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Peculiar dermatologic manifestations are present in several heritable gastrointestinal disorders. Muir–Torre syndrome (MTS) is a genodermatosis whose peculiar feature is the presence of sebaceous gland tumors associated with visceral malignancies. We describe one patient in whom multiple sebaceous gland tumors were associated with early onset colon and thyroid cancers and attenuated polyposis coli. Her family history was positive for colonic adenomas. She had a daughter presenting with yellow papules in the forehead region developed in the late infancy. Skin and visceral neoplasms were tested for microsatellite instability and immunohistochemical status of mismatch repair (MMR), APC and MYH proteins. The proband colon and skin tumors were microsatellite stable and showed normal expression of MMR proteins. Cytoplasmic expression of MYH protein was revealed in colonic cancer cells. Compound heterozygosity due to biallelic mutations in MYH, R168H and 379delC, was identified in the proband. The 11-year-old daughter was carrier of the monoallelic constitutional mutation 379delC in the MYH gene; in the sister, the R168H MYH gene mutation was detected. This report presents an interesting case of association between MYH-associated polyposis and sebaceous gland tumors. These findings suggest that patients with MTS phenotype that include colonic polyposis should be screened for MYH gene mutations. [ABSTRACT FROM AUTHOR]

The effect of intraperitoneal injection of d,l- or d-β-hydroxybutyrate on feed intake and plasma metabolites was investigated in pygmy goats. The combined intraperitoneal injection of d,l-βhydroxybutyrate or d-β-hydroxybutyrate (15 mmol/kg0.75) and 1,3-butanediol (6.6 mmol/kg0.75), a ketogenic substrate, decreased cumulative food intake while the same dose of 1,3-butanediol or dl-β-hydroxybutyrate alone had no effect. The decrease in food intake after combined injection of d-β-hydroxybutyrate and 1,3-butanediol was characterized by a significant decrease in meal frequency and a prolongation of the latency to eat. The hypophagic effect of the combined injection of d-β-hydroxybutyrate and 1,3-butanediol was significant for 8 h, while the hypophagia after d,l-β-hydroxybutyrate and 1,3-butanediol was significant for only 2 h after injection. Injection of d,l-β-hydroxybutyrate increased plasma d-β-hydroxybutyrate levels to 0.55 mmol/l and decreased plasma free fatty acids. Addition of 1,3-butanediol (6.6 mmol/kg0.75) to the injection increased plasma d-β-hydroxybutyrate level up to 0.75 mmol/l. The results show that parenteral administration of d-hydroxybutyrate reduces feed intake in pygmy goats and that the hypophagia appears to be related to the amount of d-β-hydroxybutyrate injected. The hypophagia seems to be related to elevated plasma d-β-hydroxybutyrate concentration, and the threshold concentration appeared to be about 0.7 mmol/l under the experimental conditions of this study. [ABSTRACT FROM AUTHOR]

The combined effect of the histamine receptor antagonists Dexbrompheniramine (DXB: H1-receptor antagonist) and Cimetidine (C: H2-receptor antagonist) on food and water intake was investigated in pygmy goats. DXB (1 mg/kg BW0.75) and C (16 mg/kg BW0.75) were injected together intraperitoneally (i.p.)-Cumulative food and water intake, as well as meal and draft pattern, were recorded. DXB and C significantly reduced cumulative water intake, whereas cumulative food intake did not change. Water to food ratio was also significantly diminished. Draft frequency and the percentage of drafts associated with meals were significantly reduced during the 6 h post injection, while meal frequency and meal size did not change during this period. The results show that blockade of the H1 and H2-histamine receptors attenuates the association between water and food intake in pygmy goats. Therefore, mechanisms responsible for meal-associated drinking seem to depend upon activation of histamine receptors. [ABSTRACT FROM AUTHOR]

The standard management for relapsed or refractory classical Hodgkin lymphoma (cHL) is salvage therapy followed by autologous stem cell transplantation (ASCT). This strategy allows almost 50% of patients to be cured. Post-ASCT maintenance treatment with brentuximab vedotin (BV) confers improved progression-free survival (PFS) to cHL patients at high risk of relapse. We investigated the outcome of 105 cHL patients receiving post-ASCT BV maintenance in the real-life setting of 23 Italian hematology centers. This population included naïve patients and those previously exposed to BV. Median follow-up was 20 months. Patients presented a median of two lines of treatment pre-ASCT, with 51% receiving BV. Twenty-nine percent of patients had at least two high-risk factors (refractory disease, complete response [CR] less than 12 months, extranodal disease at relapse), while 16% presented none. At PET-CT, a Deauville score (DS) of 1-3 was reported in 75% and 78% of pre- and post-ASCT evaluations, respectively. Grade 3-4 adverse events (AEs), mainly peripheral neuropathy, were observed in 16% of patients. Three-year PFS and overall survival (OS) were 62% and 86%, respectively. According to BV exposure, 3-year PFS and OS were 54% and 71%, respectively, for naïve and 77% and 96%, respectively, for previously exposed patients. Refractory disease (hazard ratio [HR] 4.46; p = 0.003) and post-ASCT DS 4-5 (HR 3.14; p = 0.005) were the only two factors significantly associated with PFS reduction in multivariable analysis. Post-ASCT BV maintenance is an effective, safe treatment option for cHL naïve patients and those previously exposed to BV., (© 2021 The Authors. Hematological Oncology published by John Wiley & Sons Ltd.)

Intracerebroventricular (I.C.V.) administration of 2-deoxy-D-glucose (2-DG) produces glucose deprivation-induced ('glucoprivic') feeding and sympathoadrenal activation. To find out whether peripheral mechanisms modulate centrally elicited glucoprivic feeding the effect of hepatic branch vagotomy (HBV) compared with sham vagotomy (SV) on the feeding response to I.C.V.-injected 2-DG (1.2 or 2.4 mg per rat) was tested in rats fed a fat-enriched diet (18% fat). 2-DG was injected into the lateral ventricle 1 h after after dark onset, at light onset or 6 h after light onset of the 12 h light-12 h dark cycle. Apart from one (1.2 mg 2-DG injected 1 h after dark onset) out of six experiments, HBV enhanced glucoprivic feeding. This finding is consistent with the assumption that centrally elicited glucoprivic feeding is antagonized by peripheral stimuli signalled to the brain through the hepatic vagus branch.