Stem cells to restore insulin production and cure diabetes.

BACKGROUND: The advancement of knowledge in the field of regenerative medicine is increasing the therapeutic expectations of patients and clinicians on cell therapy approaches. Within these, stem cell therapies are often evoked as a possible therapeutic option for diabetes, already ongoing or possible in the near future. AIM: The purpose of this document is to make a point of the situation on existing knowledge and therapies with stem cells to treat patients with diabetes by focusing on some of the aspects that most frequently raise curiosity and discussion in clinical practice and in the interaction with the patient. In fact, at present there are no clinically approved treatments based on the use of stem cells for the treatment of diabetes, but several therapeutic approaches have already been evaluated or are being evaluated in clinical trials. DATA SYNTHESIS: It is possible to identify three large potential application fields: 1) the reconstruction of the ß cell mass; 2) the immunomodulation in type 1 diabetes (T1D); 3) the treatment of complications. In this study we will limit the discussion to approaches that have the potential for clinical translation, deliberately omitting aspects of basic biology and preclinical data. Also, we intentionally omit the treatment of the complications that will be the subject of a future document. Finally, an overview of the Italian situation regarding the storage of cord blood cells for the therapy of diabetes will be given.

[Sacrococcygeal teratoma: single center experience and functional long-term follow-up]. / Teratoma sacrococcigeo: esperienza monocentrica e follow-up funzionale a lungo termine.

OBJECTIVE: To propose long-term follow-up protocol to analyse clinical and functional aspects and evaluate the Quality of Life (QoL) of patients with Sacrococcygeal Teratoma (SCT). MATERIALS AND METHODS: The long-term follow-up of 14 out of 28 patients has been assessed through the retrospective review of data related to antenatal diagnosis, obstetric pathology and surgery, collected from July 1985 to December 2009. It has been accomplished by analysing functional and aesthetic outcomes and the QoL experienced by the patients with an average observation range of 121 months for each patient. RESULTS: Concerning the anorectal functionality, 14% of patients reported impairment of fecal continence while 36% had completely normal bowel habits. Other health problems varying from urinary incontinence to neurogenic bladder were reported by 36% of patients. Dealing with the functionality of lower limbs, 20% of patients exhibited minor dysfunctions while 7% major ones. Optimal aesthetic outcome of the surgical scar has been reported only by 21% of the specimen. The evaluation of QoL pointed out that 64% of patients are moderately satisfied while 36% presented problems. CONCLUSIONS: We believe that functional sequelae should play an important role during antenatal counselling and that the urological and anorectal follow-up for SCT patients should be long-term evaluated. Furthermore, aesthetic aspects and psychological support should be taken into account carefully especially during childhood.

[Percutaneous endoscopic gastrostomy (PEG) in pediatric patients: our experience and proposal of follow-up methodology]. / Gastrostomia endoscopica percutanea (PEG) nei pazienti pediatrici: nostra esperienza e proposta operativa di metodologia osservazionale.

OBJECTIVE: To evaluate effectiveness and acceptability of percutaneous endoscopic gastrostomy (PEG) in pediatric patients and to propose a data collection and follow-up methodology. MATERIALS AND METHODS: Observational retrospective and prospective study on 33 pediatric patients and 5 adults with PEG, placed using Gauderer "push" technique, between 2000 and 2007. By means of an appropriate questionnaire, the following parameters were evaluated: complications, factors of further risk, nutritional status, management and acceptability of PEG. RESULTS: No problems occurred during placement. Complications were few and easy to resolve. In 3 patients a stomal dehiscence occurred, strongly related to the tube gauge. During replacement, in 4 patients, bumper was not taken away because of difficult removal. 8 patients had pre-PEG Gastroesophageal reflux: In 2 of them, during the PEG placement, fundoplication was realized. Subsequently PEG procedure, only 1 patient needed fundoplication for worsening of GER. All of them continued gastroprotective treatment. Respiratory tract infections decreased in our 13 patients carries of tracheostomy. CONCLUSIONS: To prefer smaller gauge reduces risk of dehiscence. If the bumper's removal is hard, to leave it inside is acceptable and quite safe, on condition of a careful surveillance of gastrointestinal obstruction signs. GER is not a contraindication of PEG. A careful follow-up is important, by recording all the evaluated parameters and by questionnaire to the family, during every hospital admission. This study, even if on few patients, confi rms PEG as the technique of choice for long-term enteral feeding, also in children. Training of family and caregivers is important to care.

Major anastomotic dehiscence after repair of esophageal atresia: conservative management or reoperation?

SUMMARY. The authors report a case of recurrent anastomotic dehiscence following surgical repair of type C esophageal atresia according to the Gross classification. Surgical repair was followed by a recurrence, which was successfully managed with conservative treatment. Esophageal atresia with fistulization of the lower pouch in a male newborn with the VACTER association was repaired with a high-tension single-layer anastomosis. On the fifth postoperative day, major anastomotic dehiscence (> 4 mm) was diagnosed. The breach was re-sutured and the anastomosis reinforced with fibrin glue, but dehiscence recurred again 4 days later. Surgery was deferred and the infant was treated conservatively with continued chest-tube drainage and total parenteral nutrition. After 43 days, complete closure of the anastomosis was documented. Even major anastomotic dehiscence can be successfully managed with conservative treatment (chest-tube drainage, suspension of oral feedings, total parenteral nutrition). If the patient is otherwise stable, we feel that this approach should be attempted even when major leakage is present.

Fibromyxoid sarcoma in a 4-year-old boy: case report and review of the literature.

The authors report on the case of a low-grade fibromyxoid sarcoma (LGFMS) with giant rosettes in a 4-year-old boy. The tumor arose in the paravertebral region and had infiltrated the vertebral canal between L2 and S1. A review of the literature indicates that this is one of the youngest patients diagnosed with a tumor of this type, and spinal involvement had never been reported with LGFMS.

[Utility and safety endoscopic digestive procedure in pediatric age]. / Utilità e sicurezza delle procedure endoscopiche digestive in età pediatrica.

The aim of this study is to verify the utility and safety of endoscopic procedures in the evaluation of children with clinically-significant gastrointestinal symptomatology. We report our experience of 87 pediatric endoscopy procedures including esophagogastroduodenoscopy, colonoscopy and tissue biopsies performed in 85 infants, children and adolescent, 3 months-15 years old, over a two-year period, june 2002-november 2004 after complete history, physical examination and basic investigations. General anesthesia was used in all patients after informed consent obtained from parents. Non significant complications were observed in this series of patients. In 81 cases (92.5%) with clinical symptoms and laboratory indications for gastrointestinal disease, the endoscopy and bioptical samples confirmed the utility and safety of procedure. Coeliac disease (39 cases), gastritis (11 cases), esophagitis (6 cases) were the most common organic cause of upper gastrointestinal disease. Allergic and indeterminate colitis (7 cases) were the most common cause of lower gastrointestinal disease. In 4.7% the procedures appear to be particularly helpful in the diagnosis of inflammatory lesions of the esophagus and stomach. In summary, the data demonstrate that endoscopy techniques show low morbidity, provide important diagnostic informations in pediatric gastrointestinal diseases and can be done safely in patients over 3 months of age.

Malignant retroperitoneal paraganglioma: case report and review of the literature.

Paraganglioma is a rare neuroendocrine neoplasm observed in patients of all ages but it has not been characterized in children. The authors describe a retroperitoneal paraganglioma diagnosed by chance in an 11-year-old boy. Many aspects of retroperitoneal paraganglioma are still under investigation. The treatment of choice is radical resection. Surgery may be possible following chemotherapeutic debulking with cyclophosphamide, vincristine, and dacarbazine. 131I-MIBG radiotherapy has proved increasingly useful in reducing the pain associated with disseminated disease and also in facilitating surgical resection in cases that appear inoperable. Conventional radiotherapy is purely palliative and used to reduce the pain of bone metastases.

Choledochal cyst in a pediatric patient: creation of a bilijejunal anastomosis with surgical staples. A case report.

Although the use of surgical staples is a well established practice in intestinal tract surgery on adults, their role in biliodigestive anastomoses in adults and children has been more limited. The Authors describe a 12-year-old girl with a type-IV choledochal cyst, who was successfully treated with cyst excision and Roux-en-Y hepaticojejunostomy created with a surgical stapler.

Bilateral renal vein thrombosis as a complication of gangrenous appendicitis.

Bilateral renal vein thrombosis after an appendectomy has never been reported in the pediatric literature. We describe the case of a 10-year-old boy who developed this very unusual complication following appendectomy for gangrenous appendicitis with peritonitis. Color duplex Doppler is the most appropriate investigation to allow correct diagnosis and immediate medical treatment. Peritoneal dialysis is a simple and effective tool to prevent permanent damage to renal function.

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

BACKGROUND: The aim of this study was to evaluate the incidence and management of complications of videosurgical procedures that occurred during a 4-year period in eight Italian pediatric surgery centers. METHODS: Between 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. RESULTS: We 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 videosurgical complications. CONCLUSIONS: We 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 is preferable to have the assistance of an expert laparoscopic surgeon to decrease the complications related to the learning curve period.

Thyroid tumors in children and adolescents: preoperative study.

Several studies indicate that in young patients (less than 21 years of age at the time of diagnosis), the prognosis of thyroid carcinoma (TC) is more favorable than in older patients. However, a more radical treatment approach is recommended in children and adolescents due to the higher prevalence of local lymph-node involvement in these cases. Since the extent of primary surgical treatment is closely related to the overall prognosis, preoperative diagnosis becomes essential in the management of thyroid neoplasms in young patients. In this retrospective study (1987-1998), we analyzed a surgical series of 50 children and adolescents with thyroid nodules in an attempt to establish the role of diagnostic studies in detecting malignant lesions prior to surgery. Our diagnostic protocol for evaluating thyroid nodules was based on clinical evaluation, measurement of thyroid-hormone and thyroglobulin (TG) levels, anti-TG and anti-TPO antibody titers, calcitonin, CEA, and TPA levels, sonography, scintigraphy, and fine-needle aspiration cytology (FNAC) of the thyroid nodules and any enlarged lymph nodes. Eleven of the 15 cases of histologically confirmed carcinoma were preoperatively identified as malignant lesions with the aid of FNAC. The authors conclude that the preoperative work-up of children and adolescents with thyroid nodules requires the collaboration of an experienced team of professionals, and recommend FNAC as the initial test.

An original technique for bladder autoaugmentation with protective abdominal rectus muscle flaps: an experimental study in rats.

BACKGROUND: Bladder autoaugmentation uses partial detrusorectomy to create a diverticular bulge in the bladder mucosa. This technique has eliminated certain serious complications of cystoplasty with gastrointestinal tissues (e.g., fluid/electrolyte/acid-base imbalances, mucous hypersecretion), but the exposed mucosa is subject to fibrosis and, sometimes, to perforation, which can annul the benefits of surgery. METHODS: We have developed an original technique based on traditional autoaugmentation with protection of the herniated mucosa by split-thickness pedunculated rectus abdominis muscle flaps that are sutured to the incised margins of the detrusor. Preliminary testing was done on 30 adult Wistar rats. A control group of 15 rats underwent laparotomy alone. Bladder capacity was measured via suprapubic cystography before and after (4 weeks, 8 weeks, 1 year) surgery, just before sacrifice. Sections of the reconstructed bladder were examined histologically. RESULTS: Twenty-three bladder-augmented rats and 13 controls survived. In the experimental group, bladder capacity increased by 38% (mean). None of the rats experienced urinary retention, although one developed bladder stones. Histology revealed no pathologic changes (other than chronic inflammatory infiltrates at suture sites) in the mucosa, detrusor, or muscle flaps, which were all viable and well integrated by the fourth postoperative week. There were no signs of mucosal or muscle fibrosis. CONCLUSIONS: Preliminary results in a rat model suggest that this new technique can produce an enlarged bladder that is fully functional and less vulnerable to fibrotic retraction and rupture. Residual contractility in the muscle flaps might theoretically be exploited to facilitate paraphysiologic micturition.

Varicocele in pediatric patients: comparative assessment of different therapeutic approaches.

OBJECTIVES: The best therapeutic approach for varicocele correction in pediatric patients is still an object of some debate. We analyzed a series of 99 patients to identify the most effective approach in terms of low recurrence rates and preservation of testicular growth. METHODS: One hundred four operations were performed on 99 patients between 9 and 16 years of age (mean 13.3). The first 18 patients underwent sclerotherapy of the internal spermatic vein. Twenty others underwent inguinal varicocelectomy. In 12 patients, a modified Palomo procedure (ie, sparing of the internal spermatic artery) was performed (four laparoscopically), and 54 were treated with the original Palomo procedure (ie, sectioning of the entire spermatic cord), using laparoscopy in 20. RESULTS: Three recurrences (16.6%) occurred among the patients treated with sclerotherapy. Similar rates of recurrence were observed in those who underwent inguinal varicocelectomy (3 [15%] of 20) and modified Palomo procedures (2 [16.6%] of 12). Only one recurrence occurred in the 54 patients treated with the original Palomo procedure (1.85%). CONCLUSIONS: Our experience, together with a review of published reports, leads us to believe that the open Palomo procedure as originally described is the most effective approach to the correction of varicoceles in adolescents.

Recurrent peptic stenosis of the esophagus: treatment with a self-expanding metallic stent.

A 12-year-old neurologically impaired boy with recurrent peptic stenosis of the esophagus was treated successfully with use of a self-expanding metallic stent that remained for 3 months.

Laparoscopic approach for nonpalpable abdominal testis.

The approach to nonpalpable testis is probably the most important indication for laparoscopy in pediatric surgery. In abdominal testis, spermatic vessels can be too short to allow for standard orchidopexy. The division of the spermatic vessels proposed by Fowler and Stephens may result, in some cases, in testicular hypotrophy or atrophy from intraoperative devascularization. In this study, we report our experience of two-stage laparoscopic orchidopexy, first proposed by Bloom. Five patients (ages 2-10 years) were treated with this technique. Laparoscopic inspection and division of spermatic vessels were successful in all patients, with no complications. Orchidopexy was performed at an average of 6 months from the first operation. During a follow-up period of from 6 to 18 months, no patient developed atrophy or hypotrophy of the testis.

[Cryptorchidism: a study of histological changes in undescended testis related to age]. / Criptorchidismo: studio delle alterazioni istologiche nei testicoli ritenuti in rapporto all'età.

A total of 46 testicular biopsies were examined to define the histological characteristics of undescended testicle. Nine of the biopsied organs were normally positioned testicles from deceased pediatric patients (aged 2 months-14 years), and the remaining 37 were undescended testes from children with various degrees of cryptorchidism (aged 18 months-14 years). As reported by others, the degree of histological damage was directly proportional to the age of the subject. Close correlation between histological findings and the level of descent was not observed.

The rationale for total hip replacement in over 60-year-old patients.

The total hip replacement (THR) survival potential is linked to the material, to surgical technique and to patient's characteristics. Cemented and cementless systems exist in a wide variety of shapes, sizes, configurations, materials and surface treatments. Aseptic loosening remain the principal mechanism of prosthetic failure and the choice of right matching for each prosthesis-patient is the main issue to obtain a very good long-term implant fixation. Cement fixation is always solidly achieved but it can break down under mechanical and biological pressure. Stability with cementless fixation must be created by the initial bone response to the implant. Clinical studies and long-term results with THR showed that in patients with age between 60 and 70 years, it is important to analyze several parameters (bone quality and morphology, life-limiting diseases and levels of activity) to make the appropriate selection of components and type of fixation.

Laparoscopic cholecystectomy in a 23-month-old infant.

We describe a laparoscopic cholecystectomy performed on a 23-month-old girl. The experience and the data in the literature indicate that this approach offers particular advantages for treatment of pediatric patients without increasing the operative risk.

[A giant lymphangioma of the trunk with a fatal outcome]. / Linfangioma permagno del tronco ad esito infausto.

The authors describe the case of a 2-month-old male brought to the emergency room in a preagonal state cause by extensive septic necrosis of an enormous lymphangioma located on the left lateral trunk. In spite of emergency treatment the child expired shortly after admission. Our observation of this case prompted a review of the literature on the treatment approaches currently used for these tumors. Emphasis is placed on the necessity for early treatment in order to avoid infectious complications that, if neglected, can lead to unmanageable and possibly fatal sepsis.