Preload dependence of gated cardiac SPECT-derived ventricular volumes in hemodialysis patients.

BACKGROUND: In hemodialysis (HD) patients, the intravascular volume expansion of the pre-HD state leads to a high preload. We aim to examine its effect on myocardial perfusion gated SPECT (MPGS)-derived left ventricular (LV) volumes. METHODS: The study comprised 50 end-stage renal disease (ESRD) patients on HD with normal 2-day stress/rest MPGS performed for kidney transplantation risk assessment. Patients (pts) comprised 23 men/27 women, with mean age of 59.4 ± 7.1 years. The time elapsed from the last HD session in hours was calculated on both days, and patients were classified according to whether it was higher (group A: 19 pts), lower (group B: 27 pts), or equal (group C: 4 pts) on the stress vs the rest day. End-diastolic, end-systolic volumes (EDV, ESV), and left ventricular ejection fraction (LVEF) were determined using QGS™ software. Transient ischemic dilation (TID) ratios were derived from the nongated images using QPS™ software. RESULTS: Volumes were significantly higher at stress in group A, at rest in group B, and similar in group C. TID ratios were significantly higher in group A vs groups B and C. CONCLUSIONS: MPGS-derived ventricular volumes are preload dependent. The high preload of the pre-HD state may mimic ischemic TID if occurring on the stress day and create confusion if coinciding with the rest day.

[Cystinosis: an infrequent cause of distal myopathy]. / Cistinosis: una causa infrecuente de miopatía distal.

INTRODUCTION: Cystinosis is a hereditary disease with clinical symptoms that are caused by the accumulation of cystine crystals in different tissues. Distal vacuolar myopathy has been reported as one of its later complications. CASE REPORT: Here, we present the case of a 20-year-old male diagnosed with cystinosis at the age of 2 years, with severe renal involvement that required a transplant. The patient gradually developed weakness and atrophy of the muscles in his hands. Neurophysiological and histological studies enabled a diagnosis of distal vacuolar myopathy to be established, and electron microscopy revealed deposits of cystine crystals. CONCLUSIONS: Cystinosis must be included within the differential diagnosis of distal myopathies. Timely treatment with cysteamine could prevent the development of this complication.

Cistinosis: una causa infrecuente de miopatía distal / Cystinosis: an infrequent cause of distal myopathy

Introducción. La cistinosis es una enfermedad hereditaria cuyas manifestaciones clínicas se producen por la acumulación de cristales de cistina en diversos tejidos. Entre sus complicaciones tardías se ha descrito una miopatía distal vacuolar. Caso clínico. Se trata de un varón de 20 años, diagnosticado de cistinosis a los 2 años, con grave afectación renal que requirió trasplante. De forma insidiosa desarrolló debilidad y atrofia de los músculos de las manos. Mediante estudios neurofisiológicos e histológicos se estableció el diagnóstico de miopatía distal vacuolar, y se objetivaron depósitos de cristales de cistina en la microscopía electrónica. Conclusiones. La cistinosis debe incluirse en el diagnóstico diferencial de las miopatías distales. Un tratamiento precoz con cisteamina podría evitar el desarrollo de esta complicación

Introduction. Cystinosis is a hereditary disease with clinical symptoms that are caused by the accumulation of cystine crystals in different tissues. Distal vacuolar myopathy has been reported as one of its later complications. Case report. Here, we present the case of a 20-year-old male diagnosed with cystinosis at the age of 2 years, with severe renal involvement that required a transplant. The patient gradually developed weakness and atrophy of the muscles in his hands. Neurophysiological and histological studies enabled a diagnosis of distal vacuolar myopathy to be established, and electron microscopy revealed deposits of cystine crystals. Conclusions. Cystinosis must be included within the differential diagnosis of distal myopathies. Timely treatment with cysteamine could prevent the development of this complication

[Cervical foreign body. An unusual case]. / Cuerpo extraño cervical. Un caso insólito.

Treatment for penetrating neck injuries remains highly controversial. Surgical intervention is mandatory in case of a patent wound or if unable to stabilize the patient. Otherwise, a selective approach is not an accepted option, although this criterion is becoming more popular due to its low morbidity and the likelihood to cure some unidentified injuries. We present a patient with a neck trauma caused by a metallic rod in a labour accident without any symptoms. The rod penetrated into his jugular vein and reached the heart area where it has remained imbedded for 5 years.

Cuerpo extraño cervical. Un caso insólito / Cervical foreign body. An unusual case

El tratamiento de heridas cervicales penetrantes está todavía sometido a controversia. Si la lesión es evidente o es imposible estabilizar al enfermo, la indicación quirúrgica es clara. En el resto de los casos la exploración selectiva es una actitud no compartida por todos los autores, aunque va ganando adeptos por la baja morbilidad y la posibilidad de subsanar lesiones no identificadas. Presentamos un paciente al que se le introduce en el cuello una viruta metálica por un accidente laboral y que, sin manifestar sintomatología de lesión vascular, evoluciona a penetrar en la vena yugular y llegar a la región cardíaca donde permanece enclavado desde hace cinco años (AU)

Treatment for penetrating neck injuries remains highly controversial. Surgical intervention is mandatory in case of a patent wound or if unable to stabilize the patient. Otherwise, a selective approach is not an accepted option, although this criterion is becoming more popular due to its low morbidity and the likelihood to cure some unidentified injuries. We present a patient with a neck trauma caused by a metallic rod in a labour accident without any symptoms. The rod penetrated into his jugular vein and reached the heart area where it has remained imbedded for 5 years (AU)

Insuficiencia cardiaca con patrón de pseudoinfarto en el electrocardiograma como forma de presentación de amiloidosis / Heart failure with electrocardiographic pseudo-infarction pattern as presentation form of amyloidosis

La afectación cardiaca por amiloidosis es una entidad infrecuente que implica un sombrío pronóstico, con una mediana de supervivencia de menos de un año. Aunque su diagnóstico es difícil al poder simular otras formas de cardiopatía, su existencia puede ser sospechada en pacientes que se presenten con la conjunción de: 1) insuficiencia cardiaca con datos ecocardiográficos de disfunción diastólica e hipertrofia ventricular; y 2) patrón de pseudoinfarto en el ECG (alteraciones electrocardiográficas que simulan un infarto de miocardio en ausencia de este antecedente). Presentamos el caso de un paciente con amiloidosis cuya enfermedad comenzó de este modo, y posteriormente revisamos la literatura al respecto (AU)

[Reversible posterior leukoencephalopathy, severe hypertension, and cocaine abuse]. / Leucoencefalopatía posterior reversible, hipertensión severa y abuso de cocaína.

Reversible posterior leukoencephalopathy syndrome is a brain disorder characterized by headache, nausea, vomiting, visual disturbance, depressed level of consciousness, convulsions and occasionally focal neurologic deficits. It is commonly associated with malignant hypertension, toxemia of pregnancy or the use of immunosuppressive agents. Early diagnosis and specific treatment is essential. We report a case of reversible posterior leukoencephalopathy in the context of a hypertensive crisis in an habitual cocaine sniffer. Reversible posterior leukoencephalopathy must be suspected in every patient with hypertensive crisis and compatible clinic manifestation. Neuroimaging studies show characteristic features which confirm the diagnosis.

Leucoencefalopatía posterior reversible, hipertensión severa y abuso de cocaína / Reversible posterior leukoencephalopathy, severe hypertension and cocaine abuse

La leucoencefalopatía posterior reversible es una alteración del sistema nervioso central que cursa clínicamente con cefalea, náuseas y vómitos, alteraciones visuales, disminución del nivel de conciencia, crisis convulsivas y ocasionalmente signos de focalidad neurológica. Suele asociarse con hipertensión arterial maligna, toxemia del embarazo o el uso de inmunosupresores. Son fundamentales un diagnóstico precoz y tratamiento adecuado. Se presenta un caso de leucoencefalopatía posterior reversible en el contexto de una crisis hipertensiva en un paciente consumidor habitual de cocaína por vía intranasal. La leucoencefalopatía posterior reversible cursa con manifestaciones clínicas sugestivas y debe ser sospechada en todo paciente con crisis hipertensiva y clínica compatible. Los estudios de neuroimagen caracterizan y apoyan el diagnóstico. (AU)