A Rare Case of Congenital Choledochal Cyst (CCC) Resulting in Recurrent Acute Pancreatitis in an Otherwise Healthy Young Woman

Introduction: Congenital choledochal cyst (CCC) is a rare cystic dilatation of intrahepatic or extrahepatic biliary ducts. We present a case of a type IVb choledochal cyst presenting as recurrent acute pancreatitis in a young healthy female with initial negative screenings. Case Description/Methods: An 18-year-old-female with a history of COVID-19 presented to the emergency department with one month of persistent abdominal pain, nausea, and vomiting. She was hospitalized once prior for similar symptoms and was diagnosed with acute pancreatitis. This admission, blood work showed elevated lipase, elevated liver enzymes, mild bilirubinemia with a normal lipid panel and urine was significant for infection. She received fluids, antiemetics and was started on prophylactic antibiotics for ascending cholangitis. A right upper quadrant ultrasound ruled out cholelithiasis or acute cholecystitis, but showed dilation of the common bile duct. MRCP confirmed dilation with bulbous termination in the periampullary region diagnosed as type IVb choledochal cyst. Discussion(s): CCCs are rare in Western countries with an incidence between 1 in 100,000 to 150,000. 80% of these cysts are diagnosed in patients under the age of 10. They are difficult to diagnose due to variable clinical presentations. A study of 214 CCC patients demonstrated the most common symptom was abdominal pain, followed by jaundice and fever. When cysts are found in adults, symptoms resemble atypical acute biliary tract disease. Surgical cyst removal may be needed for patients with significant risk factors such as older age and age of symptom onset, due to increased risk of malignant transformation. Longer periods of observation have been documented to be associated with an increased chance of developing late complications, such as anastomotic stricture, biliary calculi and recurrent cholangitis. Type IVb CCCs, as seen in this case, consist of multiple extrahepatic cysts and hepaticojejunostomy is the treatment. This patient's young age and recurrent acute pancreatitis combined with her lab and imaging findings strongly suggest the diagnosis of CCC. The anatomical location of the CCC impeded flow of pancreatic enzymes through the ampulla of vater, leading to recurrent pancreatitis in an otherwise healthy young female. CCC, although very rare, should be considered in the differential of acute pancreatitis when other causes such as gallstones and heavy alcohol consumption cannot be identified, as prompt diagnosis and surgical removal is imperative.

Characteristics of MIS-C patient cohort in Slovenia and distinguishing features to other febrile illnesses

Introduction: Multisystem inflammatory syndrome in children (MISC) was recognized during the 2020 pandemic of SARS-CoV-2 and knowledge is still limited. Objectives: To report the characteristics of patients with MIS-C and in a cohort study in Slovenia and report distinctions in regard to patients with suspected MIS-C but a different final diagnosis. Methods: This is a prospective cohort study of consecutive patients with suspected MIS-C, admitted from March 2020 to January 2021 to University Medical Centre Ljubljana, Slovenia. The inclusion criteria for the study was suspected MIS-C on referral. Inclusion criteria for MIS-C group was meeting the WHO criteria and serology was used to confirm SARS-CoV-2 infection. Results: MIS-C group. 23 patients (14male, median age 12.4 yrs), all Caucasian were enrolled and prevalence of MIS-C was 5.8/100 000 persons younger than 19 years of age. Detailed analyses were available in 20 patients. Two patients were treated in ICU and none died. Four patients had symptomatic SARS-CoV-2 infection, all had positive serology. Troponin was elevated in 15/20(75%) patients during the disease course, 7/15(47%) of these had normal level at admission. Six patients (30 %) had elevated pancreatic enzymes, 1 patient developed asymptomatic acute pancreatitis (max serum lipase 25mkat/L). All had elevated levels of D-dimer with no signs of thrombosis. All patients received IVIG and systemic corticosteroids. Four patients (20%) received high dose methylprednisolone pulse therapy. Biologic therapy with anakinra was started in 2 patients. Nineteen patients (19/20, 95%) received acetylsalicylic acid and prophylactic anticoagulation was prescribed in 15/20 (75%) of patients. The mean follow up was 50 days (14 - 122). At the last follow-up visit all patients had normal laboratory parameters of inflammation, troponin, pro-BNP, ddimer values and normal heart function. Non MIS-C group. Thirteen patients (6M, median age 7.5 years), referred as MIS-C and later diagnosed with a different disease were included. The final diagnoses were: Campylobacter gastroenterocolitis (3), staphiloccocal sepsis (2), urinary tract infection (2), renal abscess (1), appenditicis (1), adenovirus tonsilopharyngitis (1), streptococcal faringitis (1), pancreatic mass (1) and TNF receptor-associated periodic syndrome (1). Group characteristics are shown in Table 1. The main differences between the groups hystory of SARS-CoV-2 infection, lip/mouth changes, conjunctivitis and later the presence of myocarditis. The initial inflammatory parameters did not differ between groups, but in the MIS-C group significantly lower values of platelets, sodium and albumins and higher values of troponin, pro-BNP and ferritin were noted at admission. Conclusion: A very high incidence of MIS-C, estimated 5.8/100 000 persons under the age of 19 with a predominantly cardiac involvement but very good outcome was noted in European Caucasian population in Slovenia. Attention to newly described pancreatic involvement should be raised. Very different diseases can have a similar presentation at onset and attention should be payed to the specifics of the condition.

Children's perspectives on learning to look after their diet and gut: A multicenter qualitative study

Background: Children with cystic fibrosis (CF) often begin asking questions about their CF at 6 to 7 years of age. Despite this, there has been limited research focusing on the learning needs and preferences of preadolescent children with CF. In this study, we explored preadolescent children’s experiences, views, and ideas on gradually learning to look after their diet and gut. Methods: In this qualitative study, children aged 6 to 11 requiring pancreatic enzyme replacement therapy (PERT) were recruited from 3 regional CF centers in England between September 2020 and April 2021. Child-facing study documentation was developed in collaboration with a young person’s advisory group and children with CF of the same age as potential participants. Because of COVID-19, semistructured interviews were adapted from in-person to video call at home and activity packs posted to children to help build rapport and promote engagement during interviews.Written assent and consent were obtained, and children chose to be interviewed alone or with their parent(s), who also took part in an interview. Childrenwere asked what they would like to learn or learn more about and how. Phrasing of questions was tailored to the developmental stage of each child. Interviews were audio-recorded, transcribed verbatim, and analyzed using framework analysis. Results: Interviews were conducted with 16 children. From analysis to date, emerging themes onwhat children would like to learn included begin making sense of what is happening inside their bodies (e.g., not only to learn to recognize gastrointestinal symptoms, but also to begin to understand why they are experiencing them), being independent with PERT when they transition to secondary school, knowing how much PERT they need with different foods and being in the habit of remembering to take it;and knowing which foods to choose that are right for them and having the confidence to do so. For some children, this included tips on howto eat more or try newfoods. Emerging themes on howchildren would like to learn included learning through play;very fewresourceswere being used to facilitate learning, but children would like to learn through playing games (e.g., be a detective solving a mystery, doing and watching experiments [e.g., what happens in the gut if you do not drink or drink a lot]), choosing actions for characters in different scenarios using animation or comic strips, learning with other children with CF, and learning from each other together online in small groups (e.g., sharing experiences of preparing to transition to secondary school, taking part in cook-a-longs, being able to ask questions they would not ask people without CF). Having role models and hearing positive stories and seeing and hearing about people with CF doing well would help motivate them to own their CF, although a gap in role models for young girls was highlighted. Conclusion: Preadolescent children were able to clearly articulate their learning needs and preferences for how their needs could be met. These findings, as part of a larger study, will inform development of a model for self-care support of diet and the gut for children with CF. The novel approach used to interview these children could be used in future studies to inform development of resources and services for children that are appropriate and relevant and respond to their needs.