[Effect of various compositions of riboflavin eye drops on the intraoperative corneal thickness during UVA-cross-linking in keratoconus eyes]. / Entwicklung der Hornhautdicke während der UV-Vernetzungstherapie unter Anwendung verschiedener Kompositionen von Riboflavin-Augentropfen.

BACKGROUND: During the UVA-cross-linking treatment in keratoconus patients, the UVA rays are partially absorbed in the stroma of the riboflavin-loaded cornea. This effect protects the corneal endothelium from UVA irradiation damage. The intensity of UVA light reaching the endothelium is inversely correlated with corneal thickness. The common composition of riboflavin eye drops may lead to a marked reduction in corneal thickness increasing the risk of endothelial damage. PATIENTS AND METHODS: In a retrospective analysis of 23 UVA-cross-linking procedures on 23 patients we collected data about the pre-, intra- and postoperative corneal thickness (measured with ultrasound). Among these patients and depending on the preoperative state, 8 eyes received Medio Cross (TM) eye drops (group 1), 8 eyes received Medio Cross (TM) eye drops combined with riboflavin 0.1 %/methylhydroxypropylcellulose 1,5 %/NaCl 1.1 % (group 2) and 7 eyes received riboflavin 0.2 %/methylhydroxypropylcellulose 0.5 %/NaCl 0.7 % eye drops (group 3) before and during UVA irradiation. Data are presented as means ± standard deviation. A comparison of the data was performed using (one-way) ANOVA. RESULTS: The mean corneal thickness at the end of the UVA cross-linking procedure was 67 ± 9 % (means ± standard deviation) of preoperative thickness in group 1, 118 ± 14 % in group 2 and 140 ± 23 % in group 3. The values in groups 2 and 3 were significantly different from those in the reference group 1. CONCLUSIONS: Our results show a strong variability of the postoperative corneal thickness using different standard compositions of riboflavin eye drops. Further studies are needed to find a composition of riboflavin eye drops ensuring a moderate increase in intraoperative corneal thickness to protect the corneal endothelium.

Mice transgenic for exon 1 of Huntington's disease: properties of cholinergic and dopaminergic pre-synaptic function in the striatum.

In Huntington's disease (HD), neuronal loss is most prominent in the striatum leading to emotional, cognitive and progressive motor dysfunction. The R6/2 mice, transgenic for exon 1 of the HD gene, develop a neurological phenotype with similarities to these features of HD. In striatal tissue, electrically evoked release of tritiated acetylcholine (ACh) and dopamine (DA) were compared in wild-type (WT) and R6/2 mice. In R6/2 mice, the evoked release of ACh, its M2 autoreceptor-mediated maximum inhibition and its dopamine D2 heteroreceptor-mediated maximum inhibition was diminished to 51%, 74% and 87% of controls, respectively. Also, the activities of choline acetyltransferase and of synaptosomal high-affinity choline uptake decreased progressively with age in these mice. In the DA release model, however, electrical stimulation elicited equal amounts of [3H]-DA both in WT and R6/2 mice. Moreover, high-affinity DA uptake into striatal slices was similar in WT and R6/2 mice. In order to confirm these findings in vivo, intrastriatal levels of extracellular DA were measured by intracerebral microdialysis in freely moving mice: striatal DA levels were found to be equal in WT and R6/2 mice. In conclusion, in the transgenic R6/2 mice changes occur mainly in striatal cholinergic neurones and their pre-synaptic modulation, but not in the dopaminergic afferent terminals. Whether similar events also contribute to the pathogenesis of HD in humans has to be established.

[Congenital generalized dropsy and intrapericardial fetal teratoma. Report of a case of prenatal diagnosis]. / Anasarque foeto-placentaire et tératome intra-péricardique foetal. A propos d'un cas de diagnostic anténatal.

Intrapericardial teratomas are rare tumors usually occurring in early infancy. Such a case was diagnosed in a 20 week old fetus, with a lethal outcome. About fifteen cases of intrapericardial teratoma have been reported in the fetal period. They are usually symptomatic, with a more or less severe pericardial effusion, sometimes complicated by cardiac compression and/or hydrops fetalis. Prognosis of the prenatally diagnosed pericardial teratomas is more related to the occurrence of hydrops fetalis than to the behavior of the tumor itself. The main interest of sonographic ante-natal diagnosis consists of the possible improvement of hemodynamic consequences through in utero pericardiocentesis. Macroscopic and microscopic features of this cardiac tumor are similar to those of extrapericardial teratomas. Most tumors discovered in fetal period are mature and histologically benign lesions. Only one case showed immature components. The coexistence of a malignant component (germ cell or non germ cell tumor) has been described in the postnatal and adulthood.

[Ovarian metastasis of colorectal adenocarcinomas. A clinico-pathological study of 41 cases]. / Métastases ovariennes des adénocarcinomes colorectaux Etude anatomo-clinique de 41 observations.

We report a series of 41 ovarian metastases from colorectal adenocarcinomas. The patients'mean age was 57.1 years at the time the metastasis was discovered, and 55.8 years at the time the primary carcinoma was found. The diagnosis of the primary tumour was anterior to the metastasis in 25 cases (mean interval 21 months), simultaneous in 13 and posterior in 3 others. The metastases formed cystic and solid masses with a mean weight of 330 g. The endometrioid architectural type was the most frequent, either pure (71%, 29/41) or associated with a mucinous component (17%, 7/41). Pure mucinous or other architectural types were rare. The endometrioid type was characterized by glands with a garland pattern, and intraluminal dirty tumoral necrosis. Immunohistochemistry helped to distinguish the metastases of endometrioid type from serous or endometrioid primary ovarian carcinoma; 71% of the former were CK7(-)/CK20(+), and 100% of the latter had the reverse profile CK7(+)/CK20(-).

Lymphocyte reactivity to hepatitis C virus (HCV) antigens shows evidence for exposure to HCV in HCV-seronegative spouses of HCV-infected patients.

Lymphocyte reactivity against hepatitis C virus (HCV) antigens was studied in 20 couples in which 1 member had chronic hepatitis C. This was done to investigate the possibility of HCV transmission between spouses that was not followed by seroconversion. Twenty healthy subjects without any risk factors for HCV transmission served as negative controls. All the patients' spouses and the healthy controls were negative for HCV RNA and for anti-HCV antibody. Lymphocytes were cultured with recombinant HCV core and nonstructural antigens (c22, c33, c100, c200, and NS5) and with control antigens (sperm whale myoglobin, chicken lysozyme, and superoxide dismutase). Lymphocytes from 10 patients and 4 seronegative spouses proliferated in the presence of at least one HCV antigen. No proliferation was shown with nonspecific antigens or in the control group. This study gives evidence for possible in vivo priming with HCV antigens that did not lead to seroconversion in spouses of HCV-positive patients.

[Neuroectodermal differentiation of embryonal carcinoma of the testis]. / Différenciation neurectodermique d'un carcinome embryonnaire du testicule.

We report here a rare differentiation of an embryonal carcinoma of the testis to a peripheral neurectodermal tumor (PNET) with lymph nodes and lung metastases. In the present case a complete remission was obtained by a PNET oriented chemotherapy combination followed by 2 courses of classical BEP.

[Spindle cell breast sarcoma]. / Sarcome mammaire à cellules fusiformes.

A voluminous tumor of the right breast, weighing 3,950 g, is described in a 42-year-old malagasy woman. On microscopic examination eight of ten slides showed a monomorphic proliferation of spindle cells; on the two slides, they were associated with scarce ducts and rare epithelial cell clusters. This case raises the question of the association of a phyllodes tumor with a spindle cell sarcoma and their prognostic significance.

Prevalence, relation to spontaneous closure, and association of muscular ventricular septal defects with other cardiac defects.

Previous studies on muscular ventricular septal defect (VSD) have not taken into account the specific defect location in the septum. We retrospectively reviewed all patients with a muscular VSD, with and without associated malformations, diagnosed over 32 months to determine the prevalence and rate of spontaneous closure of single defects in relation to location in the muscular septum. Defects were classified into 4 groups: midmuscular, apical, anterior, and posterior. Two hundred seven patients were identified, of whom 125 had a single defect. The relative prevalence of single muscular VSD was: midmuscular 55 (44%), apical 31 (25%), anterior 33 (26%), and posterior 6 (5%). Thirty patients had signs of spontaneous closure and only 1 underwent surgery. There was no difference in rate of closure with respect to anatomic locations. Patients with multiple muscular VSD were either referred for surgery in the first year of life or had a course similar to patients with a single VSD. Muscular VSD associated with other cardiac malformations was more often encountered in patients with conoventricular VSD and coarctation of the aorta. The distribution of anatomic groups of muscular VSD in association with malformations was similar to the single VSD.

[Ultrastructural immunocytochemical study of lymphocyte subsets from patients with systemic lupus erythematosus and bearing tubuloreticular inclusions]. / Etude immunocytochimique ultrastructurale des sous-populations lymphocytaires de patients atteints de lupus erythémateux systémique et renfermant des inclusions tubulo-réticulaires.

The tubuloreticular inclusions (TRI) correspond to a modification of the endoplasmic reticulum, observed in different pathological conditions, the most frequent being immune disorders. In the systemic lupus erythematosus, they are present in 2.7% to 4.4% of circulating lymphocytes in 4 patients out of 5. Using an immunogold technique, the TRI were identified only in the T lymphocytes (26/331 T lymphocytes versus 0/79 B lymphocytes, p < 0.01 Chi square) and more precisely in the T4 "Helpers" (16/218 T4 versus 0/123 T8, p < 0.01 Chi square).

Role of early postoperative surface echocardiography in the pediatric cardiac intensive care unit.

OBJECTIVE: To compare surface echocardiographic data with catheterization and surgical observation as a way of deciding on the need to reoperate to correct hemodynamically important sequelae following pediatric cardiac surgery; to determine the false-negative diagnosis rate of surface echocardiography. DESIGN: Case series. SETTING: Tertiary-care center, pediatric cardiac intensive care unit. PATIENTS: All 39 patients who underwent reoperation because of hemodynamically significant anatomic sequelae following primary or elective secondary surgery in 1 calendar year. INTERVENTIONS: None. MEASUREMENTS: Two-dimensional and color Doppler ultrasound assessment of anatomy and physiology following cardiac surgery. RESULTS: In 85 percent, surface echocardiography provided sufficient information for surgeons to reoperate on the same admission. Detection of important residual shunts or arterial stenoses and identification of anatomic causes of pulmonary undercirculation (or overcirculation) in palliated single ventricle are feasible. CONCLUSION: Early postoperative surface echocardiography is a viable way to decide on the hemodynamic adequacy of cardiac surgery.

Changes in right ventricular geometry and heart rate early after hemi-Fontan procedure.

To document and quantitate changes in right ventricular (RV) geometry and heart rate, we prospectively examined 35 consecutive patients with hypoplastic left heart syndrome under steady-state conditions (chloral hydrate sedation) before and after a bidirectional cavopulmonary anastomosis (hemi-Fontan) procedure. Right ventricular end-diastolic volume (RVEDV) was calculated as the product of RV cavity areas in two orthogonal planes divided by RV maximal length in either plane. After the hemi-Fontan procedure, RVEDV decreased by 33% from 33 +/- 13 to 22 +/- 11 mL (mean +/- standard deviation). Indexed RVEDV decreased from 86 +/- 37 to 57 +/- 28 mL/m2. The RV wall thickness at the diaphragm in subcostal frontal view (RVWD) increased by only 11% from 8 +/- 0.2 to 9 +/- 0.2 mm (p = not significant), but RVWD/RVEDV increased by 111% from 0.36 +/- 0.22 to 0.76 +/- 0.69 mm/mL (p = 0.002). The RV anterior wall thickness in subcostal sagittal view (RVWA) increased by only 13% from 7 +/- 0.2 to 8 +/- 0.2 mm (p = not significant), but RVWA/RVEDV increased by 103% from 0.31 +/- 0.20 to 0.63 +/- 0.54 mm/mL (p = 0.002). In 11 of 35 patients (31%), resting heart rate did not change (118 +/- 14 versus 108 +/- 9 beats/min; p = not significant); however, in 24 of 35 patients (69%), heart rate increased significantly (108 +/- 9 versus 127 +/- 10 beats/min; p = 0.05). In conclusion, RV wall thickness is high before the hemi-Fontan procedure and increases slightly in the first postoperative week.(ABSTRACT TRUNCATED AT 250 WORDS)

[Magnetic resonance imaging of seminal vesicles. Normal and pathological aspects]. / Imagerie par résonance magnétique des vésicules séminales. Aspects normaux et pathologiques.

We have studied the normal and pathological aspects of seminal vesicles by MRI with body coil by correlation between imaging and pathologic findings after radical surgery of 63 patients with urogenital carcinoma. On normal T2 weighted sequence, the ampulla of the was deferens visible in 71% of cases exhibited a nodular low signal intensity. Seminal vesicles demonstrated an hypersignal in 79%, a low signal intensity in 19% and an heterogeneous signal intensity in 2%. Overall accuracy for the diagnosis of invasion was 77%. MRI was not efficient for bladder carcinoma (sensitivity: 1; specificity: 82%) but it was always gross infiltration (superior to 5 mm) than for prostatic carcinoma (sensitivity: 40%; specificity: 67%). The numerous false positives (15 cases) were related to hypointense normal (8 cases) or fibrous (6 cases) seminal vesicles and one case of atrophy. The false negatives were related to small invasion. Only, the negative predictive value was good value (overall NPV: 96%; bladder carcinoma NPV: 1; prostatic carcinoma NPV: 89%). A normal bilateral hypersignal of seminal vesicles permit to eliminate a gross invasion.

[Carcinoma cuniculatum. Apropos of 4 cases with orofacial involvement]. / Epithélioma cuniculatum. A propos de 4 cas de localisation oro-faciale.

Carcinoma cuniculatum, a rare variant of verrucous carcinoma, was first described in the foot. The authors report 4 cases of uncommon localizations of this tumor, involving oral cavity and face, 3 of them having a 6-year follow-up. The specific histological and clinical features of this tumor are remembered and the difficulties of its diagnosis are emphasized. This slow-growing, ulcerated proliferation, invading the surrounding tissues, is often responsible for chronic suppuration, but very rarely metastasizes to the regional lymph nodes. The surgical treatment consists in a wide excision; neck dissection is theoretically useless, and radiotherapy is strictly contraindicated because of the risk of transformation into an anaplasic carcinoma. For all these reasons, carcinoma cuniculatum must be considered as an anatomoclinic entity, and deserves to be known by the clinicians and the pathologists.

Accuracy of prospective two-dimensional/Doppler echocardiography in the assessment of reparative surgery.

Between January 1987 and January 1989, all 129 patients (aged 11 days to 25 years, median 39 months) undergoing both an echocardiographic examination and cardiac catheterization after reparative surgery were prospectively included in a study to assess the accuracy of combined two-dimensional and Doppler color flow imaging. The patient diagnoses were transposition of the great arteries (n = 20), tetralogy of Fallot (n = 38), coarctation of the aorta (n = 24), complete atrioventricular (AV) canal (n = 15), atrial septal defect (n = 8), ventricular septal defects (n = 3), pulmonary stenosis (n = 4), aortic stenosis (n = 8) and subaortic stenosis (n = 9). In arterial tract stenosis, there was high correlation between Doppler estimates and catheterization-derived measurements of residual right ventricular outflow tract obstruction in patients after the arterial switch operation for transposition of the great arteries (r = 0.95) as well as in patients after corrective repair of tetralogy of Fallot (r = 0.84). In semilunar/AV valve regurgitation, graded as none, mild, moderate or severe, echocardiographic estimates correlated exactly with angiographic grading in 84% and differed by one angiographic grade in the other 16%. In residual left to right shunting, no hemodynamically significant shunt was missed by echocardiography. For residual shunts at the ventricular level (n = 32), addition of Doppler color flow imaging improved the sensitivity (from 63% to 94%) and the negative predictive value (from 88% to 98%). In elevated right ventricular pressure, Doppler-derived right ventricular-right atrial pressure estimates in 24 patients correlated well with catheterization measurements (r = 0.93). Combined two-dimensional and Doppler color flow echocardiography was highly accurate in the prospective evaluation of these four types of postoperative residual.

[Neuro-endocrine carcinoma of a Bartholin's gland]. / Carcinome neuro-endocrine d'une glande de Bartholin.

This is a case of a 38 year old woman in whom a small basophil cell tumour occurred with inguinal and iliac lymph node metastases followed by pulmonary metastases and extension of the tumour into the vagina and the bladder. The patient died eight months after having started multiple chemotherapy and radiotherapy with telecobalt. Extra studies were carried out ten years later on the cells. This made it possible to diagnose the condition as a neuro-endocrine carcinoma rich in serotonin.

Absence of humoral autoimmunity in peripartum cardiomyopathy. A comparative study in Niger.

The authors report a humoral immunity study in 39 black Nigerian women with peripartum cardiomyopathy. Serum immunoglobulins (IgG, IgA, IgM) assay, evaluation of serum immune complexes (immunonephelemetric method) and of heart muscle autoantibodies (indirect immunofluorescent double layer technique in heart muscle of the rat) were made. Forty breastfeeding black Nigerian women without cardiac disease were the controls. Differences between serum immunoglobulins, circulating immune complexes and heart muscle autoantibodies are not significant. This study demonstrates the absence of a humoral autoimmunity process in peripartum cardiomyopathy.

Mycetoma in the Republic of Niger: clinical features and epidemiology.

Mycetoma is a common disease in the Republic of Niger. In two hospitals 133 cases were observed. The major site of lesions was the foot. Actinomycetomata were seen more often than eumycetomata. Streptomyces somaliensis is prevalent in the north desert zone while Actinomadura pelletieri is common in the southern part of the country. Madurella mycetomatis, the usual etiologic agent of eumycetoma, is seen in both regions. The species incidence and distribution in Niger differs from those of the west and east African endemic areas.