skip to content
Covid-19 virus
COVID-19 Resources

Reliable information about the coronavirus (COVID-19) is available from the World Health Organization (current situation, international travel). Numerous and frequently-updated resource results are available from this WorldCat.org search. OCLC’s WebJunction has pulled together information and resources to assist library staff as they consider how to handle coronavirus issues in their communities.

Image provided by: CDC/ Alissa Eckert, MS; Dan Higgins, MAM
Cistinose nefropatica: relato de 2 casos e revisao da literatura.
ClosePreview this item
Checking...

Cistinose nefropatica: relato de 2 casos e revisao da literatura.

Author: M KnoepfelmacherR RochaL R SalgadoM SemerD VossAll authors
Edition/Format:   Article : Portuguese
Publication:Revista da Associação Médica Brasileira (1992) Vol. 40, no. 1 (jan.-mar. 1994) p.43-6
Summary:
Os autores descrevem dois casos de cistinose nefropatica, da forma infantil, que se apresentaram com sindrome de Fanconi e diabetes insipidus nefrogenico. Apos a confirmacao diagnostica atraves da identificacao de cristais de cistina pelo exame de lampada de fenda e aspirado de medula ossea, os pacientes receberam tratamento convencional de reposicao das perdas renais de eletrolitos e minerais. O paciente 1 evoluiu  Read more...
Rating:

(not yet rated) 0 with reviews - Be the first.

Subjects
More like this
&AllPage.SpinnerRetrieving;

Find a copy in the library

&AllPage.SpinnerRetrieving; Finding libraries that hold this item...

Details

Genre/Form: REVISAO TUTORIAL
Relatos de Casos
Document Type: Article
All Authors / Contributors: M Knoepfelmacher; R Rocha; L R Salgado; M Semer; D Voss; B L Wajchenberg; B Liberman
OCLC Number: 69801556

Abstract:

Os autores descrevem dois casos de cistinose nefropatica, da forma infantil, que se apresentaram com sindrome de Fanconi e diabetes insipidus nefrogenico. Apos a confirmacao diagnostica atraves da identificacao de cristais de cistina pelo exame de lampada de fenda e aspirado de medula ossea, os pacientes receberam tratamento convencional de reposicao das perdas renais de eletrolitos e minerais. O paciente 1 evoluiu para obito apos quadro de broncopneumonia. No paciente 2 foi iniciado tratamento com cisteamina, que e eficaz na deplecao de cistina dos tecidos, na dose media de 50 mg/kg/dia, sendo este o primeiro caso de terapeutica com cisteamina em nosso meio. (AU).

Reviews

User-contributed reviews
Retrieving GoodReads reviews...
Retrieving DOGObooks reviews...

Tags

Be the first.
Confirm this request

You may have already requested this item. Please select Ok if you would like to proceed with this request anyway.

Linked Data


Primary Entity<\/h3>
<http:\/\/www.worldcat.org\/oclc\/69801556<\/a>> # Cistinose nefropatica: relato de 2 casos e revisao da literatura.<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Article<\/a>, schema:CreativeWork<\/a> ;\u00A0\u00A0\u00A0library:oclcnum<\/a> \"69801556<\/span>\" ;\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/diabetes_insipido_compl<\/a>> ; # Diabetes insipido--Compl<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/adolescente<\/a>> ; # ADOLESCENTE<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cistinose_quimioter<\/a>> ; # Cistinose--Quimioter<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/diabetes_insipido_diag<\/a>> ; # Diabetes insipido--Diag<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/pre_escolar<\/a>> ; # PRE-ESCOLAR<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/sindrome_de_fanconi_compl<\/a>> ; # Sindrome de fanconi--Compl<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cisteamina_uso_terap<\/a>> ; # Cisteamina--Uso terap<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cistinose_compl<\/a>> ; # Cistinose--Compl<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/fatal_outcome<\/a>> ; # Fatal outcome<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/sindrome_de_fanconi_diag<\/a>> ; # Sindrome de fanconi--Diag<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/humano<\/a>> ; # HUMANO<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/masculino<\/a>> ; # MASCULINO<\/span>\u00A0\u00A0\u00A0schema:about<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cistinose_diag<\/a>> ; # Cistinose--Diag<\/span>\u00A0\u00A0\u00A0schema:contributor<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/wajchenberg_b_l<\/a>> ; # B. L. Wajchenberg<\/span>\u00A0\u00A0\u00A0schema:contributor<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/voss_d<\/a>> ; # D. Voss<\/span>\u00A0\u00A0\u00A0schema:contributor<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/liberman_b<\/a>> ; # B. Liberman<\/span>\u00A0\u00A0\u00A0schema:contributor<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/salgado_l_r<\/a>> ; # L. R. Salgado<\/span>\u00A0\u00A0\u00A0schema:contributor<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/semer_m<\/a>> ; # M. Semer<\/span>\u00A0\u00A0\u00A0schema:contributor<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/rocha_r<\/a>> ; # R. Rocha<\/span>\u00A0\u00A0\u00A0schema:creator<\/a> <http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/knoepfelmacher_m<\/a>> ; # M. Knoepfelmacher<\/span>\u00A0\u00A0\u00A0schema:datePublished<\/a> \"1994<\/span>\" ;\u00A0\u00A0\u00A0schema:description<\/a> \"Os autores descrevem dois casos de cistinose nefropatica, da forma infantil, que se apresentaram com sindrome de Fanconi e diabetes insipidus nefrogenico. Apos a confirmacao diagnostica atraves da identificacao de cristais de cistina pelo exame de lampada de fenda e aspirado de medula ossea, os pacientes receberam tratamento convencional de reposicao das perdas renais de eletrolitos e minerais. O paciente 1 evoluiu para obito apos quadro de broncopneumonia. No paciente 2 foi iniciado tratamento com cisteamina, que e eficaz na deplecao de cistina dos tecidos, na dose media de 50 mg\/kg\/dia, sendo este o primeiro caso de terapeutica com cisteamina em nosso meio. (AU).<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0schema:exampleOfWork<\/a> <http:\/\/worldcat.org\/entity\/work\/id\/53385082<\/a>> ;\u00A0\u00A0\u00A0schema:genre<\/a> \"Relatos de Casos<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0schema:genre<\/a> \"REVISAO TUTORIAL<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0schema:inLanguage<\/a> \"pt<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"Cistinose nefropatica: relato de 2 casos e revisao da literatura.<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0schema:productID<\/a> \"69801556<\/span>\" ;\u00A0\u00A0\u00A0wdrs:describedby<\/a> <http:\/\/www.worldcat.org\/title\/-\/oclc\/69801556<\/a>> ;\u00A0\u00A0\u00A0\u00A0.<\/div>

Related Entities<\/h3>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/knoepfelmacher_m<\/a>> # M. Knoepfelmacher<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Person<\/a> ;\u00A0\u00A0\u00A0schema:familyName<\/a> \"Knoepfelmacher<\/span>\" ;\u00A0\u00A0\u00A0schema:givenName<\/a> \"M.<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"M. Knoepfelmacher<\/span>\" ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/liberman_b<\/a>> # B. Liberman<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Person<\/a> ;\u00A0\u00A0\u00A0schema:familyName<\/a> \"Liberman<\/span>\" ;\u00A0\u00A0\u00A0schema:givenName<\/a> \"B.<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"B. Liberman<\/span>\" ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/rocha_r<\/a>> # R. Rocha<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Person<\/a> ;\u00A0\u00A0\u00A0schema:familyName<\/a> \"Rocha<\/span>\" ;\u00A0\u00A0\u00A0schema:givenName<\/a> \"R.<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"R. Rocha<\/span>\" ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/salgado_l_r<\/a>> # L. R. Salgado<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Person<\/a> ;\u00A0\u00A0\u00A0schema:familyName<\/a> \"Salgado<\/span>\" ;\u00A0\u00A0\u00A0schema:givenName<\/a> \"L. R.<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"L. R. Salgado<\/span>\" ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/semer_m<\/a>> # M. Semer<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Person<\/a> ;\u00A0\u00A0\u00A0schema:familyName<\/a> \"Semer<\/span>\" ;\u00A0\u00A0\u00A0schema:givenName<\/a> \"M.<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"M. Semer<\/span>\" ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/voss_d<\/a>> # D. Voss<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Person<\/a> ;\u00A0\u00A0\u00A0schema:familyName<\/a> \"Voss<\/span>\" ;\u00A0\u00A0\u00A0schema:givenName<\/a> \"D.<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"D. Voss<\/span>\" ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Person\/wajchenberg_b_l<\/a>> # B. L. Wajchenberg<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Person<\/a> ;\u00A0\u00A0\u00A0schema:familyName<\/a> \"Wajchenberg<\/span>\" ;\u00A0\u00A0\u00A0schema:givenName<\/a> \"B. L.<\/span>\" ;\u00A0\u00A0\u00A0schema:name<\/a> \"B. L. Wajchenberg<\/span>\" ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/adolescente<\/a>> # ADOLESCENTE<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"ADOLESCENTE<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cisteamina_uso_terap<\/a>> # Cisteamina--Uso terap<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Cisteamina--Uso terap<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cistinose_compl<\/a>> # Cistinose--Compl<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Cistinose--Compl<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cistinose_diag<\/a>> # Cistinose--Diag<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Cistinose--Diag<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/cistinose_quimioter<\/a>> # Cistinose--Quimioter<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Cistinose--Quimioter<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/diabetes_insipido_compl<\/a>> # Diabetes insipido--Compl<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Diabetes insipido--Compl<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/diabetes_insipido_diag<\/a>> # Diabetes insipido--Diag<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Diabetes insipido--Diag<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/fatal_outcome<\/a>> # Fatal outcome<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Fatal outcome<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/humano<\/a>> # HUMANO<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"HUMANO<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/masculino<\/a>> # MASCULINO<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"MASCULINO<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/pre_escolar<\/a>> # PRE-ESCOLAR<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"PRE-ESCOLAR<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/sindrome_de_fanconi_compl<\/a>> # Sindrome de fanconi--Compl<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Sindrome de fanconi--Compl<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>
<http:\/\/experiment.worldcat.org\/entity\/work\/data\/53385082#Topic\/sindrome_de_fanconi_diag<\/a>> # Sindrome de fanconi--Diag<\/span>\u00A0\u00A0\u00A0\u00A0a schema:Intangible<\/a> ;\u00A0\u00A0\u00A0schema:name<\/a> \"Sindrome de fanconi--Diag<\/span>\"@pt<\/a> ;\u00A0\u00A0\u00A0\u00A0.<\/div>