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case_images: Epidemiology of biliary atresia (BA) worldwide (words in red for emphasis).
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case_images: French Polynesia has the highest rates (1:3500), with the chief reason related to intra-group marriage in isolated island populations; however, few epidemiological studies of BA have provided clues as to its etiology (Figure 1).
case_images: (A) Classification criteria of biliary atresia (BA). (B) Clinical presentation of BA (words in red for emphasis). (C) Diagnostic features in BA. (D) The present medical case belongs to type 3 (words in red for emphasis). (E) Developmental history of KPE and liver transplantation worldwide (words in red for emphasis).
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case_images: After careful re-review of our patient's medical record, we ascertained that the patient's disease was diagnosed as typical congenital BA, and that it reflected solid porta hepatis, because the most proximal part of the extrahepatic biliary tract within the porta hepatis of the patient was entirely solidified (Figure 2A). Tomita (Figure 2B). The patient had no consanguineous marriage in her immediate three generations, no similar disease in her family history, and her immediate family members were all in good health, including her elder sister. The clinical diagnosis of congenital BA primarily includes white clay-colored stools, yellow brown urine, and jaundice; accompanied by abdominal distension, dysphoria, irritability, feeding difficulties, skin itching, and other manifestations (Figure 2C). IBIL, ALT, AST, ALP, and coagulation function (Figure 2D). In addition, imaging diagnosis encompasses ultrasonography, MRI, CT, cholangiography, and nuclear medicine biliary imaging. In summary, BA can be diagnosed more accurately through the combination of clinical diagnosis, laboratory diagnosis, imaging diagnosis, and pathological diagnosis (Figure 2C). This is inconsistent with former literature, which suggests that this indicator is not a characteristic indicator of the disease (Figure 2E). ALP was consistently above the normal range, but tended to decline overall (Figure 4K, Table S2).
case_images: Clinical course of the baby and results of TDM for ISD. (A) Clinical history (timeline) of the baby (words in red for emphasis). (B) Dynamic whole-blood Tac valley concentration monitoring. ISDs are the key to anti-rejection treatment after organ transplantation in the effective therapeutic range. After liver transplantation, the ISD Tac is taken orally, but its therapeutic window is narrow and it is difficult to control its effective blood concentration with an oral dose. From 317 days of life to 383 days of life, the concentration of the drug in the blood of the baby far exceeded the normal range, resulting in renal toxicity (border colour in red for beyond the normal range). (C) Dynamic whole-blood CsA valley concentration monitoring shows a wide therapeutic window and an easily controlled oral dose of CsA. After dose adjustment, the effective blood concentration can be controlled within a stable range (black column) from 397 days of life to 1367 days of life, reducing adverse reactions to the liver and kidney (border colour in red for beyond the normal range, border colour in green for below the normal range).
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case_images: Before the first KPE surgery (at 85 days of life), all liver function indices well exceeded the normal value, suggesting severe liver dysfunction due to cholestasis (Figure 3A). Therefore, at 194 days of life, the baby underwent the second LRLT surgery (Figure 3A), followed by lifelong immunosuppressive therapy after liver transplantation (Figure 3B and C). ALP (K) and LDH (M) were beyond the normal range, although the reason for this is unclear. An immunity inhibitor was administered to prevent acute rejection and its concentration in the blood was tested regularly (Figure 3B and C). Almost all of the liver and kidney function indices of serum were in the normal range except for ALP (Figure 4K) and LDH (Figure 4M). The patient gradually recovered and gained weight, suggesting that the second surgery (LRLT) was successful. After two major surgeries, the baby girl survived, and the clinical course is shown in Figure 3A. Tacrolimus (Tac) had a narrow therapeutic window (3.0-5.0 ng/mL) (Figure 3B), and as a result, was replaced with cyclosporine A (CsA), with a wider therapeutic window (100.0-200.0 ng/mL). The dynamic blood concentrations of Tac and CsA in the baby are shown in Figure 3B and C. Therapeutic drug monitoring (TDM) results showed that the baby's blood concentration increased rapidly to the maximum value and exceeded the normal range 2 h after taking CsA orally, before decreasing slowly to the normal value (Table S1).
case_images: Postoperative dynamic monitoring of renal and liver function indices in serum. The renal function indices include UREA (A) CREA (B) and ADA (C) The liver function indices include TBIL (D) DBIL (E) IBIL (F) TBA (G), ALT (H) AST (I) GGT (J) ALP (K) CK (L) and LDH (M) Red indicates that the value is above the normal range, and green indicates that the value is below the normal range. Tac was changed to the oral ISD CsA at 397 days of life.
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case_images: ALP (K) and LDH (M) were beyond the normal range, although the reason for this is unclear. An immunity inhibitor was administered to prevent acute rejection and its concentration in the blood was tested regularly (Figure 3B and C). Almost all of the liver and kidney function indices of serum were in the normal range except for ALP (Figure 4K) and LDH (Figure 4M). The patient gradually recovered and gained weight, suggesting that the second surgery (LRLT) was successful. After two major surgeries, the baby girl survived, and the clinical course is shown in Figure 3A. Table S2). The results showed that the serum concentrations of UREA, CREA, and ADA tended to be normal (Figure 4A-C). Soon after the first operation at 113 days of life, the liver function indices including TBIL (Figure 4D), DBIL (Figure 4E), IBIL (Figure 4F), and TBA (Figure 4G) were still far beyond the normal range, which may be due to the original liver damage or the fact that the donor liver was taken from an adult. Additionally, three important indices of liver function, ALT (Figure 4H), AST (Figure 4I), and GGT (Figure 4J), gradually returned to normal after the second surgery-liver transplantation (at 197 days of life, Table S2). ALP was consistently above the normal range, but tended to decline overall (Figure 4K, Table S2). Moreover, four dynamic monitoring test results showed that CK in blood was below normal (green straight bar), but the long-term results tended to be normal (Figure 4L). Furthermore, another biochemical index of liver function, LDH, in the baby's blood was beyond the normal range, but generally tended to decline, and may be restored to the normal range in the future (Figure 4M). Healthcare Diagnostics Inc) and an easily controlled dosage in babies (Table S1). To prevent liver and kidney toxicity caused by ISD, we recorded all serum indices of liver and kidney function in the baby in detail. Serum UREA and CREA increase when renal function is impaired (Table S2). Here, from 307 days of life to 393 days of life, serum UREA continued to increase after oral Tac (Figure 4A). Additionally, most test results of serum CREA were within the normal range after oral Tac, but all were at high levels from 307 days of life to 393 days of life (Figure 4B). Here, the serum ADA levels tended to be normal when CsA was used instead of Tac after 397 days of life, suggesting that CsA was less toxic to the immune system (Figure 4C). Interestingly, most of the seven tested serum liver function indices, including TBIL (Figure 4D), DBIL (Figure 4E), IBIL (Figure 4F), ALT (Figure 4H), AST (Figure 4I), GGT (Figure 4J), and CK (Figure 4L), were within the normal range (Table S2). AST (Figure 4I), and GGT (Figure 4J). Additionally, oral ISD Tac showed that four test results of serum IBIL (Figure 4F) and CK (Figure 4L) were below the normal range (green straight bar), and after switching to CsA, only two test results were below the normal range. Additionally, the test results of serum AST (Figure 4I) were stable and within the normal range. These results suggest that the ISDs Tac and CsA have little effect on the liver function of the baby, and that the transplanted liver gradually plays a normal function in the body. Here, from 434 days of life to 669 days of life, we found that six test results of serum TBA (Figure 4G) were above the normal level (red straight bar).
case_images: Abdominal color Doppler ultrasonography showed normal transplanted liver, bile, and renal function (at 1298 days of life), and no lump, seroperitoneum, and abnormal flow in the abdominal cavity and/or around the appendix (at 1383 days of life) (words in red for emphasis). (A and B) The transplanted liver was regular in shape, smooth in surface, homogeneous in parenchymal echo and even in light spot. The portal vein blood flow velocity at the portal was 51 cm/s. The inner diameter of the inferior vena cava was not wide and the inner diameter of the common bile duct was not expanded. (C and D) Both kidneys were normal in shape and size, with a clear outline, complete and smooth renal capsule, uniform echo in parenchyma, and concentrated echo in the collecting system. No obvious abnormal echo was observed. (E-G) Abdominal color Doppler ultrasound revealed no lump, seroperitoneum, and abnormal flow in the abdominal cavity and/or around the appendix.
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case_images: The results of abdominal color Doppler ultrasound at 1298 days of life showed that the transplanted liver was complete in shape and smooth in the surface, with a portal vein flow rate of 51 cm/s, suggesting that the transplanted liver played a normal role in the body (Figure 5A-D). The follow-up was conducted at 1383 days of life, during which abdominal color Doppler ultrasound revealed no lump, seroperitoneum, and abnormal flow in the abdominal cavity and/or around the appendix (Figure 5E-G).