Biotinidase Activity in Patients with Liver Disease

Nagamine, Takahiko; Saito, S; Yamada, Shoji; Arai, Tomio; Takehara, Ken; Fukui, Toru

doi:10.3109/00365529309103132

Cited by 22 publications

(10 citation statements)

References 12 publications

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“…Hyperammonemia, which was detected in groups B and C, is reported among patients receiving VPA therapy (4). Lower concentrations of serum albumin were also found in our patients, as Nagamine et al (9) reported in patients with liver disease, who also had low biotinidase activity. Furthermore, the decreased biotinidase activities (Table 1) found in our patients were not related to the severity of their skin side effects of VPA, even though the number of patients who exhibited additional problems other than alopecia, seborrheic skin rash, and pruritus was small.…”

Section: Discussionsupporting

confidence: 90%

Low Serum Biotinidase Activity in Children with Valproic Acid Monotherapy

Schulpis¹,

Karikas

Tjamouranis³

et al. 2001

Epilepsia

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Summary:Purpose: Valproic acid (VPA) is an effective antiepileptic drug (AED), which is associated with dose-related adverse reactions such as skin rash, hair loss (alopecia), etc. Profound as well as partial biotinidase deficiency causes dermatologic manifestations similar these. Therefore, it was of interest to evaluate serum biotinidase activity in patients receiving VPA monotherapy.Methods: Seventy-five patients with seizures, mean age, 8.6 years (±1.9 years) were divided into three groups. Group A (n ‫ס‬ 25) was treated with VPA 28.7 ± 8.5 mg/kg/24 h, group B (n ‫ס‬ 25) with 41.6 ± 4.9 mg/kg/24 h, and group C with 54.5 ± 5.8 mg/kg/24 h. Their "trough" VPA serum levels were 40.9 ± 13.2, 86.25 ± 11.5, and 137 ± 14.5 g/ml, respectively. Fifty healthy children were the controls. Patients and controls underwent clinical and laboratory evaluations including liver function data, complete blood counts, NH 3 , and so on, after 45 days of VPA treatment. Biotinidase serum levels were evaluated fluorometrically.Results: Liver function data were found elevated in the groups B and C. On the contrary, biotinidase activity was significantly statistically lowered (p < 0.001) in groups B and C (1.22 ± 1.11, 0.97 ± 0.07 mmol/min/L respectively), as compared with controls (5.20 ± 0.90 mmol/min/L). Strong inverse correlations were observed between liver enzymes and VPA blood levels with the activity of the enzyme. Additionally, no inhibitory effect on biotinidase activity was found, when the enzyme was incubated in vitro with high (1.2 mM) concentrations of the drug. Skin lesions (seborrheic rash, alopecia) were improved in our patients after biotin (10 mg/day) supplementation.Conclusions: It is suggested that VPA impairs the liver mitochondrial function, resulting in a low biotinidase activity and or biotin deficiency. Biotin supplementation could restore some of the side effects of the drug.

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Section: Discussionsupporting

confidence: 90%

Low Serum Biotinidase Activity in Children with Valproic Acid Monotherapy

Schulpis¹,

Karikas

Tjamouranis³

et al. 2001

Epilepsia

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“…A report by Nagamine et al showed that the mean biotinidase activity in the liver patients was significantly lower than in control group (9). Their data indicated that the decrease of biotinidase activity tended to parallel the degree of liver disease (9). Their data indicated that the decrease of biotinidase activity tended to parallel the degree of liver disease (9).…”

Section: Discussionmentioning

confidence: 94%

Decreased serum biotinidase activity in adult patients with decompensated liver cirrhosis / Dekompanse karaciğer sirozlu erişkin hastalarda düşük serum biyotinidaz aktivitesi

Özdemir¹,

Aktuğlu-Zeybek²,

Baran³

et al. 2011

Bakirkoy

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ABSTRAa Decreased serum biotinidase aaivity in adult patients with decompensated liver cirrhosisObjective: The liver is the major source of serum biotinidase. Therefore liver cirrhosis can lead to decreased serum biotinidase activity and biotin deficiency. The aim of this study was to assess serum biotinidase activity in adult patients with liver cirrhosis and to investigate the relationship between serum biotinidase activity and the degree of compensation of liver cirrhosis. Material and Method: A total 40 cirrhotic patients were included in the study (24 decompensated and 16 compensated) (mean age 53,5 yr, range 37-78 yr). 21 and 15 patients were associated with hepatitis B virus and hepatitis C virus, respectively, and 4 patients had cryptogenic'cirrhosis. None of the patients exhibited clinical symptoms related to biotin deficiency. The control group consisted of 26 healthy persons (14 male and 12 female) (mean age 32 yr, range 20-50 yr). The statistical analyses were performed by student's t test. Results: Serum biotinidase activities were found 5,7±1,6, 7,8±2 and 8,7±1,5 ^mol/min/mL (mean±STD) (decompensated cirrhosis, compensated cirrhosis and the control group, respectively). Serum biotinidase activities in patients with decompensated cirrhosis were lower than both the patients with compensated cirrhosis and the control group's activities (p=0,005, p0,05). Conclusion: Our findings suggested that serum biotinidase activity was significantly lower in patients with decompensated cirrhosis. However, the patients with compensated cirrhosis had similar biotinidase activity in comparison with the control group. These results indicated that the decreased serum biotinidase activity was associated with severe impaired hepatocellular function although there were no clinical symptoms regarding the biotin deficiency, whether these patients can benefit from biotin supplementation needs further investigations. ÖZET Dekompanse karaciger sirozlu eri^kin hastalarda dü §ük serum biyotinidaz aktivitesiAmaç: Karaciger biyotinidaz enziminin bachea kaynagidir. Bu nedenle karaciger sirozu, serum biyotinidaz aktivitesindeki azalmaya ve biyotin eksikligine yol açabilmektedir. Çaliçmanin amaci karaciger sirozu olan erijkin hastalarda serum biyotinidaz aktivitesini degerlendirmek ve karaciger sirozunun kompansasyonuyla olan ilijkisini araçtirmaktadir. Gereç ve Yöntem: 24 dekompanse ve 16 kompanse olmak üzere 40 karaciger sirozlu hasta (25 erkek 15 kadin) çaliçmaya alindi (ortalama ya? 53.5: yac sinirlan 37-78). Sirasiyla, 21 ve 15 hasta hepatit B ve C virüsü enfeksiyonu ile iliçkiliydi. Dort hasta kriptojenik siroz idi. Hastalann hiçbirinde biyotin eksikligine ilijkin klinik bulgu gözlenmedi. Kontrol grup 26 (14 erkek, 12 kadin) saglikli bireyden olujtu (ortalama yaj 32; ya? sinirlan 20-50) istatistiksel analizler student t testi ile yapildi. Bulgular: Serum biyotinidaz aktiviteleri dekompanse kompanse ve kont...

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“…Nagamine et al 14 have shown that reduced serum biotinidase correlated with reduced serum albumin in patients with both acute and chronic liver diseases. They also showed reduced serum biotinidase correlated with reduced serum biotin and increased urinary biotin levels in patients with liver disease.…”

Section: Discussionmentioning

confidence: 99%

“…It was found that biotinidase activities in the disease groups were sig- © 2007 The Japan Society of Hepatology nificantly lower than control values, even after adjusting for bilirubin levels in this fashion. Nagamine et al 14 have shown that reduced serum biotinidase correlated with reduced serum albumin in patients with both acute and chronic liver diseases. They also showed reduced serum biotinidase correlated with reduced serum biotin and increased urinary biotin levels in patients with liver disease.…”

Section: Discussionmentioning

confidence: 99%

Serum biotinidase is a sensitive and specific biochemical marker of hepatic dysfunction: A preliminary report

Faith

Eapen

Wilfred³

et al. 2007

Hepatology Research

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Biotinidase is an enzyme synthesized predominantly by the liver. Serum activity of this enzyme has been shown to be low in chronic liver disease. In this study, we endeavored to assess the diagnostic value of serum biotinidase as a marker of hepatic biosynthetic function in acute and chronic liver dysfunction. Twenty-three patients with acute liver disease and 46 with chronic liver disease, as diagnosed by clinical examination, laboratory tests, histopathology and tests for viral markers, were inducted into the study. Forty-six healthy volunteers were selected as controls. Serum biotinidase activity was estimated in all the subjects. Biotinidase activity was found to be significantly lower in the serum of patients with acute (4.59 +/- 1.26 IU/L vs 7.56 +/- 0.82 IU/L in controls; P

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Biotinidase Activity in Patients with Liver Disease

Cited by 22 publications

References 12 publications

Low Serum Biotinidase Activity in Children with Valproic Acid Monotherapy

Low Serum Biotinidase Activity in Children with Valproic Acid Monotherapy

Decreased serum biotinidase activity in adult patients with decompensated liver cirrhosis / Dekompanse karaciğer sirozlu erişkin hastalarda düşük serum biyotinidaz aktivitesi

Serum biotinidase is a sensitive and specific biochemical marker of hepatic dysfunction: A preliminary report

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