Incomplete septal cirrhosis: an enigmatic disease

Schinoni, María Isabel; Andrade, Zilton de Araújo; Freitas, Luiz Antônio Rodrigues de; Oliveira, Rubem; Paraná, Raymundo

doi:10.1111/j.1478-3231.2004.0989.x

Cited by 25 publications

(13 citation statements)

References 14 publications

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“…NRH and incomplete septal cirrhosis both involve vascular obstruction of small hepatic arteries or portal branches and may progress to noncirrhotic portal hypertension. NRH is typically associated with systemic autoimmune diseases, hematological disorders and various drugs, particularly immunosuppressant and chemotherapeutic agents (29,30), whereas incomplete septal cirrhosis has been described in patients with viral hepatitis, alcoholism and exposure to vasculotoxic chemicals such as arsenic (31). Our patient did not have any of these conditions, nor was she on medication known to induce NRH or incomplete septal cirrhosis.…”

Section: Discussionmentioning

confidence: 81%

Liver Transplantation for Acute Intermittent Porphyria: Biochemical and Pathologic Studies of the Explanted Liver

Yasuda

Erwin

Liu

et al. 2015

Mol Med

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Acute intermittent porphyria (AIP), the most common acute hepatic porphyria, is an autosomal-dominant disorder due to the half-normal activity of the heme biosynthetic enzyme, hydroxymethylbilane (HMB) synthase (1). Symptomatic heterozygotes, most (~90%) of which are women, experience episodic life-threatening acute neurovisceral attacks that typically begin with severe abdominal pain and may include hypertension, tachycardia, constipation, motor weakness and seizures. These attacks are precipitated by certain drugs, dieting and hormonal factors that increase the hepatic expression of 5-aminolevulinic acid synthase 1 (ALAS1) (1). When hepatic ALAS1 is induced, the half-normal activity of HMB synthase becomes ratelimiting, leading to decreased heme biosynthesis and depletion of the hepatic "free" heme pool. Depletion of the"free" heme pool leads to further induction of Acute intermittent porphyria (AIP) is an autosomal-dominant hepatic disorder caused by the half-normal activity of hydroxymethylbilane (HMB) synthase. Symptomatic individuals experience life-threatening acute neurovisceral attacks that are precipitated by factors that induce the hepatic expression of 5-aminolevulinic acid synthase 1 (ALAS1), resulting in the marked accumulation of the putative neurotoxic porphyrin precursors 5-aminolevulinic acid (ALA) and porphobilinogen (PBG). Here, we provide the first detailed description of the biochemical and pathologic alterations in the explanted liver of an AIP patient who underwent orthotopic liver transplantation (OLT) due to untreatable and debilitating chronic attacks. After OLT, the recipient's plasma and urinary ALA and PBG rapidly normalized, and her attacks immediately stopped. In the explanted liver, (a) ALAS1 mRNA and activity were elevated approximately ~3-and 5-fold, and ALA and PBG concentrations were increased ~3-and 1,760-fold, respectively; (b) uroporphyrin III concentration was elevated; (c) microsomal heme content was sufficient, and representative cytochrome P450 activities were essentially normal; (d) HMB synthase activity was approximately half-normal (~42%); (e) iron concentration was slightly elevated; and (f) heme oxygenase I mRNA was increased approximately three-fold. Notable pathologic findings included nodular regenerative hyperplasia, previously not reported in AIP livers, and minimal iron deposition, despite the large number of hemin infusions received before OLT. These findings suggest that the neurovisceral symptoms of AIP are not associated with generalized hepatic heme deficiency and support the neurotoxicity of ALA and/or PBG. Additionally, they indicate that substrate inhibition of hepatic HMB synthase activity by PBG is not a pathogenic mechanism in acute attacks.

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Section: Discussionmentioning

confidence: 81%

Liver Transplantation for Acute Intermittent Porphyria: Biochemical and Pathologic Studies of the Explanted Liver

Yasuda

Erwin

Liu

et al. 2015

Mol Med

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“…Similar syndromes were reported from elsewhere in the world, and a variety of names including idiopathic portal hypertension, incomplete septal cirrhosis, hepatoportal sclerosis, nodular regenerative hyperplasia, partial nodular transformation and idiopathic noncirrhotic portal hypertension were used to describe these [4][5][6][7][8][9][10]. All these entities were characterized by intrahepatic portal hypertension that was not due to cirrhosis (when the definition of cirrhosis is applied rigorously) and where liver function remains preserved until a late date.…”

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confidence: 70%

Noncirrhotic intrahepatic portal hypertension: Towards a unifying definition and etiology

Ramakrishna¹

2014

Indian J Gastroenterol

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“…12 Liver biopsy studies revealed a ISC frequency of 0.74-1.4%. 13 Histologically, it is characterized by of hepatic parenchyma nodularity, vascularized septa, reticulin buildup between hyperplastic parenchyma zones, sinusoidal dilatation and hepatocyte hyperplasia. 14 One of the pathophysiological mechanisms suggested was the existence of obliterative portal venopathy, with consequent heterogeneity in the hepatic parenchyma portal irrigation.…”

Section: Discussionmentioning

confidence: 99%

Refractory ascites and graft dysfunction in early renal transplantation

et al. 2019

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The occurrence of ascites after Renal Transplant (RT) is infrequent, and may be a consequence of surgical or medical complications. Case report: 61 year-old, male, history of arterial hypertension, tongue carcinoma and alcoholic habits 12-20g/day. He had chronic kidney disease secondary to autosomal dominant polycystic kidney disease, without hepatic polycystic disease. He underwent cadaver donor RT in September 2017. He had delayed graft function by surgically corrected renal artery stenosis. He was admitted in January 2018 for ascites de novo, with no response to diuretics. HE had visible abdominal collateral circulation. Graft dysfunction, adequate tacrolinemia, Innocent urinary sediment, mild anemia, without thrombocytopenia. Serum albumin 4.0g / dL. Normal hepatic biochemistry. Peritoneal fluid with transudate characteristics and serum albumin gradient > 1.1. Ultrasound showed hepatomegaly, permeable vascular axes, without splenomegaly. Mycophenolate mofetil was suspended, with reduced remaining immunosuppression. He maintained refractory ascites: excluded infectious, metabolic, autoimmune and neoplastic etiologies. No nephrotic proteinuria and no heart failure. MRI: micronodules compatible with bile cysts. Upper Digestive Tract Endoscopy did not show gastroesophageal varicose veins. Normal abdominal lymphoscintigraphy. He underwent exploratory laparoscopy with liver biopsy: incomplete septal cirrhosis of probable vascular etiology some dilated bile ducts. He maintained progressive RT dysfunction and restarted hemodialysis. The proposed direct measurement of portal pressure was delayed by ascites resolution. There was further recovery of the graft function. Discussion: Incomplete septal cirrhosis is an uncommon cause of non-cirrhotic portal hypertension. Its definition is not well known, morphological and pathophysiological. We have not found published cases of post-RT ascites secondary to this pathology, described as possibly associated with drugs, immune alterations, infections, hypercoagulability and genetic predisposition.

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Incomplete septal cirrhosis: an enigmatic disease

Abstract: We concluded that Incomplete septal cirrhosis is not a disease itself but it could be considered as a stage of progression and regression of liver fibrosis.

Cited by 25 publications

References 14 publications

Liver Transplantation for Acute Intermittent Porphyria: Biochemical and Pathologic Studies of the Explanted Liver

Liver Transplantation for Acute Intermittent Porphyria: Biochemical and Pathologic Studies of the Explanted Liver

Noncirrhotic intrahepatic portal hypertension: Towards a unifying definition and etiology

Refractory ascites and graft dysfunction in early renal transplantation

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