Progressive familial intrahepatic cholestasis type 2 (PFIC2) is an autosomal recessive disorder typically presenting in infancy with cholestasis and rapidly progressing to cirrhosis. PFIC has been associated with an elevated risk of hepatocellular carcinoma (HCC), a neoplasm that is uncommon in children. PFIC type 4 has the strongest link to this type of cancer, although a few cases have also been connected to PFIC2. Herein, we report the case of a 2-year-old boy who underwent liver transplantation due to PFIC2. Histological examination showed cirrhosis and four small HCCs. Over a 20-year period following the transplantation, there was no recurrence of the disease or HCC. Although rare, HCC development can occur in PFIC and may complicate the prognosis. Liver transplantation offers a potential cure for both the metabolic disease and the neoplasm.

OBJECTIVES: The purpose of this study was to evaluate the effects of hyperthyroidism and their etiology on bone mineral density (BMD), on body soft tissue composition, on the prevalence of vertebral fractures detected by vertebral fracture assessment (VFA) and on the trabecular bone score (TBS). METHODS: From an initial population of 119 Portuguese men (78 with hyperthyroidism [HT]+ 41 controls [CTs]) admitted to the Endocrinology Department we selected 41 men aged over 50 with clinical hyperthyroidism to participate; each one was matched by age and height with a control person. BMD (g/cm²) at the lumbar spine, hip, radius 33% and whole body and the total body masses (kg) were studied by dual-energy X-ray absorptiometry (DXA). VFA with Genant semiquantitative method was used to detect fractures. The TBS was obtained from lumbar spine DXA images. No patient had been treated previously for hyperthyroidism or osteoporosis. Adequate statistical tests were used. RESULTS: In the hyperthyroidism group, total lean mass (CT 58.16 ± 7.7 vs. HT 52.3 ± 5.7, P = 0.03) and distal radius BMD (CT 0.769 ± 0.05 vs. HT 0.722 ± 0.08, P = 0.005) were lower; there was a significantly higher prevalence of osteoporosis (CT 9.7% vs. HT 29.3%, P = 0.015) and vertebral fractures (CT 2.4% vs. HT 24.4%, P = 0.007). TBS was similar in both groups (CT 1.328 ± 0.11 vs. HT 1.356 ± 0.11, P = not significant). Comparing patients with Graves' disease with patients with toxic goiter, there were no differences regarding BMD, BMD qualification, prevalence of fractures and TBS and just total lean mass was significantly lower in patients with Graves' disease. CONCLUSIONS: These results suggest that in a group of hyperthyroid men aged over 50 there are significant decreases in cortical bone BMD and lean mass and a higher prevalence of osteoporosis and silent vertebral fractures, but the etiology of the hyperthyroidism does not seem to influence it. Besides the antithyroid drugs, some patients may benefit from bone-directed treatments.
Absorptiometry, Photon ; Antithyroid Agents ; Bone Density ; Endocrinology ; Goiter ; Graves Disease ; Hip ; Humans ; Hyperthyroidism* ; Male ; Methods ; Osteoporosis* ; Prevalence* ; Radius ; Spine

Background: Intraoperative fluids are still poorly studied in veterinary medicine. In humans the dosage is associated with significant differences in postoperative outcomes. Objectives: The aim of this study is to verify the influence of three different fluid therapy rates in dogs undergoing video-assisted ovariohysterectomy. Methods: Twenty-four female dogs were distributed into three groups: G5, G10, and G20.Each group was given 5, 10, and 20 mL·kg −1 ·h −1 of Lactate Ringer, respectively. This study evaluated the following parameters: central venous pressure, arterial blood pressure, heart rate, respiratory rate, temperature, acid-base balance, and serum lactate levels. Additionally, this study evaluated the following urinary variables: urea, creatinine, protein to creatinine ratio, urine output, and urine specific gravity. The dogs were evaluated up to 26 h after the procedure. Results: All animals presented respiratory acidosis during the intraoperative period. The G5 group evidenced intraoperative oliguria (0.80 ± 0.38 mL·kg−1 ·h−1 ), differing from the G20 group (2.17 ± 0.52 mL·kg −1 ·h −1) (p = 0.001). Serum lactate was different between groups during extubation (p = 0.036), with higher values being recorded in the G5 group (2.19 ± 1.65 mmol/L). Animals from the G20 group presented more severe hypothermia at the end of the procedure (35.93 ± 0.61°C) (p = 0.032). Only the members of the G20 group presented mean potassium values below the reference for the species. Anion gap values were lower in the G20 group when compared to the G5 and G10 groups (p = 0.017). Conclusions The use of lactated Ringer's solution at the rate of 10 mL·kg −1 ·h −1 seems to be beneficial in the elective laparoscopic procedures over the 5 or 20 mL·kg−1 ·h−1 rates of infusion.

Background: Intraoperative fluids are still poorly studied in veterinary medicine. In humans the dosage is associated with significant differences in postoperative outcomes. Objectives: The aim of this study is to verify the influence of three different fluid therapy rates in dogs undergoing video-assisted ovariohysterectomy. Methods: Twenty-four female dogs were distributed into three groups: G5, G10, and G20.Each group was given 5, 10, and 20 mL·kg −1 ·h −1 of Lactate Ringer, respectively. This study evaluated the following parameters: central venous pressure, arterial blood pressure, heart rate, respiratory rate, temperature, acid-base balance, and serum lactate levels. Additionally, this study evaluated the following urinary variables: urea, creatinine, protein to creatinine ratio, urine output, and urine specific gravity. The dogs were evaluated up to 26 h after the procedure. Results: All animals presented respiratory acidosis during the intraoperative period. The G5 group evidenced intraoperative oliguria (0.80 ± 0.38 mL·kg−1 ·h−1 ), differing from the G20 group (2.17 ± 0.52 mL·kg −1 ·h −1) (p = 0.001). Serum lactate was different between groups during extubation (p = 0.036), with higher values being recorded in the G5 group (2.19 ± 1.65 mmol/L). Animals from the G20 group presented more severe hypothermia at the end of the procedure (35.93 ± 0.61°C) (p = 0.032). Only the members of the G20 group presented mean potassium values below the reference for the species. Anion gap values were lower in the G20 group when compared to the G5 and G10 groups (p = 0.017). Conclusions The use of lactated Ringer's solution at the rate of 10 mL·kg −1 ·h −1 seems to be beneficial in the elective laparoscopic procedures over the 5 or 20 mL·kg−1 ·h−1 rates of infusion.

The jugular foramen (JF) is located between the temporal and occipital bones. The JF is a primary pathway for venous outflow from the skull and passage of nerves. Variations are common in this region and may have clinical and surgical implications. To analyze the sexual dimorphism and JF morphology in skulls from Northeastern Brazil. 128 human skulls from the Anatomy Laboratory of the Federal University of Paraíba, 64 male and 64 female, were selected and the JFs analyzed for bone septation and the presence of a dome. Data analysis considered P<0.05 as significant. On at least one side, complete septation was observed in 26 skulls (20.3%), incomplete septation in 93 skulls (72.6%) and 61 skulls (47.6%) did not present septation. In 114 skulls (89%), 47.6% female and 41.4% male, have a unilateral presence of the dome and 71 (55.4%) have it bilaterally. Posterolateral compartment diameters and JF area had higher values on the right side in the total sample and separated by sex (P<0.05). Most morphometric variables of the anteromedial compartment were higher in male than in female (P<0.05), fact that was not observed in the posterolateral compartment (P>0.05). This study showed a higher prevalence of complete septation in males compared to females. Morphometric analysis presented a peculiar morphology of the JF in this study. These results suggests that the surgical approach to diseases that affect the JF may be peculiar to the studied population, confirming the importance of morphological analysis of the skull base.

Transanal endoscopic microsurgery is considered a safe, appropriate, and minimally invasive approach, and complications after endoscopic microsurgery are rare. We report a case of sepsis and pneumoretroperitoneum after resection of a rectal lateral spreading tumor. The patient presented with rectal mucous discharge. Colonoscopy revealed a rectal lateral spreading tumor. The patient underwent an endoscopic transanal resection of the lesion. He presented with sepsis of the abdominal focus, and imaging tests revealed pneumoretroperitoneum. A new surgical intervention was performed with a loop colostomy. Despite the existence of other reports on pneumoretroperitoneum after transanal endoscopic microsurgery, what draws attention to this case is the association with sepsis.
Colonoscopy ; Colostomy ; Humans ; Microsurgery ; Retropneumoperitoneum* ; Sepsis* ; Transanal Endoscopic Microsurgery

PURPOSE: Neoadjuvant chemotherapy has been shown to improve survival in locally advanced gastric cancer, but it is associated with significant toxicity. Sarcopenia and sarcopenic obesity have been studied in several types of cancers and have been reported to be associated with higher chemotherapy toxicity and morbi-mortality. The aim of this study was to assess the prevalence of sarcopenia/sarcopenic obesity in patients with gastric cancer, as well as its association with chemotherapy toxicity and long-term outcomes. MATERIALS AND METHODS: A retrospective analysis was performed using an academic cancer center patient cohort diagnosed with locally advanced gastric cancer between January 2012 and December 2014 and treated with neoadjuvant chemotherapy. We analyzed body composition (skeletal muscle and visceral fat index) in axial computed tomography images. RESULTS: A total of 48 patients met the inclusion criteria. The mean age was 68±10 years, and 33 patients (69%) were men. Dose-limiting toxicity was observed in 22 patients (46%), and treatment was terminated early owing to toxicity in 17 patients (35%). Median follow-up was 17 months. Sarcopenia and sarcopenic obesity were found at diagnosis in 23% and 10% of patients, respectively. We observed an association between termination of chemotherapy and both sarcopenia (P=0.069) and sarcopenic obesity (P=0.004). On multivariate analysis, the odds of treatment termination were higher in patients with sarcopenia (odds ratio 4.23; P=0.050). Patients with sarcopenic obesity showed lower overall survival (median survival of 6 months [95% confidence interval 3.9–8.5] vs. 25 months [95% confidence interval 20.2–38.2]; log-rank test P=0.000). CONCLUSIONS: Sarcopenia and sarcopenic obesity were associated with early termination of neoadjuvant chemotherapy in patients with gastric cancer; additionally, sarcopenic obesity was associated with poor survival.
Body Composition* ; Cohort Studies ; Diagnosis ; Drug Therapy* ; Follow-Up Studies ; Humans ; Intra-Abdominal Fat ; Male ; Multivariate Analysis ; Neoadjuvant Therapy ; Obesity ; Prevalence ; Prognosis ; Retrospective Studies ; Sarcopenia ; Stomach Neoplasms*

Ovarian stimulation with commercial preparations of equine chorionic gonadotropin (eCG) produces extremely variable responses in domestic animals, ranging from excessive stimulation to practically no stimulation, when applied on the basis of their declared unitage. This study was conducted to analyze four commercial preparations from different manufacturers via reversed-phase HPLC (RP-HPLC) in comparison with a reference preparation and an official International Standard from the World Health Organization. The peaks obtained by this qualitative and quantitative physical–chemical analysis were compared using an in vivo bioassay based on the ovarian weight gain of prepubertal female rats. The RP-HPLC data showed one or two peaks close to a main peak (t(R) = 27.9 min), which were related to the in vivo bioactivity. Commercial preparations that have this altered peak showed very little or no in vivo activity, as demonstrated by rat ovarian weight and in peripubertal gilts induced to ovulate. Overall, these findings indicate that RP-HPLC can be a rapid and reliable tool to reveal changes in the physicochemical profile of commercial eCG that is apparently related to decreased biological activity of this hormone.
Animals ; Animals, Domestic ; Biological Assay ; Chorion* ; Chorionic Gonadotropin* ; Chromatography, High Pressure Liquid ; Electrocardiography ; Female ; Humans ; Ovulation Induction ; Rats ; Weight Gain ; World Health Organization

OBJECTIVES: To evaluate the influence of the restorative technique on the mechanical response of endodontically-treated upper premolars with mesio-occluso-distal (MOD) cavity. MATERIALS AND METHODS: Forty-eight premolars received MOD preparation (4 groups, n = 12) with different restorative techniques: glass ionomer cement + composite resin (the GIC group), a metallic post + composite resin (the MP group), a fiberglass post + composite resin (the FGP group), or no endodontic treatment + restoration with composite resin (the CR group). Cusp strain and load-bearing capacity were evaluated. One-way analysis of variance and the Tukey test were used with α = 5%. Finite element analysis (FEA) was used to calculate displacement and tensile stress for the teeth and restorations. RESULTS: MP showed the highest cusp (p = 0.027) deflection (24.28 ± 5.09 µm/µm), followed by FGP (20.61 ± 5.05 µm/µm), CR (17.72 ± 6.32 µm/µm), and GIC (17.62 ± 7.00 µm/µm). For load-bearing, CR (38.89 ± 3.24 N) showed the highest, followed by GIC (37.51 ± 6.69 N), FGP (29.80 ± 10.03 N), and MP (18.41 ± 4.15 N) (p = 0.001) value. FEA showed similar behavior in the restorations in all groups, while MP showed the highest stress concentration in the tooth and post. CONCLUSIONS: There is no mechanical advantage in using intraradicular posts for endodontically-treated premolars requiring MOD restoration. Filling the pulp chamber with GIC and restoring the tooth with only CR showed the most promising results for cusp deflection, failure load, and stress distribution.
Bicuspid ; Dental Pulp Cavity ; Endodontics ; Finite Element Analysis ; Glass Ionomer Cements ; Tooth ; Weight-Bearing

Metabolic syndrome (MS) is associated with both cardiovascular and bladder dysfunction. Insulin resistance (IR) and central obesity, in particular, are the main risk factors. In these patients, vicious pathological cycles exacerbate abnormal carbohydrate metabolism and sustain an inflammatory state, with serious implications for both the heart and bladder. Ketone bodies serve as an alternative energy source in this context. They are considered a “super-fuel” because they generate adenosine triphosphate with less oxygen consumption per molecule, thus enhancing metabolic efficiency. Ketone bodies have a positive impact on all components of MS. They aid in weight loss and glycemic control, lower blood pressure, improve lipid profiles, and enhance endothelial function. Additionally, they possess direct anti-inflammatory, antioxidant, and vasodilatory properties. A shared key player in dysfunction of both the heart and bladder dysfunction is the formation of the NLRP3 inflammasome, which ketone bodies inhibit. Interventions that elevate ketone body levels—such as fasting, a ketogenic diet, ketone supplements, and sodium-glucose cotransporter 2 inhibitors—have been shown to directly affect cardiovascular outcomes and improve lower urinary tract symptoms derived from MS. This review explores the pathophysiological basis of the benefits of ketone bodies in cardiac and bladder dysfunction.