INTRODUCTION: The number of patients with necrotizing pneumonia has increased in recent years. The aim of this study is to review the incidence, management, and outcome of pediatric necrotizing pneumonia requiring surgical therapy and to prove that lung resection results in favorable development of patients. We hypothesize that overall lung function in children after lung resection does not differ from that of the healthy population. MATERIALS AND METHODS: A retrospective tertiary referral center study with a prospective follow-up spirometric study of patients with necrotizing pneumonia managed between January 2010 and December 2019 was performed. RESULTS: The study cohort consisted of 1,295 patients admitted to the pediatric department for community-acquired pneumonia; 47 patients developed necrotizing pneumonia, 36 of whom underwent parenchymal lung resection. A 5-year rise in the occurrence of necrotizing pneumonia requiring resection was 77%, with a significant increase in the last 3 years (p < 0.05). The median age at the time of surgery was 32.5 (interquartile range [IQR]: 32.25) months. Streptococcus pneumoniae was the most prevalent pathogen (83%), although 53.3% of these patients were vaccinated against the agent. In 67% of patients, preresection procedures were performed: drainage of pneumothorax (17%), drainage of empyema (46%), drainage of empyema with use of alteplase (25%), and thoracoscopic decortication (12%). Surgical procedures included lobectomy (72.2%), wedge resection (13.9%), bilobectomy (8.3%), and pneumonectomy (5.6%). The postoperative complication was bronchopleural fistula in three patients. There were two (5.5%) postoperative deaths due to multiple organ failure. The follow-up spirometry was performed 43.3 (median, IQR 23.8-66.7) months after surgical intervention. Normal lung function was detected in 35 (64.8%) patients, restrictive pattern in 6 (11.1%) patients, obstructive pattern in 11 (20.4%) patients, and combined in 2 (3.7%) patients. CONCLUSION: The number of patients with necrotizing pneumonia requiring resection has increased significantly in the last 3 years (p < 0.05). Aggressive surgical treatment results in significant clinical improvement in most cases and favorable lung function outcome. Long-term follow-up showed normal spirometry in 64.8% of cases.

• MeSH
• dítě MeSH
• empyém * chirurgie   MeSH
• lidé MeSH
• následné studie MeSH
• nekrotizující pneumonie * chirurgie   MeSH
• plíce chirurgie   MeSH
• pneumektomie škodlivé účinky   metody   MeSH
• počítačová rentgenová tomografie MeSH
• předškolní dítě MeSH
• prospektivní studie MeSH
• retrospektivní studie MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• předškolní dítě MeSH
• Publikační typ
• časopisecké články MeSH

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is the most common congenital malformation of the upper digestive tract. This study represents the first genome-wide association study (GWAS) to identify risk loci for EA/TEF. We used a European case-control sample comprising 764 EA/TEF patients and 5,778 controls and observed genome-wide significant associations at three loci. On chromosome 10q21 within the gene CTNNA3 (p = 2.11 × 10-8; odds ratio [OR] = 3.94; 95% confidence interval [CI], 3.10-5.00), on chromosome 16q24 next to the FOX gene cluster (p = 2.25 × 10-10; OR = 1.47; 95% CI, 1.38-1.55) and on chromosome 17q12 next to the gene HNF1B (p = 3.35 × 10-16; OR = 1.75; 95% CI, 1.64-1.87). We next carried out an esophageal/tracheal transcriptome profiling in rat embryos at four selected embryonic time points. Based on these data and on already published data, the implicated genes at all three GWAS loci are promising candidates for EA/TEF development. We also analyzed the genetic EA/TEF architecture beyond the single marker level, which revealed an estimated single-nucleotide polymorphism (SNP)-based heritability of around 37% ± 14% standard deviation. In addition, we examined the polygenicity of EA/TEF and found that EA/TEF is less polygenic than other complex genetic diseases. In conclusion, the results of our study contribute to a better understanding on the underlying genetic architecture of ET/TEF with the identification of three risk loci and candidate genes.

• Publikační typ
• časopisecké články MeSH

Vrozená plicní malformace dýchacích cest se svou incidencí 1:25–35 000 řadí mezi nejčastější vrozené anomálie plic. V menší míře je zastoupena plicní sekvestrace. Někdy se navzájem kombinují jako léze hybridní. Patří do diferenciální diagnostiky nejasných plicních lézí v každém věku. Lze je snadno diagnostikovat prenatálně ultrazvukem. Klinická manifestace je různá, od asymptomatických vad po respirační selhání dítěte. Terapeutický přístup se liší v závislosti na symptomech, velikosti a druhu vady či zvyklosti daného pracoviště.

Congenital pulmonary airway malformation (CPAM) with its incidence 1:25–35 000 belongs to the most frequent congenital lung anomalies. Pulmonary sequestration (PS) occures less often. These malformations can be mixed sometimes, called hybrid lesions. They need to be correctly diagnosed in patients with unknown pulmonary lesions of any age. They can be easily diagnosed prenatally by ultrasonography. Clinical presentation differs from asymptomatic lesions to respiratory failure of child. Therapeutic approach depends on symptomps, size and type of the malformation as well as personal experience.

• Klíčová slova
• vrozená plicní malformace dýchacích cest, vrozené cystické adenomatoidní malformace, kongenitální hrudní malformace, kongenitální plicní masy, plicní sekvestrace,
• MeSH
• abnormality dýchací soustavy * diagnóza   genetika   terapie   MeSH
• lidé MeSH
• vrozené vady MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• přehledy MeSH

Úvod: Soubor pacientů Dětské kliniky FNHK s vrozenou plicní malformací dýchacích cest (CPAM) a plicní sekvestrací (PS) v letech 2006 až 2020. Cíl: Zhodnocení výsledků řešení CPAM a PS s cílem shrnout stávající diagnostické a terapeutické postupy na našem pracovišti a porovnání s dostupnou literaturou. Metodika: Na Dětské klinice FNHK bylo v letech 2006 až 2020 diagnostikováno 23 pacientů s kongenitální hrudní malformací (CPAM a PS). Byla provedena analýza základních údajů z dokumentace pacientů, shrnuty základní charakteristiky, jako je věk stanovení diagnózy, metoda zobrazení malformace, symptomy, způsob řešení, věk event. operačního řešení a případné komplikace. Výsledky: Z 23 pacientů bylo 11 s diagnózou CPAM, 10 s PS a 2 s hybridními lézemi (CPAM/PS). Prenatálně bylo diagnostikováno 17 z 23 případů (74 %). Zobrazovací metodou volby bylo ve všech případech ultrazvukové vyšetření (UZ) a RTG hrudníku, před operačním řešením vždy CT hrudníku s angiografií. V 6 případech nebyly vady postnatálně detekovány pomocí UZ nebo RTG, ale až CT vyšetřením, 3x došlo ke spontánní involuci vady (2x CPAM, 1x PS). Symptomatické byly 4 z 11 dětí s CPAM a 3 z 10 s PS. Děti s hybridními lézemi byly asymptomatické. Tři z 11 dětí s CPAM podstoupily chirurgickou resekci. Věk pacientů v době operace byl 3 měsíce, 6 let a 7,5 let. Šest z 11 dětí s CPAM je zatím sledováno, věk nejstaršího dítěte je 4 roky, 2x došlo k involuci CPAM (vada nebyla detekovaná na CT). Pět z 10 dětí s PS je po lobektomii plic ve věku 5 až 15 let. Čtyři z 10 dětí s PS jsou zatím sledovány, nejstaršímu dítěti je 4,5 roku. Jednou došlo k involuci PS, pravděpodobně extralobární. Dvě děti měly hybridní léze, u jednoho byla tato léze odstraněna, druhé je zatím sledováno. Pooperační komplikace byla zaznamenána jedenkrát (pleurální výpotek), bez potřeby drenáže. Závěr: Vrozenou plicní malformaci dýchacích cest a plicní sekvestraci lze dobře diagnostikovat již prenatálně. Asymptomatické vady představují leckdy terapeutický problém, kdy, jak a zda vůbec léčit. Stále chybí dostatek dat, díky nimž by se dalo předvídat chování malformace a optimalizovat tak naši léčbu.

Introduction: A set of patients from the Department of Pediatrics, University Hospital in Hradec Králové, with congenital pulmonary airway malformation (CPAM) and pulmonary sequestration (PS) from the year 2006 to 2020. Objective: The aim of the survey is to assess the results of CPAM and PS management at our department and to compare them with literature. Methods: There were diagnosed 23 patients with congenital thoracic malformation (CPAM and PS) from the year 2006 to 2020. An analysis of data (age at the time of diagnosis, type of imaging, symptoms, management). Results: There were 11 patients with CPAM, 10 patients with PS and 2 patients with hybrid lesions (CPAM/PS) out of 23 patients with congenital thoracic malformation. Seventeen patients (74%) were diagnosed prenatally. Chest X-ray and ultrasound of the chest were done in all cases. Chest CT with angiography was always done prior to surgery. There was no detection of malformation using X-ray and ultrasound examinations in 6 cases, only with CT. Three malformations involuted (2x CPAM, 1x PS). Symptoms had 4 from 11 children with CPAM and 3 from 10 children with PS. Both children with hybrid lesions were asymptomatic. Three from 11 children with CPAM underwent surgery. The age of the patients at the time of resection were 3 months, 6 years and 7.5 years. Six from 11 children with CPAM are being monitored, the oldest is 4 years. Two CPAM involuted. Five from 10 children with PS underwent lobectomy in the age from 5 to 15 years. Four from 10 children with PS are monitored, the oldest is 4.5 years. One PS involuted, probably the extralobar. One of 2 patients with hybrid lesions underwent resection, the second is being monitored. There was one complication after surgery (a pleural effusion without the need of dranaige). Conclusion: Congenital pulmonary airway malformation and pulmonary sequestration can be easily diagnosed prenatally. The asymptomatic lesions can pose a therapeutic dilemma as to when and how to treat and if any treatment is necessary at all. We still do not have enough data to predict changes in malformations in order to be able to optimalize our treatment.

• Klíčová slova
• vrozená plicní malformace dýchacích cest, vrozené cystické adenomatoidní malformace, kongenitální hrudní malformace, kongenitální plicní masy, plicní sekvestrace,
• MeSH
• abnormality dýchací soustavy * diagnóza   genetika   terapie   MeSH
• dítě MeSH
• lidé MeSH
• retrospektivní studie MeSH
• vrozené vady MeSH
• Check Tag
• dítě MeSH
• lidé MeSH

Introduction: Sinus pilonidalis (SP) is an acquired inflammatory disease, which is relatively common in the paediatric population. Surgery is indicated in symptomatic patients. In 2017, minimally invasive pilonidal sinus treatment (EPSiT) was adapted to the paediatric population. Aim: To evaluate the first experience with minimally invasive endoscopic treatment of SP (PEPSiT) in children and adolescents in the Czech Republic. Material and methods: A retrospective review of all consecutive paediatric patients who underwent PEPSiT from November 2018 to February 2020. The monitored parameters were demographics, perioperative course of the disease, surgery, length of hospitalisation, postoperative complications, healing, disease recurrence, and follow-up. Results: Seventeen patients were enrolled in the study. The median age at surgery was 17.1 years (range: 12.5-18). The subjects comprised 76% males, and the median body mass index was 25.6 kg/m2 (range: 17-30.3 kg/m2). Thirteen patients underwent previous surgical treatment (76%) under local anaesthesia. The median duration of PEPSiT was 50 min (range: 30-85 min). The subjective evaluation of pain by patients on the VAS scale was 0 on the day of discharge. There were no postoperative complications up to the 30th postoperative day. Two disease recurrences were successfully managed by re-PEPSiT. By the end of follow-up, 14/15 patients had healed. Two patients are still within 3 months of surgery, which is too soon to definitively evaluate possible recurrence of the disease. Conclusions: These preliminary results show that PEPSiT is a highly promising method. It is safe and well-tolerated by patients (short hospital stay, quick return to normal life, low pain and analgesic consumption). Two recurrences of disease were treated by re-PEPSiT.

• Publikační typ
• časopisecké články MeSH

BACKGROUND/PURPOSE: The available literature on congenital isolated H-type tracheoesophageal fistula (TEF) is limited, and preferred approach varies among centers (cervicotomy, thoracotomy, thoracoscopy). We aimed to present one of the biggest case series of thoracoscopic approach for congenital isolated TEF and to assess the method's feasibility and outcomes. METHODS: Retrospective review of thoracoscopic TEF repair experiences at 2 European university pediatric surgery centers. RESULTS: 9 boys and 3 girls were involved in the study (age 5 days-4 years), 8 of them were newborns (mean birth weight 3013 g, mean gestational age 39 weeks). The most common presenting symptoms were desaturations on feeding in neonates and recurrent respiratory tract infections in older children. The diagnoses were established on contrast study and confirmed with rigid bronchoscopy. The fistulas were located at or below the thoracic inlet; the fistulas were 2 cm above the carina to half the height of the trachea. All patients underwent successful thoracoscopic TEF repair. There were no conversions. The postoperative course was uneventful in all but one who had rethoracoscopy for prolonged postoperative chylothorax. All patients had satisfactory vocal cord function. One patient required reoperation for fistula recurrence 8 months after primary surgery. CONCLUSION: Thoracoscopic approach for isolated H-type TEF seems to be possible as a procedure of choice with satisfactory results and all benefits of minimally invasive procedure. LEVEL OF EVIDENCE: IV (case series).

• MeSH
• atrézie jícnu * chirurgie   MeSH
• dítě MeSH
• ezofágoplastika * MeSH
• kojenec MeSH
• lidé MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• retrospektivní studie MeSH
• torakoskopie MeSH
• tracheoezofageální píštěl * chirurgie   MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND AND AIMS: SARS-CoV-2 is a worldwide serious health problem. The aim of this study was to demonstrate the number of potentially infectious particles present during endoscopic procedures and find effective tools to eliminate the risks of SARS-CoV-2 infection while performing them. METHODS: An experimental model which focused on aerosol problematics was made in a specialized laboratory. This model simulated conditions present during endoscopic procedures and monitored the formation of potentially infectious fluid particles from the patient's body, which pass through the endoscope and are then released into the environment. For this reason, we designed and tested a prototype of a protective cover for the endoscope's control body to prevent the release and spread of these fluid particles from its working channel. We performed measurements with and without the protective cover of the endoscope's control body. RESULTS: It was found that liquid coming through the working channel of the endoscope with forceps or other instruments inside generates droplets with a diameter in the range of 0.1-1.1 mm and an initial velocity of up to 0.9 m/s. The average number of particles per measurement per whole measured area without a protective cover on the endoscope control body was 51.1; with this protective cover on, the measurement was 0.0, p<0.0001. CONCLUSIONS: Our measurements proved that fluid particles are released from the working channel of an endoscope when forceps are inserted. A special protective cover for the endoscope control body, made out of breathable material (surgical cap) and designed by our team, was found to eliminate this release of potentially infectious fluid particles.

• MeSH
• COVID-19 * prevence a kontrola   přenos   MeSH
• gastrointestinální endoskopie * MeSH
• lidé MeSH
• ochranné oděvy * MeSH
• přenos infekce z pacienta na zdravotnického pracovníka prevence a kontrola   MeSH
• SARS-CoV-2 * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

Over a twenty-year period, we performed 255 ERCP procedures in infants aged up to 1 year. ERCP was indicated in cholestatic infants with suspicion of biliary obstruction. The most common diagnosis was biliary atresia (48%), choledochal cysts (13%), and choledocholithiasis (4%). The procedure complication rate was 13.7%. Hyperamylasemia occurred in 12.9%. More severe complications were rare-0.8% of ERCP procedure. There were no cases of postprocedural pancreatitis or death. Our study has proved that ERCP is a safe and reliable method in this age group. Its high specificity and negative predictive value for extrahepatic biliary atresia can prevent unnecessary surgeries in patients with normal bile ducts or endoscopically treatable pathologies.

• MeSH
• cholangiopankreatografie endoskopická retrográdní škodlivé účinky   MeSH
• choledocholitiáza * diagnostické zobrazování   chirurgie   MeSH
• cholestáza * etiologie   MeSH
• hyperbilirubinemie MeSH
• kojenec MeSH
• lidé MeSH
• pankreatitida * MeSH
• Check Tag
• kojenec MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

• Publikační typ
• abstrakt z konference MeSH

Ingestion of a foreign body is a frequent diagnosis in the pediatric population. In a small percentage of cases, foreign bodies themselves are strong magnets, and swallowing of multiple magnetic bodies can lead to serious complications in the gastrointestinal tract. Two consecutive case reports of patients who swallowed two magnetic beads are presented. In both cases, the abdominal radiograph described two magnets in contact, one in the area of the left hypochondrium and one in the right hypogastrium. Attempts of endoscopic localization and removal were unsuccessful. Due to the failure of magnet progression, laparoscopic revision of the abdominal cavity was indicated in both patients on the 25th and 4th day after swallowing. Using the magnetic forces between the magnets and the laparoscopic instruments, the foreign bodies were localized in the appendix of the first patient and in the cecum of the other one. The magnets were extracted together with the removal of the appendix in both patients. This is one of the first articles describing the successful extraction of foreign magnetic bodies from the gastrointestinal tract via laparoscopic appendectomy.

• Publikační typ
• kazuistiky MeSH