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Influenza and myoglobinuria in brothers.

Two adult brothers became ill within 48 hours of each other, and both had severe myoglobinuria. One brother died of oliguric renal failure. The other did not have renal failure and survived. Acute influenza A infection was documented serologically and from throat washings in the surviving brother, and by isolation of the influenza A virus from throat cultures and lung tissue of the brother who died. It is not certain whether a genetic myopathy made these brothers susceptible to viral-induced myoglobinuria, but a normal response of venous lactate to ischemic work excluded lack of phosphorylase or phosphofructokinase as a cause of the myoglobinuria in the surviving brother. Neither brother had a history of recurrent episodes of myoglobinuria precipitated by exercise, cold, or fasting, thus making carnitine palmityl transferase deficiency unlikely.

Acute Kidney Injury

Myoglobinuria.

Myoglobinuria is still considered to be an uncommon occurrence, however, with the advent of better diagnostic tests it is being increasingly recognized as a potentially life-threatening complication of muscle necrosis. The pathologic consequences of myoglobinuria, including respiratory failure, hyperkalemia, and acute renal failure demand recognition by all who work in areas where this syndrome may develop. This article describes the role of myoglobin in the muscle and how myoglobinuria may develop. It includes the symptoms, differential diagnosis, and treatment of myoglobinuria and possible complications.

Humans

Recurrent myoglobinuria and muscle carnitine palmityltransferase deficiency.

A 16-year-old boy with a two-year history of recurrent attacks of myalgia, muscle cramps without weakness, and myoglobinuria was shown to have a deficiency in muscle carnitine palmityltransferase. Serum concentrations of creatinine phosphokinase, serum glutamic oxalacetic transaminase, and aldolase were elevated. An electromyogram was consistent with a nonspecific myopathy as were microscopic and ultrastructural examinations of biopsied muscle. Venous lactic acid response to ischemic exercise was compatible with paroxysmal idiopathic myoglobinuria. Activities of muscle phosphorylase A and B, phosphofructokinase, muscle palmityl CoA synthetase, carnitine, and serum carnitine were normal as was the glycogen content. Activity of muscle carnitine palmityltransferase (2.7 microM/minute/mg protein), as measured by a spectrophotometric method and by radioactive assay, was significantly reduced when compared to normal control subjects (14.5 microM/minute/mg protein) and ischemic control subjects (13.8 microM/minute/mg protein). Muscle carnitine acetyltransferase (13.4 microM/minute/mg protein) was approximately 50% of normal control values (25.5 microM/minute/mg protein). This is the third reported case of myoglobinuria in a patient associated with a deficiency of muscle carnitine palmityltransferase activity.

Acyltransferases

Myoglobinemia and myoglobinuria in unconscious children.

Myoglobinemia and/or myoglobinuria was demonstrated in 12 of 17 unconscious children studied. Myoglobinemia was noted in patients with serum CPK levels above 200-250 units. Ten of the 12 patients in whom myoglobinemia was present had convulsions. Some degree of renal disturbance was noted in six of seven patients with myoglobinuria. Five patients, including four with renal disturbance, died. In unconscious children with myoglobinuria the possibility of a disturbance of renal function should always be considered.

Blood Urea Nitrogen

[Acute myoglobinuria accompanied by renal failure in high febril infection (author's transl)].

A 41 year old man developed a myolysis with myoglobinuria during a period of high fever. The clinical signs were severe myalgia with general muscular weakness without manifest localized paresis. Electromyographically a diffuse generalized floride myopathy was found. Besides the extreme increase of CPK, a myoglobinuria was already macroscopically visible, which caused an acute renal failure. The treatment included corticosteroids and hemodialysis. An illness of several weaks was followed by complete recovery. As other known factors leading to myoglobinuria have been excluded, and the complement fixation reaction for Herpes simplex was more than 1:80, a viral-toxic etiology of this disease might be considered.

Acute Kidney Injury

Low molecular weight proteinuria in association with paroxysmal myoglobinuria.

A patient with paroxysmal myoglobinuria presented with low molecular weight (LMW) proteinuria in association with an episode of exertional myoglobinuria. Since no signs of acute renal failure were present, the cause was probably competition between myoglobin and other LMW proteins for proximal tubular reabsorption. Agarose gel electrophoresis was found to be an excellent method for the investigation of myoglobinuria since this technique not only allowed the separation of myoglobin from hemoglobin but also myoglobin from metmyoglobin.

Adult

Myoglobinuria, rhabdomyolysis and marathon running.

Rhabdomyolysis, secondary to exertion is known to result in myoglobinuria and is occasionally associated with acute renal failure. In this study myoglobinaemia occurred in 25 of 44 runners completing a 99 km marathon. A marked rise in the values of myoglobin, lactate and the enzymes creatine kinase (CPK), aspartate transaminase (AST) and lactic dehydrogenase (LDH) was noted. A linear correlation was demonstrated between the level of serum myoglobin and the serum concentrations of urate, CPK, AST and LDH. Both the myoglobin itself and the increased concentration of urate may contribute to the acute renal failure. The pathophysiology of rhabdomyolysis during exertion is discussed in the context of other causes of myoglobinuria. A classification of rhabdomyolysis and myoglobinuria is suggested.

Acute Kidney Injury

Influence of pH on the simple solubility test for myoglobinuria.

We have investigated the effect of urine pH on the simple solubility test for myoglobinuria and found that the sensitivity of the test is very pH dependent. As much as a 10 fold increase in sensitivity can be obtained if the test is performed at pH 7.5 instead of pH 5.5. As such, we recommend that the urine pH should first be adjusted within the range 7.5--8.0 before performing the solubility test for myoglobinuria.

Humans

A disorder of muscle lipid metabolism and myoglobinuria. Absence of carnitine palmityl transferase.

Two brothers, 29 and 33 years of age, had recurrent myoglobinuria, renal failure and azotemia, but were otherwise normal, without apparent muscle weakness or exercise intolerance. Ischemic exercise resulted in normal lactate production. Muscle glycogen content and activities of phosphorylase and phosphofructokinase were normal. Plasma triglycerides were elevated (500 mg per deciliter) on a regular diet and rose during fasting. During a 72-hour fast, serum creatine phosphokinase rose more than 10 times, and myoglobin was detected in urine. Plasma ketone production was minimal during fasting, but prompt ketonemia ( a normal response) occurred after ingestion of medium-chain triglycerides. Carnitine palmityl transferase activity was virtually absent in crude muscle extracts and mitochondrial fractions. Lack of this enzyme impairs long-chain fatty acid utilization, reflected in increased content of plasma free fatty acids and plasma triglycerides. Depletion of ATP because of this metabolic block in muscle may account for the attacks of myoglobinuria.

Acute Kidney Injury

Semi-quantitative measurement of myoglobinuria in trauma patients with a latex-agglutination test (Rapi-Tex).

UNLABELLED: A semi-quantitative test (Rapi-Tex) for myoglobinuria was tested in trauma patients. All test results were evaluated blindly. Test specimens were obtained from 20 patients consecutively admitted with severe multiple trauma (Injury Severity Score: 29; range 26-41). The myoglobin concentration was measured semi-quantitatively by Rapi-Tex of three independent observers and quantitatively by enzyme-immuno-assay (EIA). Undiluted as well as 1:2, 1:6, 1:11, and 1:22 diluted samples were used. Test readings were done after 3 min and 5 min, respectively. The Rapi-Tex test classified the myoglobin concentrations correctly in 92.8% of the readings after 3 min and in 87.5% (mean, range: 85.5-91.6%) of the readings after 5 min. CONCLUSION: Rapi-Tex enables immediate bedside diagnosis of myoglobinuria in trauma patients. The test should be performed on both undiluted and 1:6 diluted urine samples with test reading after 5 min.

Adult

Recurrent myoglobinuria due to muscle carnitine palmityl transferase deficiency.

Three new cases of carnitine palmityl transferase deficiency are described. The syndrome consists of recurrent attacks of muscle cramps, weakness, malaise, and myoglobinuria. These attacks are especially likely to occur during prolonged exercise after fasting, eating a high-fat diet, or during cold weather. Occasionally after fasting alone, spontaneous muscle breakdown may occur. One patient studied in detail was excessively slow in producing ketones when he fasted. His mylagias and weakness appeared to be alleviated by beta-hydroxybutyrate. Of eight other patients thought to have idiopathic recurrent myoglobinuria, three were found to have myophosphorylase deficiency, whereas five did not have deficiency of either enzyme. Carnitine palmityl transferase deficiency may be more common than previously supposed, may be in part amenable to dietary therapy, can be easily distinguished from myophosphorylase deficiency, and may provide insight into the metabolism of fatty acids and ketone bodies as well as energy requirements of skeletal muscle.

Acyltransferases

Recurrent acute renal failure with idiopathic paroxysmal myoglobinuria.

A second episode of myoglobinuric renal failure requiring dialysis developed in a muscular 30-year-old man. His first episode, which was not exercise-related, had occurred in childhood and was accompanied by chills, fever, and leukocytosis, whereas the second followed unaccustomed exertion 15 years later. Despite two occurrences of severe renal failure and intermittent episodes of myoglobinuria, renal function is presently normal. Idiopathic paroxysmal myoglobinuria may produce recurrent life-threatening acute renal failure; however, the prognosis for recovery, as evidenced by this case, appears favorable.

Acute Kidney Injury

[Isolated postoperative myoglobinuria as the only sign of malignant hyperthermia. Value of spinal anesthesia with bupivacaine in a posterior surgical intervention].

The Authors report a case of biopsy-proven malignant hyperthermia in a pediatric patient who underwent general anesthesia with halothane and succinylcholine for foot surgery, in whom the presenting symptom was isolated postoperative myoglobinuria. The above syndrome, in the absence of a positive family history, may present itself with a set of minor and atypical symptoms, thus being often underestimate. The need for further investigating all the cases of postoperative myoglobinuria is stressed by the Authors, which also consider muscular biopsy for inclusion among routine investigations. In the case reported here, six months later, a new surgical operation was done safely with spinal anesthesia using hyperbaric bupivacaine.

Anesthesia, Spinal

The effect of infusion of mannitol-sodium bicarbonate on the clinical course of myoglobinuria.

Twenty patients who had evidence of myoglobinuria were treated with intravenous infusions of mannitol and sodium bicarbonate. Nine patients (group 1) responded with higher urine output, and continued infusion improved renal function; none required dialysis and all survived. Eleven patients (group 2) did not respond to the infusion, and required an average of 5.3 (range, 0 to 11) dialyses; one patient died. There was no significant difference in initial BUN level, creatinine level, BUN/creatinine ratio, or fractional sodium excretion level between the two groups. However, group 2 patients had a significantly higher creatine phosphokinase (CPK) level, serum phosphate level, and hematocrit reading initially than did group 1, indicative of more severe muscle injury and hemoconcentration. These results demonstrate that some patients with myoglobinuria will respond to infusion of mannitol and sodium bicarbonate. This treatment may be effective in altering the clinical course of myoglobinuric acute renal failure.

Acute Kidney Injury

Myoglobinuria.

Myoglobinuria has a variety of causes, ranging from trauma to heavy exercise. Symptoms usually consist of myalgia and muscle weakness; dark, bloody-looking urine may be noted. Myoglobin can be identified by readily available laboratory techniques. Acute renal failure can be a dangerous consequence of myoglobinuria but, with rapid recognition and strict monitoring, the prognosis in most cases is good.

Crush Syndrome

Myoglobinuria associated with herpes-group viral infections.

Two cases of myoglobinuria were associated with herpes-group viral infection (herpes simplex and Epstein-Barr). Muscle atrophy and acute renal failure were important complications. No evidence of direct invasion of muscle fibers by virus was found, nor was the severity of myoglobinuria correlated with histologic appearance of muscle.

Acute Kidney Injury

[Extra-cardial rise of the CPK in the screening of urgent diagnostic (among others, a case of anaesthesia induced myoglobinuria and a case of malignant hypertermia) (author's transl)].

Tests are being carried out on 37 patients and healthy volunteers, to differentiate the various, from heart independent, creatin-phosphokinase (CPK) increases, in order to recognize anaesthesia-endangered patients. A rise of the serum-CPK-level, dependent on trauma is insignificant for the anaesthesia. A significant rise of the CPK during a gener al anaesthesia and particularly extreme high values after 24 hours, (example, more than 10 000 U/l) is reason to think of a course of a malignant hyperthermia or myoglobinuria in a subclinical form. Relatives of hyperthermia- or myoglobinuria suspected families are in all cases anaesthesia-endangered, even with a very small rise of CPK-values. The knowledge of these connections is highly important also for internal medical spheres.

Acute Disease

Late components of motor unit potentials in a patient with myoglobinuria.

A 45-year-old woman had severe weakness, myoglobinuria, and elevated muscle enzyme levels in the serum after a flulike illness. Electromyography some four weeks later showed motor unit potentials with late components; these remained present on subsequent electromyographic examinations performed at approximately monthly intervals for the following year. The persistence of motor unit potentials with late components is taken to imply that the late components were caused either by sprouts that failed to undergo myelination or, more likely, by ectopically innervated muscle fibers.

Electromyography