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).
