Combined use of percutaneous and transurethral instruments in the preadolescent children with intravesical pathologies

The approach for the intravesical suspicious lesions or stones in the preadolescent children especially in boys is still a challenging problem. Open surgery, percutaneous suprapubic endoscopy or transurethral endoscopic approaches are the treatments of choice in children. However, there is a group in children between the ages of 10 and 12 years, which can be named as grey zone population; the length of the instruments is insufficient for transurethral intervention such as endoscopic stone extraction, transurethral bladder tumor resection especially for the lesion at the posterior bladder wall in this group. The aim of this study is to describe a new technique using laparoscopic instruments for percutaneous bladder stone and tumoral lesion and determines the efficacy of this procedure. Satisfactory results have been obtained in patients with these pathologies. In selected cases, operation time, urethral and mucosal damage, hospital stay, and cost can be decreased to a minimum with this simple technique.     

The Histopathologic, Pharmacologic and Urodynamic Results of Mesenchymal Stem Cell’s Injection into the Decompensated Rabbit’s Bladder

Objectives

We researched the survival of bone marrow-derived mesenchymal stem cells (MSCs) and the results of MSCs’ injected into decompensated bladders in a rabbit model.

Methods

Partial bladder neck obstruction (PBNO) and subsequent decompensation of the bladder was achieved by wrapping the bladder neck with autologous rectus fascia. In the first aspect of the experiment 18 rabbits underwent MSC injection into the decompensated bladder to prove the survivability of injected MSCs. For this purpose MSCs were isolated, transfected with Green Fluorescent Protein (GFP), and injected into the detrusor layer. Once viability was assessed in the first phase, an additional 10 rabbits underwent PBNO in the second phase. Five of these animals underwent subsequent MSC injection (group 3, stem cell) and 5 did not (group 2, obstruction). Both groups were compared to 5 controls (group 1). Urodynamics were performed in all groups. After the animals were sacrificed the groups were compared via morphometric analysis, contractile response to carbachol and KCl, and muscarinic receptor type analysis.

Results

On morphometric analysis, collagenous area rates were 43, 53 and 37 % in group 1, 2 and 3, respectively. There was no statistically significant difference between groups in terms of bladder weight, bladder capacity and vesical pressure. The contractile effects of KCl and muscarinic agonist carbachol were significantly higher in groups 1 and 3 than group 2. The response to carbachol was antagonized by muscarinic M₁ and M₃ receptor antagonist pirenzepine and abolished by muscarinic M₃ receptor antagonist 4-DAMP in all groups.

Conclusions

The injection of MSCs decreased the collagenous area, increased detrusor contractility. Functional M₃ receptors were also expressed in MSCs-injected bladder smooth muscle as well as in control group.     

Whole-genome array CGH identifies pathogenic copy number variations in fetuses with major malformations and a normal karyotype

Despite a wide range of clinical tools, the etiology of mental retardation and multiple congenital malformations remains unknown for many patients. Array-based comparative genomic hybridization (aCGH) has proven to be a valuable tool in these cases, as its pangenomic coverage allows the identification of chromosomal aberrations that are undetectable by other genetic methods targeting specific genomic regions. Therefore, aCGH is increasingly used in clinical genetics, both in the postnatal and the prenatal settings. While the diagnostic yield in the postnatal population has been established at 10-12%, studies investigating fetuses have reported variable results. We used whole-genome aCGH to investigate fetuses presenting at least one major malformation detected on ultrasound, but for whom standard genetic analyses (including karyotype) failed to provide a diagnosis. We identified a clinically significant chromosomal aberration in 8.2% of tested fetuses (4/49), and a result of unclear clinical significance in 12.2% of tested fetuses (6/49). Our results document the value of whole-genome aCGH as a prenatal diagnostic tool and highlight the interpretation difficulties associated with copy number variations of unclear significance.     

Catamenial hemoptysis

Catamenial hemoptysis is a rare condition that is associated with the presence of intrapulmonary or endobronchial endometrial tissue. We describe a case of endobronchial endometriosis with catamenial hemoptysis. The patient was a 22 years-old girl presented with recurrent hemoptysis episodes for the last two years. Bronchoscopic examination was performed within first days of menses, and indicated multiple purplish-red submucosal patches in distal one third of trachea and bilateral bronchial trees that bled easily when touched. The cytological evaluation of the bronchial brushing specimens demonstrated clusters of small cuboidal cells consistent with an endometrial origin. Follow-up bronchoscopic examination at the end of the menstrual cycle revealed that the previous tracheobronchial lesions disappeared. The patient was treated with Gonadotropin-Releasing Hormone (GnRH) analogues and hormones including estrogen and progesterone therapy. Recurrent hemoptysis stopped following the medical treatment.     

Evaluation of urinary inverted papillomas: a report of 13 cases and literature review

Inverted papilloma (IP) of the urothelium accounts for 2.2% of urothelial neoplasms. The aim of this study was to report the results of 13 patients with urinary IP, pointing out the clinical features, presentations, treatment options and outcomes. The mean age and mean follow-up periods of the patients were 60.23 +/- 3.25 (range, 44-81) years and 30 (range, 6-42) months, respectively. There was no coexistence of urothelial carcinoma with IP at presentation. Cystoscopy showed a solitary papillary tumor in the bladders of 11 patients and solid pedunculated tumors in the remaining two patients. The site of development was the bladder in 12 cases (92%) and ureter in one (8%) case. Transurethral bladder tumor resection was performed in 12 cases. For the case with IP in the ureter, nephroureterectomy was performed. Pathologic examination demonstrated that seven of the 13 cases were of the trabecular type and six were of the glandular type. Of the 13 cases, two (7%) had recurrence, at 1 year and 1 month and 1 year and 5 months from initial resection. The male to female ratio was 5.5:1. Initial symptoms included macroscopic hematuria in five cases, microscopic hematuria in four, and dysuria and microscopic hematuria in three; one case was asymptomatic. IPs of the urinary bladder are benign tumors that can be treated successfully by transurethral resection and fulguration of the tumor bed. In addition, these lesions must be followed up closely for recurrence and malignant transformation.     

Pulmonary arterial hypertension in a child with stage‐IV neuroblastoma after autologous hematopoietic stem cell transplantation and review of the literature

PH is a rare condition with high mortality rate after pediatric HSCT. As clinical presentation is non‐specific and may mimic other conditions, a high degree of suspicion is required for diagnosis. Here, we present a patient with stage‐IV neuroblastoma who developed PAH after autologous HSCT. After exclusion of other causes of PH, we regarded that this condition was secondary to HSCT.     

Evaluation of Cardiac Functions of Patients with Benign Joint Hypermobility Syndrome

We sought to investigate whether echocardiography with tissue Doppler imaging identifies myocardial dysfunction in children with benign joint hypermobility syndrome (BJHS). This cross-sectional study enrolled 75 children with BJHS and 70 healthy children. We performed detailed echocardiography in individuals with BJHS without inherited connective tissue disorders. Any congenital or acquired cardiac disease was excluded by clinical and echocardiographic examination. Both groups were similar in terms of age, sex, and body mass index. The diameter of the aortic annulus and sinus valsalva were wider in patients with BJHS. There was no significant differences in ejection fraction or mitral and tricuspid annular plane systolic excursion between the two groups. Pulsed-wave Doppler-derived E/A ratios in mitral and tricuspid valves were similar in both groups. Deceleration time of early mitral inflow was prolonged in patients with BJHS. Mitral and tricuspid annulus Ea velocity were significantly lower in children with BJHS. Ea, Aa, and Ea/Aa ratios in the interventricular septum, left ventricle posterior wall, and right ventricle free wall were lower in patients with BJHS than in the control group. The E/Ea ratio was greater in patients with BJHS than in the control group. Isovolumic relaxation time and right-ventricular (RV) and left-ventricular (LV) myocardial performance indices (MPIs) were greater in patients with BJHS. This study showed the diastolic dysfunction in patients with BJHS. In addition, we detected increased LV and RV MPI. We believe that BJHS may affect proteins of the myocardial cytoskeleton and extracellular matrix.     

Isolated metastasis of uterine leiomyosarcoma to the pancreas: Report of a case and review of the literature

INTRODUCTION

Metastatic tumors of the pancreas are uncommon and rarely detectable clinically. Metastases to the pancreas are rare. We present a patient with pancreatic metastases from a leiomyosarcoma of the uterus and review the literature about the clinical features of pancreatic metastasis and its surgical management.

PRESENTATION OF CASE

A 40-year-old woman, who underwent hysterectomy, left oophorectomy, omentectomy and lymp node dissection for leiomyosarcoma of the uterus. At the follow up, the patient complained of non-specific abdominal discomfort. Preoperative diagnosis were pancreatic pseudocyst, cystadenoma or cystadenocarcinoma. At laparotomy, a cystic mass was found in the tail of the pancreas which was invased to the transverse colon mesenterium and the spleen. Distal pancreatectomy with splenectomy and transverse colon resection was performed. Histologically, the tumor was evaluated as poorly differentiated leiomyosarcoma.

DISCUSSION

Metastatic lesions of the pancreas are uncommon and less than 2% of all pancreatic malignancies. However a few cases of leiomyosarcoma with metastases to the pancreas have been reported in the literature. Before deciding that the lesion in the pancreas was metastasis, primary leiomyosarcoma of the pancreas had to be ruled out. Histologically, leiomyosarcoma of the pancreas contains interlacing spindle cells with varying degrees of atypia and pleomorphism. The surgical approach to the pancreatic metastases must be aimed complete excision of the tumor with a wide negative margin of clear tissue and maximum preservation of pancreatic remnant if possible.

CONCLUSION

In the absence of widespread metastatic disease, aggressive surgical approach with negative margins must be aimed.