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Systemic Symptoms (systemic + symptom)
Selected AbstractsA Recurrent Drug Rash with Eosinophilia and Systemic SymptomsPEDIATRIC DERMATOLOGY, Issue 6 2007SHAKER BEN SALEM M.D. No abstract is available for this article. [source] Fine-needle aspiration diagnosis of Hodgkin lymphoma using current WHO classification,Re-evaluation of cases from 1999,2004 with new proposalsDIAGNOSTIC CYTOPATHOLOGY, Issue 6 2006Jue-Rong Zhang M.D., Ph.D. Abstract With the advent of modern therapy, the differences in prognoses and treatment regimens among different subtypes of Hodgkin lymphoma (HL) have largely vanished. Stage and the presence of systemic symptoms are much more important than histologic subtypes as predictive factors. The current (2001) WHO classification markedly de-emphasizes spatial relationships as critical to the diagnosis of lymphoma and emphasizes cell morphology, immunophenotype, genetic features, and clinical information to define the disease states. This classification, thus, greatly enhances the capability of fine-needle aspiration (FNA) to accurately diagnose HL. We searched all the FNA cases in our institute in years 1999 through 2004 and found 42 cases, for which 13 were primarily diagnosed (31.0%), 2 were recurrent (4.8%), 5 were highly suspicious (11.9%), and 22 were suspicious (52.3%) for HL. On follow-up tissue biopsy, all the primarily diagnosed, recurrent, and highly suspicious cases were confirmed to be HL (100% agreement). For the 22 suspicious cases, 13 were HL (59.1%), 5 were other lymphomas (22.8%), 1 was lymphoma unclassifiable (4.5%), and 3 were reactive processes (13.6%). The effect of immunostains on the diagnosis of HL was examined, and its importance was emphasized. Analysis of demographic data and the distribution of HL subtypes demonstrate that the study sample is representative of the general HL patient population. On the basis of these results, we propose: (1) If the FNA diagnosis of HL is confirmed both by morphology and immunostains, no further tissue confirmation, subclassification and grading is necessary, and appropriate treatment regimens should follow. (2) The nodular lymphocyte predominant HL and classical HL can be differentiated by adequate immunostaining. (3) If a definitive diagnosis cannot be achieved by FNA, a second FNA or a tissue biopsy should be recommended. Diagn. Cytopathol. 2006;34:397,402. © 2006 Wiley-Liss, Inc. [source] Aortic Tumor or Mobile Thrombus?ECHOCARDIOGRAPHY, Issue 2 2010Andrea Loiselle M.D., M.P.H. Isolated large mobile mass in the thoracic aorta can be due to thrombus or, rarely, aortic tumor. We report the case of a 61-year-old man with no history of medical problems presenting with neurologic deficits and in whom a large mobile echogenic mass in the distal aortic arch was found with transesophageal echocardiography. Given his few cardiovascular risk factors and absence of other systemic symptoms, he received anticoagulant therapy. Subsequent resolution of the aortic mass suggested a diagnosis of thrombus. This case illustrates an unusual manifestation of aortic arch atherosclerosis and underscores the utility of transesophogeal echocardiography for patients with ischemic stroke. (Echocardiography 2010;27:E21-E22) [source] Case Report: Atheroembolic renal disease in a 72-year-old patient through coronary intervention after myocardial infarctionHEMODIALYSIS INTERNATIONAL, Issue 4 2008Anna Laura HERZOG Abstract Cholesterol embolization or atheroembolic renal disease (AERD) is an often underdiagnosed issue in patients featuring a prevalent risk profile. It is a multisystemic disease with progressive renal insufficiency due to foreign body reaction of cholesterol crystals flushed into a small vessel system of the kidneys from the arteriosclerotic plaques. The most common setting in which it occurs is iatrogenic after vascular catheterization and less frequent spontaneously. Typical clinical symptoms are delayed impairment of renal function, cutaneous manifestations such as livedo reticularis or purple toes with persistingly palpable arterial pulse, myalgia, systemic symptoms such as weight loss and fever, and abdominal and neurological symptoms. Diagnosis is generally made by clinical appearance, risk profile, and interval of time from intervention; a definitive diagnosis can only be made by renal biopsy. Even though the exact incidence is not known because most patients do not undergo biopsy due to older age, comorbidity, and other explanations for loss of renal function, it is estimated to be 4% after vascular intervention. Patient and renal outcome is dependent on comorbidity, risk profile, and preexisting chronic kidney disease (CKD). About 30% of patients are estimated to require maintenance dialysis and these patients have a high risk of death within 24 months after the first renal replacement therapy. Prognosis is also influenced by severity. The case reported is a 72-year-old male patient with preexisting CKD stage 3 undergoing percutaneous coronary intervention after myocardial infarction and consecutive AERD with typical clinical appearance 6 weeks after the event. [source] Health-related quality of life improves in children and adolescents with inflammatory bowel disease after attending a camp sponsored by the Crohn's and colitis foundation of AmericaINFLAMMATORY BOWEL DISEASES, Issue 2 2005Melissa A. Shepanski MS Abstract Purpose: To describe the reported health-related quality of life (HRQOL) in children and adolescents with inflammatory bowel disease (IBD) after attending an IBD summer camp. Methods: A prospective analysis of quality of life was completed at an overnight camp that was exclusively for patients with IBD, which was sponsored by the Crohn's and Colitis Foundation of America. The IMPACT-II questionnaire (Canada and United States) and the State-Trait Anxiety Inventory for Children were administered to the campers at the beginning and at the end of a 1-week camp to assess HRQOL and anxiety. The IMPACT-II questionnaire consists of 35 questions measuring 6 quality-of-life domains (i.e., bowel domain, systemic symptoms, emotional functioning, social functioning, body image, and treatment/interventions). The State-Trait Anxiety Inventory for Children consists of 2 different 20-item sets of questions. One set assesses state anxiety, and the other, trait anxiety. A repeated-measures multivariate analysis of variance was performed to determine the differences between scores attained before and after camp on the IMPACT-II questionnaire and in each of its domains. Paired sample t tests were performed on state and trait anxiety before and after camp. Results: A total of 125 individuals consented to participate, but 61 patients (50 girls and 11 boys; age range, 9 to 16 y) completed the IMPACT-II questionnaire in full. Of those 61 patients, 47 had Crohn's disease and 14 had ulcerative colitis. There was statistically significant improvement between the mean (±SD) precamp total score (172.95 ± 36.61) and the mean postcamp total score (178.71 ± 40.97; P = 0.035), bowel symptoms scores (P = 0.036), social functioning scores (P = 0.022), and treatment interventions scores (P = 0.012). No difference was found between anxiety scores before and after camp on either the state or trait anxiety inventories (n = 55; P > 0.05). Conclusions: Overall, HRQOL improved in children after attending IBD summer camp. This exploratory study suggests that contributing factors for these improvements may be an increase in social functioning, a better acceptance of IBD symptoms, and less distress regarding treatment interventions, suggesting that a camp that is specifically designed for children with IBD may normalize the chronic illness experience. However, future research using a multimodal measurement approach is warranted to support these conclusions. [source] Erythema multiforme-like lesions associated with lesional infiltration of tumor cells occurring with adult T-cell lymphoma/leukemiaINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 4 2008Tomoyuki Ohtani MD A 66-year-old Japanese woman visited our hospital with a complaint of multiple papules on her trunk and extremities. She had a past medical history of appendicitis and blood transfusion 40 years earlier. For the last 10 years, she had noticed multiple, gradually enlarging papulonodular lesions with surrounding erythema on her trunk and extremities. ,Physical examination revealed multiple, violaceous papules or nodules, less than 10 mm in diameter, with surrounding erythema on her trunk and extremities (Fig. 1). The results of routine laboratory examinations, including blood count, liver function, renal function, serum calcium, and lactate dehydrogenase, were within the normal range. The peripheral blood picture showed a small population of atypical lymphocytes below 1% of the total white blood cells. Human T-cell lymphotropic virus type I (HTLV-I) serology was positive. A microscopic examination of a biopsy specimen from a nodule on the abdomen demonstrated diffuse infiltration of large pleomorphic T cells in the upper and middle dermis, although highly atypical lymphocytes, so-called flower cells, could not be recognized. Infiltrating lymphocytes were positive for CD2, CD3, CD4, CD5, CD7, and CD45, but negative for CD8 and CD20, immunohistologically. Bone marrow biopsy also demonstrated the infiltration of lymphocytes expressing CD2, CD3, CD4, CD5, and CD7, but not CD25. Southern blot analysis of the infiltrating cells in the skin revealed an integration of HTLV-I proviral DNA in T cells. Clonal T-cell receptor , gene rearrangement was detected in skin and bone marrow biopsies. No abnormal mass or bone defect was detected by chest or abdominal computed tomographic scanning, systemic gallium-67 citrate scintigraphy, or chest radiography. On the basis of these data, the patient was diagnosed with smouldering-type adult T-cell lymphoma/leukemia. Figure 1. Clinical features of adult T-cell lymphoma/leukemia (ATL) skin lesions. Crusted, target-like, dark-red plaques on the lower legs ,The patient was started on topical steroid and electron beam radiation therapy (27 Gy/14 days). Five days after the start of irradiation, she noticed multiple patches of edematous erythema appearing on the trunk and extremities (Fig. 2). As it was initially suspected that these newly emerging erythema multiforme or toxic eruptions were caused by irradiation, therapy was interrupted. Anti-herpes simplex virus antibody was not checked because no typical herpes simplex lesions were noticed. The patient was not taking any systemic drugs. A skin biopsy was taken from a representative lesion on the chest. The pathologic specimen showed epidermotropism, liquefaction degeneration in the basal layer, marked edema, and dense infiltration of mononuclear cells in the upper dermis. Infiltrating cells possessed abundant cytoplasm and large pleomorphic nuclei with distinct nucleoli (Fig. 3). These findings were consistent with the histopathologic findings of erythema multiforme, except for the atypical lymphoid cell infiltration. Immunohistochemical staining demonstrated that the phenotype of the skin-infiltrating cells was identical to that of the atypical cells in the initial lesions. As the eruptions did not disappear in spite of the interruption of radiation, total skin irradiation was restarted. After completion of therapy, both the erythema multiforme-like lesions and the initial adult T-cell lymphoma/leukemia nodules on the trunk and extremities had resolved, leaving brown pigmentation. The patient has been free of any recurrence of skin lesions or systemic symptoms for 6 years after the completion of total skin irradiation. Figure 2. Appearance of erythema multiforme (EM)-like lesions. Edematous red plaques involving the breast Figure 3. Microscopic examination of a biopsy specimen from (EM)-like lesions on the chest (hematoxylin and eosin staining). (a) Epidermotropism, liquefaction degeneration in the basal layer, and dense infiltration of mononuclear cells and severe edema in the upper dermis (×100). (b) High-power magnification revealed that the dermal infiltration included atypical lymphoid cells with abundant cytoplasm, convoluted large nuclei, and distinct nucleoli (×400) [source] Necrotizing fasciitis: delay in diagnosis results in loss of limbINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2006Rajat Varma MD A 58-year-old man presented to the Emergency Room with a 1-day history of severe pain in the left lower extremity preceded by several days of redness and swelling. He denied any history of trauma. He also denied any systemic symptoms including fever and chills. His past medical history was significant for diabetes, hypertension, deep vein thrombosis, and Evans' syndrome, an autoimmune hemolytic anemia and thrombocytopenia, for which he was taking oral prednisone. Physical examination revealed a warm, tender, weeping, edematous, discolored left lower extremity. From the medial aspect of the ankle up to the calf, there was an indurated, dusky, violaceous plaque with focal areas of ulceration (Fig. 1). Figure 1. Grossly edematous lower extremity with well-demarcated, dusky, violaceous plaque with focal ulceration Laboratory data revealed a white blood cell count of 6.7 × 103/mm3[normal range, (4.5,10.8) × 103/mm3], hemoglobin of 11.5 g/dL (13.5,17.5 g/dL), and platelets of 119 × 103/mm3[(140,440) × 103/mm3]. Serum electrolytes were within normal limits. An ultrasound was negative for a deep vein thrombosis. After the initial evaluation, the Emergency Room physician consulted the orthopedic and dermatology services. Orthopedics did not detect compartment syndrome and did not pursue surgical intervention. Dermatology recommended a biopsy and urgent vascular surgery consultation to rule out embolic or thrombotic phenomena. Despite these recommendations, the patient was diagnosed with "cellulitis" and admitted to the medicine ward for intravenous nafcillin. Over the next 36 h, the "cellulitis" had advanced proximally to his inguinal region. His mental status also declined, and he showed signs of septic shock, including hypotension, tachycardia, and tachypnea. Vascular surgery was immediately consulted, and the patient underwent emergency surgical debridement. The diagnosis of necrotizing fasciitis was then made. Tissue pathology revealed full-thickness necrosis through the epidermis with subepidermal splitting. Dermal edema was also present with a diffuse neutrophilic infiltrate (Fig. 2). This infiltrate extended through the fat into the subcutaneous tissue and fascia. Tissue cultures sent at the time of surgery grew Escherichia coli. Initial blood cultures also came back positive for E. coli. Anaerobic cultures remained negative. Figure 2. Necrotic epidermis with subepidermal splitting. Marked dermal edema with mixed infiltrate and prominent neutrophils. Hematoxylin and eosin: original magnification, ×20 After surviving multiple additional debridements, the patient eventually required an above-the-knee amputation due to severe necrosis. [source] Severe drug-induced skin reactions: clinical pattern, diagnostics and therapyJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 2 2009Maja Mockenhaupt Summary The spectrum of severe drug-induced skin reactions includes not only Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) but also generalized bullous fixed drug eruption (GBFDE), acute generalized exanthematous pustulosis (AGEP) and hypersensitivity syndrome (HSS), also called drug reaction with eosinophilia and systemic symptoms (DRESS). These reactions differ in clinical presentation as well as prognosis, causative agents and therapy. Therefore, the appropriate diagnostic measures should be undertaken rapidly, in order to prove the diagnosis. In addition to a thorough clinical examination, a skin biopsy should be taken and specific laboratory investigations should be done if AGEP or HSS/DRESS is suspected. Since these reactions are drug-induced, the causative agent should be rapidly identified and withdrawn. Besides adequate supportive therapy, systemic immunomodulatory treatments may be considered. Despite intensive care management, the prognosis in SJS and TEN is often poor and influenced by the amount of skin detachment as well as the age of the patients and the pre-existing underlying conditions. Severe sequelae may develop in survivors and affect especially mucosal sites. The prognosis of GBFDE is better but recurrent events may lead to more severe involvement. In HSS/DRESS sequelae have been also described as well as long lasting and recurrent courses, whereas AGEP usually heals without problems. [source] Gnathostomiasis: Import from LaosJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 5 2006Felix Hennies Creeping eruption; Gnathostomiasis; Wangenschwellung Summary Gnathostomiasis is a nematode infestation endemic in Southeast Asia, which can involve multiple organs including the liver, eyes, gastrointestinal tract and CNS. The most common manifestation is recurrent migratory subcutaneous swellings which can appear anywhere on the body and are accompanied by pruritus and systemic symptoms such as low-grade fever, loss of appetite and nausea. The diagnosis is based on the clinical picture, history of travel, peripheral blood eosinophilia and the determination of agent-specific antibody levels. The standard treatment is albendazole. We present a 37-year-old Laotian woman, who had lived in Germany for 17 years, but developed recurrent swelling of the cheek following a visit to Laos. Because of the typical clinical findings, the history of a visit to Laos, and the presence of specific anti- Gnathostoma antibodies on Western blot, the diagnosis of cutaneous gnathostomiasis was made. Zusammenfassung Die Gnathostomiasis ist eine hauptsächlich in Südost-Asien endemisch vorkommende Nematodenerkrankung, die zu einem Befall multipler Organe wie Leber, Auge, Gastrointestinaltrakt und zentralem Nervensystem führen kann. Am häufigsten kommt es jedoch zu rezidivierenden, wandernden, subkutanen Schwellungen, die am gesamten Körper auftreten können und häufig von Juckreiz und Allgemeinsymptomen wie leichtem Fieber, Appetitlosigkeit und Übelkeit begleitet werden. Die Diagnose wird anhand des klinischen Bildes, der Reiseanamnese, einer Bluteosinophilie sowie der Bestimmung Erreger-spezifischer Antikörper gestellt. Als medikamentöse Therapie hat sich das Anthelminthikum Albendazol bewährt. Vorgestellt wird eine 37-jährige, seit 17 Jahren in Deutschland lebende Patientin aus Laos, die seit einem Jahr nach Rückkehr von einem Besuch in ihrer alten Heimat unter rezidivierenden Wangenschwellungen litt. Anhand der typischen Klinik, der positiven Reiseanamnese sowie des Nachweises spezifischer Anti- Gnathostoma -Antikörper im Western-Blot wurde die Diagnose einer kutanen Gnathostomiasis gestellt. [source] Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular DysplasiaJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 5 2009SMIT C. VASAIWALA M.D. Introduction: Case studies indicate that cardiac sarcoid may mimic the clinical presentation of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); however, the incidence and clinical predictors to diagnose cardiac sarcoid in patients who meet International Task Force criteria for ARVD/C are unknown. Methods and Results: Patients referred for evaluation of left bundle branch block (LBBB)-type ventricular arrhythmia and suspected ARVD/C were prospectively evaluated by a standardized protocol including right ventricle (RV) cineangiography-guided myocardial biopsy. Sixteen patients had definite ARVD/C and four had probable ARVD/C. Three patients were found to have noncaseating granulomas on biopsy consistent with sarcoid. Age, systemic symptoms, findings on chest X-ray or magnetic resonance imaging (MRI), type of ventricular arrhythmia, RV function, ECG abnormalities, and the presence or duration of late potentials did not discriminate between sarcoid and ARVD/C. Left ventricular dysfunction (ejection fraction <50%) was present in 3/3 patients with cardiac sarcoid, but only 2/17 remaining patients with definite or probable ARVD/C (P = 0.01). Conclusions: In this prospective study of consecutive patients with suspected ARVD/C evaluated by a standard protocol including biopsy, the incidence of cardiac sarcoid was surprisingly high (15%). Clinical features, with the exception of left ventricular dysfunction and histological findings, did not discriminate between the two entities. [source] Cutaneous Rosai-Dorfman DiseaseJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L. Lin Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign disease of unknown etiology that involves the cervical lymph nodes. Histologic findings include emperiopolesis of lymphocytes and S-100 protein positivity. Extranodal involvement occurs in 30,40% of cases, often involving skin. However, cutaneous Rosai-Dorfman disease without lymph node involvement is uncommon. We describe a case of cutaneous Rosai-Dorfman disease and discuss its clinical and histopathologic findings. A 69-year-old Caucasian male presented with a two-year history of a slowly enlarging violaceous mass on the mid-upper abdomen. He had a history of renal cell carcinoma two-year ago and was asymptomatic without any systemic symptoms. Physical examination revealed a large, violaceous mass of mid-upper abdomen with several violaceous nodules surrounding the periphery. There was no palpable lymphadenopathy. Punch biopsy showed diffuse dermal infiltrates composed of central xanthomatous cells surrounded by lymphoplasmacytic cells. Focal germinal center was noted. Immunostaining revealed strong S-100 and CD68 reactivity of central xanthomatous cells and kappa and lambda polyclonal lymphoplasmacytic cells. Special stains for infectious disorder were negative. Cutaneous Rosai-Dorfman disease is a benign reactive process, often under-recognized due to lack of systemic symptoms and lymphadenopathy. A high index of diagnostic awareness is required for its recognition. [source] An Unusual Presentation of Rheumatoid MeningitisJOURNAL OF NEUROIMAGING, Issue 3 2005Vaidehi Chowdhry MD ABSTRACT Background. Central nervous system involvement in rheumatoid arthritis can rarely occur in the absence of systemic disease. Rheumatoid meningitis has not been reported to present as spells of neurologic dys-function. Patient and Methods. The authors describe a woman with a history of well-controlled rheumatoid arthritis who presented with headaches and spells of focal neurological dysfunction. Brain magnetic resonance imaging, brain biopsy, and temporal artery biopsy were required to make the diagnosis of rheumatoid meningitis with arteritis. Results. Neuroimaging revealed abnormal leptomeningeal enhancement. Necrotizing granulomatous inflammation was seen on meningeal and brain biopsy. A temporal artery biopsy showed evidence of arteritis without giant cells. Conclusions. The possibility of central nervous system involvement by rheumatoid arthritis should be considered in patients with a history of rheumatoid arthritis even in the absence of systemic symptoms. Making the diagnosis may require meningeal and brain biopsy. The condition may be steroid responsive. [source] Drug rash with eosinophilia and systemic symptoms secondary to sulfasalazineJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 4 2010Jeremy Rosenbaum Abstract A severe cutaneous eruption in an unwell patient can be a major cause of physician anxiety. With numerous differential diagnoses, an early accurate diagnosis can be challenging. infectious causes are the most important to exclude in a timely manner and drug rash and eosinophilia with systemic symptoms (DRESS) is another differential diagnosis that should be considered in children. This hypersensitivity reaction is associated with multisystem involvement. Children with underlying chronic diseases may have impairment of normal metabolic pathways and are also often on multiple medications. Therefore, drugs should always be considered in the aetiopathology of any new symptoms and signs. This case report informs readers of the association of sulfasalazine and DRESS in an 11-year-old with inflammatory bowel disease and discusses its pathogenesis and treatment. Increased awareness of this disorder will hopefully lead to increased reporting and consequently illuminate the syndrome more clearly and help guide its prevention and treatment. [source] Clinicopathlogical features and prognosis of drug rash with eosinophilia and systemic symptoms: a study of 30 cases in TaiwanJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 9 2008C-C Chiou Abstract Background, Drug rash with eosinophilia and systemic symptoms (DRESS), a group of non-blistering severe cutaneous adverse drug reactions (SCADRs), is characterized by skin rash and multiorgan involvement. Details of this reaction have not been reported in the literature so far. Aim, We investigate clinical and pathological features and prognosis of DRESS and hope this study will provide data concerning this disorder in Taiwan. Methods, From January 2001 to June 2006, a total of 30 patients, diagnosed with DRESS, were enrolled and evaluated for demographic characteristics, pathological findings, complications and outcome. Results, Patient ages ranged from 13 to 78, with an equal sex ratio. The most common offending drug was allopurinol followed by carbamazepine. Pathologic changes observed were lichenoid dermatitis, erythema multiforme, pseudolymphoma and vasculitis. Impairment of liver and renal functions and blood dyscrasia were frequent complications. Active infection or reactivation of HHV-6 was observed in 7 of 11 patients studied serologically. Two patients developed type 1 diabetes mellitus. The mortality rate was 10% (3 of 30). Conclusions, DRESS is a heterogeneous group of life-threatening conditions. The leading drug in DRESS in Taiwan is allopurinol. High eosinophil count and multiple underlying diseases are poor prognostic factors in patients with DRESS. [source] Quantifying the ,hidden' lactose in drugs used for the treatment of gastrointestinal conditionsALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 6 2009P. EADALA Summary Background, Lactose intolerance affects 70% of the world population and may result in abdominal and systemic symptoms. Treatment focuses predominantly on the dietary restriction of food products containing lactose. Lactose is the most common form of excipient used in drug formulations and may be overlooked when advising these patients. Aim, To identify and quantify the amount of lactose in medications used for the treatment of gastrointestinal disorders and to identify ,lactose-free' preparations. Methods, Medications used for the treatment of gastrointestinal disorders were identified from the British National Formulary (BNF). Their formulation including excipients was obtained from the Medicines Compendium. The lactose content and quantity in selected medications was measured using high-performance liquid chromatography (HPLC). Results, A wide range of medications prescribed for the treatment of gastrointestinal conditions contain lactose. We have quantified the lactose content in a selection of medications using HPLC. Lactose is present in amounts that may contribute towards symptoms. Lactose-free alternatives were also identified. Conclusions, Lactose is present in a range of medications and may contribute towards symptoms. This may not be recognized by the prescribing doctor as excipients are not listed in the BNF, and the quantity of lactose is not listed on the label or in the accompanying manufacturer's leaflet. [source] Pararetrovirus,crucifer interactions: attack and defence or modus vivendi?MOLECULAR PLANT PATHOLOGY, Issue 1 2000Simon N. Covey The compatible infection of plants by viruses usually leads to the development of systemic symptoms. Symptom expression of this kind is generally understood to be a host response that indicates an inability of the host to defend itself from attack. We have been studying compatible interactions between the plant pararetrovirus cauliflower mosaic virus (CaMV) and its crucifer hosts in order to understand the relationship between viral activity, symptom expression and plant defence. A CaMV protein (P6) appears to play a major role in eliciting symptom expression. This host response leads to a regulation of the viral multiplication cycle that is associated with leaf mosaics. The host regulation of CaMV appears to operate at the transcriptional level through an effect on the 35S promoter, or at the post-transcriptional level by a process that is akin to gene silencing, and can lead to host recovery depending upon the genetic background of the host. The plant apex is a focus for antiviral defence mechanisms, presumably because viral infection of the apical meristem would rapidly compromise the ability of the plant to generate new leaves and flowers for reproduction. The balance of interactions between CaMV and crucifers can provide a sustainable source of host plants to ensure viral propagation and viral exposure allows the host to adapt and develop its repertoire of defence mechanisms. [source] Clinical variant of familial amyloid polyneuropathyMUSCLE AND NERVE, Issue 3 2002Dianna Quan MD Abstract Hereditary amyloidosis with early and prominent peripheral nerve involvement is often designated familial amyloid polyneuropathy (FAP). The abnormality usually lies in the transthyretin (TTR) gene. We describe a patient with a tyr77 TTR gene mutation who presented with sensorimotor polyneuropathy but no other systemic symptoms of amyloidosis. This is one of a few documented cases of the tyr77 mutation in North America. The clinical and electrophysiologic features of this unusual cause of sensorimotor polyneuropathy are discussed. © 2002 Wiley Periodicals, Inc. Muscle Nerve 26: 417,420, 2002 [source] Chronic open angle glaucoma: patient awareness of the nature of the disease, topical medication, compliance and the prevalence of systemic symptomsOPHTHALMIC AND PHYSIOLOGICAL OPTICS, Issue 1 2004Sunil Deokule Abstract Purpose:, To study the awareness of the nature of the disease, compliance with treatment, and prevalence of systemic symptoms in a group of patients with chronic open angle glaucoma (COAG). Method:, A structured questionnaire was designed and given to 260 consecutive COAG patients attending a general ophthalmology clinic. Questions related to the increased risk of COAG amongst family members, screening of family members, nature of field defects, variation in IOP, topical treatment and availability of a free eye test for a COAG patient in the UK were asked. Compliance and systemic symptoms were also assessed. Results:, Forty-one per cent (107 of 260) of patients in the study group were aware of the increased risk of COAG in family members and 45.5% (118 of 260) of patient's family members had undergone screening for COAG. Seventy-three per cent (191 of 260) of the patients were aware of their own and their family members' entitlement to a free eye test. Seventy-seven per cent of patients claimed full compliance. Thirty per cent of patients were noted to have systemic symptoms. Conclusions:, The awareness of the nature of COAG in this population was low and incidence of perceived drug related systemic symptoms very high. Both of these may contribute to poor compliance. [source] Number IV Erythema multiformeORAL DISEASES, Issue 5 2005P Farthing Erythema multiforme (EM) is an acute mucocutaneous hypersensitivity reaction characterised by a skin eruption, with or without oral or other mucous membrane lesions. Occasionally EM may involve the mouth alone. EM has been classified into a number of different variants based on the degree of mucosal involvement and the nature and distribution of the skin lesions. EM minor typically affects no more than one mucosa, is the most common form and may be associated with symmetrical target lesions on the extremities. EM major is more severe, typically involving two or more mucous membranes with more variable skin involvement , which is used to distinguish it from Stevens-Johnson syndrome (SJS), where there is extensive skin involvement and significant morbidity and a mortality rate of 5-15%. Both EM major and SJS can involve internal organs and typically are associated with systemic symptoms. Toxic epidermal necrolysis (TEN) may be a severe manifestation of EM, but some experts regard it as a discrete disease. EM can be triggered by a number of factors, but the best documented is preceding infection with herpes simplex virus (HSV), the lesions resulting from a cell mediated immune reaction triggered by HSV,DNA. SJS and TEN are usually initiated by drugs, and the tissue damage is mediated by soluble factors including Fas and FasL. [source] Systemic Potato virus YNTN infection and levels of salicylic and gentisic acids in different potato genotypesPLANT PATHOLOGY, Issue 4 2005-Stres, H. Kre Endogenous levels of free and conjugated salicylic (SA) and gentisic (GA) acids, both putative signal molecules in plant defence, were analysed in order to investigate their involvement in the resistance of four potato (Solanum tuberosum) genotypes with different susceptibilities to Potato virus YNTN (PVYNTN) infection: the highly susceptible cv. Igor and its extremely resistant transgenic line, the extremely resistant cv. Sante and the tolerant cv. Pentland Squire. The lowest levels of free and conjugated SA were observed in the extremely resistant cv. Sante, while free GA, which was detected in all the other varieties, was absent. The extremely resistant transgenic cv. Igor contained the highest basal total SA level and the lowest level of total GA of all four cultivars. In susceptible cv. Igor, but not in resistant transgenic cv. Igor, a systemic increase of free SA was measured 1 day postinfection (dpi). Even more significant increases of free and conjugated SA and GA were detected 11 dpi when systemic symptoms appeared. In inoculated but not in upper noninoculated leaves of resistant transgenic cv. Igor, significant increase of SA conjugates occurred, but not before 11 dpi. The increase of SA and GA in susceptible cv. Igor could contribute to the general elevated levels of phenolic compounds as a response to stress caused by virus infection. It appears that basal levels of SA and GA do not correlate with resistance to PVYNTN in potato plants. [source] Clinical Symptomatology and Paranasal Sinus Involvement With Nasal Septal PerforationTHE LARYNGOSCOPE, Issue 4 2007FACS, Neil Bhattacharyya MD Abstract Objective: Determine the symptom manifestations, clinical impact, and incidence of chronic rhinosinusitis (CRS) in patients with newly diagnosed nasal septal perforation. Methods: A consecutive series of adult patients with nasal septal perforation were prospectively studied at the time of endoscopic diagnosis with the rhinosinusitis symptom inventory (RSI) and sinus computed tomography (CT). Patients' symptoms in the RSI symptom domains were computed. From the CT scan, septal perforation size and Lund scores were obtained. A separate (control) cohort of patients with CRS without septal perforation was matched to these patients for age, sex, and Lund score. RSI symptom domain comparisons were conducted between groups to determine the additional symptom burden conferred by septal perforation. Results: Thirty-three patients with septal perforation were enrolled (mean age, 48.2 yr; 69.7% female). Mean perforation size was 1.9 (SD, 2.1) cm2. The mean Lund score was 5.8 (SD, 5.3); 16 (57.1%) patients met radiographic criteria for a concurrent diagnoses of CRS. Patients with septal perforation reported significant nasal and facial symptom domain scores (56.8 and 47.0, respectively [range, 0,100]). Oropharyngeal and systemic symptoms were less severe (29.7 and 34.7, respectively). However, after comparison with the matched control patients, no statistically significant differences were identified in sinonasal symptoms between patients with and without septal perforation (all P > .131). Conclusions: Concurrent CRS may frequently accompany nasal septal perforation and may require appropriate treatment along with the perforation itself. The presence of septal perforation does not appear to significantly augment symptom severity in CRS. [source] Fulminant Liver Failure After Vancomycin in a Sulfasalazine-Induced DRESS Syndrome: Fatal Recurrence After Liver TransplantationAMERICAN JOURNAL OF TRANSPLANTATION, Issue 9 2009M. Mennicke DRESS syndrome (drug rash with eosinophilia and systemic symptoms) is a rare drug hypersensitivity reaction with a significant mortality. We describe a 60-year-old man with polyarthritis treated with sulfasalazine who developed DRESS and fulminant liver failure after additional vancomycin treatment. Liver histology revealed infiltration of granzymeB+ CD3+ lymphocytes in close proximity to apoptotic hepatocytes. After a superurgent liver transplantation and initial recovery, the patient developed recurrent generalized exanthema and eosinophilia, but only moderate hepatitis. Histology showed infiltration of FasL+ lymphocytes and eosinophils in the transplanted liver. Treatment with high-dose methylprednisolone was unsuccessful. Postmortem examination revealed extensive necrosis of the liver transplant. This case report illustrates that patients with DRESS may develop fulminant liver failure and that DRESS recurrence can recur in the transplanted liver. Histological and immunological investigations suggest an important role of granzymeB and FasL mediated cell death in DRESS associated hepatitis. [source] Azathioprine hypersensitivity presenting as a neutrophilic dermatosis in a man with ulcerative colitisAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 1 2009Eleni Yiasemides SUMMARY We report a case of a 46-year-old man with ulcerative colitis being treated with oral prednisolone and azathioprine. Two weeks after the initiation of azathioprine he presented with fever, fatigue, myalgias and arthralgias and a painful cutaneous eruption that was most marked in a sun-exposed distribution. This was accompanied by loose, non-bloody diarrhoea. Histopathological assessment of a skin biopsy supported a diagnosis of a neutrophilic dermatosis. The azathioprine was temporarily withheld and oral prednisolone was increased as it was thought that the neutrophilic dermatosis was associated with the underlying ulcerative colitis. The patient's symptoms and cutaneous eruption resolved quickly and azathioprine was re-introduced. Within 24 h, systemic symptoms returned along with a florid recrudescence of his cutaneous eruption. This rapidly improved upon withdrawal of azathioprine. [source] Necrotizing eosinophilic folliculitis with mucinosisAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 4 2003Steven Kossard SUMMARY A 23-year-old woman presented with a 2-week history of an extensive facial eruption consisting of markedly crusted arciform plaques covered with a haemorrhagic eschar that commenced as pruritic follicular pustules. These were associated with facial oedema but no systemic symptoms or fever. Two skin biopsies revealed prominent tissue eosinophilia with eosinophilic folliculitis and areas of follicular mucinosis, as well as haemorrhagic scale crust over the surface. The clinical and histological findings may represent an example of a necrotizing variant of eosinophilic folliculitis that has recently been reported in atopic individuals. This patient, however, did not have evidence of atopy and appeared to have a florid form of Ofuji's disease that responded rapidly to indomethacin treatment. [source] Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: does a DRESS syndrome really exist?BRITISH JOURNAL OF DERMATOLOGY, Issue 3 2007S.H. Kardaun No abstract is available for this article. [source] Thalidomide in advanced mastocytosisBRITISH JOURNAL OF HAEMATOLOGY, Issue 2 2008Gandhi Damaj Summary Mastocytosis is an acquired orphan disease characterized by the abnormal accumulation of mast cells responsible for organ failure and systemic symptoms. Cytoreductive drugs have been shown to be effective, but have rarely resulted in complete or long-term remission. We report two patients with advanced systemic mastocytosis (SM) who were treated successfully with thalidomide, given at the maximal tolerated dosage. B and C-findings as well as clinical symptoms rapidly improved. After a follow-up of more than 1 year, the patients remained in partial remission. Thalidomide seems to be an active drug in advanced SM. However, clinical trials are warranted to define its efficacy and safety profiles. [source] High serum levels of B-lymphocyte stimulator are associated with clinical,pathological features and outcome in classical Hodgkin lymphomaBRITISH JOURNAL OF HAEMATOLOGY, Issue 6 2007C. Tecchio Summary B-lymphocyte stimulator (BLyS) acts as survival factor for B lymphocytes. As Hodgkin and Reed-Sternberg (HRS) cells express receptors through which BLyS promotes their growth and chemotherapy resistance, we investgated whether this molecule was increased in sera from patients with classical Hodgkin lymphoma (cHL) and whether it correlates with clinical-pathological features and outcomes. Enzyme-linked immunosorbent assay was used to measure soluble BLyS (sBLyS) in sera from 87 patients and 33 donors; higher levels were detected in patients (mean ± standard error 4493·9 ± 264·9 pg/ml vs. 2687·0 ± 200·9 pg/ml; P < 0·0001). Levels above the median value (4242·0 pg/ml) were associated with age ,45 years (P = 0·042), advanced stages of disease (P = 0·005), systemic symptoms (P = 0·014) and extranodal involvement (P = 0·009). Five-year failure-free survival (FFS) of patients with sBLyS below or equal to median levels was 88·6% as compared to 65·1% of those with levels above the median (P = 0·009). Statistical analyses confirmed the prognostic significance of sBLyS (P = 0·046). When patients were analysed according to variables associated with high levels, sBLyS showed an independent predictive power in terms of FFS. Our findings support the involvement of BLyS in cHL pathogenesis. The association between high serum levels and an inferior FFS indicates that sBLyS is a possible prognostic predictor with a potential significance as a therapeutic target. [source] Predictive and discriminating three-risk-group prognostic scoring system for staging Hodgkin lymphomasCANCER, Issue 2 2007Delphine Maucort-Boulch MD Abstract BACKGROUND. Several 3-stage Ann Arbor classification-derived prognostic systems were constructed since 1980 to identify the prognosis of Hodgkin lymphoma (HL). Modern statistical tools were applied to 955 patients treated between 1981 and 1996 to build a 3-stage prognostic scoring system (PSS). METHODS. Each variable associated with 10-year overall survival (10-year OS) was assigned to 2 (0 or 1) or 3 (0, 1 or 3) values. By summing the values attributed to each variable, 3 stages were defined. 10-year OS, 5-year event-free survival (5-year EFS), and freedom from progression (5-year FFP) rates of the PSS and of other existing systems were then compared. RESULTS. Four variables were associated with 10-year OS: age (<40 = 0, ,40 = 1), number of involved lymphoid areas (1,2 = 0, 3,4 = 1, ,5 = 2), visceral disease (no = 0, yes = 1), and systemic symptoms (no = 0, yes = 1). Scores 0 and 1, 2 and 3, and ,4 were attributed to 59.7%, 30.9%, and 9.4% of the patients who had 10-year OS rates of 93.5, 75.7, and 53.4% and 5-year EFS / 5-year FFP rates of 91.2%/90.3%, 78.1%/76.3%, and 54.1%/52.6%, respectively. The discrimination and prediction abilities of the PSS were better than those of the other systems tested; moreover, the PSS adequately identified the few patients with a worse prognosis without resorting to the International Prognostic Score for advanced stages. The PSS was also highly predictive for 489 patients treated between 1997 and 2002. CONCLUSION. PSS is a useful alternative to the existing prognostic systems for evaluating HL patients. Cancer 2007. © 2006 American Cancer Society. [source] Lattice corneal dystrophy, gelsolin type (Meretoja's syndrome)ACTA OPHTHALMOLOGICA, Issue 8 2009Christian Carrwik Abstract. Purpose:, This paper reviews current knowledge about the pathogenesis, clinical manifestations and treatment of lattice corneal dystrophy, gelsolin type (LCD2, Meretoja's syndrome). Methods:, Material is derived from literature searches, a case study of a Finnish patient living in Sweden, and interviews in Helsinki with Professor Ahti Tarkkanen and Dr Sari Kiuru-Enari, both of whom have extensive first-hand experience in treating patients with the disease. Results:, The disease is now reported from several countries in Europe, as well as Japan, the USA and Iran. Treatment is symptomatic and is based on eye lubrication combined with rigorous monitoring of intraocular pressure to reduce corneal haze and postpone the need for keratoplasty. When systemic symptoms occur, the ophthalmologist should consult other specialists. Conclusions:, The disease is probably under-reported and is almost certainly to be found in more countries, including Sweden. Every ophthalmologist should be vigilant and consider this diagnosis when discovering a corneal lattice dystrophy, especially because the disease is an inherited, lifelong chronic condition with systemic symptoms. [source] Emerging and poorly known viral inflammatory eye diseasesACTA OPHTHALMOLOGICA, Issue 2009M KHAIRALLAH Arthropod vector borne diseases are among the most important emergent infections. They include a wide variety of bacterial, viral, and parasitic diseases that are transmitted to humans by the bite of mosquito, tick, or other arthropod. Most of them are prevalent in tropical and subtropical areas, but they tend to spread into new regions mainly due to increasing temperatures worldwide, movement of people, increasing human population densities, wider dispersal of competent vectors, and transportation of goods and animals. Numerous arthropod vector borne diseases have been associated with uveitis. Among them, specific viral diseases recently emerged as important causes of uveitis in the developing and developed world. They include West Nile virus (WNV) infection, Rift Valley fever (RVF) , dengue fever (DF), and Chikungunya. These viral diseases have been recently associated with an array of ocular manifestations, including anterior uveitis, retinitis, chorioretinitis, retinal vaculitis, and optic nerve involvement. Proper clinical diagnosis of any of these infectious diseases is based on epidemiological data, history, systemic symptoms and signs, and the pattern of uveitis. The diagnosis is usually confirmed by detection of specific antibody in serum. A systematic ocular examination, showing fairly typical findings, can help establish an early clinical diagnosis of a specific systemic viral infection while serologic testing is pending. Prevention remains the mainstay for control of arthropod vector borne viral diseases. [source] | ||||||||||||||||||||||||||||