Bile Duct Paucity in Infancy

Sergi, Consolato; Bahitham, Wesam; Al-Bahrani, Redha

doi:10.5772/25253

Cited by 4 publications

(3 citation statements)

References 16 publications

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“…Bile duct paucity, defined as bile duct to portal tract ratio of <0.9, has traditionally been a hallmark of ALGS liver disease. Although present in up to 85% of individuals, bile duct paucity is not a consistent feature and evident in only 60% of infants <6 months, highlighting the importance of the timing of the biopsy (1,(8)(9)(10). Ductopenia can also be a late finding in BA (11).…”

Section: Discussionmentioning

confidence: 99%

A Challenging Case of Focal Extrahepatic Duct Obstruction/Hypoplasia in Alagille Syndrome

Lin

Zoll

Russo

et al. 2017

J. pediatr. gastroenterol. nutr.

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In premature infants, adaptation of the bowel (including intestinal dilation and lengthening, growth of the villi, alterations of motility, and hormonal changes) is a important process that must be completed before adequate absorption of nutrients and weight gain can occur after intestinal surgery (2,5,6). Establishment of a high stoma can influence bowel adaptation and lead to various problems, such as fluid and electrolyte imbalance, abnormal acidbase status, nutritional deficiency/malnutrition, and specific stoma complications (stomal necrosis, prolapse, skin breakdown, parastomal hernia, and leakage from appliances) (2,4,7).The present study provides the first objective evidence that stool recycling has nutritional benefits for the preterm infant with a high stoma. As shown in Figure 1, weight gain was better in our infants who received stool recycling (recycling (þ) group) than in those without recycling. In addition, the recycling (þ) group recovered from anemia and serum albumin (an index of nutritional status) was normalized, whereas only 1 infant from the recycling (À) group had a normal level. Poor tissue integrity is considered to contribute to stomal prolapse in neonates with enterostomy (8). Although there was no significant difference of serum albumin between the recycling (þ) group and the recycling (À) group in our study, recovery from anemia and weight gain were better in the former group. Improvement of the nutritional state by recycling stool was considered to contribute to recovery of tissue strength, which may have reduced the incidence of stoma prolapse.Pataki et al (3) recently reported that storing stool from the oral side of the stoma led to a risk of bacterial overgrowth, whereas immediate or continuous aspiration from the stoma bag may prevent overgrowth. We used a system in which stool was aspirated continuously from the oral opening of the stoma and briefly stored in a sealed cylinder, after which it was recycled to the anal side of stoma as rapidly as possible. Before stool recycling was initiated, we infused probiotics with approximately 5 mL of glucose solution into the distal intestine, and then continuously infused 5% glucose solution with electrolytes (approximately 1-3 mL/h) for 1 or 2 days. To establish a more effective and safer recycling method and to better evaluate the effects of recycling, accumulation of more data on VLBW infants with a high stoma undergoing stool recycling is needed.

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Section: Discussionmentioning

confidence: 99%

A Challenging Case of Focal Extrahepatic Duct Obstruction/Hypoplasia in Alagille Syndrome

Lin

Zoll

Russo

et al. 2017

J. pediatr. gastroenterol. nutr.

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“…Alagille is a multisystem genetic disorder characterized by interlobular bile ductal paucity and chronic hepatocellular cholestasis (27). Alagille syndrome is a result of mutations on one of the two genes: JAG-1 in 90% of cases, and NOTCH2 in about 1% of cases.…”

Section: Alagille Syndromementioning

confidence: 99%

Carcinoma of the Liver in Children and Adolescents

Sergi

2021

Liver Cancer

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Liver cancer, predominantly hepatocellular carcinoma, is the second most common cause of cancer-related death in adults. Although infrequent in children, hepatocellular carcinoma is a terrifying diagnosis. Rising levels of obesity and obesity-associated lipid metabolic reprogramming of hepatocytes are increasing the prevalence of lipid-rich hepatocellular carcinoma in young adults. Most pediatric liver cancers occur in otherwise healthy liver, with some exceptions such as progressive familial intrahepatic cholestasis, hereditary tyrosinemia, alpha-1-antitrypsin deficiency, and genetic hemochromatosis. In the last decade, although aggressive multidisciplinary treatments including surgical resection and chemotherapy have remarkably improved patient outcomes in terms of decreased recurrence rate and increased overall survival rate, in children with unresectable liver cancer, the 5-year survival rate is still less than 20%. This chapter provides an overview of malignant epithelial tumors of the liver in children and adolescents. Hepatocellular carcinoma, lipid-rich hepatocellular carcinoma, fibrolamellar carcinoma, and cholangiocellular carcinoma are discussed.

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“…It is inherited as an autosomal dominant trait due to defects in NOTCH signaling pathway [2]. Nonsyndromic bile duct paucity may occur with congenital infections, metabolic disorders, endocrine disorders, chromosomal defects, immunologic disorders, and chronic rejection of transplants [3]. We have chosen to focus our study on clinically confirmed ALGS patients to eliminate confounding variables.…”

Section: Introductionmentioning

confidence: 99%

Identification of Bile Duct Paucity in Alagille Syndrome: Using CK7 and EMA Immunohistochemistry as a Reliable Panel for Accurate Diagnosis

et al. 2016

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Bile duct paucity is the absence or marked reduction in the number of interlobular bile ducts (ILBD) within portal tracts. Its syndromic variant, Alagille syndrome (ALGS), is a multisystem disorder with effects on the liver, cardiovascular system, skeleton, face, and eyes. It is inherited as an autosomal dominant trait due to defects in NOTCH signaling pathway. ALGS is characterized by vanishing ILBD with subsequent chronic obstructive cholestasis in approximately 89% of cases. Cholestasis stimulates formation of new bile ductules through a process of neoductular reaction, making it difficult to evaluate the presence or absence of ILBD. Therefore, finding a method to differentiate clearly between ILBD and the ductular proliferation is essential for accurate diagnosis. A database search identified 28 patients with confirmed diagnosis of ALGS between 1992 and 2014. Additionally, 7 controls were used. A panel of two immunostains, cytokeratin 7 (CK7) and epithelial membrane antigen (EMA), was performed. CK7 highlighted the bile duct epithelium of ILBD and ductular proliferation, while EMA stained only the brush border of ILBD. In our ALGS group, the ratio of EMA-positive ILBD to identified portal tracts was 12.6% (range, 0%-41%). However, this same ratio was 95.0% (range, 90%-100%) among control cases (P < 0.001). We propose a panel of two immunostains, CK7 and EMA, to differentiate ILBD from ductular proliferation in patients with cholestasis. With this panel, identification of bile duct paucity can be achieved. Additional studies, including molecular confirmation and clinical correlation, would provide a definitive diagnosis of ALGS.

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Bile Duct Paucity in Infancy

Cited by 4 publications

References 16 publications

A Challenging Case of Focal Extrahepatic Duct Obstruction/Hypoplasia in Alagille Syndrome

A Challenging Case of Focal Extrahepatic Duct Obstruction/Hypoplasia in Alagille Syndrome

Carcinoma of the Liver in Children and Adolescents

Identification of Bile Duct Paucity in Alagille Syndrome: Using CK7 and EMA Immunohistochemistry as a Reliable Panel for Accurate Diagnosis

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