Anales Espanoles De Pediatria最新文献

Although obstructive sleep apnea syndrome (OSAS) in children is a frequent and potentially serious respiratory disorder, it has a reliable diagnosis and treatment is highly effective. OSAS is a respiratory sleep-related disorder that forms part of sleep apnea-hypoapnea syndrome. The syndrome affects between 1 % and 3 % of children. In addition to its cardiopulmonary complications, it can retard growth and increase the risk of hyperactivity and learning difficulties. It has also been associated with attention deficit disorder and hyperactivity. When OSAS is suspected, up-to-date nocturnal polysomnography is the gold standard for the diagnosis and quantification of severity of childhood OSAS. In most children the treatment of choice is adenotonsillectomy, which has a success rate of more than 85 %. We provide an up-to-date review of the evidence on the clinical features, etiology, complications and treatment of OSAS in children. The main objective of this review is to alert pediatricians to their essential role in the early detection of this syndrome, especially among children who snore, and to provide a clinical practice guideline for the diagnosis and definitive treatment of these children.

Nijmegen breakage syndrome is a rare autosomal recessive disorder characterized by a peculiar dysmorphic syndrome (microcephaly, "bird-like" facies, short stature), combined immunodeficiency with recurrent infections, X-ray hypersensitivity and predisposition to malignancy, mainly lymphomas, as a consequence of chromosome instability due to anomalies in the repair of double-stranded DNA breaks.We present a 6-year-old boy with Nijmegen breakage syndrome, who developed a large B-cell non-Hodgkin's lymphoma, localized in the lung without nodal involvement.

Primary cutaneous involvement in B-cell lymphoblastic leukemia/lymphoma is rare in childhood. We present the case of an eleven and a half year old girl who, five months prior to being referred to our center, had undergone surgery to remove a small gluteal tumor diagnosed histopathologically as lymphoid proliferation suggestive of large cell lymphoma. On examination the presence of small nodes close to the scar where the tumor had previously been removed was observed. Hemogram revealed 2.7 x 9/l white blood cells with 0.5 x 9/l neutrophils; the remaining series and complementary investigations were normal. Bone marrow aspiration revealed 52 % blastic cells with immunophenotype and morphological characteristics of common (B-cell) acute lymphoblastic leukemia with L2 subtype in the French-American-British (FAB) classification. Ten months after finishing polychemotherapy, the patient is now in complete remission. We would like to highlight that a small slow-growing cutaneous node could be the presenting form of lymphoblastic lymphoma or acute lymphoblastic leukemia.

Backgrounds Some patients with a poor prognosis cause serious doubts about the real benefit of life-sustaining treatment. In some cases the possibility of limiting those treatments is raised. Such end-of-life decisions provoke ethical dilemmas and questions about procedure.ObjectivesTwo determine the frequency of end-of-life decisions in neonates, patient characteristics, and the criteria used by those taking decisions.Patients and methodsWe performed a multicenter, descriptive, prospective study. Neonates from 15 neonatal intensive care units who died during their stay in the hospital between 1999 and 2000, as well as those in whom end-of-life decisions were taken, were included. End-of-life decisions were defined as clinical decisions to withhold or withdraw life-sustaining treatment.ResultsA total of 330 patients were included. End-of-life decisions were taken in 171 (52 %); of these, 169 (98.8 %) died. The remaining 159 patients (48.2 %) died without treatment limitation. The main disorders involving end-of-life decisions were congenital malformation (47 %), neurologic disorders secondary to perinatal asphyxia and intracranial hemorrhage-periventricular leukomalacia (37 %). Of the 171 neonates, treatment was withheld in 80 and vital support was withdrawn in 91. The most frequently withdrawn life-sustaining treatment was mechanical ventilation (68 %). The criteria most commonly used in end-of-life decisions were poor vital prognosis (79.5 %), and current and future quality of life (37 % and 48 % respectively). The patient's external factors such as unfavorable family environment or possible negative consequences for familial equilibrium were a factor in 5 % of decisions.ConclusionsThe present study, the first of this type performed in Spain, reveals little-known aspects about the clinical practice of withholding and/or withdrawing life-sustaining treatment in critically ill neonates. End-of-life decisions were frequent (52 %) and were followed by death in most of the patients (98,8 %). The main criteria in decision-making were poor vital prognosis and the patient's current and future quality of life.