Erdheim-Chester disease and nuclear medicine imaging. A case report and brief review.

Erdheim-Chester disease (ECD) is a rare clonal myeloid neoplasm typically affecting adults over 50 years old, with bone lesions in almost all patients. The prognosis is poor in most cases if left untreated. Clinical manifestations are not specific, which hinders early diagnosis. The disease has distinct radiological features. However, three-phase bone scintigraphy exhibits the most typical pattern of all imaging modalities, which is the prominent strikingly symmetrical radiotracer uptake in the distal ends of the femurs and proximal and distal ends of the tibiae, sparing the epiphyses. We report a case of a 54-year-old female patient, presenting with atypical persistent knee joint pain. After an MRI scan, she underwent a three-phase bone scan, revealing the characteristic pattern, thus indicating a possible ECD diagnosis, which was eventually confirmed in biopsy material. Novel aspects of the pathophysiology and treatment of the disease, as well as a differential diagnosis from the perspective of an MSK radiologist and nuclear medicine physician, are also discussed.

Evaluating the long term effectiveness of a Mediterranean marine protected area to tackle the effects of invasive and range expanding herbivorous fish on rocky reefs.

Here we provide evidence, along an 8-year period time-series based on multifaceted data from a Mediterranean marine protected area (MPA), whether protection can tackle invasive and range expanding herbivore fishes, and their effects on the algal resource availability, taking into account the population trends of predatory fishes, fisheries catches of herbivore fishes and sea surface temperature (SST) through time. Our findings pointed out that an ineffective in restoring top-down control process MPA may facilitate, rather than alleviate, the sudden and enduring population burst of invasive and range-expanding herbivorous fishes at tipping points of abrupt change. This subsequently results in the deterioration of rocky reef habitats and the depletion of algal resources, with the tipping points of abrupt change for algal and herbivore fish species not overlapping chronologically. As sea temperature increases, ineffective or recently established MPAs may inadvertently facilitate the proliferation of invasive and range-expanding species, posing a significant challenge to management effectiveness and conservation objectives.

Serum angiotensin converting enzyme elevation in association with artificial stone silicosis.

BACKGROUND: Silicosis is a rapidly emerging major health concern for workers in the artificial stone benchtop industry. The association between serum angiotensin converting enzyme (sACE) levels and artificial stone silicosis is unknown. METHODS: We investigated 179 male workers (median age 40 years, interquartile range (IQR) 33-48 years) from the stone benchtop industry in Victoria, Australia. All had worked in an environment where dry processing of artificial stone had occurred and were registered with the Victorian Silica-associated Disease Registry between June 2019 and August 2020. Workers had undergone protocolised assessments including respiratory function testing, high resolution CT chest and blood tests panel, including sACE. FINDINGS: Sixty workers with artificial stone silicosis were identified and they had a higher median sACE level (64.1 U/L, IQR 51.5, 87.5), compared to 119 without silicosis (35.0 U/L, IQR 25.0, 47.0). Compared to those with a normal assessment, regression modelling noted significantly higher average differences in sACE levels for workers with lymphadenopathy alone (12.1 U/L, 95% confidence interval (CI): 1.3, 22.9), simple silicosis (28.7 U/L, 95% CI: 21.3, 36.0) and complicated silicosis (36.0 U/L, 95% CI 25.2, 46.9). There was a small negative association with gas transfer, but no associations with exposure duration or spirometry. CONCLUSION: sACE levels were noted to be higher in artificial stone workers with silicosis compared to those without disease and was highest in those with complicated silicosis. Longitudinal follow up is required to evaluate sACE as a prognostic biomarker for workers with this rapidly emerging occupational lung disease.

Impact of the Model for End-stage Liver Disease (MELD) score on liver transplantation in Greece.

INTRODUCTION: The impact of preoperative Model for End-stage Liver Disease (MELD) score in postoperative mortality remains unclear. The assumption that patients with a higher MELD score will have a higher mortality rate is not confirmed and studies are contradictory. AIM: The study of the clinical course of patients with a higher MELD score and its impact in immediate and later mortality in comparison with patients with a lower MELD score in the only liver transplantation center in Greece. METHOD: We retrospectively studied 71 patients who underwent orthotopic liver transplantation (OLT) in the time period between 1-1-2011 and 31-12-2013. The patients were divided into 2 groups: Group A with a MELD score ≥23 and Group B with a MELD score <23. RESULTS: In the patients with a MELD score ≥23 the duration of mechanical ventilation and length of stay in the ICU were prolonged (P = .013 and .009, respectively), the transfusions were more (P = .005), and the rates of ICU readmissions (22.5% vs 7.31%, P = .001) and infections (42.5% vs 17.5%, P = .011) were higher. Thirty-day mortality did not differ between the 2 groups (P = .420), but there was a statistically significant difference in morbidity and in 180-day mortality. CONCLUSION: The patients with a higher MELD score have more complex pathophysiology. This score seems to affect morbidity and late, but not early, mortality.

Comparison of RIFLE and AKIN criteria in the evaluation of the frequency of acute kidney injury in post-liver transplantation patients.

BACKGROUND: Acute renal dysfunction is presented quite often after orthotopic liver transplantation (LT), with a reported incidence of 12-64%. The "RIFLE" criteria were introduced in 2004 for the definition of acute kidney injury (AKI) in critically ill patients, and a revised definition was proposed in 2007 by the Acute Kidney Injury Network (AKIN), introducing the AKIN criteria. The aim of this study was to record the incidence of AKI in patients after LT by both classifications and to evaluate their prognostic value on mortality. METHODS: We retrospectively evaluated the records of patients with LT over 2 years (2011-2012) and recorded the incidence of AKI as defined by the RIFLE and AKIN criteria. Preoperative and admission severity of disease scores, duration of mechanical ventilation, intensive care unit length of stay, and 30- and 180-day survivals were also recorded. RESULTS: Seventy-one patients were included, with an average age of 51.78 ± 10.3 years. The incidence of AKI according to the RIFLE criteria was 39.43% (Risk, 12.7%; Injury, 12.7%; Failure, 14.1%), whereas according to the AKIN criteria it was 52.1% (stage I, 22.5%; stage II, 7%; stage II 22.55%). AKI, regardless of the classification used, was related to the Model for End-Stage Liver Disease score, the volume of transfusions, the duration of mechanical ventilation, and survival. The presence of AKI was related to higher mortality, which rose proportionally with the severity of AKI as defined by the stages of either the RIFLE or the AKIN criteria. CONCLUSIONS: AKI classifications according to the RIFLE and AKIN criteria are useful tools in the recognition and classification of the severity of renal dysfunction in patients after LT, because they are associated with higher mortality, which rises proportionally with the severity of renal disease.

Causes and incidence of renal replacement therapy application in orthotopic liver transplantation patients: our experience.

BACKGROUND: Renal dysfunction is commonly found in patients with end-stage liver disease, as well as after liver transplantation, and it often needs renal replacement therapy (RRT) with the application of continuous or intermittent methods. The aim of this study was the recording of the patients who underwent continuous venovenous hemodiafiltration (CVVHDF) in the early postoperative period, as well as the recording of mortality and risk factors. METHODS: We retrospectively examined 71 patients who underwent orthotopic liver transplantation during a 2-year period (2011-2012). We recorded the incidence of acute kidney injury (AKI), according to the Acute Kidney Injury Network criteria, and we distinguished 2 groups of patients. Group A included those who needed RRT during the 1st 7 days after transplantation, and group B included the patients who did not require RRT. The RRT method of choice was CVVHDF. RESULTS: In group A, the population of patients who presented AKI reached 52.1%, and 22.5% of the them underwent RRT. The mean Model for End-Stage Liver Disease and Acute Physiology and Chronic Health Evaluation scores were higher in group A, who also presented greater hemodynamic instability and more frequent infections and required more transfusions. The transfusion of >10 units of red blood cells was recognized as a risk factor for RRT (P = .024; odds ratio, 1.15; 95% confidence interval, 1.8-7.53). CONCLUSIONS: Patients who required RRT immediately after surgery had a significantly higher rate of mortality and it seemed that the number of transfusions played an important role in this.

The effect of hyponatremia on the outcome of patients after orthotopic liver transplantation.

Predicting the prognosis of cirrhotic patients is considered to achieve a fair allocation among patients awaiting orthotopic liver transplantation (OLT). Serum sodium (Na) concentrations are associated with reduced survival among patients with cirrhosis. The mortality risk of cirrhotic patients, as defined by the Model for End-Stage Liver Disease (MELD) score, is considered to be higher in hyponatremic patients. The aim of this study was to record complications and outcomes of severely hyponatremic patients after OLT. We retrospectively studied 75 recipients of OLT over the last 3 years. Hyponatremic patients showed lower 30-day and intensive care unit (ICU) survivals (P = .022 and .028, respectively), higher rates of neurological complications (P = .038), renal failure (P = .001), and prolonged duration of mechanical ventilation (P = .000) and ICU stay (P = .000). Severe hyponatremia was related to a greater risk for neurological and renal complications after OLT.

Acute kidney injury after orthotopic liver transplantation.

Acute kidney injury (AKI), one of the most frequent complications in the early period after liver transplantation, causes serious obstacles in the management of these patients affecting their outcomes. We studied retrospectively 79 subjects who underwent orthotopic liver transplantation (OLT). AKI was defined as an elevation of serum creatinine 1.5 times above baseline or an absolute serum creatinine level > 2 mg/dL. Our aim was to analyze the incidence, correlation with prior renal impairment, outcomes of AKI after OLT. Twenty-two patients (29.3%) developed AKI after OLT with 31.81% of the requiring renal replacement therapy. Among patients with AKI the duration of mechanical ventilation was prolonged (P = .001), length of stay in the intensive care unit was greater (P = .001), infections were more common (P = .016), and 30-day and 1-year mortality rates higher (P = .018). Logistic regression analysis showed post-OLT AKI to be an independent risk factor for 1-year mortality after OLT.

Female reproductive biology of the bluemouth Helicolenus dactylopterus dactylopterus: spawning and fecundity.

The bluemouth Helicolenus dactylopterus dactylopterus is a zygoparous species that spawns multiple batches of embryos enclosed within a gelatinous matrix. Oocyte development is asynchronous, and the recruitment of secondary growth oocytes occurs continuously during the developing phase, but stops before the start of the first spawning (i.e. fecundity is determinate). The number of developing oocytes can be estimated as a function of the total length of the fish, its ovary mass and its gonado-somatic index. Only at the onset of spawning, when potential fecundity is determined, does condition also have a significant effect. The low levels of atresia detected during most of the spawning season show that this mechanism does not substantially affect the process. There is variability both in the spawning interval (with a mean of 2 days) and in the number of embryos comprising every single batch (up to 37,000). Expected effect of fisheries on the reproductive traits of this deep-sea species is also discussed.

Use of mobile phones and changes in cognitive function in adolescents.

BACKGROUND: Several studies have investigated the impact of mobile phone exposure on cognitive function in adults. However, children and adolescents are of special interest due to their developing nervous systems. METHODS: Data were derived from the Australian Mobile Radiofrequency Phone Exposed Users' Study (MoRPhEUS) which comprised a baseline examination of year 7 students during 2005/2006 and a 1-year follow-up. Sociodemographic and exposure data were collected with a questionnaire. Cognitive functions were assessed with a computerised test battery and the Stroop Color-Word test. RESULTS: 236 students participated in both examinations. The proportion of mobile phone owners and the number of voice calls and short message services (SMS) per week increased from baseline to follow-up. Participants with more voice calls and SMS at baseline showed less reductions in response times over the 1-year period in various computerised tasks. Furthermore, those with increased voice calls and SMS exposure over the 1-year period showed changes in response time in a simple reaction and a working memory task. No associations were seen between mobile phone exposure and the Stroop test. CONCLUSIONS: We have observed that some changes in cognitive function, particularly in response time rather than accuracy, occurred with a latency period of 1 year and that some changes were associated with increased exposure. However, the increased exposure was mainly applied to those who had fewer voice calls and SMS at baseline, suggesting that these changes over time may relate to statistical regression to the mean, and not be the effect of mobile phone exposure.

Immunosuppressive therapy and clinical evolution in forty-nine patients with antineutrophil cytoplasmic antibody-associated glomerulonephritis.

Immunosuppressive therapy and clinical evolution were studied in 49 patients (29 females) with antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The mean age of patients at presentation was 55 years, and the mean (+/-SD) follow-up was 43 months (+/-33) (range, 3-140). Among the 49 patients, 10 had biopsy-proven Wegener's granulomatosis, 33 microscopic polyangiitis, 2 Churg-Strauss syndrome, and 4 idiopathic crescentic glomerulonephritis. IgG ANCA autoantibodies were detected in all patients. Induction therapy included pulses and oral administration of methylprednisolone (MP) with oral administration of cyclophosphamide (CP) and plasma exchange in patients with alveolar hemorrhage and serum creatinine (SCr) levels >/= 6 mg/dL. CP was converted to azathioprine (AZA) or mycophenolate mofetil (MMF) after 3-6 months of therapy. Low doses of MP with or without AZA or MMF were administered until the end of follow-up. Therapy institution resulted in remission of disease in all patients. The mean SCr levels decreased from 4.9 mg/dL (+/-2.5) at the onset of the disease to 2.8 mg/dL (+/-1.7) (P > 0.0001), and 3.2 mg/dL (+/-2.3) (P > 0.0001) after 3 and 6 months, respectively. At the end of follow-up, 17 (35%) patients progressed to end-stage renal disease after 34 months (+/-29) (range, 3-98), and 30 (61%) patients maintained sufficient renal function. Two patient deaths were attributed to immunosuppression. Patients with high SCr levels at diagnosis and severe interstitial fibrosis found in renal biopsy had poor renal outcome (P > 0.01 and P > 0.02, respectively). Induction therapy with MP and CP seems to be the regimen of choice in patients with ANCA-associated glomerulonephritis. Early diagnosis and therapy institution as well as long-term treatment lead to acceptable renal survival.

Management of bile duct injury during and after laparoscopic cholecystectomy.

BACKGROUND: Bile duct injury (BDI) is perhaps the most feared complication of laparoscopic cholecystectomy (LC). Proper management of iatrogenic BDI is mandatory to avoid immediate or later life-threatening sequelae. The results of surgery depend mainly on the type of injury, prompt detection of the injury, and timing of the surgery. METHODS: Twelve patients with BDI after LC were treated. Eight of them were referred to our institution for further treatment. The follow-up evaluation was focused on clinical outcome and biochemical analysis. RESULTS: Five of the patients had minor BDI with leakage. In all of them, the BDI was recognized postoperatively. Two of these patients were managed by endoscopic retrograde cholongio pancreatographic sphincterotomy and stent placement. The other three patients underwent open laparotomy and bile duct ligation. Seven of the patients had major BDI. In two patients, biliary injuries were identified at the time of LC, and the procedure was converted to laparotomy. At the time of conversion, primary suture repair with T-tube drainage of the injured bile duct was performed. Strictures developed in these patients after 2 and 6 months, respectively, and they were treated with a Roux-en-Y hepaticojejunostomy. In five additional patients, BDI was recognized postoperatively. One of these patients died because of delayed detection of biliary peritonitis. At this writing, during a median follow-up period of 52 months, neither clinical nor biochemical evidence of biliary disease has been found in the remaining patients. CONCLUSIONS: Laparoscopic BDI has a high morbidity and mortality rate. Late recognition of the BDI remains a problem.