Sindrom Nezelof

Siniţîna Lilia; Petrovici Virgil; Tomacinschi Cristina; Şciuca Svetlana

Conţinutul numărului revistei

Articolul precedent

Articolul urmator

913

Ultima descărcare din IBN:
2022-02-14 16:20

Căutarea după subiecte
similare conform CZU

616.15-008.83-06-053.1 (1)

Патология сердечно-сосудистой системы. Сердечно-сосудистые заболевания (975)

SM ISO690:2012

SINIŢÎNA, Lilia, PETROVICI, Virgil, TOMACINSCHI, Cristina, ŞCIUCA, Svetlana. Sindrom Nezelof. In: Arta Medica , 2017, nr. 4(65), pp. 25-27. ISSN 1810-1852.

EXPORT metadate:
Google Scholar
Crossref
CERIF

DataCite
Dublin Core

Arta Medica

Numărul 4(65) / 2017 / ISSN 1810-1852 /ISSNe 1810-1879

Sindrom Nezelof

Nezelof syndrome

CZU: 616.15-008.83-06-053.1

Pag. 25-27

Siniţîna Lilia¹, Petrovici Virgil¹, Tomacinschi Cristina², Şciuca Svetlana²

¹ IMSP Institutul Mamei şi Copiluluii,
² Universitatea de Stat de Medicină şi Farmacie „Nicolae Testemiţanu“

Disponibil în IBN: 25 ianuarie 2018

Descarcă PDF

Rezumat

Sindromul Nezelof reprezintă o imunodeficiență primară severă caracterizată prin alimfocitoză și disginezie a sistemului limfocitar cu displazie morfostructurală a timusului. Acest sindrom este descris de către Biroul de boli rare drept o ”boală rară”, avand o frecvență de 1:20.000 - 1:50.000 nou-născuți. Din cauza imunodeficienței severe, acești pacienți fac infecții la nivelul tuturor organelor care se complică foarte ușor, soldandu-se cu decesul pacienților la varstă fragedă. In acest articol ne propunem să prezentăm 4 pacienți diagnosticați cu sindrom Nezelof post-mortem și modificările morfopatologice determinate.

Nezelof syndrome represents a severe primary immunodeficiency characterized by alimphocytosis and dysgenezia of the lymphatic system with morphostructural dysplasia of the thymus. This syndrome is described by office of the rare disease as a "rare disease", with a frequency of 1:20,000 - 1:50,000 newborns. Due to severe immunodeficiency, these patients make infections in all the organs that are easily complicated, resulting in the death of patients at early-stage. In this article we propose to present 4 patients diagnosed with post-mortem Nezelof syndrome and the morphopathological changes determined.

Cerif XML Export

<?xml version='1.0' encoding='utf-8'?>
<CERIF xmlns='urn:xmlns:org:eurocris:cerif-1.5-1' xsi:schemaLocation='urn:xmlns:org:eurocris:cerif-1.5-1 http://www.eurocris.org/Uploads/Web%20pages/CERIF-1.5/CERIF_1.5_1.xsd' xmlns:xsi='http://www.w3.org/2001/XMLSchema-instance' release='1.5' date='2012-10-07' sourceDatabase='Output Profile'>
<cfResPubl>
<cfResPublId>ibn-ResPubl-58800</cfResPublId>
<cfResPublDate>2017-12-21</cfResPublDate>
<cfVol>65</cfVol>
<cfIssue>4</cfIssue>
<cfStartPage>25</cfStartPage>
<cfISSN>1810-1852</cfISSN>
<cfURI>https://ibn.idsi.md/ro/vizualizare_articol/58800</cfURI>
<cfTitle cfLangCode='RO' cfTrans='o'>Sindrom Nezelof</cfTitle>
<cfAbstr cfLangCode='RO' cfTrans='o'><p>Sindromul Nezelof reprezintă o imunodeficiență primară severă caracterizată prin alimfocitoză și disginezie a sistemului limfocitar cu displazie morfostructurală a timusului. Acest sindrom este descris de către Biroul de boli rare drept o &rdquo;boală rară&rdquo;, avand o frecvență de 1:20.000 - 1:50.000 nou-născuți. Din cauza imunodeficienței severe, acești pacienți fac infecții la nivelul tuturor organelor care se complică foarte ușor, soldandu-se cu decesul pacienților la varstă fragedă. In acest articol ne propunem să prezentăm 4 pacienți diagnosticați cu sindrom Nezelof post-mortem și modificările morfopatologice determinate.</p></cfAbstr>
<cfAbstr cfLangCode='EN' cfTrans='o'><p>Nezelof syndrome represents a severe primary immunodeficiency characterized by alimphocytosis and dysgenezia of the lymphatic system with morphostructural dysplasia of the thymus. This syndrome is described by office of the rare disease as a &quot;rare disease&quot;, with a frequency of 1:20,000 - 1:50,000 newborns. Due to severe immunodeficiency, these patients make infections in all the organs that are easily complicated, resulting in the death of patients at early-stage. In this article we propose to present 4 patients diagnosed with post-mortem Nezelof syndrome and the morphopathological changes determined.</p></cfAbstr>
<cfResPubl_Class>
<cfClassId>eda2d9e9-34c5-11e1-b86c-0800200c9a66</cfClassId>
<cfClassSchemeId>759af938-34ae-11e1-b86c-0800200c9a66</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
</cfResPubl_Class>
<cfResPubl_Class>
<cfClassId>e601872f-4b7e-4d88-929f-7df027b226c9</cfClassId>
<cfClassSchemeId>40e90e2f-446d-460a-98e5-5dce57550c48</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
</cfResPubl_Class>
<cfPers_ResPubl>
<cfPersId>ibn-person-17504</cfPersId>
<cfClassId>49815870-1cfe-11e1-8bc2-0800200c9a66</cfClassId>
<cfClassSchemeId>b7135ad0-1d00-11e1-8bc2-0800200c9a66</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
</cfPers_ResPubl>
<cfPers_ResPubl>
<cfPersId>ibn-person-33255</cfPersId>
<cfClassId>49815870-1cfe-11e1-8bc2-0800200c9a66</cfClassId>
<cfClassSchemeId>b7135ad0-1d00-11e1-8bc2-0800200c9a66</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
</cfPers_ResPubl>
<cfPers_ResPubl>
<cfPersId>ibn-person-55290</cfPersId>
<cfClassId>49815870-1cfe-11e1-8bc2-0800200c9a66</cfClassId>
<cfClassSchemeId>b7135ad0-1d00-11e1-8bc2-0800200c9a66</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
</cfPers_ResPubl>
<cfPers_ResPubl>
<cfPersId>ibn-person-11781</cfPersId>
<cfClassId>49815870-1cfe-11e1-8bc2-0800200c9a66</cfClassId>
<cfClassSchemeId>b7135ad0-1d00-11e1-8bc2-0800200c9a66</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
</cfPers_ResPubl>
</cfResPubl>
<cfPers>
<cfPersId>ibn-Pers-17504</cfPersId>
<cfPersName_Pers>
<cfPersNameId>ibn-PersName-17504-2</cfPersNameId>
<cfClassId>55f90543-d631-42eb-8d47-d8d9266cbb26</cfClassId>
<cfClassSchemeId>7375609d-cfa6-45ce-a803-75de69abe21f</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
<cfFamilyNames>Siniţîna</cfFamilyNames>
<cfFirstNames>Lilia</cfFirstNames>
</cfPersName_Pers>
</cfPers>
<cfPers>
<cfPersId>ibn-Pers-33255</cfPersId>
<cfPersName_Pers>
<cfPersNameId>ibn-PersName-33255-2</cfPersNameId>
<cfClassId>55f90543-d631-42eb-8d47-d8d9266cbb26</cfClassId>
<cfClassSchemeId>7375609d-cfa6-45ce-a803-75de69abe21f</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
<cfFamilyNames>Petrovich</cfFamilyNames>
<cfFirstNames>Virgil</cfFirstNames>
<cfFamilyNames>Петрович</cfFamilyNames>
<cfFirstNames>Виржил</cfFirstNames>
</cfPersName_Pers>
</cfPers>
<cfPers>
<cfPersId>ibn-Pers-55290</cfPersId>
<cfPersName_Pers>
<cfPersNameId>ibn-PersName-55290-2</cfPersNameId>
<cfClassId>55f90543-d631-42eb-8d47-d8d9266cbb26</cfClassId>
<cfClassSchemeId>7375609d-cfa6-45ce-a803-75de69abe21f</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
<cfFamilyNames>Tomacinschi</cfFamilyNames>
<cfFirstNames>Cristina</cfFirstNames>
</cfPersName_Pers>
</cfPers>
<cfPers>
<cfPersId>ibn-Pers-11781</cfPersId>
<cfPersName_Pers>
<cfPersNameId>ibn-PersName-11781-2</cfPersNameId>
<cfClassId>55f90543-d631-42eb-8d47-d8d9266cbb26</cfClassId>
<cfClassSchemeId>7375609d-cfa6-45ce-a803-75de69abe21f</cfClassSchemeId>
<cfStartDate>2017-12-21T24:00:00</cfStartDate>
<cfFamilyNames>Щука</cfFamilyNames>
<cfFirstNames>Светлана</cfFirstNames>
</cfPersName_Pers>
</cfPers>
</CERIF>