Debilidad e hipertrofia muscular como forma de presentación de un hipotiroidismo: síndrome de Hoffman / Muscle weakness and hypertrophy as a presentation form of hypothyroidism: Hoffman¿s syndrome

Las alteraciones musculares aparecen con relativa frecuencia en los pacientes con hipotiroidismo. El síndrome de Hoffman constituye una forma especial y poco común de miopatía hipotiroidea. Presentamos el caso de una varón con debilidad progresiva, hipertrofia muscular y elevación de los niveles de creatinfosfokinasa debido a un hipotiroidismo primario (AU)

Muscle involvement is common in patients with hypothyroidism.Hoffman’s syndrome is a rare form of hypothyroidmyopathy. We report the case of a man with progressive weakness,muscle hypertrophy and serum creatine kinase elevationdue to a primary hypothyroidism (AU)

[Visceral leishmaniasis and bronchial asthma: influence of steroid therapy in the development of the macrophage activation syndrome and relative adrenal insufficiency]. / Leishmaniasis visceral y asma bronquial: influencia del tratamiento esteroideo en el desarrollo del síndrome de activación macrofágica e insuficiencia suprarrenal relativa.

The risk of suffering opportunistics infections in the immunoincompetent patient is a fact perfectly established. An uncommon situation constitutes the bronchopaties, pathologies with a high prevalence among the general population that they require habitually, among other, steroid treatment. The immunosupression confers to the clinical evolution of the infections, as a consequence of the inadequate response to the physical stress, due to the inhibition of the hypothalamus-hypophysis axis being able to in particularly serious cases, to develop the denominated macrophage activation syndrome, a serious and uncommon syndrome that darkens the clinical prognosis in these patients. In presence of a feverish syndrome of uncertain origin in a patient in immunosuppressor treatment, although it is to low dose, it is necessary to carry out a exhaustive differential diagnosis, should consider, among them, the infection for Leishmania, a parasitosis whose incidence is increasing notably in the last years in the immunosuppressed population. We present the clinical case of a 63 year-old patient, immunoincompetent as a consequence of secondary chronic steroid therapy to asthmatic bronchopaty that experiences an uncommon form of visceral leishmaniasis in our area, consistent in multiorganic failure in the context of the development of a macrophage activation syndrome.

[Symptomatic secondary hyponatraemia due to combined treatment anticonvulsant and antidepressant: risk of sudden death in epilepsy?]. / Hiponatremia sintomática secundaria a tratamiento conjunto anticomicial y antidepresivo: ¿riesgo de muerte súbita en epilepsia?

The notable incidence of epilepsy in the general population, with the presence, more and more frequent of refractory therapeutic, it forces the anticonvulsant polytherapy. If we depart from the fact, that many of the epilepsies have a secondary cause, with affective disorders associate, we can understand the most habitual association of anticonvulsant and antidepressant. We present the clinical case of a 37-year-old-woman with refractory therapeutic epilepsy associated to exogenous depressive syndrome, in combined treatment with Carbamazepine, Lamotrigine and Venlafaxine, that suffers a episode of syncope secondary to symptomatic hyponatraemia, generated by syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The relevance of this case resides in the fact that the hyponatraemia generated by this pharmacological combination, up to now, it has not been described in the literature. This electrolytic anomaly can derive in secondary neurological and cardiovascular effects, in so outstanding occasions, as to produce the phenomenon denominated sudden death in the epileptic patient (SUDEP). We recommended a strict ionic control in those patients that require anti-epileptic and anti-depressant treatment combined, in order to avoiding paroxistic vascular episodes and to minimize the risk of SUDEP.

[Macro creatine kinase: illness marker. Practical guide for the management]. / Macro-creatincinasa: marcador de enfermedad. Guía práctica para su manejo.

BACKGROUND AND OBJECTIVE: The Macro creatine kinase (Macro-CK) is a complex constituted by polymerization of isoenzymes of creatine kinase (CK-BB or CK-MM together with IgG in the type I and oligomers of CK mitochondrial in the type II). Their presence in plasma generates false elevations of the CK-MB isoenzyme, upon interfering with the techniques of imunoinhibition used in the emergency room laboratories, what it constitute a serious problem in the diagnosis of squares of myocardial ischemia. The ignorance of this clinical situation has pushed us to present this study in order to begin to consider their utility like marker of illness, giving shortly, some recommendations for the correct management of this discovery in the emergency. SUBJECTS AND METHOD: They have been studied the total of patients valued in the emergency for 16 months that they gathered this requirements (25), valuing the pathology that appeared under this analytic determination. The identification of the isoenzymes of CK was carried out by means of agarose gel electrophoresis. RESULTS: The 13 cases with Macro-CK type I (9 women and 4 males) had a half age of 64 years (4-89). The levels means of CK were 274 mU/mL with a CK-MB of 440 U/L (166%). The 7 cases with Macro-CK type II (1 woman and 6 males) had a half age of 69 years (32-80). The levels means of CK were 314 mU/mL with a CK-MB of 569 U/L (191%). The 53.8% of the patients with Macro-CK type I presented any cardiovascular pathology, the 38.5% diabetes mellitus and the 30.8% pathology muscle-articulate with possible component autoimmune. The 100% of the patients with Macro-CK type II suffered processes malignant tumors, highlighting the presence of tumors of digestive origin and prostatic, with metastasis, being the more frequent in liver and bone (71.4%), and a high mortality of the 71.4% (5 deaths). The Macro-CK type II (form oligomer of mitochondrial) is liberated like consequence of a lesion of the mitochondrias of the affected fabrics tissues, associating to serious illnesses like cirrhosis and tumors. CONCLUSIONS: In all the studied cases the payees of Macro-CK presented underlying pathology, being in the case of Macro-CK type I of heart prevalence, and in the case of type II of nature tumoral. A management Macro-CK algorithm is also presented for the emergency.

Macro-creatincinasa: marcador de enfermedad. Guía práctica para su manejo / Macro creatine kinase: illness marker. Practical guide for the management

Fundamento y objetivo: La Macrocreatincinasa (Macro-CK) es un complejo constituido por polimerización de isoenzimas de creatincinasa (CK-BB o CK-MM unidas a IgG en la tipo I y oligómeros de CK mitocondrial en la tipo II). Su presencia en plasma genera falsas elevaciones de la isoenzima CK-MB, al interferir con las técnicas de inmunoinhibición empleadas en los laboratorios de urgencias, lo que constituye un grave problema en el diagnóstico de cuadros de cardiopatía isquémica. El desconocimiento de esta situación clínica nos ha empujado a presentar este estudio para empezar a considerar su utilidad como marcador de enfermedad, dando brevemente, unas recomendaciones para el correcto manejo de este hallazgo en el Servicio de Urgencias. Pacientes y Método: Se han estudiado el total de pacientes valorados en el Servicio de Urgencias durante 16 meses que reunían dichos requisitos (25), valorando la patología que subyacía bajo esta determinación analítica. La identificación de las isoenzimas de CK fueron realizadas mediante electroforesis en gel de agarosa. Resultados: Los 13 casos con Macro-CK tipo I (9 mujeres y 4 varones) tenían una edad media de 64 años (4-89). Los niveles medios de CK fueron 274 mU/mL con una CK-MB de 440 U/L (166%). Los 7 casos con Macro-CK tipo II (1 mujer y 6 varones) tenían una edad media de 69 años (32-80). Los niveles medios de CK fueron 314 mU/mL con una CK-MB de 569 U/L (191%). El 53,8% de los pacientes con Macro-CK tipo I presentaban alguna patología cardiovascular, el 38,5% diabetes mellitus y el 30,8% patología músculo-articular con posible componente autoinmune. El 100% de los pacientes con Macro-CK tipo II padecían procesos neoplásicos, destacando la presencia de tumores de origen digestivo y prostático, con afectación metastásica, siendo las más frecuentes en hígado y hueso (71,4%), y una alta mortalidad del 71,4% (5 exitus). La Macro-CK tipo II (forma oligomérica mitocondrial) es liberada como consecuencia de una lesión de las mitocondrias de los tejidos afectados, asociándose a enfermedades graves como cirrosis y tumores. Conclusiones: En todos los casos estudiados los portadores de Macro-CK presentaron patología subyacente, siendo en el caso de Macro-CK tipo I de predominio cardíaco, y en el caso de tipo II de naturaleza neoplásica. También, presentamos un algoritmo de manejo de la Macro-CK para el servicio de urgencias

Background and objective: The Macro creatine kinase (Macro-CK) is a complex constituted by polymerization of isoenzymes of creatine kinase (CK-BB or CK-MM together with IgG in the type I and oligomers of CK mitochondrial in the type II). Their presence in plasma generates false elevations of the CK-MB isoenzyme, upon interfering with the techniques of imunoinhibition used in the emergency room laboratories, what it constitute a serious problem in the diagnosis of squares of myocardial ischemia. The ignorance of this clinical situation has pushed us to present this study in order to begin to consider their utility like marker of illness, giving shortly, some recommendations for the correct management of this discovery in the emergency. Subjects and method: They have been studied the total of patients valued in the emergency for 16 months that they gathered this requirements (25), valuing the pathology that appeared under this analytic determination. The identification of the isoenzymes of CK was carried out by means of agarose gel electrophoresis. Results: The 13 cases with Macro-CK type I (9 women and 4 males) had a half age of 64 years (4-89). The levels means of CK were 274 mU/mL with a CK-MB of 440 U/L (166%). The 7 cases with Macro-CK type II (1 woman and 6 males) had a half age of 69 years (32-80). The levels means of CK were 314 mU/mL with a CK-MB of 569 U/L (191%). The 53.8% of the patients with Macro-CK type I presented any cardiovascular pathology, the 38.5% diabetes mellitus and the 30.8% pathology muscle-articulate with possible component autoimmune. The 100% of the patients with Macro-CK type II suffered processes malignant tumors, highlighting the presence of tumors of digestive origin and prostatic, with metastasis, being the more frequent in liver and bone (71.4%), and a high mortality of the 71.4% (5 deaths). The Macro-CK type II (form oligomer of mitochondrial) is liberated like consequence of a lesion of the mitochondrias of the affected fabrics tissues, associating to serious illnesses like cirrhosis and tumors. Conclusions: In all the studied cases the payees of Macro-CK presented underlying pathology, being in the case of Macro-CK type I of heart prevalence, and in the case of type II of nature tumoral. A management Macro-CK algorithm is also presented for the emergency

[Hospital registry of pancreatic tumors. Experience of the Hospital Clínic in Barcelona (Spain)]. / Registro hospitalario de tumoraciones pancreáticas. Experiencia del Hospital Clínic de Barcelona.

OBJECTIVE: To describe the characteristics of patients included in the pancreatic tumor registry of the Hospital Clínic of Barcelona. PATIENTS AND METHOD: All patients with pancreatic tumors attended between July 1990 and March 2003 were registered. Data collection included: age, gender, date of diagnosis, diagnosis, histology, size, location and tumor stage, and treatment. The correlation between tumor stage and age, date of diagnosis, and tumor location was also evaluated. RESULTS: Six hundred thirty patients with pancreatic tumors were included, representing an incidence of 60 patients/year. The mean age was 66 years and the male-to-female ratio was 1,18:1. The most frequent lesion was malignant tumor of the pancreas (92%), and the most frequent histological type was pancreatic ductal adenocarcinoma (73%). The most frequent location was the head of the pancreas (64%). In 28% of the patients, pancreatic cancer was diagnosed in stage I and II. Resection was performed in 31% of patients, whereas 48% of the patients received no treatment. The ratio between local (stage I)/disseminated (stage IV) disease was 0,34. The ratio between stage I/IV increased with age, diagnosis prior to 1994, and tumor location in the head of the pancreas. CONCLUSION: Hospital tumor registries can be used to define the profile of the attended population, which can help to delineate the best diagnostic-therapeutic strategy and can be useful in clinical research.