Startseite A rare presentation of type II Abernethy malformation and nephrotic syndrome: Case report and review
Artikel Open Access

A rare presentation of type II Abernethy malformation and nephrotic syndrome: Case report and review

  • Xin Wu , Weizhong Gu , Yongzhi Lin EMAIL logo und Lina Ye EMAIL logo
Veröffentlicht/Copyright: 21. Juli 2022

Abstract

Type II Abernethy malformation is an extremely reported congenital extrahepatic portosystemic shunt in complication with nephrotic syndrome. We present the case of an 8-year-old boy who presented with symptoms of type II Abernethy malformation and nephrotic syndrome. This diagnosis of this type II Abernethy malformation was based on physical examination, blood tests, urinalysis, nephrotic and hepatic function tests, routine clinical lipid measurements, abdominal ultrasonography, and computed tomographic angiography. A kidney biopsy revealed the pathological features of nephrotic syndrome. This is the second reported patient diagnosed with type II Abernethy malformation and nephrotic syndrome. Captopril treatment was effective in improving the symptoms of this case. A patient with type II Abernethy malformation related to immune complex-mediated glomerular injury was effectively improved with medication. Type II Abernethy malformation is a causative factor of immune complex-mediated glomerular injury in nephrotic syndrome. Captopril treatment significantly improved the symptoms in this case.

1 Introduction

Abernethy malformation or congenital extrahepatic portosystemic shunt is an anomaly of the splanchnic venous flow that bypasses the liver and drains directly into the systemic circulation [1,2]. Abernethy first described Abernethy malformation in 1793 based on a postmortem examination [3]. After that, Morgan and Superina proposed the classification of Abernethy malformation into two types (Abernethy, types I–II) [4]. Type I Abernethy malformation shows direct draining of splenic vein (SV) and superior mesenteric vein (SMV) into the inferior vena cave (IVC) (Type Ia) or has a complete diversion of portal blood into IVC (Type Ib) (Figure 1, which was originally illuminated by Jian et al. [5]). In type II Abernethy malformation, a hypoplastic intrahepatic portal vein (PV) supplies partial venous blood to the liver. This rare congenital vascular malformation of the splanchnic venous system is due to the abnormal development of the umbilical vein in the embryo [1,6].

Figure 1 
               Hand-drawn diagrams suggesting congenital extrahepatic portosystemic shunts: (a) Graphic representation of normal portal flow; (b) Type Ia showing SV and SMV directly into the IVC; (c) Type Ib showing a presence of a connection between IVC and PV; (d) Type II representing a side-to-side communication between PV and IVC. SV, splenic veins; SMV, superior mesenteric vein; IVC, inferior vena cava; and PV, portal vein.
Figure 1

Hand-drawn diagrams suggesting congenital extrahepatic portosystemic shunts: (a) Graphic representation of normal portal flow; (b) Type Ia showing SV and SMV directly into the IVC; (c) Type Ib showing a presence of a connection between IVC and PV; (d) Type II representing a side-to-side communication between PV and IVC. SV, splenic veins; SMV, superior mesenteric vein; IVC, inferior vena cava; and PV, portal vein.

The clinical manifestation of Abernethy syndrome ranges from asymptomatic to presentations related to systemic or hepatic sequelae like pulmonary hypertension, hepatopulmonary syndrome, hepatic encephalopathy, liver nodules, or tumors (focal nodular hyperplasia, nodular regenerative hyperplasia, hepatocellular adenomas, and hepatocellular carcinomas) [2,7,8,9]. It is recognized that congenital portosystemic shunts are correlated with nephrotic syndrome [10]. Membranoproliferative glomerulonephritis (MPGN) is a specific histological form of glomerulonephritis with manifestations including diffuse mesangial hypercellularity, endocapillary proliferation, thickening of the capillary wall, lobulation of the glomerular tuft, and the split of the glomerular capillary wall. Type II Abernethy malformation was reported to be associated with immune complex-mediated MPGN. In this case report, we presented a patient diagnosed with type II Abernethy malformation and nephrotic syndrome. This patient suffered from hematuria and proteinuria related to increased renal vein pressure, which may be caused by portosystemic shunts.

1.1 Case presentation

An 8-year-old boy presented with scrotal swelling and both eyelids swelling for 2 days, and both lower extremities edema for 1 day. He had a history of hemorrhoids. Physical examination on admission revealed a normal body temperature (36.6oC), pulse rate (99/min), respiratory rate (24/min), and blood pressure (102/65 mmHg). The patient was conscious, mentally fine, and mildly anemic. No enlarged lymph nodes were palpated in the superficial lymph node region. The patient’s eyelids were edematous, and small red petechiae were visible on the left eyelid. The pharynx was not red, the respiratory sounds of both lungs were clear, dry, and wet rales were not heard, the heart sounds were strong and rhythmic, and no murmurs were heard. The abdomen was slightly distended, with no varices in the abdominal wall veins and no pressure pain or rebound pain in the whole abdomen. The liver was normal in shape, with no percussion pain in the kidney area. Percussion found alternating tympanic and turbid sounds in the abdomen. Both lower extremities showed sunken edema with a capillary filling time of less than 2 s. The patient received no relevant interventions. There is no hereditary family disease.

Results of the blood test suggested a white blood cell count of 6.3 × 109/L (normal range: 4–12 × 109/L), red blood cell count of 4.0 × 1012/L (normal range: 3.5–5.5 × 1012/L), neutrophil percentage of 43.0% (normal range: 50–70%), platelet count of 165 × 109/L (normal range: 100–400 × 109/L), and hemoglobin level of 119 g/L (normal range: 110–150 g/L). There was no abnormality in the erythrocyte sedimentation rate (11 mm/h). The level of high-sensitivity C-reactive protein was less than 0.2 mg/L (normal range: 0–10 mg/L). Urinary workup showed the number of urine erythrocytes was 178/HP (normal range: 0–3), the number of urine leukocytes was 22/HP (normal range: 0–4), and urine protein level was 2.0 g/L (3+) (normal range: negative). Renal function test results showed a urea level of 2.1 mmol/L (2.9–8.2), creatinine level of 17 μmol/L (62–115), and uric acid level of 218 μmol/L (208–428). Liver function and routine lipid determination results indicated a total bilirubin of 20.9 μmol/L (3.0–25.0), alanine aminotransferase activity of 20 U/L (9–50), aspartate transaminase activity of 56 U/L (15–40), globulin of 17.4 g/L (20–40), albumin/globulin ratio of 0.95 (1.20–2.40), total cholesterol of 7.14 mmol/L (3.10–5.70), triglyceride of 1.00 mmol/L (0.56–1.70), low-density lipoprotein cholesterol of 4.75 mmol/L (0.00–3.39), total protein of 33.9 g/L (65.0–85.0), and albumin of 16.5 g/L (40.0–55.0). Color Doppler ultrasonography showed no significant abnormalities in the epididymis and testes on both sides.

Abdominal ultrasonography indicated changes in hepatic echogenicity, absence of PV, and seroperitoneum. SV, SMV, and inferior mesenteric veins were dilated and tortuous. Extrahepatic portosystemic shunts were identified. Computed tomographic (CT) angiography showed the left and right branches of PV were quietly thin. SV and SMV converged at PV and drained directly into the IVC, which they communicated with the left external iliac vein. Aneurysmal dilatation of the pelvic veins was suggested. IVC, proximal hepatic veins, both renal veins, common iliac vein, and internal and external iliac veins were thickened. It was shown that the left renal vein was compressed.

A kidney biopsy was performed. Results from hematoxylin and eosin (HE), Jones silver, and periodic acid-Schiff (PAS) staining showed diffuse proliferation of mesangial cells and endocapillary hypercellularity (Figure 2). The light microscopy appearance was diffuse membranoproliferative patterns in mesangial and endothelial proliferation, accompanied by narrowed glomerular capillary and thickened capillary loops (Figure 2a and b). There was the focal segmental mesangial insertion in the glomerular basement membrane. The proliferation of mesangial cells and stroma was noticed. It was found in mesangial and subepithelial electron-dense deposits (Figure 2c). Immunofluorescence detected the glomerular deposition of IgG, IgA, IgM, C3 (0.283 g/L), C4 (0.037 g/L), and C1q. The pathological findings of the renal puncture suggested that electron-dense deposits, i.e., immune complex deposits, were seen in mesangial and paramesangial areas. The detachment of Sertoli cells was not observed. The pathology report suggested that the lesion is consistent with the characterization of mesangial proliferative glomerulonephritis. Systemic lupus erythematosus was ruled out because the patient was negative for antinuclear antibodies, dsDNA, nuclear acidic protien (Sm), and ribonucleoprotein. Moreover, there were no characteristic changes of systemic lupus erythematosus like hematoxylin bodies. Based on these results, the body was diagnosed with type II Abernethy malformation, nephritis with nephrotic syndrome, hypoproteinemia, hypocomplementemia, and hypofibrinogenemia.

Figure 2 
                  Representative photomicrographs of native biopsy: (a) HE, (b) Jones silver, and (c) PAS staining showed diffuse proliferation of mesangial cells and endocapillary hypercellularity.
Figure 2

Representative photomicrographs of native biopsy: (a) HE, (b) Jones silver, and (c) PAS staining showed diffuse proliferation of mesangial cells and endocapillary hypercellularity.

Methylprednisolone (1 mg/kg/day) was administered to control inflammation for 2 weeks. Thereafter, the patient was administrated with prednisone (1 mg/time) in combination with tacrolimus (1 mg/time) twice per day for 2 weeks. The patient was treated with captopril (0.3–0.5 mg/kg/day) for 2 weeks and piperazine ferulate (1 tablet three times a day) for 2 weeks. Human serum albumin (200 mg/kg once) was administered to increase colloidal osmotic pressure. After the administration of the drug, the patient’s proteinuria improved, and the selling was significantly decreased. The patient is currently under follow-up in the Department of Pediatric Surgery and the Department of Nephrology.

  1. Informed consent: Informed consent has been obtained from all individuals included in this study.

  2. Ethical approval: The research related to human use has been complied with all the relevant national regulations, institutional policies and in accordance with the tenets of the Helsinki Declaration, and has been approved by the authors’ institutional review board or equivalent committee.

2 Discussion

The clinical manifestations of Abernethy malformation type II are quite diverse, ranging from invisible clinical signs to obvious severe complications such as gastrointestinal hemorrhage, liver tumors, hepatic encephalopathy, hepatopulmonary syndrome, and nephrotic syndrome [10,11,12,13,14,15]. The nephrotic syndrome is rarely reported with type II Abernethy malformation [10,15]. Reported here is the case of a patient with type II Abernethy malformation related to the immune complex-mediated glomerular injury that was successfully treated by medication therapy. In attempting to generalize the pathogenesis of this case, we reviewed previous reports about renal complications of type II Abernethy.

The symptoms of patients with type II Abernethy malformation vary from asymptomatic features to multi-organ failure [16,17]. Patients with type II Abernethy malformation presented signs of fatigue pitting edema, hyperammonemia, hypoxemia [18], anemia, hepatic encephalopathy [19], and varying degrees of hepatic impairment. Patients with a complication with complex congenital heart disease and a hepatopulmonary syndrome suggested interrupted IVC with a prominent azygos vein draining in the superior vena cava [16,17]. The combined type II Abernethy malformation and nephrotic syndrome showed decreased renal function, enlarged spleen, hypoplastic PV, portosystemic shunt, arachnoid cysts, and irregular deposition of IgG, IgA, IgM, C3, C1q, and C4 [15].

In the present case, laboratory tests revealed normal white blood cell count of 6.3 × 109/L (normal range: 4–12 × 109/L), red blood cell count of 4.0 × 1,012/L (normal range: 3.5–5.5 × 1,012/L), neutrophil percentage of 43.0% (normal range: 50–70%), platelet count of 165 × 109/L (normal range: 100–400 × 109/L), hemoglobin level of 119 g/L (normal range: 110–150 g/L), and high-sensitivity C-reactive protein less than 0.2 mg/L (normal range: 0–10 mg/L). There was no abnormality in the erythrocyte sedimentation rate (11 mm/h). Blood cultures were negative. In addition, the patient was improved without the application of antibiotics, which does not support the presence of n acute infection. The pathological findings of the renal puncture suggested that electron-dense deposits, i.e., immune complex deposits, were seen in mesangial and paramesangial areas. The detachment of Sertoli cells was not observed. The pathology report suggested that the lesion is consistent with the characterization of mesangial proliferative glomerulonephritis. The results from abdominal ultrasonography and CT angiography confirmed the diagnosis of type II Abernethy malformation. Kidney biopsy and immunofluorescence examinations indicated that this patient showed symptoms of nephrotic syndrome.

An important cause of type II Abernethy malformation is the abnormal development of the early embryo’s vascular system [13,20]. The presence of a portosystemic shunt has been revealed to be a causative factor for type II Abernethy malformation patients with multisystemic presentations (such as IgA glomerulonephritis, pulmonary hypertension, and multiple liver tumors) because its functional bypass decreases the clearance of immune complexes [10,15,21]. A postulated pathogenetic basis for IgA-predominant glomerulonephritis could be due to the increased bacteremia, vasoactive substances, and IgA-antigen complexes from the intestinal mucosa uncleared by the liver, causing deposition in the kidney after being filtered by the renal glomeruli [22,23]. Persistent chronic infection thus causes glomerulonephritis and kidney injury [22,23].

Abernethy malformation limits portal blood supply, which increases the risk of developing hepatic neoplasms [16]. As such, the early recognition of type II Abernethy malformation is particularly important, as well as other associated anomalies. Liver function examination revealed increased levels of alkaline phosphatase and aspartate transaminase [16]. In this case, a marked increase in liver enzyme levels occurred. The liver of type II malformation is still perfused due to partial shunting of blood through and side-to-side portosystemic shunt. Insufficient blood supply may lead to hepatocyte damage, which can explain the increase in liver enzyme levels. Non-invasive imaging technologies are extensively used for clinical diagnosis of type II Abernethy malformation, including ultrasound, CT, and magnetic resonance imaging (MRI). MRI and CT showed a lesion and multiple cysts in the liver, a vascular shunt between the left PV and IVC [16], interrupted IVC with a prominent azygos vein draining in the superior vena cava, polysplenia, and a bilobed liver connected to a dilated PV [17]. To confirm the congenital portosystemic shunt, a transjugular approach was used for hepatic vein and portal diagnostic venography [16]. Portal venous pressure measured by temporary balloon occlusion confirmed the patency of the right PV [16]. Cranial MRI was used to detect arachnoid cysts [15]. Kidney biopsy and immunofluorescence assays confirmed nephrotic syndromes like glomerulonephritis [15].

A treatment guideline for type II malformation has not been introduced at present. For patients without overt clinical symptoms, conservative treatment is prior [24]. Ligation of abnormal shunt vessels is considered for type II Abernethy malformation patients with excessive collateral circulation pressure, varicose veins, and hepatic encephalopathy [18,19]. Complete ligation of the portal-IVC fistula and narrowing PV and ICV improved the patient’s nonspecific abdominal pain and liver function while causing thrombus, which could be relieved by anticoagulation therapy (heparin infusion and rivaroxaban) [12]. For a type II Abernethy malformation patient with hepatocellular neoplasm, Arango et al. placed a 20-mm Amplatzer vascular plug II combined with coil embolization, which successively occluded the congenital portosystemic shunt, increased the tumor side, and improved liver function [16]. This interventional closure of the portosystemic shunt also shows the feasibility and safety for type II Abernethy malformation patients with complex congenital heart disease and hepatopulmonary syndrome [17]. Chick et al. innovatively used three-dimensional planning techniques to promote single-session Amplatzer atrial septal occlude device closure for type II Abernethy malformation, while vigilance is required for postoperative complications like splanchnic thrombosis and occlusion [25].

The presence of kidney disorder increases surgical risk [15]. Conservative medicine therapy with glucocorticoids and tacrolimus was considered because of kidney disorder, and this treatment achieved remission of the symptoms [15]. Although albuminuria was controlled by drug treatment in the short term, this long-term prognosis is not recommended [15]. Currently, there is no uniform protocol for the treatment of type II Abernethy malformation. Moreover, the patient had mild symptoms, so conservative medical treatment was chosen. In the present case, the urine protein level was decreased after captopril treatment. Captopril commonly induces the following side effects: hypotension, hyperkalemia, dry irritating cough, and angioneurotic edema. Hypotension often occurs when blood volume is insufficient. Captopril is not recommended for children with hyperkalemia. A dry irritating cough can be relieved by regulating the dosage used. Captopril reduces intra-glomerular hypertension and hyperperfusion by specifically regulating glomerular hemodynamics. Captopril decreases urinary protein and inhibits the accumulation of intracellular factors and extracellular matrix, thereby delaying the development of glomerulosclerosis, improving the prognosis, and protecting the kidney function. These effects are mainly attributed to its non-hemodynamic functions, including reduction of glomerular filtration pore size, prevention of small efferent artery constriction, and improvement of the glomerular basement membrane charge-selective barrier. Therefore, captopril has therapeutic advantages over other conservative drugs. However, persistent follow-up is recommended because of the high possibility of developing chronic renal failure, gastrointestinal bleeding, and malignant hepatic lesions.

In conclusion, the combined type II Abernethy malformation and the nephrotic syndrome were described in this case report. Type II Abernethy malformation is a causative factor for immune complex-mediated glomerular injury in nephrotic syndrome. Captopril treatment significantly improved the symptoms in this case. However, further close follow-up is necessary for observing the clinical effectiveness and disease progression.


# Equally contributed to this work.

tel: +86-576-88526190
tel: +86-576-81899120

  1. Funding information: Authors state no funding involved.

  2. Author contributions: Y.L.N. and L.Y.Z. contributed to the study conception and design. Material preparation, data collection, and analysis were performed by W.X., G.W.Z., Y.L.N., and L.Y.Z. The first draft of the manuscript was written by W.X. and G.W.Z. All authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

  3. Conflict of interest: Authors state no conflict of interest.

  4. Data availability statement: The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

References

[1] Howard ER, Davenport M. Congenital extrahepatic portocaval shunts-the Abernethy malformation. J Pediatr Surg. 1997;32(3):494–7.10.1016/S0022-3468(97)90614-XSuche in Google Scholar

[2] Baiges A, Turon F, Simón-Talero M, Tasayco S, Bueno J, Zekrini K, et al. Congenital extrahepatic portosystemic shunts (Abernethy Malformation): An international observational study. Hepatology. 2020;71(2):658–69.10.1002/hep.30817Suche in Google Scholar

[3] Abernethy J. Account of two instances of uncommon formation in the viscera of the human body: From the philosophical transactions of the royal society of London. Med Facts Obs. 1797;7:100–8.Suche in Google Scholar

[4] Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 1994;29(9):1239–41.10.1016/0022-3468(94)90812-5Suche in Google Scholar

[5] Jain V, Sangdup T, Agarwala S, Bishoi AK, Chauhan S, Dhua A, et al. Abernethy malformation type 2: varied presentation, management and outcome. J Pediatr Surg. 2019;54(4):760–5.10.1016/j.jpedsurg.2018.08.053Suche in Google Scholar PubMed

[6] Kumar P, Bhatia M, Garg A, Jain S, Kumar K. Abernethy malformation: A comprehensive review. Diagn Interv Radiol. 2022;28(1):21–8. 10.5152/dir.2021.20474. PMID: 34914605.Suche in Google Scholar PubMed

[7] Zhang XL, Duan XM, Wang FY, Zhang X, Sun Y, Ma N, et al. An infant with Abernethy malformation associated with heterotaxy and pulmonary hypertension. Chin Med J (Engl). 2017;130(18):2257–8.10.4103/0366-6999.213978Suche in Google Scholar PubMed PubMed Central

[8] Lemoine C, Nilsen A, Brandt K, Mohammad S, Melin-Aldana H, Superina R. Liver histopathology in patients with hepatic masses and the Abernethy malformation. J Pediatr Surg. 2019;54(2):266–71.10.1016/j.jpedsurg.2018.10.083Suche in Google Scholar PubMed

[9] De Vito C, Tyraskis A, Davenport M, Thompson R, Heaton N, Quaglia A. Histopathology of livers in patients with congenital portosystemic shunts (Abernethy malformation): a case series of 22 patients. Virchows Arch. 2019;474(1):47–57.10.1007/s00428-018-2464-4Suche in Google Scholar PubMed PubMed Central

[10] Schaeffer DF, Laiq S, Jang HJ, John R, Adeyi OA. Abernethy malformation type II with nephrotic syndrome and other multisystemic presentation: an illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt. Hum Pathol. 2013;44(3):432–7.10.1016/j.humpath.2012.08.018Suche in Google Scholar PubMed

[11] Mesquita RD, Sousa M, Vilaverde F, Cardoso R. Abernethy malformation: beware in cases of unexplained hepatic encephalopathy in adults-case report and review of the relevant literature. BJR Case Rep. 2018;4(2):20170054.10.1259/bjrcr.20170054Suche in Google Scholar PubMed PubMed Central

[12] Zhou M, Zhang J, Luo L, Wang B, Zheng R, Li L, et al. Surgical ligation for the treatment of an unusual presentation of type II Abernethy malformation. Ann Vasc Surg. 2020;65:285.e1–5.10.1016/j.avsg.2019.10.094Suche in Google Scholar PubMed

[13] Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD. Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol. 2002;34(5):391–4.10.1002/ppul.10182Suche in Google Scholar PubMed

[14] Emre S, Arnon R, Cohen E, Morotti RA, Vaysman D, Shneider BL. Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report. Liver Transpl. 2007;13(12):1662–8.10.1002/lt.21349Suche in Google Scholar PubMed

[15] He X, Zhu Y, Fu H, Feng C, Liu Z, Gu W, et al. Case Report: Membranoproliferative glomerulonephritis, a rare clinical manifestation of Abernethy malformation type II. Front Pediatr. 2021;9:647364.10.3389/fped.2021.647364Suche in Google Scholar PubMed PubMed Central

[16] Arango NP, Nishioka Y, Velasco J, Mahvash A, Mehran RJ, Wang LS, et al. Involution of hepatocellular neoplasm after embolization of a portosystemic vascular shunt in an adult with Abernethy Type II malformation. J Vasc Interv Radiol. 2021;32(9):1391–3.10.1016/j.jvir.2021.05.026Suche in Google Scholar PubMed PubMed Central

[17] Loureiro P, Georgiev S, Ewert P, Tanase D, Eicken A, Kammer B, et al. Successful percutaneous treatment with the Konar MF™-VSD Occluder in an infant with Abernethy syndrome-case report. Cardiovasc Diagn Ther. 2021;11(2):631–6.10.21037/cdt-20-380Suche in Google Scholar PubMed PubMed Central

[18] Zhang JS, Li L. Surgical ligation of a portosystemic shunt for the treatment of type II Abernethy malformation in 12 children. J Vasc Surg Venous Lymphat Disord. 2021;9(2):444–51.10.1016/j.jvsv.2020.08.001Suche in Google Scholar PubMed

[19] Jiang C, Ye W, Liu C, Wu W, Li Y. Surgical ligation of portosystemic shunt to resolve severe hematuria and hemafecia caused by type II abernethy malformation. Ann Vasc Surg. 2015;29(5):1020.e11–6.10.1016/j.avsg.2015.01.023Suche in Google Scholar PubMed

[20] Ghuman SS, Gupta S, Buxi TB, Rawat KS, Yadav A, Mehta N, et al. The Abernethy malformation-myriad imaging manifestations of a single entity. Indian J Radiol Imaging. 2016;26(3):364–72.10.4103/0971-3026.190420Suche in Google Scholar PubMed PubMed Central

[21] Raghuram KA, Bijulal S, Krishnamoorthy KM, Tharakan JA. Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. Pediatr Cardiol. 2013;34(8):1882–5.10.1007/s00246-012-0428-zSuche in Google Scholar PubMed

[22] Lhotta K. Beyond hepatorenal syndrome: glomerulonephritis in patients with liver disease. Semin Nephrol. 2002;22(4):302–8.10.1053/snep.2002.33671Suche in Google Scholar

[23] Hemminger J, Arole V, Ayoub I, Brodsky SV, Nadasdy T, Satoskar AA. Acute glomerulonephritis with large confluent IgA-dominant deposits associated with liver cirrhosis. PLoS One. 2018;13(4):e0193274.10.1371/journal.pone.0193274Suche in Google Scholar PubMed PubMed Central

[24] Ding PX, Han XW, Liu C. Type II Abernethy malformation in a patient with primary budd-chiari syndrome. Ann Hepatol. 2019;18(1):246–9.10.5604/01.3001.0012.7933Suche in Google Scholar PubMed

[25] Chick JFB, Reddy SN, Yu AC, Kelil T, Srinivasa RN, Cooper KJ, et al. Three-dimensional printing facilitates successful endovascular closure of a type II Abernethy malformation using an amplatzer atrial septal occluder device. Ann Vasc Surg. 2017;43:311.e15–23.10.1016/j.avsg.2017.02.012Suche in Google Scholar PubMed

Received: 2022-02-09
Revised: 2022-04-12
Accepted: 2022-04-26
Published Online: 2022-07-21

© 2022 Xin Wu et al., published by De Gruyter

This work is licensed under the Creative Commons Attribution 4.0 International License.

Artikel in diesem Heft

  1. Biomedical Sciences
  2. Effects of direct oral anticoagulants dabigatran and rivaroxaban on the blood coagulation function in rabbits
  3. The mother of all battles: Viruses vs humans. Can humans avoid extinction in 50–100 years?
  4. Knockdown of G1P3 inhibits cell proliferation and enhances the cytotoxicity of dexamethasone in acute lymphoblastic leukemia
  5. LINC00665 regulates hepatocellular carcinoma by modulating mRNA via the m6A enzyme
  6. Association study of CLDN14 variations in patients with kidney stones
  7. Concanavalin A-induced autoimmune hepatitis model in mice: Mechanisms and future outlook
  8. Regulation of miR-30b in cancer development, apoptosis, and drug resistance
  9. Informatic analysis of the pulmonary microecology in non-cystic fibrosis bronchiectasis at three different stages
  10. Swimming attenuates tumor growth in CT-26 tumor-bearing mice and suppresses angiogenesis by mediating the HIF-1α/VEGFA pathway
  11. Characterization of intestinal microbiota and serum metabolites in patients with mild hepatic encephalopathy
  12. Functional conservation and divergence in plant-specific GRF gene family revealed by sequences and expression analysis
  13. Application of the FLP/LoxP-FRT recombination system to switch the eGFP expression in a model prokaryote
  14. Biomedical evaluation of antioxidant properties of lamb meat enriched with iodine and selenium
  15. Intravenous infusion of the exosomes derived from human umbilical cord mesenchymal stem cells enhance neurological recovery after traumatic brain injury via suppressing the NF-κB pathway
  16. Effect of dietary pattern on pregnant women with gestational diabetes mellitus and its clinical significance
  17. Potential regulatory mechanism of TNF-α/TNFR1/ANXA1 in glioma cells and its role in glioma cell proliferation
  18. Effect of the genetic mutant G71R in uridine diphosphate-glucuronosyltransferase 1A1 on the conjugation of bilirubin
  19. Quercetin inhibits cytotoxicity of PC12 cells induced by amyloid-beta 25–35 via stimulating estrogen receptor α, activating ERK1/2, and inhibiting apoptosis
  20. Nutrition intervention in the management of novel coronavirus pneumonia patients
  21. circ-CFH promotes the development of HCC by regulating cell proliferation, apoptosis, migration, invasion, and glycolysis through the miR-377-3p/RNF38 axis
  22. Bmi-1 directly upregulates glucose transporter 1 in human gastric adenocarcinoma
  23. Lacunar infarction aggravates the cognitive deficit in the elderly with white matter lesion
  24. Hydroxysafflor yellow A improved retinopathy via Nrf2/HO-1 pathway in rats
  25. Comparison of axon extension: PTFE versus PLA formed by a 3D printer
  26. Elevated IL-35 level and iTr35 subset increase the bacterial burden and lung lesions in Mycobacterium tuberculosis-infected mice
  27. A case report of CAT gene and HNF1β gene variations in a patient with early-onset diabetes
  28. Study on the mechanism of inhibiting patulin production by fengycin
  29. SOX4 promotes high-glucose-induced inflammation and angiogenesis of retinal endothelial cells by activating NF-κB signaling pathway
  30. Relationship between blood clots and COVID-19 vaccines: A literature review
  31. Analysis of genetic characteristics of 436 children with dysplasia and detailed analysis of rare karyotype
  32. Bioinformatics network analyses of growth differentiation factor 11
  33. NR4A1 inhibits the epithelial–mesenchymal transition of hepatic stellate cells: Involvement of TGF-β–Smad2/3/4–ZEB signaling
  34. Expression of Zeb1 in the differentiation of mouse embryonic stem cell
  35. Study on the genetic damage caused by cadmium sulfide quantum dots in human lymphocytes
  36. Association between single-nucleotide polymorphisms of NKX2.5 and congenital heart disease in Chinese population: A meta-analysis
  37. Assessment of the anesthetic effect of modified pentothal sodium solution on Sprague-Dawley rats
  38. Genetic susceptibility to high myopia in Han Chinese population
  39. Potential biomarkers and molecular mechanisms in preeclampsia progression
  40. Silencing circular RNA-friend leukemia virus integration 1 restrained malignancy of CC cells and oxaliplatin resistance by disturbing dyskeratosis congenita 1
  41. Endostar plus pembrolizumab combined with a platinum-based dual chemotherapy regime for advanced pulmonary large-cell neuroendocrine carcinoma as a first-line treatment: A case report
  42. The significance of PAK4 in signaling and clinicopathology: A review
  43. Sorafenib inhibits ovarian cancer cell proliferation and mobility and induces radiosensitivity by targeting the tumor cell epithelial–mesenchymal transition
  44. Characterization of rabbit polyclonal antibody against camel recombinant nanobodies
  45. Active legumain promotes invasion and migration of neuroblastoma by regulating epithelial-mesenchymal transition
  46. Effect of cell receptors in the pathogenesis of osteoarthritis: Current insights
  47. MT-12 inhibits the proliferation of bladder cells in vitro and in vivo by enhancing autophagy through mitochondrial dysfunction
  48. Study of hsa_circRNA_000121 and hsa_circRNA_004183 in papillary thyroid microcarcinoma
  49. BuyangHuanwu Decoction attenuates cerebral vasospasm caused by subarachnoid hemorrhage in rats via PI3K/AKT/eNOS axis
  50. Effects of the interaction of Notch and TLR4 pathways on inflammation and heart function in septic heart
  51. Monosodium iodoacetate-induced subchondral bone microstructure and inflammatory changes in an animal model of osteoarthritis
  52. A rare presentation of type II Abernethy malformation and nephrotic syndrome: Case report and review
  53. Rapid death due to pulmonary epithelioid haemangioendothelioma in several weeks: A case report
  54. Hepatoprotective role of peroxisome proliferator-activated receptor-α in non-cancerous hepatic tissues following transcatheter arterial embolization
  55. Correlation between peripheral blood lymphocyte subpopulations and primary systemic lupus erythematosus
  56. A novel SLC8A1-ALK fusion in lung adenocarcinoma confers sensitivity to alectinib: A case report
  57. β-Hydroxybutyrate upregulates FGF21 expression through inhibition of histone deacetylases in hepatocytes
  58. Identification of metabolic genes for the prediction of prognosis and tumor microenvironment infiltration in early-stage non-small cell lung cancer
  59. BTBD10 inhibits glioma tumorigenesis by downregulating cyclin D1 and p-Akt
  60. Mucormycosis co-infection in COVID-19 patients: An update
  61. Metagenomic next-generation sequencing in diagnosing Pneumocystis jirovecii pneumonia: A case report
  62. Long non-coding RNA HOXB-AS1 is a prognostic marker and promotes hepatocellular carcinoma cells’ proliferation and invasion
  63. Preparation and evaluation of LA-PEG-SPION, a targeted MRI contrast agent for liver cancer
  64. Proteomic analysis of the liver regulating lipid metabolism in Chaohu ducks using two-dimensional electrophoresis
  65. Nasopharyngeal tuberculosis: A case report
  66. Characterization and evaluation of anti-Salmonella enteritidis activity of indigenous probiotic lactobacilli in mice
  67. Aberrant pulmonary immune response of obese mice to periodontal infection
  68. Bacteriospermia – A formidable player in male subfertility
  69. In silico and in vivo analysis of TIPE1 expression in diffuse large B cell lymphoma
  70. Effects of KCa channels on biological behavior of trophoblasts
  71. Interleukin-17A influences the vulnerability rather than the size of established atherosclerotic plaques in apolipoprotein E-deficient mice
  72. Multiple organ failure and death caused by Staphylococcus aureus hip infection: A case report
  73. Prognostic signature related to the immune environment of oral squamous cell carcinoma
  74. Primary and metastatic squamous cell carcinoma of the thyroid gland: Two case reports
  75. Neuroprotective effects of crocin and crocin-loaded niosomes against the paraquat-induced oxidative brain damage in rats
  76. Role of MMP-2 and CD147 in kidney fibrosis
  77. Geometric basis of action potential of skeletal muscle cells and neurons
  78. Babesia microti-induced fulminant sepsis in an immunocompromised host: A case report and the case-specific literature review
  79. Role of cerebellar cortex in associative learning and memory in guinea pigs
  80. Application of metagenomic next-generation sequencing technique for diagnosing a specific case of necrotizing meningoencephalitis caused by human herpesvirus 2
  81. Case report: Quadruple primary malignant neoplasms including esophageal, ureteral, and lung in an elderly male
  82. Long non-coding RNA NEAT1 promotes angiogenesis in hepatoma carcinoma via the miR-125a-5p/VEGF pathway
  83. Osteogenic differentiation of periodontal membrane stem cells in inflammatory environments
  84. Knockdown of SHMT2 enhances the sensitivity of gastric cancer cells to radiotherapy through the Wnt/β-catenin pathway
  85. Continuous renal replacement therapy combined with double filtration plasmapheresis in the treatment of severe lupus complicated by serious bacterial infections in children: A case report
  86. Simultaneous triple primary malignancies, including bladder cancer, lymphoma, and lung cancer, in an elderly male: A case report
  87. Preclinical immunogenicity assessment of a cell-based inactivated whole-virion H5N1 influenza vaccine
  88. One case of iodine-125 therapy – A new minimally invasive treatment of intrahepatic cholangiocarcinoma
  89. S1P promotes corneal trigeminal neuron differentiation and corneal nerve repair via upregulating nerve growth factor expression in a mouse model
  90. Early cancer detection by a targeted methylation assay of circulating tumor DNA in plasma
  91. Calcifying nanoparticles initiate the calcification process of mesenchymal stem cells in vitro through the activation of the TGF-β1/Smad signaling pathway and promote the decay of echinococcosis
  92. Evaluation of prognostic markers in patients infected with SARS-CoV-2
  93. N6-Methyladenosine-related alternative splicing events play a role in bladder cancer
  94. Characterization of the structural, oxidative, and immunological features of testis tissue from Zucker diabetic fatty rats
  95. Effects of glucose and osmotic pressure on the proliferation and cell cycle of human chorionic trophoblast cells
  96. Investigation of genotype diversity of 7,804 norovirus sequences in humans and animals of China
  97. Characteristics and karyotype analysis of a patient with turner syndrome complicated with multiple-site tumors: A case report
  98. Aggravated renal fibrosis is positively associated with the activation of HMGB1-TLR2/4 signaling in STZ-induced diabetic mice
  99. Distribution characteristics of SARS-CoV-2 IgM/IgG in false-positive results detected by chemiluminescent immunoassay
  100. SRPX2 attenuated oxygen–glucose deprivation and reperfusion-induced injury in cardiomyocytes via alleviating endoplasmic reticulum stress-induced apoptosis through targeting PI3K/Akt/mTOR axis
  101. Aquaporin-8 overexpression is involved in vascular structure and function changes in placentas of gestational diabetes mellitus patients
  102. Relationship between CRP gene polymorphisms and ischemic stroke risk: A systematic review and meta-analysis
  103. Effects of growth hormone on lipid metabolism and sexual development in pubertal obese male rats
  104. Cloning and identification of the CTLA-4IgV gene and functional application of vaccine in Xinjiang sheep
  105. Antitumor activity of RUNX3: Upregulation of E-cadherin and downregulation of the epithelial–mesenchymal transition in clear-cell renal cell carcinoma
  106. PHF8 promotes osteogenic differentiation of BMSCs in old rat with osteoporosis by regulating Wnt/β-catenin pathway
  107. A review of the current state of the computer-aided diagnosis (CAD) systems for breast cancer diagnosis
  108. Bilateral dacryoadenitis in adult-onset Still’s disease: A case report
  109. A novel association between Bmi-1 protein expression and the SUVmax obtained by 18F-FDG PET/CT in patients with gastric adenocarcinoma
  110. The role of erythrocytes and erythroid progenitor cells in tumors
  111. Relationship between platelet activation markers and spontaneous abortion: A meta-analysis
  112. Abnormal methylation caused by folic acid deficiency in neural tube defects
  113. Silencing TLR4 using an ultrasound-targeted microbubble destruction-based shRNA system reduces ischemia-induced seizures in hyperglycemic rats
  114. Plant Sciences
  115. Seasonal succession of bacterial communities in cultured Caulerpa lentillifera detected by high-throughput sequencing
  116. Cloning and prokaryotic expression of WRKY48 from Caragana intermedia
  117. Novel Brassica hybrids with different resistance to Leptosphaeria maculans reveal unbalanced rDNA signal patterns
  118. Application of exogenous auxin and gibberellin regulates the bolting of lettuce (Lactuca sativa L.)
  119. Phytoremediation of pollutants from wastewater: A concise review
  120. Genome-wide identification and characterization of NBS-encoding genes in the sweet potato wild ancestor Ipomoea trifida (H.B.K.)
  121. Alleviative effects of magnetic Fe3O4 nanoparticles on the physiological toxicity of 3-nitrophenol to rice (Oryza sativa L.) seedlings
  122. Selection and functional identification of Dof genes expressed in response to nitrogen in Populus simonii × Populus nigra
  123. Study on pecan seed germination influenced by seed endocarp
  124. Identification of active compounds in Ophiopogonis Radix from different geographical origins by UPLC-Q/TOF-MS combined with GC-MS approaches
  125. The entire chloroplast genome sequence of Asparagus cochinchinensis and genetic comparison to Asparagus species
  126. Genome-wide identification of MAPK family genes and their response to abiotic stresses in tea plant (Camellia sinensis)
  127. Selection and validation of reference genes for RT-qPCR analysis of different organs at various development stages in Caragana intermedia
  128. Cloning and expression analysis of SERK1 gene in Diospyros lotus
  129. Integrated metabolomic and transcriptomic profiling revealed coping mechanisms of the edible and medicinal homologous plant Plantago asiatica L. cadmium resistance
  130. A missense variant in NCF1 is associated with susceptibility to unexplained recurrent spontaneous abortion
  131. Assessment of drought tolerance indices in faba bean genotypes under different irrigation regimes
  132. The entire chloroplast genome sequence of Asparagus setaceus (Kunth) Jessop: Genome structure, gene composition, and phylogenetic analysis in Asparagaceae
  133. Food Science
  134. Dietary food additive monosodium glutamate with or without high-lipid diet induces spleen anomaly: A mechanistic approach on rat model
  135. Binge eating disorder during COVID-19
  136. Potential of honey against the onset of autoimmune diabetes and its associated nephropathy, pancreatitis, and retinopathy in type 1 diabetic animal model
  137. FTO gene expression in diet-induced obesity is downregulated by Solanum fruit supplementation
  138. Physical activity enhances fecal lactobacilli in rats chronically drinking sweetened cola beverage
  139. Supercritical CO2 extraction, chemical composition, and antioxidant effects of Coreopsis tinctoria Nutt. oleoresin
  140. Functional constituents of plant-based foods boost immunity against acute and chronic disorders
  141. Effect of selenium and methods of protein extraction on the proteomic profile of Saccharomyces yeast
  142. Microbial diversity of milk ghee in southern Gansu and its effect on the formation of ghee flavor compounds
  143. Ecology and Environmental Sciences
  144. Effects of heavy metals on bacterial community surrounding Bijiashan mining area located in northwest China
  145. Microorganism community composition analysis coupling with 15N tracer experiments reveals the nitrification rate and N2O emissions in low pH soils in Southern China
  146. Genetic diversity and population structure of Cinnamomum balansae Lecomte inferred by microsatellites
  147. Preliminary screening of microplastic contamination in different marine fish species of Taif market, Saudi Arabia
  148. Plant volatile organic compounds attractive to Lygus pratensis
  149. Effects of organic materials on soil bacterial community structure in long-term continuous cropping of tomato in greenhouse
  150. Effects of soil treated fungicide fluopimomide on tomato (Solanum lycopersicum L.) disease control and plant growth
  151. Prevalence of Yersinia pestis among rodents captured in a semi-arid tropical ecosystem of south-western Zimbabwe
  152. Effects of irrigation and nitrogen fertilization on mitigating salt-induced Na+ toxicity and sustaining sea rice growth
  153. Bioengineering and Biotechnology
  154. Poly-l-lysine-caused cell adhesion induces pyroptosis in THP-1 monocytes
  155. Development of alkaline phosphatase-scFv and its use for one-step enzyme-linked immunosorbent assay for His-tagged protein detection
  156. Development and validation of a predictive model for immune-related genes in patients with tongue squamous cell carcinoma
  157. Agriculture
  158. Effects of chemical-based fertilizer replacement with biochar-based fertilizer on albic soil nutrient content and maize yield
  159. Genome-wide identification and expression analysis of CPP-like gene family in Triticum aestivum L. under different hormone and stress conditions
  160. Agronomic and economic performance of mung bean (Vigna radiata L.) varieties in response to rates of blended NPS fertilizer in Kindo Koysha district, Southern Ethiopia
  161. Influence of furrow irrigation regime on the yield and water consumption indicators of winter wheat based on a multi-level fuzzy comprehensive evaluation
  162. Discovery of exercise-related genes and pathway analysis based on comparative genomes of Mongolian originated Abaga and Wushen horse
  163. Lessons from integrated seasonal forecast-crop modelling in Africa: A systematic review
  164. Evolution trend of soil fertility in tobacco-planting area of Chenzhou, Hunan Province, China
  165. Animal Sciences
  166. Morphological and molecular characterization of Tatera indica Hardwicke 1807 (Rodentia: Muridae) from Pothwar, Pakistan
  167. Research on meat quality of Qianhua Mutton Merino sheep and Small-tail Han sheep
  168. SI: A Scientific Memoir
  169. Suggestions on leading an academic research laboratory group
  170. My scientific genealogy and the Toronto ACDC Laboratory, 1988–2022
  171. Erratum
  172. Erratum to “Changes of immune cells in patients with hepatocellular carcinoma treated by radiofrequency ablation and hepatectomy, a pilot study”
  173. Erratum to “A two-microRNA signature predicts the progression of male thyroid cancer”
  174. Retraction
  175. Retraction of “Lidocaine has antitumor effect on hepatocellular carcinoma via the circ_DYNC1H1/miR-520a-3p/USP14 axis”
Heruntergeladen am 22.9.2025 von https://www.degruyterbrill.com/document/doi/10.1515/biol-2022-0086/html
Button zum nach oben scrollen