Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II

This report describes an 8-year-old male who presented with clinical manifestations including systemic edema, heavy proteinuria, hypoproteinemia, and persistent hypocomplementemia. Arachnoid cysts and focal nodular hyperplasia were also detected. Imaging examination and renal biopsy were performed, and Abernethy malformation type II with immune complex-mediated membranoproliferative glomerulonephritis was considered the diagnosis. Due to the persistence of embryonic vessels, Abernethy malformation is a rare congenital vascular malformation of the splanchnic venous system, which can be classified as type I (end-to-side shunt) and type II (side-to-side shunt). Abernethy malformation with glomerulonephritis remains extremely rare. In the patient described, glomerulonephritis mediated by immune complex with “full-house” positive immunohistochemistry was confirmed on renal biopsy. In addition, he was treated with glucocorticoids and tacrolimus. Whether surgical treatment is necessary should be determined according to the state of the disease in the later stages. The present case reflects the association between the congenital portosystemic shunt and the renal region and, to the authors' knowledge, may be the first report to describe arachnoid cysts as a symptom of Abernethy malformation.

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Demographic, clinical characteristics and treatment outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulonephritis in Japan: A retrospective analysis of data from the Japan Renal Biopsy Registry

PLoS ONE ◽

10.1371/journal.pone.0257397 ◽

2021 ◽

Vol 16 (9) ◽

pp. e0257397

Author(s):

Naoki Nakagawa ◽

Masashi Mizuno ◽

Sawako Kato ◽

Shoichi Maruyama ◽

Hiroshi Sato ◽

...

Keyword(s):

Renal Function ◽

Renal Biopsy ◽

Treatment Outcomes ◽

Immune Complex ◽

Membranoproliferative Glomerulonephritis ◽

Clinical Characteristics ◽

Diastolic Pressure ◽

Remission Rate ◽

Type I ◽

C3 Glomerulonephritis

The reclassification of membranoproliferative glomerulonephritis (MPGN) into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) based on immunofluorescence findings in kidney biopsies has provided insights into these two distinct diseases. C3G is further classified into dense deposit disease and C3 glomerulonephritis (C3GN) based on electron micrographic findings. Although these diseases have poor outcomes, limited Japanese literature confined to small, single-center cohorts exist on these diseases. We retrospectively analyzed 81 patients with MPGN type I and III from 15 hospitals in the Japan Renal Biopsy Registry to compare demographic, clinical characteristics and treatment outcomes of patients with IC-MPGN to those with C3GN. Of the 81 patients reviewed by immunofluorescence findings in kidney biopsies, 67 patients had IC-MPGN and 14 patients had C3GN. Age at diagnosis and systolic and diastolic pressure were higher and proteinuria and impaired renal function were significantly more prevalent in patients with IC-MPGN than those with C3GN. About 80% of the patients in both groups were treated with immunosuppressive therapy. At last follow-up (median 4.8 years), complete remission rate of proteinuria was significantly higher in patients with C3GN (64.3%) than in those with IC-MPGN (29.9%; P = 0.015). The renal survival rate was lower in patients with IC-MPGN when compared to C3GN (73.1% vs. 100%; log-rank, P = 0.031). Systolic blood pressure and renal function at baseline were independent predictors of progression to end-stage kidney disease. The overall prognosis of patients with C3GN is more favorable than for patients with IC-MPGN.

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Membranoproliferative glomerulonephritis with C3 and intramembranous dense deposits

Microscopy and Microanalysis ◽

10.1017/s1431927613000834 ◽

2013 ◽

Vol 19 (S4) ◽

pp. 43-44

Author(s):

F. Carvalho ◽

H. Viana ◽

A.P. Alves de Matos ◽

M. Amoedo

Keyword(s):

Basement Membrane ◽

Membranoproliferative Glomerulonephritis ◽

Correct Diagnosis ◽

Type I ◽

Type Ii ◽

C3 Nephritic Factor ◽

Dense Deposit ◽

Dense Deposits ◽

Capillary Walls ◽

Mild Renal Insufficiency

Membranoproliferative glomerulonephritis (MPGN) encompasses 7 to 10% of all biopsied glomerulonephritis. They are divided in: MPGN type I; MPGN type II and MPGN type III, being primary or secondary. MPGN type I are the most frequent, MPGN types II and III are very rare and difficult to diagnose without clinical and morphologic findings integration. MPGN type II or Dense Deposit Disease has a varied morphologic appearance with a few numbers of cases showing a membranoproliferative pattern by Light microscopy (LM). Electron microscopy (EM) is pivotal to confirm the diagnosis.We present a case of 35 years old man, with nephrotic proteinuria and mild renal insufficiency since 2 years. The only relevant clinical data is facial lipodystrophy. Complement 3 (C3) was low and C3 nephritic factor negative. There were not other relevant abnormalities. Renal biopsy was fixed in buffered formaldehyde 10% and performed for LM. The frozen fragment, prepared for observation by fluorescence microscopy - immunofluorescence (IMF) -, was prepared to be stained with florescent anti-serums, against immunoglobulines (IgG, IgA and IgM) and complement factors (C3, C4, and C1q). EM was later done on tissue formaldehyde fixed reprocessed from paraffin-embebbed for LM, because there was no tissue fragment fixed in glutaraldehyde.LM showed variable endocapillary hypercelullarity, with neutrophils infiltration. Capillary walls were thickened due to the deposition of elongate and ribbon-like deposits. Few double contours were visible (Figure 1a). IMF demonstrated the presence of C3 deposits in the capillary walls and mesangium (Figure 1b). EM confirmed the presence of an intramembranous dense deposit along basement membrane which was thickened (Figure 1c). LM and IMF findings favored the diagnosis of MPGN type II with C3 deposits and thickening of basement membrane. Nevertheless EM was essential to confirm intramembranous unequivocally dense deposits.MPGN type II is a rare glomerulonephritis mediated by complement deregulation. The integration of clinical and morphologic findings is essential to get a correct diagnosis. In this setting EM is highly distinctive and required for a definitive diagnosis.

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Abernethy Malformation Type II and Concurrent Nodular Hyperplasia in a Rare Female Case

Case Reports in Radiology ◽

10.1155/2018/6501490 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Zhen Kang ◽

Xiangde Min ◽

Liang Wang

Keyword(s):

Differential Diagnosis ◽

Case Report ◽

Type I ◽

Type Ii ◽

Malignant Tumours ◽

Abernethy Malformation ◽

Vascular Abnormality ◽

Female Case ◽

Nodular Hyperplasia ◽

The Right

Background. Abernethy malformation is a rare splanchnic vascular abnormality characterizing extrahepatic abnormal shunts that is classified into types I and II. Abernethy malformation type I has a female predilection and is associated with a variety of concurrent hepatic benign or malignant tumours while type II with concurrent tumours is very rare in females. Case Report. We report a rare female case of Abernethy malformation type II with concurrent occupying lesion in the right liver, which was successfully transplanted; the occupying lesion was pathologically proven to be nodular hyperplasia. Conclusion. This case might provide further knowledge regarding Abernethy malformation. On imaging, the anatomy of portal vein should be carefully investigated to categorize Abernethy malformation, and a wide variety of differential diagnosis of concurrent occupying lesions should be taken into account.

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Diverse MRI findings and clinical outcomes of acute Marchiafava-Bignami disease

Acta Radiologica ◽

10.1177/0284185120943040 ◽

2020 ◽

pp. 028418512094304

Author(s):

Wei Li ◽

Chao Ran ◽

Jun Ma

Keyword(s):

Clinical Outcomes ◽

Clinical Manifestations ◽

Acute Onset ◽

Restricted Diffusion ◽

Type I ◽

Type Ii ◽

Mri Findings ◽

Type Iii ◽

Mri Features ◽

Magnetic Resonance Imaging Mri

Background The acute onset of Marchiafava-Bignami disease (MBD) is difficult to capture, and its clinical manifestations are overlapped. Magnetic resonance imaging (MRI) is very useful in the diagnosis of acute MBD. Purpose To investigate the MRI features and clinical outcomes of acute MBD. Materials and Methods Sagittal T2-weighted (T2W) or T1-weighted (T1W) imaging, axial T1W and T2W imaging, and axial FLAIR and diffusion-weighted imaging (DWI) sequences were performed in 17 patients with acute MBD on 1.5-T MR. According to the different ranges of callosal restricted diffusion, MBD was divided into Type I (n = 7, the completely involved), Type II (n = 5, the mostly involved), and Type III (n = 5, the partly involved). The MRI findings and outcomes of each type were retrospectively analyzed. Results With the reduced range of the callosal restricted diffusion, the callosal atrophy or cavitation was more common: no case of Type I; 1 (20%) case of Type II; and 3 (60%) cases of Type III. With the increased range of callosal restricted diffusion, the extracallosal involvement was more common: 6 (86%) cases of Type I; 3 (60%) cases of Type II; and 1 (20%) case of Type III. During the follow-up, five cases had neuropsychiatric sequelae: 1 (14%) case of type I; 1 (20%) case of Type II; 3 (60%) cases of Type III. Conclusion The MRI findings and clinical outcomes of acute MBD are regular. The extensive restricted diffusion of acute MBD may present the curable condition. Callosal heterogeneity may affect the outcome of acute MBD.

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Posterior petrous meningiomas: 82 cases

Journal of Neurosurgery ◽

10.3171/jns.2005.102.2.0284 ◽

2005 ◽

Vol 102 (2) ◽

pp. 284-289 ◽

Cited By ~ 25

Author(s):

Zhe Bao Wu ◽

Chun Jiang Yu ◽

Shu Sen Guan

Keyword(s):

Facial Nerve ◽

Clinical Manifestations ◽

Petrous Bone ◽

Type I ◽

Type Ii ◽

Posterior Surface ◽

Total Resection ◽

Content Type ◽

Functional Preservation ◽

Fine Print

Object. The aim of this study was to discuss posterior petrous meningiomas—their classification, clinical manifestations, surgical treatments, and patient outcomes. Methods. A retrospective analysis was performed in 82 patients with posterior petrous meningiomas for microsurgery. According to the anatomical relationship with the posterior surface of the petrous bone and with special reference to the internal auditory canal (IAC), posterior petrous meningiomas were classified into three types: Type I, located laterally to the IAC (28 cases); Type II, located medially to the IAC, which might extend to the cavernous sinus and clivus (32 cases); and Type III, extensively attached to the posterior surface of the petrous bone, which might envelop the seventh and eighth cranial nerves (22 cases). Sixty-eight (83%) of 82 cases involved total resection. The rate of anatomical preservation of facial nerve was 97.5%, whereas the functional preservation rate was 81%. The rate of hearing preservation was 67%. All Type I tumors were completely resected, and the rate of anatomical preservation of facial nerve was 100% and functional preservation was 93%. Regarding Type II lesions, 75% of 32 cases involved total resection; the rate of anatomical preservation of facial nerve was 97% and functional preservation was 75%. For Type III lesions, 73% of 22 cases were totally resected. The rate of anatomical preservation of facial nerve in patients with this tumor type was 95%, whereas functional preservation was 73%. Conclusions. Clinical manifestations and surgical prognoses are different among the various types of posterior petrous meningiomas. It is more difficult for Types II and III tumors to be resected radically than Type I lesions, and postoperative functional outcomes are significantly worse accordingly. The primary principles in dealing with this disease entity include preservation of vital vascular and central nervous system structures and total resection of the tumor as much as possible.

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Renal transplantation for patients with type I and type II membranoproliferative glomerulonephritis

The American Journal of Medicine ◽

10.1016/0002-9343(79)90535-7 ◽

1979 ◽

Vol 66 (2) ◽

pp. A34

Keyword(s):

Renal Transplantation ◽

Membranoproliferative Glomerulonephritis ◽

Type I ◽

Type Ii

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IgG subclasses in cryoglobulins: link to composition and clinical manifestations

Arthritis Research & Therapy ◽

10.1186/s13075-020-02364-z ◽

2020 ◽

Vol 22 (1) ◽

Author(s):

Marie N. Kolopp-Sarda ◽

Pedro Ming Azevedo ◽

Pierre Miossec

Keyword(s):

Mixed Type ◽

Clinical Manifestations ◽

Igg Subclasses ◽

Type I ◽

Clinical Parameters ◽

Igg Subclass ◽

Type Ii ◽

Neurological Manifestations ◽

Inclusion Criteria ◽

Previous Series

Abstract Background Cryoglobulins (CG) are immunoglobulins which precipitate at low temperature. The analysis of IgG subclass composition of CG is poorly reported. The aim of this study was to determine the subclasses of IgG-containing type I and mixed type II and III CG in relation to clinical manifestations. Methods Out of a previous series of 1675 patients, inclusion criteria were a cryoprecipitate > 1 mL and a total IgG > 300 mg/L. IgG subclasses were quantified by immunoturbidimetry, rheumatoid factor (RF), and C4 by immunonephelometry. Clinical parameters were collected from hospital charts. Results CG samples from 86 patients were included, 10 type I CG and 76 mixed CG. Type I CG subclasses were IgG1 (6/10) and IgG2/IgG3 (4/10), never IgG4. IgG subclass in type II vs. III CG were 73.3 ± 15.2% vs. 52.5 ± 20.7% for IgG1 (p < 0.0001), 15.4 ± 8.2% vs. 25.9 ± 14% for IgG2 (p < 0.0001), 8.4 ± 12.4 vs. 21.2 ± 14% for IgG3 (p < 0.0001), and 3 ± 5.2% vs. 0.5 ± 1.2 for IgG4 (p < 0.0001). In mixed CG, the higher proportion of IgG4 was associated with RF positive CG (p = 0.01) and low C4 (p = 0.01). In type I CG, IgG1 were associated with severe vasculitis manifestations, IgG2/IgG3 with cutaneous or renal manifestations. In mixed CG, IgG2 was the only subclass associated with CG manifestations, with a higher concentration in asymptomatic (162.6 ± 29.5 mg/L) vs. symptomatic patients with cutaneous (103 ± 17.8 mg/L, p = 0.04) and neurological (108 ± 24 mg/L, p = 0.04) manifestations. Conclusion In type I IgG CG, IgG1 was the main CG subclass associated with CG vasculitis. In mixed CG, low IgG2 concentration was linked to CG cutaneous and neurological manifestations.

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Type II Abernethy Malformation in a Patient with Primary Budd-Chiari Syndrome

Annals of Hepatology ◽

10.5604/01.3001.0012.2227 ◽

2018 ◽

Vol 17 (5) ◽

pp. 0-10

Author(s):

Peng-Xu Ding ◽

Xin-Wei Han ◽

Chao Liu

Keyword(s):

Vena Cava ◽

Vascular Diseases ◽

Type Ii ◽

Budd Chiari Syndrome ◽

Abernethy Malformation ◽

Congenital Vascular Malformation ◽

Inferior Vena Cava Obstruction ◽

First Case ◽

Percutaneous Balloon ◽

Chiari Syndrome

Budd-Chiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction. Abernethy malformation is a congenital vascular malformation defined by diversion of portal blood away from the liver. Both conditions are rare vascular diseases. We report here the first case of a patient with combined type II Abernethy malformation and Budd-Chiari syndrome from China. The inferior vena cava obstruction was treated with percutaneous balloon angioplasty; close follow-up was elected for the Abernethy malformation.

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