Síndrome de Russel-Silver
Russel-silver syndrome
A.M. Martínez Nogueiras
, M.J. Teixeira Costeira, H.P. Saraiva Moreira, H. Araujo Antunes
Autor para correspondencia
henedina@mail.telepac.pt
Correspondencia: Avda. Fabril do Norte, 803, 7.° F. 4460-314 Senhora da Hora. Portugal.
Correspondencia: Avda. Fabril do Norte, 803, 7.° F. 4460-314 Senhora da Hora. Portugal.
Servicio de Pediatría. Hospital de São Marcos. Braga. Portugal
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"apellidos" => "Teixeira Costeira" ] 2 => array:2 [ "nombre" => "H.P." "apellidos" => "Saraiva Moreira" ] 3 => array:2 [ "nombre" => "H." "apellidos" => "Araujo Antunes" ] ] "afiliaciones" => array:1 [ 0 => array:1 [ "entidad" => "Servicio de Pediatría. Hospital de São Marcos. Braga. Portugal" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "*" "correspondencia" => "Correspondencia: Avda. Fabril do Norte, 803, 7.° F. 4460-314 Senhora da Hora. Portugal." ] ] ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Russel-silver syndrome" ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2000-11-30" "fechaAceptado" => "2001-02-28" "PalabrasClave" => array:2 [ "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec186680" "palabras" => array:4 [ 0 => "Síndrome de Russel-Silver" 1 => "Macrocefalia relativa" 2 => "Asimetría corporal cruzada" 3 => "Crecimiento intrauterino normal" ] ] ] "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key words" "identificador" => "xpalclavsec186681" "palabras" => array:4 [ 0 => "Russel-Silver syndrome" 1 => "Craniofacial disproportion" 2 => "Crossed asymmetry of the limbs" 3 => "Normal intrauterine growth" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "es" => array:1 [ "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Russel-Silver es un cuadro polimalformativo que presenta como características más significativas la existencia de retraso de crecimiento prenatal y/o posnatal, una facies típica y asimetría corporal.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El caso clínico que se describe corresponde a un lactante varón de 9 meses de edad, sin antecedentes familiares de interés, que en el momento del nacimiento presenta una somatometría adecuada a la edad gestacional (peso y estatura en el P<span class="elsevierStyleInf">25-50</span>), y macrocefalia relativa debido a desproporción craneofacial.</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">En la exploración se observa una cara pequeña de aspecto triangular, frente prominente, escleróticas azules, labios finos, comisuras bucales dirigidas hacia abajo, retromicrognatia, clinodactilia del quinto dedo de ambas manos y asimetría en la longitud y perímetro de las 4 extremidades con crecimiento a favor del brazo derecho y la pierna izquierda.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Desde el nacimiento presentó retraso de crecimiento (peso y talla inferior al P<span class="elsevierStyleInf">5</span>), y el perímetro cefálico se mantuvo en el P<span class="elsevierStyleInf">90</span>.</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">El desarrollo psicomotor (a los 9 meses), era adecuado a la edad, según la escala de Sheridan.</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Este cuadro clínico corresponde a un síndrome de Russel-Silver destacando como aspectos diferenciadores en nuestro paciente la asimetría corporal cruzada y la ausencia de retraso de crecimiento intrauterino.</p>" ] "en" => array:1 [ "resumen" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Russel-Silver syndrome is a pattern of malformations whose most characteristic features are intrauterine and postnatal growth retardation, a characteristic facial appearance and limb asymmetry. We report a 9-month-old male born at 37 weeks' gestation. Family history, pregnancy and delivery revealed no significant anomalies. The infant had no intrauterine growth retardation (birth weight and height ranging between the 25th and 50th percentile). The infant's head circumference was disproportionately large for the small facial mass. Clinical findings included a small triangular face with frontal bossing, blue sclerae, thin lips with down-turned corners, retromicrognathia, clinodactyly of the fifth finger in both hands and limb asymmetry with undergrowth of the right leg and the left arm. The patient presented postnatal growth deficiency with weight and height < 5th percentile; his head circumference grew normally along the 90th percentile. Psychomotor development was appropriate for his age (9 months) according to the Sheridan Scale. On the basis of this clinical evidence we diagnosed Russel-Silver syndrome, attributing particular relevance to the normal intrauterine growth and the crossed asymmetry of the limbs.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "Bibliografía" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:17 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Syndrome of congenital hemihypertrophy, shortness of stature, and elevated urinary gonadotrophins" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "H.K. Silver" 1 => "W. Kiyasu" 2 => "J. George" 3 => "W.C. 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Kaiser" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Human Genet" "fecha" => "1997" "volumen" => "100" "paginaInicial" => "415" "paginaFinal" => "419" ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "es" "url" => "/16954033/0000005400000006/v1_201307051558/S169540330177600X/v1_201307051558/es/main.assets" "Apartado" => array:4 [ "identificador" => "14234" "tipo" => "SECCION" "es" => array:2 [ "titulo" => "Medicina fetal y neonatología" "idiomaDefecto" => true ] "idiomaDefecto" => "es" ] "PDF" => "https://static.elsevier.es/multimedia/16954033/0000005400000006/v1_201307051558/S169540330177600X/v1_201307051558/es/main.pdf?idApp=UINPBA00005H&text.app=https://analesdepediatria.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S169540330177600X?idApp=UINPBA00005H" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 18 | 18 | 36 |
| 2024 Octubre | 148 | 64 | 212 |
| 2024 Septiembre | 137 | 76 | 213 |
| 2024 Agosto | 165 | 88 | 253 |
| 2024 Julio | 182 | 76 | 258 |
| 2024 Junio | 149 | 82 | 231 |
| 2024 Mayo | 158 | 116 | 274 |
| 2024 Abril | 172 | 61 | 233 |
| 2024 Marzo | 148 | 85 | 233 |
| 2024 Febrero | 195 | 96 | 291 |
| 2024 Enero | 200 | 71 | 271 |
| 2023 Diciembre | 264 | 54 | 318 |
| 2023 Noviembre | 211 | 65 | 276 |
| 2023 Octubre | 204 | 60 | 264 |
| 2023 Septiembre | 179 | 59 | 238 |
| 2023 Agosto | 149 | 38 | 187 |
| 2023 Julio | 183 | 55 | 238 |
| 2023 Junio | 150 | 48 | 198 |
| 2023 Mayo | 170 | 76 | 246 |
| 2023 Abril | 134 | 48 | 182 |
| 2023 Marzo | 162 | 65 | 227 |
| 2023 Febrero | 137 | 63 | 200 |
| 2023 Enero | 108 | 80 | 188 |
| 2022 Diciembre | 108 | 51 | 159 |
| 2022 Noviembre | 151 | 77 | 228 |
| 2022 Octubre | 154 | 74 | 228 |
| 2022 Septiembre | 164 | 59 | 223 |
| 2022 Agosto | 102 | 84 | 186 |
| 2022 Julio | 108 | 82 | 190 |
| 2022 Junio | 100 | 50 | 150 |
| 2022 Mayo | 135 | 94 | 229 |
| 2022 Abril | 150 | 89 | 239 |
| 2022 Marzo | 154 | 87 | 241 |
| 2022 Febrero | 193 | 66 | 259 |
| 2022 Enero | 177 | 87 | 264 |
| 2021 Diciembre | 135 | 77 | 212 |
| 2021 Noviembre | 165 | 98 | 263 |
| 2021 Octubre | 163 | 104 | 267 |
| 2021 Septiembre | 112 | 92 | 204 |
| 2021 Agosto | 101 | 69 | 170 |
| 2021 Julio | 172 | 60 | 232 |
| 2021 Junio | 146 | 64 | 210 |
| 2021 Mayo | 167 | 82 | 249 |
| 2021 Abril | 400 | 182 | 582 |
| 2021 Marzo | 317 | 82 | 399 |
| 2021 Febrero | 236 | 54 | 290 |
| 2021 Enero | 173 | 94 | 267 |
| 2020 Diciembre | 164 | 83 | 247 |
| 2020 Noviembre | 210 | 92 | 302 |
| 2020 Octubre | 181 | 96 | 277 |
| 2020 Septiembre | 204 | 101 | 305 |
| 2020 Agosto | 232 | 104 | 336 |
| 2020 Julio | 228 | 122 | 350 |
| 2020 Junio | 220 | 105 | 325 |
| 2020 Mayo | 197 | 116 | 313 |
| 2020 Abril | 206 | 76 | 282 |
| 2020 Marzo | 259 | 141 | 400 |
| 2020 Febrero | 444 | 152 | 596 |
| 2020 Enero | 377 | 122 | 499 |
| 2019 Diciembre | 345 | 215 | 560 |
| 2019 Noviembre | 354 | 221 | 575 |
| 2019 Octubre | 365 | 261 | 626 |
| 2019 Septiembre | 338 | 228 | 566 |
| 2019 Agosto | 207 | 195 | 402 |
| 2019 Julio | 239 | 217 | 456 |
| 2019 Junio | 322 | 142 | 464 |
| 2019 Mayo | 348 | 270 | 618 |
| 2019 Abril | 293 | 332 | 625 |
| 2019 Marzo | 282 | 184 | 466 |
| 2019 Febrero | 239 | 92 | 331 |
| 2019 Enero | 185 | 112 | 297 |
| 2018 Diciembre | 162 | 66 | 228 |
| 2018 Noviembre | 279 | 108 | 387 |
| 2018 Octubre | 226 | 93 | 319 |
| 2018 Septiembre | 154 | 62 | 216 |
| 2018 Agosto | 1 | 0 | 1 |
| 2018 Julio | 4 | 0 | 4 |
| 2018 Junio | 1 | 0 | 1 |
| 2018 Mayo | 9 | 0 | 9 |
| 2018 Abril | 30 | 0 | 30 |
| 2018 Marzo | 36 | 0 | 36 |
| 2018 Febrero | 30 | 2 | 32 |
| 2018 Enero | 60 | 0 | 60 |
| 2017 Diciembre | 35 | 0 | 35 |
| 2017 Noviembre | 62 | 0 | 62 |
| 2017 Octubre | 74 | 1 | 75 |
| 2017 Septiembre | 67 | 0 | 67 |
| 2017 Agosto | 29 | 0 | 29 |
| 2017 Julio | 63 | 7 | 70 |
| 2017 Junio | 74 | 83 | 157 |
| 2017 Mayo | 62 | 122 | 184 |
| 2017 Abril | 50 | 85 | 135 |
| 2017 Marzo | 91 | 105 | 196 |
| 2017 Febrero | 166 | 78 | 244 |
| 2017 Enero | 122 | 19 | 141 |
| 2016 Diciembre | 85 | 57 | 142 |
| 2016 Noviembre | 192 | 74 | 266 |
| 2016 Octubre | 156 | 74 | 230 |
| 2016 Septiembre | 132 | 80 | 212 |
| 2016 Agosto | 87 | 47 | 134 |
| 2016 Julio | 32 | 27 | 59 |
| 2016 Junio | 4 | 0 | 4 |
| 2016 Mayo | 5 | 0 | 5 |
| 2016 Abril | 3 | 0 | 3 |
| 2016 Marzo | 9 | 0 | 9 |
| 2016 Febrero | 7 | 0 | 7 |
| 2016 Enero | 7 | 0 | 7 |
| 2015 Diciembre | 11 | 0 | 11 |
| 2015 Noviembre | 4 | 0 | 4 |
| 2015 Octubre | 8 | 94 | 102 |
| 2015 Septiembre | 4 | 73 | 77 |
| 2015 Agosto | 2 | 0 | 2 |
| 2015 Julio | 14 | 0 | 14 |
| 2015 Junio | 13 | 19 | 32 |
| 2015 Mayo | 17 | 0 | 17 |
| 2015 Abril | 13 | 42 | 55 |
| 2015 Marzo | 7 | 0 | 7 |
| 2015 Febrero | 16 | 7 | 23 |
| 2015 Enero | 18 | 2 | 20 |
| 2014 Diciembre | 29 | 3 | 32 |
| 2014 Noviembre | 13 | 1 | 14 |
| 2014 Octubre | 33 | 1 | 34 |
| 2014 Septiembre | 24 | 5 | 29 |
| 2014 Agosto | 36 | 3 | 39 |
| 2014 Julio | 40 | 1 | 41 |
| 2014 Junio | 148 | 2 | 150 |
| 2014 Mayo | 595 | 8 | 603 |
| 2014 Abril | 545 | 5 | 550 |
| 2014 Marzo | 606 | 8 | 614 |
| 2014 Febrero | 456 | 10 | 466 |
| 2014 Enero | 444 | 9 | 453 |
| 2013 Diciembre | 255 | 8 | 263 |
| 2013 Noviembre | 385 | 13 | 398 |
| 2013 Octubre | 400 | 17 | 417 |
| 2013 Septiembre | 191 | 15 | 206 |
| 2013 Agosto | 266 | 27 | 293 |
| 2013 Julio | 120 | 11 | 131 |
| 2013 Junio | 4 | 1 | 5 |
| 2013 Mayo | 9 | 3 | 12 |
| 2013 Abril | 4 | 4 | 8 |
| 2013 Marzo | 11 | 1 | 12 |
| 2013 Febrero | 47 | 6 | 53 |
| 2013 Enero | 62 | 1 | 63 |
| 2012 Diciembre | 75 | 4 | 79 |
| 2012 Noviembre | 58 | 3 | 61 |
| 2012 Octubre | 81 | 2 | 83 |
| 2012 Septiembre | 64 | 0 | 64 |
| 2012 Agosto | 13 | 1 | 14 |
| 2001 Mayo | 12000 | 0 | 12000 |
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