Surfactante exógeno como tratamiento de la atelectasia pulmonar masiva / Exogenous surfactant as treatment for lung massive atelectasis

Fundamento: las atelectasias son un estado de colapso y falta de aire en todo el pulmón o en una zona del mismo. Una vez establecido el diagnóstico de colapso bronquial, el tratamiento debe orientarse directamente a la causa de la obstrucción y a la infección que habitualmente concomita. Objetivo: utilizar como estrategia de tratamiento el lavado broncoalveolar con surfactante exógeno (Surfacen®) unido a la fisioterapia respiratoria. Caso clínico: se presenta un caso de una paciente de 16 años de edad con el antecedente patológico personal de Epilepsia desde los dos años de vida bajo tratamiento con valproato de sodio, que ingresa en la unidad de cuidados intensivos del hospital pediátrico Dr. Eduardo Agramonte Piña, a la que se diagnostica por clínica, radiología y gasometría el síndrome de dificultad respiratoria aguda resultante de una causa pulmonar directa, al quinto día de tratamiento presenta compromiso respiratorio agudo con caída brusca de la oxigenación y se diagnostica radiológicamente una atelectasia masiva del pulmón izquierdo, se le realizó lavado broncoalveolar amplio con solución salina al 0,9 % y luego se instiló surfactante exógeno en dosis de 100 mg diluidos en 10 mL de agua para inyección, dos veces al día, administrado por vía intrabronquial. Luego del segundo lavado broncoalveolar y la kinesioterapia respiratoria, se logra la expansión total de la zona colapsada y recuperación notable de los parámetros de oxigenación. La radiografía reveló una mejoría evidente luego del tratamiento. Se retira la ventilación mecánica cinco días después de esta complicación, mantuvo buena evolución posterior. Conclusiones: la aplicación del lavado broncoalveolar con surfactante exógeno mejoró el compromiso de oxigenación que ocasiona la atelectasia masiva y permitió una rápida evolución radiográfica en la paciente, así como la disminución de los parámetros ventilatorios.

Background: atelectasis is a collapse state and shortness of breath in part or all of one lung. Once the diagnosis of bronchial collapse is established, the treatment must be directed towards the find of the cause of obstruction and the infection that is usually concomitant. Objective: to use bronchoalveolar lavage with exogenous surfactant (Surfacen®), along with respiratory physiotherapy as treatment. Clinical case: the case of a sixteen-year-old female patient with a personal-pathological history of epilepsy since she was two years old is presented. The patient was under treatment with valproate sodium. She is admitted in the intensive care unit of Dr. Eduardo Agramonte Piña Pediatric Hospital. The patient is diagnosed clinically, radiologically and gasometrically as having respiratory distress syndrome of a direct pulmonary cause. On the fifth day of treatment the patient presented acute respiratory problems with sudden oxygenation fall and is diagnosed radiologically as having massive atelectasis of the left lung. She underwent a wide bronchoalveolar lavage with 9 % saline solution and then the instillation of exogenous surfactant in 100 mg doses diluted in 10 ml of water per injection administered intrabronchially twice a day. After the second bronchoalveolar lavage and the respiratory kinesiotherapy, the total expansion of the collapsed area and a notable improvement of the oxygenation parameters were achieved. The mechanical ventilation was moved five days after the complication. The patient kept a good subsequent progress. Conclusions: the application of bronchoalveolar lavage with exogenous surfactant improved the oxygenation problem that causes massive atelectasis and allowed a rapid radiographic progress in the patient, as well as the decrease of the ventilatory parameters.

Hemoglobinas anormales en la población neonatal de Costa Rica

Abnormal haemoglobins in the newborn human population of Costa Rica. Hemoglobinopathies are hereditary autosomic recessive diseases. A total of 70 943 samples of whole blood collected by heel prick in filter paper (S&S 903) from throughout Costa Rica (October 2005-October 2006) were analyzed to detect variants of hemoglobin by the iso-electric focusing technique. Eight hundred ninety one cases presented some variant, for a frecuency of 1/79. Five cases are homozygous for hemoglobin S (sickle cell disease) and one shows the double heterozygous genotype SC. in this study the S and C variants of hemoglobin, although with some local differences, are widespread all over the country. Thus, the prevention of new cases is important through the testing of hemoglobin in the Costa Rican National Newborn Screening Program, together with a interdisciplinary National Program of Education for the disease and carrier status (AS/AC) for patients, families and medicar personnel. This is the basis for proper genetic counseling, to improve treatment and to reduce morbi-mortality. Rev. Biol. Trop. 56 (3): 995-1001. Epub 2008 September 30.

Se han analizado un total de 70 943 muestras de sangre total en papel filtro S&S 903 de neonatos de Costa Rica (octubre 2005 a Octubre 2006) con el fin de detectar variantes de hemoglobina mediante la técnica de isoelectroenfoque. Se detectaron 891 casos con alguna variante para una frecuencia de 1/79. Se clasifican 5 casos homocigotos para hemoglobina S (anemia drepanocítica o anemia falciforme) y un caso doble heterocigoto para SC. En este estudio se demuestra que las variantes fenotípicas de hemoglobina S como la C, se encuentran distribuidas por todo el país con algunas diferencias locales, razón por la cual es importante que la prevención de nuevos casos se realicé a través de nuestro Programa Nacional de Tamizaje de Hemoglobinas junto con un Programa Nacional interdisciplinario de Educación para el portador del rasgo (AS/AC) como, para el enfermo y su familia; al igual que la instauración de programas dirigidos a médicos generales y enfermeras en todas las regiones de salud del país, para asegurar consejo genético a portadores y enfermos, y a la vez, mejorar los sistemas de tratamiento a los pacientes para reducir la morbi -mortalidad.

[Abnormal haemoglobins in the newborn human population of Costa Rica]. / Hemoglobinas anormales en la población neonatal de Costa Rica.

Hemoglobinopathies are hereditary autosomic recessive diseases. A total of 70 943 samples of whole blood collected by heel prick in filter paper (S&S 903) from throughout Costa Rica (October 2005-October 2006) were analyzed to detect variants of hemoglobin by the iso-electric focusing technique. Eight hundred ninety one cases presented some variant, for a frecuency of 1/79. Five cases are homozygous for hemoglobin S (sickle cell disease) and one shows the double heterozygous genotype SC. In this study the S and C variants of hemoglobin, although with some local differences, are widespread all over the country. Thus, the prevention of new cases is important through the testing of hemoglobin in the Costa Rican National Newborn Screening Program, together with a Interdisciplinary National Program of Education for the disease and carrier status (AS/AC) for patients, families and medicar personnel. This is the basis for proper genetic counseling, to improve treatment and to reduce morbi-mortality.

Evolution and innovations of the National Neonatal and High Risk Screening Program in Costa Rica.

We present the evolution, organization and results of the National Neonatal and High Risk Screening Program in Costa Rica (PNT). This program has been working uninterruptedly for more than fourteen years. Costa Rica currently has a literacy rate of 95%. To August 2004 the rate of infant mortality was 9.74 per 1000 births and to 2003, life expectancy was 76.3 years for men and 81.1 years for women. The control of infectious and parasitic diseases, as well as of severe malnutrition, has given room to a prevalence of chronic diseases with a pathology profile similar to that of a developed country. The clinical observation, mainly starting from early 70s, of a growing number of patients with mental retardation and other disabilities caused by congenital hypothyroidism and hereditary metabolic diseases that could have been prevented in many cases with an early diagnosis and opportune treatment, led us to the decision to implement a systematically massive neonatal screening for these diseases. The presence of a single Public System of Social Security in Costa Rica, which currently includes from primary health care up to the hospitals of tertiary attention, with a single Children's Hospital for the whole country, as well as communication facilities, are factors that offered, in principle, favorable conditions for this effort, even for a developing country. To September 2004, 835,217 children have been screened. There is a coverage of 95.1% of the newborns in the country. Also to this date, 259 children with congenital hypothyroidism, 18 with phenylketonuria, 20 with the maple syrup disease, 30 with congenital adrenal hyperplasia and 10 with galactosemia have been detected, confirmed and treated, for a total of 337 children that were spared of mental retardation, other disabilities and even death. Massive neonatal screening for organic acidemias recently started in June of 2004. Cystic fibrosis is under a pilot study and the screening for hemoglobinopathies and toxoplasmosis is planned. The Center for Prevention of Disabilities, which started its functions on September 23, 2002, made feasible to integrate neonatal screening, high risk screening and diagnostic confirmation of the diseases now included in the national screening program as well as those to be added in the future.