Citations

Objective To determine if massive renal size should be a contraindication for attempting a laparoscopic approach to bilateral native nephrectomies in patients with autosomal dominant polycystic kidney disease ( ADPKD ). Patients and Methods We retrospectively reviewed all laparoscopic bilateral nephrectomies performed for ADPKD at our institution from 1 J anuary 2000 to 31 D ecember 2012. We stratified patients by kidney weight (with or without at least one kidney weighing >2500 g) and compared perioperative data, complications, and status of kidney allografts. Additionally, the subset of patients with at least one kidney weighing >3500 g was compared with the rest of the cohort. Results We identified 68 patients; mean (range) individual kidney weight was 1984 (197–5042) g. In all, 24 patients had at least one kidney weighing >2500 g, yet patients in this group were not significantly different from the rest of the cohort for complications, estimated blood loss, transfusion rate, or duration of hospitalisation. For those who underwent simultaneous renal allotransplantation, native kidney size was not associated with graft outcomes. Additionally, of the six patients with at least one kidney weighing >3500 g, only one required a blood transfusion, and the group had no intraoperative or postoperative Clavien grade ≥3 complications. None of the cohort required conversion to open surgery. Conclusion Massive size of polycystic kidneys is not a contraindication to attempting a laparoscopic approach to bilateral nephrectomies in an experienced, high‐volume centre.

Key Points ALK-negative ALCLs have chromosomal rearrangements of DUSP22 or TP63 in 30% and 8% of cases, respectively. DUSP22-rearranged cases have favorable outcomes similar to ALK-positive ALCLs, whereas other genetic subtypes have inferior outcomes.

Abstract Myopericytomas belong to a benign group of tumors consisting of perivascular myopericytes or pericytes with a characteristic myoid differentiation pattern. The tumor is histologically and radiographically heterogenous, similar to superficial myofibromas. We report on a case of a myopericytoma arising in the hypothenar eminence of a 58-year-old man with a history of over 15 years of ulnar-sided and midcarpal wrist pain. Decision was made to excise the mass after it had increased in size by over 50 % over a 10-year period. Excision of the mass led to remarkable relief of his symptoms in the hypothenar as well as midcarpal regions. Consideration of this rare perivascular tumor should be included in patients with slow-growing benign appearing masses within the subcutaneous tissues of the hand.

Background Shoulder arthroscopy is not common in the pediatric and adolescent population, but the frequency may be on the rise. The purpose of the study was to determine the incidence of acute complications of arthroscopic shoulder surgery in children and adolescents. Methods A retrospective, cross-sectional review was performed identifying patients aged 18 years or less who underwent an arthroscopic shoulder procedure from 1997 to 2009 at Institution 1 and 2007 to 2010 at Institution 2. Exclusion criteria included open procedures and missing records. Demographic and surgical data were collected, including intra-operative and post-operative complications during the first 6 months. The complications were divided into minor (no secondary treatment) and major (secondary treatment rendered). Results Two hundred children, mean age 15.9 years, met criteria and 73 % were boys. All procedures were performed under general anesthesia, but 51 % included inter-scalene regional anesthesia. There were 16 (8.0 %) total complications recorded. Major complications occurred in five (2.5 %) patients, including two tendinitis/bursitis requiring injections, one broken pain pump catheter requiring an accessory incision to retrieve, one pain control readmission, and one laceration of the cephalic vein requiring ligation. Minor complications occurred in 11 (5.5 %) patients, including allergic reactions, transient dysesthesias, headaches, bronchitis, syncope, transient hypotension, and uvula swelling. Conclusion Although we found no seriously deleterious outcomes, it is important to recognize that an additional service was rendered for 2.5 % of children undergoing shoulder arthroscopy. The events that did occur may be preventable and this study should serve as a baseline to improve quality and safety of shoulder arthroscopy in the pediatric population.

Hartsfield syndrome has been recently reported to be associated with mutations in FGFR1 however, to this date; no familial cases have been reported. In this report, we describe two siblings with Hartsfield syndrome and a novel de novo FGFR1 mutation suggesting gonadal mosaicism. The proband presented at our institution at age 6 years with a clinical diagnosis of Hartsfield syndrome and requesting further genetic evaluation. Previous studies included a normal karyotype, oligonucleotide array, and single gene testing for nonsyndromic holoprosencephaly (SHH, SIX3, ZIC2, TGIF). At the age of 6 years, exome sequencing was performed and a de novo novel missense variant was identified in FGFR1 (coding for fibroblast growth factor‐1) on chromosome 8p12: c.1880G>C (p.R627T). Subsequently, a younger sibling was born with the same phenotype (holoprosencephaly, ectrodactyly of bilateral hands and feet and bilateral cleft lip and palate). Targeted sequencing of FGFR1 revealed the identical variant that was previously identified in the proband. To our knowledge this observation is the first documentation of familial recurrence of Hartsfield syndrome. As both parents were negative for the sequence variant in FGFR1 gene by testing peripheral blood samples, this suggests gonadal mosaicism. The frequency of gonadal mosaicism in Hartsfield syndrome is not known however given our case, this possibility should be taken in to consideration for recurrence risk estimation in children of clinically unaffected parents. © 2014 Wiley Periodicals, Inc.

AbstractBackgroundThe aim of the study was to assess which aspects of an enhanced recovery programme are associated with better outcomes following laparoscopic colorectal surgery.MethodsA database of laparoscopic colorectal procedures performed in 2011 was reviewed. Elements of the enhanced recovery programme and compliance were evaluated for short-term (30-day) outcomes. Individual elements included gabapentin, celecoxib, intrathecal analgesia, diet, postoperative fluids, and paracetamol/non-steroidal anti-inflammatory drug pain management.ResultsFive hundred and forty-one consecutive procedures were included. Compliance with the enhanced recovery programme elements ranged from 82·4 to 99·3 per cent. Median length of hospital stay was 3 (i.q.r. 2–5) days, with 25·9 per cent of patients discharged within 48 h. Patients without complications had a median length of stay of 3 (i.q.r. 2–4) days if compliant and 3 (3–5) days if not (P < 0·001). Low oral opiate intake (oral morphine equivalent of less than 30 mg) (odds ratio (OR) 1·97, 95 per cent confidence interval 1·29 to 3·03; P = 0·002), full compliance (OR 2·36, 1·42 to 3·90; P < 0·001) and high surgeon volume (more than 100 cases per year) (OR 1·50, 1·19 to 1·89; P < 0·001) were associated with discharge within 48 h. Compliance with the elements of oral intake and fluid management in the first 48 h was associated with a reduced rate of complications (8·1 versus 19·6 per cent; P = 0·001). Median oral opiate intake was 37·5 (i.q.r. 0–105) mg in 48 h, with 26·2 per cent of patients receiving no opiates.ConclusionCompliance with an enhanced recovery pathway was associated with less opiate use, fewer complications and a shorter hospital stay.

Abstract Regorafenib is an oral multikinase inhibitor that inhibits several kinases relevant to tumor biology in several cancers, including colorectal carcinoma (CRC) and gastrointestinal stromal tumor (GIST). In phase III trials, regorafenib significantly improved overall survival versus placebo in patients with metastatic CRC progressing after all available standard therapies, and significantly prolonged progression-free survival in patients with advanced GIST in whom at least imatinib and sunitinib had failed. Thus, this agent holds promise as a new standard of care for CRC and GIST patients after disease progression following all other approved therapies. The clinical trials reported to date show that this new treatment has a consistent adverse event profile that is quite different from that of traditional cytotoxic chemotherapies. The most common adverse events of regorafenib include dermatologic and mucosal toxicities (especially hand-foot skin reaction, rash, and oral mucositis), constitutional symptoms (e.g., fatigue, nausea, and weight loss), vascular effects (especially hypertension), and gastrointestinal symptoms (e.g., diarrhea). To help health care professionals anticipate and manage the adverse events associated with regorafenib, we describe our experiences in clinical trials and show that such toxicities can be effectively managed with close observation of patients from initiation of dosing, along with prompt appropriate interventions, including dose modifications, if necessary.

ObjectiveTo examine trends in glucocorticoid (GC) use and dosing among patients diagnosed with rheumatoid arthritis (RA) over time.MethodsA population‐based inception cohort of RA patients diagnosed during 1980–2007 was followed longitudinally through their medical records until death, migration, or December 31, 2008. GC start and stop dates were collected, along with doses in prednisone equivalents.ResultsThe study population comprised 349 patients (68% women) diagnosed in 1980–1994 and 464 (69% women) diagnosed in 1995–2007, with a median followup of 15.3 and 5.7 years, respectively. A higher proportion of patients started GCs in their first year of disease in 1995–2007 (68% versus 36%; P < 0.001), but the starting dose (mean 8.7 versus 10.3 mg; P = 0.08) and cumulative dose in the first year of use (mean 1.8g [mean daily dose 4.9 mg] versus 2.1 gm [mean daily dose 5.8 mg]; P = 0.48) were not different. A higher proportion also discontinued GCs in their first year of disease in the 1995–2007 cohort (P < 0.001). These differences in GC initiation and discontinuation persisted throughout followup. Prevalence of GC use was higher in the 1995–2007 cohort for the first 3 years of disease.ConclusionMore patients are starting GCs early in their disease course now compared to previously, which is consistent with established treatment guidelines. A higher proportion are also discontinuing GCs, but the proportion of patients taking GCs at any given point of disease during the first 4 years is higher now than previously. Despite early addition of a disease‐modifying antirheumatic drug, some patients may not be able to discontinue GCs over the long term.