[Primary ciliary dyskinesia in children. Role of electron microscopy in countries with medium economic resources]. / Discinesia ciliar primaria en niños. Rol de la microscopia electrónica en países de medianos recursos económicos.

Primary ciliary dyskinesia (PCD) is a rare genetic disease that produces functional and structural de fects in the cilia. In Peru, no cases of this disease have been reported in the pediatric population. OBJECTIVE: To describe the clinical, radiological and ciliary ultrastructure characteristics in children with PCD, in a country with medium economic resources. CLINICAL CASE: We report 5 patients with PCD treated at the Instituto Nacional de Salud del Niño-Breña (Peru). Age range 1 to 5 years (median 3 years). Three patients were male. The most frequent clinical manifestations were chronic wet cough, rhonchi, coarse crackles, recurrent bronchial obstructive syndrome, and recurrent pneumonia. All patients had atelectasis, three had bronchiectasis, and two had dextrocardia with situs inversus. Two patients had undergone lobectomy. Other causes of recurrent pneumonias were ruled out with im munodeficiency study, chlorine test and pulmonary aspiration The electron microscopy showed ab sence of the inner arm of dynein as the most frequent pattern. All patients received treatment with antibiotics, nebulization with hypertonic saline, and respiratory physiotherapy with good adherence. CONCLUSION: In medium incomes countries, electron microscopy associated with clinical and radio logical characteristics plays an important role in the early diagnosis of this disease. This is the first Peruvian report that contributes to the casuistry and epidemiology of this rare pathology.

[Surfactant protein C dysfunction in pediatric patients: Clinical Case]. / Disfunción de la proteína surfactante C en pacientes pediátricos: Caso Clínico.

Pulmonary surfactant dysfunction disorders are caused by genetic defects that alter pulmonary surfactant metabolism. They are rare disorders and cause significant morbidity and mortality in the neonatal and pediatric populations. OBJECTIVE: To describe the clinical, histopathological, and ultrastructural findings of the lamellar body that suggest surfactant protein C (SP-C) dysfunction, where confirmatory genetic studies are not available. CLINICAL CASE: We report three pediatric cases of pul monary surfactant dysfunction disorders from a pediatric hospital in Peru. Video-assisted lung biop sy was performed in all cases. Ultrastructural studies of the lamellar body were compatible with type- C pulmonary surfactant dysfunction. The treatment used was methylprednisolone pulses monthly for six months, then every two months, varying the duration according to the clinical evolution. They also received daily hydroxychloroquine and azithromycin three times a week. Clinical evaluations, eye fundus, echocardiogram, electrocardiogram, and biochemistry were performed periodically. At follow-up, there was a good response to treatment and no adverse effects were observed. One case died despite the therapies received. CONCLUSIONS: In 3 patients with type-C surfactant dysfunction, treatment with corticosteroids, hydroxychloroquine, and azithromycin was successful in 2 of them. This is one of the first case series reported in Peru that contributes to the study of these diseases, es pecially in low- and medium-income countries.

Comportamiento del PPD en niños con diagnostico de tuberculosis pulmonar, Servicio de Neumología - INSN / Behavior of the PPD in children with diagnosis of pulmonary tuberculosis, Service of Pneumology - INSN

El diagnóstico de TBC en niños sigue siendo difícil a pesar de los actuales adelantos científicos, debido a su naturaleza paucibacilar y mayores formas extrapulmonares. Su enfoque radica principalmente en la Historia de Contactos, radiografía de tórax y PPD. Este último, sin embargo no discrimina entre infección latente y enfermedad activa. El presente estudio, describe el comportamiento del PPD en pacientes diagnosticados de TBC pulmonar, su reactividad en relación a la baciloscopía. MATERIAL Y METODOS: Es un estudio descriptivo de cohorte transversal. Se revisó las fichas de seguimiento de todos los pacientes diagnosticados de TBC Pulmonar durante los años 2009 - 2010 en el Instituto Nacional de Salud del Niño, Lima - Perú. Los datos recolectados se analizaron por el sistema SPSS V15.0. RESULTADOS: Se encontraron 209 casos (56 por ciento fueron varones), clasificados por grupos etáreos: 6.7 por ciento (menor 1 año), 27.3 por ciento (1 a 4 años), 24.9 por ciento (5 a 9 años) y 41.1 por ciento (mayores de 10 años). Los casos más frecuentes provinieron de los distritos de Lima Este (22 por ciento), Centro (22 por ciento) y Sur (20 por ciento). Predominó la TBC Pulmonar Activo evolutiva (87.6 por ciento) sobre las otras formas. La positividad de las baciloscopías fue del 22 por ciento, el grupo mayor a 10 años presentó mayor frecuencia de baciloscopías positivas (41 por ciento). La positividad al PPD se observó en el 78 por ciento de los casos estudiados y existió contacto bacilífero conocido en un 45 por ciento del total. No se observó que una mayor medida del PPD se relacione a mayor posibilidad de encontrar una baciloscopía positiva. Además los pacientes mayores de 10 años reaccionaron más al PPD que los otros grupos. CONCLUSIONES: La prueba de Tuberculina (PPD) es una herramienta útil en el enfoque diagnóstico de la TBC en niños, su mayor medida no se relaciona a una mayor frecuencia de baciloscopías positivas, y está relacionada a una mejor respuesta inmune frente al MBT