Universal screening for biliary atresia using an infant stool color card in Taiwan†

Hsiao, Cheng‐Hui; Chang, Mei–Hwei; Chen, Huey‐Ling; Lee, Hung‐Chang; Wu, Jia‐Feng; Lin, Chou-Ching K.; Yang, Yao; Chen, An-Chyi; Tiao, Mao‐Meng; Lau, Beng-Huat; Chu, Chia–Hsiang; Lai, Ming-Wei

doi:10.1002/hep.22182

“…Outros países adotaram medidas para aumentar a conscientização da sua população utilizando sinais de alerta e sistemas de triagem para AB, como as campanhas do "Alerta Amarelo" 37 e o uso da escala colorimétrica das fezes para identificar a acolia fecal 38,39 . Dentre todas estas estratégias, a escala colorimétrica das fezes, que define as fezes normais e as suspeitas por um sistema de graduação de cores das fezes, incorporada à Caderneta de Saúde da Criança e entregue a cada recém-nascido, provou ser eficaz e de fácil aplicação 40 .…”

Section: Discussionunclassified

Biliary atresia: the Brazilian experience

Carvalho¹,

Santos²,

Silveira³

et al. 2010

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Objective: To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.Methods: Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers and compared regarding decades of surgery.Results: Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year postportoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively). Conclusions:This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life. J Pediatr (Rio J). 2010;86(6):473-479:Biliary atresia, portoenterostomy, hepatic, surgery, diagnosis, differential, prognosis. ResumoObjetivo: Avaliar as características epidemiológicas, clínicas e prognósticas de crianças com atresia biliar. Os pacientes da região Nordeste foram submetidos a portoenterostomia mais tardiamente do que as crianças das regiões Sul (p = 0,008) e Sudeste (p = 0,0012), embora, mesmo nas duas últimas regiões, a idade no momento da portoenterostomia tenha sido superior ao desejável. Ao longo das décadas, houve aumento progressivo do número de TxH realizados. A sobrevida global foi de 67,6%. A sobrevida aumentou nas últimas décadas (anos 1980 versus 90, p = 0,002; anos 1980 versus 2000, p < 0,001; anos 1990 versus 2000, p < 0,001). A sobrevida de 4 anos pós-portoenterostomia, com ou sem TxH, foi de 73,4%, inversamente correlacionada à idade no momento da portoenterostomia (80, 77,7, 60,5% para ≤ 60, 61-90, > 90 dias, respectivamente). Os pacientes transplantados apresentaram taxas de sobrevida mais elevadas (88,3%). A sobrevida de 4 anos com fígado nativo foi de 36,8%, inversamente correlacionada à idade no momento da portoenterostomia (54, 33,3, 26,6% para ≤ 60, 61-90, > 90 dias, respectivamente). Método Conclusões:Este estudo multicêntrico demonstrou que o encaminhamento tardio das crianças portad...

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“…38,39 Among these strategies, the national stool color screening system, which integrated the infant stool color card into the Child-health Booklet given to every neonate, has proven to be effective and easily applicable. 40 In summary, this multicenter study on patients with BA showed that, in Brazil, overall survival of affected patients is below the desired level already attained by other groups, …”

Section: Place Of Originmentioning

confidence: 72%

Atresia biliar: a experiência Brasileira

Carvalho¹,

Santos

²

,

Silveira

³

et al. 2010

J. Pediatr. (Rio J.)

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Objective: To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.Methods: Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers and compared regarding decades of surgery.Results: Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year post-portoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively). Conclusions:This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life.

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“…1,11 In Taiwan, a universal screening system using an infant stool colour card was established to promote the early diagnosis for BA, and this enhanced early referral, timely performance of Kasai operation and better outcome. 12 The initial assessment should confirm rapidly that cholestasis is present, provide a baseline assessment of severity of liver dysfunction, and exclude potentially treatable infectious and metabolic disorders. Because the lack of specific clinical features and overlap of many diagnostic studies, most cholestatic infants require a stepwise, comprehensive evaluation.…”

Section: Resultsmentioning

confidence: 99%

Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey

Gökçebay¹,

Gülerman²,

Arda³

et al. 2015

Turkiye Klinikleri J Med Sci

4

0

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ABS TRACT Objective: Neonatal cholestasis can be the initial evidence of a heterogeneous group of diseases of different etiologies. The aim of the study is to evaluate the patients with neonatal cholestasis and analyse the etiologic factors, diagnostic tools and outcome. Material and Methods: Seventy-five patients (65% males, 45% females) between 0-6 months of age with neonatal cholestasis were retrospectively evaluated; analysed for the clinical, laboratory, radiological, scintigraphic data and histopathological findings from liver biopsies. Results: Eighty per cent of the infants' admitted to our hospital because of prolonged jaundice. The onset of the jaundice was in the first week of life in 64% of the cases, but admission time of the referral center was median two months. Biliary atresia (BA) was determined in 21 (28%), neonatal hepatitis (NH) in 54 (72%) of the patients. Biliary atresia group had significantly more frequent acholic stool, relatively lower aspartate aminotransferase, but higher gamma-glutamyl transpeptidase, and serum protein levels. Liver biopsy and hepatobiliary scintigraphy were the most sensitive methods for differentiation BA from NH (p<0.05). During the follow-up period, 13 of the patients (18%) died, whereas cholestasis improved in 14 of the patients with NH (27%) within median 6 months. Survival rate at one year was 45.5% for the patients with BA, and 87% for the patients with NH. So patients with NH had better prognosis (p<0.05). Conclusion: It has been evident that early diagnosis and intervention of treatable causes of neonatal cholestasis have a vital importance.Key Words: Jaundice, neonatal; cholestasis; biliary atresia ÖZET Amaç: Neonatal kolestaz farklı nedenlerle oluşan heterojen bir grup hastalığın ilk bulgusu olabilir. Bu çalışmanın amacı neonatal kolestazlı hastaların değerlendirilmesi ve etyolojik faktör-ler, tanı yöntemleri ve sonuçlarının incelenmesidir. Gereç ve Yöntemler: Neonatal kolestazı olan, yaşları 0-6 ay arasında değişen 75 hasta (%65'i erkek, %45'i kız) geriye dönük olarak değerlen-dirildi; klinik, laboratuvar, radyolojik, sintigrafik verileri ve karaciğer biyopsilerindeki histopatolojik bulguları incelendi. Bulgular: Bebeklerin %80'i uzamış sarılık nedeniyle hastanemize başvurmuştu. Olguların %64'ünde sarılık yaşamın ilk haftasında başlamasına rağmen ortanca başvuru zamanı iki aydı. Hastaların 21'inde (%28) bilier atrezi (BA), 54'ünde (%72) neonatal hepatit (NH) saptandı. Bilier atrezi grubunda akolik dışkı daha sık, aspartat aminotransferaz görece düşük, ancak gama-glutamil transpeptidaz ve serum protein düzeyleri yüksek saptandı. Karaciğer biyopsisi ve hepatobilier sintigrafi BA'nın NH'den ayrımında en duyarlı yöntem-lerdi (p<0,05). İzlem sırasında 13 hasta (%18) kaybedildi, ancak NH'li 14 hastada (%27) kolestaz ortanca 6 ay içinde düzeldi. Bilier atrezili hastaların bir yıllık yaşam oranı %45,5 iken, NH tanılı hastaların %87 idi. Neonatal hepatitli hastaların prognozları daha iyiydi (p<0,05). Sonuç: Erken tanı ile neonatal kolestazın tedavi edilebilir ned...

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Universal screening for biliary atresia using an infant stool color card in Taiwan†

Cited by 267 publications

References 22 publications

Biliary atresia: the Brazilian experience

Biliary atresia: the Brazilian experience

Atresia biliar: a experiência Brasileira

Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey

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