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A 14-year-old girl presented with malignant hypertension. Physical examination and abdominal computerized tomography revealed a paraaortic mass. Urinary catecholamines, serum renin, and aldesterone levels were high. The mass was totally excised and a paraganglioma of the organ of Zuckerkandl was confirmed. The hypertension resolved after surgery.

The cremaster muscle (CM) has been considered to participate in regulation of blood flow and temperature of the testis. Its contribution to testicular descent has been suggested. However, there is limited information about the CM in physiological and pathological states. Therefore, an experimental study has been conducted to evaluate and compare the contractile and electrophysiological properties of CM in boys with descended or undescended testes.Identical CM strips were obtained from eight boys who underwent orchidopexy with a mean age of 3+/-2.2 years and from eight boys who underwent herniorrhaphy with a mean age of 4+/-1.3 years. Muscle strips of 3 x 8 mm were vertically attached to an isometric force displacement transducer, and direct muscle contractions were elicited by rectangular electrical pulses. Direct isometric muscle contractions were recorded in an organ bath containing mammalian Ringer's solution. In electrophysiological experiments, conventional microelectrode techniques were used.Direct electrical stimulation of CM strips obtained from patients with descended and undescended testes elicited muscle twitches and frequency-dependent contractile responses. Tetanic contractions of undescended testes at 100 Hz were 67% greater in amplitude than that of descended testes (P.002). Muscle strips of both groups exhibited increased twitch amplitudes by 105%+/-37% when the temperature of the bathing solution was increased from 22 degrees to 37 degrees C (P.001). The electrophysiological findings were similar.Contrary to other striated muscles, elevated temperature increases the contractility of CM. If the increased contractility by an increase in temperature is a property unique for CM, it should reflect the attempts at regulating testicular blood flow or temperature. The increased amplitude of contractions encountered among the CM of boys with undescended testis suggests the CM to have a role on the location of the testis.

Between 1976-1993, 302 patients were diagnosed to have Hirschsprung's disease (HD) in Hacettepe University Children's Hospital. All patients have been searched for the occurrence of associated anomalies and other parameters including 1) sex, 2) gestational age, 3) birth weight, 4) length of aganglionic segment, 5) familial occurrence, 6) consanguinity. Forty-nine of 302 patients were proved to have 83 associated congenital anomalies together with HD. Distribution of anomalies was as follows; central nervous system and special senses 28.92%, genitourinary system 26.51%, skeletal 24.09%, gastrointestinal 12.05%, cardiovascular 8.43%. In eleven patients more than one anomaly involving different systems were detected; only one of these patients was female and six of them have shown short segment disease. Down's syndrome was present in 5 patients. Female: male ratio was 1:4.2 for the whole series and 1:6 for the patients with Hirschsprung associated congenital anomalies (HACA). In the HACA group the percentage increased in favor of long-segment and extensive aganglionosis without statistical significance (p>0.05). The majority of patients were term babies and their birth weights were between 2500g < 3500g. Consanguinity was present in 36 families. No affected parents were detected but two sisters and four brothers were proved to have HD. Three pairs of twins, one of which was monozygotic, and all showing discordance were detected. There was no difference between HACA group and HD without HACA in terms of sex, birth weight, length of aganglionic segment and consanguinity. Although HD seemed to be genetically heterogeneous HACA did not have a genetically different origin from that of HD.

Background: H-type tracheoesophageal fistula (TEF) was diagnosed in 12 patients during the 25 years from 1971 to 1996. Although all patients were symptomatic since birth, in 58% of the cases, there was a diagnostic delay ranging from 26 days to 4 years. Methods: Esophagography and/or cineesophagography, or bronchoscopy and simultaneous methylene blue administration confirmed the diagnosis of H-type TEF. Although cineesophagography is highly effective in demonstrating H-type TEF today, bronchoscopy must be used in every patient suspected of having fistula, especially when the radiological methods fail. Bronchoscopy is helpful in diagnosis, in evaluation of associated respiratory tract anomalies, and in treatment. Results: Two newborn patients could not have been operated on because of pulmonary insufficiency resulting from delayed recognition of fistula. Ten patients underwent fistula repair, nine through cervical approach, and one through thoracotomy, with seven survivors. Conclusions: The results suggest that early diagnosis is essential in the newborn period. Oversight in identification of H-type TEF, unsatisfactory radiological method, masquerading symptoms related to associated anomalies, and minute symptoms in some patients may cause delay in diagnosis. Radiological and endoscopic procedures are complementary in both diagnosis and treatment of H-type TEF.

An immunocytochemical study was performed to assess the role of beta-1 integrins in the pathogenesis of Hirschsprung's disease. Fresh tissue samples from both aganglionic and ganglionic segments of five patients who were undergoing surgery for Hirschsprung's disease were obtained. Samples were rapidly frozen in liquid nitrogen. Sections were cut and stained using anti alpha-1, 2, 3, 4, 5, 6 and beta-1 monoclonals according to the indirect immunoperoxidase method. The evaluation did not reveal any significant change of pattern in the distribution of beta-1 integrins in the non-neural elements of both aganglionic and ganglionic segments of colon, such as the epithelium, muscularis mucosa, muscularis externa, connective tissues and blood vessels. Nerve fibres in both aganglionic and ganglionic segments strongly expressed the alpha-6 chain of very late activation antigen which led to their increase in the aganglionic segment. In addition to revealing the increase, alpha-6 monoclonals also had in situ positive control due to their presence in non-neural elements. Hence, immunostaining of the suction biopsies with anti alpha-6 monoclonals may be employed as a new and simple method in the diagnosis of Hirschsprung's disease. On the other hand, beta-1 integrins do not seem to play a role in the defective migration of ganglion cells occurring in Hirschsprung's disease.

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare, diffuse progressive interstitial lung disease that affects females of childbearing age and is characterized by diffuse proliferation of abnormal smooth muscle fibers predominantly developing in the lung and leading to cystic destruction. A prepubertal 13-year-old girl with PLAM associated with renal and hepatic angiomyolipomas who was treated by nephroureterectomy and thoracoscopic pleurodesis is presented. To the best of our knowledge, this is the first reported case of PLAM associated with renal and hepatic angiomyolipomas at the prepubertal age. After evaluating the clinicopathologic features of this rare entity, the authors conclude that PLAM should be considered in the differential diagnosis of cystic pulmonary pathologies in children, particularly teenagers. The most important clinical aid is to bear this rare entity in mind when a child presents with renal and/or hepatic angiomyolipomas. Thoracoscopic pleurodesis is the most effective treatment modality for recurrent pneumothoraces.

A 1-year-old girl with Waardenburg syndrome type I presented with double collecting system of left kidney accompanied by nonobstructive hydronephrosis of lower pole and by ureteropelvic junction obstruction of right kidney. Renal involvement in Waardenburg syndrome was reported once in a 4-month-old boy with unilateral duplication of the renal collecting system and in a 16-day-old girl who had right multicystic dysplastic kidney and hydronephrosis in the left kidney. The third case of renal involvement in Waardenburg syndrome is presented here, with special emphasis on early diagnosis and management of renal anomaly. The authors conclude that urinary system anomalies should also be considered in the wide spectrum of Waardenburg syndrome clinical features to avoid life-threatening complications.

229 patients atteints de maladie de Hirschsprung qui ont eu une operation definitive entre 1976 et 1993 ont ete etudies pour preciser la survenue de complications inhabituelles dans 1'Hopital d'Enfants de l'Universite d'Hacettepe. 213 de ces patients ont ete operes dans notre Hopital et 16 nous avaient ete adresses apres l'operation definitive, la complication la moins exceptionnelle, dans cette serie, etait la fistule, en particulier recto-urinaire, recto-vaginale, ileo-rectale et peri-anale. Il patients operes par la technique de Swenson ont presente une fistule recto-uretrale qui a necessite une reintervention de Swenson apres une tentative de reparation directe non suivie de succes. Dans cette serie, nous avons detecte 2 patients avec fistule ileo-rectale, l'un entre eux avait egalement une fistule peri-anale. 2 patients ont presente une enterocolite rebelle a tout traitement; il s'agissait chez ces 2 malades d'une fistule ileo-rectale qui a pu etre reparee avec succes. Nous avons rencontre 2 fistules recto-vaginales chez des patients qui nous ont ete adresses apres operation definitive, ces deux patients ont ete reoperes. Un patient a ete adresse apres une operation de Swenson avec une fistule peri-anale et a subi une fistulotomie. Le traitement des fistules recto-urinaires et recto-vaginales doit etre une reintervention complete sinon il aboutit toujours a un echec.

It is well known that Helicobacter pylori infection is extremely common throughout the world, but most infected persons do not develop ulceration and remain asymptomatic. This study was undertaken in order to determine the prevalence of H. pylori infection, the age distribution, and the factors that may affect the frequency of H. pylori infection. We evaluated the presence of antibody against H. pylori in children and adolescents, we also assessed the efficacy of non-invasive and invasive methods for detection of H. pylori infection in children who had undergone upper gastrointestinal endoscopy. We evaluated 43 males and 18 females whose ages ranged from 1 to 17 years (mean age: 9.4 +/- 4.2). 29 of all cases were symptomatic and 32 were asymptomatic. H. pylori infection was present in 49% of the studied cases. The data were analysed by Fisher's exact chi-square and Mantel-Haenszel tests. It was found that H. pylori infection graphically increased with age in both groups from 25% at ages 3-5 to 80% at ages 16-20. But no association was determined statistically between age and H. pylori positivity (p0.05). H. pylori infection was determined in 14 (48%) and 16 (50%) cases in symptomatic and asymptomatic groups respectively. There was no significant difference between H. pylori and either group (p0.05). According to the endoscopy findings, the symptomatic group was divided into two subgroups: children with peptic ulcer and children with non-ulcer dyspepsia (NUD). An attempt was made to compare ulcer, non ulcer dyspepsia and asymptomatic cases with H. pylori positivity, but no significant relation could be established between the groups (p0.05). 14 (23%) of the cases had previous gastric and/or intestinal complaints in their parents (positive family history). There was a significant, statistically positive relation between family history and symptoms (p0.01). Nevertheless, no association was found between H. pylori infection and family history (p0.05). There were no significant differences in H. pylori infection related to sex, type of housing, location of housing, socio-economic status (SES) or source of water supply. It is concluded that H. pylori infection has a high prevalence in our country. Although endoscopic evaluation is an invasive method, under our conditions, histopathological examination with 97% is shown to be much more sensitive in determination of H. pylori infection. Since no community difference was determined in the prevalence of H. pylori infection, all children should be considered to be under the threat of the same risk.

Le mecanisme des deteriorations du testicule non descendu et de la glande controlaterale en cas de mal-descente unilaterale reste controverse. Une etude experimentale a ete planifiee pour evaluer les parametres d'hypoxie tissulaire dans le testicule ipsilateral et controlateral apres une maldescente induite chirurgicalement chez les rats qui avaient subi prealablement un traitement placebo ou une sympathectomie chimique. Cette experimentation a ete faite en pensant que des alterations dans la perfusion tissulaire pouvaient jouer un role dans la deterioration testiculaire ipsi ou controlateral. 60 rats albinos mâle ont ete utilises pour cette experimentation. La moitie, soit 30 rats, ont ete traites par placebo et l'autre moitie par sympathectomie chimique. Au 21eme jour, chaque moitie fut divisee en trois groupes, un groupe sans traitement, un groupe avec fixation d'un testicule dans l'abdomen et un groupe avec fixation des deux testicules dans l'abdomen. Les rats subirent une orchidectomie bilaterale a l'âge de 3 mois et l'acide lactique (LA) et l'hypoxanthine (HX) furent mesures dans le tissu testiculaire. La maldescente testiculaire entraine une augmentation marquee de LA et HX dans le testicule ipsilateral en comparaison avec les testicules controles et egalement entraine une augmentation de LA et HX dans le testicule controlateral. Les niveaux de LA et HX sont plus bas dans le testicule non descendu ipsilateral; en contre-partie, le niveau est plus eleve dans le testicule controlateral notamment en comparaison avec le testicule controlateral chez les rats ayant subi une sham-operation apres traitement par placebo. La maldescente bilaterale entraine une elevation similaire de LA et HX dans les deux testicules. L'administration de 6-hydroxydopamine n'entraine pas de diminution de LA et HX dans le testicule ipsilateral apres la maldescente testiculaire unilaterale. Cependant, le niveau de LA dans le testicule normalement descendu controlateral ne revele pas une difference significative en comparaison avec des testicules controlateraux des rats ayant subi une operation sham. Cette experimentation revele que la maldescente entraine une hypoxie tissulaire non seulement dans le testicule ipsilateral mais egalement dans le testicule controlateral. Une sympathectomie chimique a peu d'effets preventifs sur l'hypoxie controlaterale dans le cas d'une maldescente unilaterale. L'hypoxie tissulaire survenant pendant la maldescente peut entrainer une inadequation relative d'irrigation testiculaire pour compenser l'augmentation des demandes metaboliques sous condition hyperthermique.

Quoique la chirurgie thyroidienne soit principalement basee sur le risque de malignite, la determination des cas de malignite thyroidienne chez les patients porteurs d'une thyroide volumineuse, est un sujet de discussion chez les enfant. Une etude clinique retrospective a ete entreprise pour definir quels types d'augmentation de volume de la thyroide ont une potentialite clinique ou histologique de malignite. Au total, 137 patients qui ont subi une chirurgie thyroidienne pour tumeur sur une periode 15 ans ont ete etudies. La serie est composee de 90 cas (65,7 %) de goitre uninodulaire, 40 cas (29,2%) de goitre multinodulaire et 7 cas (5,1 %) de goitre diffus. Les examens histopathologiques ont trouve 9 patients (6,6 %) porteurs d'un carcinome thyroidien differencie. Tous les autres patients avaient des tumeurs benignes. L'analyse des resultats a montre qu'il n'y avait pas de possibilite de prediction ideale d'une malignite thyroidienne, pas plus sur le plan clinique ou les caracteristiques de laboratoire que sur les notions d'âge, de sexe, les symptomes evocateurs, une irradiation anterieure, le type de nodule, la localisation et le siege des nodules, les adenopathies lymphatiques, les tests de fonction thyroidienne, l'echographie et le scanner thyroidiens. Toutes ces caracteristiques etudiees n'ont pas de signification importante pour une valeur predictive positive ou negative. Seulement deux parametres montrent une specificite elevee au dela de 50 % pour le diagnostic d'une tumeur vraisemblablement maligne : une taille d'au moins 2,5 cm associee a des adenopathies cervicales. En consequence, de nouvelles regles plus valables doivent etre recherchees pour l'evaluation chirurgicale thyroidienne chez l'enfant ; la biopsie par aspiration a l'aiguille fine qui est utilisee couramment chez l'adulte peut etre une approche valable en depit de quelques notions restrictives chez l'enfant.

Objective To evaluate the effects of abdominal testis on ipsilateral and contralateral testicular histology, fertility and fecundity in rats which were chemically sympathectomized neonatally. Materials and methods The study comprised 60 male albino rats, 30 receiving a placebo and 30 a chemical sympathectomic agent neonatally. When 21 days old, the two groups of 30 rats were each divided into three groups and underwent a control operation or abdominal fixation of one or both testes. After they were 8 weeks old, each male rat was housed with two fertile female rats for 25 days, after which their testes were harvested. Females were observed for an additional 25 days and any pregnancies and the number of offspring were recorded. Mean seminiferous tubular diameters and mean testicular biopsy scores were determined for each testis. Results Although fertility and fecundity rates and mean testicular biopsy scores were not significantly different, chemical sympathectomy prevented the decrease in mean seminiferous tubular diameters which occurred in the contralateral testis. Conclusion Chemical sympathectomy protected contralateral testicular histology to some degree. Therefore, the sympathetic system may play a role in contralateral testicular deterioration.

Objective To investigate a possible mechanism (the vascular theory) in the development of congenital anomalies of the penis and urethra in the fetus of the goat. Materials and methods Fifteen male Angora goat fetuses underwent an operation at 110–114 days of gestation whereby branches of the internal pudendal artery within the superficial compartment of the perineum were ligated. Five male fetuses served as controls in which the perineum was explored with no ligation. Results Five treated and three control live offspring were delivered spontaneously. Although no genitourinary anomalies were observed on gross examination, histopathological examination of the penises of treated animals revealed smaller penises with increased fibrosis. Conclusion A defective vascular supply can partly explain the morphogenesis of some aspects of the congenital anomalies of the penis and urethra.

Posttraumatic prostatomembranous urethral strictures (PUS) in children have special features that warrant consideration with respect to management. Transpubic urethroplasty (TPUP) was used to treat 10 children who presented with posttraumatic PUS. All patients previously had unsuccessful repair attempts with other techniques. Urethral stricture was noted in three patients who were cured within a few months by urethral dilatations. Appropriate antibiotic therapy was begun for prolonged urinary infection in four patients. Complete urinary continence was achieved in seven patients (70%), and three (30%) are still incontinent. In the latter, the bladder neck and posterior urethra were reported to have been damaged seriously, owing to extensive dissections at the time of initial treatment. Severe retropubic tissue loss and fibrosis were noted in these patients. Thus, the authors emphasize that avoiding further injury to any of the continence mechanisms that have survived the injury is as important as performing a patent urethral anastomosis. There were no significant complications or morbidity associated with TPUP. TPUP provided excellent exposure and visualization of the involved anatomy and facilitated accurate suture placement and tissue realignment in all patients.

Pseudocyst of the pancreas is an uncommon disorder in children. During the period of 1977 to 1990, the authors reviewed the records of 10 consecutive patients with pancreatic pseudocyst (PP) who were surgically treated in our institution. In 8 patients, the surgical procedure that was used was transgastric cystogastrostomy, and in 1 patient it was total excision of the pseudocyst. The remaining 1 patient underwent external drainage. After an average follow-up of 8.5 years, none of the patients had any complaint related to previous operation except the 1 patient who was treated with external drainage. Biochemical tests, abdominal ultrasonographies, and barium meals were also normal in 9 patients. The analysis of the results indicates that despite new percutaneous therapeutic modalities, it is still an internal drainage through transgastric cystogastrostomy that should be favored in treatment of PP in children.

Inflammatory pseudotumor (IPT) of the liver is a rare entity often mistaken for malignancy. All children reported to date have been diagnosed and treated surgically, whereas successful medical diagnosis and treatment has been reported in adults. In this report, we describe the 13th child presenting with IPT of the liver and review the literature to determine diagnostic means to avoid unnecessary surgery. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish IPT from other space-occupying liver lesions. The negativity of malignant liver tumor markers and hepatitis antigens and normal liver, renal, and other systems are supportive of IPT. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained liver mass. Fine-needle aspiration is the most helpful measure to confirm the diagnosis. Surgery should be avoided in the absence of life-threatening complications.

Gastrointestinal stromal tumor is a rare pathology in childhood. It may occur anywhere along the alimentary tract and represents with a wide spectrum of signs and symptoms according to location. An infant with malignant colonic stromal tumor that has presented with a huge abdominal mass and lower gastrointestinal bleeding is reported to discuss the difficulties in diagnosis and differential diagnosis of this unusual tumor.

An experimental study was performed to investigate gastrointestinal motility and meconium passage, with simultaneous blood gas measurements, in the fetuses of eight pregnant goats at 110 to 114 days' gestation (full term, 147 to 155 days). With the goats under halothane anesthesia, a nasogastric tube and a heparinized central venous catheter were inserted into the fetuses. 24 hours after surgery, 10 mL of gastric juice from the fetus was replaced with a nonhydrosoluble contrast medium, and serial roentgenograms and blood samples (for pH, PO2, and PCO2 measurement) were taken every 4 hours. All fetuses began to pass the contrast medium into the amniotic cavity within 16 to 22 hours, and central venous blood gas values were normal. The results of this study suggest that the fetus, which urinates routinely, also defecates routinely into the amniotic cavity, even in the absence of distress. The increased incidence of meconium staining in fetal distress conditions may be indicative of impaired clearance of amniotic fluid.

Objective To evaluate the effect of chemical sympathectomy on lactic acid and hypoxanthine concentrations in both testes during unilateral testicular torsion in a rat experimental model. Materials and methods Four groups, comprising 10 rats each, were studied. Group I was the control group, Group II rats received a placebo and underwent unilateral testicular torsion, Groups III and IV received guanethidine monosulphate and 6-OH-dopamine hydrobromide respectively, and underwent unilateral testicular torsion. The levels of lactic acid and hypoxanthine in both testes were measured. Results In rats in Group II the levels of lactic acid and hypoxanthine in both testes were significantly elevated when compared to the control group. In the groups of rats that had undergone chemical sympathectomy, the levels of lactic acid and hypoxanthine were significantly decreased in the contralateral testes compared to rats in Groups I and II. Conclusion Chemical sympathectomy prevents the elevation of lactic acid and hypoxanthine levels in the contralateral testis of rats with unilateral testicular torsion. Therefore, we conclude that contralateral testicular damage during unilateral testicular torsion may result through a reflex activating sympathetic system.

An experimental study has been conducted to investigate testicular blood flow alterations through acute biochemical changes during unilateral testicular torsion and detorsion.One hundred twenty male albino rats were divided into 12 groups, each containing 10 rats. One group served to determine basal values of biochemical parameters, 4 groups were subjected to varying periods of unilateral testicular torsion (3, 6, 12, and 24 hours, respectively), 3 groups were subjected to detorsion following varying periods of torsion (6, 12, and 24 hours, respectively), and 4 groups underwent sham operations as controls. Levels of lactic acid, hypoxanthine, and lipid peroxidation products were determined in testicular tissues.Values of these 3 parameters obtained from sham operation control groups did not differ significantly from basal values (p0.05). All 3 parameters were increased significantly in both ipsilateral torted and contralateral nontorted testes after unilateral testicular torsion when compared with basal values (p0.05). Detorsion did not cause significant changes in levels of lipid peroxidation products in both ipsilateral torted and contralateral nontorted testes when compared with values obtained after torsion (p0.05).Ipsilateral testicular torsion causes a decrease not only in the ipsilateral torted but also in the contralateral nontorted testicular perfusion. The clues of reperfusion injury do not become evident following detorsion of testicular torsion lasting more than 6 hours.

All the complications requiring laparotomy among 680 children who were diagnosed as having typhoid fever between 1981 and 1990, at Hacettepe University Children's Hospital, were retrospectively analyzed. Four patients developed intestinal perforation, two gastrointestinal bleeding, and two splenic abscess, with frequencies of 0.58%, 0.29%, and 0.29%, respectively. The overall frequency was 1.17%. While the risk was 1.58% among males, it was 0.42% among females with no statistically significant difference (P > .05). The risk was higher among patients above 6 years of age (P < .05). While perforation and hemorrhagic complications developed within 10 days of initial symptoms and splenic abscess required 10 and 30 days for development, there were no clinical signs and laboratory findings predicting the occurrence of complications in the course of typhoid fever. Two patients, one with intestinal perforation and the other with gastrointestinal hemorrhage, died within 2 days following the septic postoperative courses. On the other hand, 12 patients, among those without complications requiring laparotomy, died of unremitting septic course of the disease. The mortality rate rose from 1.78% to 25% when complications occurred. The findings in this series demonstrate that typhoid fever presents a challenge to the pediatric surgeons not only because of the complications requiring laparotomy with high mortality rates, but also the absence of criteria predicting the occurrence of complications in the course of typhoid fever.

In childhood, benign tumors of the cervix and corpus uteri are very rare. Clinical and pathological findings of a benign multilocular cystic polyp of the cervix uteri in a 5-year-old girl are presented and the origin of the polyp is discussed.

Thoracic neuroblastic tumors (NBTs) are reported to have better prognosis. We aimed to review clinical characteristics, treatment results, and outcome of our patients with thoracic NBT.Files of 87 children treated at our hospital between 1973 and 2007 with the diagnoses of thoracic NBT were reviewed for clinical and pathological characteristics. Treatment results and outcomes of these cases were examined.All but one tumors were located in posterior mediastinum, one in the posterior chest wall. Median age of all was 2.1 years (range, 0.03-14; F/M: 1.42). Fifteen cases had ganglioneuromas (GN), 26 ganglioneuroblastomas (GNBL), and 46 neuroblastomas (NBLs). Stages were: I, 20.5%; II, 22.1%; III, 38.2%; IV, 14.7%; IVS, 4.5%. Stages III and IV were more common in cases over 1 year of age. In 20 patients diagnoses were incidental. Twenty-two of 87 (25.3%) had symptomatic spinal cord compression and 15 (17.3%) had Horner syndrome. Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival rates did not differ depending on the age being younger or older than 1 year. Ten-year survival rates were 88.8% in stages I, II, IVS; 65.3% in stage III and 27.8% in stage IV (P = 0.0002).Thoracic NBLs had a favorable prognosis. This might be a result of earlier diagnosis and some distinct biological characteristics. Favorable prognosis would suggest less aggressive treatment for such patients. Further studies on the biological characteristics of NBLs in the thoracic site and their association with outcome should be done.

From 1972 through 1991, 404 patients with renal tumor (Wilms' tumor and clear cell sarcoma) were seen at the Pediatric Oncology Unit of Hacettepe Children's Hospital. The genital abnormalities and renal diseases in these patients were evaluated retrospectively. Eight patients with renal tumor had genital abnormality and/or renal disease of various types and degrees. One of these patients had a clear cell sarcoma while all the others had Wilms' tumor. Two patients had all components of the Drash syndrome. Two patients with Wilms' tumor had genital abnormality and mild proteinuria. Another two patients had only Wilms' tumor and renal disease. Two patients had only renal tumor and genital abnormality of whom one was the patient with clear cell sarcoma.

Renal oncocytoma is one of the most unusual benign lesions, which presents as a complicated mass resulting in a diagnostic and therapeutic dilemma. A new case of renal oncocytoma in a 13-year-old boy is presented. The clinocopathologic features of this rare entity are discussed, with special emphasis on diagnosis and treatment. There are no specific presumptive clinical and laboratory findings, including tumor markers, ploidy analysis, and imaging techniques that distinguish oncocytoma from other renal masses. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained renal mass. Frozen section biopsies followed by partial nephrectomy are mandatory for the appropriate treatment after excluding bilateral or multifocal occurrence.

An 11-month-old girl with a prolonged history of bloody, mucoid diarrhea is presented. Although the initial diagnosis given by the rectosigmoid biopsy obtained during laparotomy was neuronal intestinal dysplasia, accompanying findings including mixed inflammatory cell infiltration of the mucosa and submucosa with mucosal ulcerations suggested nonspecific colitis. The subsequent biopsy specimen that was obtained after performing colostomy and treating with broad-spectrum antibiotics and rectal irrigations showed improvement in the structure of ganglion cells and submucous and myenteric plexuses. Although the mucosal ulcerations and inflammatory reaction improved, the colonic stricture persisted, so the Duhamel procedure was performed, and the patient had an uneventful outcome. It is claimed that inflammatory disease of the rectosigmoid colon of unknown etiology and neuronal intestinal dysplasia have occurred together in the current case or that one disease might cause the other in time.

The records of 230 adhesive small bowel obstruction (ASBO) episodes in 181 patients have been reviewed to observe the place of conservative treatment and to establish criteria to predict the success of conservative trial. Immediate operation has been reserved for 81 episodes that have presented with fever and leucocytosis and/or localized abdominal tenderness, or complete obstruction. The remaining 149 episodes have initially undergone conservative trial. Although 110 episodes (73.8%) have been cured with conservative trial, 39 (26.2%) subsequently necessitated surgical intervention. No adverse occurrences have been observed during or after delayed operations. There was no strangulated bowel nor mortality both in delayed operation and conservatively treated groups. Recurrence has occurred with rate of 18.75% and 36.47% after surgery and conservative treatment, respectively, being significantly different (P less than .01), but the treatment method of the previous episode has been without influence on the method used in the recurrent obstruction (P less than .05). Among the assumed predictive criteria, age at recent laparotomy (P less than .02), time elapsed between recent laparotomy and obstructive episode (P less than .01), the primary condition necessitating laparotomy (P less than .01), the incision of previous laparotomy (P less than .05), and duration of conservative trial (P less than .01) correlated significantly with the success of conservative trial. The number of previous laparotomies and obstructive episodes (P less than .05) have not showed correlation. By the conservative approach used in selected patients with ASBO, 40% overall have been spared operation, without any adverse occurrences. Using the proposed criteria, the success rate of conservative treatment can be predicted.

Gastric volvulus is a rare entity in children. During the last ten-year period of 1977 to 1987, 21 children were operated upon with the diagnosis of chronic gastric volvulus at The Department of Paediatric Surgery, Hacettepe University School of Medicine. This study includes retrospective analysis of these cases. The patients were investigated with respect to associated anomalies, radiological diagnosis, surgical treatment and prognosis. Results are presented and discussed.

Purpose: A retrospective clinical study was performed to find out whether serum biochemistry alterations may serve to differentiate the traumatic bladder perforation to be either intraperitoneal or extraperitoneal. Methods: Thirty-two children treated for traumatic bladder perforation between 1970 and 1997, inclusive, formed the study group. Patients were divided into two groups: intraperitoneal bladder perforation (IBP) and extraperitoneal bladder perforation (EBP) groups. The groups were compared with regard to age, sex, mechanism of injury, and hemodynamic parameters (blood pressure, hematocrit) at presentation (each group was further subdivided into two subgroups according to the duration between trauma and presentation as early (duration 24 hours) presentation groups). The subgroups were compared with each other with respect to serum concentrations of urea, creatinine, sodium, potassium, and chloride. Results: There were 21 boys and 11 girls with a mean age of 8.7 ± 4.9 years. Mechanisms of injury were motor vehicle accidents (75%), falls (19%), and gunshot wounds (6%). There was no significant difference between the IBP and EBP groups with regard to age, sex, mechanism of injury and hemodynamic parameters at presentation. The biochemical parameters were found to be within normal range in the EBP group, whereas significantly higher levels of creatinine, potassium, and lower level of sodium were noted in IBP group regardless of presentation time. Significantly higher level of serum urea was recorded in a late presentation group of IBP patients. Conclusions: The authors emphasize that patients presenting with IBP are more likely to present with significantly higher levels of creatinine, potassium, and lower level of sodium compared with the patients with EBP regardless of presentation time. Increased level of serum urea concentration is a characteristic of IBP patients with late presentation. Biochemical alterations can be used to differentiate traumatic IBP and EBP in children with subtle physical examination and radiological findings.

A newborn case with cardiac rhabdomyoma is presented to discuss the differential diagnosis of mediastinal tumors around the heart in children. The diagnosis of rhabdomyoma can be suggested by modern visualization methods such as computerized tomography, magnetic resonance imaging and echocardiography. However, surgery is clearly indicated in cases with obstruction of flow tracts, valvular dysfunction and dysrhythmia, and in cases with indefinite diagnosis, or in the absence of association with tuberous sclerosis. Cardiac rhabdomyoma should be included in the differential diagnosis of masses located adjacent to the heart in infants.

Epididymo-orchitis, an uncommon cause of acute scrotum in prepubertal boys, is infection or inflammation of epididymis and testis. Epididymo-orchitis may be associated with urinary tract infections or reflux of urine predisposed by an underlying vasal anomaly. Two infants with anorectal malformations who presented with acute scrotum are reported. The surgical exploration of the testes showed findings consistent with epididymo-orchitis. Further radiological investigations of urinary tract showed vasal anomalies in both patients. If a patient with anorectal malformation presents with acute scrotum, epididymo-orchitis should be suspected initially. Evaluations should be directed toward defining predisposing vasal anomaly, and appropriate therapeutic measures should be undertaken to prevent recurrences.

Bilateral absence of the pectoralis major muscle with accompanying abnormalities of shoulder muscles has been reported in patients without Poland anomaly (PA). However, symmetric absence of pectoralis major muscles, hypoplasia of breasts and nipples with symmetric chest wall deformity and bilateral hand anomaly has not previously been reported. A 6-year-old girl with bilateral absence of pectoralis major muscles and hand involvement and symmetric chest wall deformity is, to our knowledge, the first known case of bilateral Poland anomaly.

Etat de la Question: La vaginoplastie est indiquee chez les patientes porteuses d'une aplasie des canaux de Muller, d'un syndrome d'insensibilite aux androgenes ou d'autres pseudohermaphrodismes masculins. Plusieurs techniques de reconstruction vaginale ont ete decrites. Nous rapportons ici la technique et les resultats de la vaginoplastie utilisant le colon sigmoide chez six adolescentes. Patients et Methodes: Entre 1990 et 2003, six patientes ont eu une vaginoplastie sigmoidienne pour un deficit en 5α-reductase (n = 3), une insensibilite aux androgenes (n = 2) ou une atresie vaginale (n = 1). L'âge moyen etait de 16 ans (13 a 18 ans). Une antibiotherapie a large spectre et une preparation intestinale ont ete realises dans tous les cas. Un segment de colon sigmoide de 15 - 20 cm a ete positionne dans la filiere retro-vesicale. L'extremite proximale a ete fermee en deux plans chez les patientes avec un deficit en 5α-reductase ou un testicule feminisant. Une anastomose distale a ete realisee avec la cupule vaginale (deficit en Sa-reductase) ou avec le cul-de-sac vaginal rudimentaire (testicule feminisant). Le segment sigmoidien a ete interpose entre le cul-de-sac terminal du vagin atretique et le perine chez la patiente porteuse d'une atresie vaginale. Les patientes etaient informees de la necessite de realiser une irrigation vaginale quotidienne. Le neo-vagin etait examine et calibre sous anesthesie generale. Une dilatation vaginale reguliere n'etait pas recommandee. Resultats: Toutes les patientes sauf une ont eu des suites operatoires simples et ont pu quitter l'hopital a J7-J8 post-operatoire. Toutes les patientes ont eu un resultat cosmetique excellent, avec une longueur vaginale satisfaisante. Une des patientes a presente une stenose de l'orifice vaginal qui a bien repondu aux dilatations. La patiente avec une atresie vaginale (syndrome de Bardet-BidI) est decedee de complications a type de thrombose veineuse profonde, insuffisance renale et sepsis. Conclusion: La vaginoplastie colique sigmoidienne est une technique specifique qui semble appropriee pour la reconstruction vaginale chez l'enfant. Elle repond a tous les imperatifs demandes a la creation d'un neo-vagin. Elle donne un diametre et une longueur adaptes tout en induisant le minimum de lesions des tissus perineaux. Elle est spontanement humidifiee, facilement adaptable a l'uterus, le col et les segments vaginaux atretiques rudimentaires et ne necessite pas une dilatation reguliere. Les stenoses moderees de l'orifice vaginal peuvent etre traitees par des dilatations et des retouches chirurgicales peuvent etre realisees facilement dans les stenoses severes. Il existe neanmoins une morbidite liee a la laparotomie, et des complications severes peuvent survenir, liees a la pathologie initiale.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is an overgrowth syndrome and polyposis condition, which carries an increased risk for development of benign and malignant tumors of various tissues and organs. A 9-year-old boy with BRRS who had multiple subcutaneous mesenchymal tumors and follicular adenoma of the thyroid and a 14-year-old boy with multiple subcutaneous mesenchymal tumors and gastrointestinal polyposis are presented, with special emphasis on the surgical management of these patients.

Aim: To clarify the factors affecting the success rate of endoscopic subureteral injection (ESI) treatment of vesicoureteral reflux (VUR). Method: All patients who had ESI treatment for VUR at our unit between 1999 and 2002 were retrospectively reviewed with regard to age, gender, causes of VUR, reflux grade, type of injected materials, number of injections, results of cystourethrograms (VCUG), and outcome. The injected implants were gluteraldehyde cross-linked bovine collagen (GclBC), dextranomers in sodium hyaluronan (DiSH) and calcium hydroxylapatite (CH). A successful result was defined as absence of VUR confirmed by VCUG performed 3 months after the ESI procedure. Basic descriptive statistics were performed along with the χ2 test (p < 0.05 was significant). Results: 50 children (81 ureters) consisting of 27 girls (43 ureters) and 23 boys (38 ureters) with a mean age of 7.4 ± 4.6 years constituted the study group. Grade III VUR was the most prominent grade (42%, 34 ureters) noted in all age groups. There was no significant difference between boys and girls with regard to the number of each grade of VUR. The success rate of first ESI procedure was 55.6% and was similar in each grade of VUR varying from 50 to 66%. Repeated injections have resulted in an overall success rate of 84%. No significant difference was noted between the age groups with regard to the success rate of ESI. Repeated ESI procedures were found to be unsuccessful in grade V VUR when compared to other grades (p < 0.05).There was a significant difference between primary reflux (76.5%, 62 ureters) and exstrophic (21%, 17 ureters) patients with regard to the grade of VUR and success rate of ESI. Exstrophic patients presented with a higher incidence of grade V VUR (41%) and with a lower success rate of ESI (64.7%). DiSH was the most commonly used agent (47 ureters) followed by CH (22 ureters) and GclBC (12 ureters). The success rates of the ESI procedure by each material were 91.5, 81.8, and 58.3%, respectively. No significant difference was noted between DiSH and CH. Low success rates by GclBCwere attributed to less usage of the material. 82% of the ESI procedures were done by surgeon A (49 ureters) and B (18 ureters) with a success rate of 96 and 72%, respectively. No significant difference was noted between A and B with regard to the success rate and type of injected material and the success rate in each grade of VUR (p > 0.05). No untoward effects were noted in short- and long-term follow-up (mean 4 ± 1.2 years) of any patient. Conclusion: The ESI procedure provides a high success rate for the treatment of VUR which decreases in grade V VUR and presence of exstrophia vesica. Single injection of various materials has been found to be successful in most of the patients with grade II VUR, whereas grade IV and III patients have required repeated injections which have resulted in 100 and 94% success rates, respectively. Grade I VUR can be managed by close follow-up and appropriate antibiotic therapy without any surgical and/or endoscopic intervention. Patients presenting with grade V VUR should undergo open surgery if the first trial of ESI procedure results in failure as repeated injections have proved to be unsuccessful in this grade. The success rate of the ESI procedure does not seem to be affected by the type of injected material and different surgeons performing the procedure after achieving the learning curve.

To evaluate clinical and laboratory findings of these patients and the efficacy of liver transplantation in children with hepatocellular carcinoma (HCC) and hepatoblastoma (HB) associated with tyrosinemia. Among 113 children with liver tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular clinic visits for tyrosinemia, elevated alpha-fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given chemotherapy including cisplatin and adriamycin. In three patients, living-related liver transplantation was performed. They had no treatment after transplantation. All of them are disease free. One patient was treated with chemotherapy and right hepatectomy. She had no suitable donor for living-related liver transplantation. Three months after completing chemotherapy, she had recurrent tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living-related liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for tyrosinemia. In these patients liver transplantation is curative both for liver tumor and tyrosinemia.

Objective: To evaluate the clinical features and outcome of children withrenal cell carcinoma (RCC). Patients and Methods: Eleven patients with RCC who were diagnosed between 1972 and 2004 were retrospectively analyzed. Clinical features, histopathology, treatment regimens and outcomes of the patients were evaluated. Results: The male/female ratio was 3:8, with a median age of 10 years. The stage distribution was as follows: 3 patients in stage I, 1 patient in stage II, 3 patients in stage IIIb, and 4 patients in stage IV. Five of 7 patients with stage II-IV received an actinomycin D-based regimen, one received a cisplatin-based regimen, and the other was given 5-fluorouracil (5-FU). In the last patient, interferon-α was given in combination with 5-FU; 1 of the patients on the actinomycin D regimen received interferon-α as well. All of the stage I patients are alive without disease. Three patients with stage IIIb, stage IV and stage II disease are alive without disease 8, 14 and 26 years after their diagnosis, respectively. The other stage IV and stage IIIb patients died of the disease. Conclusion: Nephroureterectomy is the main treatment modality, and it is sufficient for stage I patients. For patients with stage II-IV RCC, interferon-α and/or actinomycin D-based chemotherapy is the treatment of choice.

OBJECTIVE To investigate lower urinary tract (LUT) functions in a prospective study in boys treated for posterior urethral distraction (PUD), as posterior urethral stricture, erectile dysfunction and incontinence can occur after various treatments for this rare injury in children. PATIENTS AND METHODS Eleven boys were treated for PUD and resultant stricture between 1980 and 2000. Their age, the cause of trauma, extent of injury, previous treatment, and continence status were evaluated, and a urodynamic study conducted. Controls were 12 age-matched males who underwent similar cystometrography (CMG) and uroflowmetry. RESULTS The mean (sd) age at injury was 8 (4) years and the cause was traffic accident in nine and crush injury in two boys. Seven boys had an isolated urethral rupture, two also had a bladder neck injury and two also had a perforated bladder. No gross neurological impairment developed after trauma. Voiding cysto-urethrograms after initial therapy showed vesico-ureteric reflux in five boys, but in only one at the time of the urodynamic studies. At the time of urodynamic study, the mean (sd) age of the patients was 15 (6) years; seven were fully continent, one had intermittent leakage, two were incontinent, and one had nocturnal enuresis. CMG-electromyography (EMG) showed a reduced maximum cystometric capacity in nine patients, reduced compliance in 10, stable detrusor in 11, synergic detrusor-sphincter activity in 11, and residual urine in one. The uroflowmetry-EMG study showed prolonged voiding time and flow time, decreased maximum flow urinary rate (Qmax) and mean flow rate (Qavg). The shape of the flow curve showed an uninterrupted low-amplitude pattern. Comparing these patients with age-matched controls, CMG and uroflowmetry studies showed that the maximum cystometric bladder capacity, compliance, Qmax and Qavg were all significantly lower in patients with PUD, while voiding time and flow time were significantly higher. CONCLUSION The LUT deteriorates after treating PUD in boys. CMG and uroflowmetry findings are consistent with partial anatomical obstruction distal to the bladder. Our results are preliminary and full urodynamics, including pressure-flow studies, will be useful to support this conclusion. A urodynamic study should be integral in the management of PUD.

Background The aim of present study is to investigate the short and long term histopathological alterations caused by submucosal injection of gluteraldehyde cross-linked bovine collagen based on an experimental rat model. Methods Sixty Sprague-Dawley rats were assigned into two groups as group I and II each containing 30 rats. 0.1 ml of saline solution and 0.1 ml of gluteraldehyde cross-linked bovine collagen were injected into the submucosa of bladder of first (control) and second groups, respectively. Both group I and II were further subdivided into 3 other groups as Group IA, IB, IC and Group IIA, IIB, IIC according to the sacrification period. Group IA and IIA, IB and IIB, IC and IIC rats (10 rats for each group) were sacrificed 3, 6, and 12 months after surgical procedure, respectively. Two slides prepared from injection site of the bladder were evaluated completely for each rat by being unaware of the groups and at random by two independent senior pathologists to determine the fibroblast invasion, collagen formation, capillary ingrowth and inflammatory reaction. Additionally, randomized brain sections from each rat were also examined to detect migration of the injection material. The measurements were made using an ocular micrometer at ×10 magnification. The results were assessed using t-tests for paired and independent samples, with p < 0.05 considered to indicate significant differences; all values were presented as the mean (SD). Results Migration to the brain was not detected in any group. Significant histopathological changes in the gluteraldehyde cross-linked bovine collagen injected groups were fibroblast invasion in 93.3%, collagen formation in 73.3%, capillary ingrowth in 46.6%, inflamatory reaction in 20%. Conclusion We emphasize that the usage of gluteraldehyde cross-linked bovine collagen in children appears to be safe for endoscopic treatment of vesicoureteral reflux.

Congenital abnormalities of the anterior urethra other than hypospadias (valve, diverticulum, and megalourethra) are rare conditions and can be difficult to diagnose. Based on five recent cases of these urethral abnormalities, we review the embryology and the English literature, and have found reports of about 100 cases of valve and diverticulum and 50 cases of megalourethra. For all these abnormalities, the clinical presentation depends on age and the diagnosis depends essentially on voiding cystourethrography, which must show the entire urethra, and careful urethroscopic examination.

Les patients porteurs de maladie de Hirschsprung (HD) ont de nombreux facteurs qui augmentent le risque de lithiases urinaires ; ce fait semble sous-estime actuellement. Sur 302 patients atteints de HD, 4 patients (1,32 %) ont ete vu porteurs de lithiases urinaires. Il faut considerer comme facteurs augmentant le risque de lithiases urinaires, l'infection du tractus urinaire, la constipation, le reflux vesico-ureteral, la resection intestinale et la malabsorption susceptibles d'etre rencontres pendant l'evolution de la maladie de HD.

Rupture is one of the rare complications of choledochal cysts. The cause of rupture is unknown in many cases and is considered to be spontaneous. Two patients admitted to the hospital with acute abdominal picture were initially thought to have postoperative abscess and appendicitis. However, the presence of bilelike fluid suggested a bile tract pathology, and evaluation showed rupture choledochal cysts. Although spontaneous rupture of a choledochal cyst is a rare complication, and can sometimes be the initial manifestation of a choledochal cyst, it should be considered in the presence of bilelike fluid and differentiated from spontaneous perforation of the bile tract. In spontaneous rupture of choledochal cyst, cystectomy, cholecystectomy, and Roux-Y hepaticoportoenterostomy is the treatment of choice.

The aim of this study was to evaluate the clinical characteristics and treatment results of 17 children with cavoatrial tumor extension of Wilms tumor. Of the 360 Wilms tumors diagnosed between 1980 and 2000, 17 patients with intracaval thrombus were identified from the medical records at the pediatric oncology department of Hacettepe University. The following data were collected and reviewed: age, sex, presenting symptoms, tumor site, presence of anaplasia, stage, associated congenital anomalies, localization of tumor thrombus, radiologic findings, type and duration of preoperative chemotherapy, response to preoperative chemotherapy, recurrences, and survival. The frequency of cavoatrial extension in this group was 4.7% (15 in the inferior vena cava and 2 in the right atrium). Fourteen patients received preoperative chemotherapy consisting of two-drug regimen (vincristine and actinomycin D) ranging from 1 to 12 weeks (median 4 weeks). Since intravascular invasion is often asymptomatic, a careful radiologic examination to detect tumor thrombus before surgery is essential. There is no need for aggressive surgery in the presence of tumor thrombus. It may be resolved by preoperative chemotherapy. Surgical removal of the thrombus should be considered in the presence of life-threatening tumor thrombosis at diagnosis and in patients who had residual thrombus after chemotherapy.

The purpose of this study was to identify the prognostic significance of pulmonary and liver involvements present at diagnosis in children with Wilms' tumor. From 1971 to 2002, 57 patients with stage IV Wilms' tumor were diagnosed, treated, and followed in the authors' institution. Metastases were detected by chest radiography, abdominal ultrasonography, and/or thoracoabdominal computed tomography. Nephrectomy with tumor resection was performed and appropriate chemotherapy was given to all the patients. The Kaplan-Meier survival method was used to calculate survival. The log-rank test was used to compare the groups with respect to survival. The age range was 1.25 to 15 years (median 4 years). Thirty-seven patients had only lung metastases, 12 had only liver metastases, 6 had both lung and liver metastases, 1 had adrenal gland metastases, and 1 had other site metastases. The overall survival rate in the whole group was 37.1%. The overall survival rates for lung and liver metastases were 50.2% and 16.6%, respectively. The overall survival rates were significantly different between the two groups (P = 0.005). Only one patient survived in the group with liver involvement. Liver involvement at diagnosis indicates a worse prognosis than lung involvement. Wilms' tumor patients with liver metastases should be treated with more intensive regimens than those with lung metastases.

Flow of a fluid through a collapsible tube is under the influence of various factors including the external compressing pressure. The intraabdominal pressure (IAP) should influence the flow through the ureter. Therefore, an experimental study was planned to investigate the effects of ureteral length and external compressing pressure onto the intrapelvic pressure (IPP) in rabbits.Nineteen adult rabbits were used for the experiment. Under general anesthesia, an intraperitoneal and an intrapelvic catheter were placed to measure IAP and IPP. A urethral catheter was placed for bladder decompression. After this standard preparation, a ureteric stent was placed in the ureter in group 1 (n = 7). Distal or proximal ureter transection was performed in group 2 (n = 6) and group 3 (n = 6), respectively. Basal pressure measurements have been recorded. Then the pressures were recorded every 5 minutes, and IAP was increased gradually for 4 cm of water pressure in each subsequent 30-minute period. All analyses were performed for a standard IAP interval (5 to 25 cm H2O).IAP did not differ between groups (P = .08). IPP values were significantly higher than the corresponding IAP values in each group (P = .0001). IPP showed significant difference between IAP values of groups (P = .0001). IPP was significantly increased in group 2 when compared with group 1 and group 3 (P = .0001; P = .0001), but no difference was encountered between groups 1 and 3 (P = .1). There has been a strong relationship between IPP and IAP values in all groups. The Rsq values were 0.912, 0.783, and 0.943 for group 1, group 2, and group 3, respectively (P.0001). Mathematic relations between IPP and IAP also were analyzed. The relations were IPP = 3.9 + 1.10 x IAP, IPP = 10.3 + 1.10 x IAP, and IPP = 3.3 + 1.12 x IAP for groups 1, 2, and 3, respectively.Renal pelvis pressure responds with augmented increases to increments in IAP in urinary tracts with different ureteric lengths. Increase in IPP is more pronounced in longer ureters possibly owing to increased resistance to flow. Prevention of ureteric wall collapse reverses the augmented increase in IPP responses. Therefore, both the length and collapsibility of the ureter play a detrimental role in the generation of augmented IPP responses to increments in IAP. The magnitude of IPP as a response to increments in IAP can be estimated by using mathematical relations between IPP and IAP. Increases in IAP may simulate proximal ureteric obstruction and may take part in the pathogenesis of hydronephrosis.

Objectif: L'obstruction de la jonction pyelo-ureterale provoque des modifications d'adaptation et/ou de compensation de la fonction contractile du bassinet. Ces modifications ayant un effet direct sur le drainage renal, une definition precise est d'une grande importance clinique. L'objectif de cette etude experimentale etait de decrire les alterations de la fonction contractile du bassinet resultant d'une obstruction complete ou partielle de la jonction pyelo-ureterale. Methodes: Quinze lapines adultes New Zealand White etaient etudiees, divisees au hasard en trois groupes de cinq animaux chacun. La meme intervention chirurgicale etait realisee chez les trois groupes pour creer une obstruction pyelo-ureterale partielle (groupe II), une obstruction pyelo-ureterale totale (groupe III) ou aucune obstruction (groupe I, temoin). Les animaux des groupes I et II etaient sacrifies a trois semaines et les animaux de groupe III a deux semaines avec preparation de bandes musculaires prelevees au niveau du bassinet. Un enregistrement isometrique de l'activite mecanique spontanee des bandes ainsi que leurs reponses a la phenylephrine (PE), a la serotonine (5-HT) et au KCl etait realise pour comparaison entre les trois groupes. Resultats: La frequence et l'amplitude des contractions spontanees etaient augmentees de facon significative dans le groupe Il (obstruction partielle) et dans le groupe III (obstruction totale) (p

Whole-bowel irrigation (WBI) has been used for different purposes. It has been the preferred method of bowel cleansing before large bowel surgery and colonoscopy. There are some studies about histopathologic alterations in the gastrointestinal system caused by WBI. It was reported that large quantities of bile-stained intraluminal fluid were seen after WBI with lactated Ringer's solution. In light of all these studies, the authors have decided to inspect whether WBI is choleretic and whether the histopathologic findings in the gastrointestinal system are caused by this choleretic effect.During the experimental procedures, animals at first were divided into 2 major groups of A and B; A consisted of the animals whose bile was not collected, and B consisted of animals whose bile was collected. Later, these 2 groups were divided into 4 subgroups consisting of 6 animals each. Sham operations were performed on the animals in group SH-A and SH-B. The animals in group saline-A and saline-B were given 0.9% of sodium chloride (isotonic sodium chloride). The animals in group LR-A and LR-B received lactated Ringer's solution. The last group (PEG-A and PEG-B) underwent WBI by using polyethylene glycol solution (PEG). After completing WBI, animals were rested for 4 hours, and at the end of resting time, laparotomy was performed to take biopsy specimens from stomach, small bowel, and large bowel. All of the specimens were evaluated and graded for congestion, edema, and inflammation by the individual blinded pathologist.According to the results, WBI has been shown to affect biliary secretion and to have a choleretic effect (P.05, Tuckey-Kramer). The choleretic effect has also been shown to be responsible for histopathologic alterations in some groups (P.05, Tuckey-Kramer). The most severe changes of congestion, edema, and inflammation have been detected in isotonic sodium chloride solutions in all groups. The least alterations have been seen in lactated Ringer's and PEG solutions.In light of these findings, it might be concluded that the use of WBI can cause different degrees of histopathologic changes in gastrointestinal system depending on the type of solutions. WBI has been shown to have choleretic effect, and it was found that there is a connection between histopathologic changes in gastrointestinal system and choleretic effect of WBI.

The treatment and early diagnosis of choledochal cyst are very important to prevent complications of the disease. Delay in treatment can cause lethal complications like biliary cirrhosis and increased morbidity in patients. We studied 26 children with choledochal cyst retrospectively for their clinical presentation, and for the diagnostic tools and treatment modalities, used. The most common findings of choledochal cyst in our series were abdominal pain, vomiting, and ictures in contrast to the classical triad of the disease of jaundice, mass and pain. The most useful diagnostic tool was abdominal ultrasonography. For the treatment, we preferred Roux-en-Y hepaticojejunostomy to the internal drainage procedures, and the results were excellent with minimal mortality and morbidity. We underline prompt diagnosis and treatment to prevent late complications of the disease.

The authors describe a patient with penile agenesis who was reared as a boy and died of chronic renal failure. A literature review was performed to clarify the management and outcome of patients with aphallia who are raised as males.

To determine the causes of perinatal mortality and to calculate perinatal mortality rates at Hacettepe University Hospital.In 1998 the Perinatal Mortality Study Group was established at Hacettepe University. The study group was constituted by the Department of Pediatrics, Units of Pediatric Pathology, Pediatric Cardiology, Pediatric Surgery, Genetics and Neonatology and the Department of Obstetrics and Gynecology, Perinatology Unit. At the end of every month, each case (including autopsy results if available) was discussed among the group, and the cause of mortality was determined according to the Modified Wigglesworth Classification, by the consensus of the group members. Perinatal mortality rates at Hacettepe University were prospectively calculated. Perinatal mortality figures of two periods were compared (1998-1999: Group A and 2000-2001: Group B).Total number of births over 500 grams was 3173 in Group A and 3013 in Group B. Perinatal mortality rate was 33.72/1000 in Group A and 16.92/1000 in Group B. Of the perinatal deaths, 61.46% were intrauterine deaths and 38.54% were early neonatal deaths in Group A. In Group B, 58.83% were intrauterine deaths and 40% were early neonatal deaths. In Group A, 72% of the deaths were1500 grams, and 53.3% were 500-1000 grams. The most common cause of death during this period was prematurity (Modified Wigglesworth Group III) (29.3%), followed by lethal congenital malformations (Group II) (26.6%) and macerated intrauterine deaths (Group I) (22.9%). Autopsy was available in 70.7% of the cases and micronecropsy was available in 12%. Genetic studies were performed in 24% of the cases and termination of pregnancy was carried out for fetal anomalies in 10.7% of the cases. In Group B, 72.6% of the cases were1500 grams and 47.1% of the cases were 500-1000 grams. The most common cause of death during this period was lethal congenital malformations (Group II) (31.4%), followed by macerated intrauterine deaths (Group I) (21.5%) and specific causes (Group V) (21.5%). Autopsy was available in 70.17% of the cases and micronecropsy was obtained in 10.52% of the cases. 20% of the cases underwent genetic studies during pregnancy and termination of pregnancy was carried out in 19.29% of the cases.Perinatal mortality rate has decreased at Hacettepe University over the last two years. The authors believe that this is a result of the multidisciplinary work which has had an impact on perinatal and neonatal care. The most common cause of mortality has changed from prematurity to lethal congenital malformations in this period. Since our institution is a referral center, around 60% of mortality is due to intrauterine deaths and around 30% of the deaths are due to lethal congenital malformations. Unfortunately lethal congenital malformations are referred to our center at an advanced stage of pregnancy.