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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Serum beta-carotene in anorexia nervosa patients: a case-control study.

OBJECTIVE: To study the prevalence of hypercarotenemia in a large cohort of patients with anorexia nervosa (AN), to compare serum beta-carotene (betaC) values among restricting and purging AN subjects, and to investigate whether hypercarotenemia is related to an increase in low-density lipoprotein (LDL) cholesterol. METHOD: Retrospective case-control study including 101 female patients and 95 age-matched normal controls in whom fasting serum betaC and lipid profiles were determined. RESULTS: The prevalence of hypercarotenemia (>200 microg/dl) in the AN population was 62%. Mean serum betaC level was significantly higher in AN patients than in controls (237 +/- 103 vs. 160 +/- 45 microg/dl, p <.0001). Among AN patients, the level was higher in restricters than in purgers (271 +/- 110 vs. 186 +/- 78 microg/dl, p <.005). Fasting serum total and LDL cholesterol levels were also significantly higher in patients with AN than in controls, but no correlation was found between serum betaC and LDL cholesterol values. DISCUSSION: Hypercarotenemia is a common finding in AN patients, especially in the restricter subgroup. The high prevalence of elevated serum betaC in AN patients supports its diagnostic value in atypical forms of eating disorders.

Heparin-induced thrombocytopaenia complicated by arterial and venous thrombosis: report of 2 cases successfully treated by a danaparoid sodium.

Heparin-induced thrombocytopaenia is a dreaded, although infrequent, complication of heparin therapy. We report two cases of heparin-induced thrombocytopaenia (HIT) type II occurring in a patient treated with standard (unfractionated) heparin and in another patient given a low-weight molecular heparin. The clinical course of the first patient illustrates the potentially severe thrombotic complications of HIT. Both cases were treated successfully by danaparoid sodium. Clues to the diagnosis and treatment are briefly discussed.

Impact of infectious diseases specialists and microbiological data on the appropriateness of antimicrobial therapy for bacteremia.

Antimicrobial therapy for 428 episodes of bacteremia in an 850-bed university hospital was prospectively evaluated for 1 year to measure the impact of two factors--blood culture results and the therapy chosen by infectious diseases specialists (IDSs)--on quality of treatment and outcome. Initial shock, a simplified acute physiology score of >15, and inappropriateness of the empirical treatment were independently associated with increased mortality. Empirical treatment was appropriate in 63% of the episodes. This proportion reached 78% for the episodes treated by IDSs, compared with 54% for the others (P < .001). After availability of blood culture results, the proportion of appropriate treatments increased to 94%, with 97% for IDS-treated patients and 89% for other patients (P = .008). IDSs more frequently shifted to oral antibiotics and used fewer broad-spectrum drugs. This study underlines the impact of blood culture results and of IDSs on the prescription of appropriate treatment for bacteremia and on the better use of antimicrobial drugs.

The prothrombin gene G20210A variant in an unselected thromboembolic population. A Belgian prospective clinical study.

The presence of the 20210A allele of the prothrombin gene has recently been shown to be a risk factor of venous thromboembolism, probably mediated through increased prothrombin levels. The aim of the study was to determine the frequency of the prothrombin 20210A allele in 193 consecutive unselected patients with venous thromboembolism and 100 healthy controls and to analyze the clinical profile associated with this new inherited thrombophilic factor. In agreement with previous reports, we found a frequency of 7.3% of heterozygous carriers of the 20210A allele among patients and 1% among controls. We confirm that plasma prothrombin levels are more elevated in the individuals bearing the prothrombin 20210A allele compared with those who do not. We did not find any relationship between the presence of the prothrombin 20210A allele and either a family history of thromboembolism, the rate of recurrences or the age at disease onset. However, the co-inheritance in the same individual of both prothrombin 20210A allele and factor V Leiden was associated with a significantly lower age at disease onset suggesting a synergistic contribution of both abnormalities.

Prevalence of activated protein C resistance and analysis of clinical profile in thromboembolic patients. A Belgian prospective study.

OBJECTIVES: To assess the prevalence of activated protein C resistance (APC-R) among healthy subjects and thromboembolic patients and to determine the clinical characteristics associated with APC-R. DESIGN: A prospective study. SETTING: One academic medical centre. SUBJECTS: 91 health controls and 126 thromboembolic patients. MEASUREMENTS: Patients and control were genotyped for the factor V Leiden (VaQ506) mutation. The anticoagulant response of the patient's plasma to activated protein C was also determined. RESULTS: The frequency of APC-R was 3.3% among healthy control subjects and 22% among thrombotic patients of whom 18% were heterozygous and 4% were homozygous. The mean age at the first thrombotic event and the severity of thrombotic disease including the proportion of proximal deep vein thrombosis and the frequency of lung embolism were identical among APC-R positive and negative patients. A family history of thromboembolic disease was elicited more frequently in APC-R positive than in APC-R negative patients (57% vs. 22%, P < 0.001). The recurrence rate was higher for APCR-R positive patients (57% vs. 34%, P < 0.05). The percentage of cases with a factor predisposing to thrombosis was very similar in APC-R positive (57%) and negative (68%) patients. CONCLUSIONS: A familial history of thromboembolic disease and recurrences are significantly more frequent among APC-R positive than APC-R negative patients.

[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.