Deep and extensive pediatric burns: retrospective evaluation of scheme of patients at the Department of Children's Developmental Defects Surgery and Traumatology SUM.

<b> Introduction:</b> Burns are one of the most common injuries among children. Despite the scale of the problem, there is no generally accepted algorithm for dealing with burn wounds in children in Poland. </br></br> <b>Aim:</b> The aim of our study was to evaluate our treatment scheme as well as the long-term effects of burn treatment in our department. </br></br> <b>Material and methods:</b> We conducted a telephone survey with the parents of patients treated at our ward in 01.01.2016-01.01.2021 due to thermal burns. To assess the cosmetic effect of treatment, the modified Vancouver Scar Scale (mVSS) was used, as well as the evaluation of parents' satisfaction with the treatment of patients on a scale from 1 to 10. Criteria to be included were the thermal burn of the skin to at least a 2b degree and/or burns with not less than 8% of the Total Body Surface Area (TBSA), as well as answering all the questions included in the questionnaire. The inclusion criteria were met by 38 out of 97 hospitalized patients. </br></br> <b>Results:</b> 26.32% of patients achieved 0 points on the mVSS, 21.05% achieved 1 point, 15.79% achieved 2 points, 15.79% achieved 3 points, 2.63% patients received 4 points, 5.26% patients received 5 points, 5.26% patients received 7 points, 2.63% patients received 8 points, 2.63% patients received 9 points, 2.63% of patients received 10 points, none of the patients received 6 and 11 points higher. 3% of parents rated their satisfaction at 5 points, 3% of caretakers as 7 points, 8% as 8 points, 8% as 9 points, and 89% as 10 points. </br></br> <b>Discussion:</b> Our treatment algorithm brings good therapeutic effects and is also very well received by the patients' parents. In order to carry out a nationwide standardization of the treatment of childhood burn wounds, it would be necessary to conduct a similarly constructed study in a multicenter setting.

Supporting Doctor's Decisions Based on Electronic Medical Documentation in Polish.

We describe new functionalities that have been added to an existing and widely used software solution in Polish public healthcare. The system automatically evaluates a number of medical scores. It provides epidemiologic monitoring concerning infectious diseases; classifies a particular patient to a specific risk group; and detects anomalies. Moreover, an analysis of a prescribed drug is performed.

Automatic mapping of atoms across both simple and complex chemical reactions.

Mapping atoms across chemical reactions is important for substructure searches, automatic extraction of reaction rules, identification of metabolic pathways, and more. Unfortunately, the existing mapping algorithms can deal adequately only with relatively simple reactions but not those in which expert chemists would benefit from computer's help. Here we report how a combination of algorithmics and expert chemical knowledge significantly improves the performance of atom mapping, allowing the machine to deal with even the most mechanistically complex chemical and biochemical transformations. The key feature of our approach is the use of few but judiciously chosen reaction templates that are used to generate plausible "intermediate" atom assignments which then guide a graph-theoretical algorithm towards the chemically correct isomorphic mappings. The algorithm performs significantly better than the available state-of-the-art reaction mappers, suggesting its uses in database curation, mechanism assignments, and - above all - machine extraction of reaction rules underlying modern synthesis-planning programs.

Antidepressive effects of targeting ELK-1 signal transduction.

Depression, a devastating psychiatric disorder, is a leading cause of disability worldwide. Current antidepressants address specific symptoms of the disease, but there is vast room for improvement 1 . In this respect, new compounds that act beyond classical antidepressants to target signal transduction pathways governing synaptic plasticity and cellular resilience are highly warranted2-4. The extracellular signal-regulated kinase (ERK) pathway is implicated in mood regulation5-7, but its pleiotropic functions and lack of target specificity prohibit optimal drug development. Here, we identified the transcription factor ELK-1, an ERK downstream partner 8 , as a specific signaling module in the pathophysiology and treatment of depression that can be targeted independently of ERK. ELK1 mRNA was upregulated in postmortem hippocampal tissues from depressed suicides; in blood samples from depressed individuals, failure to reduce ELK1 expression was associated with resistance to treatment. In mice, hippocampal ELK-1 overexpression per se produced depressive behaviors; conversely, the selective inhibition of ELK-1 activation prevented depression-like molecular, plasticity and behavioral states induced by stress. Our work stresses the importance of target selectivity for a successful approach for signal-transduction-based antidepressants, singles out ELK-1 as a depression-relevant transducer downstream of ERK and brings proof-of-concept evidence for the druggability of ELK-1.

Co-occurrence of severe Goltz-Gorlin syndrome and pentalogy of Cantrell - Case report and review of the literature.

Goltz-Gorlin syndrome is a highly variable disorder affecting many body parts of meso-ectodermal origin. Mutations in X-linked PORCN have been identified in almost all patients with a classical Goltz-Gorlin phenotype. The pentalogy of Cantrell is an infrequently described congenital disorder characterized by the combination of five anomalies: a midline supra-umbilical abdominal wall defect; absent or cleft lower part of the sternum; deficiency of the diaphragmatic pericardium; deficiency of the anterior diaphragm; and congenital heart anomalies. Etiology and pathogenesis are unknown. We report on an infant with findings fitting both Goltz-Gorlin syndrome (sparse hair; anophthalmia; clefting; bifid nose; irregular vermillion of both lips; asymmetrical limb malformations; caudal appendage; linear aplastic skin defects; unilateral hearing loss) and the pentalogy of Cantrell (absent lower sternum; anterior diaphragmatic hernia; ectopia cordis; omphalocele). The clinical diagnosis Goltz-Gorlin syndrome was confirmed molecularly by a point mutation in PORCN (c.727C>T). The presence of molecularly confirmed Goltz-Gorlin syndrome and pentalogy of Cantrell in a single patient has been reported twice before. The present patient confirms that the pentalogy of Cantrell can be caused in some patients by a PORCN mutation. It remains at present uncertain whether this can be explained by the type or localization of the mutation within PORCN, or whether the co-occurrence of the two entities is additionally determined by mutations or polymorphisms in other genes, environmental factors, and/or epigenetic influences.

Thoracoscopic repair of tracheoesophageal fistula and esophageal atresia.

PURPOSE: To evaluate the safety and efficacy of the thoracoscopic repair of esophageal atresia and tracheoesophageal fistula (EA/TEF). METHODS: From August 2005 to March 2008, 23 consecutive patients (16 boys, 7 girls) weighing 1,070 to 3,390 g underwent thoracoscopic EA/TEF repair. Nine cases had associated malformations. Two 5-mm and one 2.5-mm trocars were placed. The 5-mm 25- to 30-degree telescope was preferred. A pneumothorax was maintained with 5 to 6 mm Hg. The azygos vein was never divided. The TEF was closed with 5-mm titanic clips mainly.The esophageal anastomosis was made over 6-French nasogastric tube by three to seven simple stitches of 5-0Vicryl. The 8-French chest tube was left without suction. The enteral feeding was usually started on postoperative day 3 or 4. Barium swallow was performed on postoperative day 5 or 6, then the chest tube was removed. RESULTS: All procedures were successfully completed without conversion. The average operative time was 131 minutes (range, 55-245 minutes) with significant improvement after gaining experience (mean, 171 minutes for first 10 cases and 98 minutes for last 13 cases). There were two cases of accidental tracheal opening. The anastomotic leak rate was 13% (three cases), and all were healed on conservative treatment. Four cases required one to three courses of anastomotic stricture dilatations. There were three deaths (13%) of causes not related with performed operation. CONCLUSIONS: The thoracoscopic repair of EA/TEF is effective method, and based on our experience, it is the procedure of choice if performed by an experienced endoscopic pediatric surgeon. The intraoperative complications observed if properly managed have a good prognosis.

Bladder agenesis in a male neonate.

Bladder agenesis, especially in male, is a rare congenital anomaly. This is a case report of a male patient with several congenital anomalies including penoscrotal transposition, severe kidney's dysplasia, and agenesis of the bladder. The patient lived for 4 months and died because of severe pneumonia.

Percutaneous internal ring suturing: a simple minimally invasive technique for inguinal hernia repair in children.

BACKGROUND: Laparoscopic herniorrhaphy in pediatric surgery is usually performed through three ports in the abdominal wall with intraperitoneal suturing. Our technique of percutaneous internal ring suturing (PIRS) requires only one umbilical port and needle puncture point. We describe the technique and evaluate the efficacy of percutaneous internal ring suturing for inguinal hernia repair in children. MATERIALS AND METHODS: We performed percutaneous internal ring suturing on 106 children (ages 28 days-14.5 years) with 140 hernias. The procedures were performed under general endotracheal anesthesia. Pneumoperitoneum was established with an open technique. Under laparoscopic-guided vision an 18-gauge injection needle with a nonabsorbable thread inside the barrel of the needle was placed through the abdominal wall into the peritoneal cavity. By moving the injection needle, the thread passed under the peritoneum around the entrance into the hernia sac. The knot was tightened from outside and placed in the subcutaneous space. The contralateral open inguinal ring was closed in the same procedure. RESULTS: The average operative time was 19 minutes for unilateral and 24 minutes for bilateral hernias. Follow-up ranged between 18 and 29 months. Cosmetic results are excellent with almost invisible scars. There were 3 cases of intraoperative complication: incidental puncture of the iliac vein that required no treatment. There were 4 cases of postoperative complications: one case of ileus adhesion with bowel strangulation, and hernia recurrence in 3 boys, one of whom was reoperated with percutaneous internal ring suturing. In 5 boys, transient hydroceles were observed that disappeared spontaneously after 3 to 5 months. CONCLUSION: The percutaneous internal ring suturing method seems to be a simple and effective minimally invasive procedure with excellent cosmetic results. The rate of complications is comparable to other laparoscopic techniques of inguinal hernia repair in children. According to our experience, percutaneous internal ring suturing should be considered as a treatment alternative.