acidosis than the NS and RS groups. TBS was a feasible and safe resuscitation solution in this study and may be an alternative to NS and RS for resuscitation in hemorrhagic shock patients without liver damage.]]>
Acidosis ; Alanine Transaminase ; Animals ; Aspartate Aminotransferases ; Bilirubin ; Blood Glucose ; Blood Urea Nitrogen ; Carbon Dioxide ; Creatinine ; Electrolytes ; Enzyme-Linked Immunosorbent Assay ; Hematocrit ; Hemodynamics ; Humans ; Hydrogen-Ion Concentration ; Liver ; Magnesium ; Male ; Oxygen ; Potassium ; Rats ; Resuscitation ; Shock, Hemorrhagic ; Sodium

Pseudohypoaldosteronism (PHA) in infants is manifested by presence of hyperkalemia, hyponatremia, and metabolic acidosis. At initial stages, PAH is generally suspected as congenital adrenal hyperplasia. Transient PHA has been reported in infants with urinary tract infection and urinary tract malformation. We report a case of 5-month-old infant with failure to thrive and finally diagnosed with transient PHA due to urinary tract infection with vesicoureteral reflux.
Acidosis ; Adrenal Hyperplasia, Congenital ; Failure to Thrive* ; Humans ; Hyperkalemia ; Hyponatremia ; Infant* ; Pseudohypoaldosteronism* ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

BACKGROUND: Information on biochemical changes following rapid transfusion of blood mixtures in liver transplantation patients is limited. METHODS: A blood mixture composed of red blood cells, fresh frozen plasma, and 0.9% saline was prepared in a ratio of 1 unit:1 unit:250 ml. During massive hemorrhage, 300 ml of the blood mixture was repeatedly transfused. A blood mixture sample as well as pre- and post-transfusion arterial blood samples were collected at the first, third, fifth, and seventh bolus transfusions. Changes in pH, hematocrit, electrolytes, and glucose were measured with a point-of-care analyzer. The biochemical changes were described, and the factors driving the changes were sought through linear mixed effects analysis. RESULTS: A total of 120 blood samples from 10 recipients were examined. Potassium and sodium levels became normalized during preservation. Biochemical changes in the blood mixture were significantly related to the duration of blood bank storage and reservoir preservation (average R2 = 0.41). Acute acidosis and hypocalcemia requiring immediate correction occurred with each transfusion. Both the pre-transfusion value of the patient and the blood mixture value were significant predictors of post-transfusion changes in the body (average R2 = 0.87); however, the former was more crucial. CONCLUSIONS: Rapid infusion of blood mixture is relatively safe because favorable biochemical changes occur during storage in the reservoir, and the composition of the blood mixture has little effect on the body during rapid transfusion in liver recipients. However, acute hypocalcemia and acidosis requiring immediate correction occurred frequently due to limited citrate metabolism in the liver recipients.
Acidosis ; Blood Banks ; Blood Safety ; Blood Transfusion ; Citric Acid ; Electrolytes ; Erythrocytes ; Glucose ; Hematocrit ; Hemorrhage ; Humans ; Hydrogen-Ion Concentration ; Hypocalcemia ; Liver Transplantation* ; Liver* ; Metabolism ; Plasma ; Point-of-Care Systems ; Potassium ; Sodium

A 37-year-old male visited the hospital with multiple trauma after traffic accident. Fractures of ribs, left femur, and right humerus and spleen rupture with hemoperitoneum were founded on image studies. He was moved to operation room and general anesthesia was performed for splenectomy. During the operation, excessive high peak inspiratory pressure was observed. After abdominal closure, hypoxia, hypercapnia, and respiratory acidosis were worsened. Veno-venous extracorporeal membrane oxygenation (ECMO) was initiated after the operation. Status of the patient were improved after the application of ECMO. The patient was discharged without significant complication. Despite of several limitations in applying ECMO to patients with abdominal compartment syndrome (ACS) and multiple trauma, severe pulmonary dysfunction in ACS patients may be rescued without open abdomen treatment.
Abdomen ; Accidents, Traffic ; Acidosis, Respiratory ; Adult ; Anesthesia, General ; Anoxia ; Extracorporeal Membrane Oxygenation* ; Femur ; Hemoperitoneum ; Humans ; Humerus ; Hypercapnia ; Intra-Abdominal Hypertension ; Male ; Multiple Trauma ; Respiratory Distress Syndrome, Adult ; Ribs ; Rupture ; Spleen ; Splenectomy

Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency is a rare inborn error of ketone body utilization, characterized by episodic or permanent ketosis. SCOT deficiency is caused by mutations in the OXCT1 gene, which is mapped to 5p13 and consists of 17 exons. A 12-month-old girl presented with severe ketoacidosis and was treated with continuous renal replacement therapy. She had two previously unrecognized mild-form episodes of ketoacidosis followed by febrile illness. While high levels of ketone bodies were found in her blood and urine, other laboratory investigations, including serum glucose, were unremarkable. We identified novel compound heterozygous mutations in OXCT1:c.1118T>G (p.Ile373Ser) and a large deletion ranging from exon 8 to 16 through targeted exome sequencing and microarray analysis. This is the first Korean case of SCOT deficiency caused by novel mutations in OXCT1, resulting in life-threatening ketoacidosis. In patients with unexplained episodic ketosis, or high anion gap metabolic acidosis in infancy, an inherited disorder in ketone body metabolism should be suspected.
Acid-Base Equilibrium ; Acidosis ; Blood Glucose ; Exome ; Exons ; Female ; Humans ; Infant ; Ketone Bodies ; Ketosis ; Metabolism ; Microarray Analysis ; Renal Replacement Therapy ; Transferases

Glyphosate herbicides, which are widely used worldwide, are known to have low toxicity. However, excessive intake may cause serious life-threatening complications; therefore, caution is needed when using them. A 51-year-old man visited the hospital after ingesting glyphosate herbicide. At the time of admission, his vital signs were 80/60 mmHg-115/min-20/min-37.3℃. Electrocardiogram (ECG) showed QRS widening and corrected QT (QTc) prolongation, and blood tests showed metabolic acidosis. Treatment with gastric lavage, activated charcoal, sodium bicarbonate and intravenous lipid emulsion therapy was performed. After 2 hours, his blood pressure increased to 130/90 mg, and no QRS widening was observed on ECG.
Acidosis ; Blood Pressure ; Charcoal ; Electrocardiography ; Gastric Lavage ; Hematologic Tests ; Herbicides ; Humans ; Middle Aged ; Poisoning ; Sodium Bicarbonate ; Vital Signs

Proximal renal tubular acidosis (RTA) is caused by a defect in bicarbonate (HCO₃⁻) reabsorption in the kidney proximal convoluted tubule. It usually manifests as normal anion-gap metabolic acidosis due to HCO₃⁻ wastage. In a normal kidney, the thick ascending limb of Henle’s loop and more distal nephron segments reclaim all of the HCO₃⁻ not absorbed by the proximal tubule. Bicarbonate wastage seen in type II RTA indicates that the proximal tubular defect is severe enough to overwhelm the capacity for HCO₃⁻ reabsorption beyond the proximal tubule. Proximal RTA can occur as an isolated syndrome or with other impairments in proximal tubular functions under the spectrum of Fanconi syndrome. Fanconi syndrome, which is characterized by a defect in proximal tubular reabsorption of glucose, amino acids, uric acid, phosphate, and HCO₃⁻, can occur due to inherited or acquired causes. Primary inherited Fanconi syndrome is caused by a mutation in the sodium-phosphate cotransporter (NaPₐ-II) in the proximal tubule. Recent studies have identified new causes of Fanconi syndrome due to mutations in the EHHADH and the HNF4A genes. Fanconi syndrome can also be one of many manifestations of various inherited systemic diseases, such as cystinosis. Many of the acquired causes of Fanconi syndrome with or without proximal RTA are drug-induced, with the list of causative agents increasing as newer drugs are introduced for clinical use, mainly in the oncology field.
Acidosis ; Acidosis, Renal Tubular ; Amino Acids ; Cystinosis ; Extremities ; Fanconi Syndrome ; Glucose ; Kidney ; Nephrons ; Sodium-Phosphate Cotransporter Proteins ; Uric Acid

BACKGROUND: The trans-tracheal rapid insufflation of oxygen (TRIO) device is less commonly used and is an alternative to trans-tracheal jet ventilation for maintaining oxygenation in a “cannot intubate, cannot oxygenate” (CICO) scenario. CASE: We report the successful use of this device to maintain oxygenation after jet ventilator failure in a parturient who presented with the CICO scenario during the procedure for excision of laryngeal papilloma. CONCLUSIONS: A stepwise approach to the airway plan and preparation for an event of failure is essential for good materno-fetal outcomes. The TRIO device may result in inadequate ventilation that can lead to hypercarbia and respiratory acidosis. Hence, it should only be used as a temporizing measure before a definitive airway can be secured.
Acidosis, Respiratory ; Airway Management ; Airway Obstruction ; Anesthesia, Obstetrical ; High-Frequency Jet Ventilation ; Insufflation ; Oxygen ; Papilloma ; Ventilation ; Ventilators, Mechanical

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder of which m.3243A>G is the most commonly associated mutation, resulting in an inability to meet the energy requirements of various organs. MELAS poses a diagnostic challenge owing to its multiple organ involvement and great clinical variability due to its heteroplasmic nature. We report three cases from a family who were initially misdiagnosed with myasthenia gravis or undiagnosed. Although there is no optimal consensus treatment approach for patients with MELAS because of the disease's heterogeneity, our 21-year-long therapy regimen of l-arginine, l-carnitine, and coenzyme Q10 supplementation combined with dietary management appeared to provide noticeable protection from the symptoms and complications. Prompt early diagnosis is important, as optimal multidisciplinary management and early intervention may improve outcomes.
Acidosis, Lactic ; Arginine ; Carnitine ; Consensus ; DNA, Mitochondrial ; Early Diagnosis ; Early Intervention (Education) ; Follow-Up Studies ; Humans ; MELAS Syndrome ; Mitochondrial Diseases ; Myasthenia Gravis ; Population Characteristics

No abstract available.
Acetazolamide ; Acidosis ; Acidosis, Renal Tubular ; Hypokalemic Periodic Paralysis ; Muscle Weakness