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Browsing by Author "Bhargava, A"

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    Progeria : Pathogenesis and Oral Manifestation- A Review
    (Kathmandu University, 2012) Saigal, S; Bhargava, A
    ABSTRACT Our life span is genetically programmed and it is possible that a defect in produced proteins encoded by the ‘longevity’ gene is a cause of aging. Progeria which is a rare, fatal genetic condition which affects between one in four million and one in eight million children of both sexes equally and characterized by premature and accelerated aging. The appearance and physiology of these children resembles to elderly people but they typically have life span to their mid teens. It is also known as the Hutchinson-Gilford syndrome, which was initially reported by Johnathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. It is an autosomal recessive disorder, which means an individual has inherited a mutated gene from both parents. It is added to the expanding catalogue of ‘laminopathies’, diseases caused by mutations affecting nuclear lamina proteins known as lamin A (LMNA). In oral manifestation primary finding is micrognathia with delayed tooth eruption and incomplete formation of root of permanent tooth. Presently there are no known cures for this abnormality. KEY WORDS Hutchinson–Gilford Progeria Syndrome (HGPS), Premature aging, Progeria.
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    Superior Mesenteric Artery Syndrome: Diagnosis and Management
    (Kathmandu University, 2016) Kumar, R; Jaiswal, G; Bhargava, A; Kundu, J
    ABSTRACT Superior mesenteric artery syndrome is a life-threatening rare acquired upper gastrointestinal disorder due to mechanical compression of third part of duodenum by the acute angulation of Superior mesenteric artery, leading to obstruction. Acute loss of intervening mesenteric fat as a result of a variety of debilitating conditions is believed to be the etiologic factor causing the reduced aortomesenteric angle. Abdominal CT angiography showed the dilatation of second part of duodenum and vascular compression of the proximal third part of the duodenum between the aorta and superior mesenteric artery. We report a case of 15 year old young boy who presented with recurrent postprandial pain in the epigastric region, accompanied by epigastric fullness, nausea, postprandial bilious vomiting and weight loss. When conservative measures were ineffective, laparoscopic retrocolic duodenojejunostomy, side to side anastomosis, was performed in the patient to relieve the obstruction. This case report is unusual as it is concerned with the description of a rare disease entity and its radiological appearances for early preoperative diagnosis, better understanding and management of the disease are discussed in the pertinent light of literature. KEY WORDS CT angiography, duodenal obstruction, duodenojejunostomy, superior mesenteric artery syndrome

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