Increased risk of group B streptococcal sepsis and meningitis in HIV-exposed uninfected infants in a high-income country.

The purpose of this study is to compare group B Streptococcus (GBS) infection incidence in HIV-exposed uninfected (HEU) and HIV-unexposed (HU) infants in a Spanish cohort. We conducted a retrospective study in 5 hospitals in Madrid (Spain). Infants ≤ 90 days of life with a GBS infection were included from January 2008 to December 2017. Incidence of GBS infection in HEU and HU children was compared. HEU infants presented a sevenfold greater risk of GBS infection and a 29-fold greater risk of GBS meningitis compared to HU, with statistical significance. Early-onset infection was tenfold more frequent in HEU children, with statistical significance, and late-onset infection was almost fivefold more frequent in the HUE infants' group, without statistical significance. CONCLUSION: HEU infants presented an increased risk of GBS sepsis and meningitis. One in each 500 HEU infants of our cohort had a central nervous system infection and 1 in each 200, a GBS infection. Although etiological causes are not well understood, this should be taken into account by physicians when attending this population. WHAT IS KNOWN: • HIV-exposed uninfected infants are at higher risk of severe infections. • An increased susceptibility of these infants to group B Streptococcus infections has been described in low- and high-income countries, including a higher risk of meningitis in a South African cohort. WHAT IS NEW: • Group B Streptococcal meningitis is more frequent in HIV-exposed uninfected infants also in high-income countries. • Physicians should be aware of this increased risk when attending these infants.

Actualización en discinesia ciliar primaria: manejo actual y perspectivas de futuro / Primary cliary dyskinesia: current management and future perspectives

La discinesia ciliar primaria (DCP) es una enfermedad rara, heterogénea desde el punto de vista clínico, que se transmite con una herencia principalmente autosómica recesiva y se engloba dentro de los trastornos de la motilidad ciliar. Es causa de patología tanto a nivel de las vías respiratorias superiores como inferiores -que comienza en el primer año de vida y evoluciona a un daño pulmonar progresivo-, infertilidad y alteraciones de la posición de los órganos internos. El propósito de esta revisión es proporcionar una actualización sobre las manifestaciones clínicas, diagnóstico y tratamiento de la DCP para que exista un mayor nivel de alerta sobre esta enfermedad e incidir en la necesidad de crear registros de pacientes que nos hagan entender mejor la fisiopatología y progresión de la enfermedad (AU)

Primary ciliary dyskinesia (PCD) is a rare and heterogeneous from the clinical point of view disease, mostly autosomal recessive inherited, characterized by motile ciliary dysfunction. It causes chronic upper and lower respiratory tract disease, starting in the first year of life and resulting in progressive lung damage, infertility and situs anomalies. The aim of this review is to provide an update on the diagnosis and treatment of PCD to increase the clinicia's awareness of this disorder and high-light the need to create patient registries that give us a better understanding of the physiopathology and disease's progression

Estudio descriptivo de los casos de paludismo en la población pediátrica en un hospital general de Madrid entre 1996 y 2011 / Descriptive study of malaria cases in a general hospital in Madrid between 1996 and 2011

INTRODUCCIÓN: La malaria causa la muerte de unas 863.000 personas al año, la mayoría menores de 5 años. MATERIAL Y MÉTODOS: Se realizó un estudio retrospectivo-descriptivo de los casos diagnosticados desde 1996 hasta 2011 en el Hospital Príncipe de Asturias de Madrid. RESULTADOS: En el período estudiado se diagnosticaron 103 casos en menores de 14 años. El 60,2% de los pacientes fueron varones y la mediana de edad 4,5 años. La mayoría de los pacientes eran inmigrantes africanos recién llegados o inmigrantes residentes en España que viajaron para visitar familiares sin realizar profilaxis antipalúdica. El 25% de los casos fueron diagnosticados de malaria complicada, siendo el criterio principal la hiperparasitemia. De los pacientes que solo cumplían ese criterio, el 80% no presentó complicaciones. CONCLUSIONES: Es importante mantener un elevado nivel de sospecha en pacientes que proceden de zonas endémicas. El principal factor responsable de la infección fue la falta de quimioprofilaxis

INTRODUCTION: Malaria causes around 863,000 deaths per year, mostly of them in children under 5 years old. MATERIAL AND METHODS: We have reviewed the epidemiological data of malaria cases in a pediatric department in a Hospital in the Community of Madrid, in the period 1996-2011. RESULTS: In the period reviewed, 103 cases of malaria were diagnosed in children under 14 years old. Sixty percent were males and the average age was 4.5 years. In most cases, the infection arose during a visit to relatives in the country of origin. The vast majority did not have malaria prophylaxis. Twenty-five percent of the cases were diagnosed as complicated malaria, the main criteria being hyperparasitemia, of which 80% of the patients did not present any other complications CONCLUSIONS: A high level of suspicion must be maintained in any patient who comes from a malaria endemic area. The key factor responsible for the infection was the lack of chemoprophylaxis

[Descriptive study of malaria cases in a general hospital in Madrid between 1996 and 2011]. / Estudio descriptivo de los casos de paludismo en la población pediátrica en un hospital general de Madrid entre 1996 y 2011.

INTRODUCTION: Malaria causes around 863,000 deaths per year, mostly of them in children under 5 years old. MATERIAL AND METHODS: We have reviewed the epidemiological data of malaria cases in a pediatric department in a Hospital in the Community of Madrid, in the period 1996-2011. RESULTS: In the period reviewed, 103 cases of malaria were diagnosed in children under 14 years old. Sixty percent were males and the average age was 4.5 years. In most cases, the infection arose during a visit to relatives in the country of origin. The vast majority did not have malaria prophylaxis. Twenty-five percent of the cases were diagnosed as complicated malaria, the main criteria being hyperparasitemia, of which 80% of the patients did not present any other complications CONCLUSIONS: A high level of suspicion must be maintained in any patient who comes from a malaria endemic area. The key factor responsible for the infection was the lack of chemoprophylaxis.

Immunohistochemical alterations in invasive adenocarcinoma in endoscopically resected adenoma and factors associated with risk of residual or recurrent disease.

AIM: We determined the pattern of immunohistochemical expression in invasive adenocarcinoma in endoscopically resected adenoma, its relationship with the risk of residual or recurrent disease and the related factors. METHOD: We included individuals with malignant polyps resected endoscopically in the period 1999-2009. Clinical and endoscopic data were collected. All histological specimens were re-analysed. CD44, matrix metalloproteinase 9 (MMP-9), vascular endothelial growth factor-ß (VEGF-ß), ß-catenin, laminin and cyclooxygenase 2 (COX-2) expression were determined by immunohistochemistry. A multivariate logistic regression was performed to determine variables independently associated with the risk of residual or recurrent disease. RESULTS: One-hundred and fifty-one malignant polyps (114 pedunculated; mean size ± SD=22.61 ± 10.86 mm) were resected endoscopically. Resection was fragmented and incomplete in 26.5% and 8.6% of patients, respectively. Surgical resection was performed on 71 (47%) patients. After a median follow-up of 44 months, residual (n=12) or recurrent (n=6) disease was detected in 17 patients. Conventional histology showed that 32.1% met high-risk histological criteria. Immunohistochemical expression was positive for CD44, MMP-9, VEGF-ß, ß-catenin, laminin and COX-2 in 63.3%, 25.3%, 45%, 38.8%, 79% and 34.5% of specimens, respectively, with no differences between both groups. Variables associated with residual or recurrent disease in the univariate analysis were: nonpedunculated morphology (P=0.07); fragmented (P<0.001) or incomplete resection (P<0.001); margin infiltration (P=0.04); and histological high-risk lesion (P=0.003). Finally, incomplete resection (OR=12.16, 95% CI=3.15-46.98; P<0.001) and histological high risk (OR=4.73, 95% CI=1.33-16.74; P=0.002) were independently associated with the risk of residual or recurrent disease. CONCLUSION: Immunohistochemistry could not predict residual or recurrent disease. Only incomplete excision and histological high risk did so. The factors independently associated were histological high-risk lesion and incomplete resection.

A retrospective study of allergic diseases in children with food hypersensitivity

No disponible

No disponible

Prevalence of mutations in TSHR, GNAS, PRKAR1A and RAS genes in a large series of toxic thyroid adenomas from Galicia, an iodine-deficient area in NW Spain.

OBJECTIVE: Toxic thyroid adenoma (TA) is a common cause of hyperthyroidism. Mutations in the TSH receptor (TSHR) gene, and less frequently in the adenylate cyclase-stimulating G alpha protein (GNAS) gene, are well established causes of TA in Europe. However, genetic causes of TA remain unknown in a small percentage of cases. We report the first study to investigate mutations in TSHR, GNAS, protein kinase, cAMP-dependent, regulatory, type I alpha (PRKAR1A) and RAS genes, in a large series of TA from Galicia, an iodine-deficient region in NW Spain. DESIGN AND METHODS: Eighty-five TA samples were obtained surgically from 77 hyperthyroid patients, operated on for treatment of non-autoimmune toxic nodular goitre. After DNA extraction, all coding exons of TSHR, GNAS and PRKAR1A genes, and exons 2 and 3 of HRAS, KRAS and NRAS were amplified by PCR and sequenced. Previously unreported mutants were cloned in expression vectors and their basal constitutive activities were determined by quantification of cAMP response element (CRE)-luciferase activity in CO7 cells transfected with wild-type and mutant plasmids. RESULTS: TSHR gene mutations were found in 52 (61.2%) samples, GNAS gene mutations in 4 (4.71%) samples and no PRKAR1A or RAS mutations were found. Only three previously unreported mutations were found, two affecting the TSHR, A623F and I635V, and one affecting the G-protein alpha-subunit (Gsalpha), L203P. All mutant proteins showed higher CRE-luciferase activity than their wild-type counterparts. CONCLUSIONS: TA in a hyperthyroid population living in Galicia, a Spanish iodine-deficient region, harbours elevated frequencies of TSHR and GNAS mutations activating the cAMP pathway. However, the genetic cause of TA was undetermined in 34% of the TA samples.

[Heat-moulded plastic splint for the nose]. / Attelle plastique thermoformable de contention nasale.

The authors present a very simple and very comfortable splinting device for use after rhinoplasty. It consists of a splint made from malleable plastic which can be moulded by soaking in water at 60 degrees C, allowing perfect modelling to the shape of the nose. This splint is held in place by means of Velcro bands which offer remarkable flexibility of use.