scholarly journals Ophthalmoplegia in the Acute Phase of Hemolytic Uremic Syndrome: A Case Report

2021 ◽  
Author(s):  
Müge Ayanoğlu ◽  
Ferah Sönmez ◽  
Ayşe Tosun ◽  
Dilek Yılmaz
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Acute Phase ◽  
Fresh Frozen Plasma ◽  
Neurological Involvement ◽  
Ocular Involvement ◽  
Diffusion Weighted Images ◽  
Fresh Frozen ◽  
Uremic Syndrome ◽  
Extrarenal Complications

Hemolytic uremic syndrome (HUS) is a common form of thrombotic microangiopathies. Among its extrarenal complications, ocular involvement is very rare. We present a patient with HUS, whose first symptom was isolated abduction deficits in the eyes. Lethargy was added during the clinical course, suggesting neurological involvement. Although conventional magnetic resonance imaging was normal, symmetric diffusion restriction was detected in bilateral putamen on diffusion-weighted images. Treatment with peritoneal dialysis, fresh frozen plasma infusions, and eculizumab was initiated. The patient responded well to the treatment and was discharged with excellent neurological, hematological, and ophthalmological outcomes. Nephrological follow-up is being continued due to proteinuria To our knowledge, presenting with ophthalmoplegia in the acute phase of hemolytic uremic syndrome is very rare. The patient’s ophthalmological and neurological symptoms improved after eculizumab treatment. We suggest that eculizumab is effective in the acute phase of HUS in cases of ophthalmological involvement.

Neurological Involvement in Pediatric Hemolytic Uremic Syndrome: A Symptom-Oriented Analysis

2017 ◽  
Vol 48 (05) ◽  
pp. 363-370 ◽  
Author(s):  
Elif Özdoğan ◽  
Gülay Kaya ◽  
Nezir Özgün ◽  
Ali Cansu ◽  
Mukaddes Kalyoncu ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Neurological Symptoms ◽  
Neurological Involvement ◽  
C Reactive Protein ◽  
Protein Levels ◽  
Reactive Protein ◽  
Uremic Syndrome ◽  
Leukocyte Counts ◽  
Involvement Rate

Background Neurological involvement is common in hemolytic uremic syndrome (HUS), but each neurological symptom may be due to a variety of factors. Objective We aimed to evaluate predisposing factors to the neurological symptoms in HUS. Materials and Methods The 10-year follow-up data on HUS patients were retrospectively analyzed. Statistical comparisons were made across subgroups based on age and neurological symptoms. Results The neurological involvement rate was 37.5%. The female-to-male ratio increased in patients with neurological involvement (3.8 vs. 1.37). Regarding all HUS patients, hemoglobin levels were higher in patients with paresis. The rate of paresis was twofold higher in patients with a hemoglobin level above 11 g/dL (p < 0.05). In diarrhea-associated HUS patients, D-dimer and C-reactive protein levels were higher in patients with paresis, and leukocyte counts were higher in patients with seizures. Patients with altered consciousness had higher creatinine levels. The survival rate was significantly lower in patients with a reticulocyte percentage of less than 2% (50 vs. 100%). Conclusion Results of our study indicate that neurological involvement depends on overall findings in HUS. A symptom-oriented approach, which is different from that employed in previous studies, reveals some clues to the pathogenesis and management of these patients.

scholarly journals Complement activation in children with Streptococcus pneumoniae associated hemolytic uremic syndrome

2021 ◽  
Vol 36 (5) ◽  
pp. 1311-1315 ◽  
Author(s):  
Johannes Holle ◽  
Sandra Habbig ◽  
Alexander Gratopp ◽  
Anna Mauritsch ◽  
Dominik Müller ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Acute Phase ◽  
Alternative Pathway ◽  
Treatment Options ◽  
Laboratory Data ◽  
Infectious Complications ◽  
Pathway Activation ◽  
Uremic Syndrome

Abstract Background Hemolytic uremic syndrome caused by invasive pneumococcal disease (P-HUS) is rare in children and adolescents, but accompanied by high mortality in the acute phase and complicated by long-term renal sequelae. Abnormalities in the alternative complement pathway may additionally be contributing to the course of the disease but also to putative treatment options. Methods Retrospective study to assess clinical course and laboratory data of the acute phase and outcome of children with P-HUS. Results We report on seven children (median age 12 months, range 3–28 months) diagnosed with P-HUS. Primary organ manifestation was meningitis in four and pneumonia in three patients. All patients required dialysis which could be discontinued in five of them after a median of 25 days. In two patients, broad functional and genetic complement analysis was performed and revealed alternative pathway activation and risk haplotypes in both. Three patients were treated with the complement C5 inhibitor eculizumab. During a median follow-up time of 11.3 years, one patient died due to infectious complications after transplantation. Two patients showed no signs of renal sequelae. Conclusions Although pathophysiology in P-HUS remains as yet incompletely understood, disordered complement regulation seems to provide a clue to additional insights for pathology, diagnosis, and even targeted treatment.

scholarly journals Hemolytic Uremic syndrome associated with pregnancy: Outcome from acute Kidney Injury

2020 ◽  
Vol 36 (6) ◽  
Author(s):  
Rubina Naqvi
Keyword(s):  
Acute Kidney Injury ◽  
Hemolytic Uremic Syndrome ◽  
Pregnancy Outcome ◽  
Acute Phase ◽  
Case Series ◽  
Kidney Injury ◽  
Renal Recovery ◽  
Co Morbidity ◽  
Uremic Syndrome

Objective: To report here, case series of women developing acute kidney injury (AKI) in association with hemolytic uremic syndrome (HUS) during pregnancy or within days postpartum. Methods: Subjects for the study reported here comprised of a cohort of 49 women referred from OBGYN and diagnosed having AKI and HUS. AKI was defined according to RIFLE criteria and HUS on basis of hematological, biochemical and histological features. All patients had normal size kidneys on ultrasonography and no previous co- morbidity. Results: From January 2000 – January 2020, 49 such women were admitted/ referred to this facility. The mean age of these patients was 29.02±5.258 years. Two had HUS during pregnancy while rest during postpartum. Majority of women had more than one insults these include hemorrhages, intrauterine deaths, operative measure (lower segment caesarean section). Renal replacement was required in all women. Complete renal recovery was observed in 14 patients, while one died during acute phase of illness. CKD-V developed in 17 patients, 16 patients lost long term follow up, but were dialysis free till last follow up and one left against medical advice during acute phase of illness. Treatment with plasmapheresis revealed significantly better renal recovery (p value 0.03) in this group of patients. Conclusion: AKI with background of Hemolytic Uremic Syndrome (HUS) may remain irreversible in many of these young women. Plasmapheresis should be offered to patients with established diagnosis of HUS. doi: https://doi.org/10.12669/pjms.36.6.2931 How to cite this:Naqvi R. Hemolytic Uremic syndrome associated with pregnancy: Outcome from acute Kidney Injury. Pak J Med Sci. 2020;36(6):---------. doi: https://doi.org/10.12669/pjms.36.6.2931 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.

Thrombotic Thrombocytopenic Purpura and the Hemolytic Uremic Syndrome

2002 ◽  
Vol 126 (11) ◽  
pp. 1430-1433
Author(s):  
Joel L. Moake
Keyword(s):  
Platelet Aggregation ◽  
Hemolytic Uremic Syndrome ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Data Extraction ◽  
Fresh Frozen Plasma ◽  
Thrombocytopenic Purpura ◽  
Fresh Frozen ◽  
Uremic Syndrome ◽  
Metalloprotease Activity ◽  
Von Willebrand

Abstract Objective.—To evaluate the usefulness and feasibility of measuring plasma von Willebrand factor (vWF)-cleaving metalloprotease activity (ADAMTS 13) in the differential diagnosis of thrombotic thrombocytopenic purpura (TTP), the hemolytic uremic syndrome, and other thrombotic microangiopathies. Data Sources.—Articles published in the medical literature. Data Extraction and Synthesis.—In TTP, a multimeric form of vWF that is larger than that ordinarily found in the plasma may cause systemic platelet aggregation under the high-shear conditions of the microcirculation. ADAMTS 13 is a divalent cation-activated, vWF-cleaving metalloprotease that converts unusually large vWF multimers derived from endothelial cells into smaller vWF forms in normal plasma. ADAMTS 13 is severely reduced or absent in most patients with TTP. The vWF-cleaving metalloprotease is present in fresh-frozen plasma, cryoprecipitate-depleted plasma (cryosupernatant), and in plasma that has been treated with solvent and detergent. The enzyme is defective in children with chronic relapsing TTP. Infusion of any of the plasma products that contain the vWF-cleaving metalloprotease stops or prevents (for about 3 weeks) TTP episodes in these patients. An immunoglobulin (Ig) G autoantibody to the vWF-cleaving metalloprotease is found transiently in many adult patients with acquired acute idiopathic, recurrent, and ticlopidine/clopidogrel-associated TTP. Patients with acquired TTP require plasma exchange, that is, both infusion of a plasma product containing vWF-cleaving metalloprotease and removal of autoantibody and/or unusually large vWF multimers by plasmapheresis. The pathophysiology of platelet aggregation in bone marrow transplantation/chemotherapy-associated thrombotic microangiopathy, as well as in hemolytic uremic syndrome, is not established. In neither condition is there a severe decrease in plasma vWF-cleaving metalloprotease activity, as there is in TTP. Conclusions.—The presently available lengthy and complicated procedure for estimation of plasma vWF-cleaving metalloprotease activity is not yet practical for rapid diagnostic use. This test has supplanted the equally lengthy and difficult, less specific analysis of plasma vWF multimeric pattern. If the clinical distinction between TTP and hemolytic uremic syndrome is uncertain, it is appropriate to acquire (before therapy) a citrate-plasma sample for the ultimate determination of vWF-cleaving metalloprotease activity.

Atypical hemolytic uremic syndrome & treatment: case study

2021 ◽  
pp. 1-7
Author(s):  
Azhar Ali Khan ◽  
Muhammad Usman Ahraf ◽  
Usman Javaid
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Daily Basis ◽  
Fresh Frozen Plasma ◽  
Atypical Hus ◽  
Fresh Frozen ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Abstract Atypical Hemolytic Uremic Syndrome (aHUS) is considered as an uncommon pathology that usually affects young adults and causes acute kidney injury which can further lead to End Stage Renal Disease (ESRD). Atypical HUS usually occurs due to impairment in alternate pathway of complement system. aHUS are usually sporadic and less than 20% cases are familial. About 50% aHUS cases show no clear initiating factors. Mortality rate has been reduced in atypical HUS over the years due to progress in intensive care and dialysis facility. First treatment option should be PE, with exchange of 1.5 plasma volumes (60-75 ml/kg) per session, under the cover of fresh frozen plasma (FFP). PE needs to be performed on daily basis while waiting for the results of platelet count, LDH and Hb levels to be normal and renal functions showing improvement. Here we present a case in 15-year-old boy who was healthy previously. Continuous...

scholarly journals Hemolytic uremic syndrome related to Shiga-like toxin-producing Escherichia coli with encephalitis hiding a human herpesvirus-6 infection: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Sophie Mounier ◽  
Arthur Gavotto ◽  
Julie Tenenbaum ◽  
Pierre Meyer ◽  
Marc Fila ◽  
...  
Keyword(s):  
Escherichia Coli ◽  
Pericardial Effusion ◽  
Hemolytic Uremic Syndrome ◽  
Central Nervous System Involvement ◽  
Human Herpesvirus ◽  
Neurological Involvement ◽  
Stool Examination ◽  
Human Herpesvirus 6 ◽  
Uremic Syndrome ◽  
Herpesvirus 6

Abstract Background Cardiac and neurological involvement in hemolytic uremic syndrome are life-threatening complications. The most frequent complications of cardiac involvement in hemolytic uremic syndrome are myocarditis and cardiac dysfunction due to fluid overload. Pericarditis remains very rare in hemolytic uremic syndrome. To our knowledge, only five cases of cardiac tamponade associated with hemolytic uremic syndrome have been described in literature. Case summary A 27-month-old Caucasian girl presented with symptoms of nonbloody diarrhea and tonic-clonic seizures. The diagnosis of Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome with central nervous system involvement was made, and stool examination revealed infection with a Shiga-like toxin-producing Escherichia coli. She did not need renal replacement therapy but had severe neurological impairment. The patient’s course was complicated by pericardial effusion. A pericardiocentesis was performed via an apical approach because the pericardial effusion was predominantly surrounding the left ventricle. Effusion analysis showed an exudate and positivity for human herpesvirus-6B on polymerase chain reaction with viremia. This finding was consistent with primary human herpesvirus-6 infection with encephalitis. Conclusion We report this uncommon case of Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome associated with a severe human herpesvirus-6 infection. Secondary isolated pericardial effusion and atypical neurological involvement are uncommon in Shiga-like toxin-producing Escherichia coli hemolytic uremic syndrome and should lead the physician to perform additional investigations.

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