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Distinct Clinical Entity (distinct + clinical_entity)
Selected AbstractsClinical and phenomenological comparisons of delusional and non-delusional major depression in the Chinese elderlyINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue 6 2003Tien-Wen Lee Abstract Objective Distinct clinical entities, with and without delusions, have been reported for depressed patients. This study explores the clinical and phenomenological aspects of delusional and non-delusional major depression in elderly Chinese patients. Methods A total of 156 depressed patients (105 males and 51 females) admitted to our geriatric psychiatry ward were investigated. Sociodemographic and clinical characteristics were compared between patients divided into two groups,according to presence or absence of delusions. Results On admission, higher risk of suicide attempt, higher chance of guilt feelings, and greater daily functional impairment were observed for the deluded group. Further, the score of Hamilton Depression Rating Scale was higher and the score of Mini-Mental State Examination was lower for delusional depressives. Conclusion Our findings were grossly concordant with previous Western reports, and highlight the importance of identifying the delusional subgroup of depressive patients because of the higher risk of suicide attempt. Copyright © 2003 John Wiley & Sons, Ltd. [source] 1363: White dot syndromesACTA OPHTHALMOLOGICA, Issue 2010S ANDROUDI Purpose The white dot syndromes (WDS) are a group of distinct clinical entities characterized by one common underlying feature: the presence of multiple "spots" in the fundus, usually in the deep retina or choroid without any other systemic manifestations. Methods The disorders are relatively rare and include the following entities: acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white dot syndrome (MEWDS), birdshot retinochoroidopathy (BSRC), serpiginous choroidopathy (SC), multifocal choroiditis and panuveitis (MCP), punctate inner choroidopathy (PIC), subretinal fibrosis and uveitis syndrome, and presumed ocular histoplasmosis syndrome (POHS). Results Despite the fact that many infectious and noninfectious inflammatory diseases may present with multifocal chorioretinal lesions, the entities included in the WDS share some features which make them a particular group of ocular disorders. In fact, the WDS would be better labeled as idiopathic inflammatory multifocal chorioretinopathies, since with the exception of diffuse unilateral subacute neuroretinitis, their causes are still unknown. Conclusion Because the specific diagnosis may have profound implications on therapy and prognosis, it is important to narrow the diagnosis to the greatest extent possible, even in patients with seemingly atypical findings. The correct diagnosis of WDS is important because the management is totally different from one another. Some of them are self-limited and have good visual outcomes without treatment, while others are associated with serious retinal and choroidal sequelae, which can result in severe visual loss even after adequate immunosuppressive therapy. [source] Autoimmune gestational diabetes mellitus: a distinct clinical entity?DIABETES/METABOLISM: RESEARCH AND REVIEWS, Issue 6 2001Dídac Mauricio Abstract This review gives an update of the present knowledge on what is defined here as autoimmune gestational diabetes mellitus (GDM). Autoimmune phenomena associated with type 1 diabetes mellitus (DM) can be detected in a subgroup of women with GDM. Islet autoantibodies are present in sera from women with GDM with variable frequency. Distinct phenotypic and genotypic features may be recognised in this subset of women with GDM, which are representative of a distinct clinical entity. Furthermore, these women are at increased risk of developing type 1,DM after pregnancy. However, the eventual progression of the autoimmune destruction of beta-cells in these subjects may follow different time-course patterns thus leading to variable forms of presentation of autoimmune DM. As a high-risk group for type 1 diabetes, women with previous autoimmune GDM may be candidates for potential immune intervention strategies. Copyright © 2001 John Wiley & Sons, Ltd. [source] Panayiotopoulos Syndrome: An Important Electroclinical Example of Benign Childhood System EpilepsyEPILEPSIA, Issue 6 2007Michael Koutroumanidis Summary:, As a result of the converging evidence from multiple large independent studies, Panayiotopoulos syndrome (PS) is now formally recognized as a distinct clinical entity within the spectrum of benign focal epilepsies of childhood. Clinically, PS is manifested by predominantly autonomic seizures and electrographically with multifocal interictal spikes, while the few published ictal recordings have documented onsets of variable lobar topography. These typical electroclinical features do not allow straightforward assignment to a distinctive cortical area, rendering the term "focal",as we currently understand it,problematic. This is a critical review of the clinical and EEG features of PS, focusing on those characteristics that may shed some light on its so far elusive pathophysiology. We also explore its electroclinical similarities to other idiopathic "focal" epilepsies and its differences to symptomatic focal epilepsies that may also manifest with autonomic ictal symptoms and signs. This methodology allows the formation of a rational hypothesis on the pathophysiology of PS that seems to be emerging as a good model for the so-called "system" (nonsymptomatic) epilepsies, with potentially important taxonomic implications. [source] Follicular porokeratosis: distinct clinical entity or histologic variant?JOURNAL OF CUTANEOUS PATHOLOGY, Issue 11 2009M. Pongpudpunth Various clinical subtypes of porokeratosis, clinically characterized by annular plaques with a normal or atrophic center and a distinctive keratotic ridge, are described based on the age of onset, size, number and distribution of the lesions. Follicular involvement, identified by follicular localization of cornoid lamellae, is uncommon and has only been reported in association with other subtypes such as disseminated superficial actinic porokeratosis and porokeratosis of Mibelli. We present a case of follicular porokeratosis in a 40-year-old male who presented initially with scaly red "papules" in a follicular distribution on the upper extremity. Microscopic examination of a punch biopsy specimen revealed parakeratosis confined to the follicle and mild interface change. A repeat biopsy performed in 2008 revealed identical histologic features. In terms of etiopathogenesis, a clone of cells at the base of the follicle demonstrating abnormal keratinization is not a novel concept and has been demonstrated in other porokeratotic dermatoses. However, the presence of lesions that are solely follicular based, in terms of clinical presentation and histologic findings, and static over a 3-year period favors the concept that follicular porokeratosis is a distinct clinical entity and not merely a histologic variant of the porokeratotic dermatoses described in the literature thus far. [source] A patient with TSC1 germline mutation whose clinical phenotype was limited to lymphangioleiomyomatosisJOURNAL OF INTERNAL MEDICINE, Issue 2 2004T. Sato Abstract. Background:, Lymphangioleiomyomatosis (LAM) can occur as in isolated form (sporadic LAM) or as a pulmonary manifestation of tuberous sclerosis complex (TSC) (TSC-associated LAM). Recent studies, however, revealed that both forms of LAM are genetically related but that sporadic LAM is a distinct clinical entity caused by somatic mutations of TSC2 (not TSC1) rather than a forme fruste of TSC carrying either of the TSC1 or TSC2 germline mutations. Method:, Case presentation and in-depth molecular and histopathological examinations. A 34-year-old Japanese woman was diagnosed as having pulmonary lymphangioleiomyomatosis (LAM) when bilateral pneumothoraces were surgically treated in 1992. Although slowly progressive renal disfunction was observed due to bilateral multiple renal cysts during the past 4 years, she had no other clinical features of TSC and was diagnosed as having sporadic LAM with multiple renal cysts of undetermined aetiology. Her subsequent clinical course was complicated by an endobrochial carcinoid tumour, which eventually resulted in her death in June 1999 due to massive haemoptysis. Results:, Postmortem examination revealed the presence of LAM lesions in the lungs, mediastinal lymph nodes, kidneys and uterus. Diffuse renal LAM lesions are presumed to generate multiple renal cysts by constricting the nephron rather than epithelial hyperplasia obstructing lumina, which is analysis of the TSC genes demonstrated that she did not have TSC2/PKD1 contiguous gene syndrome but had a TSC1 germline mutation (Sato T et al. J Hum Genet 2002; 47: 20,8) that had occured de novo. Conclusion:, This patient therefore illustrates that clinical manifestations of TSC are sufficiently diverse as to allow a forme fruste of TSC that mimics sporadic LAM and that TSC1 mutation can cause multiple renal cysts resulting in renal failure. [source] Is pleomorphic lobular carcinoma really a distinct clinical entity?JOURNAL OF SURGICAL ONCOLOGY, Issue 5 2008Claire L. Buchanan MD Abstract Background Attempts to define the clinical behavior of pleomorphic lobular carcinoma (PLC) have been limited to small series, and clinical management strategies have yet to be established. We describe our experience with PLC as compared to classic ILC and invasive ductal carcinoma (IDC). Methods From 9/1996 to 5/2003, clinical and histopathologic data for 5,635 patients undergoing primary surgical treatment and sentinel lymph node biopsy for breast cancer were collected. Four hundred eighty one (8.5%) patients were diagnosed with ILC; 3,978 (70.6%) with IDC. Of those with ILC, 356 (74%) patients had material available for pathologic re-review and comprise our study population: 52 were classified as PLC; 298 were classified as classic ILC; and 6 cases were reclassified as IDC. We compared clinical, pathologic, and treatment factors for patients with PLC, ILC, and IDC using the Wilcoxon rank sum and Fisher's exact tests. Results PLC were larger than ILC and IDC (20 vs. 15 vs. 13, P,<,0.001), had more positive nodes (median 1 vs. 0 vs. 0, P,<,0.05) and more frequently required mastectomy (63.5% vs. 38.7% vs. 28.8%, P,<,0.001). In addition, more patients with PLC had developed metastatic disease compared to patients with ILC (11.5% vs. 3.7%, P,<,0.05). Conclusions These findings suggest that PLC is a distinct clinical entity that presents at a more advanced stage and may require more aggressive surgical and adjuvant treatment. J. Surg. Oncol. 2008;98:314,317. © 2008 Wiley-Liss, Inc. [source] Review article: sleep-related gastro-oesophageal reflux as a distinct clinical entityALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 1 2010W. C. ORR Summary Background, Many patients with chronic gastro-oesophageal reflux disease (GERD) have frequent nighttime heartburn as well as sleep-related gastro-oesophageal reflux (GOR). Sleep-related GOR has been shown to play an important role in the development of oesophagitis and other complications of GOR. Aim, To present a conceptual argument that nighttime heartburn and associated sleep-related GOR should be recognized as a distinct clinical entity deserving special attention with regard to the diagnosis and treatment of GERD. Methods, The data presented come from surveys of GERD patients as well as from physiological studies to include studies monitoring oesophageal pH and spontaneous reflux events during polysomnographically (PSG) monitored sleep. Results, Evidence is presented to show that nighttime heartburn is prevalent and its occurrence separates patients from those who have heartburn most exclusively in the daytime. The evidence presented also supports the notion that nighttime heartburn sufferers have a more complicated disease and they have a greater risk of developing oesophagitis and other respiratory complications. The data also show that responses to acid mucosal contact are quite different during sleep compared to responses measured during the waking state. Conclusions, Nighttime heartburn and GOR represent a distinct clinical entity which deserves specific attention in the diagnosis and optimal treatment of GERD. [source] Chronic Kidney Disease Following Non-Myeloablative Hematopoietic Cell TransplantationAMERICAN JOURNAL OF TRANSPLANTATION, Issue 1 2006A. S. Weiss Chronic kidney disease (CKD) following myeloablative allogeneic hematopoietic cell transplantation (HCT) occurs in 20% of survivors at 1 year and is believed to be due to radiation nephritis. Non-myeloablative allogeneic HCT is a recent procedure that employs significantly lower doses of chemoradiotherapy, however, incidence and risk factors for CKD following non-myleoablative HCT have not been defined. We performed a retrospective cohort study of 122 patients from three institutions who were available for analysis at 6 months following non-myeloablative HCT. Patients received two Gy of radiation; 62% received fludarabine as preconditioning. CKD was defined as at least a 25% reduction in glomerular filtration rate (GFR) from baseline using the abbreviated modified diet in renal disease (MDRD) equation. Eighty-one of 122 patients (66%) showed evidence of CKD at follow-up. Multivariate analysis revealed that acute renal failure (ARF) during the first 100 days post-transplant was associated with development of CKD (Adjusted OR 32.8 with 95% CI 4.3,250) after controlling for other variables. Previous autologous HCT, long-term calcineurin inhibitor use and extensive chronic GVHD were independently associated with CKD. CKD following non-myeloablative HCT appears to be a distinct clinical entity and likely not related to radiation nephritis. Future research should focus on possible mechanisms for alleviating chronic injury and decreasing use of calcineurin inhibitors. [source] Sjögren's syndrome and localized nodular cutaneous amyloidosis: Coincidence or a distinct clinical entity?ARTHRITIS & RHEUMATISM, Issue 7 2008Jiska M. Meijer Objective To report 8 patients with Sjögren's syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases. Methods The databases for 3 amyloidosis centers were searched for patients with localized nodular cutaneous amyloidosis and SS. Eight patients with this combination were identified, and clinical, serologic, and histologic parameters were retrospectively evaluated. Results Among the 8 patients with a clinical diagnosis of SS, 6 fulfilled the American,European Consensus Group criteria for SS. All of the patients were women in whom SS had been diagnosed at a median age of 47 years (range 30,61 years) and amyloid had been diagnosed at a median age of 60 years (range 42,79 years). The presence of the immunoglobulin light chain type of amyloid (AL amyloid) was confirmed in 4 patients. In 3 of these 4 patients as well as 2 other patients, a light chain,restricted plasma cell population was observed near the amyloid deposits. Progression to systemic amyloidosis was not observed in any patient during a median followup of 3.5 years. Conclusion SS should be considered in patients with cutaneous amyloidosis. The combination of cutaneous amyloidosis and SS appears to be a distinct disease entity reflecting a particular and benign part of the polymorphic spectrum of lymphoproliferative diseases related to SS. [source] Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitisACTA NEUROLOGICA SCANDINAVICA, Issue 4 2010H. Sakuma Sakuma H, Awaya Y, Shiomi M, Yamanouchi H, Takahashi Y, Saito Y, Sugai K, Sasaki M. Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurol Scand: 2010: 121: 251,256. © 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objective,,, We conducted a nationwide multicenter study in Japan to elucidate the clinical and laboratory characteristics of acute encephalitis with refractory, repetitive partial seizures (AERRPS). Materials and methods,,, Clinical and laboratory features, treatment, and outcome were assessed using a structured questionnaire. Results,,, Twenty-nine children were enrolled in the study. Refractory and repetitive partial seizures accompanied by fever were the cardinal clinical features. Partial seizures consisted principally of eye deviation or facial twitching, being periodically repeated during the acute phase. These seizures were refractory to conventional anticonvulsants and were only suppressed by high-dose intravenous barbiturate administration. Rhythmic activities on electroencephalography and non-specific cerebral atrophy on neuroimaging were common. Serum or cerebrospinal antibodies against GluR,2 were positive in six patients. General prognosis was unfavorable due to intractable epilepsy and cognitive deficits. Conclusion,,, Based on the peculiar and homogenous features, AERRPS can be regarded as a distinct clinical entity. [source] | ||||||||||