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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 aAO 2 < 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

To identify important factors in the differential diagnosis of renal cysts associated with hyperechogenic kidneys.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.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.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.

Objective To define a specific sonographic pattern for the appearance of the kidneys in fetuses affected by Meckel–Gruber 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. Copyright © 2006 ISUOG. Published by John Wiley & Sons, Ltd.

Resume La sequestration pulmonaire est une anomalie congenitale, definie comme un territoire pulmonaire non fonctionnel intra ou extra-lobaire, separe de ses connections bronchiques et vasculaires normales. Sa vascularisation est assuree par une artere systemique aberrante. En antenatal, l’echographie bidimensionnelle reste la premiere methode d’investigation, mais n’identifie pas toujours le vaisseau systemique aberrant pour lequel le doppler couleur apparait sensible. L’IRM avec ses sequences rapides ponderees T2 et T1 a surtout, en l’absence d’injection, un interet dans le diagnostic differentiel avec d’autres pathologies intra-thoraciques. En post-natal, l’arteriographie n’est plus pratiquee, laissant place au scanner volumique avec obtention de reconstructions MPR, MIP, 3D VRT de qualite qui a permis de modifier l’approche diagnostique de cette malformation et d’en ameliorer la prise en charge. L’analyse de quelques observations privilegiees et une revue de la litterature nous permet de preciser l’apport de ces differentes techniques d’imagerie recentes (doppler, scanner multi-detecteurs, IRM) tant au niveau du depistage antenatal que du bilan post-natal.

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. Copyright © 2004 ISUOG. Published by John Wiley & Sons, Ltd.

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. Copyright © 2004 ISUOG. Published by John Wiley & Sons, Ltd.

Mesoblastic nephroma (Bolande's tumour) is a rare renal tumour, but, overall, is the most frequent benign renal tumour in childhood and the most frequent renal tumour during the first year of life. It is increasingly diagnosed with US during the fetal period. We report the findings of two patients who showed the "ring" sign, an anechoic ring surrounding the tumour and typical of mesoblastic nephroma. We identified a Doppler signal in the ring in both cases, and pathology confirmed the vascular origin of this sign.

This experimental investigation reports the convective heat transfer coefficient around the rotor of a transonic turbine stage. Both time-resolved and time-averaged aspects are addressed. The measurements are performed around the rotor blade at 15%, 50% and 85% span as well as on the rotor tip and the hub platform. Four operating conditions are tested covering two Reynolds numbers and three pressure ratios. The tests are performed in the compression tube turbine test rig CT3 of the von Karman Institute, allowing a correct simulation of the operating conditions encountered in modern aero-engines. The time-averaged Nusselt number distribution shows the strong dependence on both blade Mach number distribution and Reynolds number. The time-resolved heat transfer rate is mostly dictated by the vane trailing edge shock impingement on the rotor boundary layer. The shock passage corresponds to a sudden heat transfer increase. The effects are more pronounced in the leading edge region. The increase of the stage pressure ratio causes a stronger vane trailing edge shock and thus larger heat transfer fluctuations. The influence of the Reynolds number is hardly visible.Copyright © 2002 by ASME

Resume Le syndrome transfuseur–transfuse est une complication severe des grossesses gemellaires monochoriales. En l’absence de traitement, l’evolution aboutit souvent au deces des jumeaux. Observations. – Deux couples de jumeaux transfuseur–transfuse ont ete etudies par echocardiographie. Le diagnostic n’a ete pose qu’a 27 et 26 semaines d’amenorrhee (SA). A la naissance, a 31 SA, le premier transfuse presentait une dysfonction biventriculaire severe avec bas debit cardiaque et augmentation des resistances vasculaires systemiques, responsables d’une hypertension arterielle systemique. L’evolution a ete favorable avec un traitement anti-hypertenseur (nicardipine), tonicardiaque vasodilatateur (dobutamine et enoximone) et une exsanguino-transfusion partielle en raison d’une polyglobulie menacante. A 28 SA, le deuxieme transfuse presentait une dilatation des cavites cardiaques avec dysfonction biventriculaire. L’evolution a ete rapidement favorable avec un traitement par dobutamine. Conclusion. – Apres extraction des transfuses une amelioration rapide de leur fonction cardiaque a ete observee, alors que l’evolution naturelle, in utero, aurait ete probablement le deces.

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.

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.

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 (R2 = 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. Conclusion: The psycho-oncology team should focus on helping doctors and nurses identify the patients’ psychological problems, dealing with them or making a referral.

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

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.

PURPOSE To evaluate the accuracy of voiding urosonography (VUS) compared to X-ray voiding cystoureterography (VCUG) for the detection and grading of vesicoureteral reflux (VUR) by standardised reading of digital clips obtained from VUS and digital images from VCUG. MATERIALS AND METHODS Approval by the ethics committee was obtained, and written, informed consent was given. 130 children (94 girls and 36 boys, mean age of 4.4 years) underwent VUS using Levovist (Schering, Germany) prior to VCUG. Digital VUS clips and digital VCUG images were reviewed by two groups of two radiologists. Results were mainly analysed in terms of reno-ureteral units (RUUs). Intra and interobserver reproducibility was estimated by calculation of kappa coefficient. Calculation of sensitivity and specificity of VUS was made in comparison with VCUG. RESULTS Intraobserver reproducibility was good to moderate for the detection of VUR using VUS (kappa = 0.67 and 0.53 for each reviewer respectively) and good for the grading of reflux (kappa = 0.64 and 0.70). Interobserver reproducibility was excellent for the detection and grading of VUR using VCUG (kappa = 0.89 and 0.91) but good to moderate for VUS (kappa = 0.73 and 0.51). Compared to VCUG, sensitivity and specificity of VUS for the detection of VUR were 62.7 % and 83.4 %. Concordance for grading was moderate, with a higher grading using VUS. CONCLUSION Real-time evaluation and diagnosis based on the review of VUS digital clips is achievable. However, there is a need for standardisation of digital records if a second reading by another radiologist or consideration by urologists is needed. Recent advances in US technology and the use of second-generation contrast agents would be promising to improve the feasibility, reproducibility and accuracy of the method.

The aims of this review article were to clarify the steps that may lead to a proper diagnosis of fetal and neonatal renal cystic diseases. All the hereditary cystic diseases are reviewed and a classification is proposed. The various sonographic patterns that can be used to ascertain the diagnosis are also reviewed. Finally, tables with differential diagnoses are presented to help the reader in the work-up of such pathologies.

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.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.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.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.

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.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.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.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.

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.Infants below 32 wk gestational age (GA) with hypoxic respiratory failure and aAO20.22 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 MPBFV0.2 m/s.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).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.

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.These cases show that even severe cardiac dysfunction may be reversed after birth unlike in utero natural evolution.

Topical antiviral drugs are frequently used. Repeated applications on a skin injured by the viral infection can lead to irritating dermatitis or contact eczema. This secondary effect is often unrecognized because imputed to an increase of the initial dermatosis.Case 1 - A 58 year-old woman had an eczema around the mouth after the use of Zovirax(R) cream (acyclovir). The patch test with this cream was positive (++ at 96 h) but the detail of all the constituents was negative. Case 2 - A 39 year-old woman had an eczema surrounding the mouth after each use of Zovirax(R) cream and Cuterpes(R) (ibacitabin). The patch tests were doubtful for Zovirax(R) cream, positive for Cuterpes(R) (++ at 96 h) and ibacitabin 1 p. 100 and 10 p. 100 in petrolatum (++ at 96 h). Case 3 - A 58 year-old man had an acute eczema of the face after the use of Zovirax(R) cream and Vira-MP(R) (vidarabin phosphate). The patch tests were positive for the both antiviral topical drugs but negative for each one of their constituents.Contact dermatitis to antiviral topical drugs are rare, due to active molecule or its excipients. The propyleneglycol, component found in the three antiviral drugs, can induce irritation or allergy. In the most of cases, like in our case number 1 and 3, the patch tests with each constituents of the topical drugs were negative, we conclude to a compound allergy.

Homocysteine is a sulfur amino acid occurring in methionine intermediary metabolism. It was recently shown to be a vascular risk factor even without high serum levels.A 29-year-old woman had painful plantar nodules and necrosis of the fifth toe on the right foot with cyanosis of the forefoot. The histology examination of a nodule biopsy gave the diagnosis of subcutaneous thrombophlebitis. Vascular explorations disclosed advanced-stage arteriopathy of the leg. The methionine loading test was abnormal and search for the heterozygous form of cystathionine ss was positive.Different diagnoses could be possible for cases associating an inflammatory venous disease and stage IV arteritis. Latent hyperhomocysteinemia was diagnosed in this case on the basis of the methionine loading test and identification of the heterozygous form of cystathionine ss. Patients with latent hyperhomocysteinemia have an increased risk of both thrombosis and arteritis. Our case emphasizes the importance of searching for hyperhomocysteinemia in patients with early arterial and/or venous thromboembolism, either as repeated episodes or occurring in an unusual territory.

Objectifs Analyse d’une cohorte de fœtus porteurs de reins hyperechogenes kystiques et dont le diagnostic final de nephropathie est connu. Materiels et methodes Etude multicentrique concernant 93 fœtus avec reins hyperechogenes. Analyse retrospective des signes echographiques de 30 d’entre eux qui presentaient des kystes renaux : 3 cas de polykystose autosomique dominante (PKAD), 9 cas de polykystose autosomique recessive (PKAR), 3 cas de Bardet-Biedl (BB), 9 cas de Meckel-Gruber (MG), 3 cas d’Ivemark II, 1 cas de Jarcho-Levine, 1 cas de Beemer et 1 cas de syndrome MG-like. Resultats Un tiers des fœtus avait des kystes renaux (30/93). Les kystes etaient presents dans 11 % des PKAD (3/28), 29 % des PKAR (9/31), 27 % des BB (3/11), 100 % des 9 MG, 50 % des 6 cas d’Ivemark II et dans tous les 3 cas d’autres syndromes (Jarcho-Levine, Beemer et MG-like). Aucun des 5 cas de trisomie 13 n’avait des kystes. Aucune malformation n’etait associee dans les 12 cas de polykystoses. Dans tous les autres cas, il existait des malformations souvent evocatrices comme une polydactylie dans les syndromes de BB et de Beemer, un defect occipital +/-Dandy-Walker dans les MG, des anomalies thoraciques ou vertebrales dans le syndrome de Jarcho-Levine. Conclusion L’association reins hyperechogenes et kystes renaux n’est pas rare. La cle du diagnostic repose souvent sur les malformations associees. En l’absence de malformation, le diagnostic principal reste celui d’une polykystose recessive ou dominante.

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.

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.

Background: Since 1 March 2010 “Galilee”, the first palliative home care unit in Hellas has been caring for patients with advanced cancer, enabling them to die at home, if they wished. This study presents prevalent reported symptoms of patients been cared in the last period of their life. Material and Methods: 23 cancer patients who were cared for until their death, by “Galilee” Palliative Home Care Unit, during the first year of the pilot unit’s operation were retrospectively studied. Data collection included patients’ demographic and clinical characteristics, their performance status measured by ECOG scale (0−4) and symptom severity assessment by Edmonton Symptom Assessment System Revised (ESAS-r), a series of nine visual analogue scales (0−10). Symptoms were evaluated at the time of referral to the service (T0), and at the last report before death (T1). Results: The majority of participants were female (60.9%) with a mean age 64.5 years and a poor mean performance status (3.39). Genital cancer was the most prevalent diagnosis (26.1%), followed by breast cancer (17.4%). About one third of patients received simultaneous aggressive treatment. The mean length of palliative home care was 78.87 days. The majority of patients died at home (65.2%). Their primary care giver was mainly the wife/husband (39.1%) or daughter (26.1%). Symptoms that scored >5 by the patients at their referral to the unit were depression (mean(T0) = 5.84), anxiety (mean(T0) = 5.60) and lack of appetite (mean(T0) = 5.32). Depression (mean(T1) = 5.46) and anxiety (mean(T1) = 5.38) followed by tiredness (mean(T1) = 5.14) were still the main prevalent symptoms at the last measurement before patients’ death. Contrary, nausea (mean(T0) = 0.85, mean(T1) = 0.93), and drowsiness (meanT0) = 1.90, mean(T1) = 1.54) were reported as the least prevalent symptoms. Although there was an improvement in all symptoms’ evaluations between the two measurements, except tiredness and nausea, there was no statistically significant difference (p > 0.050). None of patients’ demographic and clinical characteristics, nor symptoms evaluations was associated with the place of death (p > 0.050), except for depression and performance status. Patients who died at home reported less depression (p = 0.014) and had a worse performance status (p = 0.047) than those who died at hospital. Conclusion: Despite the small study sample these preliminary findings reveal that psychological symptoms play a significant role during the dying process. Further research is needed to better establish patients’ symptoms management by the palliative home care unit.

Gelatinase was purified from DFP treated human neutrophils which have been stimulated by the chemotactic peptide, N-formyl-methionyl-leucylphenylalanine. The secreted gelatinase was purified in two major steps: a gelatinase enriched fraction was recovered after a specific immunoadsorption of contaminant proteins from cytosol and immunoadsorption of proteins from specific or azurophilic granules; then gelatinase was isolated by affinity chromatography and FPLC gel filtration. Some kinetic properties of the Mr 94,000 purified enzyme were investigated.

Objectifs L’European Association of Urology, dans ses guidelines, indique que, devant une dilatation des voies urinaires superieures : « Conventional voiding cystouretrography is the method of choice for primary time diagnostic procedure ». Cette procedure est de plus en plus remise en cause car la decouverte en periode neonatale d’un reflux sans anomalie parenchymateuse ne parait plus utile et l’antibioprophylaxie n’est plus recommandee. Les defenseurs de la cystographie mettent en avant que seul cet examen permet le depistage d’un obstacle sous-vesical. L’objectif de l’etude est de determiner si l’echographie permet une presomption de diagnostic de valve afin d’eviter au maximum la pratique d’un examen invasif et inutile. Methodes Dans une etude retrospective sur 38 cas de valve de l’uretre posterieur confirme par la cystographie retrograde et l’endoscopie, l’analyse de l’echographie realisee dans le mois qui precede le diagnostic a ete faite sur : - les voies excretrices superieures - la taille de la vessie - la paroi vesicale - l’uretre posterieur. Resultats La detection des anomalies urinaires a ete evoquee en periode prenatale dans 31 cas et, en postnatal, dans 7 cas. Le detrusor est apparu epaissi dans 23 cas. Il existait une megavessie dans 11 cas et une dilatation de l’uretre posterieur a ete retrouvee dans 8 cas. Dans les 10 cas ou aucune anomalie vesicale n’a ete decrite, il existait une dilatation pyelique, pyelo-calicielle ou pyelo-ureterale dans 9 cas. Chez 4 sujets, ont ete retrouves, des signes echographiques de dysplasie parenchymateuse. Un seul patient avait une echographie normale. La sensibilite des signes echographiques vis-a-vis de la suspicion de diagnostic de VUP au niveau vesical est de 73,7 %. Conclusions En presence d’une dilatation des voies excretrices superieures reconnue en periode prenatale, la conjonction des signes echographiques renaux et vesicaux permet d’elever la probabilite de retrouver une VUP. Il n’est pas justifie de pratiquer une cystographie dans le but de diagnostiquer une valve si l’echographie realisee avec l’objectif de ce depistage ne montre pas, apres un examen attentif, d’anomalie vesicale ou parenchymateuse.

Objectifs Evaluer l’echocystographie (EC) dans le diagnostic du reflux vesico-ureteral chez l’enfant en comparaison a la cystographie retrograde (CR). Materiels et methodes Cette etude monocentrique, prospective, controlee, a inclus 113 enfants, avec realisation successive de l’EC et CR en salle de radiologie, apres sondage vesical. Une serie de 12 sequences pelviennes et renales ont ete acquises apres administration de Levovist® (Schering, 300 mg/ml) en mode harmonique sur ATL5000 (Philips) a differents temps de l’EC. Les films de CR ont ete relus par 2 radiologues referents, et les sequences digitales d’EC par 2 autres radiologues experimentes en echographie mais non entraines en EC, codant le reflux, son grade, avec consensus secondaire. Resultats Pour le diagnostic de reflux et son grade, les reproductibilites intraobservateur et interobservateur ont ete excellentes pour la CR, moderees a bonnes pour l’EC. Par rapport a la CR, les sensibilite, specificite, valeurs predictives positive et negative de l’EC ont ete de 64,7 %, 83,6 %, 50,7 % et 90 % pour le diagnostic de reflux. Conclusion L’EC est une methode dont la performance evaluee de facon independante et exterieure, apparait a la limite inferieure des series deja publiees. Non irradiante, elle peut se substituer a la CR dans certaines indications.

A case of metaphyseal dyschondroplasia is reported with the unusual feature of cone-shaped epiphyses of the tibiae and bones of the hand. An 8-year-old boy (born June 1972) was admitted in 1980 having had bowed lower limbs since the age of 4 years. The child showed reduction in stature and weight but had normal mental development. The limbs and hands were short compared with the trunk. No visceral abnormality or alopecia was present. The bilateral genu varum and genu recurvatum were considered to be secondary to the abnormal length of both fibulae. Consequently, bilateral fibular resection was performed in December 1980 and the genu varum became stabilised. We saw the child for the first time in October 1984, when dwarfism was marked. His height was 1.08 m (− 7 SD) and his weight was 21.95 kg (− 3 SD) at an age of 12 years and 4 months. At birth his weight had been 2.95 kg and he measured 47 cm in length. He had suffered from no significant disease during childhood and, in particular, had not had meningoc...