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OBJECTIVES Dyssynergic defecation is a common disorder in children with functional constipation (FC) because of relaxation disorders of the sphincter apparatus and intra-rectal pressure during defecation. The aim of the study was to determine frequency and type of dyssynergic defecation and to assess pressure in the anal canal poles during simulated evacuation and function of puborectalis muscle in defecation in children with FC. METHODS Three-dimensional (3D) high-resolution anorectal manometries (3D HRAM) were performed in 131 children with FC. In the manometric test, resting pressure measurements were assessed in 4 measuring poles of the anal canal. RESULTS One hundred thirty-one children ages 5 to 17 years (mean age 10.2; SD ± 3.8; median 10) were involved in the study (69 girls and 62 boys). Dyssynergic defecation was shown in 106/131 (80.9%) examined children. A statistically significant difference between the age of examined children (P

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

Congenital diaphragmatic hernia (CDH) is a defect involving herniation of the abdominal organs into the pleural cavity through an incompletely closed diaphragm. This defect is also accompanied by various types of pulmonary and bronchial hypoplasia and serious anomalies in the pulmonary vascular bed and pulmonary hypertension. Extracorporeal membrane oxygenation (ECMO) is used in extreme cases resistant to standard treatment. There are no reports clearly indicating the optimal time to make a decision about the repair of CDH in paediatric patients on ECMO. In high-risk neonates (with severe hypoplasia and pulmonary hypertension), this repair procedure is postponed until the cardiopulmonary parameters are stabilized and pulmonary pressure reduced.To present the experience of paediatric surgeons regarding congenital diaphragmatic hernia repair in neonates on extracorporeal membrane oxygenation.CDH was surgically repaired in 17 neonates, and of these 7 were on ECMO.Surgical problems were encountered intraoperatively: positioning of the patient forced by the cannulae inserted in the carotid vessels, significant generalized oedema, significant capillary bleeding from the surgical wound, difficulties with closing the abdominal cavity. The most common postoperative complications included bleeding from various areas.Each surgery in a neonate with CDH on ECMO is a challenge for the whole medical team involved in the procedure.

Congenital cystic adenomatoid malformation (CCAM) is a rare anomaly. The mechanisms and the time at which the abnormality develops are still unclear. The malformation is characterized by the presence of single large or multiple but smaller cysts.To present the experience of our medical centre, the Polish Mother's Memorial Hospital - Research Institute.We analysed the medical records of 32 neonates hospitalized in 2008-2017 at the Department of Paediatric Surgery and Urology ICZMP due to pre- or postnatally diagnosed congenital cystic adenomatoid malformation.In 2008-2017 32 children were hospitalized at our department due to prenatally diagnosed CCAM. An intrauterine procedure of thoracoamniotic shunting was performed in 13 fetuses. All newborns had chest computed tomography. Surgery had been performed in 21 children. Histopathological analysis of specimens prepared from the resected lung tissue confirmed type 1 CCAM in 4 cases, type 2 CCAM in 11 cases and type 3 CCAM in 1 case. Four patients were diagnosed with a bronchogenic cyst and one with extralobar pulmonary sequestration.Postnatal management of CCAM depends on the clinical status of the newborn. Newborns without clinical symptoms require radiological diagnostics to confirm the presence of malformation. Surgical treatment is recommended before the age of 10 months. Resection of the malformed lung fragment in patients with CCAM is the first-line treatment. It should be emphasized that despite the tremendous advances in diagnostic imaging, the final diagnosis of CCAM is made based on histopathological findings.Wrodzona malformacja gruczołowo-torbielowata płuc (CCAM) jest rzadką anomalią. Obecnie nieznane są zarówno mechanizmy, jak i czas powstania wady. Malformacja charakteryzuje się obecnością pojedynczych, dużych lub bardzo licznych, ale mniejszych torbieli, zwykle obejmujących pojedynczy płat płuca.Przedstawienie doświadczenia naszego ośrodka – Instytutu Centrum Zdrowia Matki Polki (ICZMP) w Łodzi.Przeprowadzono analizę dokumentacji medycznej noworodków hospitalizowanych w latach 2008–2017 w Klinice Chirurgii i Urologii Dziecięcej ICZMP w Łodzi z powodu pre- lub postnatalnie rozpoznanej CCAM.W latach 2008–2017 w klinice hospitalizowano 32 dzieci z powodu prenatalnie rozpoznanej torbielowatości płuc. U 13 płodów wykonano zabieg wewnątrzmaciczny polegający na założeniu shuntu opłucnowo-owodniowego. U wszystkich noworodków wykonano badanie tomografii komputerowej klatki piersiowej. W chwili powstawania pracy zabieg chirurgiczny przeprowadzono u 21 dzieci. Wyniki badań histopatologicznych preparatów wyciętej tkanki płucnej potwierdziły obecność CCAM typu I w 4 przypadkach, typu II w 11 przypadkach, typu III w 1 przypadku. U 4 pacjentów wynik w badaniu histopatologicznym wykazał obecność torbieli bronchogennej, a u 1 – sekwestru zewnątrzpłucnego.Postępowanie postnatalne zależy od stanu klinicznego noworodka. Noworodki bez objawów klinicznych wymagają diagnostyki radiologicznej w celu potwierdzenia obecności wady. Przeprowadzenie zabiegu rekomendowane jest w 10. miesiącu życia. Wycięcie fragmentu zmienionego płuca w przebiegu CCAM jest postępowaniem z wyboru. Należy podkreślić, że pomimo ogromnego postępu w diagnostyce obrazowej ostateczne rozpoznanie CCAM ustala się na podstawie badania histopatologicznego.

Objective: To evaluate the efficiency of thoracoamniotic shunts for drainage of macrocystic-type congenital cystic adenomatoid malformation (CCAM). Subjects and Methods: This was a retrospective study of 12 fetuses with a large thoracic cyst treated with thoracoamniotic shunting between 2004 and 2014 in a tertiary fetal therapy center. Medline was searched to identify cases of CCAM treated with thoracoamniotic shunting. Results: In all cases the thoracic cyst was associated with major mediastinal shift, the CCAM volume ratio (CVR) was >1.6, and in eight cases there was associated hydrops. Shunt insertion was successfully carried out in all cases at a median gestational age of 24 weeks (range 18-34). In 10 cases there was live birth at a median age of 38 weeks (range 35-41), but in two hydropic fetuses there was intrauterine death. A literature search identified a total of 98 fetuses with CCAM treated with thoracoamniotic shunting between 1987 and 2016. In the combined data from the previous and the current study, the survival rate was 77% (53 of 69) for hydropic and 90% (37 of 41) for nonhydropic fetuses. Conclusions: The role of thoracoamniotic shunting in macrocystic lung lesions associated with hydrops is well accepted. Intrauterine intervention is also likely to be beneficial in the subgroup of nonhydropic fetuses with a CVR >1.6.

Prenatal ultrasound at the 20th week of gestation revealed a 3-cm tumour in the sacrococcygeal area. Initially, a sacrococcygeal teratoma was suspected on the basis of fetal ultrasonography, which revealed normal heart anatomy and an increasing tumour mass. The diagnosis was then changed to fetus in fetu or teratoma. Prenatal magnetic resonance imaging at the 34th week of pregnancy confirmed the ultrasound diagnosis. No other anomalies were found. Elective caesarean section was performed at term. The care team included a paediatric surgeon, obstetricians, neonatologists, midwives, and an anesthesiologist. A female newborn was delivered in good condition. The tumour was resected in the operating room and mature teratoma was established by histopathological evaluation. Surprisingly, agenesis of the right forearm was revealed which had not been detected prenatally, despite many examinations (both in our hospital and earlier at a primary care obstetrician office).

Objective: To evaluate the efficiency of percutaneous intratumor laser ablation for fetal solid sacrococcygeal teratoma (SCT). Subjects and Methods: We carried out percutaneous ultrasound-guided intratumor laser ablation through a 17-gauge needle using an output of 40 W in 7 fetuses with large solid SCT and reviewed the literature for minimally invasive therapy for this condition. Results: Laser ablation was carried out at a median gestational age of 20 (range 19–23) weeks, and in all cases there was elimination of obvious vascularization within the tumor and improvement in cardiac function. Three (43%) babies survived and had surgical excision of the tumor within 2 days of birth, 3 liveborn babies died within 5 days of birth and before surgery, and 1 fetus died within 2 weeks after the procedure. In previous series of various percutaneous interventions for predominantly solid SCT the survival rate was 33% (2/6) (95% CI 9.7–70%) for endoscopic laser to superficial vessels, 57% (4/7) (95% CI 25–84%) for intratumor laser, 67% (8/12) (95% CI 39–86%) for intratumor radiofrequency ablation, and 20% (1/5) (95% CI 3.6–62%) for intratumor injection of alcohol. Conclusions: In solid SCT, the reported survival from intratumor laser or radiofrequency ablation is about 50%, but survival does not mean success, and it remains uncertain whether such interventions are beneficial or not because the number of fetuses is small and there were no controls that were managed expectantly.

To evaluate general health condition of children operated for esophageal atresia, including complications from gastrointestinal tract and skeletal defects. Between 1990 and 2005, 77 patients were operated for esophageal atresia in the Polish Mother’s Health Memorial Hospital. The study was based on retrospective analysis of medical case records of all children with esophageal atresia. All living patients (n=51) were invited for follow-up studies. Pathological gastroesophageal reflux was found in 46.7% of children. Scoliosis was diagnosed in 20 patients. Chest deformations were observed in 43.3% children The absence of clinical symptoms does not exclude the presence of gastroesophageal reflux in children operated for esophageal atresia. Children operated for esophageal atresia should be followed up regularly by a multispecialistic medical team.

Congenital cystic adenomatoid malformation is a rare disorder of the respiratory system which occurs with an incidence of 1/25,000-35,000. Depending on the size of the cysts, CCAM is classified into microcystic and macrocystic. Very large lesions carry a significant risk of causing both, pulmonary hypoplasia due to compression of the lung tissue and fetal hydrops, probably due to impaired cardiac function as a result of mediastinal shift and compression of the vena cava. In this report, we present a case of a fetus with prenatally diagnosed large cyst of the left lung. Despite advanced gestational age, the baby was treated by thoracoamniotic shunting owning to developing impaired cardiac function and polyhydramion. Spectacular effects of the therapy have been achieved.

We describe the presence of c-kit positive interstitial cells of Cajal-like (ICCs-like) in the walls of theurinary bladders of children. An immunohistochemical study of specimens, obtained at autopsy from either thetrigonum (Group A) or the corpus (Group B), was performed using antibodies against c-kit (CD 117). Histologicalmorphometry of the immunoexpression of c-kit positive ICCs-like was performed by means of image analysissystem. The c-kit positive ICCs-like were identified by their morphology and counted in the vesical musclelayer in ten adjacent high power fields, each of 0.0479 mm2. The areas of the epithelial and subepithelial layerscontaining c-kit positive mast cells (rounded body with no dendritic processes) were neglected. The results wereexpressed as the number of ICCs-like cells per mm2. Differences between groups were tested using unpairedStudent’s t-test preceded by evaluation of normality and Levene’s test. Results were considered statisticallysignificant if p < 0.05. In Group A, the mean number of ICCs-like cells was statistically significantly higher (41.5cells/mm2) than in Group B (30.4 cells/mm2), p < 0.05. ICCs-like cells were found within the smooth musclelayer of the urinary bladder. There was a different distribution of these cells in particular parts of the bladder,which was probably due to the different roles of the trigonum and the corpus in the bladders of children. (FoliaHistochemica et Cytobiologica 2011; Vol. 49, No. 3, pp. 431–435)

Rectal suction biopsy (RSB) is the gold standard diagnostic procedure for disorders of bowel motility. This study describes our experience with RSB stained with histochemistry as the first diagnostic approach in a large series of patients presenting with chronic constipation. Between 1993 and 2005, 766 children underwent RSB for persistent chronic constipation. The specimens were snap frozen, sectioned and stained with conventional hematoxylin and eosin (H&E) and with nicotinamide adenine dinucleotide phosphate diaphorase (NADPH-d) and acetylcholinesterase (AChE) histochemical stainings. Adequate amount of submucosa was present in 655 (85.5%) out of 766 cases and formed the basis of this study. RSB in 540 (82%) patients were reported as normal. Hirschsprung's disease was found in 47 (7.2%) patients with characteristic features of absence of ganglion cells, increased AChE activity in the lamina propria and muscularis mucosae, thick nerve fibers in the submucosa, and a lack of NADPH-d-positive fibers in muscularis mucosae. RSB in 59 (9%) patients presented features of intestinal neuronal dysplasia such as submucosal hyperganglionosis, giant ganglia, ectopic ganglia and increased AChE activity in lamina propria. Hypoganglionosis was suspected in nine (1.3%) children because of sparse or absent ganglion cells and low AChE and NAPDH-d activity in muscularis mucosae. Three patients (0.4%) developed bleeding following RSB, requiring diathermy of the bleeding point. Thus, we conclude that RSB is a simple and safe method when used as the first diagnostic approach in patients with chronic constipation. The combination of two histochemical stainings techniques provides a high level of accuracy in the diagnosis of intestinal dysganglionosis.

Interstitial cells of Cajal (ICCs) are pacemaker cells which are densely distributed throughout the whole gastrointestinal tract. ICCs have important functions in neurotransmission, generation of slow waves and regulation of mechanical activities in the gastrointestinal tract, especially for the coordinated gastrointestinal peristalsis. Therefore, a loss of ICCs could result in gastrointestinal motor dysfunction. In recent years c-kit labeling has been widely used to study pathological changes of ICCs in gastrointestinal motility disorders. Paediatric gastrointestinal motility disorders such as hypertrophic pyloric stenosis, Hirschsprung's disease, total colonic aganglionosis, hypoganglionosis, intestinal neuronal dysplasia, internal anal sphincter achalasia, megacystis microcolon intestinal hypoperistalsis syndrome have been reported to be associated with loss or deficiency of ICCs networks. This review describes the distribution of ICCs in the normal gastrointestinal tract and its altered distribution in intestinal motility disorders of childhood.

Objectives: The aim of the study was to evaluate the efficiency of intrauterine treatment of large cysts in fetal lungs using thoracoamniotic shunts. Material and methods: Our observational retrospective study was carried out on a series of 8 fetuses who underwent thoracoamniotic shunting after sonographic statement of large macrocystic lesions in the lungs at the Department of Gynecology, Fertility and Therapy of the Fetus, Polish Mother’s Research Institute, between 2009-2014. Results: Mean gestational age at shunt insertion was 26.6 (range 18-33) weeks. Marked mediastinal shift in theechocardiographic examination was observed in all of the investigated cases. Five fetuses had polyhydramion, with 4 hydropic cases. Out of the remaining 4 fetuses without impaired cardiac function, 3 had very large lesions at initial presentation and 1 had a lesion that was rapidly increasing in size. Shunt insertion was successful in all cases. Only one patient went into premature labor (at 36 weeks of gestation). Mean gestational age at delivery was 38.2 weeks. Cesarean section was necessary in the half of the patients due to obstetric complications. All newborns underwent resection of the lesions. Three of them were operated in the first month after birth. The rest of the operations were postponed. Prenatal diagnosis of congenital cystic adenomatoid malformations was confirmed by pathologists in all cases. Conclusion: Intrauterine therapy of macrocystic lesions in fetal lungs enables to achieve good perinatal outcome. It needs to be considered in every case of a fetus with developing impaired cardiac function.

OBJECTIVE To investigate the distribution of c-kit-positive interstitial cells of Cajal (ICCs) in normal bladder and bladders from patients with megacystis-microcolon-intestinal peristalsis syndrome (MMIHS, a rare congenital and generally fatal cause of functional intestinal obstruction in the newborn), the most characteristic feature of which is abdominal distension caused by a distended unobstructed urinary bladder. PATIENTS AND METHODS Full-thickness bladder specimens were obtained from four infants with MMIHS and four controls, and processed as paraffin-wax and frozen sections. Sections were assessed using single immunohistochemistry with monoclonal and polyclonal anti-c-kit antibodies. Anti-α-smooth muscle actin (SMA) antibody was used to investigate the contractile apparatus in smooth muscle cells of the urinary bladder. Specimens were examined using light and confocal scanning microscopy. RESULTS There were many c-kit positive ICCs in the normal urinary bladder, appearing as small, long, bipolar cells with only two long and several short processes. In contrast, ICCs were absent in the MMIHS bladder. α-SMA immunoreactivity was lower in MMIHS urinary bladder than in control sections. CONCLUSION This study shows for the first time the presence of c-kit-positive ICCs in the normal human urinary bladder. The lack of ICCs in the MMIHS bladder may contribute to the voiding dysfunction in this disease.

End-stage renal disease is characterized by interstitial infiltrate of inflammatory cells in association with tubular atrophy and interstitial fibrosis. Mast cells (MCs) secrete a large number of fibrogenic factors and have been implicated in chronic inflammatory processes with fibrous tissue deposition. The aim of this study was to investigate the distribution of MCs in kidneys with reflux nephropathy (RN) and to determine the relationship between MCs and the interstitial fibrotic process in RN. Kidney specimens from 12 patients (aged 2-13 years) with severe RN secondary to primary high-grade vesicoureteral reflux, obtained at the time of nephrectomy, and 5 controls were examined. Sections were investigated histochemically by acid toluidine blue (TB) and immunohistochemically with antibodies for anti MC-tryptase, MC-chymase, c- kit (CD117), and fibronectin. Double staining for fibronectin and MC-tryptase was performed and examined using confocal scanning microscopy. TB histochemistry showed a marked increase of MCs in RN specimens compared with controls. MC-tryptase, chymase, and c- kit immunopositive MC infiltration was significantly higher in RN samples (14.2+/-9.6) than controls (1.3+/-0.8), ( P0.05). In all the sections there were more MC-tryptase-positive cells than MC-chymase-positive MCs. Double staining showed increased immunoreactivity of MCs and fibrosis in the renal interstitium of kidneys with RN. The number of infiltrating tryptase-positive MCs was correlated with the degree of interstitial renal scarring. This study demonstrates for the first time the increased expression of MCs in RN, suggesting that MCs may be involved in the development of scarring in RN.

Reflux nephropathy (RN) is a major cause of end stage renal failure in children and hypertension is a frequent complication. Cyclooxygenase-2 (COX-2) is an enzyme responsible for the prostaglandin synthesis. It has been shown that COX-2 up-regulates renin production leading to renovascular hypertension. We investigate COX-2 expression in the kidneys of children with RN.Kidney specimens from 12 patients 2 to 13 years old with severe RN secondary to primary high grade vesicoureteral reflux obtained at the time of nephrectomy and 5 controls were examined. Single labeled immunohistochemistry was performed with 2 COX-2 antibodies using light and confocal microscopy. Quantification of COX-2 was determined by Western blotting analysis. COX-2 gene expression was evaluated by real-time quantitative reverse transcription polymerase chain reaction.There was a strong COX-2 immunoreactivity in the proximal tubules and tubulointerstitial space in the RN samples compared to controls. Immunoreactive COX-2 protein expression was markedly increased in RN samples compared to controls. Real-time reverse transcription polymerase chain reaction showed a significant increase in COX-2 mRNA expression in the RN samples compared to controls (p0.05).Over expression of COX-2 in reflux nephropathy suggests that COX-2 may be involved in the pathogenesis of tubulointerstitial damage associated with severe reflux nephropathy.

Intestinal neuronal dysplasia (IND) is an intestinal motility disorder, which clinically resembles Hirschsprung's disease (HD). Adventitial fibromuscular dysplasia (AFMD) consists of proliferation of smooth muscle cells and collagen fibers in the adventitia of blood vessels. The purpose of this study was to investigate vascular abnormalities in large bowel biopsies from patients with isolated HD, IND associated with HD and isolated IND. Large bowel biopsies from patients presenting with isolated HD ( n=23), IND associated with HD ( n=11), isolated IND ( n=16) and normal bowel as controls ( n=6) were investigated using acetylcholinesterase (AChE) histochemistry, van Gieson staining and alpha-smooth muscle actin (alpha-SMA) immunohistochemistry. Increased AChE activity around submucosal vessels was found in 9/16 (56%) cases with isolated IND, 3/11 (27%) cases of IND associated with HD, 5/23 (21%) isolated HD cases and 0/6 controls. AFMD was found in 10/16 (62%) of the isolated IND cases, 4/11 (362) of the cases with IND associated with HD and 4/23 (17%) cases of HD without IND using van Gieson staining. None of the control specimens revealed AFMD. Increased alpha-SMA immunoreactivity filaments were demonstrated in the submucosal vessel wall in 9/16 (56%) of isolated IND and 2/11(18%) of IND associated with HD cases. Normal alpha-SMA immunoreactivity around submucosal vessels was seen in isolated HD and controls. Abnormal submucosal vasculature is a common histological finding in isolated IND and IND associated with HD and may be a useful additional diagnostic feature in these patients.

Purpose: Interstitial cells of Cajal (ICCs) are pacemaker cells that play an important role in the control of gut motility. Carbon monoxide (CO) has been proposed as an endogenous messenger molecule between ICC and smooth muscle cells in the gastrointestinal tract (GIT). Heme oxygenase-2 (HO-2) is the main physiologic mechanism for generating CO in human cells. The aim of this study was to investigate the immunocolocalization of the HO-2 and ICCs in normal and aganglionic bowel of Hirschsprung's disease (HD). Methods: Full-thickness specimens were obtained from aganglionic colon during pull-through operation from 10 patients diagnosed as having HD. Normal control large bowel specimens were collected from 4 patients during bladder augmentation procedures. Double immunostaining was carried out using c- kit and HO-2 antibodies. Immunolocalization was detected by means of confocal laser scanning microscopy. Results: HO-2 immunoreactivity (IR) was found in many ICCs present around the myenteric plexus, within the longitudinal and circular muscle layers and at the innermost part of the circular muscle layer in normal colon. In the aganglionic colon there was absence of HO-2 IR in the sparsely found ICCs. In the transitional zone of HD bowel the colocalization of HO-2 IR and ICCs was much reduced compared with controls. Conclusions: The results of this study provide the first evidence for the presence of HO-2 immunoreactivity in the ICCs in normal human colon and absence of HO-2 immunoreactivity in sparsely appearing ICCs in the bowel of HD patients. The lack of HO-2 in the ICCs in the bowel of HD patients may result in impaired intracellular communication between ICCs and SMCs causing motility dysfunction. J Pediatr Surg 38:73-77. Copyright 2003, Elsevier Science (USA). All rights reserved.

Two pairs of omphalopagus twins were encountered at the Polish Mother's Health Institution in Lodz, Poland during the past 15 years. In the first set the prenatal diagnosis was not established and the delivery of the twins in a regional hospital was a complete surprise. Both babies died. In the second case the conjoined twins were diagnosed prenatally, surgical separation was successful, and both twins survived. The prenatal identification of conjoined twins is of cardinal importance for the planning of delivery and possible separation.

Objective: The goal of the work was a comparative analysis of intrauterine therapy among pregnant women with diagnosed fetal uropathy and unilateral hydronephrosis. Methods: The study was conducted on a group of 98 pregnant women hospitalized at the Department of Gynecology, Fertility and Fetal Therapy, Polish Mother’s Memorial Hospital between 2006-2012. The study group included 77 cases of fetal obstructive uropathy (79%) and 21 cases of unilateral hydronephrosis (21%). Both groups have been analyzed in terms of prognosis. Follow-up has been conducted and results have been statistically analyzed. Results: In 11% of cases of obstructive uropathy therapy early complications were observed. The remaining 89% of the patients delivered at a later date, of which 54% after 34 weeks of pregnancy. The average weight of a newborn was 2645g and Apgar score – 7.45. 73% of newborns were discharged home in good condition, with preserved diuresis. Conclusions: 1. The use of intrauterine therapy has a positive effect on fetal condition and newborn postnatal state. 2. Unilateral hydronephrosis group was characterized by a significantly longer duration of pregnancy, higher birth weight, Apgar score, and greater survival of newborns. 3. In the group of obstructive uropathy survival improvement was achieved and dieresis was preserved. Intrauterine interventions were associated with a low risk of complications.

Bezoars are concretions of human or vegetable fibers that accumulate in the gastrointestinal tract of humans and animals. Patients may remain asymptomatic for many years, and symptoms develop as bezoars increase in size to the point of obstruction or perforation. THE AIM OF THE STUDY WAS TO PRESENT: 3 cases of bezoars treated in our centre and review of the current knowledge about this disorder, emphasizing the diagnostic difficulties.Physicians should consider bezoars as a differential diagnosis of acute abdomen. As bezoars may remain asymptomatic for many years, monitoring of pathogenetic and predisposing factors is essential, in particular in compulsive disorders.

Peristaltic contractions in the upper urinary tract serve to move urine from the kidney through the ureter to the bladder. Ureteropelvic junction (UPJ) obstruction is the most common cause of congenital hydronephrosis in children. To our knowledge the pathophysiology of UPJ obstruction is unknown. C-kit positive interstitial cells of Cajal (ICCs) are pacemaker cells that facilitate active propagation of electrical events and mediate neurotransmission. We investigated the expression of c-kit positive cells in the muscle layer of normal and obstructed UPJ specimens.A total of 19 human formalin fixed, paraffin embedded specimens of intrinsic UPJ obstruction from children with a mean age of 2.3 years (range 2 months to 12 years) and 7 control samples from children with a mean age of 4.5 years (range 11 months to 9 years) were investigated immunohistochemically for the expression of c-Kit oncoprotein and peripherin by light and laser scanning microscopy. Quantification of immunolabeled structures was quantified using computerized image analysis.Peripherin immunoreactivity was strong in the muscle layer of normal UPJ specimens, while in UPJ obstructed specimens there was a decrease in peripherin positive nerve fibers. In normal UPJ specimens there were many c-Kit positive ICCs between the muscle bundles. The density of ICCs was markedly decreased in the muscle layers of UPJ obstructed specimens.To our knowledge this study shows for the first time the immuno-expression of c-Kit positive ICCs in the proximal part of the normal human upper ureter. The altered density of c-Kit positive cells in UPJ obstruction may have a role in the failure of transmission of peristaltic waves across the UPJ.

Ileus due to adhesions is a common postoperative complication. However, there are only rare reports of this condition occuring in children. The aim of this study was to determinate the risk of incidence of intestinal adhesion ileus in children after abdominal operations.Retrospective studies were carried out on a group of 94 children operated in the Department of Pediatric Surgery and Urology of the Polish Mother's Memorial Hospital - Research Institute in Łódź in the years 1996-2005, in whom adhesions were found on relaparotomy. The following parameters were analysed:indication for operation age at first laparotomy, timeintervalbetweenl aparotomyandrelaparotomy, localization and extent of adhesions, complications after adhesiolysis, indications for relaparotomy.Among 1987 children in whom laparotomy was performed, in 94 intestinal adhesions were found. The majority of relaparatomies were performed in the first 6 months after the primary operation.Adhesive bowel obstruction is a frequent and serious complication after laparotomy especially in the neonatal period. It occurs most frequently in the 6 months after the primary operation.

Congenital diaphragmatic hernia (CDH) in most cases presents immediately or within hours after birth with signs of respiratory failure: dyspnea, tachypnea, and cyanosis. Late presentation of CDH (beyond the neonatal period) is less common and represents true diagnostic challenge. Diagnosis of the defect is difficult. Symptoms are non-specific and can be misleading. Late onset of this anomaly must be considered in the differential diagnosis of chronic gastrointestinal or respiratory symptoms. The aim of this report is a case of 17 year old boy with delayed presentation of congenital diaphragmatic hernia.

Obesity, which is a chronic systemic disease, is one the main health problems in contemporary society. Prevalence of obesity in childhood obesity and promotes the occurrence of many chronic diseases into adulthood. The aim of the study was to assess the relationship between obesity and child obesity, and lifestyle conditions in the family.The study group included 100 children aged 4-18 years, with a body mass index (BMI) above 97 centile. Respondents (44 girls and 56 boys) were selected from among children treated in the period from April 2008 to May 2009, the Department of Pediatric Surgery and Urology with general diseases, scheduled treatments not associated with obesity. In each patient, based on a prepared questionnaire, has been interviewed for dietary habits, family history in the direction of obesity and other comorbidities. The measurements of height and weight and abdominal circumference and waist were also made.The studies show that obesity is most common in children aged 9-13 years. The majority of obese children has errors habits. In the study group found the relationship between the prevalence of obesity in children and obesity occurring in the immediate family. It was observed that, in the oldest patients (14-18 years) most attempts to treat obesity.The percentage of obesity in children and their parents were similar and remained tied with overweight and obesity in the family. Therapeutic interventions are undertaken mainly in older children. In addition, children aged 9-13 years usually reach for foods with a fast food, which can negatively associated with rarely undertaken interventions aimed at weight loss.

Context.—Interstitial cells of Cajal (ICCs) are pacemaker cells, which are of fundamental importance in regulating gastrointestinal motility. Recent evidence suggests that carbon monoxide is a neurotransmitter involved in neurotransmission between ICC and smooth muscle cells. Heme oxygenase-2 (HO-2) is the major physiological mechanism for the generation of carbon monoxide in the enteric nervous system. Objective.—To investigate the immunocolocalization of HO-2 and ICCs in the normal pylorus and in infantile hypertrophic pyloric stenosis (IHPS). Design.—Specimens from 18 infants with IHPS and 8 control specimens were examined using double-immunostaining with c-Kit and HO-2 antibodies. The immunolocalization was detected with the help of confocal laser scanning microscopy. Results.—Abundant HO-2 immunoreactivity was found in ICCs in the smooth muscle layer of normal pylorus. There was a decrease in the number of ICCs and lack of HO-2 immunoreactivity in ICCs in IHPS. Conclusions.—The results of the present study provide the first evidence for the presence of HO-2 in ICCs in the normal human pylorus. The lack of ICCs and HO-2 in IHPS suggests impaired intracellular communication between ICCs and smooth muscle cells, contributing to motility dysfunction in IHPS.

Context.—Interstitial cells of Cajal (ICCs) are pacemaker cells in the smooth muscles of the gut. The internal anal sphincter (IAS) is the most caudal part of gastrointestinal tract. It has the important function of maintaining fecal continence. It has been proposed that ICCs in the IAS mediate the inhibitory innervation of the recto-anal reflexes. Objective.—To investigate the distribution of ICCs in the normal IAS and in the IAS of children diagnosed with internal anal sphincter achalasia (IASA) and Hirschsprung disease (HD). Methods.—At the time of IAS myectomy, specimens of the IAS were taken from 8 patients with IASA, 4 patients with HD, and 4 normal controls. All specimens were examined using anti–c-Kit and antiperipherin antibodies; immunolocalization was detected with light microscopy. Density of the ICCs was graded by computerized image analysis. Results.—There was strong peripherin immunoreactivity in the ganglia cells and nerve fibers in the normal IAS. The number of peripherin-positive nerve fibers was markedly reduced in the IAS in patients with IASA. In HD patients, there was lack of peripherin immunoreactivity in the IAS, but hypertrophic nerve trunks stained strongly. Many c-Kit–positive ICCs were present among the muscle fibers and between the muscle bundles in the normal IAS. In HD and IASA patients, ICCs were absent or markedly reduced. Conclusion.—Altered distribution of ICCs in the internal sphincter in IASA and HD may contribute to motility dysfunction in these patients.

The aim of this study was to investigate the expression and distribution of SK2 and SK3 channels in the normal and Hirschsprung's disease (HD) bowel.Full-thickness colonic specimens were collected at pull-through operation from 10 patients with HD and from 6 patients during bladder augmentation. Reverse transcriptase-polymerase chain reaction (RT-PCR) analysis for SK2 and SK3 channels and double immunostaining using anti SK2/c-kit, SK3/c-kit, SK2/alpha-SMA, and SK2/PGP 9,5 antibodies was performed. Immunolocalization was detected using laser scanning microscopy.RT-PCR analysis showed strong expression of SK2 and SK3 mRNA in the normal human bowel and significantly reduced SK3 expression in the aganglionic bowel (P.05). In the normal colon, double labeling immunohistochemistry showed strong SK3 immunoreactivity (IR) colocalizing in the c-kit-positive ICCs. In the aganglionic bowel, SK3 IR was reduced markedly in the sparsely found ICCs. There was strong SK2 IR mainly in smooth muscles in the normal and aganglionic bowel.The results of this study provide the first evidence for the presence of SK2 and SK3 channels and for the immunocolocalization of SK3 channels in the ICCs in the normal human colon. Decreased expression SK3 channels in the aganglionic bowel may contribute to motility dysfunction in HD.

Routine contralateral groin exploration in girls with unilateral inguinal hernia (UIH) continues to be controversial. The aim of this study was to determine the incidence of contralateral hernia development in girls after UIH repair.Between 1972 and 2000, 391 girls underwent repair of UIH. Ninety-one (23%) of the 391 girls underwent routine contralateral exploration during UIH repair and were excluded from the study. The median age at operation in the remaining 300 girls was 3.3 years (range, 1 month to 14 years). Two hundred ten (70%) girls had right-sided and 90 (30%) had left-sided UIH. Familial history of hernia was identified in 8 (3%) patients. The follow-up ranged from 1.5 to 14 years. Mann-Whitney U test was used for intergroup comparison.A contralateral hernia developed in 24 (8%) of the 300 patients who had unilateral repair of inguinal hernia. Median time from operation to occurrence of the contralateral hernia was 3 years (range, 1 to 4 years). Age at operation, side of hernia, and familial history did not influence the development of contralateral hernia.These data suggest that the low incidence of contralateral hernia development in girls undergoing UIH repair does not justify routine contralateral groin exploration.