Infectious and Bleeding Complications in Patients With Glycogenosis lb

Ambruso, Daniel R.

doi:10.1001/archpedi.1985.02140090053027

Cited by 45 publications

(44 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Before G-CSF therapy, the patient had an elevated platelet count. This is a common observation in patients with GSD lb, and the reason for this elevation is not known (12). Interestingly, G-CSF therapy resulted in a reduction of the patient's platelet count to values that stabilized within the normal range.…”

Section: Eflect Of G-csf Administration On Peripheral Blood Countsmentioning

confidence: 95%

“…Organisms that are most 202,1990) demonstrated that decreased 02-production in frequently isolated include Staphylococcus aureus, group A neutrophils was associated with impaired Ca2+ mobiliza-Streptococcus, Streptococcus pneumoniae, and Escherichia coli tion. In vivo administration of G-CSF increased f-Met- (12). Such infections usually respond poorly to treatment, and Leu-Phe-triggered Ca2+ mobilization by neutrophils to many patients require repeated, prolonged hospitalizations for 43% of control by mo 1 of G-CSF therapy and to 93% of i.v.…”

mentioning

confidence: 99%

See 1 more Smart Citation

In Vitro and In Vivo Effects of Granulocyte Colony-Stimulating Factor on Neutrophils in Glycogen Storage Disease Type IB: Granulocyte Colony-Stimulating Factor Therapy Corrects the Neutropenia and the Defects in Respiratory Burst Activity and Ca2+ Mobilization

et al. 1994

View full text Add to dashboard Cite

Section: Eflect Of G-csf Administration On Peripheral Blood Countsmentioning

confidence: 95%

mentioning

confidence: 99%

In Vitro and In Vivo Effects of Granulocyte Colony-Stimulating Factor on Neutrophils in Glycogen Storage Disease Type IB: Granulocyte Colony-Stimulating Factor Therapy Corrects the Neutropenia and the Defects in Respiratory Burst Activity and Ca2+ Mobilization

et al. 1994

View full text Add to dashboard Cite

“…Em ordem decrescente de freqüência, os principais sítios de infecção foram otite, pneumonia, infecções cutâneas (particularmente perianal), infecções do trato urinário, raramente sepse e osteomielite 36 . As infecções são predominantemente por estafilococos e alguns gram-negativos (E. coli e Pseudomonas aeruginosa).…”

Section: Glicogenose Ibunclassified

“…Vários estudos comprovaram a baixa contagem e as anormalidades da função dos neutrófilos 36,37 . As alterações dos neutrófilos incluem mobilidade e aderência reduzidas, defeitos na atividade bactericida e fagocitose, redução na produção de ânion superóxido e da atividade do shunt da hexose-monofosfato (HMP) 39 .…”

Section: Glicogenose Ibunclassified

See 1 more Smart Citation

Glycogenosis type I

Reis

Penna²,

Oliveira³

et al. 1999

J Pediatr (Rio J)

View full text Add to dashboard Cite

ResumoObjetivo: Divulgar os conhecimentos atuais acerca da glicogenose tipo I -doença de depósito de glicogênio resultante da deficiência da enzima glicose-6-fosfatase -e dotar o pediatra das informações necessárias para o diagnóstico precoce e a conduta adequada nos casos acometidos por esse distúrbio metabólico.Métodos: Foram selecionados, através da Medline, os artigos de periódicos médicos nacionais e internacionais mais relevantes dos últimos 20 anos, com ênfase especial ao tratamento dietético da glicogenose tipo I.Resultados: O metabolismo do glicogênio e as conseqüências metabólicas da glicogenose tipo I são discutidos, em especial, a hipoglicemia, o principal distúrbio metabólico da doença. São descritos os quadros clínico e laboratorial e a histopatologia.O uso do amido de milho cru e da infusão de glicose por sonda são recursos empregados para a manutenção da normoglicemia. O controle da hiperuricemia, da hiperlipidemia e da disfunção plaquetária são outros aspectos do tratamento, assim como o controle das infecções e o uso do G-CSF na glicogenose Ib. O transplante hepático e as suas principais indicações são comentados. Os adenomas hepáticos, que abrigam um potencial para transformação maligna, são resultantes do tratamento incompleto.Conclusões: Embora de ocorrência rara, a glicogenose tipo I é uma causa importante de hepatomegalia volumosa associada à hipoglicemia entre os lactentes. O tratamento dietético dessa afecção tem alterado significativamente o curso clínico e melhorado o prognóstico. Portanto, é indispensável que o pediatra geral se familiarize com o diagnóstico dessa entidade clínica para atuar com rigor no seu controle dietético.J. pediatr. (Rio J.). 1999; 75(4): 227-236: doença do armazenamento de glicogênio tipo I, hepatomegalia, hipoglicemia, glicose-6-fosfatase, amido. AbstractObjective: To o present up-to-date knowledge about Glycogen storage disease type I (GSD-type I) -a disease caused by the deposit of glycogen resulting from the deficiency of the enzyme glucose-6-phosphatase -and to provide the pediatricians with the necessary information for a precocious diagnosis and an adequate conduct for those cases where this metabolic disturbance is present.Methods: Through Medline, the most significant articles published during the last 20 years were selected from national and international journals of medicine, with special attention to dietary treatment of glycogen storage disease type I.Results: The metabolism of glycogen and the metabolic consequences of glycogen storage disease type I were discussed, especially hypoglycemia, the principal metabolic disturbance of the disease. The clinical and laboratory findings are described together with the histopathology.The use of uncooked cornstarch and enteral carbohydrate infusion are the means used for the maintenance of normoglycemia. The control of hyperuricemia, hyperlipidemia and platelet disorders are other aspects of the treatment as well as the prevention of infections and the use of G-CSF for glycogen storage type Ib. Hepatic trans...

show abstract

Portacaval Shunt as Treatment for Glycogenosis Ib

Özsoylu¹

1986

Arch Pediatr Adolesc Med

View full text Add to dashboard Cite

Infectious and Bleeding Complications in Patients With Glycogenosis lb

Cited by 45 publications

References 30 publications

In Vitro and In Vivo Effects of Granulocyte Colony-Stimulating Factor on Neutrophils in Glycogen Storage Disease Type IB: Granulocyte Colony-Stimulating Factor Therapy Corrects the Neutropenia and the Defects in Respiratory Burst Activity and Ca2+ Mobilization

In Vitro and In Vivo Effects of Granulocyte Colony-Stimulating Factor on Neutrophils in Glycogen Storage Disease Type IB: Granulocyte Colony-Stimulating Factor Therapy Corrects the Neutropenia and the Defects in Respiratory Burst Activity and Ca2+ Mobilization

Glycogenosis type I

Portacaval Shunt as Treatment for Glycogenosis Ib

Contact Info

Product

Resources

About