case_images: PMC9201381_01
case_images: Abdominal x-rays before (A) and after (B-D) the first surgery.
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case_images: PMC9201381_01_fped-10-917116-g0001.jpg
case_images: F1
case_images: An upright abdominal X-ray showed signs of intestinal obstruction (Figure 1A). An abdominal X-ray on the first postoperative day showed low bowel inflation (Figure 1B). Two days after the operation, abdominal X-ray showed a large bubble of the stomach, and hydro-aerial(HA) levels of dilated small bowel (Figure 1C). The intestine was gradually filling with air (Figure 1D).
case_images: (A) Intestinal wall calcification. (B) Intestinal atresia with multinucleated giant cells. (C,D) Hepatic cell swelling, cholestasis, fibrosis in the portal area, inflammatory cell infiltration, small bile duct hyperplasia, vascular hyperplasia, and bile duct plate malformation. (E) Gallbladder atrophy, fibrous hyperplasia, surrounding bulky nerve fibers. (F) Fibrous tissue shows branching bile ducts and nerve fibers infiltrated with inflammatory cells.
case_images: fped-10-917116-g0002.jpg
case_images: PMC9201381_01_fped-10-917116-g0002.jpg
case_images: F2
case_images: Pathology (Figures 2A,B): (ileum), the bowel is congested and edematous with localized stenosis, atresia, and calcification. Pathology (Figures 2C-F): hepatocytes were swollen and bilious with ballooning and hepatic giant cell formation.
case_images: X-ray of the cholangiography during the second operation.
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case_images: PMC9201381_01_fped-10-917116-g0003.jpg
case_images: F3
case_images: The diagnosis of BA was confirmed during an intraoperative cholangiography procedure (Figure 3) at 46 days of age, and a KPE followed.
case_images: (A) The left Y-axis indicates DBIL, TBIL, and ALB; the right Y-axis indicates GGT. The day of Kasai's surgery is marked with the vertical dotted line. (B) Growth curve of the patient. Parenteral nutrition was used during hospitalization. After the enterostomy was closed, his weight gradually approached the normal range after discharge.
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case_images: F4
case_images: At present, the follow-up to 7 months of age revealed no cholangitis, gradual improvement of liver function index (Figure 4A), and body weight showed a catch-up trend (Figure 4B).