Isolation of an "Early" Transit Amplifying Keratinocyte Population in Human Epidermis: A Role for the Low Affinity Neurotrophin Receptor CD271.

In the interfollicular epidermis (IFE), stem cells (KSC) generate transit amplifying (TA) cells that, after symmetric divisions, produce differentiating daughters. Here, we isolated and characterized the highly proliferative interfollicular epidermal basal cell population "early" TA (ETA) cells, based on their capacity to adhere to type IV collagen. Proliferation and colony-forming efficiency in ETA cells are lower than in KSC but higher than in "late" TA (LTA). Stemness, proliferation, and differentiation markers confirmed that ETA cells display a unique phenotype. Skin reconstructs derived from ETA cells present different features (epidermal thickness, Ki67, and Survivin expression), as compared to skin equivalents generated from either KSC or LTA cells. The low-affinity neurotrophin receptor CD271, which regulates the KSC to TA cell transition in the human epidermis through an on/off switch control mechanism, is predominantly expressed in ETA cells. Skin equivalents generated from siRNA CD271 ETA cells display a more proliferative and less differentiated phenotype, as compared to mock-derived reconstructs. Consistently, CD271 overexpression in LTA cells generates a more proliferative skin equivalent than mock LTA cells. Finally, the CD271 level declines with cellular senescence, while it induces a delay in p16INK4 expression. We conclude that ETA cells represent the first KSC progenitor with exclusive features. CD271 identifies and modulates ETA cells, thus participating in the early differentiation and regenerative capacity of the human epidermis.

Hypospadias: clinical approach, surgical technique and long-term outcome.

BACKGROUND: Hypospadias is one of the most common congenital abnormalities in male newborn. There is no universal approach to hypospadias surgical repair, with more than 300 corrective procedures described in current literature. The reoperation rate within 6-12 months of the initial surgery is most frequently used as an outcome measure. These short-term outcomes may not reflect those encountered in adolescence and adult life. This study aims to identify the long-term cosmetic, functional and psychosexual outcomes. METHODS: Medical records of boys who had undergone surgical repair of hypospadias by a single surgical team led by the same surgeon at a single centre between August 2001 and December 2017 were reviewed. Families were contacted by telephone and invited to participate. Surgical outcome was assessed by combination of clinical examination, a life-related interview and 3 validated questionnaires (the Penile Perception Score-PPS, the Hypospadias Objective Score Evaluation-HOSE, the International Index of Erectile Function-5-IIEF5). Outcomes were compared according to age, severity of hypospadias, and respondent (child, parent and surgeon). RESULTS: 187 children and their families agreed to participate in the study. 46 patients (24.6%) presented at least one complication after the repair, with a median elapsed time of 11.5 months (6.5-22.5). Longitudinal differences in surgical corrective procedures (p < 0.01), clinical approach (p < 0.01), hospitalisation after surgery (p < 0.01) were found. Cosmetic data from the PPS were similar among children and parents, with no significant differences in child's age or the type of hypospadias: 83% of children and 87% of parents were satisfied with the cosmetic result. A significant difference in functional outcome related to the type of hypospadias was reflected responses to HOSE amongst all groups of respondents: children (p < 0.001), parents (p=0.02) and surgeon (p < 0.01). The child's HOSE total score was consistently lower than the surgeon (p < 0.01). The HOSE satisfaction rate on functional outcome was 89% for child and 92% for parent respondents. CONCLUSION: Surgeons and clinicians should be cognizant of the long-term outcomes following hypospadias surgical repair and this should be reflected in a demand for a standardised approach to repair and follow-up.

Thoracoscopic treatment of a rare bilateral extralobar lung sequestration in a 3-years old girl.

Majority of sequestrations fall into two categories: Intra-Lobar (ILS) and Extra-Lobar (ELS). Rarely the abnormal lung could be attached to the gastrointestinal tract, Bronchopulmonary Foregut Malformation (BPFM). We described a case of a girl of 3-years-old with antenatal diagnosis of left intrathoracic mass of the inferior lobe. Postnatal Computed-Tomography (CT) revealed a bilateral ELS with an isthmic bridge crossing the vertebral spine. She follows a MRI follow-up at 18months/30months confirming the lesion. Before surgery, a three-dimensional-CT-angiography was performed to study the mass, its blood supply and to plan surgery. She underwent to thoracoscopic resection. Two aberrant blood vessels were dissected from the thoracic aorta and ligated. The postoperative course was uneventful. She was discharged after 3 days. The rarity of our case is due to the bilateral extension. An appropriate preoperatory imaging study is necessary for the success of surgery while thoracoscopy is particularly appropriate in surgical treatment.

First case of laparoscopic partial splenectomy in a child with hamartoma: Case report and review of the literature.

INTRODUCTION: To date, laparoscopic surgery has played a key role in the treatment of not only splenic hematologic pathologies but also solid ones. Hamartoma is a rare disease; only twenty percent of them are of pediatric relevance; it is a benign tumor, but radiological features never allow proper differentiation from malignant neoplasms. In children, hamartoma may be associated with other morbid conditions, such as sickle cell disease or other hematological alterations. PRESENTATION OF THE CASE: We report a case of hamartoma in a 7-year-old child treated with partial laparoscopic splenectomy. After a multidisciplinary evaluation, the indication of laparoscopic splenectomy was decided; upon evaluating the age of the patient and the affected spleen portion, a partial splenectomy was proposed. The histological examination during surgery was performed to exclude any form of malignancy. The intraoperative frozen section of the specimen was negative for malignancies, and a partial splenectomy was performed. DISCUSSION: Surgery remains the first choice in the definitive treatment of solid lesions of the spleen; minimally invasive technique, namely, laparoscopy, has set itself as the technique of choice for surgical treatment. In this case, the possibility of obtaining an intraoperative pathological diagnosis by frozen section of the specimen, confirming the benign nature of the lesion, allowed the surgeon to decide in favor of a laparoscopic partial splenectomy. CONCLUSION: Partial laparoscopic splenectomy can be considered a safe, effective and reproducible alternative in patients suffering from benign solid diseases, safeguarding the hematological functions of the organ itself in pediatric age.

Transperitoneal laparoscopic adrenalectomy in children.

PURPOSE: The use of a minimally invasive approach for adrenalectomy is poorly defined in pediatric patients, although laparoscopic adrenalectomy is considered a standard procedure in adults. The aim of our study was to describe the safety and feasibility of minimally invasive adrenalectomy in children on the basis of surgical skills and results. MATERIALS AND METHODS: This was a retrospective study of 4 pediatric laparoscopic adrenalectomies performed at our center between 2009 and 2012. All patients underwent transperitoneal lateral laparoscopic adrenalectomies (2 right and 2 left adrenalectomies). RESULTS: Four laparoscopic adrenalectomies were performed. Indications for surgery were neuroblastoma in 2 patients, secernent adrenocortical tumor in 1 patient, and adrenocortical nodular hyperplasia in 1 patient. Patients had a mean age of 87 months (range, 17-156 months) at diagnosis, and the average lesion size was 3.23 cm (range, 0.7-6.4 cm). All laparoscopic adrenalectomies were successful, no conversions to open surgery were required, and no postoperative complications or deaths occurred. The average operating time was 105 minutes (range, 80-130 minutes), blood loss during surgery was minimal, and the mean postoperative hospital stay was 3.75 days (range, 3-5 days). None of the patients showed signs of recurring disease at 15-month follow-up. CONCLUSIONS: Laparoscopic adrenalectomy is a safe, feasible, and reproducible technique offering numerous advantages, including shortening of operating times and postoperative hospital stays, as well as reduction of blood loss and complications. It also provides good visibility and easy access to other organs.

A case of frozen pelvis: primary actinomycosis of urinary bladder in a young boy.

We report a case of urinary bladder actinomycosis in childhood. In children abdominal actinomycosis is rare and unlikely involves the urinary tract, so it is often misdiagnosed. An 7-year-old boy was referred to a secondary level hospital because of abdominal pain and dysuria. Physical examination revealed a left hypochondrial mass. Hypothesizing a pelvic rhabdomyosarcoma, a biopsy with mini-laparotomy access was performed. The first histopathological analysis did not show any malignant cells, and a 14-day antibiotic course was ineffective. Reoperation and biopsy was needed, and the histopathological examination made the diagnosis possible.

Bilateral xanthogranulomatous funiculitis and orchiepididymitis in a 13-year-old adolescent boy.

Xanthogranulomatous orchitis is an extremely rare inflammatory nonneoplastic lesion of the testis. We report a case of a 13-year-old adolescent boy who presented a painless left hemiscrotal swelling. The subsequent ultrasonography and magnetic resonance imaging revealed the presence of abnormal expanding tissue located in both testes and spermatic cord, reaching the internal inguinal ring. Testicular tumor markers were normal. The frozen section examination of the surgical specimen showed only inflammatory tissue and not neoplastic tissue. No orchiectomy was performed. Definitive histopathologic diagnosis was xanthogranulomatous inflammation. To our knowledge, this is the youngest case of xanthogranulomatous orchiepididymitis and funiculitis found in medical literature.

Jejunal obstruction as initial presentation of polyarteritis nodosa in a 13-month-old boy.

Systemic polyarteritis nodosa (PAN) is a rare disease in childhood affecting small and midsized arteries. The typical presentation in children is of isolated 1- or 2-organ involvement, and the diagnosis is often based on the histopathology. We report a case of pediatric PAN whose presenting symptom was jejunal obstruction owing to ischemic necrosis of the small bowel. The postoperative recovery was uneventful, and the patient responded well to steroid therapy. There are only a few cases of PAN with intestinal involvement described in the literature, some of them fatal. The diagnosis of PAN should be considered in children with acute intestinal obstruction with no obvious etiology, as early recognition and treatment may reduce its high mortality.

Once-daily intrapleural urokinase treatment of complicated parapneumonic effusion in pediatric patients.

In this paper, we describe our experience in the treatment of childhood empyema using urokinase. Patients' ages ranged from 2 to 12 years. Urokinase (dosage: 3,100 IU/kg/day) was diluted in normal saline to produce 1000 IU/ml (maximum dosage 100,000 IU in 100 ml of normal saline). After 2 hours, the clamped catheters were released and connected to water-seal suction at a negative pressure of 10 cm H2O. Pleural irrigations were continued once a day until thoracostomy tube output decreased to less than 10 ml/day (urokinase treatment mean duration: 11.5 days). The complete resolution of the chest effusion was assessed on chest ultrasound scan and radiographs. None of the patients experienced any side effects due to urokinase. It would now seem reasonable to advocate small chest tube thoracostomy and intrapleural urokinase as first-line treatment of pleural empyema in children, with surgery indicated as a secondary intervention.

Three small testes in left hemiscrotum: a rarer case of polyorchidism.

Polyorchidism is a rare congenital anomaly. A review of literature has yielded about 100 cases. We describe a rarer case of polyorchidism consisting of 3 testes on left hemiscrotum and one normal testis in right hemiscrotum. Diagnosis and management are discussed.