ESPE Abstracts (2019) 92 P3-276

ESPE2019 Poster Category 3 Late Breaking Abstracts (69 abstracts)

Exocrine Pancreatic Insufficiency and Vitamin K Deficiency Associated to Octreotide Therapy in Congenital Hyperinsulinism: An Under-Recognized Potential Adverse Effect

Purificación Ros-Pérez 1,2 , Luz Golmayo 3 , M. Luz Cilleruelo 4 , Carolina Gutierrez 4 , Patricia Celaya 3 , Nerea Lacamara 3 , Itziar Martinez-Badás 3 , María Güemes 5 & Jesús Argente 5,6,7

1Department of Pediatric Endocrinology, Hospital Universitario Puerta de Hierro-Majadahonda, MADRID, Spain. 2Department of Pediatrics, Universidad Autónoma de Madrid, MADRID, Spain. 3Department of Pediatrics, Hospital Universitario Puerta de Hierro-Majadahonda, MADRID, Spain. 4Department of Pediatric Gastroenterology, Hospital Universitario Puerta de Hierro-Majadahonda, MADRID, Spain. 5Department of Endocrinology, Hospital Infantil Universitario Niño Jesús, Instituto de Investigación la Princesa, MADRID, Spain. 6CIBER de obesidad y nutrición (CIBEROBN), Instituto de Salud Carlos III, MADRID, Spain. 7IMDEA Food Institute, CEI UAM + CSIC. Madrid, Spain., MADRID, Spain

Abstract: Congenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn. Octreotide, a long-acting somatostatin analogue (SSA), is a second line treatment for diazoxide unresponsive CH patients. Although it has been found to be a safe and effective treatment, long-term benefits and side effects have not been thoroughly evaluated. Furthermore, some authors have emphasized that exocrine pancreatic insufficiency is a common but under-recognized adverse reaction in adults treated with octreotide. To our knowledge, no pediatric patient with somatostatin analogue-induced pancreatic exocrine insufficiency has been reported to date.

Objective: Our aim is to report the first case of an infant with CH and exocrine pancreatic insufficiency and secondary vitamin K deficiency, associated to Octreotide therapy.

Case report: A 7 month and 3 week old male with diazoxide unresponsive diffuse CH (heterozygous autosomal dominantly mutation in the ABCC8 gene; NM_000352.4:c.357del) was found with bruising of legs, back and forearms after two months of SSA treatment onset (8.9 µg/kg/day divided into 4 daily doses). In addition to intermittent capillary blood glucose measurement, Real-time subcutaneous continuous glucose monitoring was used for glycemic control (Guardian TM Sensor 3; Medtronic Diabetes, Northridge, CA, USA). Bruises and bleeding remnants were also observed at the puncture points of the sensor. Laboratory findings identified vitamin K deficiency as the cause of the cutaneous hemorrhagic syndrome with an abnormal coagulation values [prothrombin time 117.4 seconds - Reference range (RR) 11.5-15.3 seconds-; International Normalized Ratio 9.1 - RR 0.8-1.2 -; Activated Partial Thromboplastin Time 88.4 seconds - RR 35.0-46.0 seconds-; serum fibrinogen 340 mg/dl - RR 150-380 mg/dl-] and a decrease in all vitamin K-dependent proteins (Factor II: 4% -RR 70-120 %-; Factor VII: 10 % -RR 55-170 %-; Factor IX: 8 % -RR 60-150%- and Factor X: 3% -RR 70-120 %-). Coagulopathy was resolved with vitamin K treatment (5 mg/day intravenous; 3 days). The patient was discharged without incidents. Further investigations revealed association of steatorrhea, (fat fecal quantification: 18.8 -19 g fat/day -RR < 6 g/d-) and stool fecal elastase-1: 120 mcg/g -RR > 200 mcg/g-), both markers of malabsorption. Other causes (cystic fibrosis and bacterial overgrowth syndrome) were excluded.

Conclusion: We report the first pediatric case of exocrine pancreatic insufficiency and vitamin K deficiency associated to Octreotide therapy in congenital hyperinsulinism, emphasizing the potential adverse effects and clinical relevance of the exocrine pancreatic insufficiency associated to Octreotide treatment.

Volume 92

58th Annual ESPE

Vienna, Austria
19 Sep 2019 - 21 Sep 2019

European Society for Paediatric Endocrinology 

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