Mucin-producing hepatic cystic neoplasms: an uncommon but challenging disease often misdiagnosed and mismanaged.

Background: Mucin-producing hepatic cystic neoplasms (MHCN) are uncommon and potentially malignant.Methods: Nine MHCN were encountered in our centre for over 32 years. Patients' clinical, biological, radiological and pathological features were reviewed. Lesions were classified into Mucinous Cystic Neoplasms (MCN) and Intraductal Papillary Neoplasms of the Bile duct (IPNB) (WHO 2010 classification).Results: Five MCN and 4 IPNB were reviewed. Serum and intracystic tumour markers were insufficient to diagnose malignancy. Complications were encountered in five out of nine patients (56%), mean symptom duration was 26 months (range: 1-132). Three patients were mismanaged pre-referral. Radiological features enabled preoperative diagnosis in eight out of nine patients (89%). Greater tumour size, unilocular lesion and mural nodularity indicated malignancy. Radical tumour excision was achieved in eight patients. One IPNB patient was misdiagnosed and underwent unroofing. For 103 months median follow-up, five out of six patients with benign tumours were alive and disease-free, whereas the misdiagnosed IPNB recurred with fatal malignant transformation seven years later. Among the three patients with malignancies (median follow-up: 77 months), two IPNB died, one from cancer recurrence and one from unrelated causes, whereas the malignant MCN was alive and disease-free.Conclusions: Appropriate MHCN diagnosis is crucial, yet it is often misdiagnosed and mismanaged. The prognosis after complete excision is favourable.

Epidemiology and mortality of candidemia both related and unrelated to the central venous catheter: a retrospective cohort study.

Our objective was to compare mortality, epidemiology, and morbidity in hospitalized patients with candidemia which was both related and unrelated to the central venous catheter (CVC). This was a monocentric, retrospective cohort study of candidemia. The sample consisted of 103 patients with laboratory-confirmed nosocomial candidemia hospitalized between 2006 and 2013 in a tertiary care public hospital. We included 65 (63.1 %) patients (24 in the CVC-positive group, 41 in the CVC-negative group). Demographic data and risk factors were recorded using a structured case report form. In the group of candidemia associated to the CVC, survival at day 50 was 58.6 ± 11.9 %, compared to 26.5 ± 8.9 % for the CVC-negative group (p-value = 0.012); the hazard ratio of death was 0.38 (95 % confidence interval 0.17-0.85, p-value = 0.019). Compared with the CVC-positive patients, CVC-negative patients were often colonized with yeast (41.5 % vs. 16.7 %, p-value = 0.041), had a shorter previous in-hospital stay (20 days vs. 34 days, p-value = 0.023), and were more severely ill (severe sepsis 85.4 % vs. 58.3 %, p-value = 0.016). In this study, when the origin of candidemia was not the CVC, patients were more seriously ill, had a higher mortality rate, and the removal of the catheter seemed to lead to disappointing results. It would be useful to explore the impact of retention of the CVC on survival in the CVC-negative patients, where the CVCs are essential to treating these patients.

Reproducibility of three-dimensional posterior cranial base angles using low-dose computed tomography.

OBJECTIVES: One of the key aspects of three-dimensional (3D) craniofacial cephalometry is the measurement of posterior cranial base angle as this area is deeply involved in craniofacial development. The purpose of our retrospective study was to define the best reproducible 3D posterior cranial base angles among five 3D angles transposed from 2D cephalometry (Cousin, BL1 of Ross and Ravosa, Bjork, Delaire, CBA4 of Liberman) and seven 3D angles based on physical anthropology studies and on new concepts (R1 to R7). The null hypothesis was that all 3D posterior cranial base angles were equally reproducible. MATERIAL AND METHODS: We used a preoperative low-dose computed tomography (CT) data from 20 adult patients undergoing orthognathic surgery after approval by local ethical committee. Two independent observers performed two series of 23 3D landmark identifications on 3D CT surface rendering of each patient using Maxilim software. Then, the same observers performed twice 3D cephalometric analyses (23 landmarks, 4 midpoints, 19 planes) that provided the automatic measurement of 12 posterior cranial base angles. RESULTS: Inter-observer correlation coefficient varied from 0.545 (Cousin) to 0.695 (CBA4 of Liberman) and from -0.177 (R2) to 0.827 (R4). CONCLUSIONS: The null hypothesis was rejected. The most reproducible angle was 3D angle R4 based on "basion," "superior optic" (right, left), and "crista galli inferior" landmarks. CLINICAL RELEVANCE: R4 angle might be used as reference 3D posterior cranial base angle in further clinical studies involving 3D cephalometry as a diagnostic tool for orthodontics and for orthognathic surgery.

Reproducibility of three-dimensional cephalometric landmarks in cone-beam and low-dose computed tomography.

OBJECTIVES: The purpose of this study is to compare the reproducibility of three-dimensional cephalometric landmarks on three-dimensional computed tomography (3D-CT) surface rendering using clinical protocols based on low-dose (35-mAs) spiral CT and cone-beam CT (I-CAT). The absorbed dose levels for radiosensitive organs in the maxillofacial region during exposure in both 3D-CT protocols were also assessed. MATERIALS AND METHODS: The study population consisted of ten human dry skulls examined with low-dose CT and cone-beam CT. Two independent observers identified 24 cephalometric anatomic landmarks at 13 sites on the 3D-CT surface renderings using both protocols, with each observer repeating the identification 1 month later. A total of 1,920 imaging measurements were performed. Thermoluminescent dosimeters were placed at six sites around the thyroid gland, the submandibular glands, and the eyes in an Alderson phantom to measure the absorbed dose levels. RESULTS: When comparing low-dose CT and cone-beam CT protocols, the cone-beam CT protocol proved to be significantly more reproducible for four of the 13 anatomical sites. There was no significant difference between the protocols for the other nine anatomical sites. Both low-dose and cone-beam CT protocols were equivalent in dose absorption to the eyes and submandibular glands. However, thyroid glands were more irradiated with low-dose CT. CONCLUSIONS: Cone-beam CT was more reproducible and procured less irradiation to the thyroid gland than low-dose CT. CLINICAL RELEVANCE: Cone-beam CT should be preferred over low-dose CT for developing three-dimensional bony cephalometric analyses.

High suspicion of bacillary angiomatosis in a kidney transplant recipient: a difficult way to diagnose--case report.

Bacillary angiomatosis is an infection caused by Bartonella, which has first been described in human immunodeficiency virus (HIV)-infected patients. We report an unusually located lesion, in a totally asymptomatic kidney transplant recipient. The diagnosis was strongly suggested based on the iconography and our histological analysis, but was not confirmed using polymerase chain reaction (PCR) and immunohistochemical studies. We illustrate our difficult way to the diagnosis as well as the course of the disease and our therapeutic strategy.

Three-dimensional computed tomography cephalometric craniofacial analysis: experimental validation in vitro.

The development of three-dimensional (3D) cephalometric analysis is essential for the computer-assisted planning of orthognathic surgery. The aim of this study was to transform and adapt Delaire's two-dimensional cephalometric analysis into the third dimension; this transposition was then validated. The comparative advantage of using 3D computed tomography (CT) surface renderings over profile X-rays was analysed. Comparison was made of inter- and intra-observer reproducibility of the cephalometric measurements done on profile X-rays and on 3D CT surface renderings on the same 26 dry skulls. The accuracy was also tested of the measurements done on 3D CT surface renderings (ACRO 3D) in relation to those directly taken on dry skulls with the help of a 3D measuring instrument. Inter- and intra-observer reproducibility proved significantly superior (p<0.0001) following the 3D CT method. There were no significant differences in the accuracy of measurements between the ACRO 3D software and the 3D measuring instrument. The ACRO 3D software was confirmed as being a reliable tool for developing 3D CT cephalometric analyses. Further research may entail clinical validation of the 3D CT craniofacial cephalometric method of analysis.

Successful treatment of Acanthamoeba rhinosinusitis in a patient with AIDS.

Acanthamoeba infection is a rare, difficult-to-treat, and often fatal, opportunistic parasitic infection in immunocompromised hosts, such as patients infected with HIV. We describe an aggressive nasal and sinus infection by Acanthamoeba spp. in a person with AIDS. The resolution of this Acanthamoeba infection was secondary to a multidisciplinary treatment approach involving a combination of surgery as well as high-dose amphotericin B plus 5-fluorocytosine. In the era of the HIV/AIDS pandemic, the present report underscores the need for early identification and prompt aggressive treatment to ensure successful management of this rare but potentially fatal opportunistic infection.

Evaluation of the hypothalamic-pituitary-adrenal axis and its relationship with central respiratory dysfunction in children with Prader-Willi syndrome.

BACKGROUND: Children with Prader-Willi Syndrome (PWS) have been considered at risk for central adrenal insufficiency (CAI). Hypothalamic dysregulation has been proposed as a common mechanism underlying both stress-induced CAI and central respiratory dysfunction during sleep. OBJECTIVE: To evaluate CAI and sleep-related breathing disorders in PWS children. PATIENTS AND METHODS: Retrospective study of cortisol response following either insulin tolerance test (ITT) or glucagon test (GT) in 20 PWS children, and comparison with 33 non- Growth Hormone deficient (GHD) controls. Correlation between sleep related breathing disorders and cortisol response in 11 PWS children who received both investigations. RESULTS: In PWS children, the cortisol peak value showed a significant, inverse correlation with age (Kendall's τ = -0.411; p = 0.012). A similar though non-significant correlation was present between cortisol increase and age (τ = -0.232; p = 0.16). Similar correlations were found in controls. In only 1 of 20 PWS children (5 %), ITT was suggestive of CAI. Four patients had an elevated central apnea index but they all exhibited a normal cortisol response. No relationship was found between peak cortisol or cortisol increase and central apnea index (respectively p = 0.94 and p = 0.14) or the other studied polysomnography (PSG) parameters. CONCLUSIONS: CAI assessed by ITT/GT is rare in PWS children. Our data do not support a link between CAI and central respiratory dysregulation.

Elevation of serum thyroxine-binding globulin (but not of cortisol-binding globulin and sex hormone-binding globulin) associated with the progression of human immunodeficiency virus infection.

PURPOSE: In order to assess the relation of thyroid function tests to human immunodeficiency virus (HIV) infection, we determined the levels of serum thyroid hormones, serum binding proteins [thyroxine-binding globulin (TBG), cortisol-binding globulin (CBG), and sex hormone-binding globulin (SHBG)], and serum tumor necrosis factor (TNF) in HIV-seropositive subjects at different clinical stages. PATIENTS AND METHODS: Thirty-seven HIV-seropositive patients were studied: 7 at stage II, 13 at stage III, and 17 at stage IV (eight ambulatory and nine hospitalized) according to the Centers for Disease Control's criteria. RESULTS: As compared with stage II and stage III patients, stage IV patients had significantly higher mean TBG and total thyroxine (TT4) values, similar and normal total triiodothyronine (TT3) levels, and similar and abnormally low reverse triiodothyronine (rT3) concentrations. However, stage IV hospitalized patients had significantly lower TT3 values than stage IV ambulatory patients. In contrast to TBG, mean levels of CBG and SHBG were comparable in the three groups and within normal limits. For the whole population of HIV patients, there was a highly significant correlation between the CD4 lymphocyte count and TBG (r = -0.529, p less than 0.001) but not with CBG and SHBG levels. Finally, TNF values higher than 10 pg/mL were detected in six of the 17 stage IV patients and in only one of the 13 stage III patients (p = 0.059); elevated TNF levels correlated with a lower CD4 count (p less than 0.01) but not with serum TBG levels. CONCLUSION: The progression of HIV infection is associated with an elevation of serum TNF and TBG, but not of CBG or SHBG. HIV-infected patients have an unexpectedly normal TT3-low rT3 state.

Clinical use and bacteriological studies of catheter contamination sleeves.

A system combining a valved introducer sheath and a plastic protective sleeve enabling repositioning of pulmonary artery catheters was tested in 73 cardiac surgical patients. It was used for a mean time of 70.3 h and allowed for improved hemodynamic monitoring. A 12 percent incidence of valve contamination was found and makes the safety of the device questionable.

[Aids and chorioretinal opportunistic infections]. / SIDA et infections opportunistes de la choriorétine.

Retrospective study of 19 cases of opportunistic infections of the chorioretina in patients with the Acquired Immunodeficiency Syndrome. We observed 14 cases of CMV retinitis, 2 cases of toxoplasmic chorioretinitis, 1 case of cryptococcal choroiditis and 2 cases of herpes zoster retinitis. Review of the clinical, angiographical and histopathological aspects of these infections. Review of the vital and visual prognosis after treatment.

[Septic arthritis of a posterior vertebral inter-apaphysial articulation. Apropos of a case]. / Arthrite septique d'une articulation inter apophysaire vertébrale postérieure. A propos d'un cas.

A report of a case of haematogenous infection of a lumbar zygapophysial joint. In spite of radiographic and bone scan findings that localised the lesion, the diagnosis was not made until an operative exploration was made, which also effected a cure. This is an unusual site for sepsis and the clinical picture can easily lead to confusion with spondylosis which is much more common.

Determinants of weight loss in an interdisciplinary long-term care program for childhood obesity.

Background. Efforts are needed to improve the long-term efficiency of childhood obesity treatment. To adapt strategies, the identification of subgroups of patients with a greater weight loss may be useful. Objective. To analyze the results of a chronic care program for childhood obesity and to determine baseline factors (medical, dietary, and psychosocial) associated with successful weight loss. Subjects and Method. We set up a family-targeted and individually adapted interdisciplinary long-term care program. We reviewed the medical files of 144 children (59 boys and 85 girls; 10.5 ± 3.1 y; mean BMI-z-score: 2.73 ± 0.62) who had ≥2 interdisciplinary visits and ≥1-year treatment. Results. Mean treatment length was 2.2 y (1-6.7 y) with 3 ± 1 visits/year. The duration of treatment did not depend on the initial weight loss, but this was predictive of the weight change over time. Furthermore any additional weight loss was observed with time whatever the initial weight change. High levels of physical activity and daily water intake from baseline conditions were associated with a greater weight loss after 9 months of intervention. In contrast, a high baseline consumption of soft drinks resulted in lower weight loss. Family specific factors such as being a single child or the child's family support were identified as baseline factors which may contribute to better results. Conclusion. Our study suggests that the benefit of a chronic weight control program supports the need for its integration into the current concept of treatment. Better prevention policy and parental support may improve the success of the childhood obesity treatment.

European and American suspected and confirmed pulmonary embolism populations: comparison and analysis.

BACKGROUND: If the prevalence of pulmonary embolism (PE) differs significantly between the US and Europe, this observation could reduce the generalizability of diagnostic protocols for PE derived in either location. OBJECTIVE: To determine possible causes and potential clinical consequences of these PE prevalence differences. METHODS: Secondary analysis of three prospectively collected multicenter samples (two French and one from the US) including 3174 European and 7940 American PE-suspected patients in Emergency departments (ED) (117 for Europe and 12 for US). Comparison of clinical features, resource use and outcomes of European- and US-suspected PE populations in ED. RESULTS: European patients evaluated for PE were significantly older and had a higher clinical pretest probability (CPP) for PE. The final PE prevalence was significantly higher in Europe, in the overall sample (26.5% vs. 7.6%) and in each level of CPP. Suspected European patients categorized as low CPP had a higher posttest probability than US low CPP patients. Suspected US patients categorized as high CPP had a much lower posttest probability of PE than in Europe. The mean number of tests performed for one PE diagnosis was lower in Europe (7.4 vs. 21.6). Among patients diagnosed with PE, European patients had a higher mean severity of illness score and a higher PE-mortality rate (3.4% vs. 0.7%). CONCLUSIONS: Among patients suspected of a PE and those ultimately diagnosed with a PE, European patients had higher acuity, a higher pretest probability and worse outcome than US patients. The present study underscores the importance of disease prevalence for pretest probability scoring approaches and for significance interpretation of imaging tests.

Accuracy of three-dimensional (3D) craniofacial cephalometric landmarks on a low-dose 3D computed tomograph.

OBJECTIVES: The aim of this paper is to compare the accuracy of cephalometric landmark identification using three-dimensional CT (3D-CT) surface rendering with "high-dose" (200 mAs) and "low-dose" (35 mAs) CT protocols. The absorbed dose levels for radiosensitive organs in the maxillofacial region during the exposure for both 3D-CT protocols were also measured. METHODS: The study population consisted of 15 human dry skulls examined with spiral 3D-CT. 12 cephalometric anatomical landmarks at 7 sites were identified on the 3D-CT surface renderings by 2 observers independently, twice each, using high-dose and low-dose protocols. In total, 1440 imaging measurements were made. Thermoluminescent dosemeters (TLDs) were placed at ten sites around the thyroid and submandibular glands and the eyes in an Alderson phantom for measuring the absorbed dose levels. RESULTS: The intraobserver mean distances between 3D landmarks were smaller for all sites with the high-dose protocol (P = 0.37). There was a significant difference among the observers (P = 0.000004). Interobserver mean distances between 3D landmarks were smaller for four of the seven sites with the low-dose protocol. However, the global interobserver mean distances between 3D landmarks for all sites were smaller with the high-dose protocol (P = 0.028). The low-dose protocol reduced the radiation dose to the thyroid by 6.12, to the submandibular salivary glands by 5.91 and to the eye by 5.44, resulting in a global reduction factor of 5.71. CONCLUSIONS: The accuracy in the landmark's identification was maintained when the milliampere-second values were reduced from 200 mAs to 35 mAs. We recommend use of the low-dose protocol for clinical 3D-CT cephalometric applications.

A retrospective study about the use of cotrimoxazole as diagnostic support and treatment of suspected cerebral toxoplasmosis in AIDS.

The aim of this retrospective study was to define a diagnostic strategy and to evaluate the efficacy of cotrimoxazole (CTMX) for presumed cerebral toxoplasmosis in patients with AIDS. Twelve patients with toxoplasma encephalitis were reviewed. The best diagnostical signs of reactivated acute cerebral toxoplasmosis were the association of neurological symptoms indicative of focal cerebral lesions, and a radiological picture showing ring contrast enhanced hypodense mass-lesions; serology was unreliable for the diagnosis. Five patients out of twelve were treated without delay and until death with CTMX. Only these improved their clinical and radiological status obviously. Moreover, their median survival time was clearly longer (160 days, versus 9 days) and their autopsy demonstrated the absence of active necrotizing lesions of toxoplasma encephalitis. So, CTMX seems to be an efficient therapy for suspected cerebral toxoplasmosis in AIDS. Nevertheless, further prospective randomized therapeutic trials are required to confirm this impression.