scholarly journals A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizumab

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Prianka Puri ◽  
Anida Hanxhiu ◽  
Daniel V. O’Hara ◽  
Danny Hsu ◽  
Mirna Vucak-Dzumhur
Keyword(s):  
Kidney Injury ◽  
Rare Condition ◽  
Foetal Death ◽  
Case Presentation ◽  
Haemolytic Uremic Syndrome ◽  
Genetic Sequencing ◽  
Planned Pregnancy ◽  
Life Threatening ◽  
Uremic Syndrome ◽  
Antenatal Visits

Abstract Background Pregnancy-related Atypical Haemolytic Uremic Syndrome (P-aHUS) is a rare condition affecting genetically predisposed women during pregnancy. It is often difficult to diagnose and has a significant impact on maternal and foetal outcomes. It is characterised by microangiopathic haemolytic anaemia and kidney injury from thrombotic microangiopathy. Case presentation A 27-year-old female of Lebanese descent presented at 36 weeks’ gestation with foetal death in-utero (FDIU) with placental abruption on a background of previously normal antenatal visits. She was coagulopathic and anaemic with anuric acute kidney injury, requiring emergency Caesarean section, intubation and dialysis. Her coagulopathy rapidly resolved, however, her anaemia and renal dysfunction persisted. A diagnosis of P-aHUS was made, and she was empirically treated with Eculizumab. Her ADAMTS13 level was normal, effectively excluding thrombotic thrombocytopenic purpura. Within 2 weeks of treatment her haematological parameters improved, and her renal function began to recover and within 2 months she became dialysis independent. Conclusion This case highlights the challenges of a timely diagnosis of P-aHUS from other pregnancy-related diseases. Although our patient is dialysis-independent, her risk of relapse remains high with subsequent pregnancies. Currently we are awaiting her genetic sequencing to complete her assessment for underlying mutations and are determining the safest approach to a future planned pregnancy.

scholarly journals Haemolytic uremic syndrome: diagnosis and management

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 1690 ◽  
Author(s):  
Neil S. Sheerin ◽  
Emily Glover
Keyword(s):  
Clinical Presentation ◽  
Tissue Injury ◽  
Thrombus Formation ◽  
Kidney Injury ◽  
Neurological Involvement ◽  
Haemolytic Uremic Syndrome ◽  
Clinical Scenarios ◽  
Recent Developments ◽  
Life Threatening ◽  
Uremic Syndrome

The thrombotic microangiopathies (TMAs) are a group of diseases characterised by microangiopathic haemolysis, thrombocytopenia, and thrombus formation leading to tissue injury. Traditionally, TMAs have been classified as either thrombotic thrombocytopenic purpura (TTP) or haemolytic uremic syndrome (HUS) based on the clinical presentation, with neurological involvement predominating in the former and acute kidney injury in the latter. However, as our understanding of the pathogenesis of these conditions has increased, it has become clear that this is an over-simplification; there is significant overlap in the clinical presentation of TTP and HUS, there are different forms of HUS, and TMAs can occur in other, diverse clinical scenarios. This review will discuss recent developments in the diagnosis of HUS, focusing on the different forms of HUS and how to diagnose and manage these potentially life-threatening diseases.

scholarly journals Life-Threatening Intrapulmonary Hemorrhage due to Vancomycin-Induced Thrombocytopenia: A Case Report

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Haneen Abdalhadi ◽  
Yazan Fahmawi ◽  
Abhijin Das ◽  
Brian Fouty
Keyword(s):  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Ventilator Associated Pneumonia ◽  
Disseminated Intravascular Coagulopathy ◽  
Heparin Induced Thrombocytopenia ◽  
Platelet Antibodies ◽  
Intravascular Coagulopathy ◽  
Life Threatening ◽  
Uremic Syndrome ◽  
Life Threatening Complication

Thrombocytopenia is a rare and sometimes life-threatening complication of Vancomycin. A 52-year-old male patient with acute kidney injury was treated with Vancomycin for ventilator-associated pneumonia. Three days later, his platelets decreased from 172×109/L to 3×109/L over a 36-hour period. The patient developed significant intrapulmonary bleeding leading to profound hypoxemia. Workup was negative for thrombotic thrombocytopenic purpura, disseminated intravascular coagulopathy, atypical hemolytic uremic syndrome, heparin-induced thrombocytopenia, and autoimmune diseases. All recently started medications were discontinued, and the patient was started empirically on methylprednisolone and intravenous immunoglobulin. The patient’s platelets increased, and his airway bleeding stopped within 48 hours; his platelet count returned to normal by 18 days. Vancomycin-dependent anti-platelet antibodies were identified in the patient’s serum by flow cytometry. Thrombocytopenia is an underrecognized complication of Vancomycin that can lead to life-threating bleeding. Stopping Vancomycin may be sufficient to reverse the thrombocytopenia in patients with normal renal function, but more aggressive measures such as steroids, IVIG, and dialysis may be required to stop bleeding and reverse thrombocytopenia in patients with underlying kidney injury who cannot effectively excrete Vancomycin.

scholarly journals Recurrent case of pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS)

2019 ◽  
Vol 12 (1) ◽  
pp. bcr-2018-226571 ◽  
Author(s):  
Dileep Kumar ◽  
Mary King ◽  
Belinda Jim ◽  
Anjali Acharya
Keyword(s):  
Renal Failure ◽  
Haemolytic Anaemia ◽  
Postpartum Period ◽  
Multidisciplinary Approach ◽  
Post Partum ◽  
Rare Condition ◽  
Disease Manifestation ◽  
Haemolytic Uremic Syndrome ◽  
Recurrent Case ◽  
Uremic Syndrome

Pregnancy-induced atypical haemolytic uremic syndrome (P-aHUS) is a rare condition characterised by microangiopathic haemolytic anaemia, thrombocytopenia and renal failure. It accounts for approximately 7% of total HUS cases. Here, we present a case of recurrent P-aHUS in a 25-year-old Hispanic woman. Pregnancy was the clear trigger in both instances, and the disease manifested in first week of the postpartum period. Because of her significant obstetric history, a multidisciplinary approach was adopted to monitor her second pregnancy antepartum and post partum. As the patient developed recurrence of P-aHUS 4 days after her delivery, she was immediately administered eculizumab within few hours of disease manifestation. The patient normalised her haematological parameters within 1 week but sustained dialysis-requiring renal failure for a total of 6 weeks. This case highlights the advances as well as the ongoing uncertainties, especially with respect to the use of eculizumab, in this rare but morbid disease.

scholarly journals Atypical haemolytic uremic syndrome from multiple missenses to a full-blown disease

2019 ◽  
Vol 12 (6) ◽  
pp. e230026 ◽  
Author(s):  
Filipe Santos Mira ◽  
Ana Luísa Nunes ◽  
Ana Rita Elvas ◽  
Nuno Oliveira
Keyword(s):  
Kidney Injury ◽  
Age Related Macular Degeneration ◽  
Activity Levels ◽  
Haemolytic Uremic Syndrome ◽  
Age Related ◽  
Elevated Liver Enzymes ◽  
Factor Inhibitor ◽  
History Of ◽  
Uremic Syndrome ◽  
Endothelial Growth Factor

A 72-year-old woman was admitted to the hospital because of dorsal, lumbar and lower abdomen pain that had started 4 days before. She had a history of age-related macular degeneration (treated with intraocular bevacizumab). Blood tests showed anaemia, thrombocytopaenia, acute kidney injury, elevated liver enzymes and total bilirubin (mainly because of the indirect fraction). Viral serologies and ADAMTS13 activity levels were normal, and stool testing was negative for Escherichia coli-producing Shiga toxins. E. coli was isolated in urine. Atypical haemolytic uremic syndrome triggered by a urinary tract infection or by the vascular endothelial growth factor-inhibitor bevacizumab were the most likely hypothesis. The patient started urgent plasmapheresis and dialysis that lasted for a total of 18 days. There was complete remission and recovery of kidney function allowing for treatment discontinuation, and she was discharged home. After 6 months of follow-up, she shows no signs of relapse.

scholarly journals Plasma exchange for thrombotic microangiopathy secondary to dermatomyositis associated with acute kidney injury and complement activation: a case report with literature review

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Norifumi Hayashi ◽  
Keiichirou Okada ◽  
Yuko Tsuruyama ◽  
Yu Kagaya ◽  
Sho Kumano ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Intensive Care ◽  
Case Report ◽  
Plasma Exchange ◽  
Complement Activation ◽  
Thrombotic Microangiopathy ◽  
Kidney Injury ◽  
Ivig Therapy ◽  
Case Presentation ◽  
Life Threatening

Abstract Background Thrombotic microangiopathy (TMA) in patients with connective tissue disease is rare but life-threatening. In particular, the survival rate of patients with dermatomyositis (DM) that develop TMA is low. The effectiveness of plasma exchange (PEX) therapy is unclear for the treatment of TMA secondary to DM. Case presentation We describe a case of a 28-year-old woman who developed severe DM complicated by aspiration pneumonia from dysphagia and acute kidney injury. The patient was unresponsive to corticosteroids and intravenous immunoglobulin (IVIG) therapy and developed TMA. In this case, immunofluorescence of skin biopsy revealed that complement activation was involved in the pathogenesis of DM. After 6 PEX therapies, thrombocytopenia improved. She was successfully treated by intensive care and PEX therapy. Conclusions PEX therapy was effective to treat TMA secondary to DM associated with complement activation.

scholarly journals Eculizumab therapy on a patient with co‐existent lupus nephritis and C3 mutation‐related atypical haemolytic uremic syndrome: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Mi Jung Kim ◽  
Haekyung Lee ◽  
Yon Hee Kim ◽  
So Young Jin ◽  
Hee-Jin Kim ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Immunosuppressive Therapy ◽  
Lupus Erythematosus ◽  
Gene Mutations ◽  
Kidney Injury ◽  
Complement C3 ◽  
Complement Factor ◽  
Haemolytic Uremic Syndrome ◽  
Uremic Syndrome ◽  
Poor Outcomes

Abstract Background Thrombotic microangiopathy (TMA), a rare but serious complication of systemic lupus erythematosus (SLE), is associated with poor outcomes to conventional immunosuppressive therapy. Recently, eculizumab, a humanised monoclonal antibody that blocks the complement factor 5, has been known to effectively treat atypical haemolytic uremic syndrome (aHUS). Here, we report a case of aHUS co-existing with lupus nephritis that was successfully treated with eculizumab. Case presentation A 23-year-old man presented with abdominal pain and diarrhoea. Initial laboratory tests have shown thrombocytopaenia, microangiopathic haemolytic anaemia, and acute kidney injury. Immunologic tests were consistent with SLE. Kidney biopsy have revealed lupus nephritis class IV-G with TMA. Genetic analysis have shown complement C3 gene mutations, which hints the co-existence of lupus nephritis with aHUS, a form of complement-mediated TMA. Although initial treatment with haemodialysis, plasma exchange, and conventional immunosuppressive therapy (steroid and cyclophosphamide) did not appreciably improve kidney function and thrombocytopaenia, the patient was able to respond to eculizumab therapy. Conclusions Due to the similar features of TMA and SLE, clinical suspicion of aHUS in patients with lupus nephritis is important for early diagnosis and prompt management. Timely administration of eculizumab should be considered as a treatment option for aHUS in lupus nephritis patients to yield optimal therapeutic outcomes.

scholarly journals A Case of De Novo Antiglomerular Basement Membrane Disease Presenting during Pregnancy

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
F. A. K. Lodhi ◽  
T. Akcan ◽  
J. N. Mojarrab ◽  
S. Sajjad ◽  
R. Blonsky
Keyword(s):  
Basement Membrane ◽  
De Novo ◽  
Kidney Injury ◽  
Young Female ◽  
Unique Challenge ◽  
Case Presentation ◽  
Oral Steroids ◽  
Life Threatening ◽  
Fetal Safety ◽  
Hypoxic Respiratory Failure

Background. Acute kidney injury (AKI) requiring dialysis during pregnancy is uncommon. We present a case of a young female diagnosed with antiglomerular basement membrane (anti-GBM) disease during pregnancy. Case Presentation. A 23-year-old woman approximately 15 weeks pregnant experienced weakness, nausea, vomiting, and anorexia for one week and anuria for 48 hours. No known drug allergies and no significant social or family history for kidney or genitourinary disease were reported. Laboratory analysis revealed anemia, life-threatening hyperkalemia, AKI, and elevated antiglomerular basement membrane (GBM) antibodies. Renal biopsy revealed 100% cellular crescents, confirming the diagnosis. The patient was treated using plasmapheresis and methylprednisolone followed by oral steroids, azathioprine, and tacrolimus. At 24 weeks and 4 days of gestation, the patient had hypoxic respiratory failure as well as preterm premature rupture of membranes. Due to the development of infection and lack of renal recovery, immunosuppression was discontinued. At 28 weeks and 0 days of gestation, the patient developed uncontrollable hypertension requiring emergent delivery. Postpartum, her hypertension improved without signs of preeclampsia though still requires dialysis. Discussion. Pregnancy presents a unique challenge for providers treating patients with anti-GBM disease. Fetal safety should be considered and risks thoroughly discussed with the patient when choosing an immunosuppressive regimen for this condition.

scholarly journals Severe Adverse Toxic Effects of Low-Dose Methotrexate Treatment on an Ectopic Pregnancy Patient With Methylenetetrahydrofolate Reductase Mutations: A Case Report

2021 ◽  
Vol 8 ◽  
Author(s):  
Huan Yu ◽  
Wenhui Wang ◽  
Haiyan Liang ◽  
Kun Wang ◽  
Bin Ling
Keyword(s):  
Ectopic Pregnancy ◽  
Low Dose ◽  
Methylenetetrahydrofolate Reductase ◽  
Rare Condition ◽  
Skin Lesions ◽  
Toxic Effects ◽  
Literature Report ◽  
Case Presentation ◽  
Dose Methotrexate ◽  
Life Threatening

Background: Low-dose methylenetetrahydrofolate (LD-MTX) has been widely used for the treatment of the ectopic pregnancy (EP) for many decades, and related severe adverse toxic effects are rare. Current studies have shown that the polymorphisms of methylenetetrahydrofolate reductase (MTHFR) gene can decrease the MTX clearance, leading to the metabolite accumulation. However, there is a lack of literature report on an MTHFR gene polymorphism associated with adverse toxic effects resulting from the use of LD-MTX in an EP.Case Presentation: We report a rare case of a 38-year-old female who developed persistent fever, grade IV myelosuppression, skin lesions, mucositis, and liver injury after single dose of LDMTX to treat EP. The personalized genetic testing showed that MTHFR TT (677C>T) and MTHFR AA (1298A>C) were detected. Gradually, the symptoms improved after calcium leucovorin (CF) rescue, continuous renal replacement therapy (CRRT), promoting blood system regeneration, and multiple supportive treatments.Conclusion: This is the first report on the serious adverse toxic effects of LD-MTX on an EP patient with MTHFR mutations. We aim to alert obstetricians and gynecologists to this rare condition. The unexpected life-threatening toxicity with LD-MTX should be highly considered and recognized early. In particular, some easily overlooked gastrointestinal, skin, and mucosal symptoms occur earlier than severe myelosuppression. When toxic effects are suspected, detecting the polymorphisms of an MTHFR gene and monitoring MTX concentration in blood could assist us to formulate individualized and active treatments.

scholarly journals A rare presentation of Kikuchi Disease with Hemolytic Uremic Syndrome

2019 ◽  
Vol 35 (2) ◽  
Author(s):  
Salwa Tauseeq Khan ◽  
Rubina Naqvi ◽  
Rahma Rashid ◽  
Sana Abbas Naqvi
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Case Reports ◽  
Kidney Injury ◽  
Rare Condition ◽  
Pathological Findings ◽  
Rare Presentation ◽  
Benign Condition ◽  
Creative Commons ◽  
Uremic Syndrome ◽  
Open Access Article

Kikuchi disease (KD) or also known as Kikuchi Fujimoto disease is named after scientists Kikuchi and Fujimoto who describe the disease in Japan in 1972. KD originally reported from Asia but later case reports from different regions of world have been published. It is a benign condition of necrotizing histiocytic lymphadenitis which mimic like Lymphoma, diagnosis of KD is based on histo-pathological findings from lymphnodes. It is a rare condition and mostly case reports have been published, it can have an association with other pathologies. We aim to report a case where KD has been found in a young woman in association with hemolytic uremic syndrome and acute kidney injury. How to cite this:Khan ST, Naqvi R, Rashid R, Naqvi SA. A rare presentation of Kikuchi Disease with Hemolytic Uremic Syndrome. Pak J Med Sci. 2019;35(2):586-588. doi: https://doi.org/10.12669/pjms.35.2.735 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

scholarly journals Lemierre’s Syndrome: Case Presentation of a Rare and Possibly Life-Threatening Condition

Medicina ◽  
2021 ◽  
Vol 57 (10) ◽  
pp. 1102
Author(s):  
Lucian Giubelan ◽  
Livia Dragonu ◽  
Vlad Pădureanu ◽  
Alexandru Neacșu ◽  
Mirela Mănescu ◽  
...  
Keyword(s):  
Jugular Vein ◽  
Anaerobic Bacteria ◽  
Rare Condition ◽  
Fusobacterium Necrophorum ◽  
Lemierre’S Syndrome ◽  
Case Presentation ◽  
Oropharyngeal Infection ◽  
Threatening Condition ◽  
Life Threatening ◽  
Lemierre's Syndrome

Lemierre’s syndrome is, presently, a very rare condition, but a life-threatening one. The syndrome was first described in 1936 by Andre Lemierre and comprises an oropharyngeal infection (most commonly associated with anaerobic bacteria Fusobacterium necrophorum), internal jugular vein thrombophlebitis and, possibly, secondary septic metastasis (common sites are lungs or brain). We describe such a rare case diagnosed at our Infectious Diseases Department in September 2019.

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