An unexpected infection in loss-of-function mutations in STAT3: malignant alveolar echinococcosis in liver

Loss-of-function (LOF) mutations in signal transducer and activator of transcription 3 (S TAT 3) gene causes autosomal dominant hyper immunoglobulin E syndrome (AD-HIES or Job's Syndrome), a rare and complex primary immunodeficiency (PID) syndrome characterized by increased levels of IgE (>2000 IU/mL), eosinophilia, recurrent staphylococcal skin abscesses, eczema, recurrent pneumonia, skeletal and connective tissue abnormalities. Although bacterial and fungal infections are common in AD-HIES, susceptibility to parasitic infections has not been reported. Alveolar echinococcosis (AE), a zoonosis caused by the growth of the Echinococcus multilocularis (EM) metacestode, mimics slow-growing liver cancer. The mortality rate of AE is very high when it is diagnosed late or undertreated. Here, we report a 14-year-old boy with AE infections of the liver and the lung resulting in liver failure and diagnosed as STAT3-LOF. To our knowledge, the association between these two conditions has not been reported in the literature before

The Effects of Local and Systemic Growth Hormone Treatment on Germ Cell Population and Fertility in an Experimental Unilateral Testicular Torsion and Orchiectomy Model

Purpose: We evaluated the effects of local and systemic growth hormone on the germ cell population of the contralateral testes of pubertal rats subjected to unilateral testicular torsion and orchiectomy 24 hours later

Increase in pro-apoptotic Bax expression and decrease in anti-apoptotic Bcl-2 expression in newborns with necrotizing enterocolitis.

The aim of the present study was to find out if there is an increase in the expression of pro-apoptotic Bax and reduction in expression of anti-apoptotic Blc-2A1 in newborn intestines with necrotizing enterocolitis (NEC)

The effects of local and sustained release of fibroblast growth factor on wound heating in esophageal anastomoses

Background/ Purpose: Postsurgical complications, such as anastomotic leaks in patients with esophageal atresia, have remained unchanged during the last 3 decades. Growth factors enhance healing in several wound-healing models. Therefore, an experimental study was used to evaluate the effects of local and sustained release of basic fibroblast growth factor (FGF) on wound healing in esophageal anastomoses

Effects of Local and Sustained Release of FGF, IGF, and GH on Germ Cells in Unilateral Undescended Testis in Rats

OBJECTIVES To evaluate the effects of the local release of fibroblast growth factor (FGF), insulin-like growth factor (IGF), and growth hormone (GH) on a germ cell population of ipsilateral undescended and contralateral descended testes of rats with a surgically created unilateral abdominal testis for 12 weeks and after the application of growth factors after orchidopexy

Increase in pro-apoptotic Bax expression and decrease in anti-apoptotic Bcl-2 expression in newborns with necrotizing enterocolitis

Background/Aim. The aim of the present study was to find out if there is an increase in the expression of pro-apoptotic Bax and reduction in expression of anti-apoptotic Blc-2A1 in newborn intestines with necrotizing enterocolitis (NEC). Material and Methods. We compared 8 consecutive newborn patients undergoing bowel resection for NEC with 8 neonates undergoing intestinal resection for ileal atresia. Histopathological evaluation of tissue injury and apoptosis was performed by using light microscopic examination and TUNEL method. The mRNA level of apoptotic (CASP3, CASP6, CASP7, Bax, BIRC2) and anti-apoptotic genes were evaluated by PCR array method. Protein expression was assessed by immunohistochemistry. Results. Tissue injury scores and mean apoptosis scores were significantly higher in NEC group when compared with control group (p < 0.01). Expression of pro-apoptotic genes were significantly increased in NEC group when compared with control group (p < 0.01). Expression of anti-apoptotic Bcl-2A1 gene was significantly decreased in NEC group, (p < 0.01). Protein expression of Bax and CASP3 was significantly increased in NEC group, (p < 0.01). Conclusion. Our data in human newborns suggest that alteration of the balance between pro-apoptotic Bax expression and antiapoptotic Bcl-2A1 expression in the site of injury is a possible mechanism in the pathogenesis of NEC

Early Postoperative Serum Phosphate Drop Predicts Sufficient Hypertrophy after Liver Surgery

OBJECTIVE The aim of this study was to assess the impact of postoperative hypophosphatemia on liver regeneration after major liver surgery in the scenario of ALPPS (Associating Liver Partition with Portal vein ligation for Staged hepatectomy) and living liver donation (LLD). BACKGROUND Hypophosphatemia has been described to reflect the metabolic demands of regenerating hepatocytes. Both, ALPPS and LLD, are characterized by an exceptionally strong liver regeneration and may be of particular interest in the context of posthepatectomy hypophosphatemia. METHODS Serum phosphate changes within the first 7 postoperative days after ALPPS (n=61) and LLD (n=54) were prospectively assessed and correlated with standardized volumetry after one week. In a translational approach, postoperative phosphate changes were investigated in mice and in vitro. RESULTS After ALPPS stage-1 and LLD, serum phosphate levels significantly dropped from a preoperative median of 1.08 mmol/L (IQR 0.92-1.23) and 1.07 mmol/L (IQR 0.91-1.21) to a postoperative median nadir of 0.68 mmol/L and 0.52 mmol/L, respectively. A pronounced phosphate drop correlated well with increased liver hypertrophy (P<0.001). Patients with a low drop of phosphate showed a higher incidence of posthepatectomy liver failure after ALPPS (7 vs. 31%, P=0.041). Like in human, phosphate drop correlated significantly with degree of hypertrophy in murine ALPPS and hepatectomy models (P<0.001). Blocking phosphate transporter (Slc20a1) inhibited cellular phosphate uptake and hepatocyte proliferation in vitro. CONCLUSION Phosphate drop after hepatectomy is a direct surrogate marker for liver hypertrophy. Perioperative implementation of serum phosphate analysis has the potential to detect patients with insufficient regenerative capacity at an early stage

Early Postoperative Serum Phosphate Drop Predicts Sufficient Hypertrophy after Liver Surgery

Objective: The aim of this study was to assess the impact of postoperative hypophosphatemia on liver regeneration after major liver surgery in the scenario of Associating Liver Partition with Portal vein ligation for Staged hepatectomy (ALPPS) and living liver donation (LLD). Background: Hypophosphatemia has been described to reflect the metabolic demands of regenerating hepatocytes. Both ALPPS and LLD are characterized by an exceptionally strong liver regeneration and may be of particular interest in the context of posthepatectomy hypophosphatemia. Methods: Serum phosphate changes within the first 7 postoperative days after ALPPS (n=61) and LLD (n=54) were prospectively assessed and correlated with standardized volumetry after 1 week. In a translational approach, postoperative phosphate changes were investigated in mice and in vitro . Results: After ALPPS stage 1 and LLD, serum phosphate levels significantly dropped from a preoperative median of 1.08 mmol/L [interquartile range (IQR) 0.92-1.23] and 1.07 mmol/L (IQR 0.91-1.21) to a postoperative median nadir of 0.68 and 0.52 mmol/L, respectively. A pronounced phosphate drop correlated well with increased liver hypertrophy ( P &lt;0.001). Patients with a low drop of phosphate showed a higher incidence of posthepatectomy liver failure after ALPPS (7% vs 31%, P =0.041). Like in humans, phosphate drop correlated significantly with degree of hypertrophy in murine ALPPS and hepatectomy models ( P &lt;0.001). Blocking phosphate transporter (Slc20a1) inhibited cellular phosphate uptake and hepatocyte proliferation in vitro. Conclusion: Phosphate drop after hepatectomy is a direct surrogate marker for liver hypertrophy. Perioperative implementation of serum phosphate analysis has the potential to detect patients with insufficient regenerative capacity at an early stage.</p