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The aim of this study is to describe a case of haemoglobinuria in a cat after near-drowning. A 6-year-old male neutered domestic short hair cat weighing 6.5 kg with a pre-existing seizure disorder presented to an emergency hospital after near-drowning in a swimming pool during a seizure episode. On presentation, the patient was obtunded, dyspnoeic, bradycardic and hypothermic. Imaging revealed evidence of severe bilateral pulmonary infiltrates. Treatment with intravenous diazepam, amoxicillin, fluid therapy, active warming and oxygen therapy was administered. The cat developed haemoglobinuria approximately 6 h after nearly drowning. Despite improvements in mentation, pulse quality and heart rate, respiratory compromise and poor oxygen saturation persisted, prompting euthanasia approximately 10 h after admission. To the author's knowledge, this is the first reported clinical case of haemoglobinuria following near-drowning in veterinary medicine., (© 2024 The Authors. Veterinary Medicine and Science published by John Wiley & Sons Ltd.)

Background: High-voltage pulses can cause hemolysis., Objectives: The authors evaluated the occurrence of hemoglobinuria after pulsed-field ablation (PFA) and its impact on renal function in patients with atrial fibrillation (AF)., Methods: A consecutive series of patients with AF undergoing PFA were included in this analysis. The initial patients who did not receive postablation hydration immediately after the procedure were classified as group 1 (n = 28), and the rest of the study patients who received planned fluid infusion (0.9% sodium chloride ≥2 L) after the procedure were categorized as group 2 (n = 75)., Results: Of the 28 patients in group 1, 21 (75%) experienced hemoglobinuria during the 24 hours after catheter ablation. The mean postablation serum creatinine (S-Cr) was significantly higher than the baseline value in those 21 patients (1.46 ± 0.28 mg/dL vs 0.86 ± 0.24 mg/dL, P < 0.001). Of those 21 patients, 4 (19%) had S-Cr. >2.5 mg/dL (mean: 2.95 ± 0.21 mg/dL). The mean number of PF applications was significantly higher in those 4 patients than in the other 17 patients experiencing hemoglobinuria (94.63 ± 3.20 vs 46.75 ± 9.10, P < 0.001). In group 2 patients, no significant changes in S-Cr were noted. The group 2 patients received significantly higher amounts of fluid infusion after catheter ablation than did those in group 1 (2,082.50 ± 258.08 mL vs 494.01 ± 71.65 mL, P < 0.001). In multivariable analysis, both hydration (R 2  = 0.63, P < 0.01) and number of PFA applications (R 2  = 0.33, P < 0.01) were independent predictors of postprocedure acute kidney injury., Conclusions: On the basis of our findings, both the number of PFA applications and postablation hydration were independent predictors of renal insult that could be prevented using planned fluid infusion immediately after the procedure., Competing Interests: Funding Support and Author Disclosures Dr. Chierchia has received compensation for teaching purposes and proctoring from Medtronic, Abbott, Biotronik, Boston Scientific, and Acutus Medical. Dr. Burkhardt is a consultant for Biosense Webster and Stereotaxis. Dr. Di Biase is a consultant for Biosense Webster, Boston Scientific, Stereotaxis, and St. Jude Medical and has received speaker honoraria from Medtronic, Atricure, EPiEP, and Biotronik. Dr. de Asmundis has received research grants on behalf of the center from Biotronik, Medtronic, Abbott, LivaNova, Boston Scientific, AtriCure, Philips, and Acutus and compensation for teaching purposes and proctoring from Medtronic, Abbott, Biotronik, Livanova, Boston Scientific, Atricure, Acutus Medical, and Daiichi Sankyo. Dr. Dello Russo is a consultant for Abbott Medical. Dr, Natale is a consultant for Abbott, Biosense Webster, Biotronik, Boston Scientific, and iRhythm. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Bloody urine is classified in farm animals as hematuria, hemoglobinuria, and myoglobinuria. In small ruminants, discolored urine is reported due to several etiologies which is sometimes fatal. Of these causes are babesiosis, bacillary hemoglobinuria, copper toxicity, and hypophosphatemia., Aim: This study was designed to investigate the clinical, etiological, hematobiochemical, ultrasonographic, and pathological findings in rams and bucks with red urine syndrome., Methods: Eighteen male animals (nine rams and nine bucks) of 6 months to 3 years were examined. Parallel, 10 healthy controls were used. They were admitted due to red urine, voiding of only urine drops, straining during the act of urination, grunting during urination, ventral abdominal edema, and abdominal distension. The duration of the disease ranged from 2 to 30 days. A history of chronic copper toxicosis was informed in two bucks and a ram. Two blood samples were collected from diseased as well as from controls in EDTA tubes (for complete blood count testing) and in plain tubes (for serum collection)., Results: Hematuria was found in 11 animals (seven bucks and four rams) while hemoglobinuria was detected in seven animals (five bucks and two rams). Sonographic findings in diseased animals included ruptured urinary bladder in 3, ruptured urethra in 5, penile calculi, uroperitoneum in 6, distended urinary bladder in 7, hydronephrosis in 5, echogenic deposits in the bladder in 3, and ventral urine accumulation in four animals. Laboratory evaluation of a Geimsa-stained blood smear confirmed the infection with Babesia in three bucks and a ram. Hemolytic anemia was marked in two bucks and a ram due to chronic copper toxicity. Biochemical abnormalities included hypoalbuminemia, hyperglobulinemia, increased blood urea nitrogen and creatinine concentration, and hyperglycemia. Postmortem examination was carried out on six animals (four rams and two bucks)., Conclusion: Discolored urine in rams and bucks in this study resulted from hematuria due to urinary calculi and pelvic abscessation or from hemoglobinuria due to Babesia infection or due to copper toxicity. Hemolytic anemia was the outstanding hematological finding and hypoalbuminemia, hyperglobulinemia, increased blood urea nitrogen (BUN) and creatinine, and hyperglycemia were the characteristic biochemical findings. Sonography of the urinary tract was very helpful in assessing the renal parenchyma, urinary bladder, and abdominal cavity for the verification of urolithiasis, hydronephrosis, intact or ruptured urinary bladder, uroperitoneum, and perforated urethra., Competing Interests: The authors declare that there is no conflict of interest.

Background: March hemoglobinuria is caused by a hemolytic mechanism due to transient hematuria after physical exercise which, although rare, may lead to acute kidney injury. We report a case of a patient with march hemoglobinuria induced by kendo, which was diagnosed by the presence of Berlin blue iron staining in the proximal tubules through renal biopsy., Case Presentation: A 15-year-old male complained of fever (37 °C), general malaise, and nausea after hard kendo sessions. Laboratory findings revealed indirect bilirubin dominant hyperbilirubinemia (total bilirubin 3.8 mg/dL), high lactate dehydrogenase (LDH), and acute kidney injury (serum creatinine: 3.11 mg/dL and estimated glomerular filtration rate: 26 mL/min/1.73m 2 ). Urine test was positive for occult blood but without hematuria. Renal biopsy was performed to clarify the cause of renal injury, which showed minor glomerular abnormalities. Meanwhile, hemosiderin deposition was identified in the proximal tubules by Berlin blue iron staining, and lysosomes were observed to contain granular iron. In addition to clinical background of strenuous kendo exercise, renal biopsy led to a definitive diagnosis of march hemoglobinuria., Conclusions: March hemoglobinuria is a hemolytic disease that can occur after intense exercise, especially kendo. Considering its rarity due to the lack of critical symptoms, it is important to note that occult blood-positive findings may be indicative of march hemoglobinuria if the patient underwent strenuous exercise. Therefore, clinicians should be aware of this possibility to provide timely and appropriate treatment., (© 2022. The Author(s).)

Objectives: To compare the safety and efficacy of microwave ablation (MWA) and radiofrequency ablation (RFA) for such hemangiomas (5-9.9 cm in diameter)., Methods: This multicenter retrospective cohort study investigated the differences in technical success, ablation time, complete ablation, complications, hospital stay, and clinical response between MWA and RFA. A total of 452 patients with hepatic hemangiomas were screened. Propensity score matching was performed. Univariable and multivariate regression analyses were used., Results: Among the 452 patients, 394 met the eligibility criteria and completed the follow-up. After the propensity score matching analysis, 72 pairs of patients were created. No technical failures were found. The RFA group had a longer ablation time (48.63 ± 18.11 min versus [vs.] 37.18 ± 15.86 min, p < 0.001), higher morbidity of hemoglobinuria (77.78% vs. 50.00%, p < 0.001), and longer hospital stay (5.01 ± 1.56 days vs. 4.34 ± 1.42 days, p < 0.05) than the MWA group. The treatment methods (p = 0.032, OR = 0.105, 95% CI = 0.013-0.821), size of the hemangioma (p = 0.021, OR = 5.243, 95% CI = 1.285-21.391), and time of ablation (p = 0.031, OR = 1.145, 95% CI = 1.013-1.294) were significant independent risk factors associated with hemoglobinuria. No recurrence or delayed complications were observed. There were no differences in complete ablation, clinical response, and health-related quality of life between the groups., Conclusions: MWA and RFA appear to be effective treatments for large hepatic hemangiomas. However, MWA had a shorter ablation time than RFA, and MWA was associated with fewer hemolysis-related complications and shorter hospital stays., Key Points: • MWA and RFA appear to be effective treatments for large hepatic hemangiomas. • MWA had a shorter ablation time than RFA. • MWA was associated with fewer hemolysis-related complications and shorter hospital stays., (© 2022. The Author(s), under exclusive licence to European Society of Radiology.)

Background: Information on risk factors of hemoglobinuria after hematopoietic stem-cell transplant (HSCT) and its association with AKI, mortality, and engraftment is limited., Methods: We conducted a retrospective cohort study on all consecutive adults that underwent HSCT from January 6, 1999, to November 6, 2017. The study included 6039 patients that underwent bone marrow transplantation (BMT), umbilical cord blood, and peripheral blood stem-cell transplantation (PBSCT)., Results: Early post-HSCT, AKI occurred in 393 (7%) patients, and 52 (0.9%) patients had post-HSCT hemoglobinuria. Post-HSCT hemoglobinuria was associated with graft type (BMT+Cord), underlying disease (lymphoma, acute leukemia), and fludarabine-based conditioning regimen. Post-HSCT hemoglobinuria was associated with early (48-72 hours) post-HSCT AKI. Graft type (BMT+Cord) was associated with AKI among patients with hemoglobinuria. AKI in patients with hemoglobinuria was associated with delayed platelet engraftment and delayed WBC engraftment but not 100-day mortality., Conclusion: Close monitoring is recommended in this patient group to facilitate a good engraftment outcome., Competing Interests: K. Nath reports being a scientific advisor or member of the Journal of the American Society of Nephrology and Mayo Clinic Proceedings. N. Leung reports consultancy agreements with AbbVie, Lilly, and Omeros; reports having an ownership interest in Checkpoint Therapeutics; and reports receiving research funding from Alnylam and Omeros; reports being a scientific advisor or member of the Journal of Nephrology. All remaining authors have nothing to disclose., (Copyright © 2021 by the American Society of Nephrology.)

A man in his early 50s presented with jaundice, mild shortness of breath on exertion and dark urine. He had had coryzal symptoms 2 weeks prior to admission. Medical history included obstructive sleep apnoea and hypertension. His initial blood tests showed a mild hyperbilirubinaemia and acute kidney injury stage 1. Chest X-ray and CT pulmonary angiogram were negative for features suggestive of COVID-19. He later developed a drop in haemoglobin and repeat bloods showed markedly raised lactate dehydrogenase and positive direct antiglobulin test. These results were felt to be consistent with a haemolytic anaemia. A nasopharyngeal swab came back positive for COVID-19. We suspect the cause of his symptoms was an autoimmune haemolytic anaemia secondary to COVID-19 which has recently been described in European cohorts., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Nil.

Snakebite envenoming is a major health issue in many parts of the world, especially in rural areas. Vipera ammodytes is the commonest cause of snakebite in Greece. We report our experience with a patient bitten by such a snake, who developed massive intravascular hemolysis characterized by a spherocytic rather than microangiopathic hemolytic picture. This case illustrates the potential of snakebite envenoming to cause spherocytic hemolytic anemia associated with hemoglobinuria and acute renal failure, and represents the first report of V. ammodytes in this context. Another important point is that antivenom was rapidly effective in reversing spherocytic hemolytic anemia, even though several hours had elapsed since the bite.

Introduction: The purpose of this study was to analyze the epidemiological, diagnostic, therapeutic and evolutionary features of hemoglobinuria in children hospitalized in the Pediatric University Hospital Charles de Gaulle, Ouagadougou., Methods: We conducted a cross-sectional descriptive study over the period 01 st July-31 st December 2014. All children aged 0-15 years hospitalized in the Department of Medical Pediatrics of the Pediatric University Hospital Charles de Gaulle and diagnosed with macroscopic hemoglobinuria during the study period were enrolled., Results: Thirty-eight patients were included in the study. Hospitalization rate for hemoglobinuria was 1.9%. The average age of patients was 80.8 ± 44.1 months (ranging from 21 to 168). The study involved 23 boys (60.5%) and 15 girls (39.5%). The major clinical signs were: fever (86.8%), dark urines like « coca cola » (86.8%), pallor (63.2%), hepatomegaly (50%). Glomerular filtration flow was less than 80 mL/min/1.73m 2 in 23 patients (69.7%); 21 patients had Glucose-6-phosphate dehydrogenase (G6PD) deficiency. The main suspected causes of hemoglobinuria were: severe malaria, bacterial and viral infections, G6PD deficiency, biliary haemoglobinuric fever. Treatments included: artemisinin derivatives, antibiotics and antipyretics. One patient underwent dialysis., Conclusion: Hemoglobinuria is a symptom mainly causing diagnostic problems in our context. It is a severe disorder which can result in acute renal failure (ARF)., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts., (© Hamidou Savadogo et al.)

Objectives: Sclerotherapy is an essential component of the treatment for venous malformations, and ethanolamine oleate (EO) is known as a useful sclerosing agent. However, macroscopic haemoglobinuria (MH) and subsequent renal impairment are severe complications after sclerotherapy using EO. The present study aimed to clarify the MH risk factors for better peri-operative management of venous malformations., Methods: Data collected during 130 procedures involving 94 patients who were undergoing sclerotherapy using EO for venous malformation were retrospectively analysed. Pre-operative and operative variables, including sex, age, pre-operative body mass index, location, depth, type of lesion, size, number of procedures, type of drainage vein, ratio of sclerosant to air, and injected total dose of 5% EO per body weight (BW), were examined. Univariable analysis and multivariable logistic regression were performed to determine the possible risk factors for MH., Results: Following sclerotherapy, MH occurred in 27.7% of patients, but no patient developed post-operative renal impairment because of aggressive hydration and haptoglobin administration. On univariable analysis, diffuse lesion, lesion size ≥50 cm 2 , and total injected dose of 5% EO ≥ 0.18 mL/kg were found to be the MH risk factors. Multivariable logistic regression analysis identified a total injected dose of 5% EO ≥ 0.18 mL/kg as the significant independent factor contributing to MH risk., Conclusions: Macroscopic haemoglobinuria is a reversible complication if immediate and appropriate interventions with aggressive hydration and haptoglobin administration are performed; therefore, it should be closely monitored following sclerotherapy, especially when using 5% EO ≥ 0.18 mL/kg., (Copyright © 2019 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.)

Aim: To explore the risk factors predicting haemoglobinuria after ultrasound-guided percutaneous microwave ablation (MWA) of liver tumours and discuss the treatments and outcomes., Materials and Methods: The present study comprised 2,829 patients admitted for liver tumours treated with MWA from Jan 2011 to April 2017. Ethics committee approval was waived and informed consent for treatment procedures were obtained from the patients. Haemoglobinuria after MWA was found in 149 patients. The influence of 19 risk factors was assessed. Binary logistic regression and receiver operating characteristic (ROC) curve analysis were used for statistical analysis. The treatments and outcomes of patients with haemoglobinuria were summarised., Results: By univariate analysis, histopathology, liver cirrhosis, MWA volume, MWA energy, and MWA duration were significant risk factors. By multivariate analysis and ROC curve, MWA energy, duration, and volume were identified as predictors of haemoglobinuria after MWA. Drug treatments including kidney protection, adequate hydration, alkalisation of urine, and diuresis were administrated to the patients with haemoglobinuria. One patient progressed to acute kidney injury (AKI) while others had good clinical outcomes., Conclusion: Haemoglobinuria is a controllable side effect after MWA of liver tumours, which is related to high MWA energy, long MWA duration, and great MWA volume. It usually caused few side effects on renal function with correct treatment., (Copyright © 2018 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Competing Interests: Competing interests We have read and understood The BMJ policy on declaration of interests and declare that we have no competing interests.

Haemolytic anaemia caused by a paravalvular leak presenting as progressively worsening red urine. Haemoglobinuria was easily mistaken for gross haematuria, resulting in extensive invasive urological investigation that proved to be futile. Further investigation following an emergency admission led to the realisation that intravascular haemolysis secondary to a paravalvular leakâ€"presenting 43 years following metallic valve insertionâ€"was the cause of discoloured urine and newly presenting symptomatic anaemia. This case highlights that there remains other causes of what often appears to be haematuria, and further exploration of alternative causes should be considered when no urological cause is found., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Here, we present a case of a deceased-donor kidney transplant. The brain-dead donor had received a massive blood transfusion during cardiopulmonary bypass, which lead to hemolysis, hemoglobinuria, acute kidney injury, and renal replacement therapy. The kidney appeared red after in situ flush. Postoperatively, the recipient developed delayed graft function. Protocol biopsy during the postoperative period revealed the widespread deposition of heme pigment in the renal tubules. Massive blood transfusion and cardiopulmonary bypass surgery are associated with hemolysis and heme pigment deposition in the renal tubules, which subsequently lead to acute kidney injury. Kidneys from such donors appear red and, while this does not preclude transplant, are likely to develop delayed graft function.

Intravascular hemolysis can result in hemoglobinuria with acute kidney injury. In this study we systematically explored two in vivo animal models and a related cell culture system to identify hemoglobinuria-triggered damage pathways. In models of stored blood transfusion and hemoglobin (Hb) exposure in guinea pigs and beagle dogs we found that hemoglobinuria led to intrarenal conversion of ferrous Hb(Fe(2+)) to ferric Hb(Fe(3+)), accumulation of free heme and Hb-cross-linking products, enhanced 4-hydroxynonenal reactivity in renal tissue, and acute tubule injury. These changes were associated in guinea pigs with activation of a renal cortex gene expression signature indicative of oxidative stress and activation of the unfolded protein response (UPR). Tubule cells of hemolytic animals demonstrated enhanced protein expression of heme oxygenase and heat shock protein and enhanced expression of acute kidney injury-related neutrophil gelatinase-associated lipocalin. These adverse changes were completely prevented by haptoglobin treatment. The in vivo findings were extrapolated to a MS-based proteome analysis of SILAC-labeled renal epithelial cells that were exposed to free heme within a concentration range estimate of renal tubule heme exposure. These experiments confirmed that free heme is a likely trigger of tubule barrier deregulation and oxidative cell damage and reinforced the hypothesis that uncontrolled free heme could trigger the UPR as an important pathway of renal injury during hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is a non-malignant, acquired clonal hematopoietic stem cell disease that can present with bone marrow failure, hemolytic anemia, smooth muscle dystonias, and thrombosis. We present a case of a 32 year-old-female, G2P2A0 with no past medical history of any systemic illnesses who refers approximately 2 months of progressively worsening constant heartburn with associated abdominal discomfort. CBC showed leukopenia (WBC 2.9 x 103 /µL) with neutropenia (segmented neutrophils 48%), macrocytic anemia (Hgb 6.1 g/dL, hematocrit 20%, MCV,113 fL) and thrombocytopenia (platelet count 59 x 109/L). Abdomino-pelvic CT scan revealed a superior mesenterc vein thrombosis, which was treated initially with low-molecular-weight heparih for full anticoagulation. Peripheral blood flow cytometry assays revealed diminished expression of CD55 and CD59 on the erythrocytes, granulocytes and monocytes.' Paroxysmal nocturnal hemoglobinuria is a rare, clonal, hematopoietic stem-cell disorder whose manifestations are almost entirely explained by complement-mediated intravascular hemolysis. The natural history of PNH is highly variable, ranging from indolent to life-threatening. The median survival is 10 to 15 years, but with a wide range. Thrombosis is the leading cause of death, but others may die of complications of bone marrow failure, renal failure, myelodysplastic syndrome, and leukemia. Anticoagulation is only partially effective in preventing thrombosis in PNH; thus, thrombosis is an absolute indication for initiating treatment with Eculizumab. Nevertheless, bone marrow transplantation (BMT) is still the only curative therapy for PNH but is associated with significant morbidity and mortality.

This study aimed to assess the relevance of laboratory tests in Henoch-Schönlein purpura nephritis (HSPN) classification, and determine accurate classification factors. This prospective study included 694 HSPN patients who underwent ultrasound-guided percutaneous renal biopsy (PRB). Renal specimens were scored according to International Study of Kidney Disease in Children (ISKDC) classification. Meanwhile, blood samples were immediately collected for laboratory examination. The associations between laboratory parameters and HSPN classification were assessed. Significant differences in levels of serum Th1/Th2 cytokines, immunoglobulins, T-lymphocyte subsets, complement, and coagulation markers were obtained between HSPN patients and healthy children. Interestingly, 24h urinary protein (24h-UPRO) levels and urine protein/urine creatinine ratios could determine HPSN grade IIb, IIIa, and IIIb incidences, with areas under ROC curve of 0.767 and 0.731, respectively. At 24h-UPRO >580.35mg/L, prediction sensitivity and specificity were 75.2% and 70.0%, respectively. These values became 53.0% and 82.3%, respectively, with 24h-UPRO exceeding 1006.25mg/L. At urine protein/urine creatinine > 0.97, prediction sensitivity and specificity were 65.5% and 67.2%, respectively, values that became 57.4% and 80.0%, respectively, at ratios exceeding 1.2. Cell and humoral immunity, coagulation and fibrinolytic systems are all involved in the pathogenesis of HSPN, and type I hypersensitivity may be the disease trigger of HSPN. 24h-UPRO levels and urine protein/creatinine ratios could probably forecast the pathological classification of HSPN.

Background: Transfusion of ABO major-incompatible red blood cells (RBCs) can activate the complement system and can cause severe and even lethal acute hemolytic reactions. The activation of the complement system with formation of C3a and C5a (anaphylatoxins) and the release of hemoglobin from the lysed RBCs are thought to mediate clinical signs like fever, hypotension, pain, and acute renal failure. Therapeutic inhibition of the complement cascade in case of ABO-incompatible RBC transfusion would be desirable to ameliorate the signs and symptoms and to improve the outcome of the reaction., Study Design and Methods: A patient with blood group B was erroneously transfused with a unit of group A2 RBCs. Within 1 hour after transfusion she received eculizumab, a monoclonal antibody that binds to the complement component C5 and blocks its cleavage. Clinical and immunohematologic observations are reported here., Results: Hemoglobinemia and hemoglobinuria were present for several hours after transfusion, but she developed no hypotension, no renal failure, and no disseminated intravascular coagulation. As shown by flow cytometry, group A cells survived in the peripheral blood for more than 75 days. No immunoglobulin G was detectable by column agglutination technique on these cells., Conclusion: A low isoagglutinin titer and blood group A2 of the erroneously transfused cells most likely were the reason for the absence of clinical signs during and immediately after the ABO-incompatible transfusion. In the further course, eculizumab successfully protected the incompatible RBCs from hemolysis for several weeks., (© 2014 AABB.)

A 25-year-old man developed macroscopic haemoglobinuria after a persistent dry cough. Although chest radiograph findings were normal, since the serum antibody for Mycoplasma pneumoniae was significantly elevated, a diagnosis infection with this organism was made. Despite the absence of apparent anaemia, a marked increase in serum haemolytic markers and positive result for urine haemoglobin indicated the haemolysis of red blood cells, which was likely to have occurred secondarily to M. pneumoniae infection. Shortly after the initiation of a macrolide antibiotic, clarithromycin, the patient's haemoglobinuria completely disappeared together with a complete resolution of his respiratory symptoms. In this case, due to the lymphocyte-stimulatory nature of M. pneumoniae, an enhanced immune response, such as the production of cold agglutinins, was likely to be involved in the pathogenesis of erythrocyte haemolysis. The immunomodulatory property of clarithromycin was thought to repress the increased immunological reaction and thus enable the resolution of the urine abnormality.

Atypical hemolytic uremic syndrome (aHUS) is a life-threatening thrombotic microangiopathy, and as many as 70% of patients with aHUS have mutations in the genes encoding complement regulatory proteins. Eculizumab, a humanized recombinant monoclonal antibody targeting C5, has been used successfully in patients with aHUS since 2009. The standard maintenance treatment requires life-long eculizumab therapy, but the possibility of discontinuation has not yet been tested systematically. We report the safety of discontinuing eculizumab treatment in 10 patients who stopped treatment with the aim of minimizing the risk of adverse reactions, reducing the risk of meningitis, and improving quality of life while also reducing the considerable treatment costs. Disease activity was monitored closely at home by means of urine dipstick testing for hemoglobin. During the cumulative observation period of 95 months, 3 of the 10 patients experienced relapse within 6 weeks of discontinuation, but then immediately resumed treatment and completely recovered. Our experience supports the possibility of discontinuing eculizumab therapy with strict home monitoring for early signs of relapse in patients with aHUS who achieve stable remission., (Copyright © 2014 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

A 2-year-old African-American male patient with sickle cell trait developed cough, red coloured urine, pallor and fatigue. The patient was hospitalised. Diagnostic workup showed that he was glucose 6 phosphate dehydrogenase (G6PD) deficient in erythrocytes. He also had chest X-ray findings of pneumonia. His urine examination showed the presence of haemoglobin and myoglobin. On repeated questioning it was found that he had a moth ball in his mouth a few days prior to this medical episode. This case illustrates a rarely described complication of myolysis in G6PD deficient persons on exposure to a strong oxidant. A review of the literature showed that most people with G6PD deficiency tolerate exercise well without untoward effect in muscles. However, assay of myoglobin in urine has not been routinely performed in these patients during acute haemolytic episode, and thus it is uncertain how frequent myoglobulinaemia occurs in a similar stress situation., (2014 BMJ Publishing Group Ltd.)

Hemolysis and consequent hemoglobinuria is a well-known cause of acute kidney injury (AKI). Hemolysis has been associated with malpositioned prosthetic valves, but other prosthetic devices may rarely be associated with red cell shear stress. We report a case of a 56-year-old man who presented with hemolysis, AKI, and anemia. During workup, he was found to have anti-phospholipid antibodies, leading to a presumptive diagnosis of catastrophic anti-phospholipid antibody syndrome. However, further investigation revealed the cause of hemolysis to be from aortic root graft dysfunction. Following replacement of the prosthesis, there was complete resolution of his hemolysis and recovery of renal function. This case underscores the need to consider cardiac prosthetic devices in patients presenting with hemoglobinuric AKI.

Microwave assisted liver resection is a useful technique, especially when haemostasis could be difficult to achieve with conventional methods. However, prolonged administration of microwaves can be responsible for intraoperative haemoglobinuria. We describe the first case of acute haemolysis secondary to microwave assisted liver resection.

To evaluate the association between haemoglobinuria and chronic kidney disease (CKD) in sickle cell anaemia (SCA), we analysed 356 adult haemoglobin SS or Sβ(o) thalassaemia patients from the University of Illinois at Chicago (UIC) and 439 from the multi-centre Walk-Treatment of Pulmonary Hypertension and Sickle Cell Disease with Sildenafil Therapy (Walk-PHaSST) cohort. CKD was classified according to National Kidney Foundation Kidney Disease Outcomes Quality Initiatives guidelines. Haemoglobinuria, defined as positive haem on urine dipstick with absent red blood cells on microscopy, was confirmed by enzyme-linked immunosorbent assay in a subset of patients. The prevalence of CKD was 58% in the UIC cohort and 54% in the Walk-PHaSST cohort, and haemoglobinuria was observed in 36% and 20% of the patients, respectively. Pathway analysis in both cohorts indicated an independent association of lactate dehydrogenase with haemoglobinuria and, in turn, independent associations of haemoglobinuria and age with CKD (P < 0·0001). After a median of 32 months of follow-up in the UIC cohort, haemoglobinuria was associated with progression of CKD [halving of estimated glomerular filtration rate or requirement for dialysis; Hazard ratio (HR) 13·9, 95% confidence interval (CI) 1·7-113·2, P = 0·0012] and increasing albuminuria (HR 3·1, 95% CI: 1·3-7·7; logrank P = 0·0035). In conclusion haemoglobinuria is common in SCA and is associated with CKD, consistent with a role for intravascular haemolysis in the pathogenesis of renal dysfunction in SCA., (© 2013 John Wiley & Sons Ltd.)

Objective: The purpose of this article is to study the incidence, risk factors, and treatment of gross hemoglobinuria and oliguria following sclerotherapy for venous malformations., Materials and Methods: The clinical records and imaging studies of 131 patients with venous malformations (57 male and 74 female patients; age range, 2-58 years) who underwent sclerotherapy at our institution between July 1993 and August 2007 were reviewed. Demographic data, the location and estimated size of the malformation, the type and dose of the sclerosing agents, development of postprocedural hemoglobinuria and oliguria, and the treatment given were documented and analyzed., Results: Four hundred seventy-five sclerotherapy procedures were performed on 131 patients, with the number of procedures per patient ranging from 1 to 21 (mean, 3.6 procedures). Sodium tetradecyl sulfate was used in 47% of the procedures, ethanol in 27%, and both agents in 26%. Transient hemoglobinuria occurred after 34% of the sclerotherapy procedures, and 57% of these were associated with transient oliguria, with increased risk with higher adjusted doses (sclerosant volume/weight of patient) for both agents. Resolution of the hemoglobinuria and oliguria with hydration, alkalinization, and diuretics occurred in all patients. The risk of hemoglobinuria increased with higher adjusted dose (sclerosant volume/weight of patient) for both agents and with sclerotherapy of venous malformations affecting the lower extremities and multiple locations., Conclusion: Transient hemoglobinuria and oliguria are common complications of sclerotherapy for venous malformation. Nevertheless, with proper fluid management, all the patients promptly recovered. The risk correlates with the volume of sclerosant (adjusted to patient's weight) and is higher for lower extremity and multiple locations.

Objective: Balloon-occluded retrograde transvenous obliteration has been traditionally based on liquid sclerotherapy. However, overdose and systemic spillage of liquid sclerosant can cause severe complications, such as hemolysis, which lead to hemoglobinuria, allergy, acute respiratory distress syndrome, and other disorders. The purpose of this study was to evaluate the performance of foam sclerotherapy with C-arm CT guidance to reduce the amount of sclerosant and to optimize the safety of balloon-occluded retrograde transvenous obliteration while preserving its efficacy., Materials and Methods: Twenty consecutively registered patients with gastric varices underwent balloon-occluded retrograde transvenous obliteration with polidocanol foam. C-arm CT guidance was used to confirm gas filling of the target vessels. In this retrospective analysis of a prospectively encoded database, total net doses of polidocanol used for transvenous obliteration and of contrast medium used for venography before transvenous obliteration were compared, and subsequent complications, including hemoglobinuria, were documented., Results: In all patients, foam was observed in the target vessels at C-arm CT. The mean dose of polidocanol used for balloon-occluded retrograde transvenous obliteration (3.9 ± 1.5 mL) was significantly smaller (p < 0.001) than the dose of contrast medium used for venography (16.4 ± 7.9 mL). Hemoglobinuria was found in only one patient. Except in one instance of recanalization, full variceal thrombosis was confirmed at contrast-enhanced CT 1 week after transvenous obliteration (success rate, 95%). In one patient, air migrated into the liver during transvenous obliteration but was spontaneously absorbed. No serious complication occurred., Conclusion: Balloon-occluded retrograde transvenous obliteration with polidocanol foam under C-arm CT guidance allowed significant reduction of sclerosant dose and resulted in a low complication rate while a high technical success rate and efficacy were maintained.

Background: Anti-IH is usually a clinically insignificant antibody that may complicate a serologic workup. However, it can occasionally cause hemolysis. We report a rare case of acute hemolysis caused by anti-IH., Case Report: A 60-year-old man with a long history of chronic myelomonocytic leukemia and anemia, blood group A, D+ was found to have an unidentified antibody on serologic workup. He received an A, D+ red blood cell (RBC) unit that was crossmatch compatible by immunoglobulin G indirect antiglobulin test and then experienced an acute hemolytic transfusion reaction with fever, hemoglobinuria, and acute renal failure. The antibody was later identified as an anti-IH with a wide thermal amplitude. The transfused RBCs were later typed as A(2). The patient was subsequently typed as an A(1) individual. The patient recovered completely from the effects of this reaction and was transfused with A(1) RBCs over the next few days with no adverse effect., Conclusion: Anti-IH, which is usually clinically insignificant and often found in A(1), B, and A(1) B individuals, can, on rare occasions, cause acute hemolytic transfusion reactions, especially when an A(2) unit is transfused to an A(1) patient., (© 2011 American Association of Blood Banks.)

The erythrocyte lifespan in haemolytic anemia is shortened while erythropoesis is increased. Important labaratory findings are increased reticulocytes, LDH, indirect bilirubin and a decreased haptoglobin level. The most important diagnostic tool for further work up of hemolytic anemia is the direct antiglobulin test (DAT, Coombs test) to differentiate autoimmune hemolytic anemia (AIHA) from other causes. Another important group are fragmentation syndroms (hemolytic uremic syndrome and thrombotic thrombocytopenic purpura). In these forms of haemolytic anemia fragmented red blood cells can be found in the blood smear together with thrombocytopenia. A severe problem in paroxysmal nocturnal hematuria is the incidence of thrombosis. The following review describes the most important forms of hemolytic anemia in the adult and the diagnostic and therapeutic strategies., (© Georg Thieme Verlag KG Stuttgart · New York.)

Intravascular hemolysis is a rare but potentially life threatening cause of red urine characterized by brisk hemolysis and release of large amounts of hemoglobin into the urine. We present an unusual case of red urine in a 20-year-old male who was subsequently diagnosed with intravascular hemolysis due to an aorto-atrial fistula. Fistula formation was likely secondary to a recently implanted atrial septal occluder, which is a reported but exceedingly rare complication of the device. We discuss the diagnostic approach to hemolytic anemia and conclude with a literature review of other cases of device associated fistula formation and hemolysis.

Introduction: The Twin-Barred Tree Snake, Chrysopelea pelias, is a colubrine that, like other members of the genus Chrysopelea, is able to glide in the arboreal strata. Little is known about the effects of its bite. This report is the first clinically documented bite by this relatively uncommon rear-fanged species., Case Report: The patient was a 19-year-old female who arrived at the Emergency Department (ED) of an urban teaching hospital 6 h after being bitten by a snake that was later provisionally identified as a C. pelias. Noted on presentation were bite marks on the right middle toe with minimal inflammation and tenderness. There was slight numbness over the dorsum of the right foot and discomforting sensation radiating up the thigh that persisted for several days. There was mild pyrexia, but no evidence of any systemic effects. The full blood count did show neutrophil leucocytosis, and transient hemoglobinuria was noted in an initial urine analysis., Discussion: The properties of Duvernoy's secretion of C. pelias remain uncharacterized. In this case, the clinical course featured only the local effects noted above. However, the significant local pain reported by the patient suggests that bites by C. pelias are not necessarily trivial and do require full evaluation and observation in a medical facility. Discussed also is the importance of the establishment of a national registry for animal bites and stings in Malaysia., Conclusion: Such a facility could expedite safe and appropriate management of envenomed patients.

Although delayed hemolytic transfusion reaction (DHTR) has been widely recognized as a serious complication of red blood cell transfusion in patients with sickle cell disease (SCD), there is no consensus on its optimal management. Discontinuation of transfusion is recommended, whereas corticosteroids and immunoglobulins are considered to be beneficial. We report 2 children with sickle cell anemia who were diagnosed with DHTR and experienced a subsequent neurologic event in the course of treatment with corticosteroids. The role of corticosteroids as possible precipitating factors of neurologic complications is discussed. Pending a better understanding of the chain of events of DHTR, SCD children with DHTR should receive steroids with great caution.

Aims: To determine the factors responsible for poor glycaemic control in diabetes and whether any such factors are associated with likely improvement in glycaemic control., Methods: A prospective cohort study of 130 diabetic patients with poor glycaemic control (HbA(1c )> or = 10.0%) with 1-year follow-up in a teaching hospital Diabetes Clinic. Changes in HbA(1c) were measured after 1 year., Results: Poor glycaemic control was attributed to one of 15 possible causes. Those cases due to recent diagnosis of diabetes, inadequate treatment with diet, oral glucose-lowering agents or insulin, exacerbation of co-existent medical problems, recent stressful life-events and missed clinic appointments were all associated with significant improvement in HbA(1c) at 12 months. Patients with low mood or alcohol excess, inadequate blood glucose monitoring, poor exercise/sedentary lifestyle, refusal to take tablets or underdosing and refusal to take insulin at all or to increase the dose were all associated with continuing poor glycaemic control at 12 months. The patients were divided almost equally between the two groups., Conclusions: In patients with poor glycaemic control, it is possible by simple features identified at clinic to predict which individuals are likely to show improvement in control and which will not. These findings have not been reported previously and suggest that about half of individuals with poor control will improve within our current diabetes clinic practice. Additional strategies will be required to address those individuals who are not likely to respond.

A 54-year-old woman was scheduled for the total hip replacement. She was anesthetized with continuous epidural blockade combined with general anesthesia using oxygen, sevoflurane, and propofol. When she received her autologous blood transfusion, we noticed the obstruction of the venous line, and pushed 30 ml of blood with 10 ml syringe. However, it was difficult to return her blood through the venous line. At that time, a sudden onset of red urine without any special symptom occurred. We investigated the cause of the red urine, whether this urine is attributable to hemoglobinuria, myoglobinuria, or porphyria. According to the patient's symptoms during and after the operation, and blood as well as urine examination, a microfilter in the venous line might have led to red blood cell destruction. The filter-induced hemolysis can be life-threatening especially in a child. We conclude that the early recognition and management of reactions associated with blood transfusion is essential during the operation.

A 57-year-old man with lung tumor was scheduled for right middle lobectomy under general anesthesia. The patient received glycerin enema 2 hours before anesthesia. Anesthesia was induced with propofol, fentanyl, ketamine and vecuronium. After the induction, urine of dark-red color was drained through the urinary catheter. Massive (3+) occult blood and few erythrocytes in the urine sediment were observed. Furthermore, blood analysis showed hemolysis with mild renal dysfunction (Cr 1.3 mg x dl(-1)). Although serum CPK and myoglobin increased, there was no apparent symptom that supported the onset of rhabdomyolysis induced by anesthetics, acute myocardial infarction or malignant hyperthermia. At this time, we noticed that blood sample taken before the induction had been hemolysed. With all the above information in mind, we suspected that the main cause of the hemoglobinuria could be the enema and the surgery was canceled. The patient made a good progress with laboratory data normalized on the 4th postanesthesia day. However, rectal ulcer developed as a possible late complication of the enema. Although it is well-known that glycerin enema could cause hemolysis, renal failure and rectal ulcer, the increase of CPK and myoglobin in serum made the diagnosis difficult from other conditions leading to rhabdomyolysis in this case.

Our way of life has led to a massive increase in the prevalence of obesity in adults and children. Therefore diabetes type 2 has also become a pediatric disease. Therapy consists above all of implementing modifications of life style such as a healthy diet and regular physical activity in order to achieve a decrease in body weight. If these measurements prove to be insufficient, medical treatments are introduced, either using metformine or insulin. The screening and treatment of complications (retinopathy, nephropathy) and comorbidities (arterial hypertension, dyslipidemia) will help to decrease mortality on the long haul.