ISSN (electrónico): 1699-5198 - ISSN (papel): 0212-1611 - CODEN NUHOEQ S.V.R. 318 Nutrición Hospitalaria Nota Clínica Hernia enlargement and pancreatitis in a patient with short bowel syndrome treated with teduglutide Crecimiento de hernias y pancreatitis en una paciente con síndrome de intestino corto tratada con teduglutida Irene Conejo1, Núria Mas-Malagarriga1, Pau Riera1,2, Joana Cardenete1, Rocío Puig-Piña3, Manuel Rodríguez Blanco4, Jan T. De Pourcq1 1 Pharmacy Department; 2U-705. Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER). Instituto de Salud Carlos III; 3Endocrinology and Nutrition Service; and 4Department of General Surgery. Hospital de la Santa Creu i Sant Pau. Barcelona, Spain Abstract Introduction: teduglutide (TED) is indicated for the treatment of patients with short-bowel syndrome (SBS) who are dependent on parenteral support. Case report: we report the case of a 60-year-old woman with SBS treated with TED. She had previously undergone multiple surgical resections due to Crohn’s disease. Her remnant bowel included only the duodenum and 50-60 centimeters of jejunum. The patient was dependent on Keywords: intravenous fluids (2,320 mL/48 h) and had a high stoma output (3,000 mL/day). After four months of TED the jejunostomy output had decreased to 2,200 mL/day with a thicker consistency, and intravenous fluid therapy was reduced to 2,010 mL/48 h. TED was withdrawn due to acute Teduglutide. Short pancreatitis and enlargement of two supraumbilical hernias with high strangulation risk. bowel syndrome. Pancreatitis. Hernia Discussion: pancreatitis has been reported in clinical studies, and determination of amylase and lipase is recommended in all patients receiving enlargement. Case TED. In contrast, there are no recommendations for the surveillance of hernia enlargement in patients on TED therapy, but we suggest the need report for surveillance based on this case report. Resumen Introducción: la teduglutida (TED) está indicada para el tratamiento de pacientes con síndrome de intestino corto (SBS) que precisen soporte parenteral. Caso clínico: mujer de 60 años con SBS tratada con TED. Previamente se había sometido a múltiples resecciones quirúrgicas por su enfermedad de Crohn. Su intestino remanente incluía el duodeno y 50-60 centímetros de yeyuno. La paciente era dependiente de líquidos por vía intravenosa Palabras clave: (2320 ml/48 h) y tenía una ostomía de alto débito (3000 ml/día). Después de cuatro meses de TED, el débito de la yeyunostomía disminuyó a 2200 ml/día, con una consistencia más espesa, y la fluidoterapia intravenosa se redujo a 2010 ml/48 h. Se retiró la TED por pancreatitis aguda Teduglutida. y agrandamiento de dos hernias supraumbilicales con alto riesgo de estrangulamiento. Síndrome de intestino corto. Pancreatitis. Discusión: se han descrito casos de pancreatitis en estudios previos, por lo que se recomienda la determinación de la amilasa y la lipasa en los Crecimiento de pacientes tratados con TED. Sin embargo, no hay recomendaciones específicas sobre la vigilancia del agrandamiento de hernias, pero sugerimos hernias. Caso clínico. su idoneidad basada en este caso clínico. Received: 20/09/2021 • Accepted: 07/11/2021 Irene Conejo and Núria Mas-Malagarriga contributed equally to this case report. Consent to publish: the participant has consented to the submission of this case report to the journal. Financial disclosure: no funding was received. Conflicts of interest: the authors declare no conflicts of interest. Conejo I, Mas-Malagarriga N, Riera P, Cardenete J, Puig-Piña R, Rodríguez Blanco M, T. De Pourcq J. Correspondence: Hernia enlargement and pancreatitis in a patient with short bowel syndrome treated with teduglutide. Pau Riera. Pharmacy Department. Hospital Nutr Hosp 2022;39(1):230-232 de la Santa Creu i Sant Pau. Carrer de Sant Quintí, 89. 08041 Barcelona, Spain DOI: http://dx.doi.org/10.20960/nh.03879 e-mail: priera@santpau.cat © Copyright 2022 SENPE y ©Arán Ediciones S.L. Este es un artículo Open Access bajo la licencia CC BY-NC-SA (http://creativecommons.org/licenses/by-nc-sa/4.0/). HERNIA ENLARGEMENT AND PANCREATITIS IN A PATIENT WITH SHORT BOWEL SYNDROME TREATED WITH TEDUGLUTIDE 231 INTRODUCTION CASE REPORT Short bowel syndrome (SBS) is characterized by a signifi- A 60-year-old woman with Crohn’s disease underwent mul- cant decrease in effective intestinal surface due to anatomical tiple surgical resections (colectomy, duodenectomy, jejunosto- or functional losses. The main causes of SBS are surgical re- my, proctectomy) between December 2008 and October 2014, sections, congenital defects, and diseases affecting the bowel. which resulted in a remnant bowel including only the duodenum Crohn’s disease is one of the underlying conditions that may and 50-60 centimeters of jejunum distal to the ligament of Treitz. require intestinal resection. Symptoms of SBS are variable, but Her medical history included obesity, hypothyroidism, fibromyal- normally characterized by diarrhea, fatty stools, abdominal pain, gia, rheumatoid arthritis, scoliosis, hiatus hernia, and gallblad- malnutrition, and dehydration (1). Patients suffering from SBS der removal. No previous history of pancreatitis was noted. Her may require long-term IV supplementation (1). regular medications included levothyroxine, ranitidine, sodium Teduglutide (TED) is an analogue of glucagon-like peptide-2 bicarbonate, fluoxetine, azathioprine, benzbromarone, magnesi- (GLP-2), a hormone-based treatment that helps intestinal re- um, calcium, vitamins D2 and D3, sodium chloride, vitamin B12, habilitation by reinforcing the structural and functional integrity omega-3 fatty acids, and taurine. of the remaining intestine in SBS (2). GLP-2 receptor activation The patient became dependent on intravenous fluids (saline stimulates mucosal growth, thereby leading to the expansion of with vitamins, electrolytes, minerals, and trace elements) and epithelial surface area (3). This can enhance absorption of ma- had a high stoma output (3,000 mL/day) despite support with cronutrients and drugs (4). slowing agent therapies. TED was started in February 2019, TED is used in SBS patients requiring chronic parenteral sup- with a starting dose of 3.9 mg/day (0.05 mg/kg). Eighteen port (PS). The pivotal trials of TED demonstrated that, in patients weeks after starting TED, the jejunostomy output had decreased with intestinal failure (IF) who required PS ≥ 3 times per week from 3,300 to 2,254 mL/day (32 % reduction) and had a thicker for at least 12 months, 63 % of those treated with TED had a consistency, so intravenous fluids were reduced from 2,320 to relative reduction in their PS volume of > 20 % from baseline at 2,010 mL/48 h (13 % reduction). The clinical follow-up data are weeks 20 and 24, compared with 30 % of patients receiving pla- detailed in table I. cebo (p < 0.01) (5,6). TED decreases dependence on parenteral Twenty-four hours after starting treatment the patient devel- support and, in some cases, independence from parenteral sup- oped a fever attributed to an intestinal virus. After a month of port can be achieved. This can markedly improve patient quality TED therapy, the patient experienced an episode of radiculopa- of life as well as decrease the risk of PS-related complications. thy, not attributable to TED. To treat this, pregabalin was initiated. However, as TED is a relatively new drug, the potential side ef- After two months of TED therapy, two peristomal supraumbilical fects are not well known yet. We report the case of a patient who hernias enlarged, thus increasing the risk of hernia strangula- presented with mild acute pancreatitis and enlargement of two tion. They were managed conservatively; had they grown larg- supraumbilical hernias with peristomal location probably due to er, a further bowel resection would have been needed. During TED therapy. the fourth month the patient experienced an episode of sudden Table I. Clinical follow-up data Baseline Month 1 Month 2 Month 3 Month 4 Month 5 Weight (kg) 78.1 78.7 79.1 78.9 78.6 78.7 BMI (kg/m2) 31.3 31.5 31.7 31.6 31.5 31.5 Oral volume intake (mL/day) 3300 2829 2807 2835 2771 3010 PS volume (mL/48 h) 2320 2320 2320 2110 2010 2010 Feces output (mL/day) 3300 2961 2822 2700 2376 2254 Urine output (mL/day) 1525 1571 1438 1349 1269 1549 Urea (mg/dL) 40 31 38 25 40 34 Creatinine (mg/dL) 0.76 0.79 0.80 0.84 0.81 0.76 PS: parenteral support. [Nutr Hosp 2022;39(1):230-232] 232 I. Conejo et al. abdominal pain, intolerance of oral intake, and increased acute lipase and amylase elevation. Likewise, Lee et al. reported the phase reactants and pancreatic lipase, and she was diagnosed case of a 70-year-old female with Crohn’s disease in whom an with mild acute pancreatitis. TED was considered a possible increase in amylase and lipase was detected during TED treat- cause of pancreatitis, and therefore the drug was discontinued. ment. In our case, the increase in lipase levels, along with the Lipase levels increased suddenly from normal levels (< 100 U/L) abdominal pain reported by the patient, led to the diagnosis of to 17,335 U/L when diagnosed, and returned to normal levels acute pancreatitis. A biliary origin was considered unlikely due to after two weeks. After analgesic treatment and 24 h of digestive her history of gallbladder removal. Although pancreatitis could rest, a progressive oral diet was restarted and well tolerated. have been caused by drugs concomitantly taken by the patient Treatment with TED was permanently withdrawn, the stoma (ranitidine, azathioprine, pregabalin or fluoxetine) or the coexist- output increased significantly again and, consequently, the same ence of all of them, the time sequence suggests the onset of initial parenteral support of 2,320 mL/48 h was reintroduced. acute pancreatitis could be attributable to TED. Because of the The risk-benefit of TED treatment for this patient is currently be- high risk of hernia strangulation and her short bowel remnant, ing reassessed due to difficulties in preserving her central venous TED rechallenge has been considered inaproppriate. access and the high risk of hernia strangulation and pancreatitis. In conclusion, our patient experienced benefits from TED treat- ment, but also some complications attributable to this drug that led to its withdrawal. Our experience suggests that patients un- DISCUSSION dergoing TED warrant close monitoring in order to detect possi- ble complications over time. In this case report we describe the first experience of a pa- tient with SBS receiving TED in our hospital. In the randomized, placebo-controlled trials of TED, responders were those subjects REFERENCES demonstrating a reduction ≥ 20 % in parenteral volumes from 1. Kelly DG, Tappenden KA, Winkler MF. Short bowel syndrome: Highlights baseline at weeks 20 and 24 (5,6). 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