scholarly journals ECMO with Systemic Heparinization as a Rescue Therapy for Acute Life-Threatening Pulmonary Thromboembolism Complicating Nephrotic Syndrome

2020 ◽  
Author(s):  
Kyusang You ◽  
So Mi Kim ◽  
Dongmin Kim ◽  
Kyoungmin Ryu ◽  
Mee Jeong Lee
Keyword(s):  
Nephrotic Syndrome ◽  
Pulmonary Thromboembolism ◽  
Rescue Therapy ◽  
Systemic Heparinization ◽  
Life Threatening

Coeliac crisis mimicking nephrotic syndrome in a post-partum patient

2019 ◽  
Vol 64 (3) ◽  
pp. 116-118
Author(s):  
Özant Helvacı ◽  
Seyma Yıldız ◽  
Berfu Korucu ◽  
Ulver Derici ◽  
Turgay Arinsoy
Keyword(s):  
Nephrotic Syndrome ◽  
Coeliac Disease ◽  
Post Partum ◽  
Lower Extremity Weakness ◽  
Case Presentation ◽  
Multisystem Involvement ◽  
Life Threatening ◽  
History Of ◽  
Rare Occasion

Background Coeliac crisis is a life-threatening presentation of coeliac disease. Severe diarrhoea, weight loss, electrolyte imbalances and malnutrition are prominent features. Although mainly a disease of childhood, it can on the rare occasion be diagnosed in adults. Case presentation A 25-year-old female with severe generalised oedema, lower extremity weakness, hypokalemia and profound hypoalbuminemia was referred with an initial diagnosis of nephrotic syndrome. Three months previously she had given birth to a healthy child following an uneventful pregnancy. She did not have proteinuria. She had a history of diarrhoea with gluten-containing food since childhood but lacked a formal diagnosis of coeliac disease. A duodenal biopsy confirmed the suspected diagnosis. Coeliac crisis was diagnosed with life-threatening multisystem involvement. Introduction of a gluten-free diet abolished all disease symptoms and ameliorated laboratory parameters at six months’ follow-up. Conclusion Coeliac crisis is a rare, yet dangerous presentation of coeliac disease in adults. As this case suggests, it can present with generalised oedema and hypoalbuminemia mimicking nephrotic syndrome. Rapid diagnosis is the key to successful treatment.

scholarly journals Life-threatening COVID-19 presenting as stroke with antiphospholipid antibodies and low ADAMTS-13 activity, and the role of therapeutic plasma exchange: A case series

2020 ◽  
Vol 8 ◽  
pp. 2050313X2096408
Author(s):  
Abdulrahman Alharthy ◽  
Fahad Faqihi ◽  
Abdullah Balhamar ◽  
Ziad A Memish ◽  
Dimitrios Karakitsos
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Plasma Exchange ◽  
Plasma Volume ◽  
Antiphospholipid Antibodies ◽  
Rescue Therapy ◽  
Antiviral Treatment ◽  
Case Series ◽  
Therapeutic Plasma Exchange ◽  
Life Threatening

We present a case series of three patients with COVID-19 who were admitted to our intensive care unit due to acute respiratory distress syndrome, brain infarction, pulmonary embolism, and antiphospholipid antibodies. We applied therapeutic plasma exchange on all cases. On intensive care unit admission, all patients had low (<10) Glasgow Coma Scale, and central nervous imaging showed multiple brain infarctions. COVID-19 was confirmed by reverse transcriptase polymerase chain reaction assays. Patients underwent rescue therapeutic plasma exchange using the Spectra OptiaTM Apheresis System (Terumo BCT Inc., USA), which operates with acid-citrate dextrose anticoagulant as per Kidney Disease Improving Global Outcomes 2019 guidelines. A dose of 1.5 plasma volume was used for the first dose and then 1 plasma volume daily for a total of five doses. Plasma was replaced with Octaplas LG® (Octapharma AG, USA), which is an artificial fresh frozen plasma product that has undergone viral inactivation by prion reduction technology. We administered ARDS-net/prone positioning ventilation, empiric antiviral treatment, therapeutic anticoagulation, and intensive care unit supportive care. Laboratory tests showed lymphocytopenia; elevated levels of D-dimer, fibrinogen, total bilirubin, C-reactive protein, lactate dehydrogenase, and ferritin; as well as low levels of ADAMTS-13 activity and antibody. Serology tests depicted positive IgM and IgG antiphospholipid antibodies (anti-cardiolipin and anti-β2-glycoprotein I antibodies). No side effects of therapeutic plasma exchange were recorded. After the completion of therapeutic plasma exchange, patients improved clinically and gradually recovered neurologically (after 27–32 days). To conclude, in life-threatening COVID-19, especially when immune dysregulation features such as antiphospholipid antibodies exist, therapeutic plasma exchange could be an effective rescue therapy.

scholarly journals Anakinra in the treatment of protracted paradoxical inflammatory reactions in HIV-associated tuberculosis in the United Kingdom: a report of two cases

2020 ◽  
Vol 31 (8) ◽  
pp. 808-812 ◽  
Author(s):  
Alexander J Keeley ◽  
Vivak Parkash ◽  
Anne Tunbridge ◽  
Julia Greig ◽  
Paul Collini ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Therapeutic Option ◽  
Interleukin 1 ◽  
High Dose ◽  
Inflammatory Reactions ◽  
Amyloid A ◽  
The United Kingdom ◽  
First Case ◽  
Life Threatening ◽  
Dose Corticosteroid

Paradoxical reactions, including immune reconstitution inflammatory syndrome (IRIS), are common in patients co-infected with human immunodeficiency virus (HIV) and tuberculosis (TB). Paradoxical reactions may confer substantial morbidity and mortality, especially in cases of central nervous system (CNS) TB, or through protracted usage of corticosteroids. No high-quality evidence is available to guide management in this scenario. Interleukin-1-mediated inflammation has been implicated in the pathophysiology of TB–IRIS. We describe two cases where anakinra (human recombinant interleukin-1 receptor antagonist) was used as steroid-sparing therapy for life-threatening protracted paradoxical inflammation in HIV-associated TB. In the first case of disseminated TB with lymphadenitis, protracted TB–IRIS led to amyloid A amyloidosis and nephrotic syndrome. In the second case of disseminated TB with cerebral tuberculomata, paradoxical inflammation caused unstable tuberculomata leading to profound neuro-disability. In both cases, paradoxical inflammation persisted for over a year. Protracted high-dose corticosteroid use led to adverse events yet failed to control inflammatory pathology. In both patients, anakinra successfully controlled paradoxical inflammation and facilitated withdrawal of corticosteroid therapy. Following anakinra therapy, nephrotic syndrome and neuro-disability resolved, respectively. Anakinra therapy for protracted paradoxical inflammation in HIV-associated TB may be a viable therapeutic option and warrants further research.

scholarly journals Cerebral Sinovenous Thrombosis in a Child with Idiopathic Nephrotic Syndrome

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
L. Ghedira Besbes ◽  
S. Haddad ◽  
A. Gabsi ◽  
M. Hassine ◽  
Ch. Ben Meriem ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Magnetic Resonance ◽  
Cranial Computed Tomography ◽  
Renal Disorder ◽  
Heavy Proteinuria ◽  
Steroid Dependent ◽  
Cerebral Sinovenous Thrombosis ◽  
Sinovenous Thrombosis ◽  
Life Threatening ◽  
Nephrotic Children

Nephrotic syndrome (NS) is a renal disorder characterized by heavy proteinuria, hypoalbuninemia, edema and hypercholesterolemia. Nephrotic syndrome in children is known to be associated with an hypercoagulable state and thromboembolic complications. However cerebral sinovenous thrombosis (CSVT) is very rare. Here we report a seven-year-old child with steroid-dependent idopathic nephrotic syndrome resulting from a minimal change disease, developed multiple cerebral sinovenous thrombosis, presenting with headache, left sixth nerve palsy, and papilledema. The diagnosis of CSVT was established by cranial computed tomography, magnetic resonance imaging, and magnetic resonance angiography. He gradually recovered after anticoagulant therapy. CSVT is very rare in nephrotic children. The diagnosis of CSVT should be considered in any patient with nephrotic syndrome who develops neurologic symptoms. This report highlights the importance of suspecting and recognizing this potentially life threatening complication and initiating early treatment.

scholarly journals Pneumocystis jirovecii Pneumonia in Patients with Nephrotic Syndrome: Application of Lymphocyte Subset Analysis in Predicting Clinical Outcomes

2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Yang Liu ◽  
Ke Zheng ◽  
Yecheng Liu ◽  
Huadong Zhu
Keyword(s):  
Nephrotic Syndrome ◽  
T Lymphocyte ◽  
Lymphocyte Count ◽  
Clinical Manifestations ◽  
Multivariate Logistic Regression Analysis ◽  
Pneumocystis Jirovecii ◽  
Pneumocystis Jirovecii Pneumonia ◽  
Confirmatory Tests ◽  
Life Threatening ◽  
Hiv Negative

Purpose. With immunosuppressants being widely used, Pneumocystis jirovecii pneumonia (PCP) has been increasing and could be life-threatening among HIV-negative patients. This study aimed at identifying prognostic factors of PCP in patients with nephrotic syndrome. Methods. We retrospectively investigated patients with nephrotic syndrome who were diagnosed with PCP. The diagnosis of PCP was based on clinical manifestations, radiological findings, and microbiological confirmatory tests. Predictors of outcome were determined with multivariate logistic regression analysis. Results. A total of 57 patients were included in this study. The PCP mortality was 33.3%, which increased to 48.6% if ICU admission was required and to 60% when mechanical ventilation was needed. The T lymphocyte count and CD4/CD8 ratio independently predicted the outcome of PCP, so did the CD4+ T lymphocyte count (OR, 0.981; 95% CI, 0.967–0.996; p=0.001). The cut-off value of 71 cells/μl for the CD4+ T lymphocyte count was determined to identify patients with poor prognosis. No association was found between PCP mortality and the type of immunosuppressant used. Conclusions. PCP is a fatal complication among nephrotic syndrome patients receiving immunosuppressive therapy. The CD4+ T lymphocyte count is suggested as an independent predictor of prognosis, which can be used clinically to identify patients with high risk of unfavorable outcomes.

Rationale and efficacy of interleukin-1 targeting in Erdheim–Chester disease

Blood ◽  
2010 ◽  
Vol 116 (20) ◽  
pp. 4070-4076 ◽  
Author(s):  
Achille Aouba ◽  
Sophie Georgin-Lavialle ◽  
Christian Pagnoux ◽  
Nicolas Martin Silva ◽  
Amédée Renand ◽  
...  
Keyword(s):  
Rescue Therapy ◽  
Interleukin 1 ◽  
C Reactive Protein ◽  
Reactive Protein ◽  
Erdheim Chester Disease ◽  
Poor Tolerance ◽  
Life Threatening ◽  
And Control ◽  
Erdheim Chester ◽  
Chester Disease

Abstract Erdheim–Chester disease (ECD) pathophysiology remains largely unknown. Its treatment is not codified and usually disappointing. Interferon (IFN)-α therapy lacks efficacy for some life-threatening manifestations and has a poor tolerance profile. Because interleukin (IL)-1Ra synthesis is naturally induced after stimulation by IFN-α, we hypothesized that recombinant IL-1Ra (anakinra) might have some efficacy in ECD. We treated 2 patients who had poor tolerance or contraindication to IFN-α with anakinra as a rescue therapy and measured their serum C-reactive protein, IL-1β, IL-6, and monocytic membranous IL-1α (mIL-1α) levels before, under, and after therapy. Another untreated ECD patient and 5 healthy subjects were enrolled as controls. After treatment, fever and bone pains rapidly disappeared in both patients, as well as eyelid involvement in one patient. In addition, retroperitoneal fibrosis completely or partially regressed, and C-reactive protein, IL-6, and mIL-1α levels decreased to within the normal and control range. Beside injection-site reactions, no adverse event was reported. Therefore, our results support a central role of the IL-1 network, which seemed to be overstimulated in ECD. Its specific blockade using anakinra thereby opens new pathophysiology and therapeutic perspectives in ECD.

scholarly journals Nephrotic syndrome after insect sting: a case report

2020 ◽  
Author(s):  
Vânia Junqueira ◽  
Beatriz Donato ◽  
Catarina Teixeira ◽  
Maria Isabel Mascarenhas ◽  
Isabel Costa Silva ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Case Report ◽  
Minimal Change Disease ◽  
Pulmonary Thromboembolism ◽  
Adult Population ◽  
Minimal Change ◽  
Sustained Remission ◽  
Insect Sting ◽  
Insect Stings ◽  
New Onset

ABSTRACT Minimal change disease accounts for up to 25% of the cases of nephrotic syndrome in adult population. The allergic mechanism has been associated with minimal change disease and allergens have been implied, namely insect stings. We present a case report of a woman with new onset of nephrotic syndrome after a non-hymenoptera insect sting, with biopsy-proven minimal change disease, that was accompanied by a pulmonary thromboembolism process. Complete remission with glucocorticoid therapy was observed, with sustained response for 6 months after discontinuation. A new exposure to insect sting in the same geographical region and season triggered a nephrotic syndrome relapse. Subsequent avoidance of the place resulted in a sustained remission for more than 4 years.

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