Mario Messina

Telomere length and obesity.

Aim: To assess the telomere length in apparently healthy obese and normal‐weight subjects.

Complications and conversions of pediatric videosurgery: the Italian multicentric experience on 1689 procedures.

BackgroundThe aim of this study was to evaluate the incidence and management of complications of video-surgical procedures that occurred during a 4-year period in eight Italian pediatric surgery centers.MethodsBetween 1996 and 1999, 2305 videosurgical procedures were performed in 11 centers of pediatric surgery. The data from 3 centers, for a total of 616 procedures, were largely incomplete and were thus excluded from the study. We analyzed the data from 8 centers only, for a total of 1689 laparoscopic or thoracoscopic operations on patients aged between 15 days and 16 years. The type of operations performed ranged from basic videosurgical interventions, such as varicocelectomy and cryptorchidism, to advanced laparoscopic procedures, such as splenectomy, total colectomy, and esophageal achalasia. Each patient’s file was examined for any complications that may have occurred during the surgical procedure and for a record of how these were managed.ResultsWe recorded 79 complications (4.6%) in our series. In 57 cases (72.2%) the problem was solved by videosurgery. Twenty-two cases (27.8%) required conversion to open surgery. There was no mortality in our series. At a maximum follow-up of 4 years, all children were alive and had no problems related to the video-surgical complications.ConclusionsWe believe that the routine use of open laparoscopy in pediatric patients is a key factor to avoiding complications related to the Veress needle and blind introduction of the first trocar. Moreover, the surgeon’s laparoscopic experience, the correct indications for laparoscopic surgery, and the verification of the laparoscopic equipment before surgery are also important rules to follow to reduce the incidence of complications. In the beginning, it ispreferable to have the assistance of an expert laparoscopic surgeon to decrease the complications related to the learning curve period.

Italian multicenter survey on laparoscopic treatment of gastro-esophageal reflux disease in children

Background: Skepticism is still present today about the laparoscopic treatment of gastro-esophageal reflux (GER) in children. We present the prospective experience and short-term results of eight Italian pediatric surgical units. Methods: We included all the children with complicated GER, operated after January 1998 by single surgeons from eight different centers. Diagnostic aspects, type of fundoplication, and complications were considered. All the patients were followed for a minimum period of 6 months in order to detect complications or recurrences. Results: 288 children were prospectively included. Mean age was 4.8 years (3 m–14 y). Nissen fundoplication was done in 25%, floppy Nissen in 63%, Toupet in 1.7%, and anterior procedures (Lortat Jacob, Thal) in 10%. Gastrostomy was associated, if neurological impairment or feeding disorders were present. Mean follow-up was 15 months and reoperation was necessary in 3.8% of cases. Conclusions: This experience underlines that minimal invasive access surgery in children is safe and that the laparoscopic approach is considered in eight centers the golden standard for surgical repair of gastro-esophageal reflux disease maintaining the same indications and techniques of the open approach.

Voluminous perinatal pelvic mass: a case of congenital hydrometrocolpos

Imperforate hymen is the most frequent congenital malformation of the female genital tract; it usually does not show symptoms until puberty. Only rarely, imperforate hymen manifests itself as an abdominal mass detectable in the prenatal period. We describe a rare case of voluminous hydrometrocolpos, antenatally diagnosed and successfully treated immediately after birth.

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-1β 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.

Management of status dystonicus in children. Cases report and review

Status dystonicus (SD) is a medical emergency weighed by a relevant morbidity and mortality. It mainly affects patients with primary or secondary dystonia and is often triggered by events such as fever, infections, exposure medications or their abrupt cessation. We report on three patients presenting with SD. Two of them were affected by a static encephalopathy and the other one by a neurodegenerative disorder such as megalencephalic leukoencephalopathy with subcortical cysts (MLC). To our knowledge this is the first patient affected by MLC presenting with SD. All our patients underwent continuous infusion of midazolam, in association with pimozide and trihexyphenidyl, which led to complete resolution of muscular spasms in two patients. In the other one a complete cessation of dystonic spasms was obtained after intrathecal baclofen. From a therapeutic point of view there are no evidence-based management guidelines in SD. The approach is empiric and based on very limited anecdotal reports. On the basis of our observations and an extensive review of the literature we delineated a possible therapeutic strategy of SD in children.

Preoperative distraction in children: hand-held videogames vs clown therapy.

Anxiety in children undergoing surgery is characterized by feelings of tension, apprehension, nervousness and fear which may manifest differently. Postoperative behavioural changes such as nocturnal enuresis, feeding disorders, apathy, and sleep disturbances may stem from postoperative anxiety. Some Authors pointed out that over 60% of children undergoing surgery are prone to developing behavioural alterations 2 weeks after surgery. Variables such as age, temperament and anxiety both in children and parents are considered predictors of such changes.1 Studies were published describing how psycho-behavioural interventions based on play, learning and entertainment in preparing children for surgery, may reduce preoperative anxiety. Clown-therapy is applied in the most important paediatric facilities and has proved to diminish childrens emotional distress and sufferance, as well as consumption of both analgesics and sedatives and to facilitate the achievement of therapeutic goals. The aim of our study was to evaluate the efficacy of clown-therapy during the childs hospital stay, with a view to optimizing treatment and care, preventing behavioural alterations and enhancing the childs overall life quality.

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.

The protean ocular involvement in monogenic autoinflammatory diseases: state of the art

Ocular involvement is frequent in the monogenic autoinflammatory disorders and generally occurs as spontaneously recurring inflammatory events at different ocular sites caused by the aberrant release of proinflammatory cytokines, mainly IL-1β. Over the past decade, we witnessed a significant growth of eye abnormalities associated with idiopathic granulomatous disorders, familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, mevalonate kinase deficiency, and cryopyrin-associated periodic syndrome. The pathogenetic mechanisms of these disorders have shown the evidence of disrupted cytokine signaling, but the explanation for the heterogeneous ocular involvement remains to be elucidated. We herein review the monogenic autoinflammatory disorders affecting the eye, describing their main clinical features with specific regard to the ocular involvement, which can lead to decreased visual acuity and even blindness, if the primary disorder is undetected or left untreated.

Congenital cystic adenomatoid malformation of the lung associated with esophageal atresia and tracheoesophageal fistula

Abstract Bronchopulmonary malformations associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) are extremely rare. The authors describe a case of type II congenital cystic adenomatoid malformation (CCAM) of the right lower lobe associated with EA and TEF (Vogt-Gross type C) in a full-term female infant. The CCAM presented as an incidental radiologic finding, and a contralateral tension pneumothorax developed shortly after surgical repair of the EA. Early recognition of this rare association is essential for correct operative management.