Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia

Background Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID). Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH. Case presentation A 24-year-old man was diagnosed with CVID at the age of 1.25 years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750–1200 mg/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22 years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure. Conclusions Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients.

Large Primary Neuroendocrine Tumor of the Liver in a 57-year-Old Female Presenting With MSSA Bacteremia

Primary neuroendocrine tumors (NETs) are rare forms of malignancy, representing just .5% of known cancers and having an overall incidence of 0.2/100,000. The most common sites of origin are bronchopulmonary and gastrointestinal, most commonly the appendix, pancreas, and ileum. We report the case of a 57-year-old female who was admitted for refractory MSSA bacteremia and several weeks of abdominal pain. CT imaging done on presentation demonstrated a 12.5 x 19.4 x 17.3 cm heterogeneous right liver mass with associated mass effect. The patient was taken to the operating room and a right hepatectomy and cholecystectomy were performed without complication. Histological examination revealed necrotic tumor in sheets and nests with marked nuclear pleomorphism. Immunohistochemistry demonstrated positive staining for pancytokeratin, synaptophysin, chromogranin, and TTF-1, consistent with undifferentiated NET. While rare, NETs can originate from a variety of organs outside the gastrointestinal and bronchopulmonary tract, including the liver.

Prenatal yolk corticosterone exposure promotes skeletal growth and induces oxidative imbalance in yellow-legged gull embryos

Maternally-derived hormones induce variation in offspring phenotype, with consequences that can carry-over into post-natal life and even into adulthood. In birds, maternal egg corticosterone (CORT) is known to exert contrasting effects on offspring morphology, physiology and behaviour after hatching. However, information on the effects of CORT exposure on pre-hatching embryonic development is limited. We experimentally increased yolk CORT levels in yellow-legged gull (Larus michahellis) eggs, and assessed the effects on embryo pre-hatching development and oxidative status of brain and liver. CORT-supplemented embryos reached a larger skeletal size and liver mass compared to controls. Embryos from CORT-injected last-laid eggs showed decreased activity of the hepatic antioxidant enzymes superoxide dismutase and catalase, while intermediate-laid eggs showed an increased levels of lipid peroxidation. However, elevated yolk CORT did not affect oxidative stress endpoints in the brain. Our results indicate that elevated yolk CORT levels affect prenatal embryo development by promoting skeletal growth, and induce laying sequence- and organ-specific oxidative imbalance, with potential adverse consequences during postnatal life especially for late-hatched offspring.