Experimental acoustic scene analysis using One-Eighth spherical fraction microphone array.

This paper investigates the performance of one-eighth Spherical Fraction Microphone Array through experimental measurement to analyze acoustic scenes in one-eighth of space. The array geometry is designed to be placed in a room corner at the junction of three acoustically rigid walls. Two prototypes are built with 8 and 16 microphones, respectively. The sampling strategy is discussed and a spatial aliasing analysis is carried out both analytically and by numerical simulations. The array performances are evaluated through Spherical Fraction Beamforming (SFB). This approach is based on the decomposition of the acoustic pressure field in a rigid bounded domain. The localization angular error and Directivity Index criterion are evaluated for both arrays. In a first experiment, the arrays are mounted in an eighth of space built inside an anechoic room. The results are compared with simulation and show consistency. The theoretical limitations of SFB in a rigid bounded one-eighth of space are retrieved experimentally. These limitations are also observed in a real configuration: an office room. Further investigations on SFB are also conducted in the case of a virtual scene constructed with two sound sources.

Vitis Methods to Understand and Develop Strategies for Diagnosis and Sustainable Control of Grapevine Trunk Diseases.

Vitis vinifera is affected by many diseases every year, depending on causal agents, susceptibility of cultivars, and climate region. Some are caused by a single agent, such as gray mold caused by Botrytis cinerea or powdery mildew caused by Erysiphe necator. Others result from the actions of a complex of pathogens such as grapevine trunk diseases (GTDs). GTDs are presently among the most devastating diseases in viticulture worldwide because both the economic losses and the long-term sustainability of vineyards are strongly affected. The complexity of GTDs results from the diversity of associated fungi, the undetermined period of latency within the vine (asymptomatic status), the erratic foliar symptom expression from one year to the next, and, probably correlated with all of these points, the lack of efficient strategies to control them. Distinct methods can be beneficial to improve our knowledge of GTDs. In vitro bioassays with cell suspensions, calli, foliar discs, full leaves, or plantlets, and in vivo natural bioassays with cuttings, grafted plants in the greenhouse, or artificially infected ones in the vineyard, can be applied by using progressive integrative levels of in vitro and in vivo, depending on the information searched. In this review, the methods available to understand GTDs are described in terms of experimental procedures, main obtained results, and deliverable prospects. The advantages and disadvantages of each model are also discussed.

Histologically Proven Bronchial Neuroendocrine Tumors in MEN1: A GTE 51-Case Cohort Study.

OBJECTIVE: To evaluate the natural history of MEN1-related bronchial endocrine tumors (br-NETs) and to determine their histological characteristics, survival and causes of death. br-NETs frequency ranges from 3 to 13% and may reach 32% depending on the number of patients evaluated and on the criteria required for diagnosis. METHODS: The 1023-patient series of symptomatic MEN1 patients followed up in a median of 48.7 [35.5-59.6] years by the Groupe d'étude des Tumeurs Endocrines was analyzed using time-to-event techniques. RESULTS: br-NETs were found in 51 patients (4.8%, [95% CI 3.6-6.2%]) and were discovered by imaging in 86% of cases (CT scan, Octreoscan, Chest X-ray, MRI). Median age at diagnosis was 45 years [28-66]. Histological examination showed 27 (53%) typical carcinoids (TC), 16 (31%) atypical carcinoids (AC), 2 (4%) large cell neuroendocrine carcinomas (LCNEC), 3(6%) small cell neuroendocrine carcinomas (SCLC), 3(6%) TC associated with AC. Overall survival was not different from the rest of the cohort (HR 0.29, [95% CI 0.02-5.14]). AC tended to have a worse prognosis than TC (p = 0.08). Seven deaths were directly related to br-NETs (three AC, three SCLC and one LCNEC). Patients who underwent surgery survived longer (p = 10-4) and were metastasis free, while 8 of 14 non-operated patients were metastatic. There were no operative deaths. CONCLUSIONS: Around 5% of MEN1 patients develop br-NETs. br-NETs do not decrease overall survival in MEN1 patients, but poorly differentiated and aggressive br-NETs can cause death. br-NETs must be screened carefully. A biopsy is essential to operate on patients in time.

Source sparsity control of sound field reproduction using the elastic-net and the lasso minimizers.

Sound field reproduction is aimed at the reconstruction of a sound pressure field in an extended area using dense loudspeaker arrays. In some circumstances, sound field reproduction is targeted at the reproduction of a sound field captured using microphone arrays. Although methods and algorithms already exist to convert microphone array recordings to loudspeaker array signals, one remaining research question is how to control the spatial sparsity in the resulting loudspeaker array signals and what would be the resulting practical advantages. Sparsity is an interesting feature for spatial audio since it can drastically reduce the number of concurrently active reproduction sources and, therefore, increase the spatial contrast of the solution at the expense of a difference between the target and reproduced sound fields. In this paper, the application of the elastic-net cost function to sound field reproduction is compared to the lasso cost function. It is shown that the elastic-net can induce solution sparsity and overcomes limitations of the lasso: The elastic-net solves the non-uniqueness of the lasso solution, induces source clustering in the sparse solution, and provides a smoother solution within the activated source clusters.

Picometer-scale surface roughness measurements inside hollow glass fibres.

A differential profilometry technique is adapted to the problem of measuring the roughness of hollow glass fibres by use of immersion objectives and index-matching liquid. The technique can achieve picometer level sensitivity. Cross validation with AFM measurements is obtained through use of vitreous silica step calibration samples. Measurements on the inner surfaces of fibre-sized glass capillaries drawn from high purity suprasil F300 tubes show a sub-nanometer roughness, and the roughness power spectrum measured in the range [5 · 10(-3) m(-1) 10(-1) m(-1)] is consistent with the description of the glass surface as a superposition of frozen capillary waves. The surface roughness spectrum of two capillary tubes of differing compositions can be quantitatively distinguished.

Experimental assessment of the accuracy of genomic selection in sugarcane.

Sugarcane cultivars are interspecific hybrids with an aneuploid, highly heterozygous polyploid genome. The complexity of the sugarcane genome is the main obstacle to the use of marker-assisted selection in sugarcane breeding. Given the promising results of recent studies of plant genomic selection, we explored the feasibility of genomic selection in this complex polyploid crop. Genetic values were predicted in two independent panels, each composed of 167 accessions representing sugarcane genetic diversity worldwide. Accessions were genotyped with 1,499 DArT markers. One panel was phenotyped in Reunion Island and the other in Guadeloupe. Ten traits concerning sugar and bagasse contents, digestibility and composition of the bagasse, plant morphology, and disease resistance were used. We used four statistical predictive models: bayesian LASSO, ridge regression, reproducing kernel Hilbert space, and partial least square regression. The accuracy of the predictions was assessed through the correlation between observed and predicted genetic values by cross validation within each panel and between the two panels. We observed equivalent accuracy among the four predictive models for a given trait, and marked differences were observed among traits. Depending on the trait concerned, within-panel cross validation yielded median correlations ranging from 0.29 to 0.62 in the Reunion Island panel and from 0.11 to 0.5 in the Guadeloupe panel. Cross validation between panels yielded correlations ranging from 0.13 for smut resistance to 0.55 for brix. This level of correlations is promising for future implementations. Our results provide the first validation of genomic selection in sugarcane.

New Insights into Esca of Grapevine: The Development of Foliar Symptoms and Their Association with Xylem Discoloration.

A new study on the development of foliar symptoms of esca was carried out from 2004 to 2006 in five mature vineyards in Aquitaine, France. Symptoms were monitored for severity and changes over time. Initial foliar symptoms were characterized by the presence of drying zones or discolorations (reddening or yellowing), which are symptoms that have also been attributed to Black Dead Arm (BDA). Then, the less-severely affected leaves persisted throughout the summer and developed into typical "tiger-stripe" symptoms of esca. The most severely symptomatic leaves fell soon after symptoms appeared. Severely diseased vines showed typical apoplectic or acute forms of esca that did not differ from the severe BDA forms. The appearance of leafsymptomatic vines increased uniformly over time, reaching a maximum incidence by the end of July. A second survey in 41 European and Lebanese vineyards showed that longitudinal discolorations were visible under the bark of 95% of the vines showing foliar esca symptoms. These wood symptoms, also previously attributed to BDA, appeared as xylem orange-brown stripes. Thus, foliar symptoms of esca showed transitory phases which overlapped with some BDA descriptions. Most of these symptoms, in the west-palearctic regions that were investigated, were commonly associated with the presence of one or several xylem discolorations.

"Global" and "local" predictions of dairy diet nutritional quality using near infrared reflectance spectroscopy.

The objective of the study was to evaluate performance of classic (global) and innovative (local) calibration techniques to monitor cattle diet, based on fecal near infrared reflectance spectroscopy (NIRS). A 3-yr on-farm survey (2005-2008) was carried out in Vietnam and La Reunion Island to collect animal, feed intake, and feces excretion data. Feed and feces were scanned by a Foss NIRsystem 5000 monochromator (Foss, Hillerød, Denmark) to estimate diet characteristics and nutrient digestibility. A data set including 1,322 diet-fecal pairs was built and used to perform global and local calibrations. Global equations gave satisfactory accuracy [coefficient of determination (R(2)) >0.8, 10% ≤ relative standard error of prediction (RSEP) ≤20%], whereas local equations gave good accuracy (R(2) >0.8, RSEP <10%) or excellent accuracy (R(2) >0.9, RSEP <10%) for the prediction of diet intake, quality, and digestibility. When validating the equations using the external individual data, both techniques were robust, with similar RSEP (8%) and R(2) (0.82) values. The predictive performance of global and local equations was improved (RSEP = 5% and R(2)=0.90) when averaged animal data from farm, visit, and similar milk production were used. In particular, local equations reduced RSEP by 43% and increased R(2) by 15%, on average, compared with those obtained from individual data. The low RSEP (4%), high R(2) (0.96), and good ratio performance deviation (RPD=5) illustrated the excellent accuracy and robustness of the local equations. Findings suggest the ability of fecal NIRS to successfully and more accurately predict diet properties (intake, quality, and digestibility) with local calibration techniques compared with classic global techniques, especially on an averaged data set. Local calibration techniques represent an alternative promising method and potentially a decision support tool to decide whether diets meet dairy cattle requirements or need to be modified.

Role of adiponectin receptors, AdipoR1 and AdipoR2, in the steroidogenesis of the human granulosa tumor cell line, KGN.

BACKGROUND: Adiponectin is involved in the regulation of energy homeostasis and more recently in the reproductive functions. We have previously shown that adiponectin receptors (AdipoR1 and AdipoR2) are expressed in human granulosa cells. However, it remains to be investigated whether both AdipoR1 and AdipoR2 or only one of these receptors serve as the major receptor(s) for adiponectin in human granulosa cells. METHODS: The RNA interference (RNAi) technology was used to specifically knockdown the expression of either AdipoR1 or AdipoR2. Progesterone and estradiol levels in the conditioned media were measured by radioimmunoassay, and determination of cell proliferation by tritiated thymidine incorporation. The levels of adiponectin receptors and proteins involved in the steroidogenesis and in the signalling pathways were examined by western blot. RESULTS: We generated AdipoR1 (R1) and AdipoR2 (R2) knockdown KGN cell lines. R1 cells were apoptotic and had increased expression levels of cleaved caspase 3 and decreased levels of BAD phosphorylation and PCNA as compared with control or parental KGN cells. R2 cells had similar morphology to control or KGN cells. However, they produced less progesterone and estradiol and expressed lower levels of StAR protein in response to FSH or IGF-1 stimulation compared with control cells. Furthermore, the increase of MAPK ERK1/2 phosphorylation in response to human recombinant adiponectin and FSH was lower in R2 than control cells. CONCLUSIONS: In the human granulosa KGN cell-line, AdipoR1 seems to be involved in the cell survival whereas AdipoR2, through MAPK ERK1/2 activation, may be implicated in the regulation of steroid production.

Tobacco rattle virus mediates gene silencing in a plant parasitic root-knot nematode.

Root-knot nematodes (RKNs) are sedentary biotrophic parasites that induce the differentiation of root cells into feeding cells that provide the nematodes with the nutrients necessary for their development. The development of new control methods against RKNs relies greatly on the functional analysis of genes that are crucial for the development of the pathogen or the success of parasitism. In the absence of genetic transformation, RNA interference (RNAi) allows for phenotype analysis of nematode development and nematode establishment in its host after sequence-specific knock-down of the targeted genes. Strategies used to induce RNAi in RKNs are so far restricted to small-scale analyses. In the search for a new RNAi strategy amenable to large-scale screenings the possibility of using RNA viruses to produce the RNAi triggers in plants was tested. Tobacco rattle virus (TRV) was tested as a means to introduce double-stranded RNA (dsRNA) triggers into the feeding cells and to mediate RKN gene silencing. It was demonstrated that virus-inoculated plants can produce dsRNA and siRNA silencing triggers for delivery to the feeding nematodes. Interestingly, the knock-down of the targeted genes was observed in the progeny of the feeding nematodes, suggesting that continuous ingestion of dsRNA triggers could be used for the functional analysis of genes involved in early development. However, the heterogeneity in RNAi efficiency between TRV-inoculated plants appears as a limitation to the use of TRV-mediated silencing for the high-throughput functional analysis of the targeted nematode genes.

A call to incorporate the prevention and treatment of geriatric disorders in the management of diabetes in the elderly.

The prevalence of type 2 diabetes increases with age. However, the management of diabetes in the elderly has received surprisingly little attention. Diabetes in the elderly is associated with a high risk of geriatric syndromes including malnutrition and sarcopenia, functional impairments, falls and fractures, incontinence, depression and dementia. Tight glycaemic control for the prevention of vascular complications is often of limited value in the elderly. However, glycaemic control and non-pharmacological therapy may prevent diabetes symptoms and delay geriatric syndromes. The prevention, screening and treatment of both conventional diabetic complications and geriatric syndromes should be integrated in a management plan to optimize the patients' overall health status and quality of life.

Self-monitoring of blood glucose in people with type 1 and type 2 diabetes living in France: the Entred study 2001.

OBJECTIVE: To describe the practice of self-monitoring blood glucose (SMBG) testing and to determine factors linked to SMBG in people with diabetes living in France. METHODS: The 2001 Entred study, a French national survey of people being treated for diabetes, is based on a representative sample of 10,000 adults who claimed reimbursement for oral hypoglycaemic agents and/or insulin in October to December 2001 and who were randomly extracted from the database of the major National Health Insurance System. A questionnaire was mailed to all these people and was returned by 36% of them, who were then classified into three groups: type 1 diabetes (T1D, N=235); type 2 diabetes treated with insulin (iT2D, N=635); and type 2 diabetes treated with oral hypoglycaemic agents (oT2D, N=2689). Factors associated with SMBG were analyzed using logistic regression models with a step-by-step forward approach. RESULTS: HbA(1c) was greater than or equal to 8% in 42% of people with T1D, 48% of those with iT2D and 21% of those with oTD2. Almost all of those treated with insulin performed SMBG. The frequency of self-monitoring was higher in T1D than in iT2D. In T1D, 58% of people reported they took at least three tests a day, as recommended in guidelines, which was more frequent in those who knew what HbA(1c) meant and in women. In iT2D, 74% reported that they took at least two tests a day, as recommended, and it was more frequent in those who knew what HbA(1c) meant, who reported at least one severe hypoglycaemic episode in 2001 and who received dietary advice from their practitioner. In oT2D, 38% reported using SMBG (six tests a week on average), even though no official recommendation had been provided for these patients. SMBG was also more frequent in patients being treated with multiple oral hypoglycaemic agents, in those who benefited from a waiver of co-payment due to a chronic disease and in those, who had visited a diabetes specialist in 2001, reported they knew what HbA(1c) meant, received dietary advice and reported at least one severe hypoglycaemic episode in 2001 and/or a history of diabetes complications. CONCLUSION: In France, as per the official recommendations, almost all people on insulin treatment use a SMBG device while, overall, their glucose control remains poor. More than one-third of those with oT2D regularly perform SMBG. In only 3% of people, the regular use of SMBG does not appear to be related to any special needs or events (such as insulin treatment, occurrence of severe hypoglycaemia or chronic complications).

Relationships between chemical properties of larval media and development of two Stomoxys species (Diptera: Muscidae) from Reunion Island.

The development of two cattle pests, Stomoxys calcitrans L. and Stomoxys niger niger Macquart (Diptera: Muscidae), was studied in the laboratory using seven potential larval media from a dairy farm on Reunion Island. The media were six types of cattle feed and an old manure medium. Egg-to-adult survival, duration of development, and adult live weight at emergence were determined for both fly species on each medium. The media were analyzed for pH, nitrogen, organic matter, and structural compounds (cellulose, hemicellulose, lignin). For S. calcitrans, immature survival was significantly higher on sugarcane leaves, Rhodes grass, and elephant grass; for S. niger, survival was significantly higher on the same substrates plus sugarcane tops. These substrates were characterized by slightly acid pH values (range, 5.4-6.0). In both species, there were significant bell-shape relationships between immature survival and substrate pH. The developmental time of both fly species was significantly shorter on Rhodes grass, Rhodes grass hay, and elephant grass. These substrates were characterized by high cellulose contents and low soluble organic fractions. In both species, there were significant linear relationships between developmental time and cellulose content of substrates. Similarly, there were significant linear relationships between adult live weight and cellulose content of substrates. The C:N ratio of the most favorable substrates was highly variable. Although the relationships revealed in this study do not establish causation, it is suggested that pH and cellulose content may have direct and indirect effects on Stomoxys development.

Postcalving factors affecting conception risk in Holstein dairy cows in tropical and sub-tropical conditions.

The objective was to identify postpartum risk factors between nutritional imbalance and health disorders affecting first-service conception risk (FSCR) in 21 commercial Holstein herds in Reunion Island. Multivariate logistic-regression models including herd as a random effect were used to analyze the relationship between FSCR and energy status, nitrogen status, hepatic function, mineral deficiencies, and postpartum health disorders. Two models (A and B) were built on two subsets of data (n=446 and n=863) with risk indicators measured during the first month of lactation and around time of first service, respectively, adjusted for season, breed, parity, origin, milk yield, calving to first service interval (CS1), and type of estrus (spontaneous vs. induced). The averaged conception risk was 0.266+/-0.015 (n=913) (mean+/-S.E.M.). In both models, FSCR was decreased by CS1 < or = 60 d and induced estrus. In model A, FSCR was decreased (p<0.05) for cows with mean cumulative 100 d daily milk yield < or =23 kg/d and >27 kg/d, with losses of body condition score >1.5, and with retained placenta. In model B, FSCR was decreased (p<0.05) for cows inseminated during wet season, previously raised out of the farm as nulliparous, with blood magnesium concentration < or =0.9 mmol/L, and for high-yielding cows (100 d milk yield > 27 kg/d) with glutamate deshydrogenase>17 UI/L. Hence, high-body-lipid mobilization during the first month of lactation was a strong nutritional predictor of low FSCR together with liver damage in high-yielding cows. Interestingly, our models revealed that infertility is better related to nutritional factors than to postpartum health disorders occurrence.

[Elastosonography of thyroid lesions]. / Elastographie ultrasonore des lésions thyroïdiennes.

UNLABELLED: While ultrasound is the imaging modality of choice for diagnosis of thyroid lesions, characterization remains limited and tissue diagnosis frequently is required for management. The availability of additional tools such as elastography may improve lesion characterization and direct management. MATERIALS AND METHODS: A total of 96 patients (11 males and 85 females; 58+/-24 years) referred for fine needle aspiration (FNA) of mainly solid thyroid nodules 9-32 mm in diameter underwent conventional US and elastosonography. Results on elastography were correlated with histological results from FNA and classified as follows: suspected malignant lesion, suspected benign lesion, suspicious, indeterminate. RESULTS: The nodules were classified as follows: 95 nodules were soft (classes I and II) and 13 nodules were hard (classes III and IV). No cancers were detected in class and II lesions and 6 cancers were detected in class III and IV lesions. FNA provided insufficient cellular material for diagnosis in 5 class I-II nodules and 2 class III-IV nodules. CONCLUSION: Real-time elastosonography may be a useful adjunct to conventional US in the evaluation and characterization of thyroid nodules allowing identification of patients at high risk of malignancy for whom tissue diagnosis and/or close follow-up is required.

Non pharmacological treatments in elderly diabetics.

Among the therapeutic resources available for the elderly diabetic, diet and exercise are often neglected because patients are reluctant to make changes and significant amount of time of healthcare providers and physicians is required for patient education. Diet and exercise work in synergy to lower the biological parameters of diabetes control. Diet in the elderly diabetic patient is based essentially on the nutritional recommendations for the elderly subject, diabetic or non diabetic. Recent studies on exercise demonstrate the value of resistance training in increasing muscle mass, preferably over endurance training. The benefits obtained also involve autonomy and quality of life. Taking up exercise is not devoid of disadvantages because of the frequent co-morbidity at this age.

Five-year predictive factors of type 2 diabetes in men with impaired fasting glucose.

AIM: The outcome of 743 French men (age 20-60) with impaired fasting glucose (IFG) [blood glucose 6.1-6.9 mmol/l] at T1 was evaluated 5 years later, at T2. METHODS: Personal and family medical history, smoking, nutritional habits, physical activity, blood pressure, body mass index (BMI) and waist girth, fasting biological data were collected at T1 and T2. Predictive factors for developing diabetes were compared between those who returned to normal fasting glucose and those who had diabetes, before and after adjustment for age, BMI, glucose and triglyceride (TG) levels. RESULTS: At T2, 44%, 39%, 17% were classified as normal fasting plasma glucose (FPG), IFG or diabetic, respectively. Odd ratios for diabetes were 4.2 for men with a family history of diabetes (FHD), 3.4 if BMI > or = 25 kg/m(2), 2.9 if waist girth > or = 90 cm, 2.8 if TG > or = 2 mmol/l and 1.9 if no daily dairy products were eaten. Still significant after adjustment for age, BMI, glucose and TG levels were: FHD (P=0.001), no daily dairy products (P=0.001), high alcohol intake (P=0.02) and low physical activity (P = 0.02). CONCLUSION: No daily dairy products, high alcohol intake and low physical activity were independent predictive factors of a 5-year onset of diabetes after adjusting for BMI, FHD, triglyceride and glucose levels at baseline. For a better prevention of diabetes, these findings give clues for behaviour modifications as soon as IFG is detected.

Ageing and reproduction: is polycystic ovary syndrome an exception?

BACKGROUND: Polycystic ovary syndrome (PCOS) is a frequent cause of infertility. Despite an impressive number of reports, few have evaluated the influence of age upon fertility. We present the outcomes of three infertile women with PCOS who achieved spontaneous pregnancies when ageing. CASE REPORTS: Three patients with PCOS were monitored for more than 20 years. PCOS was confirmed by clinical data (oligo/amenorrhoea, infertility, hirsutism), hormonal measures and ovarian ultrasonography. All three infertile patients were treated for several years using numerous ovulation induction protocols with varying responses. When ageing, they gained more regular cycles and spontaneously became pregnant at 39, 40 1/2 and 36 years of age, more than 5 years after induction treatment was stopped, and in spite of increasing weight in each of them. CONCLUSIONS: These clinical observations suggest improved fertility in some PCOS ageing women. The positive impact of ageing on cycle regularisation in PCOS has recently been claimed but the fertility outcome was not evaluated. Ovary ageing results in diminution of the follicular cohort in both normal and PCOS women, associated with decreased inhibin B and anti-müllerian hormone (AMH) levels. Lower inhibin B levels induce FSH enhancement, with a rise in FSH rate per follicle which may determine better follicle maturation, regular and ovulatory cycles in PCOS ageing women. The best proof of this improved fertility was the occurrence of spontaneous pregnancies which never occurred previously.

[A Multiple endocrine neoplasia type-1 observatory in a French-speaking area. A tool from the Endocrine Tumor study Group (GTE)]. / Observatoire francophone des néoplasies endocriniennes multiples de type 1. Un outil du Groupe d'étude des Tumeurs Endocrines (GTE).

Wermer's syndrome or Multiple Endocrine Neoplasia Type-1 (MEN1) is an autosomal dominant inherited disease, related to mutations in MEN1, an approximately 10-kb gene encoding menin, localized on chromosome 11q13. The Endocrine Tumor Group (GTE) has set up a MEN1 observatory of 1001 regularly followed MEN1 cases. This observatory aims at registering and evaluating MEN1 cases in a large cohort. Any new study on a particular unexplored aspect of the disease may be proposed by a physician to the GTE. This article describes the way to diagnose a new MEN1 case and to register it. Procedures for participating in a new study are presented. Some original results are quoted.

Clinical characterization of familial isolated pituitary adenomas.

CONTEXT: Familial pituitary adenomas occur rarely in the absence of multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC). OBJECTIVE: Our objective was to characterize the clinical and genealogical features of non-MEN1/CNC familial isolated pituitary adenomas (FIPA). DESIGN AND SETTING: We conducted a retrospective study of clinical and genealogical characteristics of FIPA cases and performed a comparison with a sporadic population at 22 university hospitals in Belgium, Italy, France, and The Netherlands. RESULTS: Sixty-four FIPA families including 138 affected individuals were identified [55 prolactinomas, 47 somatotropinomas, 28 nonsecreting adenomas (NS), and eight ACTH-secreting tumors]. Cases were MEN1/PRKAR1A-mutation negative. First-degree relationships predominated (75.6%) among affected individuals. A single tumor phenotype occurred in 30 families (homogeneous), and heterogeneous phenotypes occurred in 34 families. FIPA cases were younger at diagnosis than sporadic cases (P = 0.015); tumors were diagnosed earlier in the first vs. the second generation of multigenerational families. Macroadenomas were more frequent in heterogeneous vs. homogeneous FIPA families (P = 0.036). Prolactinomas from heterogeneous families were larger and had more frequent suprasellar extension (P = 0.004) than sporadic cases. Somatotropinomas occurred as isolated familial somatotropinoma cases and within heterogeneous FIPA families; isolated familial somatotropinoma cases represented 18% of FIPA cases and were younger at diagnosis than patients with sporadic somatotropinomas. Familial NS cases were younger at diagnosis (P = 0.03) and had more frequently invasive tumors (P = 0.024) than sporadic cases. CONCLUSIONS: Homogeneous and heterogeneous expression of prolactinomas, somatotropinomas, NS, and Cushing's disease can occur within families in the absence of MEN1/CNC. FIPA and sporadic cases have differing clinical characteristics. FIPA may represent a novel endocrine neoplasia classification that requires further genetic characterization.