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Bansal Arpana V AV Reader, Department of Pedodontics and Preventive Dentistry, People's Dental Academy, Bhanpur Bypass Road, Bhopal-462010, Madhya Pradesh, India, e-mail: - - 2012
Dens invaginatus is a rare developmental anomaly. It is unusual to find this anomaly in primary dentition. Diagnosis of this dens invaginatus is important due to possible pulpal involvement. Not only that, simultaneous presence of other dental anomaly may require long-term treatment planning. Dens invaginatus can be detected clinically in ...
Carano Nicola - - 2012
Acquired complete heart block, in pediatric age is mainly the results of direct injury to conduction tissue during cardiac surgery or cardiac catheterisation. It can also be observed in different clinical settings as infectious diseases, neoplasia, and inflammatory diseases. It has a wide range of presentation and in some settings ...
Jeanmonod Rebecca R St. Luke's Hospital and Health Network, Bethlehem, PA 18015, - - 2012
The Joint Commission accreditation manual contains standards in improving organization performance related to report and review of patient care issues causing unexpected harm. In spite of regulations mandating reporting, it remains inconsistent, varying by provider type and hospital. Our purpose was to determine current attitudes, knowledge, and practice of error ...
Remo Andrea - - 2011
Rhabdoid colon tumors (RCTs) are rare lesions whose existence as an independent distinct entity remains controversial. To date, 6 RCTs have been reported. This study reports a novel case associated with polyposis coli in a 73-year-old woman. Histologically, the neoplasia was heterogeneous consisting of an adenocarcinoma associated with rhabdoid features. ...
Laouri M - - 2011
Retinal vein occlusion (RVO) is the second most common cause of vision loss due to retinal vascular disease. A literature review was undertaken to understand the epidemiology, clinical consequence, current practice patterns, and cost of RVO. Pertinent articles were identified by computerized searches of the English language literature in MEDLINE ...
Papadia Marina - - 2011
Purpose: To report the case of a patient who presented with unilateral papillitis that turned out to be the visible feature of bilateral tubercular choroiditis. Design: Retrospective review of chart and clinical history of the patient. Methods: Case report of a 54-year-old Hispanic woman with visual complaints and papillitis in ...
Purvin Valerie - - 2011
Neuroretinitis (NR) is an inflammatory disorder characterized by optic disc edema and subsequent formation of a macular star figure. The underlying pathophysiology involves increased permeability of disc vasculature, but the etiology is not fully defined. In some cases, NR is probably due to an infectious process involving the disc; in ...
Hartney Kimberly E - - 2011
Charles Bonnet syndrome (CBS) is a clinical entity in which patients develop vivid visual hallucinations in the absence of psychiatric illness. In the great majority of cases, a decline in visual acuity precedes the development of CBS. The patient maintains intact reality testing and recognizes that the hallucinations are not ...
Bair Brooke - - 2011
BACKGROUND: Atopic dermatitis (AD) is a common skin disorder of increasing prevalence. Many ophthalmologic conditions are associated with AD, including cataract formation. Posterior and anterior subcapsular cataracts have been described in AD. Topical and systemic corticosteroids have been implicated in the development of cataracts. The precise pathogenic mechanisms and risk ...
Chaudhry M - - 2011
Abstract Posterior capsular opacification, also known as after cataract develops in 33 % of patients after uneventful cataract surgery. Nd-YAG Laser capsulotomy is the treatment of choice for PCO and is routinely performed by the general ophthalmologist on an OPD basis. Endophthalmitis, although a rare complication after Nd-YAG Laser capsulotomy, ...
Gogaki Eleni - - 2011
Iridoschisis is a rare condition that consists of the separation of the anterior mesodermic layer of the iris. In more than two-thirds of cases it is associated with glaucoma. We report the case of an 80-year-old patient who had bilateral iridoschisis and presented with acute angle-closure glaucoma. The patient was ...
Thomas Ravi - - 2010
PURPOSE OF REVIEW: Clear lens extraction is being advocated for primary angle closure disease (PACD). It is important that this advocacy should be based on a sound, peer-reviewed evidence base. This review attempts to establish the extent of that evidence and provide some management guidelines. RECENT FINDINGS: There are only ...
Tsipursky Michael S - - 2011
We report three new cases of patients with Sturge-Weber Syndrome and symptomatic retinal detachments from diffuse choroidal hemangiomas successfully treated with photodynamic therapy (PDT) and review medical literature on the available treatment options for choroidal hemangiomas. All patients were treated with a single session of PDT with verteporfin infused at ...
Chowdhury R K - - 2010
Sideroblastic anemia is a rare cause of microcytic hypochromic anemia. In Bangladesh, most common causes of microcytic anemia are iron deficiency anemia, anemia of chronic diseases and thalassemia. Serum ferritin is usually done to differentiate them. If serum ferritin is low, the diagnosis of iron deficiency is entertained. When serum ...
Aynaoğlu Gülşah - - 2010
To present a rare case with factor VII deficiency (FVIID) that first diagnosed during pregnancy, and to disccuss the ante-, peri- and postpartum management. A case report and review of the literature. FVIID was diagnosed for the first time in a 23-year-old pregnant woman at the 24th week of gestation ...
Rauh Michael J - - 2011
Despite modern technological advancements in laboratory hematology, the blood film remains an important diagnostic aid. Herein, we report the case of a patient with a history of gastric cancer, who presented seven years following apparently successful surgery and adjuvant chemo-radio-therapy, with blood film findings of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, ...
Girolami A - - 2010
The occasional occurrence of thrombosis in patients with congenital bleeding disorders has received considerable attention during the past decade. Myocardial infarction, ischemic strokes and venous thromboembolism have been reported in hemophilia A or B patients, in von Willebrand disease and, also, in rare coagulation disorders, especially in factor VII (FVII) ...
Dogan Murat - - 2011
Mucopolysaccharidosis type VI (MPS VI), or Maroteaux-Lamy syndrome, is a lysosomal storage disorder that results from a deficiency of the enzyme N-acetylgalactosamine-4-sulfatase or arylsulfatase B (ASB). It is a relatively rare disorder, with an estimated incidence of 1 in 248,000 to 1 in 300,000. The diagnosis is made on the ...
Guss David A - - 2011
Lice infestation is a commonly encountered disorder in emergency medicine. The louse survives from a blood meal from its host; hence, iron deficiency anemia is a theoretic possibility. A limited number of reports of severe iron deficiency anemia have appeared in the veterinary literature, but a thorough review of the ...
Peacocke Monica - - 2010
Desquamative inflammatory vaginitis (DIV) is a chronic disorder associated with yellow vaginal discharge, vulvovaginal burning and pruritus, and dyspareunia. The cause of DIV is unknown; however, infectious, hormonal, and inflammatory etiologies have been proposed. In this series, we observe the association of DIV and vitamin D deficiency by reporting 4 ...
Allegri Gabriella - - 2010
Fumaric aciduria is a rare metabolic disease, with 40 cases reported so far. Fumarase deficiency leads mainly to brain abnormalities, developmental delay, and great accumulation of fumaric acid in urine. This work presents the first case of fumaric aciduria described in Brazil, which presented with some interesting clinical and biochemical ...
Schwalfenberg G K - - 2010
There is disturbing evidence of widespread vitamin D deficiency in many population groups, particularly within nations at high latitude. Numerous recent studies in the scientific literature associate vitamin D deficiency with a colossal increase in morbidity and mortality. Since Canada is at higher latitude, this review assesses the vitamin D ...
Zerzri Yusr - - 2010
BACKGROUND: The complement system is one of the main effectors of both innate and adaptive immunity. Hereditary complement deficiency, mainly those of the terminal pathway (C5-C9), is at increased risk for septic meningitides particularly meningococcal ones. AIM: to assess clinical and biochemical features of 3 Tunisian adults with C5 hereditary ...
Kelly Emer - - 2010
To review the topic of alpha-1 antitrypsin (AAT) deficiency. Narrative literature review. Much work has been carried out on this condition with many questions being answered but still further questions remain. AAT deficiency is an autosomal co-dominantly inherited disease which affects the lungs and liver predominantly. The clinical manifestations, prevalence, ...
Yildiz Baris - - 2010
Antral gastric vascular ectasia is a rare cause of chronic anemia. When encountered, the diagnosis is usually delayed. Endoscopic findings are well established, although radiologic findings are not. Patients respond well to surgery. Our case was of a 62-year-old female with chronic anemia who required multiple blood transfusions and iron ...
Al-Qattan M M - - 2010
Ulnar ray deficiency is rare and has a variable presentation. As a result, there are many different classification systems for this anomaly. Furthermore, the developmental biology of the anomaly is still not fully understood. The aim of this article is to review the previous classification systems, present the clinical features ...
Gazit Yael - - 2010
INTRODUCTION: Leukocyte adhesion deficiency (LAD) is a group of rare inherited disorders characterized by immune deficiency and peripheral neutrophilia. There are only seven reported cases of LAD type II worldwide, and no long-term follow-up data. CASE REPORT: We reviewed the medical file of a 20-year-old man with LAD II. Clinical ...
Win Nay - - 2010
Hyperhemolytic transfusion reaction (HHTR) is a serious and potentially life-threatening complication of red blood cell (RBC) transfusion and has been well described in patients with sickle cell disease (SCD) and non-SCD patients. Awareness of this condition is important because subsequent transfusion may exacerbate hemolysis and may lead to a chronic ...
Paul Siba P - - 2010
Coeliac disease (CD) occurs in individuals sensitive to gluten protein contained in wheat products. It affects at least 1:100 children and may present with extra-intestinal manifestations such as iron deficiency anaemia, short stature and delay in puberty. A case of severe iron deficiency anaemia as a manifestation of CD is ...
Kalotychou Vassiliki - - 2010
A 40-year-old Greek male was admitted to the hospital because of acute respiratory infection. The patient has been undergoing regular venesection for erythrocytosis for 20 years; he has also been taking oral anticoagulants for thrombosis for 15 years. The molecular defect for erythrocytosis was detected together with the rare Hb ...
Gopakumar H - - 2010
It is presently a universal practice to administer vitamin K at birth. Hence, the serious bleeding manifestations from vitamin K deficiency are nowadays very rare. We describe a case of late vitamin K deficiency bleeding presenting as intracranial hemorrhage with impending coning and the related review of literature. Such severe ...
Dziuban Eric J - - 2011
Microscopic polyangiitis (MPA) is an autoimmune systemic vasculitis of small vessels. The condition has been best characterized in older adults and little is known of the natural history of this disease in children and adolescents. In this report, a case of an adolescent presenting with symptomatic anemia and syncopal episodes ...
Todd T - - 2010
The rare inherited coagulation factor deficiencies (deficiencies of factors I, II, V, VII, XI, XIII, combined FV + FVII deficiency, combined deficiency of the vitamin K dependent factors and von Willebrand disease type 3) have an aggregate prevalence of approximately 1:100,000. They may cause recurrent life or function threatening haemorrhage. ...
Puntambekar Preeti - - 2009
Vitamin B12 deficiency is an important nutritional disorder causing neurological manifestations of myelopathy, neuropathy and dementia. Sub-acute combined degeneration (SCD) with involvement of the posterior columns in the cervical and thoracic cord is a common presentation of this disorder. In this case report, we describe a 43 year old woman ...
Lai Jiun-I - - 2009
OBJECTIVE: To report a case of immune mediated hemolysis occurring after oxaliplatin infusion in a patient with rectal cancer. CASE SUMMARY: We report a 69 year old male patient who presented with acute onset anemia after infusion of oxaliplatin as chemotherapy. A positive direct Coombs test and good response to ...
Saluja S - - 2009
Normocytic anaemia is caused either by hypoproliferation of haemopoietic tissue or increased destruction of red cell. Osteopetrosis is a rare cause of anaemia. The leading clinical features are pallor, growth failure, hepatosplenomegaly.On hematological examination, anemia, thrombocytopenia, leucocytosis and myelophthisic anemia are commonly observed in this disease. We are highlighting osteopetrosis ...
Godbole Koumudi - - 2009
Neural tube defects (NTDs) are one of the commonest birth defects with a high incidence in India. However, few studies have systematically looked into the etio-pathogeneis of NTDs, which mainly includes nutritional deficiencies and genetic predisposition. Efforts are afoot for universal food fortification with folic acid in the hope of ...
Yamshchikov Alexandra V AV Division of Infectious Diseases, Department of Medicine, Emory University-School of Medicine, Atlanta, GA 30303, USA. - - 2009
Vitamin D regulates calcium homeostasis in the body and may play a major role in regulating immune responses to tuberculosis (TB). Pilot studies suggest that vitamin D supplementation may improve outcomes in pulmonary TB (PTB), but clinical evidence using vitamin D in TB treatment is limited. We present a case ...
Brown Jennifer - - 2009
Internationally, there have been isolated case reports published of children presenting with dilated cardiomyopathy (DCM) in the setting of undiagnosed rickets. Although there has been an increased prevalence of rickets in the United States, there has been only one documented case of associated DCM. At our institution, a hospital database ...
Sillery John J - - 2009
Hemolytic anemia and methemoglobinemia are well-known adverse effects that follow ingestion of naphthalene mothballs. They are only rarely reported in association with ingestion of paradichlorobenzene mothballs. An asymptomatic boy presented to our pediatric emergency department after ingesting paradichlorobenzene mothballs. Three daysafter the ingestion, the boy returned with hemolysis and mild ...
Naseem Shano - - 2009
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematologic disorder, characterized by intravascular hemolysis, thrombosis, or bone marrow failure. It is very rare in children. The clinical manifestations are due to deficiency of a family of membrane proteins that are anchored into the cell membrane through glycosylphosphatidylinositol (GPI). Currently, flow cytometric ...
Schuurman Marijn - - 2009
Reduced concentrations of glucose-6-phospate dehydrogenase (G6PD) render erythrocytes susceptible to hemolysis under conditions of oxidative stress. In favism, the ingestion of fava beans induces an oxidative stress to erythrocytes, leading to acute hemolysis. The simultaneous occurrence of methemoglobinemia has been reported only scarcely, despite the fact that both phenomena are ...
Ciantelli M - - 2009
We report the case of a 6-week-old female who presented an intracranial hemorrhage due to late vitamin K deficiency bleeding (VKDB). No other evident bleeding sites were present at the moment of diagnosis. Intramuscular vitamin K (1 mg) was administered at birth. She was exclusively breast-fed. No other risk factors ...
Khan Fahmi Yousef - - 2009
We report a case of severe hemolytic anemia following Mycoplasma pneumoniae infection in a 29-year-old male patient who was treated with azithromycin. Direct Coombs' test was strongly positive and the cold agglutinin titer was high, with anti-I specificity. Antimycoplasma antibody titer by complement fixation was high 1:10,240. The patient was ...
Daher Elizabeth F - - 2009
BACKGROUND: The use of anabolic steroids and vitamin supplements has reached alarming proportions in the last decades. Adverse effects have been documented and include virilization, feminization, adverse lipid profile, psychiatric disorders, cardiac and liver disease. Acute kidney injury (AKI) is not frequently described. The purpose of this study is to ...
Patne Shashikant C U - - 2009
The distribution of hemoglobin E (alpha2beta2 26Glu (R)Lys ) is mostly restricted to Northeastern India. While evaluating the patients of jaundice, we came across two cases of hemoglobin E (Hb E) disorders. The first case is in a 22-year-old Bengali male and the second case of Hb E/beta thalassemia in ...
Nightingale Laura M - - 2009
The link between nutritional status and either optic or peripheral neuropathies is well established with tobacco, ethanol, deficiencies in thiamine, vitamin A, B12, B3 and B6 and protein-energy malnutrition all being causative. We describe the case of a 32-year-old Afro-Caribbean of Jamaican origin presenting with blurred vision and a painful ...
Suzuki Yuhko - - 2009
A 61-year-old Japanese man was referred to our hospital in 2002 due to severe pancytopenia. Bone marrow and peripheral blood findings indicated he had severe aplastic anemia (AA). A whole-body CT scan and Ga scintigraphy revealed no abnormal findings. Antithymocyte globulin and cyclosporine A (CyA) were administered and he got ...
Belgemen Tugba - - 2009
Selective immunoglobulin M (IgM) deficiency is a rare disorder defined by a decreased serum level of IgM and normal levels of other immunoglobulin classes. The disease has not been well described and the cause remains unknown. Patients with IgM deficiency may present with a wide spectrum of clinical manifestations, from ...
Sreejith P - - 2009
Adenine phosphoribosyl transferase deficiency is a rare metabolic abnormality presenting with 2,8 dihydroxyadenine urolithiasis. The stones are characteristically radiolucent and therefore need to be differentiated from uric acid stones which are also radiolucent and have identical chemical reactivity. No cases of 2, 8- dihydroxyadenine urolithiasis have been reported from India. ...
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