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Biomedical subjects

F Didier

Publications and source records attributed to F Didier.

At least 19 recordsLinked to original sources
PubMed2006

Meckel-Grüber syndrome: sonography and pathology.

OBJECTIVE: To define a specific sonographic pattern for the appearance of the kidneys in fetuses affected by Meckel-Grüber syndrome (MGS). METHODS: This was a retrospective analysis of 30 cases, collected from five centers, with ultrasound features suggestive of MGS. Only fetuses with a confirmed diagnosis of MGS were finally included. Analysis included a detailed evaluation of the sonographic findings and comparison with pathological follow-up. RESULTS: Seventeen cases met the pathological criteria for a diagnosis of MGS and were included in the study. In all cases, a typical sonographic pattern was seen: the kidneys were enlarged (mean, + 4.8 SD) and showed unusual corticomedullary differentiation, occurring as early as the first trimester. In most cases, the medullary areas appeared excessively large and mottled due to the presence of multiple small cysts. CONCLUSIONS: The kidneys of fetuses with MGS are enlarged, cystic and have unusual corticomedullary differentiation. These features can be observed as early as the first and early second trimesters.

Central Nervous System↗
PubMed2006

Differential diagnosis of fetal hyperechogenic cystic kidneys unrelated to renal tract anomalies: A multicenter study.

OBJECTIVES: To identify important factors in the differential diagnosis of renal cysts associated with hyperechogenic kidneys. METHODS: This was a retrospective multicenter study. We identified 93 fetuses presenting between 1990 and 2002 with hyperechogenic kidneys and which had a diagnosis of nephropathy confirmed later. We analyzed retrospectively the prenatal ultrasound findings of those fetuses which were found sonographically to have renal cysts. RESULTS: Of the 93 fetuses presenting with hyperechogenic kidneys and with a later diagnosis of nephropathy, there were 28 with autosomal dominant polycystic kidney disease (ADPKD), 31 with autosomal recessive polycystic kidney disease (ARPKD), 11 with Bardet-Biedl syndrome, nine with Meckel-Gruber syndrome, six with Ivemark II syndrome, one with Jarcho-Levin syndrome, one with Beemer syndrome and one with Meckel-like syndrome. One third of the fetuses (30/93) had renal cysts. Cystic characteristics (size, location, number) were not very useful for diagnosis; more useful was diagnosis of an associated malformation. Three (11%) of the fetuses with ADPKD had cysts, as did nine (29%) of those with ARPKD, three (27%) of those with Bardet-Biedl syndrome, all (100%) of those with Meckel-Gruber syndrome, three (50%) of those with Ivemark II syndrome, and each of the three cases with other syndromes (Jarcho-Levin, Beemer and Meckel-like syndromes). None of the cases with trisomy 13 had cysts. There were no associated malformations in the 12 cases with renal cysts and polycystic kidney disease; the other 18 cases with renal cysts were associated with malformations that were often specific, such as polydactyly in Bardet-Biedl and Beemer syndromes, occipital defect and Dandy-Walker malformation in Meckel-Gruber or Meckel-Gruber-like syndromes, and thoracic and/or vertebral abnormalities in Jarcho-Levin and Beemer syndromes. CONCLUSION: Renal cysts associated with hyperechogenic kidneys are not rare. The clue to diagnosis is the demonstration of an associated malformation. If no malformation is found, the main diagnosis remains polycystic kidney disease, i.e. ARPKD or ADPKD.

Bardet-Biedl Syndrome↗
PubMed2005

When mastectomy becomes inevitable: the nipple-sparing approach.

The preservation of the nipple areola complex (NAC) could improve the quality of life in cases of mastectomy. A novel radiosurgical treatment combining subcutaneous mastectomy with intraoperative radiotherapy is proposed. Three hundred nipple-sparing mastectomies (NSM) were performed. Invasive (58%) and in situ (42%) carcinomas were included. Clinical complications, aesthetic results, oncological and psychological results were recorded. The NAC necrosed totally in 10 cases and partially in 29 and it was removed in 12. Nine infections (3%) were observed and 10 prostheses removed. Good results were rated by 82.3% of the patients and by 84.8% of the surgeons. In 7.5% a radiodystrophy was observed. The sensitivity of the NAC recovered partially in 48%. Two local recurrences occurred outside the radiated field. Overall, we observed three metastases and no deaths. Sixty-eight of the patients were satisfied with their reconstructed breast and 85.5% were satisfied having preserved the NAC.

Adult↗
PubMed2004

Antenatal renal sonographic anomalies and postnatal follow-up of renal involvement in Bardet-Biedl syndrome.

OBJECTIVES: To describe an antenatal sonographic renal pattern encountered in Bardet-Biedl syndrome, a rare autosomal recessive disorder whose definitive diagnosis is often delayed, and to describe the evolution of the sonographic appearance of the kidneys after birth. METHODS: Among a large group of fetuses with hyperechoic kidneys, we retrospectively analyzed the prenatal sonographic findings and clinical and postnatal renal sonographic evolution of 11 patients who were found to be affected by Bardet-Biedl syndrome. RESULTS: All 11 fetuses presented enlarged homogeneously hyperechoic kidneys without corticomedullary differentiation. The diagnosis was established before birth in three fetuses thanks to their familial history. It was confirmed during childhood in the remaining eight based on the development of the classic features of the syndrome. In the postnatal period, the prenatal pattern persisted for a few months in all 11 cases. The sonographic aspects of the kidneys normalized in most cases between 1 and 2 years after birth. CONCLUSIONS: In affected families, the prenatal appearance of enlarged hyperechoic kidneys without corticomedullary differentiation should prompt a diagnosis of recurrence in the family of Bardet-Biedl syndrome, especially when polydactyly is present. In non-affected families, Bardet-Biedl syndrome should be included in the differential diagnosis whenever such an appearance is discovered in utero. The postnatal evolution of the renal sonographic findings is variable and normalization generally occurs by the age of 2 years.

Bardet-Biedl Syndrome↗
PubMed2004

Prenatal sonographic patterns in autosomal dominant polycystic kidney disease: a multicenter study.

OBJECTIVE: To determine whether a specific prenatal sonographic pattern can be identified for autosomal dominant polycystic kidney disease (ADPKD) and if so whether it would be helpful in orienting complementary analysis, properly counseling parents and adapting pregnancy management. METHODS: A retrospective multicenter study was conducted in four prenatal diagnostic centers. The records of fetuses with a prenatal ultrasound examination revealing abnormal kidneys and with a final diagnosis of ADPKD were analyzed. Ultrasound analysis included: amount of amniotic fluid, bladder size, renal length, presence or absence of renal cysts and size of renal pelves, and was focused on parenchyma echogenicity and status of corticomedullary differentiation. Postnatal follow-up was reviewed. RESULTS: Of the 27 patients included in the study, 25 had hyperechogenic renal cortex and 20 had hypoechogenic medulla resulting in increased corticomedullary differentiation (CMD). In six cases, the medulla was hyperechogenic leading to absent or decreased CMD. One patient had normal cortical echogenicity and CMD. Renal cysts were present during the prenatal period in four patients (at 22 weeks in one case and after 30 weeks in three cases). In 12 patients, the cysts appeared after birth (within the first 6 months of postnatal life in 10 cases and by the age of 1 year in two cases). Elevated blood pressure was observed in only two cases and moderate chronic renal failure in one case. CONCLUSION: We have described the sonographic presentation in fetuses with ADPKD: moderately enlarged hyperechogenic kidneys with increased CMD. Although not specific to ADPKD, these findings should prompt familial screening. Other prenatal sonographic features (absent or decreased CMD and cortical cysts) are less frequent.

Amniotic Fluid↗
PubMed2004

Inhaled nitric oxide improves oxygenation in very premature infants with low pulmonary blood flow.

AIM: Inhaled nitric oxide (iNO) is used to reduce right-to-left extrapulmonary shunting by decreasing pulmonary vascular resistance in term or near-term infants. The objectives of this study were to determine, first, the pulmonary blood flow status of very preterm infants with hypoxaemic respiratory failure, then the response of oxygenation to iNO therapy according to pulmonary blood flow (PBF) and, finally, to verify the lack of adverse side effects of iNO on the ductus arteriosus. METHODS: Infants below 32 wk gestational age (GA) with hypoxic respiratory failure and aAO2 < 0.22 were randomized as the control or iNO group. PBF was evaluated by pulsed Doppler measurement of mean pulmonary blood flow velocity (MPBFV) in the left pulmonary artery. Low PBF (LPBF) was defined as MPBFV < 0.2 m/s. RESULTS: Seventy infants of 23 to 31 wk GA with hypoxic respiratory failure were randomized either to receive or not to receive 5 ppm iNO in addition to optimal care. Twenty-eight infants were diagnosed with LPBF (11/35 in iNO vs 17/35 in the control groups). Thirty minutes after receiving iNO the number of LPBF infants dropped to 8/35. In the iNO group, aAO2 increased significantly from 0.14 +/- 0.05 to 0.24 +/- 0.08 after iNO, but only in the LPBF infants (mean +/- SD; p = 0.027). CONCLUSION: In infants below 32 wk GA with hypoxic respiratory failure, Doppler echocardiographic assessment of LPBF seems to be able to determine which patients are likely to benefit from iNO therapy on systemic oxygenation.

Administration, Inhalation↗
PubMed2002

[Twin-twin transfusion syndrome: report of two cases with hemodynamic complications].

CASE REPORTS: We report two cases of cardiac dysfunction in twin-twin transfusion syndrome (TTTS) evaluated with serial echocardiography. Two cases of TTTS were referred at 27 and 26 weeks. At delivery at 31 weeks, the first recipient twin had evidence of severe cardiac dysfunction with decreased ventricular function and transient systemic hypertension. There was polycythaemia. Favorable outcome was observed after treatment with arterial vasodilating (nicardipine) and inotropic agents (dobutamine, enoximone), and reduction of haematocrit. At 28 weeks the other recipient twin had cardiac dilatation with hypokinetic myocardium. These alterations were cured by dobutamine. CONCLUSION: These cases show that even severe cardiac dysfunction may be reversed after birth unlike in utero natural evolution.

Adult↗
PubMed2001

Antenatal diagnosis of Multicystic Renal Dysplasia.

Multicystic Renal Dysplasia (MRD) was discovered during antenatal ultrasound examination in 138 fetuses between 1980 and 1995. Associated malformations were present in 66 % (42 % urological) and 22 % of the fetuses did not survive the pregnancy or the peri-natal period.Anatomical analysis showed a wider variety of MRD than in classical descriptions. Obstruction of the urinary tract was almost invariable. Like the hypothesis published by Beck in 1971, our view is that, with a very early obstruction of the urinary tract (during the first trimester), there is a dysplastic evolution of renal tissue, while later in pregnancy the same obstruction can induce a hydronephrosis with corticomedullary dysplasia.We advise complete neonatal urological investigation, and surgical removal of multicystic kidneys, to avoid multiple and inadequate evaluations of those children with a single functioning renal unit.

Female↗
PubMed2001

Assessment of quality of care in an oncology institute using information on patients' satisfaction.

OBJECTIVE: To evaluate the feasibility of conducting a patient satisfaction survey in the oncology hospital setting, using a multidimensional patient satisfaction questionnaire to be completed at home. METHODS: Socio-demographic and clinical data were collected for 133 consecutive patients. Patients were asked to complete the European Organisation for Research and Treatment of Cancer QLQ-C30 (version 2.0) just before hospital discharge and the Comprehensive Assessment of Satisfaction with Care at home 2 weeks after discharge. RESULTS: Respondents (73% of patients approached) were younger, hospitalized for a shorter time and presented less appetite loss, nausea and vomiting and better physical and role functioning than non-responders. The aspects of care for which patients wanted the most improvement were associated with the provision of medical information. In multivariate analyses, longer hospital stay was associated with higher satisfaction with all aspects of medical and nursing care, most probably because patients discharged early were not assured of continuity of care and lacked information regarding self-care at home. Higher global quality of life was associated with higher satisfaction with all aspects of care, suggesting the potential contribution of patient satisfaction to the patients' well-being. CONCLUSIONS: Conducting a patient satisfaction survey in an oncology hospital setting proved feasible; however, further surveys should attempt to obtain the opinion of patients with more severe physical conditions. The assessment of the patients' satisfaction provided indications for improvement of care in a particular hospital. Although the results of this study are specific to one hospital, the methods could be reproduced in other hospital settings, but may possibly lead to other conclusions.

Adult↗
PubMed2000

The potential role of rodents in the enzootic cycle of Rift Valley fever virus in Senegal.

Wild rodents (214) of fourteen species were trapped at seven sites in Senegal. Arvicanthis niloticus and Mastomys erythroleucus were among the most frequently collected species (77.2% of total capture). All rodents were examined for the presence of anti-Rift Valley fever virus (RVFV) antibody; the prevalence over all sampled species was 3.8%, varying widely with respect to species and location. Four of 14 species of rodents were found to have anti-RVFV antibodies: Rattus rattus (one positive of two tested) Mastomys huberti (13.5%), A. niloticus (4.3%), and M. erthroleucus (2.4%). The highest prevalence of anti-RVFV antibody was recorded within the enzootic area of the Senegal River delta, at Richard Toll (9.6%). A. niloticus and M. erythroleucus and a strain of laboratory-bred mice were experimentally inoculated with two strains of RVFV and examined for viremia, illness, seroconversion and mortality. A. niloticus and M. erythroleucus demonstrated a limited resistance to infection, thus potentially allowing for the replication of virus in these animals and making these species possible candidates as hosts in the maintenance cycle of RVFV in nature.

Animals↗
PubMed1999

A comprehensive assessment of satisfaction with care: preliminary psychometric analysis in an oncology institute in Italy.

BACKGROUND: Little is known about patients' perception of the quality of the care they receive in oncology hospitals. We developed a 61-item comprehensive assessment of satisfaction with care (CASC) to evaluate the competence of hospital physicians and nurses, as well as aspects of care organisation and hospital environment. The aims of this study were to define the structure of the CASC and assess the internal consistency and convergent and discriminant validity of its scales. PATIENTS AND METHODS: Three hundred ninety-five consecutive cancer patients discharged from an oncology institute in Italy were asked to complete the CASC at home and return it in a self-addressed envelope. RESULTS: Two percent of the patients refused to participate and 25% failed to return the questionnaire. Separate factor analyses of the CASC sub-scales disclosed the perceived extent of doctors' and nurses' availability, coordination, human quality, technical competence, provision of psychosocial care and information, as well as the patients' general satisfaction, perception of the organisation of their care, access and comfort. Multi-trait scaling analysis was carried out on item-grouping resulting from factor analyses. High levels of internal consistency and convergent validity were obtained but discriminant validity could be improved. CONCLUSIONS: Results of present psychometric testing of the CASC forecast adequate properties. This will be confirmed by repeating these analyses in a cross-cultural setting.

Adult↗
PubMed1999

Psychological distress in cancer patients attending the European Institute of Oncology in Milan.

BACKGROUND: The determination of the extent and specific features of the psychological distress to be expected in a cancer centre may influence the choice of interventions to be implemented for addressing these problems. This study was aimed at estimating the prevalence of psychological distress in patients attending a second reference cancer centre in Milan (Italy), and at identifying associated factors. PATIENTS AND METHODS: 190 consecutive patients were assessed within 3 days of hospital discharge using the Hospital Anxiety and Depression Scale. RESULTS: Major depressive disorders or generalised anxiety disorders were estimated in 16% of the patients. Only 2 of these patients were referred to the psycho-oncology unit, hence the psychological distress of many patients was not considered during their hospital stay. In the multiple regression analysis, independent predictors of psychological distress were female gender, experience of disturbance in family and social life due to illness, nausea and vomiting, and perception of being in a poor state of health (R(2) = 0.31, p value < 0.001), while physical functioning, fatigue and pain, significant factors in univariate analysis (p < 0. 05), sociodemographic and clinical factors were not predictors. CONCLUSIONS The psycho-oncology team should focus on helping doctors and nurses identify the patients' psychological problems, dealing with them or making a referral.

Adult↗
PubMed1996

A follow-up study of pre-natally detected primary vesico-ureteric reflux: a review of 61 patients.

OBJECTIVE: To describe the pre- and post-natal characteristics of primary vesico-ureteric reflux (VUR) in a retrospective study of babies presenting with VUR suspected from pre-natal ultrasonography. PATIENTS AND METHODS: Between 1984 and 1994. 61 children (41 boys and 20 girls) with primary VUR were followed for 2 years after the pre-natal detection of urinary tract anomalies. Patients with VUR secondary to infravesical obstruction and duplex systems were excluded. The mean age at ante-natal diagnosis was 28.4 weeks of gestation; in 37 the reflux was bilateral and 98 refluxing units were reviewed. RESULTS: According to the international classification of VUR, 8% were grade 1, 32% grade 2, 38% grade 3, 16% grade 4 and 6% grade 5. Twelve patients (22 refluxing units) underwent ureteric re-implantation. Six kidneys showed renal scars on isotope renography and two nephrectomies were carried out in patients < 2 years old: in 40 patients (64 renal units) the VUR resolved spontaneously. Of the latter, eight were grade 1, 20 grade 2, 27 grade 3 and nine grade 4: seven patients (10 refluxing units) are still being followed and awaiting a decision on treatment. CONCLUSION: This study confirms the predominance of boys in those with ante-natally suspected VUR. The spontaneous resolution during the first 2 years of life was apparent in most cases, even in those with severe reflux (grade 3-5).

Child, Preschool↗
PubMed1995

Mortality after an antenatal diagnosis of foetal uropathy.

A longitudinal study identified 987 foetal uropathies over a 13-year period. There were 147 deaths. Forty infants died as a result of a lethal uropathy in the presence of associated congenital anomalies. Sixty-six infants with an isolated uropathy died. There were 4 cot deaths, 2 obstetric related deaths, and 34 deaths caused by associated congenital anomalies. There was 1 termination of pregnancy following a false-positive diagnosis of uropathy. Of the 147 deaths, 34 occurred postnatally, 20 within 24 hours. Twenty-nine infants were spontaneously aborted. There were 78 terminations of pregnancy, 43% occurring after 24 weeks of gestation. There was complete concordance in antenatal and postnatal diagnoses in 113 (77%) cases and incomplete concordance in 19 (13%) cases. There were 14 false-negative diagnoses (9.5%). The relative frequency of different lethal congenital uropathies is detailed. Accurate in utero diagnosis of foetal uropathy and hence prediction of outcome is possible. The relatively late gestational age at time of diagnosis remains a constraint when fatal malformations would otherwise prompt termination of pregnancy.

Abnormalities, Multiple↗
PubMed1994

Patent foramen ovale with left to right shunt in bronchopulmonary dysplasia: coincidental or associated complication?

Despite the use of exogenous surfactants, the incidence of bronchopulmonary dysplasia (BPD) has not decreased as much as expected. Of 208 newborns involved in trials with Exosurf at our center, 51 had BPD. Among these newborns, 8 were found retrospectively to have secondary worsening of respiratory distress syndrome after initial improvement with surfactant treatment. Oxygen requirements decreased from 89 +/- 15% to 58 +/- 16% (mean +/- SD), respectively, before and 48 h after surfactant, and then reached a plateau of 34 +/- 10% before dramatic deterioration at 22 +/- 9 days of life. The patients had signs of cardiac failure and increased oxygen requirements of up to 76 +/- 19% within 6 h. A patent foramen ovale (PFO) was demonstrated at echocardiography with a left to right shunt at Doppler. No other cause of worsening was found. Five infants had significant improvement when treated with digoxin and furosemide. This retrospective study raises questions about the relationship between PFO and BPD that should be explored in a prospective study.

Bronchopulmonary Dysplasia↗
PubMed1994

[Imaging of bronchogenic cysts in children].

The authors report a retrospective series of 22 cases of bronchogenic cysts in children. The development of antenatal ultrasonography allowed the diagnosis in 3 cases. When the diagnosis is suggested for the time first postnatally, chest x-ray and oesophagography remain the essential initial investigations. Thoracic ultrasonography needs to be developed, as in some cases it can reveal the cystic nature of the tumour. In difficult cases, the work-up should be completed by CT scan, while keeping in mind that not all bronchogenic cysts present in the form of low-density images. A detailed review of the literature shows that mediastino-pulmonary opacities are more frequent than exclusively pulmonary images in the form of hyperlucent, fluid level or solid opacity images.

Bronchogenic Cyst↗
PubMed1993

Human neutrophil gelatinase is a collagenase type IV.

Secreted gelatinase from human neutrophils was purified as a 94 kDa polypeptide. Gelatinolytic and type IV collagenolytic activities of the purified protein were measured and compared. Immunoglobulins purified from antisera raised against gelatinase inhibited both the gelatinase and type IV collagenase activities. There was no cross-reaction in the inhibition with type I collagenase while the three metalloproteases were similarly inhibited by recombinant tissue inhibitor of metalloproteases. Purified gelatinase degraded denatured type I and native type IV collagens; there was no proteolysis of native type I collagen.

Collagenases↗