[Kawasaki disease: Diagnosis and treatment]. / Enfermedad de Kawasaki: diagnóstico y tratamiento.

INTRODUCTION: Kawasaki disease is the leading cause of acquired heart disease in children. In spite of the efficacy of intravenous immunoglobulin (IGIV), the absence of a specific diagnostic test and due to there being IGIV-refractory patients, Kawasaki disease is a major cause of coronary artery abnormalities (CAA). OBJECTIVES: To analyze the clinical and epidemiological characteristics of cases of Kawasaki disease, to evaluate the efficacy of treatments used and the CAA observed. METHODS: We retrospectively reviewed the medical records of children diagnosed with Kawasaki disease between January 2002 and December 2008 in a tertiary public Hospital in the South of Madrid. The diagnosis of Kawasaki disease was based on the clinical criteria proposed by the American Academy of Pediatrics in 2004. RESULTS: Twenty three children were identified. Median age was 26 months (range: 2 months-10 years). Nineteen children (82%) were younger than 5 years old. Fever and changes in the lips and oral cavity were present in all cases. Twenty-one patients (91%) received IGIV, all of them before the 10th day of disease. One child (4.7%) required the administration of more than one dose of IGIV, because persistence of fever. CAA was recorded in three patients [13.0%, (95% CI: 1-26%)], including a four month-old boy. All patients with CAA were treated with the recommended dose of IGIV, 2g/kg, between the 5th and 8th day of disease. CONCLUSIONS: Kawasaki disease was more common in children less than five years old. We observed a high rate of CAA in children with Kawasaki disease in spite of appropriate and timely treatment.

[Neonatal lactic acidosis caused by severe pyruvate carboxylase deficiency]. / Acidosis láctica neonatal debida a deficiencia aislada de piruvato carboxilasa.

A new patient with neonatal lactic acidosis due to pyruvate carboxylase deficiency is described. Since birth he developed vomiting, hypothermia, lethargy, irritability, hypoglycemia and severe metabolic acidosis. During admission a progressive deterioration was observed. Despite different attempted therapies patient died at 4 1/2 months of age. High levels of plasma and urine lactate and pyruvate were detected. Enzymatic studies in cultures skin fibroblasts and postmortem tissues showed a severe deficiency of pyruvate carboxylase.

Administración de metamizol, insuficiencia renal y constricción intrauterina del conducto arterioso. Una asociación poco conocida / Administration of metamizol, renal failure and intrauterine ductus arteriosus constriction. An uncommon association

El metamizol, o dipirona magnésica, es un antiinflamatorio no esteroideo (AINE) ampliamente utilizado en Europa como analgésico y antipirético. Como con la indometacina y el ibuprofeno, se han descrito casos de oligoamnios, insuficiencia renal y cierre intrauterino del conducto arterioso fetal después del uso de metamizol en el tercer trimestre de la gestación, aunque su asociación con estos efectos adversos es menos conocida que con otros AINE. En el presente artículo presentamos un caso de hidropesía fetal con oligoamnios y constricción intrauterinaductal, que cursó con insuficiencia renal e hipertensión pulmonar, en el hijo de una madre que había recibido metamizol en el contexto de una pielonefritis aguda. Asimismo, se discutirá la fisiopatología de esta entidad y se revisará la bibliografía científica existente sobre el tema(AU)

The metamizole or magnesium dipyrone is a nonsteroidal anti-inflammatory drugs (NSAIDs) widely used in Europe as an analgesic and antipyretic. As with indomethacin and ibuprofen cases of oligohydramnios, renal failure and intrauterine closure of ductus arteriosus after its use in the third trimester of pregnancy have been reported, although its association with these adverse effects is less well known than other NSAIDs. In this article we present a case of hydrops fetalis with oligohydramnios and intrauterine ductal constriction with pulmonary hypertension and renal failure in the child of a mother who had received metamizole in the context of an acute pyelonephritis. We also will discuss the pathophysiology of this entity and review the existing scientific literature on this topic(AU)