Hidropesía vesicular como forma de presentación de la enfermedad de Kawasaki / Hydrops of the gallbladder as a form of presentation of Kawasaki disease

La enfermedad de Kawasaki (EK) es una vasculitis aguda de etiología desconocida. Clínicamente, se caracteriza por la presencia de fiebre, conjuntivitis, cambios en las extremidades, exantema, afectación de los labios y la mucosa oral, y linfadenopatía. La hidropesía vesicular se ha descrito en aproximadamente el 10-15% de los pacientes con EK. Esta enfermedad es el proceso febril más frecuentemente asociado a hidropesía vesicular. Es necesario sospechar la EK ante una hidropesía vesicular aguda febril para no demorar la instauración del tratamiento adecuado. Aportamos el caso de un paciente de 8 años de edad, ingresado por presentar fiebre e hidropesía vesicular, en el que se diagnosticó una EK (AU)

Kawasaki disease (KD) is an acute vasculitis of unknown aetiology. Clinically it is characterised by fever, conjunctivitis, changes in the extremities, exanthema, involvement of the lips and oral mucosa and lymphadenopathy. Hydrops of the gallbladder has been described in approximately 10-15% of patients with KD. This disease is the febrile condition most frequently associated with hydrops of the gallbladder. KD must be suspected in the event of acute febrile hydrops of the gallbladder so as to avoid delays in the start of an appropriate treatment. We present the case of an 8-year-old patient admitted for fever and hydrops of the gallbladder, and diagnosed with KD (AU)

[Afebrile convulsions and acute gastroenteritis: a more common than expected combination]. / Convulsiones afebriles y gastroenteritis aguda: una asociación más frecuente de lo esperado.

Afebrile seizures in association with acute gastroenteritis without electrolyte disturbances have been reported in children from Asiatic countries, but only few references are from Spain. It is a benign, self-limiting process, with no specific examinations or therapy needed. There is no increased risk of epilepsy or impaired neurodevelopment in the children affected. We present five children with seizures during acute gastroenteritis admitted to Hospital Universitari Arnau de Vilanova of Lleida (Spain) between December 2005 and March 2006, during the last seasonal rotavirus outbreak. Patients with febrile seizures, electrolyte disturbances or previous seizures have been excluded. Association between acute gastroenteritis and afebrile benign seizures is also common in our setting, and some cases associated to seasonal rotavirus outbreaks have been published. All papers agree with the good prognosis of this problem. To consider this diagnosis is important in order avoid unnecessary examinations or anticonvulsive therapies.

Convulsiones afebriles y gastroenteritis aguda: una asociación más frecuente de lo esperado / A febrile convulsions and acute gastroenteritis: a more common than expected combination

La presentación de convulsiones afebriles en el marco de una gastroenteritis aguda leve sin alteraciones hidroelectrolíticas es una entidad descrita en pediatría con relativa frecuencia en países asiáticos. En nuestro medio, las referencias son más reducidas. Se trata de un proceso benigno que no precisa exploraciones complementarias agresivas ni tratamiento anticomicial posterior, y que no aumenta el riesgo de epilepsia ni de deterioro neuropsíquico. Se presentan 5 casos de convulsión afebril asociada a gastroenteritis aguda, ingresados en el Servicio de Pediatría del Hospital Universitario Arnau de Vilanova desde diciembre de 2005 hasta marzo de 2006. Se han excluido las convulsiones febriles y los casos con alteraciones hidroelectrolíticas o antecedentes previos de convulsión. La asociación entre gastroenteritis aguda y convulsiones benignas afebriles es también frecuente en nuestro medio, y se han publicado casos asociados a brotes estacionales de infección por rotavirus. Todos los trabajos publicados coinciden en la benignidad y el buen pronóstico del proceso. Es importante considerar esta entidad para evitar exámenes complementarios innecesarios y tratamientos antiepilépticos prolongados

Afebrile seizures in association with acute gastroenteritis without electrolyte disturbances have been reported in children from Asiatic countries, but only few references are from Spain. It is a benign, self-limiting process, with no specific examinations or therapy needed. There is no increased risk of epilepsy or impaired neurodevelopment in the children affected. We present five children with seizures during acute gastroenteritis admitted to Hospital Universitari Arnau de Vilanova of Lleida (Spain) between December 2005 and March 2006, during the last seasonal rotavirus outbreak. Patients with febrile seizures, electrolyte disturbances or previous seizures have been excluded. Association between acute gastroenteritis and afebrile benign seizures is also common in our setting, and some cases associated to seasonal rotavirus outbreaks have been published. All papers agree with the good prognosis of this problem. To consider this diagnosis is important in order avoid unnecessary examinations or anticonvulsive therapies

Enfermedad invasiva por Streptococcus pyogenes en lactante sano de 38 días / Invasive disease due to Streptococcus pyogenes in a healthy, 38-day-old infant

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Secuencia de Poland: aportación de dos nuevos casos / Poland sequence: two new cases

La secuencia de Poland es una anomalía musculosquelética congénita caracterizada por la ausencia de la porción esternal del músculo pectoral mayor asociada a braquisindactilia. Se presenta con una incidencia aproximada de 1/20.000-30.000 nacidos vivos (un 75% de los casos corresponde a varones), y en un 70% está afectado el lado derecho. Además de la asociacióncon la braquisandactilial, aausencia del pectoral mayor puede presentarse aislada o bien asociada a otras malformaciones, especialmente de la zona pectoral o del brazo y la mano homolaterales, como sindactilia, ausencia del pectoral menor, hipoplasia de la extremidad o amastia. También se puede asociar a alteraciones de otros territorios, como dextrocardia, síndrome de Moebius o neurofibromatosis. La etiología es desconocida y habitualmente no presenta base hereditaria. Se ha propuesto como teoría patogénica una hipoplasia de la arteria subclavia fetal. El tratamiento es quirúrgico, fundamentalmente con fines estéticoso para corregirlas a1teraciones funcionales de la mano que esta anomalía conlleva. Aportamos dos casos tratados en nuestro servicio de pediatría: uno corresponde a un varón recién nacido, con afectacióndel pectoral mayor e hipoplasia del arco anterior de las costillas 2-5, y el otro a una niña de 9 años afectada de asma mediada por inmunoglobulina E (lgE), que asocia a la agenesia del pectoral mayor una hipoplasia de la mama, una hipoplasia de la mano y braquisindactilia

Poland sequence is a congenital musculoskeletal disorder characterized by agenesis of the sternal portion of the pectoralis major muscle and brachysyndactyly. The incidence is about 1/20,000 to 1/30,000 live births. The ratio of males to females is 3:1 and the right is the side aftected in 70% of cases. The agenesis of the pectoralis major muscle can be the only manifestation or can be associated with other lesions in the pectoral region and ipsilateral upper extremity: brachysyndactyly, absence of the pectoralis minar muscle, hypoplasia of the arm or hand or amastia. Its association with other conditions, like dextrocardia, Moebius syndrome or neurofibromatosisis also possible. The etiology is unknown, and it is usually not hereditary. Hypoplasia of the fetal subclavian artery has been proposed as an underlying cause. The treatment is surgical, mainly for cosmetic reconstruction or improvement in the function of the affected hand. We report two new cases observed in our service. One involves a male infant with agenesis of pectoralis major and hypolasia of anterior arch of the 2nd to 5th ribs. The other patient isa 9-year-old girl with IgE-mediated asthma and agenesis of the pectoralis, breast hypoplasia, Sprengel's deformity and brachysyndacty

[Bacteremia during the course of Salmonella gastroenteritis]. / Bacteriemia en el curso de gastroenteritis a Salmonella.

OBJECTIVE: Salmonella is the most frequent cause of bacterial acute gastroenteritis (AGE) in our setting. Usually its course is self-limited, but it sometimes can lead to bacteremia, principally in young infants and malnourished or immunosuppressed children. Bacteremia is less frequent in healthy people and in those over one year of age. This study was carried out to assess the incidence of bacteremia during Salmonella GE and to detect parameters that could lead to bacteremia. PATIENTS AND METHODS: A retrospective study of positive stool cultures in our hospital between 1991 and 1993 was performed. Data about the epidemiology, clinical features cultures and analytical procedures were drawn from clinical records. Data were analyzed using the Mann-Whitney test and Fisher's exact test. RESULTS: During this period of time, 860 cases of AGE were observed with 86 stool cultures positive for Salmonella (10%). Six of them also had positive blood cultures (7%). All 6 patients with bacteremia were healthy previously and five were over 12 months old. The outcome was good in all cases, without focalizations of the bacteremia. We could not detect any differences between patients with positive blood cultures and the patients without bacteremia. CONCLUSIONS: Bacteremia during Salmonella AGE is not infrequent and is not limited to young infants or patients with underlying diseases. None of the parameters analyzed were useful in predicting the possibility of bacteremia.

[An outbreak of neonatal meningitis caused by enterovirus]. / Brote neonatal de meningitis por enterovirus.

Six newborn infants affected by acute aseptic neonatal meningitis were attended during the months of June and July 1987. Fever, good general physical appearance and no associate focal symptomatology was the onset in five cases, whereas one of them had apnea spells. An increase in total cell count was present in CSF with polymorphonuclears predominance in two cases, being lymphocites more prevalent in the others. Enterovirus were demonstrated as a cause in three of them. After describing clinical facts and evolution, some epidemiological aspects are commented.

[Epidemic outbreak of necrotizing enterocolitis coincident with an epidemic of neonatal rotavirus gastroenteritis]. / Brote epidémico de enterocolitis necrotizante, coincidiendo con una epidemia de gastroenteritis neonatal a rotavirus.

Authors present four cases of neonatal necrotizing enterocolitis occurred during an outbreak of acute enteritis. From november 1985 to beginning of february 1986, forty-seven out of 588 newborn infants were involved. In 22 affected infants (46.8%) rotavirus were isolated from stools, whereas no bacterial or viral agent was recovered in 25 (53.2%). Four cases (8.6%) developed necrotizing enterocolitis.

[Sepsis, as the presenting form of hereditary tyrosinemia type I]. / Sepsis, como forma de presentación de tirosinemia hereditaria tipo I.

A case of hereditary tyrosinemia type I with a septic onset is reported. Diagnosis was established by the urinary presence of succinylacetone (SA) and deficiency in fumarylacetoacetate hydrolase (FAAH) in a culture of fibroblasts. Response to treatment with a diet in which the aminoacids phenylalanine, tyrosine and methionine were restricted is commented upon.