Osmotic gradient ektacytometry: A valuable screening test for hereditary spherocytosis and other red blood cell membrane disorders.

INTRODUCTION: New generation osmotic gradient ektacytometry has become a powerful procedure for measuring red blood cell deformability and therefore for the diagnosis of red blood cell membrane disorders. In this study, we aim to provide further support to the usefulness of osmotic gradient ektacytometry for the differential diagnosis of hereditary spherocytosis by measuring the optimal cutoff values of the parameters provided by this technique. METHODS: A total of 65 cases of hereditary spherocytosis, 7 hereditary elliptocytosis, 3 hereditary xerocytosis, and 171 normal controls were analyzed with osmotic gradient ektacytometry in addition to the routine red blood cell laboratory techniques. The most robust osmoscan parameters for hereditary spherocytosis diagnosis were determined using receiver operating characteristic curve analysis. RESULTS: The best diagnostic criteria for hereditary spherocytosis were the combination of decreased minimal elongation index up to 3% and increased minimal osmolality point up to 5.2% when compared to the mean of controls. Using this established criterion, osmotic gradient ektacytometry reported a sensitivity of 93.85% and a specificity of 98.38% for the diagnosis of hereditary spherocytosis. CONCLUSION: Osmotic gradient ektacytometry is an effective diagnostic test for hereditary spherocytosis and enables its differential diagnosis with other red blood cell membrane diseases based on specific pathology profiles.

Hipertensión intracraneal asociada a trombosis de senos venosos cerebrales y mastoiditis. A propósito de dos casos pediátricos / Intracranial hypertensión associated with cerebral venous sinus thrombosis and mastoiditis. Two paediatric case reports

Introducción. La trombosis de senos venosos cerebrales es una entidad poco frecuente, en general asociada a procesos agudos, entre los que destaca la mastoiditis. En aproximadamente la mitad de los casos, cualquiera que sea su etiología, pueden detectarse trastornos protrombóticos asociados. Habitualmente los pacientes presentan clínica de cefalea, convulsiones y disminución de la conciencia, siendo este último factor de mal pronóstico. Casos clínicos. Presentamos dos pacientes pediátricos con clínica de hipertensión intracraneal en el contexto de mastoiditis como complicación de una otitis media aguda, en los que se identificó una trombosis de senos venosos cerebrales. Ambos se trataron con punciones lumbares evacuadoras y tratamiento diurético. En el segundo paciente se añadió tratamiento anticoagulante. No obstante, ante la persistencia del cuadro de hipertensión intracraneal, este paciente precisó una derivación ventriculoperitoneal. Conclusiones. El método diagnóstico de elección es la resonancia magnética con venografía, si bien, en caso de urgencia, la tomografía computarizada puede orientar el diagnóstico en un tercio de los casos. Además del tratamiento sintomático, se acepta la anticoagulación (grado de evidencia 1B) para evitar la progresión (AU)

Introduction. Cerebral venous sinus thrombosis is a rare condition that is generally associated with acute processes, one of the most important being mastoiditis. Associated prothrombotic disorders can be detected in approximately half of all cases, regardless of the aetiology. Patients usually present clinical symptoms like headache, convulsions and diminished level of awareness, this latter factor being linked to a poor prognosis. Case reports. We report the cases of two paediatric patients with a clinical picture of intracranial hypertension within a context of mastoiditis as a complication of an acute ear infection, in whom cerebral venous sinus thrombosis was identified. Both patients were treated with lumbar punctures to evacuate erebrospinal fluid and diuretic therapy. Anticoagulant therapy was added in the case of the second patient. Nevertheless, because of the persistence of the symptoms of intracranial hypertension, a ventriculoperitoneal shunt had to be performed in this patient. Conclusions. The preferred diagnostic method is magnetic resonance imaging with venography, although in an emergency computerised tomography can help reach a diagnosis in a third of cases. In addition to treating the symptoms, anticoagulation is also accepted (degree of evidence 1B) to prevent further progression (AU)

[Intracranial hypertension associated with cerebral venous sinus thrombosis and mastoiditis. Two paediatric case reports]. / Hipertensión intracraneal asociada a trombosis de senos venosos cerebrales y mastoiditis. A propósito de dos casos pediátricos.

INTRODUCTION: Cerebral venous sinus thrombosis is a rare condition that is generally associated with acute processes, one of the most important being mastoiditis. Associated prothrombotic disorders can be detected in approximately half of all cases, regardless of the aetiology. Patients usually present clinical symptoms like headache, convulsions and diminished level of awareness, this latter factor being linked to a poor prognosis. CASE REPORTS: We report the cases of two paediatric patients with a clinical picture of intracranial hypertension within a context of mastoiditis as a complication of an acute ear infection, in whom cerebral venous sinus thrombosis was identified. Both patients were treated with lumbar punctures to evacuate cerebrospinal fluid and diuretic therapy. Anticoagulant therapy was added in the case of the second patient. Nevertheless, because of the persistence of the symptoms of intracranial hypertension, a ventriculoperitoneal shunt had to be performed in this patient. CONCLUSIONS: The preferred diagnostic method is magnetic resonance imaging with venography, although in an emergency computerised tomography can help reach a diagnosis in a third of cases. In addition to treating the symptoms, anticoagulation is also accepted (degree of evidence 1B) to prevent further progression.