John of Brienne known as John I, was King of Jerusalem from 1210 to 1225 and Latin Emperor of Constantinople from 1229 to 1237. He was the youngest son of Erard II of a wealthy nobleman in Champagne. John destined for an ecclesiastical career, became a knight and owned small estates in Champagne around 1200. After the death of his brother, Walter III, he ruled the County of Brienne on behalf of his minor nephew Walter IV; the barons of the Kingdom of Jerusalem proposed that John marry Maria. With the consent of Philip II of France and Pope Innocent III, he left France for the Holy Land and married the queen. After Maria's death in 1212 John administered the kingdom as regent for their infant daughter, Isabella II. John was a leader of the Fifth Crusade. Although his claim of supreme command of the crusader army was never unanimously acknowledged, his right to rule Damietta was confirmed shortly after the town fell to the crusaders in 1219, he claimed the Armenian Kingdom of Cilicia on behalf of his second wife, Stephanie, in 1220.
After Stephanie and their infant son died that year, John returned to Egypt. The Fifth Crusade ended in failure in 1221. John was the first king of Jerusalem to visit Europe to seek assistance for the Holy Land, he gave his daughter in marriage to Holy Roman Emperor Frederick II in 1225, Frederick ended John's rule of the Kingdom of Jerusalem. Although the popes tried to persuade Frederick to restore the kingdom to John, the Jerusalemite barons regarded Frederick as their lawful ruler. John administered papal domains in Tuscany, became the podestà of Perugia and was a commander of Pope Gregory IX's army during Gregory's war against Frederick in 1228 and 1229, he was elected emperor in 1229 as the senior co-ruler of the Latin Empire, was crowned in Constantinople in 1231. John III Vatatzes, Emperor of Nicaea, Ivan Asen II of Bulgaria occupied the last Latin territories in Thrace and Asia Minor, besieging Constantinople in early 1235. John directed the defence of his capital during the months-long siege, with the besiegers withdrawing only after Geoffrey II of Achaea and united fleets from Italian towns defeated their fleet in 1236.
The following year, John died as a Franciscan friar. John was the youngest of the four sons of Erard II, Count of Brienne, Agnes of Montfaucon, he seemed "exceedingly old... about 80" to the 14-year-old George Akropolites in 1231. However, no other 13th-century authors described John as an old man, his father referred to John's brothers as "children" in 1177 and mentioned the tutor of John's oldest brother, Walter III, in 1184. Modern historians agree that John was born after 1168 during the 1170s. Although his father destined John for a clerical career, according to the late 13th-century Tales of the Minstrel of Reims he "was unwilling". Instead, the minstrel continued, John fled to his maternal uncle at the Clairvaux Abbey. Encouraged by his fellows, he earned a reputation in tournaments and fights. Although elements of the Tales of the Minstrel of Reims are invented, historian Guy Perry wrote that it may have preserved details of John's life. A church career was not unusual for youngest sons of 12th-century noblemen in France.
John "clearly developed the physique, necessary to fight well" in his youth, because the 13th-century sources Akropolites and Salimbene di Adam emphasize his physical strength. Erard II joined the Third Crusade and died in the Holy Land in 1191, his oldest son, Walter III, succeeded him in Brienne. John was first mentioned in an 1192 charter issued by his brother, indicating that he was a prominent figure in Walter's court. According to a version of Ernoul's chronicle, John participated in a war against Peter II of Courtenay. Although the Tales of the Minstrel of Reims claimed that he was called "John Lackland", according to contemporary charters John held Jessains, Onjon and two other villages in the County of Champagne around 1200. In 1201, Theobald III granted him additional estates in Mâcon and elsewhere. Theobald's widow, Blanche of Navarre, persuaded John to sell his estate at Mâcon, saying that it was her dower. Walter III of Brienne died in June 1205 while fighting in southern Italy, his widow, Elvira of Sicily, gave birth to a posthumous son, Walter IV.
John assumed the title of count of Brienne, began administering the county on his nephew's behalf in 1205 or 1206. As a leading vassal of the count of Champagne, John frequented the court of Blanche of Navarre. According to a version of Ernoul's chronicle, she loved John "more than any man in the world"; the two versions of Ernoul's chronicle tell different stories about John's ascent to the throne of Jerusalem. According to one version, the leading lords of Jerusalem sent envoys to France in 1208 asking Philip II to select a French nobleman as a husband for their queen, Maria. Taking advantage of the opportunity to rid himself of John, Philip II suggested him. In the other version an unnamed knight encouraged the Jerusalemite lords to select John, who accepted their offer with Philip's consent. John visited Pope Innocent III in Rome; the pope
Severe achondroplasia with developmental delay and acanthosis nigricans, is a rare genetic disorder. This disorder is one that affects bone growth and is characterized by skeletal and skin abnormalities; those affected by the disorder are short in height and possess shorter arms and legs. In addition, the bones of the legs are bowed and the affected have smaller chests with shorter rib bones, along with curved collarbones. Other symptoms of the disorder include extra folds of skin on the arms and legs. Developmentally, many individuals who suffer from the disorder show a higher level in delays and disability. Seizures are common due to structural abnormalities of the brain; those affected may suffer with apnea, the slowing or loss of breath for short periods of time. Many of the features of SADDAN are similar to those seen in other skeletal disorders achondroplasia and thanatophoric dysplasia. Achondroplasia is a form of short-limbed dwarfism; this type of dwarfism is caused by the inability of the cartilage of the skeleton to ossify and turn to bone.
Acanthosis nigricans is a skin condition in which areas of the skin is of a dark and velvety discoloration seen in the body folds and creases such as the armpits and neck. Within those affected by SADDAN, acanthosis nigricans develops early on in infancy or early childhood; the mutated gene responsible for the disorder is the FGFR3 gene, more specifically. This gene codes for the instructions of a protein, integral in the development and maintenance of bone and brain tissue. Mutations of this gene cause the protein to be overly active, causing many characteristics of this disorder. SADDAN is an autosomal dominant genetic disorder. Autosomal means that the gene responsible for the mutation and disorder is found on a non-sex chromosome and that either the mother or father can pass on the gene, while dominant means that only one copy of the gene is required for the individual to have the disorder; the disorder is rare and has only been described in a few number of cases worldwide. While the disorder can be genetically inherited, no instances of inheritance have been recorded as of yet.
Rather, of the few cases documented, the individual affected by the disorder is affected as a product of a random mutation called a de novo mutation, of the FGFR3 gene only, not by inheritance of the mutated gene. Medical diagnosis is required. Clinical tests can be performed, as well as molecular genetic testing; the available tests include: Sequence analysis of the entire coding region Severe achondroplasia with developmental delay and acanthosis nigricans - Sanger Sequencing: Diagnosis, Mutation Confirmation, Pre-symptomatic, Risk Assessment, Screening Craniosynostosis: Diagnosis Invitae FGFR3-Related Disorders Test: Pre-symptomatic, Therapeutic managementMutation scanning of select exons Skeletal Dysplasia Panel: Diagnosis, PrognosticSequence analysis of select exons Severe Achondroplasia with Developmental Delay and Acanthosis Nigricans: Diagnosis, Mutation Confirmation, Risk Assessment Severe Achondroplasia, Developmental Delay, Acanthosis Nigricans: Diagnosis, Mutation ConfirmationDeletion/duplication analysis Invitae FGFR3-Related Disorders Test: Pre-symptomatic, Therapeutic management Life with SADDAN is manageable, although therapy and lifelong doctor surveillance may be required.
Bellus GA, Bamshad MJ, Przylepa KA, Dorst J, Lee RR, Hurko O, Jabs EW, Curry CJ, Wilcox WR, Lachman RS, Rimoin DL, Francomano CA. "Severe achondroplasia with developmental delay and acanthosis nigricans: phenotypic analysis of a new skeletal dysplasia caused by a Lys650Met mutation in fibroblast growth factor receptor 3". Am J Med Genet. 85: 53–65. Doi:10.1002/1096-862885:1<53::AID-AJMG10>3.0. CO. PMID 10377013. Cohen MM Jr. "Some chondrodysplasias with short limbs: molecular perspectives". Am J Med Genet. 112: 304–13. Doi:10.1002/ajmg.10780. PMID 12357475. Vajo Z, Francomano CA, Wilkin DJ. "The molecular and genetic basis of fibroblast growth factor receptor 3 disorders: the achondroplasia family of skeletal dysplasias, Muenke craniosynostosis, Crouzon syndrome with acanthosis nigricans". Endocr Rev. 21: 23–39. Doi:10.1210/er.21.1.23. PMID 10696568
Arthur John MacLean was an Anglican bishop in the decades of the 19th century and first four of the 20th century. Maclean was born into an ecclesiastical family, his father, the Rev Arthur J. Macleane, began a career in the East India Company before returning to England, obtaining a degree Trinity College Cambridge, being ordained and securing appointment as inaugural Principal of Brighton College, he held two subsequent headships and was editor of various Classical texts Horace and Juvenal. Maclean was educated at King's College, Cambridge, he was ordained in 1882 and he was head of the Archbishop of Canterbury's Assyrian Mission from 1886 to 1891 and Rector of Portree. In 1882 he became Dean of Argyll and The Isles and after this was Rector of Selkirk before a spell as Principal of the Scottish Episcopal Theological College and a nearly 40 years episcopacy as Bishop of Moray and Caithness. Late in his life he was additionally elected Primus of the Scottish Episcopal Church. An eminent author, he died on 24 February 1943.
Grammar of Vernacular Syriac in English, 1895 A Dictionary of the Dialects of Vernacular Syriac, 1901 The Ancient Church Orders, 1910 The Didache, 1922 J. F. Coakley; the Church of the East and the Church of England:a history of the Archbishop of Canterbury's Assyrian Mission, Oxford, 1992