanej przez niego interpretacji zjawiska mitu. Grecki reżyser traktował mit jako zbiór archetypicznych sytuacji i wzorów postępowania odtwarzanych nieustannie w dziejach świata. Elementy antycznych opowieści splatał w filmach z historią Grecji i tradycją bizantyjską, ujawniając ich uniwersalny charakter. W Podróży komediantów wykorzystał historię zdradzonego i zamordowanego Agamemnona, który zostaje pomszczony przez swoje dzieci: Orestesa i Elektrę, ale przeniósł ją w czasy współczesne, portretując Grecję lat 40. i 50. XX wieku. Mit zostaje zreprodukowany z modulacjami: kluczowe wydarzenia następują w wyniku interwencji historii, nie fatum czy decyzji bogów. Ponadto konflikty między postaciami zostają wzbogacone o wymiar polityczny, ponieważ Angelopoulos ukazuje rozdźwięk między ich postawami ideologicznymi. Jednak członkowie trupy aktorskiej pozostają wobec wydarzeń tak samo bezradni jak rodzina władcy Argolidy.

scholarly journals Pheochromocytoma of the urinary bladder - a case report

2016 ◽  
Vol 73 (6) ◽  
pp. 584-587 ◽  
Author(s):  
Predrag Maric ◽  
Novak Milovic ◽  
Vladimir Bancevic ◽  
Branko Kosevic ◽  
Predrag Aleksic ◽  
...  
Keyword(s):  
Case Report ◽  
Urinary Bladder ◽  
Bladder Wall ◽  
Rare Condition ◽  
Excessive Sweating ◽  
Efficient Treatment ◽  
The Face ◽  
131I Mibg ◽  
History Of ◽  
One Year

Introduction. Pheochromocytoma of the urinary bladder is a rare tumor and presents less than 0.06% of all urinary bladder tumors. Case report. We presented a 49-year-old female patient with a history of daily paroxysmal hypertension accompanied with flushing of the face and upper chest, palpitations and excessive sweating prior to micturition. Ultrasonography reported a 3 cm bladder wall tumor. The 131I-metaiodobenzylguanidine (131I-MIBG) scan showed a pathological isotope accumulation in the projection of the bladder. The patient underwent a partial cystectomy. One year following the operation the patient was normotensive and without recurrence. Conclusion. The most efficient treatment option for bladder pheochromocytoma is surgical resection. The most important fact in the diagnostics is suspicion on this rare condition.

Antithrombin III “Northwick Park”: A Variant Antithrombin with Normal Affinity for Heparin but Reduced Heparin Cofactor Activity

1985 ◽  
Vol 53 (03) ◽  
pp. 314-319 ◽  
Author(s):  
D J Howarth ◽  
Diana Samson ◽  
Yvonne Stirling ◽  
M J Seghatchian
Keyword(s):  
Venous Thrombosis ◽  
Fast Moving ◽  
Autosomal Dominant ◽  
Antithrombin Iii ◽  
Two Dimensional ◽  
Antithrombin Activity ◽  
The Family ◽  
At Iii ◽  
History Of ◽  
Cofactor Activity

SummaryFurther studies have been carried out in a previously reported family with congenital antithrombin III (AT III) deficiency due to an abnormal variant of AT III (AT III Northwick Park). The variant has been identified in five members of the family, three of whom had a history of venous thrombosis. Inheritance followed an autosomal dominant pattern. The affected family members have reduced levels of antithrombin heparin cofactor (41–67%) and progressive antithrombin activity (44–62%) but normal levels of immunoreactive AT III (91–162%). Two dimensional immunoelectrophoresis (2 DIE) of AT III in the absence of heparin revealed an abnormal fast-moving peak in addition to the normal peak but 2 DIE in the presence of heparin appeared normal. Further studies confirmed that the abnormal AT III binds completely to heparin but has no heparin cofactor or progressive antithrombin activity. These results would be consistent with a mutation affecting the binding site for thrombin.

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