Table 1:

Chronology of events for a 28-year-old woman with familial Mediterranean fever

Date	Admission duration, d	Symptoms			Laboratory abnormalities^*		Diagnosis	Investigations, procedures, interventions, intraoperative findings, pathology, complications and treatments
Date	Admission duration, d	Fever	Abdominal pain	Other	Max. CRP	Other	Diagnosis
February 2015	Outpatient genetics	No	No	Back pain, hypermobility	NA	EDS gene panel negative	Hypermobile EDS
March 2015	Outpatient medicine	No	Yes	Back pain, shortness of breath	NA	Hemoglobin 74 g/L	NSAID-induced gastric ulceration	Endoscopy
July 2016	6	Yes	Yes, LLQ	No	163	Lipase 100–155 U/L	Preoperative: ovarian torsion Postoperative: endometritis and urinary retention	Ultrasonography of abdomen and pelvis: left ovarian cyst, could not rule out torsion Laparoscopic ovarian cystectomy: left ovarian hemorrhagic cyst, no torsion Complications: postoperative fever, urinary retention and pain treated as endometritis
July–August 2016	19	No	Yes, RLQ	Nausea, vomiting	82	NA	Admission: appendicitis Discharge: abdominal pain NYD, postoperative ileus	Ultrasonography of abdomen: hyperemia to appendix, free fluid in RLQ Laparoscopic appendectomy: no evidence of appendicitis Pathology: mild inflammatory changes, lymphoid hyperplasia Complications: postoperative abdominal pain with ileus
October 2016	35	Yes (after procedure)	Yes, RUQ	Nausea, vomiting	201	Lipase 75–81 U/L, amylase 143 IU/L	Admission: pancreatitis Discharge: sphincter of Oddi dysfunction	Ultrasonography of abdomen: gall bladder wall thickening, biliary sludge ECRP and stent placement with papillotomy Complications: postprocedure spike in CRP, RLQ pain and fever
February–March 2017	51	Yes	Yes, RUQ	Nausea, vomiting	111	Lipase 102–189 U/L	Admission: cholecystitis, pancreatitis Discharge: malnutrition, culture-negative sepsis	Ultrasonography of abdomen: gall bladder wall thickening, biliary sludge Cholecystectomy Pathology: lymphocytic inflammatory infiltrate PICC line insertion Complications: postprocedure fever diagnosed as culture-negative sepsis Sepsis treated with antibiotics
May 2017	6	No	No	Chest pain, shortness of breath	NA	NA	Bilateral pleural effusions, cause unclear	Chest radiography: pleural effusions, lung fields clear Supportive, low-flow oxygen for 48 h
August 2017	14	Yes	Yes	Obstipation, abdominal distention	NA	Lipase 77–220 U/L	Admission: volvulus Discharge: transverse colon dilatation secondary to EDS and constipation	CT of abdomen: concerning for sigmoid volvulus Sigmoidoscopy, colonoscopy, exploratory laparotomy: no volvulus evident, redundant colon, thinning of bowel wall Complications: postoperative pain and fever
October 2017	7	Yes	Yes, epigastric	Left hand pain, redness, swelling	55	Leukocyte 16 × 10⁹/L	Cellulitis	Intravenous antibiotics, analgesia
November 2017	10	Yes	No	Neck pain, headache	NA	Leukocyte 18 × 10⁹/L	Septic thrombophlebitis (Lemierre syndrome)	Ultrasonography of neck: evidence of clot extending to right jugular vein and tributaries Intravenous antibiotics
December 2017	6	Yes	Yes	Headache	NA	Blood culture positive for CONS	Admission: central line infection Discharge: bacteremia	CT of head: normal Lumbar puncture, PICC line removal, port-a-cath insertion Intravenous antibiotics
December 2017	34	Yes	Yes, RUQ	Headache, blurred vision, constipation, weight loss	NA	Lipase 103 U/L, AST and ALT > 1000 U/L	Admission: small bowel obstruction, possible stroke Discharge: small bowel obstruction, migraine	MRI brain with MRA and MRV: normal Ultrasonography of abdomen: hepatomegaly, splenomegaly Abdominal radiography: air fluid levels, concerning for small bowel obstruction. Laparoscopy with lysis of adhesions Gastrostomy tube insertion
December 2017	4	No	Yes	Headache, nausea, feeding intolerance	NA	NA	Colonic and gastric dysmotility, feeding intolerance	Gastrostomy tube replaced with gastrojejunal tube
April 2018	87	Yes	Yes	Abdominal distension, nausea	88	Blood culture positive for Escherichia coli, Enterococcus species	Admission: sepsis Discharge: colonic distension with bacterial translocation, bacteremia	CT of abdomen: redundant sigmoid colon, colonic distension, free fluid Total colectomy and ileostomy Complications: postoperative fevers, pain and feeding intolerance
November 2018	21	Yes	Yes, RLQ	NA	76	NA	Admission: ileostomy reversal Discharge: postoperative pain, surgical site infection	Planned Ileostomy reversal Intravenous antibiotics Complications: postoperative RLQ pain, fever managed as infection
November–December 2019	Recurrent outpatient emergency visits	Yes	Yes	Pelvic pain, tenesmus, bloody diarrhea	94	NA	Pouchitis	CT of abdomen and pelvis: bowel wall thickening consistent with pouchitis Pouchoscopy: colonic mucosal inflammation, lymphoid aggregation Intravenous and oral antibiotics, intravenous steroids
March 2020	Emergency department visit	Yes	Yes, RLQ	Pelvic pain, urinary retention	83	NA	Pouchitis	CT of abdomen and pelvis: RLQ fluid collection concerning for infection, abscess Analgesia, discharge with oral antibiotics, intermittent self-catheterization
March–May 2020	47	Yes	Yes	Chest pain, vomiting, rectal bleeding	151	ALT 134–368 U/L Blood culture positive for E. coli	Urosepsis	Ultrasonography of abdomen: hepatomegaly (19.6 cm), splenomegaly (15.5 cm) CT of chest: pericardial effusion, pulmonary nodules
June–December 2020	Outpatient genetics	No	No	NA	NA	NA	FMF	Whole exome sequencing (GeneDx Laboratory, United States): positive for mutations causing FMF Urgent referral to rheumatology, colchicine started

Note: ALT = alanine aminotransferase, AST = aspartate aminotransferase, CONS = coagulase-negative Staphylococcus aureus, CRP = C-reactive protein, CT = computed tomography, EDS = Ehlers–Danlos syndrome, ERCP = endoscopic retrograde cholangiopancreatography, FMF = familial Mediterranean fever, LLQ = left lower quadrant, Max. = maximum, MRA = magnetic resonance angiography, MRI = magnetic resonance imaging, MRV = magnetic resonance venography, NA = not measured or not relevant, NSAID = nonsteroidal anti-inflammatory drug, NYD = not yet diagnosed, PICC = peripherally inserted central catheter, RLQ = right lower quadrant, RUQ = right upper quadrant.
↵* Normal reference ranges are as follows: ALT = 7–55 U/L, amylase = 28–100 U/L, AST = ≤ 32 U/L, CRP = 3–5 mg/L, hemoglobin = 115–160 g/L, lipase = 13–60 U/L, leukocyte = 4–10 × 10⁹/L.