A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

VATS: first step in the parapneumonic empyema*.

Pneumonia is a common cause of pediatric hospitalization and almost 50% of children hospitalized for pneumonia develops meta pneumonic pleural effusion, most of which resolve spontaneously (1). The meta pneumonic effusion remains a major source of morbidity and mortality in the pediatric population and is a complication on the rise in both the U.S. (2) and Europe (3-6). There is no uniformity of treatment of the meta pneumonic effusion in its early stages and are still questioning some aspects of proper management, remains uncertain and not always shared the operative timing (7). The treatment options are represented, in combination with antibiotic therapy, the thoracentesis (8), the positioning of one or more pleural drainage (9), fibrinolytic therapy (10), the toilet of the pleural cavity by means of video-assisted thoracoscopic surgery (VATS) (11) or "open" with thoracotomy (12) or traditional mini thoracotomy. We report our experience concerning the processing of meta pneumonic effusion, suggesting how the video thoracoscopy may be the treatment of choice in the early stages of the disease.

[Gartner's duct cyst: report of three cases]. / Cisti del dotto di Gartner: presentazione di tre casi.

Gartner's duct cyst is a rare condition consequent to an unfinished disappearance of the mesonephric duct in females. In this report we present three cases of mesonephric duct remnants cysts: two of these were originated from its proximal part ,paraooforon, whereas in the third case the cyst was originated from its paraurethral distal end. These lesions are an uncommon and extremely rare manifestations in paediatric age and more in neonatal age: therefore we believe very interesting and useful to report these cases, describe their clinical data, debate the embryogenic origins, discuss diagnostic and therapeutic problems according to international literature.

The Lortat-Jacob operation by laparoscopic access to treat gastroesophageal reflux in pediatric patients. Preliminary results.

BACKGROUND: Even in the pediatric field, the technique most commonly used in the laparoscopic treatment of GERD is 360 degrees fundoplication (according to Nissen and variants), but this is not devoid of serious complications or sequelae, such as persistent dysphagia or the "gas bloat syndrome." In fact, there has been no lack of proposals of alternative techniques in literature aiming to reduce these negative postoperative events, especially in pediatric patients. At our pediatric surgical clinic at Bari University, the first choice technique is the Lortat-Jacob operation, used in traditional surgery for over 20 years and that has yielded excellent results both as regards control of GER and the complication and sequelae rate. Aim of this study, based on purely preliminary results, was to demonstrate the feasability of the Lortat-Jacob operation by laparoscopic access in pediatric patients, even younger than 1 year old. To our knowledge, there are no other references in literature to the use of this technique by laparoscopic access in pediatric patients. METHODS: Antireflux plasty sec. Lortat-Jacob by laparoscopic access was performed in 10 patients of ages ranging between 10 months and 11 years. The technique adopted took into account all the recognized principles of the traditional surgical approach. The most delicate stage was the extensive mobilization of the distal esophagus at the level of the mediastinum, owing to the risk of bleeding, and of pleural and vagal lesions. Mean operative time was 100' (80'-120'). All the operations were performed laparoscopically, and no intraoperative complications were recorded. The nasogastric probe was removed within 24 h postoperatively, and liquid feeding was recommenced within 36 h. All patients were discharged within 72 h. RESULTS: No complications or short or medium term sequelae were observed. Follow-up is still in the early stages, but the first radiological endoscopic, and pH monitoring controls have shown excellent results. CONCLUSION: The initial data on our recent, limited experience show that the Lortat-Jacob operation can be performed by laparoscopic access in expert hands, provided scrupulous attention is paid to the timing and principles laid down for the traditional surgical approach. Moreover, laparoscopic access allows even greater care to be taken to prevent damage to the vagal nerves during mobilization of the terminal esophagus at the mediastinic level, as they are easier to identify even in children under 1 year old, thanks to the magnification of the image. The good control of GER and absence of complications or short or medium term sequelae justify our choice to use this operation. However, our results are still preliminary and need to be confirmed by an increasing number of patients and longer term follow-up.

MCP-1 and EGF renal expression and urine excretion in human congenital obstructive nephropathy.

BACKGROUND: Obstructive nephropathy is characterized at the histologic level by tubular atrophy and interstitial monocyte infiltration. The molecular mechanisms underlying these histologic changes are still poorly defined. Epidermal growth factor (EGF) produced by tubular cells seems to play a pivotal role in the modulation of tubular cell growth, while monocyte chemotactic peptide-1 (MCP-1) is a powerful and specific chemotactic and activating factor for monocytes. METHODS: Twenty-four patients with congenital ureteropelvic junction obstruction [UPJO; 10 with recurrent urinary tract infection (UTI) and 10 with no UTI] and 15 healthy children were studied. Diagnosis was made by renal ultrasound, intravenous pielography, and MAG3 scan. Urinary samples were collected before and after surgery. In 10 patients, urine was also collected directly from the affected pelvis at the time of surgery. Urinary EGF and MCP-1 levels were measured by enzyme-linked immunosorbent assay. MCP-1 and EGF gene expression were evaluated by in situ hybridization in 15 biopsies from congenital UPJO and in 10 normal kidneys. RESULTS: In normal kidneys, there was a high expression of EGF mRNA, whereas MCP-1 mRNA was undetectable. MCP-1 gene expression was strikingly increased at the tubulointerstitial level in UPJO biopsies compared with controls and was directly correlated with the extent of monocyte infiltration. In addition, UPJO kidney sections showed a marked reduction in EGF gene expression that was directly correlated with the degree of tubular damage. EGF urine concentration was significantly reduced in UPJO when compared with control and directly correlated with its renal gene expression. On the other hand, the MCP-1 urine concentration was strikingly increased in UPJO patients. It is noteworthy that a significant and inverse correlation was observed between the MCP-1 concentration in the urine collected from the obstructed pelvis and the MAG3 clearance of the obstructed kidney (r = -0.76). The presence of recurrent UTI was associated with a significantly higher MCP-1 excretion and a slight reduction in EGF urine concentration. The surgical correction of UPJO was followed by an improvement of renal function together with a significant reduction in MCP-1 excretion and a marked increase in EGF urine concentrations. Interestingly, EGF urine concentration measured before surgery was significantly correlated with the difference between the MAG3 clearance of the obstructed kidney before and after surgery. CONCLUSIONS: MCP-1 and EGF seem to be involved in the pathogenesis of tubulointerstitial damage in congenital obstructive nephropathy, and their urine excretion may represent a powerful prognostic marker in this form of renal disease.

Renal expression of monocyte chemotactic protein-1 and epidermal growth factor in children with obstructive hydronephrosis.

BACKGROUND/PURPOSE: The authors studied the potential role of ureteropelvic junction obstruction (UPJ-O) in causing progressive renal damage in children through the renal expression of epidermal growth factor (EGF) and monocyte chemotactic protein-1 (MCP-1) mRNA. METHODS: Renal tissues were harvested from 11 children with UPJ-O and from 10 normal kidneys to study the renal expression of EGF and MCP-1 detected by means of in situ hybridization. Five of the patients were found to have a history of urinary tract infection (UTI). RESULTS: Children with UPJ-O had marked reduction of EGF gene expression when compared with controls. Interstitial expression of MCP-1 mRNA was present in all UPJ-O cases. Both EGF and MCP-1 expression did not correlate with age, with differential renal function, and with renal thickness measured through MAG3 renal scan. Children with a history of UTI had a more severe reduction of the renal thickness of the affected kidney compared with those without UTI. MCP-1 expression was higher and EGF more reduced in children with a history of UTI. CONCLUSIONS: Our results suggest a potential role of EGF and MCP-1 in the pathogenesis of renal damage and growth failure in UPJ-O, especially in children with UTI. These important functional changes begin early in life, possibly during fetal life.

Urothelium damage as the primary cause of ureteropelvic junction obstruction: a new hypothesis.

Ten infants under 6 months old underwent surgery for obstruction of the ureteropelvic junction. Craniocaudal light microscopy showed subdivision of the resected ureteropelvic junction into three portions: prestenotic, stenotic, and poststenotic. The prestenotic portion was characterized by dilatation of the ureteral lumen, flattening of its mucosal folds and thinning of the urothelium; the stenotic tract showed partial or total loss of the epithelium and fibrosis of the mucosal and fibromuscular coats. No modifications were detected in the poststenotic portion. We advance the hypothesis that a primary epithelial break might cause urine to spread inside the ureteral wall and consequently the mastocytes to migrate and degranulate within the mucosal and fibromuscular coats. The histamine and prostaglandins produced by the mastocytes could induce prolonged muscular spasm, in turn responsible for increasing the intrapelvic pressure and so causing enlargement of the epithelial break. A connective tissue reaction of the ureteral wall would thus occur, which should be considered a secondary event leading to fibrotic stenosis of the ureteropelvic junction.

Effect of aging and acetyl-L-carnitine on the activity of cytochrome oxidase and adenine nucleotide translocase in rat heart mitochondria.

The effect of aging and treatment with acetyl-L-carnitine on the activity of cytochrome oxidase and adenine nucleotide translocase in rat heart mitochondria was studied. It was found that the activity of both these mitochondrial protein systems was reduced (by around 30%) in aged animals. Treatment of aged rats with acetyl-L-carnitine almost completely reversed this effect. Changes in the mitochondrial cardiolipin content appear to be responsible for these effects of acetyl-L-carnitine.

[Ulcerative rectocolitis in the pediatric age group. Radical surgical treatment by the pull-through procedure and direct ileo-anal anastomosis]. / La rettocolite ulcerosa nell'età pediatrica. Terapia chirurgica radicale mediante il procedimento dell'endo-rectal pull through ed anastomosi ileo anale diretta.

Ulcerous rectocolitis (URC) is today seen more frequently than in the past. Extended forms and/or those recalcitrant to medical therapy, make use today of radical surgical treatment with fewer functional and anatomic sequelae. Personal experience of seven cases admitted to the Paediatric Surgery Department of the University of Bari is reported, stress being laid in particular on the results of surgical therapy obtained with total colectomy associated with proctomucosectomy according to the Romualdi-Soave technique. After presenting some general remarks on the disease in paediatric age, stress is laid on the need to resort as quickly as possible to radical surgery, respecting the technical suggestions of Lester-Martin. It is maintained that before carrying out surgical therapy it is always advisable to carry out temporary ileostomy as it offers particular advantages deriving from the greater effectiveness of pharmacological therapy on the defunctionalides colon, the noteworthy recovery in general condition favoured by associated NPT and by recognition of the real extent of the disease which often does not correspond to the endoscopic aspects. Finally, it is maintained that Lester-Martin's technical modification if applied correctly leads to normal functional results (continence and number of alvus emptyings) and definitive, stable cure of the disease, thus rendering inopportune, at least in paediatric age, of the use of the ileal pocket.

[Germ cell tumors in children. Evaluation of regional cases over a 23 year period]. / Tumori a cellule germinali in età pediatrica. Valutazione della casistica regionale osservata nell'arco di 23 anni.

UNLABELLED: A retrospective study on germ cell tumors on children is reported. The purpose of this study was to evaluate some prognostic factors (age, sex, site, histology, treatment) as well as to compare the casistic of Puglia Region with the results of the National literature. 45 patients (30 females, 15 males) aging 2 days-12 years observed in three Pediatric Centres of Bari into 23 years. Primary site of the tumor was: ovary 9, testis 9, sacrococcygeal 16, and others site 11 (head 4, mediastinum 2, pelvis 2, abdomen 2, gluteus 1). HISTOLOGY: teratoma 31, choriocarcinoma 1, embryonal carcinoma 7, endodermal sinus tumor 5, germinoma 1. TREATMENT: surgery 35 patients, surgery+chemotherapy 6, surgery+chemotherapy+radiotherapy 4. Results of treatment: 37 of 41 valuable patients achieved a response (34 complete, 3 partial response); 4 patients had no response. Of the 37 patients that have had a complete or partial remission, 8 subsequently relapsed; of these 8 patients, 5 obtained a second remission after second line therapy, 3 are dead for progressive disease. Better prognosis was observed in children with gonadal tumors treated with surgery alone.

Effect of aging on the activity of the phosphate carrier and on the lipid composition in rat liver mitochondria.

The effect of aging on the activity of the phosphate carrier and on the lipid composition in rat liver mitochondria has been investigated. It was found that the rate of phosphate transport in mitochondria from aged rats (28 months old) is significantly reduced (around 40%) compared to that obtained in mitochondria from young control rats (5 months old). Kinetic analysis of the phosphate transport indicates that only the Vmax of this process is affected, while there is no change in the Km values. The lower activity of the phosphate carrier in mitochondria from aged rats is also documented by swelling experiments. The age-related decrement in the activity of the phosphate carrier was found not to be due neither to a change in the endogenous content of phosphate nor to a change in the transmembrane delta pH value. Inhibitor titrations with mersalyl provide no evidence for a lower content of functional phosphate translocase in mitochondria from aged rats. There is no difference either in the respiratory control ratios or in the ADP/O ratios between mitochondria from young and aged animals. The hepatic mitochondrial lipid composition is altered significantly in aged rats: the total cholesterol increases (31%), the phospholipids decrease (12%), and the cholesterol/phospholipid molar ratio increases (44%). Among the phospholipids cardiolipin shows the greatest alteration (30% decrease with age). Alterations were also found in the pattern of fatty acids. The age-related decrement in the activity of the phosphate carrier appears to be dependent on changes in the lipid domain surrounding the carrier protein molecule in the mitochondrial membrane.

Transport of pyruvate in mitochondria from different tumor cells.

A comparative study of the transport of pyruvate in mitochondria isolated from normal rat liver and from three tumors has been carried out. The Km for net pyruvate uptake in mitochondria isolated from Ehrlich ascites tumor cells is practically equal to that measured in normal rat liver mitochondria while, on the other hand, it is higher in Morris hepatomas 44 and 3924A. The Vmax of pyruvate uptake is depressed in all three types of tumor mitochondria as compared to that in the rat liver mitochondria, with the depression being higher in Morris hepatoma 3924A mitochondria. The lower activity of pyruvate translocator in mitochondria isolated from tumor cells as compared to that in rat liver mitochondria is also shown by depression of the rate of pyruvate-supported oxygen uptake. The results document a decreased activity of the pyruvate translocator in tumor mitochondria which seems to be correlated with the growth rate of the tumor cells.

Pyruvate transport in tumour-cell mitochondria.

Tumour-cell mitochondria contain a pyruvate-transporting system exhibiting the same general properties as those described in rat liver mitochondria. The Km for net pyruvate uptake in tumour-cell mitochondria is practically similar to that measured in rat liver mitochondria but the V is lower. This difference is also shown by swelling experiments. The possible implication of these observations in the context of lactate accumulation in tumour-cell is discussed.

Transport of anionic substrates and glutamate metabolism in mitochondria from ascites tumor cells.

A study is presented of alpha-oxoglutarate and glutamate transport and of glutamate oxidation in ascites tumor cell mitochondria. Kinetics analysis of alpha-oxoglutarate transport in mitochondria from two strains of Ehrlich ascites tumor cells, the hyperdiploid and the hyperdiploid Lettré mutant, shows that the activity of the alpha-oxoglutarate carrier and its affinityfor substrates are higher in the mutant than in the wild strain. Evidence is presented showing the occurrence of carrier mediated glutamate-OH-exchange-diffusion in mitochondria from both strains. The activity of the glutamate carrier is apparently higher in the hyperdiploid Lettré mutant. Glutamate oxidation occurs mainly through transamination to asparatate in both tumor stains. The rate of deamination in the two strains correlates directly with the level of glutamide dehydrogenase (EC 1.4.1.3.), which is higher in the wild than in the mutant strain. Thus glutamate dehydrogenase per se, and not glutamate penetration, constitutes the control step for gluttochondria of glutamate with externally added oxaloacetate (arsenite present) that exclude an obligatory transport of oxaloacetate on the alpha-oxoglutarate carrier.