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Severe Episodes (severe + episode)
Selected AbstractsA case report of systemic capillary leak syndrome (Clarkson's disease)ACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 5 2010J. HOLLENBERG Systemic capillary leak syndrome (SCLS) is a very rare disorder also known as Clarkson's disease. The condition is characterized by recurrent episodes of severe capillary hyperpermeability resulting in severe hemoconcentration, hypoalbuminemia, hypovolemia and shock. We describe a 41-year-old previously healthy man who was admitted to hospital on several occasions with rapidly developing hypovolemic shock accompanied by extreme hemoconcentration and hypoalbuminemia. Our case is similar to other reports describing patients with SCLS where the initial suspicions have been pointing towards septic shock. He received a combination of prophylactic treatment with theophylline, ,-agonists, immunoglobulins and statins but eventually died after a severe episode of SCLS that ended with recurrent cardiac arrest. Clinical autopsy revealed pulmonary edema and acute and chronical organic fluid overload. SCLS should be kept in mind when treating patients suffering from attacks of severe idiopathic edema and mimics recurrent septic shock where no pathogen is found. The pathogenesis is unknown and the attacks may be lethal. [source] Histopathology and Immunophenotype of the Spleen During Acute Antibody-Mediated RejectionAMERICAN JOURNAL OF TRANSPLANTATION, Issue 5 2010B. Kaplan Splenectomy has been reported to have a beneficial effect in treating Acute antibody-mediated rejection (ABMR). This reason for this often rapid and profound beneficial effect is not readily apparent from what is known about normal splenic immunoarchitecture. While the spleen is rich in mature B cells, it has not been noted to be a repository for direct antibody-secreting cells. We present a case of a Native American female who received a renal transplant and developed a severe episode of ABMR. The patient was initially refractory to both plasmapheresis and IVIG. The patient underwent an emergent splenectomy with almost immediate improvement in her renal function and a rapid drop in her DR51 antibodies. Immunohistochemical stains of the spleen demonstrated abundant clusters of CD138+ plasma cells (>10% CD138 cells as opposed to 1% CD138 cells as seen in traumatic controls). Though this is a single case, these findings offer a rationale for the rapid ameliorative effect of splenectomy in cases of antibody rejection. It is possible that the spleen during times of excessive antigenic stress may rapidly turn over B cells to active antibody-secreting cells or serve as a reservoir for these cells produced at other sites. [source] Blue rubber bleb nevus syndromeACTA PAEDIATRICA, Issue 4 2010M Agnese Abstract Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare condition characterized by multiple venous malformations involving the skin and internal organs. The gastrointestinal tract is always involved and intestinal haemorrhage is the most frequent clinical manifestation associated with iron deficiency anaemia. We describe a 10-year-old girl who, since birth, presented numerous venous malformations all over her body and a lymphangioma in the right leg. At the age of 5 years, she also had a severe episode of gastric bleeding requiring a blood transfusion. From this episode, she is suffering from chronic anaemia and this is the reason for admission into our hospital. The endoscopic examination of the gastrointestinal tract revealed multiple giant venous malformations in the oesophagus, stomach, duodenum and in all visible sections of the colon. Endoscopy is the gold standard technique for the diagnosis of BRBNS with GI lesions and also allows immediate therapeutic measures such as argon plasma coagulation, laser photocoagulation, sclerotherapy or band ligation. In addition, pharmacological treatments based on corticosteroids, interferon alfa, vincristine or octreotide have been described for BRBNS. Conclusion:, Blue Rubber Bleb Nevus Syndrome is a congenital cutaneous and gastrointestinal haemangiomatosis. Its morbidity and mortality depends on involvement of visceral organs and particularly on GI bleeding. The treatment is based on pharmacological or surgical therapy. Overall, the most important step is the follow-up to the presence and the evolution of GI lesions and the possible bleeding. [source] A reduction in severe hypoglycaemia in type 1 diabetes in a randomized crossover study of continuous intraperitoneal compared with subcutaneous insulin infusionDIABETES OBESITY & METABOLISM, Issue 11 2009A. Liebl Aim: Continuous intraperitoneal insulin infusion (CIPII) with the DiaPort system using regular insulin was compared to continuous subcutaneous insulin infusion (CSII) using insulin Lispro, to investigate the frequency of hypoglycemia, blood glucose control, quality of life, and safety. Methods: In this open, randomized, controlled, cross-over, multinational, 12-month study, 60 type 1 diabetic patients with frequent hypoglycemia and/or HbA1c > 7.0% with CSII were randomized to CIPII or CSII. The aim was to obtain the best possible blood glucose while avoiding hypoglycemia. Results: The frequency of any hypoglycemia was similar (CIPII 118.2 (SD 82.6) events / patient year, CSII 115.8 (SD 75.7) p = 0.910). The incidence of severe hypoglycemia with CSII was more than twice the one with CIPII (CIPII 34.8 events / 100 patient years, CSII 86.1, p = 0.013). HbA1c, mean blood glucose, and glucose fluctuations were not statistically different. Treatment-related severe complications occurred mainly during CIPII: port infections (0.47 events / patient year), abdominal pain (0.21 events / patient year), insulin underdelivery (0.14 events / patient year). Weight gain was greater with CSII (+ 1.5 kg vs. , 0.1 kg, p = 0.013), quality of life better with CIPII. Conclusions: In type 1 diabetes CIPII with DiaPort reduces the number of severe episodes of hypoglycemia and improves quality of life with no weight gain. Because of complications, indications for CIPII must be strictly controlled. CIPII with DiaPort is an alternative therapy when CSII is not fully successful and provides an easy method of intraperitoneal therapy. [source] Hypoglycaemia in Type 2 diabetesDIABETIC MEDICINE, Issue 3 2008S. A. Amiel Abstract The primary cause of hypoglycaemia in Type 2 diabetes is diabetes medication,in particular, those which raise insulin levels independently of blood glucose, such as sulphonylureas (SUs) and exogenous insulin. The risk of hypoglycaemia is increased in older patients, those with longer diabetes duration, lesser insulin reserve and perhaps in the drive for strict glycaemic control. Differing definitions, data collection methods, drug type/regimen and patient populations make comparing rates of hypoglycaemia difficult. It is clear that patients taking insulin have the highest rates of self-reported severe hypoglycaemia (25% in patients who have been taking insulin for > 5 years). SUs are associated with significantly lower rates of severe hypoglycaemia. However, large numbers of patients take SUs in the UK, and it is estimated that each year > 5000 patients will experience a severe event caused by their SU therapy which will require emergency intervention. Hypoglycaemia has substantial clinical impact, in terms of mortality, morbidity and quality of life. The cost implications of severe episodes,both direct hospital costs and indirect costs,are considerable: it is estimated that each hospital admission for severe hypoglycaemia costs around £1000. Hypoglycaemia and fear of hypoglycaemia limit the ability of current diabetes medications to achieve and maintain optimal levels of glycaemic control. Newer therapies, which focus on the incretin axis, may carry a lower risk of hypoglycaemia. Their use, and more prudent use of older therapies with low risk of hypoglycaemia, may help patients achieve improved glucose control for longer, and reduce the risk of diabetic complications. [source] Recurrent palmar,plantar erythrodysaesthesia following high-dose cytarabine treatment for acute lymphoblastic leukemiaEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5-6 2002Julie H. Crawford Abstract: Palmar,plantar erythrodysaesthesia (PPE) is an uncommon cutaneous complication of cytotoxic chemotherapy which generally presents as a painful erythema involving the palms and soles. It has been suggested that PPE caused by cytarabine does not recur with subsequent cytarabine re-challenge. We report a patient with recurrent, increasingly severe episodes of PPE, ultimately complicated by a severe bullous eruption, following successive cycles of high-dose cytarabine for the treatment of acute lymphoblastic leukaemia. Contrary to previous recommendations, our experience cautions against the further use of high-dose cytarabine in patients who develop PPE, and is a timely reminder of the potential toxicity of this agent, which is now increasingly being used as first-line treatment in the management of haematologic malignancies. [source] Hypnic Headache Responsive to Low-Dose Topiramate: A Case ReportHEADACHE, Issue 2 2008Massimo Autunno MD This is a clinical report of a 63-year-old woman, with a 3-year history of severe episodes of hypnic headache responding to low-doses of topiramate (25 mg at bedtime). Topiramate has been used at the dosage of 100 mg/day for hypnic headache prevention in one recent case report with benefit. This report confirms the efficacy of topiramate in hypnic headache even using low-dose regimen therapy. [source] Human caliciviruses detected in Mexican children admitted to hospital during 1998,2000, with severe acute gastroenteritis not due to other enteropathogensJOURNAL OF MEDICAL VIROLOGY, Issue 4 2010Ana Lorena Gutiérrez-Escolano Abstract Few studies exist regarding the frequency of human caliciviruses as single etiologic agents in sporadic cases, or in outbreaks occurring in children hospitalized for acute gastroenteritis. In this study, a total of 1,129 children of <5 years of age and hospitalized due to acute diarrhea were enrolled from three main hospitals in Mexico City during a period of 3 years (March 1998 to December 2000). After analyzing all fecal samples for several enteropathogens, 396 stools that remained negative were further screened for human caliciviruses by RT-PCR using a primer set specific to norovirus and sapovirus. Human caliciviruses were detected in 5.6% (22/396) of the children. The minimum incidence rate for 1999 were 5.3% (7/132) for 1999 and 7.8% (13/167) for 2000, since only fecal specimens that tested negative to other enteric pathogens were examined. Positive samples were further characterized using specific GI and GII primers and sequencing. Norovirus GII was detected in 19/22 samples, most of them were GII/4, while sapovirus GI/2 was detected in one sample. Associations between the presence of human calicivirus and clinical and epidemiological data revealed that diarrhea occurred with a seasonal pattern, and that children hospitalized due to human calicivirus disease scored an average of 13,±,3.2 (SD) points on the Vesikari scale, which corresponded to severe episodes. These results highlight that human caliciviruses, by themselves, are enteropathogens of acute severe diarrhea among young Mexican children requiring hospitalization and that their detection is important in order to reduce the diagnosis gap. J. Med. Virol. 82:632,637, 2010. © 2010 Wiley-Liss, Inc. [source] Cyclosporin A treatment in severe childhood psoriasisJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2006TM Pereira Abstract Though used occasionally, systemic therapies in severe childhood psoriasis have not been systematically investigated. Cyclosporin A (CysA) is effective in adults with severe psoriasis but there are no extensive data regarding the efficacy and safety of its use in childhood psoriasis. In this paper, we describe six children aged between 11 months and 13 years (average: 7.6 years) treated with CysA microemulsion formulation for severe psoriasis, who had been unresponsive to other treatments. The CysA dose ranged from 2 to 4 mg/kg/day, for periods varying from 8 to 105 weeks (mean: 54 weeks). Dose tapering was gradual after lesion improvement and adjusted according to clinical response. Adjuvant therapy with topical steroids, vitamin D3 ointments, coal tar preparations or anthralin was used in all children. Acitretin was used in three patients for short periods. The children were regularly monitored for serum renal and liver function and blood pressure. Improvement of skin lesions was achieved after between 4 and 30 (mean: 12) weeks of treatment, with complete remission in three children. Relapse of lesions occurred in the other children during CysA reduction, but they responded to a dose increase. The treatment was found to be well tolerated and with no significant side-effects. CysA can be used in carefully selected and monitored patients and may represent an alternative tool for severe episodes of psoriasis in children, when other therapies are unsuccessful. [source] PERCUTANEOUS THERMORHIZOTOMY IN A CLINICAL SERIES OF 80 PATIENTS WITH TRIGEMINAL NEURALGIA.JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2002F. Rychlicki At the time of this report, 80 patients have been operated upon for typical trigeminal neuralgia by the percutaneous rhizotomy approach. Through follow-up evaluation extending over a period of 1 to 20 years, it has been completed for 65 of these patients. The average age was 63 years and 60% of patients were female. In 60% of patients pain was located on the right, and the second and third division of the trigeminal nerve were commonly involved. Isolated pain in the first or third division was less frequent than the second division. The disease had been present for an average of 8 years and was characterized by increasingly severe episodes of paroxismal pain and shortening period of remission. Nearly all patients had been treated with either diphenylhydantoin or carbamazepine, as well as other forms of medical and physical remedies. Response to follow-up was obtained for all 65 patients. All were contacted by questionnaire on phone and a family member was contacted if the patient had died. At the time of evaluation, 94% of patients reported excellent to good results from the procedure. The remaining patients obtained only fair results because of undesirable side-effects or recurrence of pain. The authors emphasize the importance of surgical therapy in trigeminal neuralgia when medical therapy fails. [source] Kikuchi Fujimoto lymphadenitis: Case report and literature reviewAMERICAN JOURNAL OF HEMATOLOGY, Issue 1 2003Giuseppe Famularo Abstract We describe a young woman with two severe episodes of Kikuchi Fujimoto disease occurring 16 years apart. Both episodes were proven by biopsy, and on the second occasion the patient remained dependent on high-dose prednisone for more than 6 months in order to control inflammation and achieve a reduction in cervical lymph node size. The second lymph node biopsy showed leukocytoclastic vasculitis in addition to the typical features of Kikuchi Fujimoto disease, but, even though the clinical interpretation of this finding was unclear, we documented no clinical or laboratory evidence of the development of other serious systemic disease over 20 years of follow-up. Kikuchi Fujimoto disease is considered a disorder with a self-limited course and a favorable outcome. However, on the basis of our experience with this patient and data from peer-reviewed literature, we suggest that this generally accepted postulate should be revised. Am. J. Hematol. 74:60,63, 2003. © 2003 Wiley-Liss, Inc. [source] Proinflammatory action of the antiinflammatory drug infliximab in tumor necrosis factor receptor,associated periodic syndromeARTHRITIS & RHEUMATISM, Issue 2 2009Belinda Nedjai Objective Tumor necrosis factor receptor (TNFR),associated periodic syndrome (TRAPS) is an autosomal-dominant autoinflammatory condition caused by mutations in the TNFRSF1A gene. Unlike other autoinflammatory diseases in which anti-TNF therapy is largely a successful treatment option, therapy with the anti-TNF drug infliximab is often ineffective in patients with TRAPS. Moreover, in certain cases, infliximab actually triggers severe episodes of inflammation. The aim of this study was to elucidate the mechanisms underlying such a reaction. Methods Peripheral blood mononuclear cells (PBMCs) were obtained from patients with TRAPS. Both caspase 3 activity and NF-,B subunit activity were determined by enzyme-linked immunosorbent assay. Cytokine secretion was assessed using a specific customized human multiplex bead immunoassay kit. Results Unlike findings in controls, cells from a family of 9 patients, all of whom carried the T50M mutation in TNFRSF1A, failed to respond to infliximab through proapoptotic induction of caspase 3 activity. Instead, we observed enhanced antiapoptotic c-Rel subunit activity, accompanied by a significant increase in secretion of the proinflammatory cytokines interleukin- 1, (IL-1,), IL-1 receptor, IL-6, IL-8, and IL-12. Conclusion Altered extracellular conformation of TNFRI, resulting from the T50M mutation in TNFRSF1A, results in failure of PBMCs to induce an apoptotic response to infliximab. We hypothesize that failure to shed infliximab-bound TNF/TNFRI from the cell surface of cells from patients with the T50M mutation triggers c-Rel activation, and that this leads to a marked increase in cytokine secretion and an increased proinflammatory response. In light of these findings, we strongly advise caution when prescribing infliximab as anti-TNF therapy to patients with TRAPS. [source] | |||||||||||||||||||