Noonan syndrome associated with central giant cell granuloma

We report a case of Noonan syndrome associated with central giant cell granuloma. The patient was a 101/2-year-old boy with the chief complaint of proptosis of the right eye. He also had various malformations such as short stature, webbed neck, pectus excavatum, cubitus valgus, pulmonary valve stenosis and patent foramen ovale, a characteristic face appearance and cryptorchidism and so on. Chromosome analysis showed a 46, XY karyotype. A computed tomographic scan and magnetic resonance imaging showed a mass originated from the lateral wall of the right maxillary sinus. The patient underwent Caldwell-Luc operation. Histological examination of the mass showed the characteristics of central giant cell granuloma. This case report describes a patient with the features of the recently described Noonan-like/multiple giant cell lesion syndrome.     

Outcome of acute intussusception in a regional paediatric centre

It has been suggested recently that acute intussusception should only be managed in a specialised paediatric centre. The aim of this study is to assess the incidence and outcome of acute intussusception in a comprehensive paediatric unit in Waterford Regional Hospital. It is a retrospective medical records study over a ten year period from June 1990 to June 2000. Our results showed that we had 24 cases giving an incidence of 2.4 cases per year. Contrast enema was performed in 23(95%) cases, successfully reduced in 14(61%) cases, unsuccessfully reduced in 7(30%). Surgical intervention was necessary in 7(30%) cases. Three (12.5%) cases were transferred to a specialised paediatric surgical centre. There were no perforation or death. We conclude from this study that management of acute intussusception can be successfully undertaken in a regional paediatric centre. It requires adequate throughput of cases and close co-operation between paediatrician, radiologist, anaesthetist, surgeons and of course a dedicated paediatric nursing staff.     

Does center or surgeon volume influence adoption of minimally invasive versus open pancreatoduodenectomy? A systematic review and meta-regression

BackgroundThere has been increasing uptake of minimally invasive pancreatoduodenectomy during the past decade, but it remains a highly specialized procedure as benefits over open pancreatoduodenectomy remain contentious. This study aimed to evaluate current evidence on minimally invasive pancreatoduodenectomy versus open pancreatoduodenectomy in terms of impact of center volume on outcomes.MethodsA systematic review of articles on comparative cohort and registry studies on minimally invasive pancreatoduodenectomy versus open pancreatoduodenectomy published until 31st December 2019 were identified, and meta-analyses were performed. Primary endpoints were International Study Group on Pancreatic Fistula grade B/C postoperative pancreatic fistula and 30-day mortality.ResultsAfter screening 7,390 studies, 43 comparative cohort studies (8,755 patients) with moderate methodological quality and 3 original registry studies (43,735 patients) were included. For the cohort studies, the median annual hospital minimally invasive pancreatoduodenectomy volume was 10. No significant differences were found in grade B/C postoperative pancreatic fistula (odds ratio: 0.98, 95% confidence interval: 0.78–1.23) or 30-day mortality (odds ratio: 1.14, 95% confidence interval: 0.65–2.01) between minimally invasive pancreatoduodenectomy when compared with open. No publication biases were present and meta-regression identified no confounding for grade B/C postoperative pancreatic fistula, center volume or 30-day mortality. Minimally invasive pancreatoduodenectomy was only strongly associated with significantly lower rates of postoperative pulmonary complications and surgical site infection, shorter length of stay, and significantly higher rates of R0 margin resections.ConclusionMinimally invasive pancreatoduodenectomy remains noninferior to open pancreatoduodenectomy for grade B/C postoperative pancreatic fistula but is strongly associated with significantly lower rates of postoperative pulmonary complications and surgical site infection. Minimally invasive pancreatoduodenectomy can be adopted safely with good outcomes irrespective of annual center resection volume.     

ASO Visual Abstract: The Landmark Series—Minimally Invasive Pancreatic Resection

Annals of Surgical Oncology -     

Free-radicals dependent and independent pathways of cddp-mediated cytotoxicity and apoptosis in ovarian tumor-cell lines

Cis-platinum (CDDP) is a chemotherapeutic drug widely used alone or in combination with other drugs in the treatment of cancer. However, many tumors become resistant to CDDP and the mechanisms of resistance are complex. The present study investigated the role of free radicals in CDDP-mediated cytotoxicity and apoptosis. Four human ovarian cancer cell lines were chosen for the study: two lines, 222 and A2780, are sensitive to CDDP and two lines, AD10 and C30 are resistant to CDDP. The importance of free radical formation was tested by the use of an inhibitor, 2,6 di-tert-butyl-4-methoxy phenol (BHA), that inhibits the production of free radicals and detoxifies free radicals. Cytotoxicity was assessed by the MTT assay and apoptosis assessed by flow cytometric analysis of DNA hypoploidy. Three of the 4 cell lines, 222, AD10, and C30, were completely inhibited by BHA in CDDP-mediated cytotoxicity. The CDDP-sensitive A2780 cell line, however, was not inhibited by BHA, even at high non-toxic concentrations of BHA. The DNA analysis for apoptosis paralleled the findings obtained in the cytotoxicity assay. In order to rule out that the A2780 cell line was not reactive to BHA, VP-16 mediated cytotoxicity was examined. All four cell lines were sensitive to VP-16 and all four were inhibited by BHA. In contrast, there was no detectable inhibition by BHA of actinomycin-D-mediated cytotoxicity in all four lines tested. Overall, the findings demonstrate that either free-radical dependent (CDDP, VP-16) or free radical-independent (CDDP, actinomycin-D) pathways of cytotoxicity and apoptosis are utilized by different drugs. Further, a single agent like CDDP can mediate killing by both a free radical-dependent and by a free-radical independent pathway. Therefore, new agents that are developed to reverse resistance by a particular drug must take into consideration alternative cytotoxic pathways mediated by the same drug.     

Lipoprotein levels in patients with pregnancy induced hypertension

The purpose of this prospective study was to investigate the levels of Lp(a), Apo(a), VLDL, LDL and HDL in 23 patients with pregnancy induced hypertension (PIH) and in 20 control. The Mann-Whitney U tests was used for comparisons between the two groups. Serum Lp(a) and Apo(a) levels were sigificantly raise in the PIH group (p < 0.05 andp < 0.05 respectively) and no significant correlations could be demonstrated for other lipoproteins.     

Relation between bone mineral content and clinical, hormonal and biochemical parameters in postmenopausal women

We studied factors related to bone mass after a natural or surgical menopause in 73 healthy women attending the menopause clinic of a university hospital. In the natural menopause group we found inverse correlations between bone mineral density (BMD) vs. menopausal duration; BMD vs. body mass index (BMI) and BMI vs. inorganic phosphate (Pi), borderline correlations between weight vs. thyroxin (T4) and weight vs. luteinising hormone (LH) and a positive correlation between androstenedione (D4A) vs. urinary calcium (Uca). In the surgical menopause group we found some negative correlations (BMD vs. menopausal duration, BMI vs. Pi; BMI vs. dehydroepiandrosterone sulphate (DS), weight vs. DS and cortisol vs. Uca) and some positive correlations (BMD vs. free testosterone (fT), BMD vs. calcium (Ca), and BMD vs. Uca). We concluded that the serum hormone levels we measured were not useful markers of current bone mineral status. Received: 3 January 1997 / Accepted: 3 January 1997