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Volume 86 - 2023 - Fasc.3 - Clinical images

A remarkable presentation of a massive Budd-Chiari syndrome

A 59-year-old female presented to the emergency department with malaise, significant weight loss, abdominal discomfort, dyspnoea and severely swollen peripheral extremities. She had a past medical history of sickle cell anaemia, a latent tuberculosis infection and a chronic hepatitis B that was treated with PEG-interferon alpha-2 in 2008. Since 2018, she was lost to follow-up. The patient was critically ill, sarcopenic and lethargic. Clinical examination revealed icteric sclerae and a markedly distended and diffusely tender abdomen and peripheral oedemas. Her vital signs included a blood pressure of 105/64 mmHg, tachycardia of 130 bpm, hypothermia of 35.6°C and a SpO2 of 100% in ambient air. Laboratory workup demonstrated a total bilirubin of 12.9 mg/dL, AST 500 U/L, ALT 218 U/L, ALP 178 U/L and GGT 126 U/L, a thrombocytopenia of 34 x10E3/ μL, 16,01 x10E3/μL leukocytes, a CRP of 94.8 mg/L and a lactate of 10,12 mmol/L. The serum creatinine was 1.04 mg/dL, eGFR 54.1 ml/min. The INR was 2.77 and there was a severe hypoalbuminemia (22 g/L). D-dimers were >20 000 ng/ml. The patient presented also with spontaneous hypoglycaemia. A multiphasic contrast-enhanced thoracic and abdominal computed tomography (CT) was performed (figure 1). Given the results of the CT, a 2D echocardiogram was urgently requested (figure 2).


Melanoma of the gallbladder

An 80-year-old man, with no medical history, was referred to the gastroenterologist due to cholestasis and altered general condition. Physical examination revealed mild abdominal tenderness in the right hypochondrium, with no other clinical signs. Laboratory examinations showed a cholestasis (gamma-glutamyl transferase: 160 U/L and alkaline phosphatase: 120 U/L) and an elevated CRP level of 25 mg/L. Abdominal MRI was performed (Fig 1, Fig 2A). Subsequently, a PET-CT and brain MRI for seizures (Fig. 2B) were realized.


An underrecognized cause of hepatomegaly in uncontrolled type I diabetes

A 29-year-old woman with a past medical history of poorly controlled type 1 diabetes and autoimmune hypothyroidism presented to the emergency department for evaluation of abdominal pain, nausea and vomiting. The patient was a cocaine user, but she did not use alcohol and did not smoke. Her current medications included Insulin aspart, Insulin degludec, L-thyroxine and Pantoprazole. She reported no use of over-thecounter medication or herbal and dietary supplements. acute distress. The abdomen was nondistended and soft with diffusely tenderness and a smooth palpable liver 3 to 4 cm below the costal margin. Laboratory studies was significant for glucose 539 mg/dL (normal 65-110), alkaline phosphatase 153 U/L (normal 35-105), gamma-GT 44 U/L (normal <36), lactate 42,1 mg/dL (normal 4,5-19,8), arterial pH 7,32 (normal 7,35-7,45) and HbA1c 11% (4,5-6). A computed tomography (figure 1; panel A and B) and liver biopsy (figure 1; panel C and D) were performed.


An unusual cause of portal hypertension

A 66-year-old male with a medical history of coronary artery disease presented with nonspecific symptoms of anorexia, lethargy and an increase in abdominal girth. He was diagnosed 3 months ago with chronic liver disease of undetermined aetiology. He did not smoke and was a social drinker till 4 months ago. He was taking tests showed progressive cholestatic liver enzymes (total bilirubin 1,09 mg/dL, AST 130 U/L, ALT 129 U/L, GGT 517 U/L and ALP 641 U/L) and a severe hypoalbuminemia. Laboratory workup made a metabolic, autoimmune, and viral aetiology of the suspected cirrhosis less probable. Urinalysis showed a rapidly progressive proteinuria in the nephrotic range accompanied by the presence of serum spike of monoclonal lambda light Furosemide, Aldactone, Pantoprazole, Acetylcysteine and Atorvastatin. He had no personal or familial history of liver diseases. Physical examination revealed a tender but distended abdomen and large ascites was present. The laboratory chain protein on electrophoresis with immunofixation. Doppler echocardiography revealed a hypertrophic cardiomyopathy based on diffuse left and right ventricular hypertrophy with relative apical sparing. A diagnostic paracentesis was undertaken and the results were consistent with portal hypertension (SAAG 1,6 g/ dL). Considering the concomitant presence of hypertrophic cardiomyopathy, nephrotic syndrome and a presentation of portal hypertension of undetermined aetiology in presence of a monoclonal IgG lambda light chain spike, a transjugular liver biopsy (figure 1 and 2) was performed with HVPG measurement consistent with significant portal hypertension (15 mmHg).