Recurrent acute kidney failure due to mcardle disease

We present an adolescent with McArdle disease and recurrent acute kidney failure due to rhabdomyolysis. The patient was admitted with acute kidney failure for 3 times and due to a history of proximal weakness, fatigue, and muscular cramps after physical activities a glycogen-storage disease was suspected. Serum creatine phosphokinase and urine myoglobin were found to be elevated. McArdle disease was diagnosed based on pathologic examination of muscle tissue specimen. Patients presenting with rhabdomyolysis following strenuous exercise should be evaluated for McArdle disease

role of pulmonary surfactants [protein A and phospholipids], catecholamines and myoglobin in the diagnosis of the cause of death

An important and often one of the most difficult tasks of a medical practitioner or pathologist is the investigation of the cause of death. The aim of this work is to assess the post mortem levels of serum protein A [SP-A], pulmonary as well as serum phospholipids, catecholamines and myoglobin in forensic autopsies as a guide to diagnose the cause of death. This study was done on 120 cadavers 75 males and 45 females with ranging age from newborn up to 62 years old. They included 2 groups: the first group consisted of 110 cases died from various types of trauma or due to some diseases [experimental group] and the second group consists of 10 cases died without trauma or disease [control group]. The experimental group subdivided into eleven subgroups of various types of trauma or disease. Each was 10 cases for each of the followings:- strangulation, hanging, drowning, blunt head injuries, burns, sharp weapon injuries, heat stroke, electrical fatalities, hemorrhagic shock, myocardial infarction and interstitial pneumonia. It was found that the level of serum protein A [SP-A] showed a very highly significant increase in cases of interstitial pneumonia and highly significant increase in cases of drowning burns and sharp weapon injuries. Also it showed a significant increase in cases of strangulation hanging, blunt head injuries and electrical injuries, while insignificant increase in, heat stroke hemorrhagic shock and myocardial infarction as compared to the control had been shown. Serum phospholipids showed a very highly significant increase in strangulation, hanging, drowning, electrical injuries and interstitial pneumonia and highly significant increase in blunt head injuries, burn, sharp weapon injuries and heat stroke. Also it showed a significant increase in hemorrhagic shock and myocardial infarction as compared to the control. Lung surfactant phospholipids showed insignificant, increase in different cases of death as compared to the control. Adrenaline and noradrenoline showed highly significant increase in case of strangulation, hanging, drowning, myocardial infarction and interstitial pneumonia respectively. Adrencline showed a significant increase in blunt head injuries, burns, sharp weapon injuries, heat stroke and hemorrhagic shock as compared to the control. Noradrenaline showed insignificant increase in heat stroke as compared to the control. Myoglobin showed a significant increase in cases of blunt head injuries, burns, sharp weapon injuries, heat stroke and electrical injuries. While insignificant increase in cases of strangulation, hanging, drowning, hemorrhagic shock, myocardial infarction and interstitial pneumonia, as compared to the control

Poder pronóstico precoz de la mioglobina en los síndromes coronarios agudos sin elevación del segmento ST / Prognostic value of myoglobin in non-ST elevation acute coronary syndromes

Antecedentes: La mioglobina es un marcador precoz de necrosis miocárdica. Sin embargo, su valor pronóstico aún no se ha aclarado en forma prospectiva en las primeras horas de evolución de pacientes con síndromes coronarios agudos (SCA) sin supradesnivel del segmento ST. Objetivos: Determinar la relación entre los valores de mioglobina al ingreso y la ocurrencia de muerte o infarto a los 30 días en pacientes con SCA sin supradesnivel del ST. Material y métodos: Se realizó un estudio de cohortes prospectivo y multicéntrico que incluyó 366 pacientes para evaluar el valor pronóstico de la mioglobina en el momento de la admisión. Se determinó también la concentración de troponina T, la proteína C reactiva (PCR) y la CK-MB masa a las 12 horas del comienzo de los síntomas. Las determinaciones fueron realizadas a doble ciego por un laboratorio central e independiente. El punto final primario fue muerte y/o reinfarto no fatal a los 30 días. Se realizó un análisis multivariado para determinar la asociación entre los niveles de mioglobina y el punto final clínico. Resultados: La mediana de tiempo síntomas-admisión fue de 4 horas. La tasa de eventos a los 30 días fue del 10,1 por ciento. De acuerdo con la regresión logística, se observó una asociación entre los niveles elevados de mioglobina al ingreso y los eventos clínicos (OR 3,5 [IC 95 por ciento 1,6-7,6]), independiente de marcadores clínicos y bioquímicos convencionales. Conclusiones: La elevación precoz de la mioglobina agrega información pronóstica independiente a marcadores clínicos y bioquímicos convencionales y además se asocia con mala evolución clínica a los 30 días en pacientes con SCA sin supradesnivel del ST.

Serum cardiac troponin-I in active rheumatic carditis.

OBJECTIVE: The study was performed to investigate the level of serum cardiac troponin I (cTnI), a specific marker for myocardial cell damage, in the acute rheumatic carditis (RC). METHODS: Twenty seven consecutive patients with acute RC and 23 healthy children were enrolled. RESULT: cTnI level in both groups showed no statistical difference (p > 0.05). CONCLUSION: Serum cTnI level did not gain clinical use.

Valor diagnóstico da CK-MB assa, à mioglobina e à troponina I nas síndromes isquêmicas agudas / Diagnostic Value of CK-MB Activity Compared to Mecglobin, CK-MB Mass, Traponin 1 in Acute Coronary Syndrome

A síndrome isquêmica aguda varia de angina instável a infarto agudo do miocárdio, e seu manuseio atual requer rapidez e eficácia. O diagnóstico do infarto agudo do miocárdio foi estabelecido pela Organização Mundial da Saúde, e a CK-MB atividade foi considerada a enzima padrão. O objetivo deste estudo foi comparar o valor diagnóstico e o desempenho da CK-MB atividade à CK-MB massa, à troponina I e à mioglobina em portadores de dor precordial prolongada, atendidos no pronto-socorro. A casuística incluiu 217 pacientes, com dor precordial de duração igual ou superior a 20 minutos, distribuídos em três grupos: grupo I, 29 pacientes com infarto agudo do miocárdio, diagnosticado por supradesnivelamento do segmento ST e dor; grupo II, 24 pacientes com infarto agudo do miocárdio, diagnosticado por elevação da CK-MB atividade e dor, e grupo III, 164 pacientes, com dor, sem alteração da CK-MB atividade e do eletrocardiograma compatível com infarto agudo. As dosagens dos marcadores bioquímicos foram realizadas na ocasião da inclusão do paciente no estudo, duas, quatro, seis, oito, dez, 12 e 24 horas após, e as análises foram realizadas em relação à hora do início da dor. As características demográficas dos grupos foram semelhantes, exceto o tabagismo, que foi menor no grupo III. Quanto às alterações eletrocardiográficas, todos os pacientes com eletrocardiograma normal situaram-se no grupo III, e os níveis dos marcadores bioquímicos foram mais elevados nos pacientes com alterações eletrocardiográficas compatíveis com isquemia aguda. A sensibilidade e a especificidade da CK-MB massa (5,6 ng/ml), da cTnI (1,5 ng/ml), da CK-MB massa (9,4 ng/ml) e da mioglobina (110 ng/ml), em relação à CK-MB atividade, foram 98% e 78,6%, 98% e 74,4%, 98% e 84,5%, 85,7% e 85,1% respectivamente. O valor preditivo positivo variou de 52,7% a 64,8% e o valor preditivo negativo variou de 95,3% a 99,3%. A CK-MB atividade não se elevou em níveis compatíveis com IAM em 13,8% dos pacientes do grupo I, tendo ocorrido um resultado falso positivo no grupo II...

Hypertonic-hyperosmotic solution modifies myoglobin levels in early reperfusion after ischemic cardiac arrest. Experimental model

To evaluate the effect of reperfusison with hypertonic-hyperosmotic solution, cardiectomy was performed in 25 New Zealand white rabbits. Seven isolated hearts were submitted to 30 min of global ischemia and reperfused with oxygenated uffer for 60 min. Myoglobin and isoensyme MB of creatine kinase concnetrations were each measured in the effluent 15 min, and values were correlated (r=0.5011, p=0.015). After this procedure, 18 isolated hearts were radomized in two groups. Hearts of group I were reperfused with hypertonic-hyperosmotic solution (NaCl 7.5 percent dextran 60,000 MW) diluted in oxygenated buffer, and group II with oxygenated buffer. Myoglobin and coronary flow were measured in both groups, group I showed lower levels of myoglobin (p=0.0069) and higher coronary flow (p = 0.028) than group II. In conclusion, changes in myoglobin concentration in the heart effluent are more sensitive than changes in isoenxyme MB of creatine kinase; thus, evaluation of this parameter may be useful in the detection of ishcemia reperfusion injury. Additionally, hypertonic-hyperosmotic solution improves the coronary flow and has a protective effect against ichemia-reperfusion injury

Determinación de la mioglobina por radioinmunoenzayo en pacientes con coronariografía / Myoblobin determination by radioimmunoassay in patients with coronarography

Se estudiaron 15 pacientes adultos en forma consecutiva, ingresados en el Instituto de Cardiológia Cardiovascular con el diagnóstico de cardiopatía isquémica , entre los meses de septiembre y noviembre de 1988 para ventricolografía y coronorariografía selectiva bilateral, con el objetivo de determinar si se presentaban variaciones en los niveles de la mioglobina en los pacientes a los cuales se les realizó coronariografía. A todos se les cuantificó la mioglobina por radioinmunoensayo 1 hora antes, al finalizar y 4 horas después de haber concluido la coronariografía y el valor promedio para cada tiempo fue de 34, 40 y 42ng/ml, respectivamente. En nuestra investigación los valores obtenidos de la mioglobina oscilaron dentro del rango normal(8 a 80 ng x mL). No se presentaron complicaciones en el estudio. No se encontró diferencias estadísticamente significativas entre los diferentes horarios(p> 0,05).