Kidney transplantation in a child with Pierson syndrome.

Congenital nephrotic syndrome is commonly associated with mutations in genes that encode podocyte and slit diaphragm proteins or the structural and regulatory proteins of the GBM. These mutations lead to the formation of dysfunctional proteins, which account for the resistance of the renal manifestations to conventional treatment methods. Consequently, patients become renal replacement therapy dependent. Mutation of the LAMB2 gene is associated with Pierson syndrome, which is an autosomal recessive disorder characterized by congenital nephrotic syndrome and ocular abnormalities. In this report, a 2-year-old male patient who was diagnosed with Pierson syndrome is presented. He had bilateral microcoria and developmental delay in addition to nephrotic syndrome. His renal function deteriorated rapidly, and he underwent a deceased donor kidney transplantation. He showed dramatic improvement after kidney transplantation; in addition to having good renal function, he started to catch up to his peers in terms of growth. Pierson syndrome should be considered during the diagnostic investigations of children with renal manifestations and ocular abnormalities. Children with Pierson syndrome must be evaluated in terms of kidney transplantation as soon as they are diagnosed.

Parastomal skin metastasis in a patient with history of urinary bladder cancer.

Transitional cell cancer of the urinary bladder very rarely metastasize to the skin. Herein, we present a patient with urinary bladder cancer who underwent radical cystoprostatectomy and ileal conduit surgery. During follow-up, he developed lung and skin metastases. However, the latter presented as a solitary sessile polypoid lesion at the parastomal area.

An unusual cause of small bowel perforation: apricot pit.

Ingestion of foreign bodies can be a common problem, especially among children, alcoholics, and psychiatric and senile patients. Foreign bodies with smooth edges usually do not pose significant problems, but a sharp foreign object that is not retrieved immediately may penetrate the wall and cause complications. Ingested foreign bodies usually pass the intestinal tract uneventfully, and perforation occurs in less than 1%. In this study, we report a case of small bowel obstruction with perforation in a 73-year-old female due to the accidental swallowing of an apricot pit.

Prosthetic repair of acutely incarcerated groin hernias: a prospective clinical observational cohort study.

BACKGROUND AND AIM: Mesh usage in repair of acutely incarcerated hernia is still a concern owing to infectious complications. The aim of this prospective clinical observational cohort study was to evaluate factors that increase the risk of bowel necrosis and to document the clinical outcome of the patients with acutely incarcerated groin hernias treated by non-absorbable mesh. MATERIALS AND METHODS: Ninety-five adult patients with acutely incarcerated groin hernias who underwent prosthetic herniorrhaphy from 1997 to 2005 were prospectively included. The patients were evaluated in two groups, which were based on whether the bowel resection was required (group 1) or not (group 2). Demographics and characteristics of patients in each group were compared. Chi-square, Fisher's exact, and Mann-Whitney U test were used to determine the statistical significance (p < 0.05). RESULTS: Bowel resection was required in 14 (14.7%) and not required in 81 (85.3%) patients. Duration of symptoms longer than 6 h was an important factor for determining the need for resection (p = 0.026). No significant difference was noted concerning the development of wound infection, postoperative recurrence, morbidity, and mortality rates between the two groups. CONCLUSIONS: Our results suggest that duration of symptoms longer than 6 h was an important factor for determining the need for resection. The use of non-absorbable mesh for acutely incarcerated groin hernia repair is effective and may be used with an acceptable incidence of wound infection and recurrence even when intestinal necrosis was present.

Benign solitary cecal ulcer: a condition that mimics plastron appendicitis.

The benign solitary cecal ulcer is a rare clinical entity that is not usually included in the differential diagnosis of the cecal diseases. The etiology is unknown, and there are no pathognomonic lesions or symptoms. Pre-operative and intra-operative diagnosis is difficult. Definitive diagnosis is generally obtained by histologic evaluation of the surgical specimen after a right hemicolectomy performed for a suspected neoplasm of the cecum. We herein describe a 70-year-old woman with solitary cecal ulcer presenting with abdominal pain, palpable mass on the right lower quadrant and leukocytosis, mimicking plastron appendicitis on initial evaluation.

What is the role of the abdominal perfusion pressure for subclinical hepatic dysfunction in laparoscopic cholecystectomy?

BACKGROUND: Subclinical hepatic dysfunction after laparoscopic cholecystectomy (LC) has been described in the literature. However, this alteration is not encountered in all patients. In order to address this situation, a prospective study was conducted to investigate the effect of abdominal perfusion pressure (APP) on liver function tests after LC performed under constant intra-abdominal pressure (IAP). PATIENTS AND METHODS: Of 78 patients who underwent LC between May 2007 and October 2007, 40 patients were eligible for the study. In all the patients, six parameters of liver function (aspartate aminotransferase, alanine aminotransferase, direct bilirubin, indirect bilirubin, alkaline phosphatase, and gamma-glutamyltransferase) were assessed before and 24 hours after surgery. Patients who showed more than a 100% increase in at least one parameter (group 1) were compared to those who did not (group 2) regarding age, sex, body weight, body height, operation time, pneumoperitoneum time, IAP, preoperative, and intraoperative APP. RESULTS: Of the patients, 37.5% showed more than a 100% increase in at least one parameter of liver function. No significant difference was found between the two groups with regard to age, sex, body weight, body height, operation time, pneumoperitoneum time, and IAP. There were significant increase in AST and ALT at 24 hours postoperatively in group 1, as compared with group 2 (P = 0.000, P = 0.001). In comparison of preoperative APP with intraoperative APP values, group 1 showed a statistically significant decrease (P = 0.000), while no difference was found in group 2. CONCLUSION: Subclinical hepatic dysfunction after LC could mostly be attributed to the negative effects of the pneumoperitoneum on hepatic blood flow. For the evaluation of hepatic hypoperfusion, APP may be a new criterion as a determinant of interaction with mean arterial pressure (MAP) and IAP.

Surgical Drains Do Not Decrease Complication Rates But Are Associated with a Reduced Need for Imaging After Kidney Transplant Surgery.

BACKGROUND: Perigraft collections and wound complications are common after kidney transplantation. The aim of this study was to determine whether intraoperative drain placement had an effect on the risk of these complications. MATERIAL AND METHODS: Adult patients who underwent kidney transplantation in our center between January 2006 and December 2014 were included. Information regarding absence/presence of drain, imaging studies, and complications (perigraft collection and wound complications) were collected. The effect of drains on outcomes was analyzed using logistic regression after adjustment for baseline characteristics. RESULTS: Baseline characteristics were similar for 'drain' (n=374) and 'no drain' (n=283) groups. Forty-eight percent (n=317) of the patients were imaged. Fewer patients with a drain (40%) were imaged to diagnose a perigraft collection compared to those without a drain (60%, p<0.001). Perigraft collections and wound complications were detected in 28% (n=186) and 14% (n=90) of the cohort, respectively. Presence of a drain was associated with a significantly lower rate of perigraft collections (odds ratio 0.62, 95% CI [0.43-0.88], p=0.011). However, risk of wound complications was similar for those with a drain versus without a drain (odds ratio 0.67, 95% CI 0.42-1.07, p=0.096). Among the 225 patients with a complication, the subsequent intervention rate was the same for those with or without a drain (adjusted odds ratio 1.23, 95% CI 0.61-2.46. p=0.562). CONCLUSIONS: Drain placement is not associated with a significant reduction in wound complications following kidney transplant and does not reduce the risk of clinically significant perigraft collections. Since it is associated with reduced need for imaging to diagnose collections, it has the potential to reduce transplant costs.

Effects of mTOR Inhibitors in Prevention of Abdominal Adhesions.

PURPOSE OF THE STUDY: Postsurgical adhesions can occur after laparotomy and can cause morbidity. Local delivery of sirolimus prevented adhesion formation in various experiments. We analyzed the impact of orally dosed mammalian target of rapamycin inhibitors on abdominal adhesion formation and wound tensile strength in an experimental model. MATERIALS AND METHODS: Wistar albino rats were divided into sirolimus, everolimus, and control groups (n = 6 per group). Experimental animals underwent midline laparotomy and adhesion induction procedure which included cecum abrasion and mesh implantation. Animals were administered oral sirolimus (4 mg/kg), everolimus (3 mg/kg), or placebo starting on postoperative day 1. Treatments were given until postoperative day 7. At postoperative day 21, adhesions were scored. Meshes were resected with the attached abdominal wall and cecal segment and stained with Sirius red for collagen density analysis. Midline scars were excised for tensile strength measurement. Effects of sirolimus and everolimus on fibroblast proliferation were also assessed. RESULTS: Mean adhesion score of the everolimus group (7.83 ± 1.17) was significantly lower compared to sirolimus (11.00 ± 0.63) and control (11.66 ± 0.51) groups. Mean collagen density of the everolimus group (33.5 ± 7.8) was significantly lower compared to sirolimus (50.7 ± 9.69) and control (53.8 ± 12.4) groups. Mean tensile strength of the control group (26.41 ± 2.10) was significantly higher compared to sirolimus (17.89 ± 1.9) and everolimus (21.37 ± 1.25) groups. It was significantly lower in sirolimus group than everolimus group. Both sirolimus and everolimus treated media inhibited fibroblast proliferation significantly compared to media alone. CONCLUSIONS: Everolimus effectively reduced adhesions. Nevertheless, it also reduced wound tensile strength: an effect which seemed to be due to inhibition of fibroblast proliferation.

Gastrointestinal stromal tumour in a recipient with kidney transplantation.

Immunosuppression is associated with an increased risk of post-transplant malignancies. Gastrointestinal stromal tumours are the most common mesenchymal tumours of the gastrointestinal tract. However, they have seldom been reported recipient with transplantation. In this report, a 46-year-old woman, a recipient with kidney transplantation, who developed a gastric tumour is presented. Removal of this tumour required a partial gastrectomy. Histopathology and immunohistochemistry examinations revealed a high-risk gastric stromal tumour. Adjuvant imatinib mesylate treatment was initiated. There was no evidence of tumour recurrence at 12-month follow-up.

Kidney transplantation in an adult patient with VACTERL association.

The vertebral, anal, cardiac, tracheoesophageal, renal, and limb birth defects (VACTERL) association is a rare, non-random constellation of congenital abnormalities among which urinary tract anomalies can be included. In the presence of these anomalies, patients are suspected to have a higher rate of renal failure than average. We report a case of a 22-year-old woman with VACTERL association and consequent end stage renal failure. A live-related kidney transplant was carried out successfully and the postoperative course was uncomplicated. The patient had immediate graft function. Risk factors that may complicate kidney transplant surgery in this patient population as well as considerations relevant to peritransplant management are discussed.

Advances in loco-regional palliation of unresectable cholangiocarcinomas.

Cholangiocarcinoma (CC) represents the second most common primary hepatic malignancy after hepatocellular carcinoma, accounting for 10% to 15% of primary liver tumors. Its prognosis is often poor due to its late presentation and its relative resistance to current chemotherapy and radiotherapy regimens. At the time of diagnosis, more than half of the patients are not candidates for surgical therapy due to either invasion of vascular structures or distant metastases. Therefore, for a large proportion of patients, palliation remains the only option for better quality of life and, in some circumstances, longer survival. The main goals of palliative interventions for CC are: restoration of the biliary drainage and resolution of pruritis caused by obstructive jaundice, improvement of the nutritional status and reduction of pain. During the last decades, novel therapies have been introduced to optimize the management of patients with unresectable CC. These therapies can be resource intensive and have some adverse effects; therefore patient selection is crucial. This article discusses the current options for palliation of CC.

Implications of Hyponatremia in Liver Transplantation.

Although there are a limited number of quality studies, appropriate peri-operative management of serum electrolytes seems to reduce adverse outcomes in liver transplantation. Hyponatremia is defined as the presence of serum concentration of sodium equal ≤130 mmol/L and it is detected in approximately 20% of patients with end stage liver disease waiting for a liver transplant (LT). This paper will focus on the pathogenesis of dilutional hyponatremia and its significance in terms of both candidacy for LT and post-operative outcomes.