Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment

Sinus venosus atrial septal defect (SV‐ASD) usually coexists with partial anomalous pulmonary vein connection (PAPVC). It is a difficult diagnosis in transthoracic echocardiography (TTE) due to eccentric position of defects. We present a rare case of atypical anatomical variation in PAPVC, which was never described before. Two right pulmonary veins drained into superior vena cava, which overrode SV‐ASD and interatrial septum, a third pulmonary vein into the right atrium. Complete diagnosis could not be set after TTE, nor transesophageal echocardiography, whereas angio‐CT was finally conclusive. This diagnostic approach allowed the surgical planning.     

Nephrology and renal replacement therapy in Romania--transition still continues (Cinderella story revisited).

INTRODUCTION. This report describes the current status of nephrology and renal replacement therapy (RRT) in Romania, a country with previously limited facilities, highlighting national changes in the European context. METHODS: Trends in RRT development were analysed in 2003, on a national basis, using the same questionnaires as in previous surveys (1991, 1995). Survival data and prognostic risk factors were calculated retrospectively from a large representative sample of 2284 patients starting RRT between January 1, 1995 and December 31, 2001 (44% of the total RRT population investigated). RESULTS: In 2003, RRT incidence [128 per million population (p.m.p.)] and prevalence (250 p.m.p.) were six and five times higher, respectively, than in 1995. The annual rate of increase in the stock of RRT patients (11%) was supported mainly by an exponential development of the continuous ambulatory peritoneal dialysis (CAPD) population (+600%), while the haemodialysis (HD) growth rate was stable (+33%) and renal transplantation made a marginal contribution. Renal care infrastructure followed the same trend: nephrology departments (+100%) and nephrologists (+205%). The characteristics of RRT incident patients changed accordingly to current European epidemiology (increasing age and prevalence of diabetes and nephroangiosclerosis). The estimated overall survival of RRT patients in Romania was 90.6% at 1 year [confidence interval (CI) 89.4-91.8] and 62.2% at 5 years (CI 59.4-65.0). Patients' survival was negatively influenced (Cox regression analysis) by age >65 years (P < 0.001), lack of pre-dialysis monitoring by a nephrologist [P = 0.01, hazards ratio (HR) = 0.8], severe anaemia, lack of erythropoetin treatment (P < 0.001, HR = 0.6), and co-morbidity, e.g. cardiovascular diseases (P < 0.001, HR = 1.8) and diabetes mellitus (P < 0.001, HR = 2.2). CONCLUSIONS: Although the rate of increase in RRT patient stock in 1996-2003 in Romania was the highest in Europe, the prevalence remained below the European mean. As CAPD had the greatest expansion, followed by HD, an effective transplantation programme must be set up to overcome the imbalance. The quality of RRT appears to be good and survival was similar to that in other registries. Further evolution implies strategies of prevention, based on national surveys, supported by the Romanian Renal Registry.     

Pitfall in diagnosis of Crohn's disease in a cystic fibrosis patient

Cystic fibrosis (CF) and Crohn's disease may both present as failure to thrive and recurrent intestinal obstruction. Proper treatment and adequate nutrition may reverse these manifestations and improve the patient's quality of life. We describe a girl with CF who, despite appropriate management, failed to grow and had several episodes of bowel obstruction. After the additional diagnosis of Crohn's disease was reached, the patient improved on antiinflammatory and nutritional therapy. This patient illustrates the pitfall in the diagnosis of Crohn's disease in a CF patient due to the clinical overlap between the two conditions. We suggest that therapeutic failure in a chronic disease justifies additional diagnostic efforts resulting in a completion of diagnosis and significant changes in management.     

Surgical treatment of paravalvular abscess: long-term results.

OBJECTIVE: To examine the outcomes of surgery for active infective endocarditis with paravalvular abscess. METHODS: Paravalvular abscess was defined as infective necrosis of the valve annulus that required patch reconstruction before implanting a new valve. Of 383 patients with active infective endocarditis who underwent surgical treatment, 135 (35%) had paravalvular abscess. Patients' mean age was 51+/-16 years and 68% were men. The infected valve was native in 69 patients and prosthetic in 66. The abscess involved the aortic annulus in 73 patients, the mitral annulus in 27, the aortic and mitral annuluses in 33, and the aortic and tricuspid and/or pulmonary annuluses in 2. Surgery consisted of radical resection of the abscess, reconstruction of the annulus with patches and valve replacement. Mean follow-up was 6.2+/-5.2 years and complete. RESULTS: There were 21 (15.5%) operative deaths. Preoperative shock and abscess in the aortic and mitral annuluses were independent predictors of operative death. There were 34 (25%) late deaths. Survival at 15 years was 43+/-6% for all patients, 50+/-8% for native valve endocarditis and 35+/-9% for prosthetic (p=0.41). Age by increments of 5 years and recurrent endocarditis were independent predictors of late death. There were 16 episodes of recurrent endocarditis in 15 patients, and the freedom from recurrent endocarditis was 82+/-4% at 15 years. Fifteen reoperations were performed in 14 patients. Freedom from reoperation was 72+/-9% at 15 years. CONCLUSIONS: Surgery for active endocarditis with paravalvular abscess was associated with high operative mortality, particularly in patients in shock and abscess of both mitral and aortic annuluses. Long-term survival was adversely affected by age and recurrent bouts of endocarditis.     

The hypotransferrinaemic mouse: Ultrastructural and laser microprobe analysis observations

Homozygote hypotransferrinaemic mice (hpx/hpx) have cytopathological features similar to those of human congenital atransferrinaemia, genetic haemochromatosis, and neonatal haemochromatosis. These conditions all have in common high levels of cytotoxic non-transferrin-bound serum iron. This study describes the ultrastructural features of iron overload in liver, pancreas, heart, and small intestine of 2- and 12-month-old hypotransferrinaemic mice. Electron microscopic studies of unstained sections showed early parenchymal cell siderosis, with accumulation of numerous ferritin particles and clusters in the cytosol, as well as ferritin and haemosiderin in lysosomes (siderosomes). In the 12-month-old animals, iron was also found in Kupffer cells and macrophages in other tissues. In addition, there were conspicuous iron-containing compounds in the bile canaliculi, and marked iron deposition in the pancreas and heart. Laser microprobe mass analysis (LAMMA) enabled localization and relative quantitation of iron deposition in subcellular compartments providing in situ documentation of iron accumulation in siderosomes and contributed in assessing total cytosolic iron in various cell types. Moreover, it demonstrated the importance and magnitude of the biliary route for iron excretion in these animals.     

The Lipofuscin-Iron Association in Pigmentosis Tubae

Pigmentosis tubae (PT) is a rare condition characterized by the presence of numerous lipofuscin-laden macrophages in the lamina propria of the fallopian tube. Two women, who also had endometriotic ovarian cysts, showed polypoid pigmented tubal mucosae. In addition to lipofuscin, occasional cells showed spotty positivity for iron. Ultrastructural examination of the tubal mucosa showed the lipofuscin-containing bodies, which were similar to lipofuscin-containing lysosomes found in other pigmented conditions. Cytoplasmatic ferritin and hemosiderin in siderosomes were observed in macrophages and endothelial cells of the lamina propria. The present study is the first to demonstrate the presence of iron-containing particles and lipofuscin in the residual bodies of PT. The origin of the excess iron is not clear, but erythrophagocytosis and an abnormal tubal environment could play a role. Iron-promoted lipid peroxidation may alter the lysosomal membranes and contribute to the excessive accumulation of lipofuscin in these cells.     

Hepatic iron overload in birds: Analytical and morphological studies

Hepatic iron content was determined in post mortem specimens from a wide range of avian species collected over a 12-month period. The majority (> 90%) of these specimens (n = 40) showed high iron content (up to 12 mg Fe/g tissue). The highest concentrations were associated with fibrosis and regenerative nodules. Dietary analysis indicated that the iron intake was not excessive, suggesting that iron-loading was due to enhanced intestinal absorption.     

Change of appetite in patients with functional digestive disorder. Association with psychological disorders: A cross‐sectional study

Background and Aims

Changes in appetite are a frequent complaint in patients with functional gastrointestinal disorders (FGIDs). The aims of this study are to evaluate whether the changes in appetite are associated with specific FGIDs and to explore associations of these changes with symptoms of anxiety or depression.

Methods

This study included 1009 consecutive FGID patients (71% female), aged 48.9 years who all filled out a Rome III questionnaire for the evaluation of FGIDs, submitted to a psychological evaluation of symptoms of anxiety, and completed the Beck Depression Inventory questionnaire. The patients were classified according to their appetite change using a 7‐point grading scale and split into three groups: those with appetite loss, those with no change in appetite, and those with increased appetite.

Results

Among the 1009, 496 patients (49%) reported a change in appetite, of which 332 (33%) patients reported a decrease in appetite and 164 (16%) patients reported an increase in appetite. Appetite was not affected in 51% of patients. Changes in appetite depended on gender, body mass index and psychometric evaluation scores. Increased appetite did not have specific FGIDs associations, while decreased appetite was associated with esophageal, gastroduodenal, bowel, and anorectal symptoms. The presence of depressive symptoms was also a predictor for the majority of FGIDs in decreased appetite, while anxiety trait was significant for globus and dysphagia.

Conclusions

Decreased appetite was associated with FGIDs, especially in the presence of depressive symptoms. A reduced appetite would help to predict psychological disorders associated with FGIDs.

Financial disclosure

None declared.

Legal registration

This study was a registered study in the French National Drug Agency (ANSM, Agence Nationale de Securité du Medicamentet des produits de santé, Study Number 2016‐A01120‐51).

Competing interests

Michel Bouchoucha, Marinos Fysekidis, Florence Mary, Gheorghe Airinei, Cyriaque Bon, and Robert Benamouzig have no competitive interests.