Sirolimus-Induced Hepatitis in Two Patients with Hyperinsulinemic Hypoglycemia

Autor: Belma Haliloglu, Avni Kaya, Mehmet Nuri Ozbek, Heybet Tüzün, Sarah E. Flanagan, Muhittin Çelik, Sian Ellard
Přispěvatelé: Haliloğlu, Belma, Tüzün, Heybet, Flanagan, Sarah E., Çelik, Muhittin, Kaya, Avni, Ellard, Sian, Özbek, Mehmet Nuri, Yeditepe Üniversitesi
Jazyk: angličtina
Rok vydání: 2018
Předmět:
0301 basic medicine
medicine.medical_specialty
Endocrinology
Diabetes and Metabolism
Hyperinsulinemic hypoglycemia
Octreotide
030209 endocrinology & metabolism
Endokrinoloji ve Metabolizma
Case Report
medicine.disease_cause
Sulfonylurea Receptors
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Endocrinology
Internal medicine
medicine
Humans
hepatitis
cardiovascular diseases
Adverse effect
Hepatitis
Sirolimus
business.industry
Infant
Newborn
medicine.disease
equipment and supplies
3. Good health
Discontinuation
liver enzymes
030104 developmental biology
surgical procedures
operative
Pediatri
Pediatrics
Perinatology and Child Health
Congenital hyperinsulinism
cardiovascular system
Congenital Hyperinsulinism
Female
Liver function
Chemical and Drug Induced Liver Injury
business
Immunosuppressive Agents
medicine.drug
Zdroj: Journal of Clinical Research in Pediatric Endocrinology
ISSN: 1308-5735
1308-5727
Popis: Sirolimus has been reported to be effective in the treatment of the diffuse form of congenital hyperinsulinism (CHI), unresponsive to diazoxide and octreotide, without causing severe side effects. Two newborns with CHI due to homozygous ABCC8 gene mutations were started on sirolimus aged 21 and 17 days, due to lack of response to medical treatment. A good response to sirolimus was observed. At follow-up after ten and two months of treatment, liver enzymes were found to be increased [serum sirolimus level 1.4 ng/mL (normal range: 5-15), aspartate aminotransferase (AST): 298U/L, alanine aminotransferase (ALT): 302U/L and serum sirolimus level: 9.9 ng/mL, AST: 261U/L, ALT: 275U/L, respectively]. In Case 1, discontinuation of the drug resulted in normalization of liver enzymes within three days. Two days after normalization, sirolimus was restarted at a lower dose, which resulted in a repeated increase in transferases. In Case 2, a reduction of sirolimus dose caused normalization of liver enzymes within ten days. When the dose was increased, enzymes increased within three days. Sirolimus was discontinued in both cases. The rapid normalization of liver enzyme levels after sirolimus withdrawal or dose reduction; elevation of transaminases after restart or dose increase and rapid normalization after sirolimus withdrawal were findings strongly suggestive of sirolimus-induced hepatitis. To the best of our knowledge, this is the first report of sirolimus-induced hepatitis in CHI. Sirolimus is a promising drug for CHI patients who are unresponsive to medical treatment, but physicians should be vigilant for adverse effects on liver function.
Databáze: OpenAIRE
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