Tuberculosis infantil: presentación de 3 casos y discusión / Pediatric tuberculosis: case report and discussion

Se presentan tres casos de tuberculosis infantil. Los dos primeros casos son dos hermanos de 5 años y 20 meses de edad con tuberculosis pulmonar, contactos intradomiciliarios del caso índice, la madre de ambos afecta de tuberculosis pulmonar bacilífera, con antecedente de viaje hace 4 meses a su pais de origen (Marruecos); ambos diagnosticados por positividad de la prueba de tuberculina y estudio radiográfico. El tercero es una niña de 9 años afecta de artritis idiopática juvenil en tratamiento inmunosupresor que desarrolló tuberculosis miliar con afectación encefálica, diagnosticada inicialmente en contexto de estudio de adenopatía (AU)

Three cases of childhood tuberculosis are presented: first and second cases are two brothers of 5 years and 2.0 months old respectively with pulmonary tuberculosis, household contacts of the index case: their mother, who was affected by smear-positive pulmonary tuberculosis, four months after visiting their country of origin (Morocco); both brethren were diagnosed by positivity of the tuberculin test and radiographic study. The third case is a 9 ycar old girl, juvenile idiopathic arthritis patient treated With immunosuppressive therapy, who has developed miliary tuberculosis with meningoencephalic involvement and she was initially diagnosed by the study of an adenopathy (AU)

Cojera en una niña de 20 meses, ¿es siempre una sinovitis de cadera? / Lameness in a 20 months girl, is it always a synovitis of the hip?

La cojera o el dolor musculoesquelético es una consulta frecuente en la infancia. Su etiología es diversa y frecuentemente banal. En algunas ocasiones la cojera puede ser el síntoma inicial de procesos neoplásicos. Presentamos el caso de una lactante de 20 meses de edad que presentó una cojera de dos semanas de evolución. En la valoración inicial en Atención Primaria no se apreció patología de gravedad, se realizó una ecografía de caderas y fue diagnosticada de sinovitis transitoria. Tras un periodo de reposo relativo y antiinflamatorios no se apreció mejoría. Varias semanas después presentó dolor nocturno de extremidades de forma intermitente. Se realizó una analítica en la que se apreciaba una neutropenia. Ante la persistencia de la cojera, la presentación del dolor nocturno y la neutropenia, se procedió a derivar a la paciente al Servicio de Oncohematología de referencia donde se diagnosticó de leucemia linfoblástica aguda (AU)

Lameness or musculoskeletal pain is a frequent consultation in the infancy. Its etiology is diverse and frequently not severe. In some cases lameness can be the initial symptom of neoplasic processes. Caucasic woman aged 20 months who presented lameness of two weeks of evolution. In the initial evaluation in Health primary care center we didn't appreciate any severity process. An ultrasound scan of hips was realized and she was diagnosticated of transient sinovitis of the hip. After a period of relative rest and anti-inflammatory treatment medical staff didn't appreciate any improvement. Several weeks later she presented intermitent night pain in the legs. A blood sample was taken and discovered neutropaenia. Given the persistence of the lameness, night pain and neutropenia, paediatrician sent the patient to paediatric oncohematology department where the diagnosis of linfoblastic leukaemia was realized after bony marrow aspiration (AU)

[Central sleep apnea syndrome as the first sign of type I Chiari malformation]. / Síndrome de apnea central del sueño como primera manifestación de malformación de Chiari tipo I.

INTRODUCTION: Type I Chiari malformation consists on the caudal displacement of cerebellar tonsils through the foramen magnum. It is often asymptomatic, although it may display symptoms as a result of cerebellum, brainstem, high cervical spinal cord or the lower cranial nerve, involvement. OBJECTIVE: We report our experience over the last 16 years. We have identified 16 patients with type I Chiari malformation. Only 2 cases showed common type I Chiari symptoms and just one had respiratory disorder as the first clinical sign. CLINICAL CASE: A 15 year old girl presented with a 5 years' history of chronic daily cough aggravated by exercise. Snoring and sleep apnea had been noted by her mother for 1 year. The girl eventually suffered from migraine and diurnal hypersomnolence. The physical and neurological examination was normal with the only exception being the absence of bilateral nauseous reflex. A nocturnal polysomnography study demonstrated a pseudoperiodic pattern with apnea pauses associated to cycles of deep breathing, resulting in severe gasometric repercussion and bradycardia. Magnetic resonance imaging of the brain showed Chiari I malformation. Non-invasive mechanical ventilation treatment significantly improved the clinical symptoms and gasometric analysis. DISCUSSION: Surgical posterior fossa decompression is discussed. Early decompression before appearance of irreversible neurological damage is recommended. It is associated with a significant reduction in the number of central apneas and sleep arousals. Surgical intervention is recommended in symptomatic patients and in cases of radiographic Chiari malformation or syrinx progression.

Síndrome de apnea central del sueño como primera manifestación de malformación de Chiari tipo I / Central sleep apnea syndrome as the first sign of type I Chiari malformation

Introducción: La malformación de Chiari tipo I consiste en el descenso de las amígdalas cerebelosas a través del foramen magno. Con frecuencia es asintomática, pero puede presentar clínica por afectación de cerebelo, troncoencéfalo, porción superior de la médula cervical y pares craneales bajos. Objetivo: Se presenta nuestra experiencia de 16 años con 16 casos de malformación de Chiari tipo I; sólo en 2 existen síntomas atribuibles a la anomalía de Chiari tipo I, y en una de ellas la primera manifestación clínica consistió en un trastorno respiratorio durante el sueño. Caso clínico: Niña de 15 años con tos crónica diaria, agravada con el ejercicio, de 5 años de duración. La madre observaba desde hacía 1 año que al dormir la niña roncaba y realizaba repetidamente pausas de apnea. Tenía cefaleas ocasionales e hipersomnolencia diurna. Las exploraciones física y neurológica fueron normales, salvo ausencia de reflejo nauseoso bilateral. El estudio polisomnográfico nocturno evidenció un patrón seudoperiódico con alternancia de pausas de apnea con ciclos de respiraciones profundas, con graves repercusiones gasométricas y sobre la frecuencia cardíaca. La resonancia magnética mostró anomalía de Chiari tipo I. La ventilación mecánica no invasiva supuso una mejoría clínica y gasométrica en la paciente. Discusión: La descompresión quirúrgica es discutida. Está indicada de forma precoz, antes de que aparezca daño neurológico irreversible. Se asocia con una reducción significativa en el número de apneas centrales y microdespertamientos. Se recomienda en casos sintomáticos o en casos con progresión radiológica de la anomalía de Chiari o de la siringomielia asociada (AU)

Introduction: Type I Chiari malformation consists on the caudal displacement of cerebellar tonsils through the foramen magnum. It is often asymptomatic, although it may display symptoms as a result of cerebellum, brainstem, high cervical spinal cord or the lower cranial nerve, involvement. Objective: We report our experience over the last 16 years. We have identified 16 patients with type I Chiari malformation. Only 2 cases showed common type I Chiari symptoms and just one had respiratory disorder as the first clinical sign. Clinical case: A 15 year old girl presented with a 5 years history of chronic daily cough aggravated by the exercise. Snoring and sleep apnea had been noted by her mother for 1 year. The girl eventually suffered from migraine and diurnal hypersomnolence. The physical and neurological examination was normal with the only exception being the absence of bilateral nauseous reflex. A nocturnal polysomnography study demonstrated a pseudoperiodic pattern with apnea pauses associated to cycles of deep breathing, resulting in severe gasometric repercussion and bradycardia. Magnetic resonance imaging of the brain showed Chiari I malformation. Non-invasive mechanical ventilation treatment significantly improved the clinical symptoms and gasometric analysis. Discussion: Surgical posterior fossa decompression is discussed. Early decompression before appearance of irreversible neurological damage is recommended. It is associated with a significant reduction in the number of central apneas and sleep arousals. Surgical intervention is recommended in symptomatic patients and in cases of radiographic Chiari malformation or syrinx progression (AU)

[Facial paralysis reported in a paediatric emergency department: actuation protocol reviewed and verified]. / Parálisis facial en urgencias de pediatría: actualización de nuestro protocolo y autoevaluación.

INTRODUCTION: As result of our aim to improve the quality standard of our emergency system, work has been carried out in relation to the development and monitorization of effective clinical protocols in the department of paediatric practice. PATIENTS AND METHODS: An evidence based review approach was taken to design a clinical protocol about Bell's palsy condition for the paediatric emergency department. Previous protocol approved in March 2003 was reviewed accordingly with the new designed protocol's quality standards. The Bell's palsy cases reported since March 2003 until June 2006 to paediatric emergency department were analyzed. RESULTS: A total of 27 patients affected by Bell's palsy were reported to the hospital's emergency department. Facial expression was described in 85.19% of the cases. Cranial nerves normal function was reported in 77.78%. Fundoscopic examination was described in 77.78% and otoscopic findings in 44.44%; the absence of herpes vesicles was analyzed only in 11.11%. All patients received steroid therapy (prednisone) and the treatment resulted in the complete recovery. The mean time to resolution was 58.6 days. CONCLUSIONS: In order to improve hospital's quality standards, clinical protocols should be designed and verified regularly to ensure the proper performance. Medical auditing also contributes to improve effectiveness in health attendance.

Parálisis facial en urgencias de pediatría: actualización de nuestro protocolo y autoevaluación / Facial paralysis reported in a paediatric emergency department: actuation protocol reviewed and verified

Introducción. En nuestro hospital se está trabajando en un plan de mejora de calidad de las urgencias pediátricas que incluye, entre otras actuaciones, la protocolización de los procedimientos (mediante la revisión de la evidencia disponible) y la monitorización de su aplicación. Pacientes y métodos. Se revisa, según la evidencia disponible, el protocolo de urgencias de la parálisis facial. Se evalúa la aplicación del previo, consensuado en sesión hospitalaria en marzo del 2003, y se aplican de forma retrospectiva los indicadores diseñados en la revisión de junio de 2006. Se analizan los casos de parálisis facial a frigore valorados en urgencias de Pediatría desde marzo de 2003 hasta junio de 2006. Resultados. En el período de estudio fueron evaluados en Urgencias 27 pacientes con parálisis facial. En el 85,19% de los casos se describió la alteración de la mímica facial en el informe de urgencias. En el 77,78% de los casos constaba la normalidad de los demás pares craneales, y en el 77,78% del fondo de ojo. En el 44,44% se recogió la normalidad de la otoscopia; sólo en el 11,11% se especificaba la ausencia de vesículas herpéticas. Todos recibieron tratamiento con prednisona, fueron seguidos en la consulta de Neuropediatría y se resolvieron completamente, con una media de 58,6 días. Conclusiones. En el proceso de mejora de calidad es fundamental la elaboración de protocolos, y la evaluación de su cumplimiento mediante indicadores de calidad. El audit. y sus reevaluaciones facilitan la implantación del ciclo de aprendizaje y mejora en la atención sanitaria, vinculados a la excelencia del trabajo

Introduction. As result of our aim to improve the quality standard of our emergency system, work has been carried out in relation to the development and monitorization of effective clinical protocols in the department of paediatric practice. Patients and methods. An evidence based review approach was taken to design a clinical protocol about Bell’s palsy condition for the paediatric emergency department. Previous protocol approved in March 2003 was reviewed accordingly with the new designed protocol’s quality standards. The Bell’s palsy cases reported since March 2003 until June 2006 to paediatric emergency department were analyzed. Results. A total of 27 patients affected by Bell’s palsy were reported to the hospital’s emergency department. Facial expression was described in 85.19% of the cases. Cranial nerves normal function was reported in 77.78%. Fundoscopic examination was described in 77.78% and otoscopic findings in 44.44%; the absence of herpes vesicles was analyzed only in 11.11%. All patients received steroid therapy (prednisone) and the treatment resulted in the complete recovery. The mean time to resolution was 58.6 days. Conclusions. In order to improve hospital’s quality standards, clinical protocols should be designed and verified regularly to ensure the proper performance. Medical auditing also contributes to improve effectiveness in health attendance

Síndrome de Gorlin neonatal asociado a hemimegalencefalia confirmado por estudio genético / Neonatal Gorlin syndrome associated to hemimegalencephaly confirmed by genetic study

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