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Biomedical subjects

C Granata

Publications and source records attributed to C Granata.

At least 19 recordsLinked to original sources

Lichen sclerosus et atrophicus in children with phimosis and hypospadias.

This prospective study was designed to evaluate the incidence of lichen sclerosus et atrophicus (LSA) in a pediatric population with hypospadias and phimosis in order to discuss the indications for circumcision and utilization of preputial skin for urethral surgery. All 115 boys, 55 with congenital phimosis, 45 with acquired phimosis, 13 with hypospadias, and 2 with recurrent chronic balanitis, underwent full-thickness biopsies of the foreskin that were examined by a single pathologist. Of the patients with acquired phimosis, 88% showed inflammatory features in the foreskin; 60% had LSA. Of the patients with congenital phimosis, 82% showed inflammatory disease in the prepuce; 30% had LSA. Of the patients operated upon for hypospadias, 61% showed histologic findings of chronic inflammation of the foreskin and LSA was evident in 15%. The high incidence of LSA in the prepuce of patients with phimosis suggests that circumcision should be performed to correct this disease. The frequent presence of chronic inflammation is a possible cause of stenosis when the foreskin is used to perform a urethroplasty in patients with hypospadias.

Adolescent↗

Outbreak of African tick-bite fever in six Italian tourists returning from South Africa.

In May 1999, a cluster of cases of African tick-bite fever was detected in six Italian tourists who had returned from South Africa. All of the patients had moderate fever and cutaneous eschars. Regional lymphangitis was observed in three of the patients and skin rash in two. By comparing the number of eschars with the number of detectable bite sites it was suggested that at least two-thirds of the biting vectors were capable of transmitting Rickettsia africae. The clinical course of disease was mild in all cases, and all but one of the patients recovered spontaneously before antibiotic treatment was initiated. The diagnosis of African tick-bite fever was confirmed serologically using both microimmunofluorescence and Western blot tests.

Animals↗

Vesicoureteral reflux and ureteropelvic junction obstruction in children with horseshoe kidney: treatment and outcome.

PURPOSE: Horseshoe kidney is the most common renal fusion anomaly. We determined the treatment and outcome of vesicoureteral reflux and ureteropelvic junction obstruction in children with horseshoe kidney. MATERIALS AND METHODS: We reviewed the medical and radiological records of 52 consecutive children, including 32 boys and 20 girls, in whom horseshoe kidney was diagnosed at 2 children's hospitals during 1990 to 1999. Patient age at diagnosis was 1 day to 12 years (mean 3.9 years). In 2 children with horseshoe kidney neuropathic bladder was secondary to spina bifida and they were excluded from study. The diagnosis was made in all cases by abdominal ultrasound and confirmed by excretory urography or (99m)technetium-dimercaptosuccinic acid scan. Voiding cystourethrography was performed in 40 cases (80%). Patients were followed for 2 to 11 years (mean 4.2). RESULTS: Associated urological anomalies were identified in 26 patients (52%) with horseshoe kidney, including primary vesicoureteral reflux in 13, ureteropelvic junction obstruction in 12 and ectopic ureter in 1. Surgical intervention to correct the anomalies in 15 of the 26 children (58%) involved pyeloplasty in 8, ureteral reimplantation in 2, endoscopic treatment for vesicoureteral reflux in 2, ureterolithotomy in 1, upper pole heminephrectomy in 1 and valve fulguration in 1. No significant complications were observed in surgically treated patients. CONCLUSIONS: More than half of the patients with a clinically symptomatic horseshoe kidney have vesicoureteral reflux or ureteropelvic junction obstruction. Many patients with horseshoe kidney require surgical intervention for associated urological anomalies with good results.

Child↗

Medium-term results after cholecystectomy in patients younger than 10 years.

BACKGROUND: Gallbladder stones are a well-known and widely studied problem in children. Hematological disorders are the most common diseases that can cause cholelithiasis. However, in the last few years, the proportion of children with idiopathic cholelithiasis has increased 50%. Herein, we present a prospective study on laparoscopic cholecystectomy in a selected group of patients aged < 10 years. METHODS: Fifty-eight patients aged < 10 years underwent laparoscopic cholecystectomy for stones in the period 1992-99. The female/male ratio was 1.5, the mean age was 8 years (range, 2-10), and the mean weight was 30 kg. In all patients, parenteral nutrition, fasting state, and prolonged use of antibiotics had been suspended for > or = 6 months, and conservative treatment had been tried for > or = 12 months in the absence of symptoms of stone migration. All the patients were followed up after surgery: clinically at 1,6,12, and 36 months and by ultrasound at 1, 12, and 36 months. Liver function and hematological tests were performed in case of symptoms or if hemolytic disorders were the cause of stones. RESULTS: The mean operative time was 63 min (range, 30-120) in children undergoing cholecystectomy alone and 150 min in children undergoing associated splenectomy. There were no major complications or reoperations. Minor complications included bleeding from accessory cystic artery (n = 3) and insufflation of the omentum (n = 2). One case was converted to an open procedure due to technical problems. All the children were followed up and no complications were observed. CONCLUSION: We consider the laparoscopic approach the gold standard for cholecystectomy in children. This procedure does not have a complication rate any higher than open cholecystectomy, and patient follow-up is as good as that of open surgery. Previous abdominal surgery is not a contraindication to laparoscopy.

Child↗

Conformationally restricted PACAP27 analogues incorporating type II/II' IBTM beta-turn mimetics. Synthesis, NMR structure determination, and binding affinity.

To probe the importance of a proposed beta-turn within residues S9-R12 of PACAP for recognition by VIP/PACAP receptors, compounds 1 and 2, two conformationally restricted analogues of PACAP27 incorporating respectively (S)- or (R)-IBTM as type II or II' beta-turn dipeptide mimetic at the Y10-S11 position, were synthesized. According to 1H NMR conformational analyses in aqueous solution and 30% TFE, both PACAP27 and the [S-IBTM(10,11)]PACAP27 analogue 1 adopt similar ordered structures. PACAP27 shows an N-terminal disordered region (residues H1-F6) and an alpha-helical conformation within segment T7-L27. For residues S9-R12, our data seem more compatible with a segment of the alpha-helix than with the beta-turn previously proposed for this fragment. In compound 1 the alpha-helix, also spanning T7-L27 residues, appears slightly distorted at the N-terminus relative to the native peptide. Although this distortion could lead to the marked decrease in binding affinity of this compound at the VIP/PACAP receptors, the lack of the Y10 side chain in analogues 1 and 2 could also significantly affect the binding of these compounds.

Amino Acid Sequence↗

Pleuropulmonary blastoma.

We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass was achieved by a bilobectomy. Microscopic examination of the histologic sections showed the typical findings of blastemal and mesenchymal areas with focal zones of rhabdomyoblastic and liposarcomatous differentiation. Monthly cycles of chemotherapy consisting of ifosfamide, vincristine and actinomycin D were given for 10 months after surgical resection. Our patient is presently alive and disease-free two years after diagnosis. As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.

Antineoplastic Agents↗

N-terminal fatty acid substitution increases the leishmanicidal activity of CA(1-7)M(2-9), a cecropin-melittin hybrid peptide.

In order to improve the leishmanicidal activity of the synthetic cecropin A-melittin hybrid peptide CA(1-7)M(2-9) (KWKLFKKIGAVLKVL-NH(2)), a systematic study of its acylation with saturated linear fatty acids was carried out. Acylation of the N(epsilon)-7 lysine residue led to a drastic decrease in leishmanicidal activity, whereas acylation at lysine 1, in either the alpha or the epsilon NH(2) group, increased up to 3 times the activity of the peptide against promastigotes and increased up to 15 times the activity of the peptide against amastigotes. Leishmanicidal activity increased with the length of the fatty acid chain, reaching a maximum for the lauroyl analogue (12 carbons). According to the fast kinetics, dissipation of membrane potential, and parasite membrane permeability to the nucleic acid binding probe SYTOX green, the lethal mechanism was directly related to plasma membrane permeabilization.

Acylation↗

Importance of local treatment in pediatric soft tissue sarcomas with microscopic residual after primary surgery: results of the Italian Cooperative Study RMS-88.

BACKGROUND: The goal of primary excision in soft tissue sarcomas is the complete removal of the tumor by a nonmutilating procedure. However, microscopic residuals may be left after a conservative procedure because of inadequate preoperative assessment or difficulties during the operation. The purpose of this report is to describe the treatment and the outcome in patients, enrolled in the Italian Cooperative Study RMS-88, with microscopic residuals after primary excision (IRS Group IIa). PROCEDURE: Microscopic residuals were evident at histology in 52 of 90 patients who had a macroscopic complete primary excision: 25 rhabdomyosarcomas (RMS) and 27 nonrhabdo-soft tissue sarcomas (NRSTS). Eighteen patients were treated with primary reexcision (PRE) and chemotherapy (CT) using VA or IVA regimens; 27 patients received radiation therapy (RT; 40 Gy) and IVA; 7 children in whom PRE was not feasible and RT could not be administered for age <3 years were treated with CT (IVA) alone. RESULTS: Of the 18 patients who underwent a successful PRE + CT, the local relapses were 3 (16.6%); of 27 cases who had RT + CT there were 4 local relapses (14.8%); 3 local relapses occurred in those 7 patients in whom CT alone was administered (43%). CONCLUSIONS: Microscopic residuals after primary surgery were difficult to manage because of the absence of a measurable target. PRE represented the treatment of choice for children <3 years of age who cannot receive RT and for paratesticular sites. PRE and RT showed similar results in achieving local control in extremity and trunk sites, but they could not always avoid local recurrence. In particular PRE was not effective in tumors larger than 5 cm. If microscopic residuals could not be avoided and PRE was not possible, adequate RT was effective both for RMS and for NRSTS.

Adolescent↗

Features and outcome of neuroblastoma detected before birth.

BACKGROUND/PURPOSE: The growing use of routine ultrasonography during pregnancy is leading to an increasing number of prenatally diagnosed neuroblastomas. Optimal strategy has not yet been defined for these patients, because knowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NB (ADNB) reported in the Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NB. METHODS: The Italian Neuroblastoma Registry was reviewed for the period January 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NB stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or magnetic resonance imaging) of the primary tumor and bone marrow biopsy before surgical resection. RESULTS: Seventeen patients were identified. Primary tumour site was adrenal glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution was stage I, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery was 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shimada classification. N-myc gene amplification was studied in 14 patients. N-myc amplification was detected only in a newborn with stage II-A NB, who died of massive bleeding 2 days after tumor resection. DNA index and 1p deletion were studied in 11 and 8 patients, respectively. Both diploidy and deletion of 1p were observed in a newborn who subsequently died of disease progression despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently are alive and free of disease, and one with IV-S NB and short follow-up is alive with disease. CONCLUSIONS: Our data give evidence that in most cases infants with ADNB represent a subset of patients with excellent outcome. Aggressive treatment may not always be necessary. Infants with ADNB with unfavorable features should undergo early surgical excision, whereas patients with favourable features could be observed awaiting spontaneous regression of the mass, reserving delayed surgery for tumors that increase in size or do not regress.

Adrenal Gland Neoplasms↗

[Intractable diarrhea and neuroblastoma: report of a clinical case].

One of the typical presentations of neuroblastoma is intractable diarrhea or wdha (watery diarrhea, hypokalemia, achloridria). The case admitted to our Pediatric Surgery Department presented watery diarrhea due to VIP hyperincretion caused by a stage 1 neuroblastoma, whose ablation allowed a complete resolution of the clinical conditions. This case report can be useful in the discussion of the differential diagnosis of the most common clinical pictures.

Adrenal Gland Neoplasms↗

Apoptosis and bcl-2 oncogene expression in Wilms' tumor.

Wilms' tumor (WT) usually has a good outcome, although a poor prognosis is often related to more advanced stages and anaplastic features. Apoptosis occurs with variable frequency in malignant tumors, and may have a role in reducing their growth rate. The bcl-2 proto-oncogene inhibits apoptosis, and the consequent increase in the number of cells may play a role in the development of tumors. The aim of this study was to analyze the role of apoptosis and bcl-2 expression in WT. Twenty-six resected WT specimens were studied; 12 patients had stage I tumor, 4 stage II, 5 stage III, 3 stage IV, and 2 stage V. Twenty-three tumors were classified as favorable histology (FH) and 3 as unfavorable (UH). The mean follow-up was 34 months; 22 patients were alive and 4 were dead (2 with FH: 1 stage III and 1 stage IV, and 2 with UH stages 4). Apoptosis was detected by the in-situ end-labelling technique; bcl-2 expression was detected by immunohistochemistry. An apoptotic index (AI) was calculated as the ratio of apoptotic to normal cells in each specimen. The AI was lower in higher tumor stages, with a significant difference between stages I and IV (P < 0.05). In cases with UH, Al was lower than in tumors with FH (P < 0.01). The AI was also lower in patients who died than in those who survived (P < 0. 01). In all specimens no correlation between AI and bcl-2 expression was observed. Progression to advanced stages of WT and a poor prognosis f anaplastic tumors may be linked with disruption of the mechanisms that control apoptosis. Bcl-2 does not play a role as a regulator of apoptosis in WT, other oncogenes and tumor-suppression genes may be more involved in inhibiting apoptosis in WT.

Apoptosis↗

Pancreatitis caused by duodenal duplication.

The authors present the investigations and surgical treatment of two cases of duodenal cystic duplication. Abdominal pain and gastroesophageal reflux were the most important symptoms and signs associated with an history of recurrent acute pancreatitis. Computed tomography scan, ultrasound examination, and cholangiography confirmed preoperatively the diagnosis, and a transduodenal surgical approach was carried out in both children. A simple marsupialization of the cyst was performed in the former, and a sphincterotomy with papillosphincteroplasty was associated in the latter. The diagnosis was confirmed by microscopy, and both the children are asymptomatic after a 14 and 18 months of follow-up. This report focuses on the importance of the cholangiopancreatography for every child presenting with recurrent, unexplained bouts of acute pancreatitis, and underlines the technical surgical aspects on the basis of the anatomic identification of the malformation.

Acute Disease↗

Treatment of vesico-ureteric reflux in children with neuropathic bladder: a comparison of surgical and endoscopic correction.

BACKGROUND/PURPOSE: Vesico-ureteric reflux (VUR) is a common problem in children with neuropathic bladder. Lesser-degree VUR may be manageable by intermittent catheterization or by anticholinergics, but higher grades usually require surgical treatment. If left untreated, two thirds of such patients may experience deterioration of the upper renal tracts. The aim of this study was to compare the results of the STING (Subureteric Teflon Injection) technique with surgical ureteric reimplantation as treatment for VUR in neuropathic bladder. METHODS: From January 1981 to December 1996, 58 children with NB (81 ureters) were treated for VUR. STING and Cohen ureteroneocystotomy were performed in 40 and 41 ureters, respectively. Mean age was 4.5 years (STING) and 5.1 years (Cohen). RESULTS: Twenty-nine of 40 refluxing ureters (72.5%) were cured by STING, whereas Cohen eradicated reflux in 39 of 41 ureters (95.5%). No complications were observed in either group. All the ureters in which STING failed were treated successfully by Cohen ureteroneocystostomy. The 2 ureters still refluxing after surgical reimplantation were cured successfully by a single STING. The mean follow-up was 6.8 years in the Cohen group and 4.8 years in the STING group. During follow-up, no recurrence was observed in patients cured by open reimplantation. In the STING group, 2 previously cured ureters showed recurrence of VUR: both were treated successfully by a further STING. CONCLUSIONS: Open ureteral reimplantation is more effective than STING in correcting VUR in children with neuropathic bladder dysfunction. Nevertheless, the good success rate, the relative technical simplicity, outpatient nature, and rapid recovery point to STING as a safe and effective procedure for the initial treatment of VUR. Failure of STING does not preclude a successful open operation.

Adolescent↗

Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation.

A type I congenital cystic adenomatoid malformation (CCAM) in the left lower lobe was removed from a 11-year-old boy with a 3-month history of recurrent pneumonia. As incidental finding, a bronchioloalveolar carcinoma (BAC) was found in the lung parenchyma adjacent to the cyst. A left lower lobectomy was performed. At 18 months after surgery the patient is well and free of neoplastic disease. To the best of our knowledge, this association has not been reported previously in a pediatric patient. Malignancies complicating CCAM are rarely seen, but have been reported in adults. Including our case, eight cases of BAC and five cases of rhabdomyosarcoma (RMS) in association with CCAM have been reported so far. As CCAM can host metaplastic mucous cells, primitive mesenchymal cells and differentiated but poorly organized striated muscle fibers, it has been proposed that CCAM may act as a predisposing condition for oncogenesis. Our experience adds further support that CCAM can act as a premalignant lesion. Previous reports of both BAC and RMS in asymptomatic CCAM suggest prompt resection shortly after diagnosis.

Adenocarcinoma, Bronchiolo-Alveolar↗

Lung resection in pediatric patients.

An evaluation of all pediatric patients with primary or secondary pulmonary disease operated upon from January 1993 to July 1996 by the same senior surgeon was carried out. The inclusion criterion was a lung resection in patients aged less than 14 years. Children were divided into two categories according to the neoplastic or non-neoplastic nature of their disease. In the first group a lobectomy was performed for primary lesions and wedge resection for secondary ones. In the second group lobar emphysema and cystic dysplasia were the major indications for lobectomy, while diagnostic wedge resections were performed for interstitial/infiltrative lesions. Several groups of techniques were identified according to the type of approach and the suture method. Video-assisted thoracoscopic surgery and a muscle-sparing approach were compared to classic posterolateral thoracotomy. The mechanical stapler-suturing method was compared to the manual suturing. Our results demonstrate the importance of mechanical suturing, particularly in decreasing anesthesia time and reducing the risk of dehiscence. The minimally invasive approach associated with mini-thoracotomy was particularly useful for patients with reduced oxygen saturation due to ventilatory and gas-exchange problems. The roles of staplers in lung parenchymal resection and minimally invasive procedures for improving the postoperative thoracic compliance of pediatric patients are stressed.

Adolescent↗

Multicenter survey of endoscopic treatment of vesicoureteral reflux using polytetrafluoroethylene.

PURPOSE: Endoscopic subureteral polytetrafluoroethylene (Teflon) injection has been used successfully to treat primary and secondary vesicoureteral reflux in children. We review the results of subureteral polytetrafluoroethylene injection in terms of its effectiveness, long-term followup and morbidity. MATERIALS AND METHODS: A total of 53 pediatric urologists and/or pediatric surgeons at 41 centers worldwide answered an inquiry regarding experience with subureteral polytetrafluoroethylene injection in vesicoureteral reflux. Data were collected from a completed standard questionnaire. From 1984 to 1996, 1,921 boys and 6,411 girls with a mean age of 4.5 years (12,251 refluxing ureters) were treated endoscopically with subureteral polytetrafluoroethylene injection. Reflux was grades I to V in 407 (3.3%), 3,832 (31.2%), 5,213 (42.5%), 2,218 (18.1%) and 581 (4.7%) ureters, respectively. In the majority of patients subureteral polytetrafluoroethylene injection was performed on an outpatient basis. Most urologists monitored patients with voiding cystourethrography and renal ultrasound at 3 months, 1 year and 3 years. All patients were followed 1 to 13 years. RESULTS: Reflux resolved in 9,226 ureters (75.3%) after 1 polytetrafluoroethylene injection, in 1,478 (12%) after 2 and in 250 (2%) after 3 or 4. Vesicoureteral reflux improved to grade I after 1 or 2 injections in 743 (6%) ureters, which needed no further treatment. Subureteral injection failed to correct reflux in 554 ureters (4.5%), which were then treated with ureteral reimplantation. Vesicoureteral junction obstruction requiring ureteral reimplantation developed in 41 ureters (0.33%). More than 90% of patients have been followed for more than 2 years. At followup reflux recurred in 326 (2.8%) endoscopically corrected refluxing ureters. No clinically untoward effects were reported in any patient due to the use of polytetrafluoroethylene as an injectable material. CONCLUSIONS: The results of this multicenter survey confirm that endoscopic subureteral polytetrafluoroethylene injection is a simple, safe and effective outpatient procedure for treating all grades of vesicoureteral reflux.

Adolescent↗