Maple syrup urine disease (branched-chain oxoaciduria)
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop in Atlas of Inherited Metabolic Diseases, 2020
A number of variants of branched-chain oxoaciduria has been described in addition to the classic, severe, or neonatal form [41]. Each is milder in its clinical presentation than classic MSUD. The first of these to be described [42–45] and the second most common form has been referred to as intermittent branched-chain ketoaciduria. Involved individuals may have no problems except in the presence of some special stress, such as infection or surgery. On the other hand, this disorder too can be lethal. Patients with no symptoms at all for a period of years can suddenly develop coma, convulsions, and death following an apparently mild infection [45]. More commonly, these patients have intermittent bouts of acute ataxia. In one report, severe acidosis was a prominent clinical finding [44]. In previous editions of this book, we have cited our experience that the biochemical abnormality of the accumulation of amino acids and oxoacids in body fluids is not intermittent, even though the clinical manifestations may be. In all of these patients, the biochemical features were always demonstrable, except of course when successfully treated. We have now encountered patients who clearly fit the definition of intermittent even of the biochemistry.
Halogenated hydrocarbons
Bev-Lorraine True, Robert H. Dreisbach in Dreisbach’s HANDBOOK of POISONING, 2001
Special problems: Bronchospasm complicating pulmonary edema or bronchial pneumonia is treated by aminophylline given intravenously; repeat as necessary.Treat renal failure.Treat acidosis.Treat methanol intoxication if necessary.Treat bacterial pneumonia with organism-specific chemotherapy.
Blood gas interpretation
Philip Woodrow in Nursing Acutely Ill Adults, 2015
Metabolic acidosis can have varied causes, including kidney disease (hydrogen ions not excreted)liver failure (little bicarbonate produced)tissue acids (e.g. lactic, ketoacids)hyperchloraemic.
Preparation and exchange transfusion effect of a double polymerization human umbilical cord haemoglobin of red blood cell substitute
Published in Artificial Cells, Nanomedicine, and Biotechnology, 2023
Wentao Zhou, Shen Li, Shasha Hao, Xintong Xie, Honghui Zhang, Jiaxin Liu, Hong Wang, Chengmin Yang
The results showed that the Beecf in the control group was higher than the GDA-HCHb group and the DBBF-GDA-HCHb group at end of ET (−13.78 ± 3.34 vs. −6.57 ± 2.50, −6.75 ± 2.42) (p < 0.05), and the Lac in the DBBF-GDA-HCHb group were significantly lower than the control group and the GDA-HCHb group at the end of ET (3.12 ± 0.63, 3.23 ± 0.48 vs. 1.70 ± 0.16) (p < 0.05). BE, Beecf and Lac are all important indicators to evaluate the body’s acidosis. The higher the Lac content in the body, the more obvious the state of acidosis. The important reason for acidosis is the lack of oxygen in the body. Hypoxic state, causing the body’s anaerobic respiration, results in excess lactic acid [30–32]. If the delivery of oxygenated blood is disrupted, hypoxia occurs and rat’s metabolism shifts to the anaerobic pathway with the production of lactic acid. With high lactic acid production, the buffer system becomes insufficient leading to rat’s acidosis, low pH. And the lower pH was positively correlated with BE increase and a trend towards higher pCO2 levels [30]. Therefore, compared with the control group, the HBOC group can better supply oxygen to the hypoxic tissues and organs. Due to the lower oxygen affinity of DBBF-GDA-HCHb, it can provide sufficient oxygen for the hypoxic body in a better and more timely manner, resulting in a significant decreasing in the lactic acid content of DBBF-GDA-HCHb group.
Vascular injury at laparoscopy: a guide to management
Published in Journal of Obstetrics and Gynaecology, 2018
Victoria Asfour, Edward Smythe, Rizwan Attia
Damage control resuscitation tolerates up to moderate hypotension (systolic of 90 mmHg) with judicious replacement of fluids and blood. Traumatic coagulopathy may be prevented with proactive replacement of platelets and clotting factors, calcium supplementation and red cell transfusions. Acidosis is strongly associated with adverse outcomes. It occurs when the peripheral tissues are hypo-perfused. There is a conversion to anaerobic respiration producing lactate (acidosis). Acidosis directly affects myocardial function leading to a low cardiac output state. Hypothermia exacerbates acidosis, promotes coagulopathy, arrhythmias, enzymatic activity and further reduces cardiac output (Sandadi et al. 2010; Miller 2013). Hypothermia can be avoided by using warmed intravenous fluids, use of the bear hugger blanket, a prompt diagnosis and the repair of the bleeding point (Figures 1–3).
Arterial blood gas analysis: base excess and carbonate are predictive of noninvasive ventilation adaptation and survival in amyotrophic lateral sclerosis
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Umberto Manera, Maria Claudia Torrieri, Cristina Moglia, Antonio Canosa, Rosario Vasta, Fulvia Ribolla, Francesca Palumbo, Luca Solero, Gabriele Mora, Alessio Mattei, Adriano Chiò, Andrea Calvo
The correlation between ABG parameters and survival was found to be stronger before NIV starting (8): this could be due to the real efficacy of ventilatory therapy, that can normalize pCO2 and HCO3− blood levels if performed correctly, thus reducing the strength of correlation between ABG values and survival. The inverse correlation between pH and survival could be related to the chronic HCO3− increase in ALS patients with nocturnal hypoventilation (9); so a higher pH is a sign of chronic compensation during a respiratory failure of longer duration and thus a marker of worse prognosis in ALS. Indeed acidosis is not a common event in our patients, being usually associated with an acute respiratory failure concomitant to pneumonia or other precipitant causes (10).
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