[Benign idiopathic external hydrocephalus (benign subdural collection) in 39 children: its natural history and relation to familial macrocephaly]. / Hidrocefalia externa idiopática benigna (efusión subdural benigna) en 39 niños: evolución natural y relación con la macrocefalia familiar.

INTRODUCTION: Benign idiopathic external hydrocephalus (BIEH) is an age-dependent disorder that is self-limiting in time and has an uncertain aetiology. PATIENTS AND METHODS: A retrospective study was conducted involving 39 patients (16 girls and 23 boys) with BIEH. The following data were analysed for each patient: age, sex, family history, history concerning pregnancy, childbirth and neonatal period, postnatal history, data from clinical records and from physical examinations, progress of psychomotor development, findings from the first and successive neuroimaging studies, results of other complementary examinations, clinical and neuroimaging situation at the last check-up that was carried out, length of clinical control, age at which subdural effusion disappeared, and any other relevant associated facts. RESULTS: Age at diagnosis ranged from 1.33 and 25 months (mean: 8.4 months); in 38.46% of cases there was a history of macrocephalia in one of the progenitors; in four of them the presence of congenital macrocephalia was noted; in five, there was motor retardation and one of them displayed psychomotor retardation; in 15, there was an association with a slight dilatation of the lateral ventricles; the mean time of clinical control was 3.36 years; the process was seen to resolve in 14 cases; the minimum age for the disappearance of the subdural effusion was 9 months and the maximum was 8 years; macrocephalia persisted until the clinical control ended in 22 of the cases. We also noted the presence of two cases of mitochondrial encephalomyopathy, one craniosynostosis of the sagittal suture, one microdeletion 22q11.2, one a-1 antitrypsin deficiency, and one case of idiopathic bilateral congenital palpebral ptosis. CONCLUSIONS: The subdural effusion and/or macrocephalia persist in a high percentage of these patients and sometimes there is a close relationship between this condition and benign familial macrocephalia. Despite its benignity, it can influence psychomotor or motor retardation and behavioural disorders. On rare occasions it may be associated to mitochondrial encephalomyopathy and to the microdeletion 22q11.2.

Hidrocefalia externa idiopática benigna (efusión subdural benigna) en 39 niños: evolución natural y relación con la macrocefalia familiar / Benign idiopathic external hydrocephalus (benign subdural collection) in 39 children: natural history and relationship to familial macrocephaly

Introducción. La hidrocefalia externa idiopática benigna (HEIB) es un trastorno dependiente de la edad, autolimitado en el tiempo y de etiología incierta. Pacientes y métodos. Se llevó a cabo un estudio retrospectivo de 39 pacientes, 16 niñas y 23 niños, con HEIB, en los que se analizaron los siguientes datos: edad, sexo, antecedentes familiares, antecedentes del embarazo, el parto y el período neonatal, antecedentes posneonatales, datos de la historia clínica y de la exploración física, evolución del desarrollo psicomotor, hallazgos de la primera y sucesivas pruebas de neuroimagen, resultados de otras exploraciones complementarias, situación clínica y de neuroimagen en el último control realizado, tiempo de control clínico, edad a la que desapareció la efusión subdural, y otros hechos relevantes asociados. Resultados. La edad en el momento del diagnóstico osciló entre 1,33 y 25 meses (media: 8,4 meses); en el 38,46% existía el precedente de macrocefalia en alguno de los progenitores; en cuatro se observó la presencia de macrocefalia congénita; en cinco se detectó la presencia de retraso motor, y en uno, de retraso psicomotor; en 15 se asociaba una ligera dilatación de los ventrículos laterales; el tiempo medio de control clínico fue de 3,36 años; se observó la resolución del proceso en 14; la edad mínima para la desaparición de la efusión subdural fue de 9 meses, y la máxima, de 8 años; la macrocefalia persistió al final del control clínico en 22 niños; resalta la presencia de una encefalomiopatía mitocondrial en dos, de craneosinostosis de la sutura sagital en uno, de microdeleción 22q11.2 en otro, de deficiencia de -1 antitripsina en otro, y ptosis palpebral congénita bilateral idiopática en otro. Conclusiones. En un alto porcentaje de los pacientes persiste la efusión subdural y/o la macrocefalia; en ocasiones existe una estrecha relación entre esta entidad y la macrocefalia familiar benigna; a pesar de su benignidad, puede condicionar retraso psicomotor o motor y trastornos conductuales; de forma excepcional, se puede asociar a una encefalomiopatía mitocondrial y a la microdeleción 22q11.2

Introduction. Benign idiopathic external hydrocephalus (BIEH) is an age-dependent disorder that is self-limiting in time and has an uncertain aetiology. Patients and methods. A retrospective study was conducted involving 39 patients (16 girls and 23 boys) with BIEH. The following data were analysed for each patient: age, sex, family history, history concerning pregnancy, childbirth and neonatal period, postnatal history, data from clinical records and from physical examinations, progress of psychomotor development, findings from the first and successive neuroimaging studies, results of other complementary examinations, clinical and neuroimaging situation at the last check-up that was carried out, length of clinical control, age at which subdural effusion disappeared, and any other relevant associated facts. Results. Age at diagnosis ranged from 1.33 and 25 months (mean: 8.4 months); in 38.46% of cases there was a history of macrocephalia in one of the progenitors; in four of them the presence of congenital macrocephalia was noted; in five, there was motor retardation and one of them displayed psychomotor retardation; in 15, there was an association with a slight dilatation of the lateral ventricles; the mean time of clinical control was 3.36 years; the process was seen to resolve in 14 cases; the minimum age for the disappearance of the subdural effusion was 9 months and the maximum was 8 years; macrocephalia persisted until the clinical control ended in 22 of the cases. We also noted the presence of two cases of mitochondrial encephalomyopathy, one craniosynostosis of the sagittal suture, one microdeletion 22q11.2, one -1 antitrypsin deficiency, and one case of idiopathic bilateral congenital palpebral ptosis. Conclusions. The subdural effusion and/or macrocephalia persist in a high percentage of these patients and sometimes there is a close relationship between this condition and benign familial macrocephalia. Despite its benignity, it can influence psychomotor or motor retardation and behavioural disorders. On rare occasions it may be associated to mitochondrial encephalomyopathy and to the microdeletion 22q11.2

Allergy to drugs: antioxidant enzymic activities, lipid peroxidation and protein oxidative damage in human blood.

Reactive oxygen species lead to lipid peroxidation and specific oxidation of some specific enzymes, proteins and other macromolecules, thus affecting many intra- and intercellular systems. Recently, antioxidant functions have been linked to anti-inflammatory properties. Cell defences against toxic oxygen include antioxidant enzymes. We studied the enzymic antioxidant capacity in human blood of both erythrocytes and mononuclear cells from patients suffering from an allergic reaction to different drugs. We determined superoxide dismutases (SODs), glutathione peroxidase (GSHPx) and catalase (CAT) activities in each cell type. We also determined the extent of thiobarbituric acid reactive substances (TBARS) and the oxidative damage to proteins, in order to study the correlation between the cellular enzymic activities, the oxidative status and the allergic reaction. In mononuclear cells from allergic patients, SODs and CAT activities were enhanced compared with controls. Conversely, a decrease in GSHPx activity was found. In erythrocytes, higher values for CAT, GSHPx and SODs activities were found in allergic patients. TBARS were also enhanced in both types of cells, and the carbonyl content of serum was equally increased. The respective enzymic imbalances in mononuclear cells and erythrocytes, namely, GSHPx/SOD and CAT/SOD, and their consequences are discussed. To our knowledge, this is the first global study of antioxidant enzyme determinations, including TBARS level and carbonyl content, in patients suffering from allergies to drugs.

Chemical and biological activity of free radical 'scavengers' in allergic diseases.

Reactive oxygen species (ROS) are generated constantly in vivo. They can lead to lipid peroxidation and oxidation of some enzymes, as well as protein oxidation and degradation. Cells possess several biological systems, defined as 'scavengers', to protect themselves from the radical-mediated damage. Immune cells may discharge their arsenal of toxic agents against host tissues, resulting in oxidative damage and inflammation. Therefore, free radical production and disturbance in redox status can modulate the expression of a variety of immune and inflammatory molecules, leading to inflammatory processes, both exacerbating inflammation and effecting tissue damage. Recently, abnormal immunity has been related to oxidative imbalance, and antioxidant functions are linked to anti-inflammatory and/or immunosuppressive properties. Currently, allergy is one of the most important human diseases. We studied the role of the primary antioxidant defence system, constituted by the antioxidant enzymes superoxide dismutase, catalase and glutathione peroxidase, protecting cells from toxic oxygen. We analyzed how they are involved in blood cells detoxification, and how the imbalance of reactive oxygen species is related to inflammation in allergic diseases by affecting immune cells. Finally, we discuss the published data that relates anti-free radical therapy to the management of human allergic diseases.

Interrelationship between oxidative damage and antioxidant enzyme activities: an easy and rapid experimental approach.

This article describes a method for determining some antioxidant enzyme activities (catalase and/or glutathione peroxidase) and the oxidative status (protein oxidative damage and/or lipid peroxidation) of human blood. However, the main objective of the work is to illustrate the relationship between antioxidant defences and oxidative damage, showing to students their correlation and the general importance of the biochemical regulation in health and diseases.

Antioxidant activity levels and oxidative stress as blood markers of allergic response to drugs.

Antioxidant enzymes work together in human blood cells against toxic reactive oxygen species. Although their relationship with several pathophysiologic processes has been stated, not much is known about the connection between antioxidant defence and allergy. This study was designed to determine the enzymatic activities and the oxidative indices in the blood and serum proteins in patients suffering from allergy to drugs. We hypothesize that serum and blood reactions may serve as useful clinical marker for the allergic state. We used enzymatic antioxidant activities, thiobarbituric acid reactive substances, and carbonyl contents of proteins as suitable markers. We determined superoxide dismutases, glutathione peroxidase and catalase activities in each cell type. After antihistaminics plus steroids were given as part of a protocol treatment, enzymatic antioxidant activities, thiobarbituric acid reactive substance levels, and carbonyl contents were used as recovering markers for the disease. We found a relationship between antioxidant enzymatic activities, thiobarbituric acid reactive substance levels, and carbonyl contents for allergic reactions belonging to several type I and type IV allergies, as well as cross-reactive intolerance to nonsteroidal anti-inflammatory drugs and an anaphylactoid reaction to a radiocontrast media. A similar pattern also exists for analogous allergic manifestations and disease-like status.

Antioxidant enzymatic activities in human blood cells after an allergic reaction to pollen or house dust mite.

Several diseases have been related to oxidative stress. Recently, antioxidant functions have also been linked to anti-inflammatory properties. Cell defenses against reactive oxygen species include antioxidant enzymes. We studied the enzymatic antioxidant capacity in human blood of both red blood and mononuclear cells from patients suffering from an allergic reaction to pollen or house dust mite. We determined superoxide dismutases (SODs), glutathione peroxidase (GSHPx) and catalase (CAT) activities in each cell type. We also determined the extent of thiobarbituric acid reactive substances (TBARS), in order to study the correlation between the cellular enzymatic activities, the redox status and the disease. In mononuclear cells from allergic patients, SODs and CAT activities were enhanced compared to controls. Conversely, a decrease in GSHPx activity was found. In erythrocytes, higher values for GSHPx and SODs and similar CAT activities were found in allergic patients and controls. Interestingly, CuZnSOD and MnSOD activities were enhanced in the same proportion for both, erythrocytes and mononuclear cells. TBARS were also enhanced in both types of cells. The respective enzymatic imbalances in mononuclear cells and erythrocytes, namely, GSHPx/SOD and CAT/SOD, and their consequences are discussed. To our knowledge, this is the first global study of antioxidant enzymes, including TBARS level determinations, in allergy.

Antioxidant enzymes and human diseases.

OBJECTIVES: To describe the importance of the antioxidant enzymes superoxide dismutase, glutathione peroxidase, and catalase working together in human cells against toxic reactive oxygen species, their relationship with several pathophysiologic processes and their possible therapeutic implications. CONCLUSIONS: Reactive oxygen species (ROS) are involved in the cell growth, differentiation, progression, and death. Low concentrations of ROS may be beneficial or even indispensable in processes such as intracellular signaling and defense against micro-organisms. Nevertheless, higher amounts of ROS play a role in the aging process as well as in a number of human disease states, including cancer, ischemia, and failures in immunity and endocrine functions. As a safeguard against the accumulation of ROS, several nonenzymatic and enzymatic antioxidant activities exist. Therefore, when oxidative stress arises as a consequence of a pathologic event, a defense system promotes the regulation and expression of these enzymes.