Re-evaluation of histological findings of nonparasitic splenic cysts.

OBJECTIVE: The pathogenesis of nonparasitic splenic cysts (NPSCs) has not been clarified completely. The aim of this multinational and multicentre retrospective study was to further elucidate the origin of NPSCs. METHODS: From 1980 to 2006, 50 children and adolescents were surgically treated for NPSC at six paediatric surgical centres in four European countries. The initial histology report of 35 NPSCs, 22 epidermoid cysts, 11 pseudocysts or post-traumatic cysts and two mesothelial cysts was available. Additional re-evaluation, including immunohistochemistry, to detect cytokeratin, carcino-embrionic antigen and mesothelioma antibody in the inner surface of the cysts was carried out. Special attention was given to the possibility of preceding trauma to the splenic area and whether it played a role in the genesis of NPSC. RESULTS: The pathological re-evaluation showed 30 epidermoid cysts, four mesothelial cysts and one pseudocyst. Immunohistology revealed eight epidermoid and two mesothelial linings of the cysts in those 11 patients in whom pseudocyst was diagnosed originally. No pseudocyst was documented in those patients who had a history of previous blunt abdominal trauma but was not proved by ultrasound and computed tomography scan. CONCLUSION: In contrast with the prevailing belief, it has been demonstrated that NPSCs are congenital in origin, and there is no clinically proven evidence that trauma does play a role in their genesis.

Claudin-1 and claudin-2 differentiate fetal and embryonal components in human hepatoblastoma.

Claudins (CLDNs), a family of transmembrane proteins, are major constituents of tight junctions (TJs). They have been shown to be differentially regulated in malignant tumors and play a role in carcinogenesis and progression. We aimed to explain the molecular mechanism underlying the main epithelial components of hepatoblastomas (HBs) based on the composition of TJs. Fourteen formalin-fixed, paraffin-embedded surgical resection specimens were analyzed by immunohistochemistry for CLDN-1, -2, -3, -4, -7; proliferating cell nuclear antigen (PCNA); Ki-67; beta-catenin; cytokeratin-7 (CK-7); and hepatocyte-specific antigen; messenger RNA was isolated for real-time reverse transcriptase polymerase chain reaction analysis of the CLDNs from dissected fetal and embryonal cell types. Significantly increased protein and messenger RNA expression of CLDN-1 and -2 was detected in the fetal compared with the embryonal component. Both cell types displayed negative or weak immunostainings for CLDN-3, -4, and -7. Hepatocyte-specific antigen was dominantly expressed in the fetal component. PCNA and Ki-67 labeling indices were significantly higher in embryonal compared with fetal cells. beta-catenin cytoplasmic/nuclear immunoreaction was frequent, although not showing significant differences between fetal and embryonal cells. Mutational analysis of beta-catenin detected mutation in two cases. Our results suggest that increased expression of CLDN-1 and -2 characterizes the more differentiated fetal component in HBs and is a reliable marker for differentiating fetal and embryonal cell types in HBs. The results proved that the embryonal and fetal components of HBs differ in such important feature as the protein composition of TJs. The expression of CLDN-1 and -2 is inversely correlated with cell proliferation. The more aggressive, rapidly proliferating embryonal phenotype is associated with the decrease/loss of CLDN-1 and -2. However, there are no data indicating association with the nuclear translocation of beta-catenin.

Myoelectrical activity of the stomach after surgical correction of esophageal atresia.

AIM: The aim of this study was to investigate the motor activity in the stomach of infants with repaired esophageal atresia (EA). METHOD: Gastric myoelectrical activity was investigated by cutaneous electrogastrography in 15 infants after the surgical correction of EA. Ten infants with no gastrointestinal upset served as controls. Studies were done before and after a milk feed. The pH of the lower esophagus was measured for 24 hours to assess the gastroesophageal reflux (GER) in the infants with repaired EA. RESULTS: After feeding, a significant increase in bradygastria and decrease in tachygastria were observed as compared with the preprandial period. Compared with healthy infants, the electrogram showed pathological patterns in 73.3% (11/15) of EA patients. Twelve of 15 EA patients showed some clinical sign of GER, and 60% of the EA patients proved to be GER-positive on esophageal pH monitoring. There was no difference in the distribution of gastric myoelectrical waves between the GER-positive and GER-negative EA patients either before or after meal. CONCLUSION: Cutaneous electrogastrography is a noninvasive, harmless method for obtaining indirect information about the motor function of the stomach. The abnormal changes in physiological gastric myoelectrical activity in EA patients can serve as markers of disturbed neuromuscular function and can play a role in the pathogenesis of feeding disturbances after operative correction of EA. Gastroesophageal reflux, which often occurs after surgical repair of EA, seems to be connected not only with disordered gastric myoelectric activity, but also probably with other factors such as artificially straightened esophagogastric angle or brachyesophagus.

[Malignant rhabdoid tumour in the liver]. / Malignus rhabdoid tumor a májban.

The authors describe the case of a 6-months-old child with liver tumour. The newborn was healthy until 6 months of age. Prior to hospitalization meteorism and remarkably enlarged liver were observed. A tumour occupying the right lobe of the liver was found with ultrasound and computer tomography, which proved to be inoperable. Intraoperative liver biopsy and few days later the autopsy histology showed a malignant rhabdoid tumour. Authors describe the clinical and morphological features of a rare case of primary hepatic rhabdoid tumour.

[Urinary enzyme excretion in childhood uropathy]. / Obstruktív uropathiás gyermekek enzimürítése.

INTRODUCTION: Renal tissue, and tubules are rich in enzymes but enzyme measurement in the urine as diagnostic parameters in renal diseases have not yet been generally accepted as routine procedures. AIMS: To study urinary enzyme excretion in childhood obstructive uropathy and compares the diagnostic efficiency of them. METHODS: Excretion of the enzymes alkaline phosphatase (ALP), gamma-glutamyltransferase (gamma-GT), N-acetyl-beta-D-glucosaminidase (NAG) and leucine aminopeptidase (LAP) has been investigated in urine of 34 children suffering from obstructive uropathy and in 31 healthy controls by photometric kinetic analysis using synthetic substrates. RESULTS: Urinary excretion of both enzymes significantly increase--2-10 times higher than normal controls--indicates tubular damage. In this study the following relations were found in specificity and in sensitivity: ALP < LAP < gamma-GT < NAG. CONCLUSIONS: Elevated urinary enzyme excretion may be helpful in identifying upper tract obstruction, which if left untreated may cause progressive renal deterioration. It has a role in helping to determine the surgical correction or can be safely followed without fear of parenchymal damage.

[Multicystic dysplastic kidney: natural history of the affected and the contralateral kidney compared to the normal and solitary kidney]. / Multicystás dysplasiás vese: kórlefolyás, az ellenoldali vese növekedésének összehasonlítása a solitaer és a normális vese növekedésével.

INTRODUCTION: Complications of multicystic dysplastic kidney are rare, but hypertension and malignant transformation represent real danger. PATIENTS/METHODS: In a 10-year period, 94 infants (60 boys, 34 girls) with multicystic dysplastic kidney were diagnosed. The data obtained from these patients were compared to 70 children with solitary kidney of which 36 were newborns. In 80 cases (85%) the diagnosis of multicystic dysplastic kidney was already suspected by prenatal sonography. RESULTS: Abnormalities of the contralateral kidney were found in 15 of the 94 patients (16%). Complete involution of the multicystic dysplastic kidney was observed in 19, and a decrease in size in 42%. In 37 children (39%) the dysplastic kidney has been removed at the age of about 1 year because of no involution. The length of the contralateral kidney, compared to the normal standard was already significantly larger at birth. In newborn babies with unilateral renal agenesia the solitary kidney was also significantly longer. CONCLUSIONS: Compensatory hypertrophy of single functioning kidneys occurs in utero both in patients with multicystic dysplastic kidney and in those with unilateral renal agenesia. Based on the results of these and previous studies, early nephrectomy cannot be recommended in the newborn period. Surgery remains an option for patients who have no involution at the time of about 1 year of age.