Late-Onset Subcutaneous Scalp Calcifications in a Patient with Pseudo-Pseudohypoparathyroidism

Ectopic calcifications and even bone formation have been linked to GNAS gene mutations. A 51-year-old Caucasian female had been diagnosed of pseudo-pseudohypoparathyroidism (PPHP) in 1989. She has always had normal serum parathyroid hormone, calcium, and phosphorus levels. A non-contrast computed tomography of the head was done in 2013 and it showed finely speckled subcutaneous calcifications in the high convexity of the head. Cutaneous exploration did not show any abnormality. We herein report an unusual case of late-onset scalp calcifications in a patient with PPHP.     

A systematic review of the literature to support an evidence-based Precepting Program

Aim

To provide a systematic review of the literature regarding development of an evidence-based Precepting Program for nurses transitioning to burn specialty practice.

Background

Burned patients are admitted to specialty Burn Centers where highly complex nursing care is provided. Successful orientation and integration into such a specialized work environment is a fundamental component of a nurse's ability to provide safe and holistic patient care.

Design

A systematic review of the literature was performed for the period 1995–2011 using electronic databases within PUBMED and Ovid search engines.

Data sources

Databases included Medline, CINHAL, ProQuest for Dissertations and Thesis, and Cochran Collaboration using key search terms: preceptor, preceptee, preceptorship, precept*, nurs*, critical care, personality types, competency-based education, and learning styles.

Review methods

Nurses graded the level and quality of evidence of the included articles using a modified 7-level rating system and the Johns Hopkins Nursing Quality of Evidence Appraisal during journal-club meetings.

Results

A total of 43 articles related to competency (n = 8), knowledge acquisition and personality characteristics (n = 8), learning style (n = 5), preceptor development (n = 7), and Precepting Programs (n = 14).

Conclusions

A significant clinical gap existed between the scientific evidence and actual precepting practice of experienced nurses at the Burn Center. Based on this extensive review of the literature, it was determined that a sufficient evidence base existed for development of an evidence-based Precepting Program.     

Health-related quality of life (HRQoL) in cystine compared with non-cystine stone formers

Cystinuria is a genetic cause of recurrent kidney stones which may be more recurrent and larger than more common non-cystine stones. They may have a greater impact on health-related quality of life (HRQoL). We measured this impact by surveying HRQoL in patients with stones, comparing non-cystine stone formers (NCSF) to cystine stone formers (CYSF) and both groups to normative values of the US population. We used SF-36v2 via an internet instrument. CYSF patients were recruited via cystinuria-related websites, two patient advocacy groups, and an active endourology practice. NCSF patients were recruited from the same practice and by email. Total n surveyed with scorable data: 214 CYSF and 81 NCSF. The participants included 128 men and 161 women. The group of CYSF were significantly younger (39 vs. 54 years) and suffered longer from kidney stones (255 vs. 136 months). CYSF patients had significantly more episodes of stones than NCSF patients in the last year (N = 108 CYSF, N = 20 NCSF). More frequent stones in the last year and mental comorbidities most often predicted worse scores in the individual HRQoL domains. However, cystine stone composition was a significant predictor of worse scores only for role emotional. Better scores in all SF-36 domains were associated with greater time since the last kidney stone event. Although kidney stones are often transient, kidney stone formers, regardless of stone composition, have a worse HRQoL than the standard US population, which has a normative score of 50, such as general health (41.2 ± 12.8), bodily pain (46.5 ± 11.8) and mental health (45.1 ± 12.6). CYSF are more frequent and severe stone formers compared with NCSF with a resulting greater, direct impact on the HRQoL of CYSF patients. Whether preventive strategies for cystinuria are being properly utilized by practitioners, and which strategies are most effective, should be established.     

Electrochemotherapy Treatment of Locally Advanced and Metastatic Soft Tissue Sarcomas: Results of a Non-Comparative Phase II Study

Aims

Our aim was to evaluate the activity, toxicity, and feasibility of electrochemotherapy (ECT) in patients with soft-tissue sarcomas (STS).

Methods

A two-stage phase II trial was conducted between October 2006 and March 2012. Patients (N = 34) with locally advanced or metastatic STS, unsuitable for standard oncological treatments and with maximum 3-cm deep tumors, received an intravenous bolus of bleomycin (15,000 IU/m²), followed by tumor electroporation according to the European Standard Operating Procedures of ECT. Outcome measures included local response according to response evaluation criteria in solid tumors (RECIST), toxicity and tumor control. Feasibility measures included the accuracy of electrode placement and the intensity of electric current flowing in tumor tissue.

Results

Median tumor size was 4.0 cm (range 2–12). Objective response, assessed on 71 target lesions, was 92.2 % (complete 32.3, 95 % CI 28–64). A total of 15 patients received up to four cycles due to incomplete response, but re-treatment did not significantly improve outcome (p = 0.205). After a median follow-up of 19.3 months, 2-year local control rate was 72.5 %. Median time to local failure (N = 11 patients) was 5.1 months. Tumor response (p = 0.041) and control (p = 0.047) correlated with histological grading. Relevant toxicity consisted of G3 skin ulceration and soft tissue necrosis (35 and 23 % of patients, respectively), although this was manageable on an outpatient basis. The accuracy of electrode placement was 47.1 %, and the adequacy of electroporative current 85.3 %.

Conclusions

ECT may represent an active and safe treatment to achieve local control in advanced STS patients with symptomatic disease. Future research challenges include the improvement of electrode placement and voltage delivery together with the containment of soft tissue toxicity.     

Bone marrow derived stem cells in joint and bone diseases: a concise review

Stem cells have huge applications in the field of tissue engineering and regenerative medicine. Their use is currently not restricted to the life-threatening diseases but also extended to disorders involving the structural tissues, which may not jeopardize the patients’ life, but certainly influence their quality of life. In fact, a particularly popular line of research is represented by the regeneration of bone and cartilage tissues to treat various orthopaedic disorders. Most of these pioneering research lines that aim to create new treatments for diseases that currently have limited therapies are still in the bench of the researchers. However, in recent years, several clinical trials have been started with satisfactory and encouraging results. This article aims to review the concept of stem cells and their characterization in terms of site of residence, differentiation potential and therapeutic prospective. In fact, while only the bone marrow was initially considered as a “reservoir” of this cell population, later, adipose tissue and muscle tissue have provided a considerable amount of cells available for multiple differentiation. In reality, recently, the so-called “stem cell niche” was identified as the perivascular space, recognizing these cells as almost ubiquitous. In the field of bone and joint diseases, their potential to differentiate into multiple cell lines makes their application ideally immediate through three main modalities: (1) cells selected by withdrawal from bone marrow, subsequent culture in the laboratory, and ultimately transplant at the site of injury; (2) bone marrow aspirate, concentrated and directly implanted into the injury site; (3) systemic mobilization of stem cells and other bone marrow precursors by the use of growth factors. The use of this cell population in joint and bone disease will be addressed and discussed, analysing both the clinical outcomes but also the basic research background, which has justified their use for the treatment of bone, cartilage and meniscus tissues.     

Bochdalek hernia and repetitive pancreatitis in a 33 year old woman

INTRODUCTION

Bochdalek hernia presentation in adulthood is rare. The presentation in newborns is the most common, manifesting with data from respiratory failure secondary to pulmonary hypoplasia, requiring urgent surgical intervention with high morbidity and mortality.

PRESENTATION OF CASE

We present the case of a 33 year old woman admitted in the emergency room with severe abdominal pain in the left upper quadrant and disnea. After physical examination and laboratory test we diagnose mild acute pancreatitis. The patient haven’t colelitiasis by ulstrasound or any risk factor for pancreatitis. Initially she received medical treatment and was discharged after one week. After four weeks she presented the same symptoms in two different occasions, with severe and mild pancreatitis respectively. A computed tomography report a left posterolateral diafragmatic hernia. In spite of the rare association of pancreatitis and Bochdalek hernia, we realized it as the etiology until the second event and planned his surgery. We made a posterolateral torachotomy and diafragmatic plasty with a politetrafluoroetileno mesh and after a 6 months follow up she has coursed asymptomatic.

DISCUSSION

The high rate of complications in this type of hernia requires us to perform surgical treatment as the hernia is detected. In this case it is prudent medical treatment prior to surgical correction despite this being the origin of the pancreatitis, because the systemic inflammatory response added by the surgical act could result in a higher rate of complications if not performed at the appropriate time. There is no precise rule to determine the type of approach of choice in this type of hernia which thoracotomy or laparotomy may be used.

CONCLUSION

Bochdalek hernia is a rare find in adults who require treatment immediately after diagnosis because of the high risk of complications. When presented with data from pancreatitis is recommended to complete the medical treatment of pancreatitis before surgery to obtain the best results, unless it exist another abdominal complication.     

Peri-operative complications in pediatric and adolescent shoulder arthroscopy

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.