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Background Chronic granulomatous disease (CGD) is a primary immunodeficiency resulting from the absence or malfunction of oxidative mechanism in phagocytic cells. The disease is due to a mutation in one of four genes that encode subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Affected patients experience severe infections and granuloma formation due to exuberant inflammatory responses. Some evidence suggests that eosinophilic cystitis (EC) is included in the spectrum of inflammatory manifestations. EC is an inflammatory disease, rare in childhood, which may require different, nonstandardized therapeutic approaches, ranging from antihistamines to cyclosporine. Case-Diagnosis/Treatment Herein we describe the cases of two CGD patients with CGD who experienced EC during hospitalization for a severe infection. Conclusions EC in immunocompetent children seems to have a self-limiting course, unlike in CGD patients, in whom it presents a prolonged and recurrent course. We focus on the effective therapy administered to our patients with CGD and review the corresponding literature., Introduction Chronic granulomatous disease (CGD) is an uncommon immunodeficiency disorder of phagocyte oxidative metabolism. To date, the cases of only six CGD patients suffering from eosinophilic cystitis (EC) have been [...]

The SIVI (Italian Society of Videosurgery in Infancy) guidelines are clinical practice guidelines edited and approved by the Society's steering committee. They are the products of a detailed systematic review of the literature, integrated with expert opinion in the field of pediatric minimally invasive surgery. These guidelines are intended to assist the pediatric surgeons experienced or not experienced in minimally invasive surgery, with the goal to inform choices related to the indications, approach, and techniques to use when treating the major pediatric surgical pathologies. Given the complexities of congenital malformations and other pediatric surgical conditions, as well as large variations in available regional health services, we must note that these guidelines are not intended as a cookbook recipe to follow for all possible patients. Rather, the guidelines should serve as a flexible framework, to be used by the physician in concert with the parents, to choose the best approach for each individual patient. Decisions tailored to available scientific knowledge and the needs and desires of the patient's family serve both patient autonomy and medical science. All guidelines are published in this scientific Journal, in order to ensure their availability to all physicians. The Guidelines project has been approved by the SIVI General Assembly of the 2016 Madrid congress. Review of guidelines has been performed by the Steering Committee of SIVI and experts. [ABSTRACT FROM AUTHOR]

Introduction Testicular microlithiasis (TM), characterized by the presence of intratubular calcifications in a single or both the gonads, is an uncommon entity with unknown etiology and outcome in pediatric and adolescent age. In this study, the results of a multicenter long-term survey are presented. Materials and Methods From 11 units of pediatric urology/surgery, patients with TM were identified and yearly, followed up in a 7-year period, adopting a specific database. The recorded items were: age at diagnosis, presenting symptoms/associated abnormalities, ultrasonographic finding, surgery and histology at biopsy, if performed. Results Out of 85 patients, 81 were evaluated yearly (4 patients lost to follow-up). TM was bilateral in 66.6% of the patients. Associate genital abnormalities were present in 90%, more frequently undescended/retractile testis (23.4%) and varicocele (22.2%). TM remained unchanged at 4.7 years follow-up in 77 patients (93.8%) and was reduced in 4 patients after 1 to 5 years of inguinoscrotal surgery. Orchiectomy was performed in three patients (3.7%), one for severe testicular hypoplasia and two for seminoma (2.5%), respectively, concurrent and metachronous to diagnosis of TM. Tumorectomy with parenchymal sparing surgery was performed in a teratoma associated with TM. Conclusion TM is a controversial entity, often associated with several inguinogenital features, which rarely can recover. Testicular malignancy, although present in TM, has not proven definitively associated to microliths. Proper counseling, yearly ultrasound, and self-examination are long-term recommended. [ABSTRACT FROM AUTHOR]

Different minimally invasive methods have been proposed to treat renal manifestation of tuberous sclerosis complex (TSC), in order to preserve kidney parenchyma and avoid nephrectomy. The case of a boy with bleeding giant angiomyolipoma treated by selective arterial embolization is discussed. The extensive renal lesions resulted significantly decreased, with resolution of arterial hypertension and abdominal symptoms. Differential renal function increased from 26.6% to 32.6%. Renal involvement in TSC requires careful parenchymal-sparing procedures. Selective embolization of the main renal lesions should be considered as first line therapy. [ABSTRACT FROM AUTHOR]