Handling shock in idiopathic systemic capillary leak syndrome (Clarkson ’s disease): less is more

AbstractIdiopathic systemic capillary leak syndrome (ISCLS) presents with recurrent potentially life-threatening episodes of hypovolemic shock associated with severe hemoconcentration and hypoproteinemia. Timely recognition is of paramount importance because ISCLS, despite resembling other kinds of hypovolemic shock, requires a peculiar approach, to prevent life-threatening iatrogenic damage. Due to the rarity of this condition with only scattered cases described worldwide, evidence-based recommendations are still lacking. Here, we summarize our 40  years’ experience in treating shock in ISCLS patients to derive a therapeutic algorithm. Records from 12 ISCLS patients (mean follow-up is 6 years, with a mean age at symptoms’ onset of 51.5 years) were informative for treatment modalities and outcome of 66 episodes of shock. Episodes are di vided in three phases and treatment recommendations are the following: prodromal symptoms-signs (growing malaise, oligo-anuria, orthostatic dizziness) last 6–12 h and patients should maintain rigorous bed rest. The acute shock phase lasts 24–36 h. Patients should be admitted to ICU, placed on restrictive infusion of fluids favoring cautious boluses of high-molecular-weight plasma expanders when SAP 
Source: Internal and Emergency Medicine - Category: Emergency Medicine Source Type: research

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Publication date: Available online 3 August 2020Source: Journal of the Formosan Medical AssociationAuthor(s): Yi-Ting Chen, Chieh-Kai Chan, Wen-Yi Li, Tao-Min Huang, Tai-Shuan Lai, Vin-Cent Wu, Tzong-Shinn Chu, National Taiwan University Hospital Study Group on Acute Renal Failure (NSARF)
Source: Journal of the Formosan Medical Association - Category: General Medicine Source Type: research
Authors: Disoteo OE, Zampetti B, Garascia A, Attanasio R, Cozzi R Abstract A 24-yo female was admitted for acute renal failure, melanoderma, hyponatremia and hyperkalemia. The clinical suspicion of Addison's disease was confirmed by laboratory test and the appropriate replacement therapy with corticosteroids and fludrocortisone was started. In the mean-time primary hypothyroidism and diabetes mellitus type 1 were disclosed and treated, thus fulfilling a diagnosis of autoimmune polyendocrine syndrome type 2. Eighteen months later she was admitted for right sided heart failure. The work-up allowed to diagnose pulmona...
Source: Endocrine, Metabolic and Immune Disorders Drug Targets - Category: Drugs & Pharmacology Tags: Endocr Metab Immune Disord Drug Targets Source Type: research
Condition:   COVID-19 Interventions:   Drug: Apixaban 2.5 MG;   Drug: Apixaban 5MG;   Drug: Aspirin;   Drug: Placebo Sponsors:   Frank C Sciurba;   National Heart, Lung, and Blood Institute (NHLBI) Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Authors: Owens J, Otero JE, Noiseux NO, Springer BD, Martin JR Abstract Background: As the population ages, rate of total knee arthroplasty increases and thus, it is important to maximize efficiency and minimize risk. Identifying patients who are at higher risk for transfusion can help streamline care provided and minimize superfluous, costly hemoglobin monitoring in low risk patients. Methods: Adult patients who underwent total knee arthroplasty (TKA) in 2015 were identified in the National Surgical Quality Improvement Project (NSQIP) database. Patients were divided into two cohorts: those who required transfu...
Source: Iowa orthopaedic journal - Category: Orthopaedics Tags: Iowa Orthop J Source Type: research
The current pandemic of coronavirus disease 2019 (COVID-19) spotlighted the vulnerability of patients with chronic kidney disease stage 5 on maintenance hemodialysis (HD) to the viral infection. Social distancing is the most effective preventive measure to reduce the risk of infection. Nonetheless, the necessity to frequently reach the dialysis center and the inherent social gathering both impede social distancing and also self-quarantine for infected individuals. A baseline hyperinflammatory state driven by factors such as the retention of uremic toxins afflicts these patients. Concomitantly, a condition of relative immun...
Source: Nephron - Category: Urology & Nephrology Source Type: research
We report the first case of COVID-19 presenting with generalized seizure secondary to cerebral venous sinus thrombosis.Case Rep Neurol 2020;12:260 –265
Source: Case Reports in Neurology - Category: Neurology Source Type: research
Metformin-associated lactic acidosis is a rare side effect in metformin poisoning. There is conflicting data about mortality rates changing from 3% to 83%. We aimed to discuss a case that developed lactic acidosis and acute renal failure progressing to mor...
Source: SafetyLit - Category: International Medicine & Public Health Tags: Alcohol and Other Drugs Source Type: news
Publication date: September–October 2020Source: Journal of Orthopaedics, Volume 21Author(s): Peter A. Gold, Terence Y. Ng, Josephine R. Coury, Luke J. Garbarino, Nipun Sodhi, Michael A. Mont, Giles R. Scuderi
Source: Journal of Orthopaedics - Category: Orthopaedics Source Type: research
Purpose of review Nonsevere hemophilia A (NSHA) patients have received relatively little clinical and research attention as compared with their severe counterparts. There is increasing recognition that despite their milder bleeding phenotype, the management of NSHA can be a challenge, with most management decisions largely inferred from severe hemophilia A data. This review focuses on some of the more recent developments in the field of NSHA. Recent findings Epidemiologic studies suggest that NSHA remain under-recognized and under-diagnosed globally. As the NSHA population ages, they are susceptible to age-related com...
Source: Current Opinion in Hematology - Category: Hematology Tags: HEMOSTASIS AND THROMBOSIS: Edited by Paul F. Bray Source Type: research
Purpose of review Advances in medical care and preventive measures have contributed to increasing life expectancy. Therefore, it is critical to expand our understanding of the physiological and pathophysiological adaptations of the hematological system in aging. We highlight and review the findings from recent investigations aimed at understanding the effects of aging on megakaryocytes and platelets. Recent findings Biochemical and transcriptomic studies of megakaryocytes and platelets from older humans and mice have advanced our understanding of the molecular and functional characteristics of megakaryocytes and plate...
Source: Current Opinion in Hematology - Category: Hematology Tags: HEMOSTASIS AND THROMBOSIS: Edited by Paul F. Bray Source Type: research
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