Congenital Portosystemic Shunt

Sokollik, Christiane; Bandsma, Robert; Gana, Juan Cristóbal; Heuvel, Meta van den; Ling, Simon C.

doi:10.1097/mpg.0b013e31828b3750

Cited by 141 publications

(108 citation statements)

References 34 publications

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“…Although clinical manifestations of CPSS can be variable, diagnosis is usually made due to symptoms or biochemical abnormalities related to hepatic dysfunction [1,7,13,14,15]. However, presentation of CPSS with sole endocrine manifestations in otherwise asymptomatic children is reported for the first time.…”

Section: Discussionmentioning

confidence: 99%

Premature Pubarche, Hyperinsulinemia and Hypothyroxinemia: Novel Manifestations of Congenital Portosystemic Shunts (Abernethy Malformation) in Children

Baş

Güran²,

Atay³

et al. 2015

Horm Res Paediatr

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Congenital portosystemic shunt (CPSS) is persistence of an anomalous embryological connection of the portal vein with a large vein of the vena cava system. Clinical presentations include neonatal cholestasis, liver tumors, and encephalopathy, but can be variable in timing and symptomatology. We report 2 girls who presented 10 years apart with the same complaint of early pubarche at age 7 years, with inappropriately low DHEAS levels. In addition to hyperandrogenemia (elevated testosterone and androstenedione) and advanced bone age, both had hyperinsulinemia, and hypothyroxinemia. The 2nd case also had symptomatic hypoglycemia. Presentation of CPSS with this combination of findings in prepubertal children has not been reported previously. With further investigations, we proposed novel mechanisms explaining these manifestations. Hyperandrogenemia is caused by decreased hepatic sulfation of DHEA to less active DHEAS due to shunting of DHEA to systemic circulation. Elevated DHEA is then used for synthesis of more potent androgens. Shunting of postabsorbtive glucose from portal to systemic circulation causes early hyperglycemia leading to exaggerated insulin secretion. Insulin bypasses the hepatic metabolism directly entering into the systemic circulation, which results in hyperinsulinemia, then in turn causes late hypoglycemia. Finally, hypothyroxinemia was linked to thyroxin-binding globulin deficiency, which has not been reported in CPSS.

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Section: Discussionmentioning

confidence: 99%

Premature Pubarche, Hyperinsulinemia and Hypothyroxinemia: Novel Manifestations of Congenital Portosystemic Shunts (Abernethy Malformation) in Children

Baş

Güran²,

Atay³

et al. 2015

Horm Res Paediatr

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“…Many authors have tried to define types of PSS with different schemes [3, 5, 31]. One of the most confusing terms is “spontaneous,” because it is controversial whether a portal hypertensive PSS should be included in spontaneous PSS or not [30, 32].…”

Section: Discussionmentioning

confidence: 99%

“…The definition and classification of PSS are in a chaotic status in respect to its cause, location, and anatomical inflow/outflow vessels. This situation probably arose because of lacking consensus, due to most of the relevant literature being composed of case reports or small series [4, 5]. The blood flow is basically similar to an electric current, in that it is determined by pressure difference and resistance, governed by Ohm's law [6].…”

Section: Introductionmentioning

confidence: 99%

Understanding the Pathophysiology of Portosystemic Shunt by Simulation Using an Electric Circuit

Kim

Lee

2016

BioMed Research International

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Portosystemic shunt (PSS) without a definable cause is a rare condition, and most of the studies on this topic are small series or based on case reports. Moreover, no firm agreement has been reached on the definition and classification of various forms of PSS, which makes it difficult to compare and analyze the management. The blood flow can be seen very similar to an electric current, governed by Ohm's law. The simulation of PSS using an electric circuit, combined with the interpretation of reported management results, can provide intuitive insights into the underlying mechanism of PSS development. In this article, we have built a model of PSS using electric circuit symbols and explained clinical manifestations as well as the possible mechanisms underlying a PSS formation.

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“…In intrahepatic shunt occurs connection between the portal vein (or one of its branches) with the hepatic vein or inferior vena cava 13 , 5 , 6 Thus, the intestinal blood passes straight into the systemic circulation without passing through the liver 7…”

Section: Discussionmentioning

confidence: 99%

Shunt portossistêmico congênito intra‐hepático diagnosticado na vida intrauterina

Bellettini

Wagner

Balzanelo

et al. 2016

Revista Paulista de Pediatria

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Portosystemic shunt can lead to various complications such as hepatic encephalopathy, hypergalactosemia, liver tumors, and hepatopulmonary syndrome. Most diagnoses are done after one month of age, after such complications occur. The prenatal diagnosis of this patient provided greater security for the clinical picture management, as well as regular monitoring, which allows the anticipation of possible complications and perform interventional procedures when needed.

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Congenital Portosystemic Shunt

Cited by 141 publications

References 34 publications

Premature Pubarche, Hyperinsulinemia and Hypothyroxinemia: Novel Manifestations of Congenital Portosystemic Shunts (Abernethy Malformation) in Children

Premature Pubarche, Hyperinsulinemia and Hypothyroxinemia: Novel Manifestations of Congenital Portosystemic Shunts (Abernethy Malformation) in Children

Understanding the Pathophysiology of Portosystemic Shunt by Simulation Using an Electric Circuit

Shunt portossistêmico congênito intra‐hepático diagnosticado na vida intrauterina

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