Clinical Case Summary

A 30-year-old-female presented with a one-week history of jaundice and pedal edema. Initial laboratory investigations revealed elevated liver enzymes and total bilirubin of 37.90 mg/dL. Over the following three months her condition worsened, a transjugular liver biopsy revealed 50% necrosis, indicative of severe liver injury for which she underwent a Liver transplant. On POD 7 she developed severe thrombocytopenia (8000/μL) and neutropenia (858/μL), along with elevated LFTs (AST=473 IU/L, ALT=992 IU/L), challenging the post-operative management. Some differentials were Acute transplant rejection, CMV, infection, drug induced hepatotoxicity etc. CMV PCR testing was ordered which revealed a positive result, prompting antiviral therapy. Due to persistent pancytopenia a bone marrow biopsy was performed confirming the presence of hemophagocytosis, leading to a diagnosis of Hemophagocytic Lymphohistiocytosis. Diagnosis was supported by other Lab values (platlets=8000/μL, neutrophils=858/μL, LFTs; AST=473 IU/L, ALT=992 IU/L, fibrinogen=508 mg/dl and ferritin=2269 Ng/ml).
Appropriate treatment with high-dose steroids and etoposide was initiated promptly adhering to the HLH-2004 protocol [1], however the patients condition continued to worsen. On POD 16 she eventually succumbed to multi-organ failure due to mucormycosis and septic shock.
The incidence of HLH in liver transplant patients is not well documented, only forty-one previous post-LT HLH cases have been reported in present literature, out of which only 5 were initially diagnosed with acute liver failure [2,3] ,This case adds to the existing literature on HLH and highlights the importance of including HLH as a differential diagnosis in immunocompromised patients, especially in post-liver transplant cases, due to its severe outcomes. Integrating novel diagnostic tools like the HScore may enhance the ability to diagnose HLH in complex clinical situations [4], although they cannot replace astute clinical judgment.

References

1. Henter J, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb 25;48(2):124–31.
2. Chesner J, Schiano TD, Fiel MI, Crismale JF. Hemophagocytic lymphohistiocytosis occurring after liver transplantation: A case series and review of the literature. Clin Transplant. 2021 Aug 8;35(8).
3. Iseda N, Yoshizumi T, Toshima T, Morinaga A, Tomiyama T, Takahashi J, et al. Hemophagocytic syndrome after living donor liver transplantation: a case report with a review of the literature. Surg Case Rep. 2018 Dec 29;4(1):101.
4. Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome. Arthritis & Rheumatology. 2014 Sep 26;66(9):2613–20.

Introduction

Superior Mesenteric Artery Syndrome (SMAS), also known as Wilkie’s syndrome, is a rare gastrointestinal condition characterized by compression of the distal duodenum between the abdominal aorta (AA) and the superior mesenteric artery (SMA). Evidence on Wilkie’s syndrome is limited, reviews are based on small cohorts and case studies^1-3. Patients with SMAS typically present with postprandial epigastric discomfort, nausea, vomiting, stomach distention, and reflux, leading to malnutrition and weight loss. As mesenteric fat decreases due to the weight loss, the duodenal obstruction worsens, increasing SMAS symptoms. SMAS mostly affects young females aged 20-30 years and is challenging to diagnose due its overlap with dyspepsia, anorexia nervosa and median arcuate ligament syndrome^1-3. SMAS can present acutely, after weight loss from bariatric surgery, malnutrition or (hyper)metabolic disorders. It also presents chronically, due to an anatomically high duodenal position or a low aortic SMA origin^1-3. Diagnostic imaging, including computed tomography (CT) typically reveals a reduced SMA-AA angle <25° and a characteristic vertical, abrupt duodenal obstruction on barium X-ray series^1,2. Conservative treatment focuses on improving gastrointestinal motility and increasing the SMA-AA angle by weight gain to regain mesenteric fat¹. If conservative treatment fails, surgical intervention is considered, with duodenojejunostomy being most commonly performed. Yet, this carries potential severe complications such as anastomotic leakage and non-physiological gastro-duodenal changes. Recent evidence suggests laparoscopic Treitz ligament release may offer a viable and low-risk alternative^3,4.

Aims & Methods

This study aims to introduce a standardized, low risk treatment protocol for SMAS. The protocol includes: 1) Jejunal tube feeding for six-eight weeks, 2) Laparoscopic release of the Treitz ligament, and 3) SMA reimplantation⁵. The protocol was implemented at three hospitals in the Netherlands, Medisch Spectrum Twente, Franciscus, and Erasmus MC.

Results

A total of 12 patients were treated according to this protocol from 2020 until 2024. The cohort consisted of 7 (58.3%) female patients, with a mean age of 29.2 years (SD 11.8). The average weight and body mass index were 60.33 kg (SD 6.5) and 20.01 (SD 3.4). The mean SMA-AA angle measured on CT scans was 22.55° (SD 13.1). All patients started treatment with jejunal feeding. Nine patients underwent laparoscopic Treitz ligament release, with 7 experiencing complete resolution of symptoms. One patient with co-existing Nutcracker syndrome received primary SMA infra-renal reimplantation, with complete symptom release after the surgery. In two patients, symptoms persisted following Treitz ligament release, resembling gastroparesis and irritable bowel syndrome. The 2 remaining patients are planned for laparoscopic Treitz ligament release.

Conclusion

The combination of jejunal feeding followed by laparoscopic Treitz ligament release, represents a safe and effective treatment approach for SMAS; 7/9 patients who underwent the Treitz ligament release experienced resolution of all symptoms, demonstrating the efficacy of protocol. This approach offers an alternative to traditional surgical procedures, such as duodenojejunostomy, and reduces the risks of substantial short- and long-term complications. Further research with larger sample sizes is required to confirm the long-term outcomes and advantages of this protocol compared to other treatment strategies for SMAS.

References

1. Oka A, Awoniyi M, Hasegawa N, Yoshida Y, Tobita H, Ishimura N, Ishihara S. Superior mesenteric artery syndrome: Diagnosis and management. World J Clin Cases. 2023 May 26;11(15):3369-3384. doi: 10.12998/wjcc.v11.i15.3369. PMID: 37383896;
2. Navandhar PS, Shinde RK, Gharde P, Nagtode T, Badwaik N. Understanding Superior Mesenteric Artery Syndrome: Etiology, Symptoms, Diagnosis, and Management. Cureus. 2024 Jun 2;16(6):e61532. doi: 10.7759/cureus.61532. PMID: 38957238; PMCID: PMC11218894
3. Beita AKV, Whayne TF. The Superior Mesenteric Artery: From Syndrome in the Young to Vascular Atherosclerosis in the Old. Cardiovasc Hematol Agents Med Chem. 2019;17(2):74-81. doi: 10.2174/1871525717666190920100518. PMID: 31538906.
4. Fang JF, Wei B, Zheng ZH, Lei PR, Chen TF, Wei HB. Laparoscopic lysis of the ligament of treitz for superior mesenteric artery syndrome. Dig Surg. 2014;31(4-5):291-6. doi: 10.1159/000367670. Epub 2014 Oct 17. PMID: 25342434.
5. Ali T, Tomka J, Bakirli I, Bakirov I. Surgical Treatment of Wilkie's Syndrome by Vascular Transposition. Cureus. 2022 Apr 18;14(4):e24251. doi: 10.7759/cureus.24251. PMID: 35475250; PMCID: PMC9018456.
6. Pourhassan S, Grotemeyer D, Fürst G, Rudolph J, Sandmann W. Infrarenal transposition of the superior mesenteric artery: a new approach in the surgical therapy for Wilkie syndrome. J Vasc Surg. 2008 Jan;47(1):201-4. doi: 10.1016/j.jvs.2007.07.037. Epub 2007 Oct 18. PMID: 17949939

Clinical Case Summary

Background: Syphilis is a chronic systemic infection caused by the spirochete Treponema pallidum, often referred to as "the great imitator" due to its ability to affect nearly any organ. Syphilitic hepatitis, or "luetic jaundice," is a rare manifestation, indicating spirochete dissemination to the liver.
Case Report: A 26-year-old female was admitted to the hospital with fever (39.5 °C), fatigue, sweating, tremor, dark urine, pale stools, and significant weight loss (15 kg over two months). She also reported lower extremity muscle and joint pain, cough and slowing of speech. On examination, she had icteric skin, a generalized maculopapular rash, and enlarged cervical lymph nodes. No history of alcohol or drug use was noted.
Laboratory tests revealed elevated levels of both cytolytic and cholestatic liver enzymes, along with increased bilirubin an elevated C-reactive protein.
Abdominal ultrasound and contrast CT showed hepatomegaly, hilar and retroperitoneal lymphadenopathy and splenomegaly.
Viral hepatitis markers (HBsAg, HBV PCR, HCVAb, HCV PCR, anti-HAV IgM, anti-HEV IgM) and antibodies for CMV, EBV, HIV, HSV-1,2 were negative. Wright-Hedelson test for brucellosis and serological markers for autoimmune hepatitis were negative while IgG and cerulopasmin levels were within normal range. Given systemic findings and history, syphilis was considered. Serologic tests were positive for Treponema pallidum (ELISA, TPHA, MRP). Liver biopsy revealed mild active hepatitis. The diagnosis of ^secondary syphilitic hepatitis was made. The patient was started on antimicrobial therapy. During follow-up, liver function tests normalized, and systemic symptoms resolved.
Conclusion: This case underscores the importance of considering syphilis in the differential diagnosis of unexplained hepatitis. Although rare, syphilitic hepatitis should be considered in patients with liver enzyme elevations and nonspecific symptoms, particularly when no clear alternative diagnosis is found.

References

1) Malvar G, Cardona D, Pezhouh MK, Adeyi OA, Chatterjee D, Deisch JK, Lamps LW, Misdraji J, Stueck AE, Voltaggio L, Gonzalez RS. Hepatic Secondary Syphilis Can Cause a Variety of Histologic Patterns and May Be Negative for Treponeme Immunohistochemistry. Am J Surg Pathol. 2022 Apr 1;46(4):567-575. doi: 10.1097/PAS.0000000000001848. PMID: 34864775.
2)Supronowicz Ł, Rogalska M. Syphilitic hepatitis. Clin Exp Hepatol. 2024 Mar;10(1):9-13. doi: 10.5114/ceh.2024.136235. Epub 2024 Mar 12. PMID: 38765910; PMCID: PMC11100338.
3)Al Dallal HA, Narayanan S, Alley HF, Eiswerth MJ, Arnold FW, Martin BA, Shandiz AE. Case Report: Syphilitic Hepatitis-A Rare and Underrecognized Etiology of Liver Disease With Potential for Misdiagnosis. Front Med (Lausanne). 2021 Nov 29;8:789250. doi: 10.3389/fmed.2021.789250. PMID: 34912834; PMCID: PMC8666961.
Figure 1: Histological images of the liver biopsy
Figure 2: Abdominal CT images
Figure 3: Images of maculopapular rash

Introduction

Inflammatory Bowel Disease (IBD) is emerging as a significant but under-recognized health challenge in Africa. Diagnostic delays, limited awareness, and a lack of educational opportunities for healthcare professionals persist. The Inaugural Inflammatory Bowel Disease in Africa Symposium was held on April 10th in Abuja, Nigeria, to foster collaboration in IBD diagnosis and management, promote awareness of medical and surgical care options, and support patient advocacy.

Aims & Methods

A hybrid (in-person and virtual) full-day symposium was designed and developed, bringing together specialists in gastroenterology, surgery, nursing, pathology, radiology, and oncology from across the continent. The program featured expert-led lectures and multidisciplinary team case-based discussions. A post-symposium electronic survey was deployed to assess participants’ satisfaction, knowledge gains, practice impacts, and IBD management challenges and provide recommendations for enhancing future symposia. Quantitative responses were analyzed descriptively; qualitative feedback was thematically categorized.

Results

A total of eighty-four individuals registered, with speakers and faculty representing Nigeria, South Africa, Ghana, Zambia, Zimbabwe, the United States, the United Kingdom, Egypt, and Kenya. Fifty-eight (69%) of 84 completed the survey. These comprise nurses (34.5%), surgeons (27.6%), gastroenterologists (17.2%), radiologists (8.6%), pathologists (6.9%), and oncologists (5.2%). Overall, 93% rated the program as Satisfied, with 88% reporting significant knowledge gains in African-context IBD management. Diagnostic and treatment confidence improved in 83% and 76% of participants, respectively, with 69% gaining skills in biopsy techniques and 67% differentiating IBD mimickers. Major challenges reported included limited access to endoscopy and histopathology, low disease awareness among patients, high diagnostic costs, misdiagnosis as infectious diarrhea or tuberculosis, and lack of referral networks. The absence of multidisciplinary care teams was also noted. A strong majority (95%) supported establishing an African IBD registry, emphasizing the need for sustainable funding, technical infrastructure, and training. Hybrid learning and case-based approaches were preferred for future symposia, with interest in pediatric IBD and resource-adapted care.

Conclusion

The symposium significantly enhanced knowledge and confidence in IBD care among participants while exposing critical gaps in diagnostics, treatment access, and care coordination. These findings highlight the need for sustained collaborative efforts to strengthen IBD education and care. Supporting regional training and cross-disciplinary engagement will be essential to building capacity, improving outcomes, and advancing equitable IBD management across the continent.

Disclosure

Nothing to disclose

Clinical Case Summary

A 64-year-old male patient presented with acute epigastric pain over 7 days, worsening after meals, nausea, vomiting 2 times without signs of bleeding, diarrhea 5-6 times/day, high blood pressure 180/100 mmHg. He had a 6-year history of recurrent epigastric pain, after multiple EGDSs was diagnosed as gastric ulcer and atrophic gastritis. The patient had undergone HP eradication and received standard anti-ulcer therapy several times, with no clinical improvement. On physical examination, the patient appeared pale. The abdomen was soft, without pain, guarding or rebound tenderness. The patient's medical history was significant for arterial hypertension. Lab abnormalities included WBC 16.32-10x10⁹/L, ESR 94mm/sec, CRP at 184.3 mg/L, FBG 767 mg, Total protein 50.9 g/L, CREA 147 mmol/L, Urea 13.42 mmol/L, Na 129 mmol/L. Intestinal infections were negative. New EGDS revealed an ulcerative lesion with 0.8cm diameter, in the region from antrum to greater curvature, covered with fibrin. Colonoscopy was normal. Duplex ultrasound revealed a 70% stenosis of the celiac trunk and superior mesenteric artery, with atherosclerotic wall deformation and intima-media thickening. A thrombosis was detected in the region of the aortic bifurcation. CT angiography revealed up to 70% stenosis of the suprarenal abdominal aorta, no contrast enhancement was observed in the origins of the celiac trunk, superior mesenteric artery, left renal artery (left kidney appeared atrophic), total occlusion of bilateral common, external, and internal iliac arteries.
Conclusion: Gastric ulcers are mostly associated with HP. However, in rare cases, underlying vascular pathology may be critical in their pathogenesis. This case highlights an overlooked cause of gastric ulcers, particularly in patients with atherosclerotic risk factors. In patients with persistent symptoms despite medical therapy ischemic ulceration should be included in the differential diagnosis.