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The complement system in health and disease
Published in Gabriel Virella, Medical Immunology, 2019
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder on the surface of selected hemopoietic stem cell lines and their erythrocyte progeny. The patients develop hemolytic anemia manifested by the intermittent passage of dark urine (due to hemoglobinuria), which usually is more accentuated at night. The spontaneous hemolysis is due to an increased susceptibility of the abnormal population of erythrocytes to complement-mediated lysis. The erythrocytes are not responsible for the activation of the complement system; rather, they are lysed as innocent bystanders when complement is activated.
Therapeutic Pheresis: Precautions and Nursing Intervention
Published in James L. MacPherson, Duke O. Kasprisin, Therapeutic Hemapheresis, 2019
Jeane E. Blust, Judith Parrish
Throughout any pheresis procedure, the nurse should be aware of potential hemolysis. Whenever blood is exposed to a mechanical manipulation there is the potential for hemolysis. Visual inspection of the connection tubings and restricting aspects of the apparatus must be made throughout the entire procedure. In addition, at our facility, upon completion of the first pass, two capillary specimens are collected and centrifuged for visual examination for the presence of hemolysis in the plasma. If there is any suspicion of hemolysis resulting from mechanical destruction of red cells, such as the presence of a pinkish coloring to the plasma layer, the procedure should be discontinued immediately. However, the venous access should be maintained, and this can be accomplished by the addition of 0.9% sodium chloride, injection (normal saline) at a keep vein open (KVO) or maintenance rate of infusion. The medical director of the pheresis unit should be alerted, and a blood specimen from both the patient and the reinfusion bag should be obtained. The specimen from the patient should be collected in an anticoagulated tube. Since the reinfusion product is anticoagulated, a tube without anticoagulant can be used to collect this specimen. If at all possible, a urine specimen should be collected to observe for the presence of hemoglobinuria depending on the clinical condition of the patient. Increased fluid intake is encouraged.
Burns
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
Manage haemoglobinuria by encouraging a high urine output, e.g. forced alkaline diuresis: 12.5 g/L mannitol25 mmol/L NaHCO3
Bilateral anterior segment dysgenesis and peripheral avascular retina with tractional retinal detachment in an infant with multiple congenital anomalies-hypotony-seizures syndrome 3
Published in Ophthalmic Genetics, 2021
Frances Wu, Paula C. Goldenberg, Shizuo Mukai
Genetic testing for familial exudative vitreoretinopathy (FEVR), Norrie disease, and Wagner syndrome were negative. Whole-exome sequencing (WES) revealed compound heterozygous mutations in the PIGT gene, consisting of a maternally inherited c.918dupC pathogenic variant and paternally inherited c.1580A>G variant of uncertain significance (VUS). The patient was found to have developmental delay, microcephaly with head circumference less than 1st percentile for age, and hearing impairment treated with hearing aids. Facial features were consistent with those described in MCAHS3; specifically, depressed nasal bridge, short anteverted nose, prominent philtrum, full cheeks, and open mouth characteristic of hypotonia (1). There was one seizure-like episode, but subsequent electroencephalogram and brain MRI were normal. Testing for paroxysmal nocturnal hemoglobinuria (PNH) was negative. Echocardiogram and ultrasound of the kidneys and bladder were normal.
Macroscopic hematuria in wasp sting patients: a retrospective study
Published in Renal Failure, 2021
Maohe Wang, Singh Prince, Yong Tang, Xiang Zhong, Shasha Chen, Guisen Li, Li Wang, Wei Wang
Our research also has limitations. Ours is a retrospective study, hence there may be selection bias in addition to possible confounding. The study data came from a single center and we did not have complete information such as wasp species, sting site, prognosis and follow-up of patients with AKI. We did not consider the possible effect of microscopic hematuria, and we also didn't distinguish between hemoglobinuria and myoglobinuria. Although the PSS has a high accuracy in predicting the prognosis of wasp sting patients, the severity of wasp sting patients from our study highlighted that the number of stings, time of the year also correlate with worse outcomes. PSS criteria may benefit with incorporation of these variables. Twelve patients had diabetes, but no evidence of diabetic nephropathy was found. Our study did not adjust for risk of AKI with preexisting diabetes. To our knowledge, this is the largest study in terms of study subject number to review macroscopic hematuria in wasp sting patients in terms of clinical features and outcomes.
Microwave ablation versus transcatheter arterial embolization for large hepatic hemangiomas: clinical outcomes
Published in International Journal of Hyperthermia, 2020
Yaoping Shi, Jingjing Song, Min Ding, Xiaoyin Tang, Zhi Wang, Jiachang Chi, Tao Wang, Jiansong Ji, Bo Zhai
The main disadvantage of MWA for the treatment of large hepatic hemangiomas is hemolysis attributable to the generous blood supply of hepatic hemangiomas. Hemolysis can lead to various degrees of hemoglobinuria, hemolytic jaundice, anemia or even renal damage accordingly, depending on its severity. It is worth noting that intraoperative quick infusion followed by intravenous use of 125 mL of sodium bicarbonate solution and 20 mg of furosemide was effective for preventing acute renal dysfunction. In our study, three early patients who did not receive preventive measures experienced acute renal dysfunction. However, none of the later patients suffered acute renal dysfunction. Furthermore, to prevent injury to the surrounding organisms, such as the diaphragm, gastrointestinal tract, gall bladder and biliary tract, it is not necessary to pursue complete ablation of a hemangioma. In this study, all nine patients with incomplete ablation had tumors located abutting either the diaphragmatic dome or hepatic hilum. However, none of them need further treatment because of the small size and slow growth of the residual tissue.