Síndrome de apnea obstructiva del sueño con repercusión sistémica. A propósito de un caso / Obstructive sleep apnea syndrome with systemic repercussion. Case report

Presentamos el caso de un niño de 4 años que ingresa en la unidad de cuidados intensivos pediátricos (UCIP) por mal estado general, dificultad respiratoria severa con débil esfuerzo y ronquido inspiratorio. La auscultación cardiopulmonar muestra hipoventilación y ritmo de galope, tiene hepatomegalia y, desde el punto de vista neurológico, presenta disminución del nivel de conciencia (Glasgow 6/15). En las pruebas complementarias, se observa un daño hipóxico-isquémico generalizado(creatinina sérica de 1,8 mg/dL; GOT de 23.730 UI/L y GPT de 5.771 UI/L; actividad de protrombina del 31% y troponina de1,73 ng/mL). La radiografía de tórax muestra una discreta cardiomegalia y la ecocardiografía hipertensión pulmonar. En la eco-Doppler abdominal se observa una severa hiperecogenicidad cortical renal y hepatomegalia. En el electroencefalograma hay signos de afectación cerebral generalizada, y en la tomografía computarizada (TC) craneal aparecen dos áreas cerebrales sugestivas de infartos isquémicos e hipertrofia adenoidea. A los pocos días del ingreso, se realiza una adenoamigdalectomía. En el momento del alta, los parámetros analíticos son normales, y en la ecocardiografía no se observa hipertensión pulmonar (AU)

We present a case report of a four year old child who is admittedat the PICU due to general bad condition, serious respiratorydifficulty with low effort and inspiratory snoring. The cardiopulmonarauscultation shows hypoventilation and gallop rhythm.He also shows hepatomegaly and awareness reduction at aneurological level (Glasgow 6/15). The complementary testsshow a generalized hypoxic-ischemic injury (serum creatinine of1.8 mg/dL; GOT of 23,730 IUI/L and GPT of 5,771 IU/L; prothrombinactivity 31% and troponin 1.73 ng/mL). Thorax X-rayshows cardiomegaly and the echocardiography pulmonary hypertension.The abdominal echo-doppler shows a serious renalcortical hyperechogenicity and hepatomegaly. At the electroencephalographthere are some signs of generalized cerebraldamage and in the cranial scan there are two cerebral areasthat suggest ischemic infarctions and adenoid hypertrophy. Atonsillectomy is performed some days after admission. At thedischarge, the analytical parameters are normal and there is nopulmonary hypertension (PHT) in the echocardiography (AU)

[Pneumococcal conjugate vaccine failure in HIV-infected child. Clinical case]. / Fallo de la vacuna antineumocócica conjugada en un niño VIH positivo. A propósito de un caso.

Despite appropriate antimicrobial therapy and vaccination, invasive pneumococcal infections remain associated with significant mortality, especially in selected high-risk groups (asplenic, humoral immunity deficient patients, etc.). We present a 13-year-old caucasian boy with HIV infection (vertical transmission). He received treatment with highly-active antiretroviral therapy (amprenavir, lamivudine and zidovudine) and vaccination with 23-valent vaccine (6 years old) and 7-valent pneumococcal conjugate vaccine (10 years old). His CD4 count and his viral load at these times were 2,063/microl and 13461 cop/ml, when he was 6 years old and 1,315/microl and 32400 cop/ml when he was 10 years old, respectively. The latest CD4 count (1,000/microl) and his viral load (3800 cop/ml) confirmed satisfactory control of the disease. He was referred to our emergency department presenting with fever, head and stomach-ache and vomiting. In the following hours his condition continued to deteriorate and depressed level of consciousness and meningismus were observed. Streptococcus pneumoniae, serotype 18 C, was detected in blood and cerebrospinal fluid cultures. Despite appropriate treatment with antibiotics (cefotaxime and vancomycin) and anti-oedema medications, brain-death was confirmed 24 hours after his admittance.

Fallo de la vacuna antineumocócica conjugada en un niño VIH positivo. A propósito de un caso / Pneumococcal conjugate vaccine failure in HIV-infected child

La infección neumocócica sigue asociada con una importante mortalidad, especialmente en grupos de riesgo (esplenectomizados, déficit de la inmunidad humoral, etc.) a pesar de vacunas y antibióticos adecuados. Presentamos a un niño de 13 años VIH positivo por transmisión vertical tratado con triple terapia (amprenavir, lamivudina y zidovudina). Fue vacunado con vacuna 23-valente a los 6 años y conjugada heptavalente a los 10 años de edad. El recuento de células CD4 y su carga viral a los 6 años eran de 2.063/μl y 13.461 copias/ml, respectivamente. A los 10 años el recuento de CD4 y su carga viral eran de 1.315/μl y 32.400 copias/ml, respectivamente. El último recuento de CD4 (1.000/μl) y la carga viral (3.800 copias/ml) confirmaban un buen control de la enfermedad 15 días antes del ingreso. Acude a urgencias por fiebre, dolor abdominal y vómitos. Hay un progresivo deterioro del nivel de conciencia y signos meníngeos. En el hemocultivo y en el cultivo de LCR crece Streptococcus pneumoniae serotipo 18C, y es tratado con cefotaxima y vancomicina, así como medidas antiedema cerebral, pero evoluciona a muerte cerebral en 24 h (AU)

Despite appropriate antimicrobial therapy and vaccination, invasive pneumococcal infections remain associated with significant mortality, especially in selected high-risk groups (asplenic, humoral immunity deficient patients, etc.). We present a 13-year-old caucasian boy with HIV infection (vertical transmission). He received treatment with highly-active antiretroviral therapy (amprenavir, lamivudine and zidovudine) and vaccination with 23-valent vaccine (6 years old) and 7-valent pneumococcal conjugate vaccine (10 years old). His CD4 count and his viral load at these times were 2,063/μl and 13461 cop/ml, when he was 6 years old and 1,315/μl and 32400 cop/ml when he was 10 years old, respectively. The latest CD4 count (1,000/μl) and his viral load (3800 cop/ml) confirmed satisfactory control of the disease. He was referred to our emergency department presenting with fever, head and stomach-ache and vomiting. In the following hours his condition continued to deteriorate and depressed level of consciousness and meningismus were observed. Streptococcus pneumoniae, serotype 18 C, was detected in blood and cerebrospinal fluid cultures. Despite appropriate treatment with antibiotics (cefotaxime and vancomycin) and anti-oedema medications, brain-death was confirmed 24 hours after his admittance (AU)

Enuresis asociada a crisis de ausencia / Enuresis associated with petit mal seizures

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Ataxia cerebelosa aguda, eritema nodoso y orquiepididimitis tras gastroenteritis pro “Salmonella” D9 / Acute cerebellar ataxia, erythema nododum adn epidídimo-orchitis are complications of gastrointestinal infection by Salmonella D9

Se presenta el cado de un niño de siete años que sufre una gastroenteritis por Salmonella D9. A los quince dias de empezar la clínica, aparecen mialgias e inestabilidad en la marcha, dolor testicular y lesiones cutáneas. Fue diagnosticado de ataxia cerebelosa aguda, orquiepididimitis y eritema nodoso. Aunque todas estas complicaciones están descritas en la bibliogradía, resulta excepcional encontrarlas juntas en un mismo paciente

We present the case of a seve-year-old boy with gastroenteritis caused by Salmonella D9. Fifteen days after the clinical onset, he began to develop muscular pain, unsteady gait, testicular pain and skin lesions. The diagnoses were acute cerebellar ataxia, epididymo-orchitis and erythema nodosum. Each of these complications has been reported in the literature, but it is highly unusual to find all of them in the same patient

El síndrome de Costello a propósito de un caso clínico / Costello syndrome based on one clinical case

El síndrome de Costello ha sido descrito en 115 pacientes hasta la fecha. Reúne una serie de características fenotípicas que sugieren el diagnóstico ya que, en el momento actual, no existe ningún estudio que lo confirme. El interés de este caso radica en el reconocimiento de este síndrome debido al importante incremento de riesgo tumoral que presentan estos pacientes y a que, por tanto, precisan ser sometidos a un protocolo de despistaje de neoplastias: ecografía abdomino-pélvica desde el nacimiento y cada 3-6 meses hasta 8-10 años con objeto de detectar rabdomiosarcoma; determinación de catecolaminas en orina cada 6-12 meses y hasta los 5 años de vida para objetivar neuroblastoma y búsqueda de hematuria por su relación con la presentaciónde cáncer de vejiga (AU)

Costello Síndrome has been described in 115 patients. It consists in different clinical data that suggest the diagnosis. It is important to recognize this syndrome early because these children have an important tumoral risk so they should undergo different tests in order to find neoplasias: an abdo-pelvic ECO should be done from birth till 8-10 years of age every months to look for rhabdomyosarcoma, urinary catecholamine excretion measurements every 6-12 months up the age of 5 years to find neuroblastoma, and annual screening for hematuria from the age of ten onwards because it is related with the bladder cancer (AU)