Oral sodium phenylbutyrate for hyperammonemia associated with congenital portosystemic shunt: a case report

AbstractObjectivesThe efficacy of sodium phenylbutyrate (SPB) for hyperammonemia associated with congenital portosystemic shunt (CPSS) remains unknown. We show the effectiveness of oral SPB.Case presentationOur patient had CPSS with severe hypoplasia of extrahepatic portal veins. At 9 months of age, to assess the efficacy of oral SPB, we evaluated the 24 h fluctuations of venous ammonia levels. In the first two days without SPB, ammonia levels were above 80 μmol/L for half a day. On the third and fourth days, administration of oral SPB three times a day decreased ammonia to acceptable levels, except at midnight. On the fifth day, another oral SPB administration at 8 pm decreased ammonia at midnight. Low levels of branched-chain amino acids, as well as coagulation disturbances, were observed without apparent symptoms. At 12 months of age, he showed normal psychomotor development.ConclusionsOral SPB may be effective for hyperammonemia associated with CPSS.

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Is there a rationale to supplement hepatotropic factors to dogs with multiple acquired portosystemic shunts secondary to congenital portosystemic shunt attenuation?

Vlaams Diergeneeskundig Tijdschrift ◽

10.21825/vdt.v90i4.20717 ◽

2021 ◽

Vol 90 (4) ◽

pp. 209-215

Author(s):

L. Van Acker ◽

H. De Rooster ◽

G. Serrano ◽

D. Paepe ◽

W. De Spiegelaere ◽

...

Keyword(s):

Clinical Trials ◽

Branched Chain Amino Acids ◽

Portosystemic Shunt ◽

Therapeutic Benefits ◽

Pathological Conditions ◽

Portosystemic Shunts ◽

Congenital Portosystemic Shunt ◽

Show Evidence ◽

Branched Chain ◽

Hepatocyte Growth

Experimental rat models and clinical trials in human patients with liver cirrhosis show evidence that supplementation with hepatotropic factors provides therapeutic benefits. This form of support has not yet been described in dogs with multiple acquired portosystemic shunts (MAPSS) despite similarities between both pathological conditions. Especially hepatocyte growth factor (HGF) and branched chain amino acids (BCAA) deserve closer attention. High-quality vegetable rather than animal proteins have been suggested to form an excellent dietary source of BCAA, and leucine seems the best candidate for supplementation given its stimulating effects on liver function in general and on HGF secretion specifically. Research on optimal ways of administration of HGF in dogs with MAPSS secondary to congenital portosystemic shunt attenuation is necessary before clinical trials can be initiated.

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Vasoactive intestinal peptide in canine portosystemic shunt in the absence of portal hypertension

Open Veterinary Journal ◽

10.4314/ovj.v11i1.7 ◽

2021 ◽

Vol 11 (1) ◽

pp. 39-41

Author(s):

Mitsuhiro Isaka ◽

Hiroshi Ueno

Keyword(s):

Blood Flow ◽

Portal Hypertension ◽

Vasoactive Intestinal Peptide ◽

Vascular Anomaly ◽

Portosystemic Shunt ◽

Portal System ◽

Enzyme Linked Immunosorbent Assay ◽

Congenital Portosystemic Shunt ◽

Significant Difference ◽

Portal Veins

Background: The congenital portosystemic shunt (PSS) is a common vascular anomaly in dogs. Vasoactive intestinal peptide (VIP) is produced in various organs (including the small intestine, large intestine, and pancreas), leading to abdominal vasodilation, increased blood flow, increased pancreatic blood flow, and promotion of pancreatic endocrine and exocrine secretions. However, there have been no reports on the concentration of VIP in the portal and peripheral veins in canine PSS. Aim: The aim of this pilot study was to evaluate whether dogs with PSS have a different VIP concentration in their portal system in general. Methods: Six dogs with an extrahepatic portosplenic shunt were included in the study. Blood samples were taken from the saphenous and portal veins during PSS ligation surgery with an amerid constrictor, to evaluate and compare the VIP concentration in both samples. VIP was measured using a commercial canine enzyme-linked immunosorbent assay kit. Results: The breeds included Mongrels (n = 2), Norfolk Terriers (n = 1), Miniature Dachshunds (n = 1), and Maltese (n = 2), and their ages were 9.3 ± 6.5 months; the bodyweight was 3.3 ± 0.8 kg. The concentration of VIP in the saphenous vein was 17.75 ± 13.88 pg/ml; on the contrary, the concentration of VIP in the portal vein was 29.7 ± 20.29 pg/ml. There was no significant difference in the VIP concentration between veins. Conclusion: There was no difference in the VIP concentration between the portal and saphenous veins, suggesting a non-association between VIP and the PSS, in the absence of portal hypertension.

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ADRENOCORTICAL FUNCTION IN PATIENTS WITH ACUTE SEVERE BRAIN AND PITUITARY LESION

Acta Endocrinologica ◽

10.1530/acta.0.0400254 ◽

1962 ◽

Vol 40 (2) ◽

pp. 254-262 ◽

Cited By ~ 1

Author(s):

H. H. Bassøe ◽

R. Emberland ◽

E. Glück ◽

K. F. Støa

Keyword(s):

Pituitary Gland ◽

Adrenal Cortex ◽

Artificial Ventilation ◽

Practical Significance ◽

Adrenocortical Function ◽

The Third ◽

Low Levels ◽

Pituitary Lesion ◽

Steroid Excretion ◽

The Brain

ABSTRACT The steroid excretion and the plasma corticosteroids were investigated in three patients with necrosis of the brain and of the pituitary gland. The patients were kept alive by artificial ventilation. In two of the patients the neutral 17-ketosteroids and the 17-hydrocorticosteroids fell to extremely low levels. At the same time, the number of eosinophil cells showed a tendency to increase. Corticotrophin administered intravenously twice to the third patient had a stimulating effect on the adrenal cortex. The theoretical and practical significance of these findings is discussed.

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