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)

[Amino acid changes in blood of children with severe liver disease. The evaluation of differences according to distinct physiopathology]. / Alteraciones de los aminoácidos en sangre en niños con enfermedad hepática severa. Valoración de las diferencias en relación a la distinta fisiopatología.

OBJECTIVE: The aim of this study was to know the serum amino acid profiles in children with terminal hepatic diseases and to assess the differences between the two main physiopathological groups of hepatic damage: cholestasis and cellular necrosis. PATIENTS AND METHODS: We studied twenty-six pediatric patients with severe hepatic diseases admitted to the Pediatric Intensive Care Unit. Patients were divided into two groups according to the predominant hepatic lesion: cellular damage (fourteen children) and cholestasis damage (twelve cases). RESULTS: Overall, there is a significant increase in the aromatic amino acids (AAA) phenylalanine (p < 0.04) and tyrosine (p < 0.0003) and a decrease in the branched-chain amino acids (BCAA) leucine, isoleucine and valine (p < 0.00001), with a reduction in the BCAA/AAA ratio (p < 0.00001). However, we found a significant decrease in glutamine, cysteine, taurine, serine, threonine, tryptophan, total amino acids and essential amino acids, together with higher levels of glutamic acid, ornithine and citrulline, which reflects a more complex metabolic disturbance. The group with cholestatic damage shows very low taurine levels (p < 0.0003). Patients with predominantly cellular damage have higher increases in tyrosine (p < 0.01), phenylalanine and hydroxyproline (p < 0.01). CONCLUSIONS: These findings may help us to better understand the complex physiopathology of amino acid metabolism in different liver diseases. Moreover, the extremely low levels of taurine found prompted us to recommend additional dietary support particularly in children with cholestatic hepatopathy.