Uretero-pelvic junction obstruction in children: Is vascular hitch an effective and safe solutions in very long term outcome? Report of 25 years follow-up.

Vascular (VH) according to Hellstrom-Chapman technique is considered a safe and effective alternative approach to pure extrinsic Ureteropelvic Junction Obstruction (UPJO) with good results in short and medium term, but few data are available on long and verylong term outcomes. Our aim is to evaluate VH long and very-long term outcomes in patients treated in pediatric age focusing on relapse, development of hypertension and/or inferior polar kidney hypotrophy during puberty and adulthood. From 1990 to 2015 in our Department 76 children were treated by open or laparoscopic VH for pure extrinsic-UPJO. We were able to contact 54 of 76. 41 patients (25 males, 16 females) accepted to be studied. Mean follow- up time was 12.7 years (range 6-27 years); mean age at the assessment was 22.2 years. We excluded patients who were younger than 13 (if females) or 14 (if males) at the assessment (upper limits of physiological puberty onset). Patients were followed with US, MAG-3-scan and arterial blood pressure measurement. Collected data were compared with the preoperative ones by Student t-test. 95% of US images and MAG-3-scan reports were compatible with complete resolution of obstruction with good renal functionality. 87% of patients were completely healthy. We recorded 3 cases of hypertension (7%) not secondary to renovascular origin; 2 cases with recurrent flank pain (5%) with slightly dilated pelvis at the US and sub-obstructive pattern at MAG-3-scan with preserved renal function. Our experience confirms that VH, (open/laparoscopic) is a safe and effective procedure with good outcomes at very longterm follow-up. No patients at puberty and in adulthood required reoperation or presented polar hypotrophy and related vascular hypertension. VH is an alternative approach to pure extrinsic-UPJO. There were few data about long and very-long term outcomes in patients after this kind of surgery. We followed-up 41 patients confirming that VH (open/laparoscopic) is safe and effective with good long-term outcomes.

Testicular torsion during the COVID-19 pandemic: Results of a multicenter study in northern Italy.

INTRODUCTION: The literature reported an increased avoidance of the Emergency Department (ED) during COrona VIrus Disease 19 (COVID-19) pandemic, causing a subsequent increase of morbidity and mortality for acute conditions. Testicular torsion is a surgical emergency, which can lead to the loss of the affected testicle if a delayed treatment occurs. As testicular loss is time-related, outcome was hypothesized to be negatively affected by the pandemic. OBJECTIVE: The aim is to investigate whether presentation, treatment and outcomes of children with testicular torsion were delayed during COVID-19. STUDY DESIGN: Medical records of pediatric patients operated for testicular torsion of six Paediatric Surgical Units in Northern Italy between January 2019 and December 2020 were retrospectively reviewed. Patients were divided as for ones treated during (dC) or before the pandemic (pC). To reflect possible seasonality, related to lockdown restrictions, winter and summer calendar blocks were also analysed. For all cohorts, demographic data, pre-operative evaluation, operative notes and post-operative outcomes were reviewed. Primary outcomes were referral time, time from diagnosis to surgery and ischemic time, while secondary outcomes were orchiectomy and atrophy rates. Statistic was conducted as appropriate. RESULTS: A total of 188 patients with acute testicular torsion were included in the study period, 89 in the pre-COVID-19 (pC) period and 99 during COVID-19 (dC). Time from symptom onset to the access to the Emergency Department (T1) was not different among the two populations (pC: 5,5 h, dC: 6 h, p 0.374), and similarly time from diagnosis to surgery (pC: 2,5 h, dC: 2,5 h, p 0.970) and ischemic time (pC: 8,2 h, dC: 10 h, p 0.655). T1 was <6 h in 46/99 patients (46%) pC and 45/89 patients (51%) dC (p = 0.88, Fisher's exact test). Subgroup analysis accounting for different lockdown measures, confirm the absence of any difference. Orchiectomies rate was 23% (23/99) dC and 21% (19/89) pC (p = 0.861, Fisher's exact test) and rate of post-operative atrophy was 9% dC (7/76) and 14% pC (10/70), p = 0,44, Fisher's exact test. DISCUSSION: Despite worldwide pediatric ED accesses reduction, we reported that neither ischemic time nor the long-term outcomes in children with testicular torsion increased during the COVID-19 pandemic. In the available literature, few studies investigated the topic and are controversial on the results. Similarly to our findings, some studies found that timing and orchiectomy rates were not significantly different during the pandemic, while others reported a correlation to pandemic seasonality. Furthermore, in the recent pediatric literature it has been reported a delayed testicular torsion diagnosis due to shame in informing parents. Strengths of this study are the large numerosity, its multicentric design and a long study period. Its main limitation is being retrospective. CONCLUSIONS: We reported our large cohort from one of the most heavily COVID-19-affected regions, finding that referral, intra-hospital protocols and ischemic time in testicular torsion were not increased during to the pandemic, as well as orchiectomy rate and atrophy.

Epithelial thymic tumours in paediatric age: a report from the TREP project.

BACKGROUND: Thymic epithelial tumours (thymoma and carcinoma) are exceptionally rare in children. We describe a national multicentre series with a view to illustrating their clinical behaviour and the results of treatment. METHODS: From January 2000 all patients under 18 years of age diagnosed with "rare paediatric tumours" were centrally registered by the Italian centres participating in the TREP project (Tumori Rari in Età Pediatrica [Rare Tumours in Paediatric Age]). The clinical data of children with a thymic epithelial tumour registered as at December 2009 were analyzed for the purposes of the present study. RESULTS: Our series comprised 4 patients with thymoma and 5 with carcinoma (4 males, 5 females; median age 12.4 years). The tumour masses were mainly large, exceeding 5 cm in largest diameter. Based on the Masaoka staging system, 3 patients were stage I, 1 was stage III, 1 was stage IVa and 4 were stage IVb.All 3 patients with stage I thymoma underwent complete tumour resection at diagnosis and were alive 22, 35 and 93 months after surgery. One patient with a thymoma metastasizing to the kidneys died rapidly due to respiratory failure.Thymic carcinomas were much more aggressive, infiltrating nearby organs (in 4 cases) and regional nodes (in 5), and spreading to the bone (in 3) and liver (in 1). All patients received multidrug chemotherapy (platinum derivatives + etoposide or other drugs) with evidence of tumour reduction in 3 cases. Two patients underwent partial tumour resection (after chemo-radiotherapy in one case) and 4 patients were given radiotherapy (45-54 Gy). All patients died of their disease. CONCLUSIONS: Children with thymomas completely resected at diagnosis have an excellent prognosis while thymic carcinomas behave aggressively and carry a poor prognosis despite multimodal treatment.

Intraoperative examination (IOE) in pediatric extracranial tumors.

BACKGROUND AND OBJECTIVES: Intraoperative evaluation of surgical specimens by frozen sections (IOE) is required to distinguish benign and malignant lesions, assess surgical margins, and determine sample adequacy of biopsies. In the last years, it has been used also for therapeutic decisions, particularly in children, who may need other ancillary procedures, in case of malignancies. Our purpose was the evaluation of diagnostic accuracy, limits, and different role of IOE in pediatric pathology. PATIENTS AND METHODS: From 1990 to 2001, 416 IOEs were performed in 341 children, affected by lymph node pathology, soft tissue tumors, neuroblastic tumors, gonadal germ cell, and stromal tumors, hepatic lesions, renal tumors, and others; the technique was also used to assess surgical margins during major surgeries. IOEs were obtained from Tru-cut biopsies (<1 cm(3)), wide biopsies (>1 cm(3)), or from the whole lesions, and the subsequent final diagnoses were classified as conordant, discordant, and deferred. RESULTS: Three hundred seventy cases (88.9%) were concordant, 10 (2.4%) discordant, and 36 (8.6%) deferred. The disagreement was found in two small lymph node samples, three soft tissue tumors, one hepatoblastoma, one metastasis, and three surgical margins. The deferred diagnoses were related to lymph node and soft tissue lesions. CONCLUSIONS: IOE in pediatric oncology may integrate the diagnostic process and supports the therapeutic guidelines of different tumors. In our study, the diagnostic concordance was satisfactory. A rational use of the technique and the awareness of its limits are, however, prerequisites to avoid the risk of overtreatment.

Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee.

BACKGROUND: To evaluate the effect of radiotherapy (RT) in association with complete second look operation, histologically confirmed, on outcome of patients with IRS Gr.III non-alveolar RMS. PROCEDURE: We analyzed data from 39 patients (age: 0.5-194 months, median 52) who were enrolled between 1988 and 2005 in 2 consecutive Italian Studies, RMS 88 and RMS 96. All achieved a complete resection of the residual tumor after neoadjuvant chemotherapy; 27 did not receive any other local treatment: pelvic 8, extremities 6, head-neck-non-parameningeal 5, orbit 1, genito-urinary-bladder-prostate 3, trunk 2, abdomen 1, vagina 1; 12 were given RT (32-45 Gy), 5 before and 7 after the operation: genito-urinary-bladder-prostate 3, pelvic 3, abdominal 1, extremities 1, head-neck-parameningeal 1, head-neck-non-parameningeal 1, vagina 1, orbit 1. All received postoperative chemotherapy. RESULTS: Median follow-up was 81 months (range 17-219 months). With RT: 10/12 patients are in first complete remission; 2/12 had a metastatic relapse (1 also local relapse), and both of them died of disease. Without RT: 16/27 maintained the first complete remission, however 1/16 died due to a second tumor; 8 suffered from local relapse (4 pelvic, 1 orbit, 1 vagina, 1 head-neck-non-parameningeal, 1 abdomen) and 3 of them died, 3 showed a metastatic recurrence (2 extremities, 1 pelvic) and 1 died. CONCLUSIONS: Local relapses were more frequent for patients without RT, especially in pelvic sites. The two relapses after RT occurred in huge bladder-prostate RMS. Although the limited number of patients does not allow statistically significant conclusions, our experience suggests that RT may have a positive influence on local control for completely resected non-alveolar RMS.

Monitoreo de anticuerpos HLA en insuficientes renales crónicos en lista deespera uruguaya para trasplante renal 2005 / Monitoring of HLA antobodies in chronic renal failure (CRI) patients on the waiting list for renal transplant in Uruguay

Introducción: en el trasplante renal, la investigación en los pacientes en lista de espera de anticuerpos (Ac) contra Human Leucocyte Antigen (HLA) resulta necesaria para adjudicar el órgano y para adecuar el tratamiento inmunomodulador que promueva mayor sobrevida al trasplante. Material y método: realizamos búsqueda de anticuerpos HLA en 488 pacientes en lista de espera nacional de trasplante renal (2005). Definimos como inmunizados a aquellos con reactividad > 20% (41 pacientes), y altamente inmunizados con > 80% (6 pacientes), por técnica de microlinfocitotoxicidad enfrentados a un panel linfocitario, detectando especificidad por ELISA y citometría de flujo. Resultados y conclusiones: detectamos anticuerpos HLA clase I y II por ELISA, e identificamos especificidades HLA por citometría de flujo: 41 (8,4%) pacientes inmunizados presentaronanticuerpos HLA clase I y 22 (4,5%) asocian clase II. Las especificidades de estos anticuerpos más frecuentemente encontradas fueron: A24, A23, BW6, B44, CW6, CW2, DR8, DR7, DQ2,DQ7. Comparamos distribución por edad, sexo, retrasplante, grupo sanguíneo, transfusiones previas, tiempo en lista de espera y diagnóstico en población inmunizada respecto a noinmunizada. Constatamos que en el grupo de inmunizados predominan los candidatos a un segundo trasplante (X2=130,47), quienes han recibido transfusiones previas (X2=119,2) yaquellos con mayor tiempo en lista de espera (p<0,0001). No hay diferencias en la distribución por edad, sexo ni diagnóstico etiológico de insuficiencia renal crónica, predominando la nefropatía indeterminada y glomerulopatías. La distribución por grupo sanguíneo mostró diferencias significativas: en el grupo de inmunizados hay más pacientesdel grupo 0 (X2 =7,9) y menos del grupo A (X2 =3,94).

Introduction: tests of Human Leucocyte Antigen (HLA), antibodies for patients on the waiting list for renal transplant,are necessary to allocate organs and to define theappropriate immunomodulator treatment that result in the best tranplant survival rates.Methods: we looked for HLA antobodies in 488 patients on the national waiting list for renal transplant. We defined patients with reactivity > 20% (41 pacients) as immunized, and those with reactivity > 80% (6 patients) ashighly immunized, by using the microlymphocitotoxicity technique against a lymphicyte test panel, detecting specificity by ELISA and flow cytometry.Results and conclusions: we found HLA antibodies class I and class II by ELISA ,and identified HLA specificitiesby flow cytometry: 41 (8.4%) of immunized patients showed HLA antibodies class I and 22 (4.5%) evidenced class II. The most frequently found specificities for these antibodies were : A24, A23, BW6, B44, CW6, CW2, DR8, DR7, DQ2, DQ7.We compared the distribution by age, sex,retransplantation, blood type, previous blood transfusions, time on the waiting list and diagnosis of the immunizedpopulation with those non-immunized. We found that in the immunized group most of them are candidates for asecond transplant (X2=130,47), have received previous transfusions (X2=119,2) and have been on the waiting list longer(p<0,0001). No differences were found in the distributionby age, sex or etiological diagnosis of chronic renal failure (CRF), being the non-determined nephropatyand glomerulopathies, the most frequent types. Distribution by blood type showed significant differences: in theimmunized group there were more patients belonging to group 0 (X2 =7,9) and less of them belonging to group A(X2 =3,94).

Introdução: no transplante renal, a pesquisa de anticorpos (Ac) contra Human Leucocyte Antigen (HLA) em pacientes em lista de espera é necessária para alocar o órgão epara a adequar o tratamento imunomodulador visando umasobrevivência mais longa do transplante.Material e método: realizamos pesquisa de anticorpos HLA em 488 pacientes em lista de espera nacional detransplante renal (2005). Definimos como imunizados os pacientes com reatividade > 20% (41 pacientes), e altamente imunizados com > 80% (6 pacientes),empregando a técnica de microlinfocitotoxicidade contra painel delinfocitos, detectando especificidade por ELISA e citometria de fluxo.Resultados e conclusões: detectamos anticorpos HLA classe I e II por ELISA, e identificamos especificidade HLApor citometria de fluxo: 41 (8,4%) pacientes imunizados tinham anticorpos HLA classe I e 22 (4,5%) classe II. Asespecificidades desses anticorpos más freqüentes foram: A24, A23, BW6, B44, CW6, CW2, DR8, DR7, DQ2, DQ7.Comparamos a distribuição por idade, sexo, retransplante, grupo sanguíneo, transfusões anteriores, tempo na listade espera e diagnóstico na população imunizada com relação a não imunizada. Constatamos que no grupo deimunizados predominam os candidatos a um segundo transplante (X2=130,47), os que receberam transfusões previas (X2=119,2) e os que estavam a mais tempo na lista de espera (p<0,0001). Não observamos diferenças nadistribuição por idade, sexo nem pelo diagnóstico etiológico da insuficiência renal crônica, predominando anefropatia indeterminada e as glomerulopatias. A distribuição por grupo sanguíneo mostrou diferenças significativas: o grupo de imunizados tinha mais pacientes do grupo 0 (X2 =7,9) e menos do grupo A (X2 =3,94).

Fibrous hamartoma of infancy: an Italian multi-institutional experience.

BACKGROUND: Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass. Complete excision is curative. OBJECTIVE AND METHODS: The clinical features and treatment results of 18 children with FH are described. RESULTS: Local excision was the most common procedure. Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis. One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing well, awaiting plastic surgery. LIMITATIONS: The results did not reach statistical significance due to difficulties in collecting cases. CONCLUSIONS: FH should be treated by complete excision; in our experience a nonradical excision was also able to achieve the cure. An aggressive approach should be avoided, as the overall prognosis is excellent.

HLA class I antigen and HLA-A, -B, and -C haplotype frequencies in Uruguayans.

HLA class I antigens were determined for 959 unrelated Uruguayans. The predominant HLA alleles were A2, Cw4, and B35, and the most frequently observed two-loci haplotypes were A2-B44 and B35-Cw4. The most frequent three-loci HLA haplotype was A2-Cw5-B44. We compared the Uruguayan sample with similar data from other populations.