The "V-I penoscrotal reconfiguration": A simple technique for the surgical treatment of congenital webbed penis.

OBJECTIVES: To describe a new penoscrotal reconfiguration technique, named "V-I penoscrotal reconfiguration" for the surgical reconstruction of a congenital webbed penis (CWP). METHODS: Twenty-one patients who underwent the "V-I penoscrotal reconfiguration technique" were included in this retrospective study. The CWP severity was assessed according to El-Koutby's classification. Demographic and clinical data, surgical data, and postoperative outcomes were scheduled and analyzed. Specifically, the postoperative follow-up included both physical and psychological assessments at 2 weeks, 1, 6, and 12 months after surgery. Parents' satisfaction degree was quantified by the Likert scale. RESULTS: CWP was grade 3 in 11 (52%) patients, 2 in five (24%), and 1 (24%) in five. Five (24%) CWP were isolated malformations, 11 (52%) were associated with phimosis, three (14%) with hypospadias, and two (10%) with hypospadias and phimosis. There were no postoperative complications and no cases of redo surgery. The cosmetic outcomes were excellent in all cases: the parents' satisfaction score was 4 in 17 (81%) cases and 3 (9%) in the other four cases. CONCLUSIONS: CWP may cause psychological distress and functional problems, especially during sexual intercourse. Its correction in childhood is advocated to prevent psychological and sexual issues. The "V-I reconfiguration technique" is simple, and easy with excellent cosmetic and functional outcomes.

Ovarian torsion in the pediatric population: predictive factors for ovarian-sparing surgery-an international retrospective multicenter study and a systematic review.

STUDY OBJECTIVE: Ovarian torsion (OT) in pediatric age is a challenging condition to diagnose and treat. To date, there is still no clear consensus about its management. Our aim was to assess some possible associated factors that can help surgeons in decision-making. DESIGN: We conducted a retrospective multicentric study of pediatric OT surgically treated between 2010 and 2020 in six Italian and German institutions, comparing our findings with a literature review of the last 10 years (2010-2020). PARTICIPANTS: Patients aged 0-18 years with a diagnosis of OT intraoperatively confirmed and surgically treated at the involved institutions. RESULTS: Ninety-seven patients with a mean age at diagnosis of 8.37 years were enrolled in the study. Severe abdominal pain was present in 82 patients (84.5%). Eighty children (82.5%) presented an enlarged ovary with an US diameter > 5 cm and only 32 (40%) of them underwent conservative surgery. A laparoscopic approach was performed in 60 cases (61.9%) although in 15 (15.5%) conversion to open surgery was deemed necessary. A functional cyst was present in 49 patients (50.5%) while 11 children (11.3%) suffered from OT on a normal ovary. CONCLUSIONS: Our results showed that a post-menarchal age (p = .001), a pre-operative US ovarian size < 5 cm, (p = .001), the presence of severe abdominal pain (p = .002), a laparoscopic approach (p < .001), and the presence of a functional cyst (p = .002) were significantly associated with conservative surgery.

Pleural drainage vs video-assisted thoracoscopic debridement in children affected by pleural empyema.

BACKGROUND: Both thoracic drainage and video-assisted thoracic surgery (VATS) are available treatment for pleural empyema in pediatric patients. MATERIALS AND METHODS: This retrospective multicenter study includes pediatric patients affected by pleural empyema treated from 2004 to 2021 at two Italian centers. Patients were divided in G1 (traditional approach) and G2 (VATS). Demographic and recovery data, laboratory tests, imaging, surgical findings, post-operative management and follow-up were analyzed. RESULTS: 70 patients with a mean age of 4.8 years were included; 12 (17.1%) in G1 and 58 (82.9%) in G2. Median surgical time was 45 min in G1, 90 in G2 (p < 0.05). Mean duration of thoracic drainage was 7.3 days in G1, 6.2 in G2 (p > 0.05). Patients became afebrile after a mean of 6.4 days G1, 3.9 in G2 (p < 0.05). Mean duration of antibiotic therapy was 27.8 days in G1, 25 in G2 (p < 0.05). Mean duration of postoperative hospital stay was 16 days in G1, 12.1 in G2 (p < 0.05). There were 4 cases (33.3%) of postoperative complications in G1, 17 (29.3%) in G2 (p > 0.05). 2 (16.7%) patients of G1 needed a redosurgery with VATS, 1 (1.7%) in G2. CONCLUSIONS: VATS is an effective and safe procedure in treatment of Pleural Empyema in children: it is associated to reduction of chest tube drainage, duration of fever, hospital stay, time of antibiotic therapy and recurrence rate.

Pediatric robotic surgery: issues in management-expert consensus from the Italian Society of Pediatric and Neonatal Anesthesia and Intensive Care (SARNePI) and the Italian Society of Pediatric Surgery (SICP).

BACKGROUND: Pediatric robotic-assisted surgeries have increased in recent years; however, guidance documents are still lacking. This study aimed to develop evidence-based recommendations, or best practice statements when evidence is lacking or inadequate, to assist surgical teams internationally. METHODS: A joint consensus taskforce of anesthesiologists and surgeons from the Italian Society of Pediatric and Neonatal Anesthesia and Intensive Care (SARNePI) and the Italian Society of Pediatric Surgery (SICP) have identified critical areas and reviewed the available evidence. The taskforce comprised 21 experts representing the fields of anesthesia (n = 11) and surgery (n = 10) from clinical centers performing pediatric robotic surgery in the Italian cities of Ancona, Bologna, Milan, Naples, Padua, Pavia, Perugia, Rome, Siena, and Verona. Between December 2020 and September 2021, three meetings, two Delphi rounds, and a final consensus conference took place. RESULTS: During the first planning meeting, the panel agreed on the specific objectives, the definitions to apply, and precise methodology. The project was structured into three subtopics: (i) preoperative patient assessment and preparation; (ii) intraoperative management (surgical and anesthesiologic); and (iii) postoperative procedures. Within these phases, the panel agreed to address a total of 18 relevant areas, which spanned preoperative patient assessment and patient selection, anesthesiology, critical care medicine, respiratory care, prevention of postoperative nausea and vomiting, and pain management. CONCLUSION: Collaboration among surgeons and anesthesiologists will be increasingly important for achieving safe and effective RAS procedures. These recommendations will provide a review for those who already have relevant experience and should be particularly useful for those starting a new program.

Surgical management of ovarian teratomas in childhood: a multicentric study on 110 cases and a literature review.

AIM OF THE STUDY: To compare a multicentric surgical experience on ovarian teratomas in childhood with the current management trends. DESIGN: A retrospective multicentric pediatric ovarian teratomas surgically treated between January 2000 and August 2020 at four Italian institutions. PubMed database was used to search for Reviews and Systematic Reviews published between January 2010 and August 2020: 15 manuscripts reported 3633 ovarian neoplasms in pediatric age, 1219 (33,5%) of which were ovarian teratomas. RESULTS: A hundred-ten patients with a mean age at diagnosis of 11.8 years were enrolled. Mature cystic teratomas accounted for the 90% of the masses. At surgery, 78 were oophorectomies and 32 were ovary sparing surgeries. Laparoscopy occurred in 16.3% of the surgeries.As regarding the current management trends, the mean age at diagnosis was 11.9 years and 80.5% of the cases were represented by mature teratomas. Of 430 procedures, 331 were oophorectomies while 99 were ovary sparing surgeries and laparoscopy was performed in 23.8% of cases. CONCLUSIONS: Ovary-sparing surgery with laparoscopic approach is increasingly offered as standard treatment for benign masses that fit the criteria for mature teratomas, in the attempt to achieve the best compromise between the preservation of fertility and the prevention of recurrences. Awareness should be raised among pediatric surgeons to reduce unnecessary radical surgery.

Clinically Differentiated Abnormalities of the Architecture and Expression of Myosin Isoforms of the Human Cremaster Muscle in Cryptorchidism and Retractile Testis.

AIM: To describe architecture and expression of myosin isoforms of the human cremaster muscle (CM) and to individuate changes in clinically differentiated abnormalities of testicular descent: cryptorchidism or undescended testis (UDT) and retractile testis (RT). BACKGROUND: The CM is a nonsomitic striated muscle differentiating from mesenchyme of the gubernaculum testis. Morphofunctional and molecular peculiarities linked to its unique embryological origin are not yet completely defined. Its role in abnormalities of testicular descent is being investigated. SUBJECTS AND METHODS: Biopsy samples were obtained from corrective surgery in cases of cryptorchidism, retractile testis, inguinal hernia, or hydrocele. Muscle specimens were processed for morphology, histochemistry, and immunohistology. RESULTS AND CONCLUSIONS: The CM differs from the skeletal muscles both for morphological and molecular characteristics. The presence of fascicles with different characterization and its myosinic pattern suggested that the CM could be included in the specialized muscle groups, such as the extrinsic ocular muscles (EOMs) and laryngeal and masticatory muscles. The embryological origin from the nonsomitic mesoderm is, also for the CM, the basis of distinct molecular pathways. In UDT, the histological alterations of CM are suggestive of denervation; the genitofemoral nerve and its molecular messengers directed to this muscle are likely defective. Compared with the other samples, RT has a distinct myosinic pattern; therefore, it has been considered a well-defined entity with respect to the other testicular descent abnormalities.

The DATE Association: A Separate Entity or a Further Extension of the VACTERL Association?

BACKGROUND: The combination of esophageal atresia, congenital duodenal obstruction, and anorectal malformation has seldom been reported. We describe the largest series of patients with such association, which we summed up with the mnemonic acronym DATE [D-duodenal obstruction, A-anorectal malformation (ARM), and TE-tracheoesophageal fistula with esophageal atresia]. METHODS: This was a multicenter retrospective review of 13 patients recruited from 8 institutions over a nearly 5-decade period (1968-2017). Information gathered included type of DATE malformations, other associated anomalies, type and timing of surgery, and clinical outcomes. RESULTS: The DATE association consisted of type C esophageal atresia (13), complete (9) or incomplete (4) congenital duodenal obstruction (CDO), and high or intermediate (8) or low (5) ARM. Eight patients had at least one additional component feature of VACTERL association. A total of 6 patients died. Overall, 9 patients achieved complete restoration of gastrointestinal continuity, 7 of whom are alive at a median follow-up of 4 y (range, 1 to 9). Survivors received a median of 6 major operations (range, 4 to 14) to overcome their anomalies and surgical complications. Two incomplete duodenal obstructions were initially overlooked. All survivors with high or intermediate ARM defects required some form of bowel management to keep them clean. CONCLUSIONS: The DATE association is a low-frequency entity, often occurring among the wider spectrum of VACTERL association. Functional outcomes largely depend on the severity of ARM or other major associated malformations. Awareness of the DATE association may avoid untoward diagnostic delays of subtler component features of the spectrum, such as an incomplete CDO.

Efficacy and safety of certolizumab pegol and golimumab in the treatment of non-infectious uveitis.

OBJECTIVES: The aim of the study was to evaluate the efficacy of golimumab (GOL) and certolizumab pegol (CZP) as additional treatment options for the treatment of uveitis. METHODS: Patients with longstanding uveitis receiving either GOL or CZP were retrospectively evaluated in terms of frequency of ocular flares, drug survival, changes in best corrected visual acuity (BCVA) and steroid-sparing effect. RESULTS: Twenty-one patients (30 eyes), 17 of whom being female, were enrolled in the study; 16 out of 21 patients had been previously treated with other tumour necrosis factor (TNF)-α blockers. A significant reduction in ocular flares (from 128.6 bouts for 100 patients-year to 42.9 events for 100 patients-year) was observed between the 12 months prior to the start of GOL or CZP and the 12 months thereafter (p=0.01). The 36-month drug survival was 54.5% for CZP and 50.0% for GOL with no statistically significant differences between the two biologic agents. No differences were detected concerning BCVA values and the mean corticosteroid intake between baseline and the last follow-up. The safety profile was excellent. CONCLUSIONS: GOL and CZP represent effective and safe treatment choices for patients with uveitis also when unsuccessfully treated with other anti-TNF-α drugs, permitting a significant reduction in the frequency of ocular flares and preserving visual function with a good long-term retention rate.

Relapsing Polychondritis: an Update on Pathogenesis, Clinical Features, Diagnostic Tools, and Therapeutic Perspectives.

Relapsing polychondritis is a rare multisystemic disease widely accepted as a complex autoimmune disorder affecting proteoglycan-rich structures and cartilaginous tissues, especially the auricular pinna, cartilage of the nose, tracheobronchial tree, eyes, and heart's connective components. The clinical spectrum may vary from intermittent inflammatory episodes leading to unesthetic structural deformities to life-threatening cardiopulmonary manifestations, such as airway collapse and valvular regurgitation. The frequent association with other rheumatologic and hematologic disorders has been extensively reported over time, contributing to define its complexity at a diagnostic and also therapeutic level. Diagnosis of relapsing polychondritis is mainly based on clinical clues, while laboratory data have only a supportive contribution. Conversely, radiology is showing a relevant role in estimating the rate of systemic involvement as well as disease activity. The present review is aimed at providing an update on scientific data reported during the last 3 years about relapsing polychondritis in terms of pathogenesis, clinical features, diagnosis, and new treatment options.

Untangling the web of systemic autoinflammatory diseases.

The innate immune system is involved in the pathophysiology of systemic autoinflammatory diseases (SAIDs), an enlarging group of disorders caused by dysregulated production of proinflammatory cytokines, such as interleukin-1ß and tumor necrosis factor-α, in which autoreactive T-lymphocytes and autoantibodies are indeed absent. A widely deranged innate immunity leads to overactivity of proinflammatory cytokines and subsequent multisite inflammatory symptoms depicting various conditions, such as hereditary periodic fevers, granulomatous disorders, and pyogenic diseases, collectively described in this review. Further research should enhance our understanding of the genetics behind SAIDs, unearth triggers of inflammatory attacks, and result in improvement for their diagnosis and treatment.

Evolution of outcomes and complications of flip flap laparoscopic repair for inguinal hernia in children: 5 years' experience and practical implication in a third level Italian center.

BACKGROUND: Inguinal hernia repair is one of the most common pediatric surgeries that can be approached with an open or laparoscopic approach. The aim of this study was to describe outcomes and complications of flip flap inguinal hernia repair analyzing our experience in the last 5 years. METHODS: Between 2015 and 2020, 280 children admitted at our department with inguinal hernia requiring surgery were included. Secondary aim was to investigate how confidence of surgeons regarding VLS approach increase during time and how this particular technique represents a primary procedure useful to increase surgeons' skills in performing laparoscopic sutures. RESULTS: One hundred sixty children (57%) underwent open approach, whereas 120 (43%) laparoscopic ligations of hernia. For each group, clinical and surgical data were recorded. We focused on comparing postoperative complications: the most frequent complication was hydrocele (N.=4, 1.4%). Reported postoperative complication were not statistically different between two groups (open N.=3, 1.8% vs. laparoscopy N.=1, 0.8%). No other postoperative complications were documented in our cohort for both groups. In laparoscopic group we registered 3 cases of ipsilateral recurrence (1.6%) and no cases of metachronous hernia. The choice of laparoscopic approach significantly increased from 22% in 2015 to 74% in 2020 (P<0.05) as well as the percentage of male underwent to laparoscopic procedure (38% in 2015 to 74% in 2020, P<0.05). CONCLUSIONS: It is known that the rate of complication or recurrence is similar for open or minimally invasive inguinal hernia repair. Laparoscopy offers advantages such as the possibility of visualizing contralateral internal inguinal ring, reducing the incidence of metachronous inguinal hernia. Moreover, taking confidence with a minimally invasive technique such Flip Flap hernioplasty by performing it repeatedly over time, leads to an improvement of surgeons' skills also in performing laparoscopic sutures, that can be helpful for other complex or tricky procedures.

Alteration of Immunoregulatory Patterns and Survival Advantage of Key Cell Types in Food Allergic Children.

All allergic responses to food indicate the failure of immunological tolerance, but it is unclear why cow's milk and egg (CME) allergies resolve more readily than reactivity to peanuts (PN). We sought to identify differences between PN and CME allergies through constitutive immune status and responses to cognate and non-cognate food antigens. Children with confirmed allergy to CME (n = 6) and PN (n = 18) and non-allergic (NA) (n = 8) controls were studied. Constitutive secretion of cytokines was tested in plasma and unstimulated mononuclear cell (PBMNC) cultures. Blood dendritic cell (DC) subsets were analyzed alongside changes in phenotypes and soluble molecules in allergen-stimulated MNC cultures with or without cytokine neutralization. We observed that in allergic children, constitutively high plasma levels IL-1ß, IL-2, IL-4, IL-5 and IL-10 but less IL-12p70 than in non-allergic children was accompanied by the spontaneous secretion of sCD23, IL-1ß, IL-2, IL-4, IL-5, IL-10, IL-12p70, IFN-γ and TNF-α in MNC cultures. Furthermore, blood DC subset counts differed in food allergy. Antigen-presenting cell phenotypic abnormalities were accompanied by higher B and T cell percentages with more Bcl-2 within CD69+ subsets. Cells were generally refractory to antigenic stimulation in vitro, but IL-4 neutralization led to CD152 downregulation by CD4+ T cells from PN allergic children responding to PN allergens. Canonical discriminant analyses segregated non-allergic and allergic children by their cytokine secretion patterns, revealing differences and areas of overlap between PN and CME allergies. Despite an absence of recent allergen exposure, indication of in vivo activation, in vitro responses independent of challenging antigen and the presence of unusual costimulatory molecules suggest dysregulated immunity in food allergy. Most importantly, higher Bcl-2 content within key effector cells implies survival advantage with the potential to mount abnormal responses that may give rise to the manifestations of allergy. Here, we put forward the hypothesis that the lack of apoptosis of key immune cell types might be central to the development of food allergic reactions.

Gallbladder Stones in Pediatric Age: An Emerging Problem: The Risk of Difficult Cholecystectomy and the Importance of a Preoperative Evaluation.

The need for cholecystectomy during pediatric age has significantly increased in the last two decades. As biliary pathology increases, the probability of complicated cholecystectomies increases too. The aim of this article is to analyze our experience with difficult laparoscopic pediatric cholecystectomy, focusing on the importance of an accurate pre-operative imaging study. We retrospectively analyzed all patients affected by cholelithiasis who underwent laparoscopic cholecystectomy at the Pediatric Surgery Department of San Camillo Forlanini hospital of Rome and Santa Maria alle Scotte University Hospital of Siena from 2017 to 2022. Demographic data, body mass index (BMI), recovery data, laboratory tests, imaging exams, surgical findings, post operative management and outcome were taken into account. Overall, 34 pediatric patients, with a mean age of 14.1 years (6-18 years) were included, with a mean BMI of 29. All patients underwent abdominal ultrasonography and a liver MRI with cholangiography (cMRI). We identified five cases as "difficult cholecystectomies". Two subtotal cholecystectomies were performed. Cholecystectomy in pediatric surgery can be difficult. The surgeon must be able to find alternative strategies to total cholecystectomy to avoid the risk of possible bile duct injury (BDI). Pre-operative imaging study trough ultrasound and especially cMRI is crucial to recognize possible difficulties and to plan the surgery.

Robotic approach to the uretero-vesical junction in children: An international multicentric retrospective study.

BACKGROUND: Robot-assisted approach to UVJ is getting more and more used in pediatric patients. METHODS: In this retrospective study 26 patients affected by nephro-urological malformations, robotic-surgically treated from 2016 and 2021 at 3 Pediatric Surgery Department were included: 3 (11.5%) primary obstructive megaureter, 2 (7.7%) dysplastic kidneys, 3 (11.5%) duplex collecting system, 18 (69.2%) primary vescico-ureteral reflux (VUR). RESULTS: Mean age at surgery was 6 years old. 22 (84.6%) underwent Lich Gregoire extravesical ureteral reimplantation, 4 (15.4%) total nephroureterectomy. Mean operative time was 230 min. No conversions or intraoperative complications. Median hospital stay was 4 days. There were 4 (15.38%) postoperative complications: 3 (11.54%) persistent VUR and 1 (3.84%) refluxing megaureter. 2 (7.7%) redo-surgery. CONCLUSIONS: Robotic Surgery should be considered a safe and effective technique for treatment of UVJ anomalies in children, because it firstly allows surgeons to approach both upper and lower ureteral ends without modifying trocars' placement.

Folliculosebaceous units are a frequent finding in early lichen sclerosus of the foreskin.

BACKGROUND: Folliculosebaceous units (FSU) has been considered an early target of inflammation in vulvar lichen sclerosus (VLS). This diagnostic clue is not reported in lichen sclerosus (LS) of the foreskin (FLS) that is normally hairless. We evaluated the presence and inflammation of FSU and sebaceous glands (SG) in LS of the foreskin. METHODS: Histological specimens from therapeutic circumcision were assessed in order to evaluate the frequency and inflammation of FSU and SG in LS. RESULTS: Ninety-eight cases, grouped into 46 early (group 1) and 52 overt (group 2) FLS were included in the study. SG-FSU were found in 95.7% of group 1, and 65.4% of group 2 cases. Their density was inversely correlated with patient age (P=0.0014). We observed perifollicular inflammation in all cases with visible SG-FSU and frequent FSU abnormalities. CONCLUSIONS: SG and FSU were frequent in early FLS and decreased in advanced disease and adults. We hypothesize that SG and FSU are involved in the inflammatory process leading to FLS. These data, which need further investigation, could help to better understand the pathogenesis of FLS.

Lichen sclerosus in pediatric age: A new disease or unknown pathology? Experience of single centre and state of art in literature.

Lichen Sclerosus (LS) is a chronic inflammatory skin disease with unknown etiology. In pediatric age the main disease "lichenlinked" is the phimosis in male. This is a retrospective study that reports the experience of our clinic and review of the literature. We included all patients affected by pathological phimosis, treated by circumcision between January 2015 and May 2020, older than 6 years old and with an histopathological diagnosis of lichen sclerosus. The aim was to identify prognostic factors based on histological report to plan the clinical management of patients. Statistical analysis was done. We included 207 patients. The mean age of children was 9,78 years (5-18 years, DS±3.29). Based on the histological features we divided patients in 2 groups: early lesions (70/207, 34%) and advanced (137/207, 66%). In term of complications lichen linked we considered meatal stenosis that needed of urethral dilatations. We included 7 patients (7/207, 3,4%). We report P value Statistical Significance in many aspects. An early diagnosis of LS and surgical treatment of foreskin are essential to prevent early and late complications in children. The size of sample is a limit of the study but results encourage our management.

Pediatric Surgical Care During the COVID-19 Lockdown: What Has Changed and Future Perspectives for Restarting in Italy. The Point of View of the Italian Society of Pediatric Surgery.

Background: The coronavirus disease 2019 (COVID-19) time exacerbated some of the conditions already considered critical in pediatric health assistance before the pandemic. A new form of pediatric social abandonment has arisen leading to diagnostic delays in surgical disorders and a lack of support for the chronic ones. Health services were interrupted and ministerial appointments for pediatric surgical healthcare reprogramming were postponed. As a result, any determination to regulate the term "pediatric" specificity was lost. The aim is, while facing the critical issues exacerbated by the COVID-19 pandemic, to rebuild future perspectives of pediatric surgical care in Italy. Methods: Each Pediatric Society, including the Italian Society of Pediatric Surgery (SICP), was asked by the Italian Federation of Pediatric Associations and Scientific Societies to fill a questionnaire, including the following the main issues: evaluation of pre-pandemic criticalities, pediatric care during the pandemic and recovery, and current criticalities. The future care model of our specialty was analyzed in the second part of the questionnaire. Results: Children are seriously penalized both for surgical treatment as well as for the diagnostic component. In most centers, the pediatric surgical teams have been integrated with the adult ones and the specificity of training the pediatric operating nursing is in danger of survival. "Emotional" management of the child is not considered by the general management and the child has become again an adults patient of reduced size. Conclusion: A new functional pediatric surgical model needs to be established in general hospitals, including activities for day surgery and outpatient surgery. To support the care of the fragile child, a national health plan for the pediatric surgery is required.

Human microvascular lymphatic and blood endothelial cells produce fibrillin: deposition patterns and quantitative analysis.

Fibrillin microfibrils constitute a scaffold for elastin deposition in the wall of arteries and form the anchoring filaments that connect the lymphatic endothelium to surrounding elastic fibers. We previously reported that fibrillin is deposited in a honeycomb pattern in bovine arterial endothelial cells, which also deposit microfibril-associated glycoprotein (MAGP)-1, whereas thoracic duct endothelial cells form an irregular web. The present immunohistochemical study was designed to verify whether lymphatic and blood human dermal microvascular endothelial cells (HDMECs) isolated from human foreskin by the sequential use of a pan-endothelial marker, CD31, and the lymphatic specific marker, D2-40, deposit fibrillin and MAGP-1. In both cell types, fibrillin and MAGP-1 co-localized and were deposited with different patterns of increasing complexity co-existing in the same culture. Fibrillin microfibrils formed a wide-mesh honeycomb leaving fibrillin-free spaces that were gradually filled. This modality of fibrillin deposition, similar to that of bovine large artery endothelial cells, was basically the same in blood and lymphatic HDMECs. In some lymphatic HDMECs, fibrillin was initially deposited as uniformly scattered short fibrillin strands probably as a result of anchoring filaments carried over from the vessels of origin. Our findings show that blood and lymphatic endothelial cells participate in fibrillin deposition in human skin.

Silent increase of urinary ethylmalonic acid is an indicator of nonspecific brain dysfunction.

Our aim was to compare urinary ethylmalonic acid (EMA) levels in subjects who had no apparent clinical reason to have increased levels of this substance but were suffering from non-specific CNS impairment, and healthy controls. Urinary EMA concentrations detected by (1)H-NMR spectroscopy were studied in 130 subjects with CNS impairment of unknown origin (with no definite diagnosis, no specific symptoms or signs, and normal common biochemical and metabolic screening results) and 130 age- and sex-matched healthy subjects. EMA levels exceeding two standard deviations (SD) above normal (i.e. 8.1 mmol/molCn) were found in a subgroup of CNS-impaired patients and healthy controls. EMA levels exceeding 2 SD above normal were fourfold prevalent in the urine of patients with non-specific CNS impairment compared to from the EMA levels in healthy controls. Moreover, we found that the level exceeding > 8.1 mmol/molCn (i.e. > + 2 SD) had sufficient discrimination accuracy in identifying subjects with non-specific CNS impairment; the level exceeding 12 mmol/molCn (i.e. > + 6 SD) reaches suitable accuracy (i.e. 100% specificity and 78.6% sensitivity). These observations are of importance, as we found that subtle increases in urinary EMA levels are frequent in patients with non-specific CNS impairment. The reasons for this association remain unknown.

Isolated fallopian tube torsion in pediatric age: An Italian multicenter retrospective study.

PURPOSE: The purpose of this study was to quantify the experience of five Italian centers on the diagnosis and management of isolated fallopian tube torsion (IFTT) in children. METHODS: We retrospectively reviewed the data of 20 patients aged 1-16 years of age with surgically diagnosed IFTT between 1991 and 2017 from five Italian centers of pediatric surgery. We analyzed common presenting signs, symptoms, and radiographic findings, as well as surgical interventions to describe management offering further insight into the diagnosis and treatment of this rare entity. RESULTS: Twenty cases of IFTT were collected. Median age was 13.1 years. Menarche was present in 14 cases. A clinical history of abdominal pain was present in 13 patients, whereas in 7 patients the clinical picture was an acute abdomen. Ultrasonography was the first diagnostic examination in 16 cases. Surgical approach was by laparoscopy in 16 cases and laparotomy in 4 cases. In 11 patients IFTT was associated with another pathologic condition. In 7 of the remaining 9 IFTT without pathologic association, the girls played sports. Salpingectomy was performed in 13 patients and de-torsion in 7. CONCLUSIONS: IFTT is a rare condition that seems to occur in younger adolescents. Vague clinical presentation contributes to low preoperative suspicion. IFTT should be considered in girls with abdominal pain who practice sports with sudden body movements. Preoperative suspicion may be increased based on radiographic findings of an enlarged tubular/cystic structure with adjacent normal ovary. Conservative management is controversial but could be preferred in order to provide the best option for future fertility of these girls. TYPE OF STUDY: Treatment Study (Retrospective Study) - Level IV.