Sensitive Trypsin Assay for Dried-Blood Specimens as a Screening Procedure for Early Detection of Cystic Fibrosis

King, David; Walsh, Micheal; Heeley, A F; Kuzemko, J A

doi:10.1016/s0140-6736(79)92336-5

Cited by 49 publications

(14 citation statements)

References 2 publications

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“…o aumento do tripsinogênio no sangue foi observado pela primeira vez por Crossley 35 e confirmado por outros autores 36,38 . Admite-se que o aumento da tripsina seja secundário ao refluxo de secre-ção pancreática, provocado pela obstrução dos dutos pancreáticos.…”

Section: Tripsina Imunorreativa (Tir)unclassified

Controvérsias na fibrose cística: do pediatra ao especialista

Ribeiro¹,

Ribeiro²,

Ribeiro³

2002

J. Pediatr. (Rio J.)

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ResumoObjetivo: nos últimos 70 anos, a fibrose cística emergiu da obscuridade para o reconhecimento como a mais importante doença hereditária, potencialmente letal, incidente na raça branca. Embora seja uma doença genética, na qual o defeito básico acomete células de vários órgãos, nem todos os indivíduos expressam respostas clínicas na mesma intensidade. Várias manifestações clínicas, principalmente pulmonares e digestivas, podem ocorrer durante a vida dos pacientes fibrocísticos. O objetivo deste artigo é propiciar ao pediatra geral uma visão atualizada dos principais assuntos referentes à fibrose cística. Fontes dos dados:revisão sistemática e atualizada em fonte de dados oficial (Medline). Síntese dos dados:foram revisados 79 artigos sobre fibrose cística, de periódicos internacionais, colocando, de modo atual e crítico, os principais eventos relacionados com a incidência, a fisiopatogenia, as manifestações clínicas, o diagnóstico e o tratamento da fibrose cística.Conclusões: apesar de não existir cura para essa doença, muitos conhecimentos novos sobre a etiologia e a fisiopatologia, adquiridos nas duas últimas décadas, propiciaram uma nova abordagem para o tratamento da fibrose cística. A compreensão dos mecanismos básicos da doença pulmonar, bem como das manifestações digestivas na fibrose cística, decorrente dos conhecimentos de pesquisas recentes, tem sido a chave para o aumento da sobrevida e a melhora da qualidade de vida dos pacientes.J Pediatr (Rio J) 2002; 78 (Supl.2):S171-S186: fibrose cística, doenças pulmonares, criança. AbstractObjective: cystic fibrosis has become, in the last 70 years, the most important potentially fatal inherited disease that affects white individuals around the world. Although it is considered a genetic disorder, which strikes cells of different organs, not all patients present similar clinical response. Many clinical manifestations, mainly pulmonary and gastrointestinal, may develop in cystic fibrosis patients. The aim of this article is to offer pediatricians an updated review of the controversies and recent advances in the treatment of cystic fibrosis. Sources: systematic review in the Medline database.Summary of the findings: seventy-nine articles about cystic fibrosis published in international journals were reviewed. This article presents an updated and critical review of the main events related to the incidence, pathophysiology, diagnosis and treatment of cystic fibrosis.Conclusions: even though no treatment is available for this disease, new findings about its etiology and pathophysiology have been discovered in the last two decades, improving treatment and survival of cystic fibrosis patients.J Pediatr (Rio J) 2002; 78 (Supl.2): S171-S186: cystic fibrosis, respiratory tract diseases, child. Controvérsias na fibrose cística -do pediatra ao especialista Controversies in cystic fibrosis -from pediatrician to specialist IntroduçãoNos últimos 70 anos, a fibrose cística (FC) emergiu da obscuridade para o reconhecimento como a mais importante doença hereditária, potencia...

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Section: Tripsina Imunorreativa (Tir)unclassified

Controvérsias na fibrose cística: do pediatra ao especialista

Ribeiro¹,

Ribeiro²,

Ribeiro³

2002

J. Pediatr. (Rio J.)

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“…Patients with cystic fibrosis (CF) have been found to have elevated immunoreactive trypsin in serum or dried blood spots obtained in the newborn period and during early infancy (2,8,19). In C F children 2 years of age or older, however, Crossley et al (8) found serum immunoreactive trypsin levels to be similar to or slightly below those in other children without CF.…”

Section: Speculationmentioning

confidence: 99%

“…We hav;used a radi;mmunoassay technique developed in this laboratory which can detect circulating cationic trypsinogen as well as trypsin bound to a,-protease inhibitor (al-antitrypsin) (12). In contrast to methods described in other reports (2,8,19), this procedure involves chemical blocking of the active site of the radioiodinated trypsin tracer used in the assay to prevent it from binding to plasma protease inhibitors while maintaining full immunoreactivity.…”

Section: Speculationmentioning

confidence: 99%

Plasma Immunoreactive Pancreatic Cationic Trypsinogen in Cystic Fibrosis: a Sensitive Indicator of Exocrine Pancreatic Dysfunction

et al. 1981

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SummaryPlasma immunoreactive cationic trypsin(ogen) levels were determined in 32 control subjects and 43 patients with varying degrees of pancreatic insufficiency including 35 with cystic fibrosis (CF) and eight with Shwachman's syndrome. In six CF infants less than 2 years of age, plasma trypsin(ogen) levels were significantly elevated (97.3 + 62.2 ng/ml) above the normal range for nine controls (7.0 + 5.9 ng/ml, P < 0.025). Four of these infants had steatorrhea, three of whom had undetectable duodenal trypsin activity after stimulation with secretin-cholecystokinin. In two CF infants, molecular size fractionation by gel filtration of plasma followed by radioimmunoassay of the column fractions demonstrated that trypsinogen was the only immunoreactive species in the circulation.In contrast, in older CF patients with steatorrhea (mean age, 15.3 + 4.6 years), plasma cationic trypsin(ogen) levels were undetectable or low (1.1 2 1.7 ng/ml). This finding clearly distinguished them from older CF patients without steatorrhea (mean age, 14.3 f 3.9 years) in whom cationic trypsin(ogen) levels were significantly higher (23.3 + 17.6 ng/ml; P < 0.01). The mean trypsin(ogen) concentration in the older CF patients without steatorrhea did not differ from the mean value for 23 normal subjects of similar age. Plasma cationic trypsin(ogen) levels in two Shwachman's patients with steatorrhea (0.19 and 0.86 ng/ml) were significantly lower than the values found in six Shwachman's patients without steatorrhea (5.9 + 2.3 ng/ml; P < 0.025). Furthermore, in nine older CF patients and eight Shwachman's patients, circulating trypsin(ogen) levels were highly correlated with duodenal trypsin output after secretin-cholecystokinin stimulation (r = 0.946, P < 0.01; r = 0.899, P < 0.01, respectively). These results suggest that in CF infants high levels of circulating trypsin(ogen) persist even in those with complete pancreatic insufficiency. In older CF patients and those with Shwachman's syndrome, however, circulating trypsin(ogen) accurately reflects residual pancreatic function.

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“…These results compare favourably with those from strategies based on measurement of immunoreactive trypsinogen concentration alone. 2(1 In addition, the recalled families whose infants were found subsequently not to have cystic fibrosis were alerted to the fact that at least one parent carries a cystic fibrosis mutation. Counselling included discussion about the investigation of both parents for the presence of recognised cystic fibrosis mutations to define the risks to future pregnancies.…”

Section: Discussionmentioning

confidence: 99%