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We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.

A case of false-negative discrepancy between results of chorionic villi (direct preparation) and those of fetal tissue with an isochromosome 18q [i(18q)] in amniotic cells and fetal blood is reported. Fluorescence in situ hybridization (FISH) confirmed this uncommon chromosomal rearrangement. The fetus showed cyclopia and multiple congenital anomalies which have never been reported in cases of i(18q).

Density functional theory (DFT) was used to study reactions involving small molecules. Relative energies of isomers and transition structures of diazene, formaldehyde, and methylenimine were determined using various DFT functionals and results were compared with MP2 and MP4 calculations. DFT reaction barriers were found to be consistently lower. For some reactions, such as OH + H2 H2O + H, gradient-corrected functionals predict very low or nonexistent barriers. The hybrid Hartree–Fock–DFT adiabatic connection method (ACM) often provides much better results in such cases. The performance of several density functionals, including ACM, was tested in calculations on over 100 atomization, hydrogenation, bond dissociation, and isodesmic reactions. The ACM functional provides consistently better geometries and reaction energetics than does any other functional studied. In cases where both HF and gradient-corrected DFT methods underestimate bond distances, the ACM geometries may be inferior to those predicted by gradient-corrected DFT methods. © 1995 John Wiley & Sons, Inc.

First principles, density functional theory embodied in the DMol program has been applied to agostic ethyl-Ti-complexes, including the dmpe complex, [Ti(-CH 2 CH 3 )C1 3 (dmpe)], where dmpe=(Me 2 PCH 2 ) 2 and its model complex, [Ti(−CH 2 CH 3 )Cl 3 (PH 3 ) 2 ]. The ethyl moiety of the complexes can adopt two limiting conformations, staggered and eclipsed. In the model complex, [Ti(−CH 2 CH 3 )C1 3 (PH 3 ) 2 ], both conformers are found to form agostic structures upon geometry optimization subject to Cs symmetry constraint, with the agostic eclipsed structure being the lower in energy. Full geometry optimization of the dmpe complex, [Ti(−CH 2 CH 3 )C1 3 (dmpe)], yields an agostic structure with geometrical features similar to those measured by single crystal X-ray analysis. It is shown that the HOMO orbital contributes substantially to the agostic bonding.

The authors have undertaken a systematic study of the various low-lying electronic states of the H/sub 2/P/sub 2/ system, about which little is known experimentally, using the methods of ab initio molecular electronic structure theory. For each state they have predicted its molecular geometry, energy, dipole moment, vibrational frequencies, and their associated normal modes. The ground state is trans-HP=PH, 3.5 kcal/mol below cis-HP=PH. The H/sub 2/P=P isomer lies at a relative energy of 28 kcal/mol above the ground state. For the ground state the highest occupied molecular orbital (HOMO) is n/sub +/, a symmetric lone-pair combination; the second HOMO is the P-P ..pi.. bond. In H/sub 2/P=P a lone pair n/sub y/ is the HOMO, and the P-P ..pi.. bond is the second HOMO. For both isomers the lowest unoccupied molecular orbital (LUMO) is the antibonding P-P ..pi..* orbital. A variety of states involving n-..pi..* and ..pi..-..pi..* excitations have been studied for various molecular geometries. The lowest energy open-shell singlet is a pyramidal H/sub 2/PP /sup 1/A'' state (out-of-plane angle 77/sup 0/) at a relative energy of 46 kcal/mol. A skewed HPPH /sup 1/B state (torsion angle 109/sup 0/) is at 64 kcal/mol. Highly distorted geometries are also characteristic ofmore » the triplet states. A pyramidal H/sub 2/PP /sup 3/A'' state (out-of-plane angle 79/sup 0/) has a relative energy of only 21 kcal/mol, and thus it is predicted to lie below the closed-shell planar H/sub 2/PP state. A skewed HPPH /sup 3/B state (torsion angle 90/sup 0/) is at 29 kcal/mol. An unsuccessful search has been made for a stable doubly bridged PH/sub 2/P structure, analogous to SiH/sub 2/Si.« less

Full density functional geometry optimizations on hydrogen peroxide and heptane/dimethyl pentane using six different numerical grids are presented. The grids vary in quality and gradients are calculated (1) assuming a fixed grid and no weight derivatives, and (2) with full allowance for a ‘‘moving’’ atom‐centered grid and inclusion of the weight derivatives. The results clearly demonstrate that accurate energies and geometries can be obtained with around 3500 points per atom for medium‐sized systems (up to say 30 atoms) without the necessity of including the weight derivatives. The latter only begin to influence the results for grids which are of insufficient quality to guarantee reliable values in any case.

STUDY OBJECTIVE--The aims were (1) to assess whether termination of pregnancy after prenatal screening by ultrasound affected the prevalence of congenital anomalies at birth, and (2) to examine the trend of this pattern over time. DESIGN--This study deals with congenital anomalies, possibly detectable prenatally or at birth, which were classified as isolated and multiple anomalies; chromosomal anomalies were not included. The prevalence rates of congenital anomalies at birth were determined from case registration data in the Marseille district, France, from the registry of congenital malformations (Eurocat no 22), which covers 23,500 births a year. The chi 2 test for homogeneity in proportions was used to test whether the differences in the total prevalence rates were significant over time. SETTING--The population was defined as all children born to parents living in the Marseille district between January 1 1984 and December 31 1990. PATIENTS--Among the 164,509 pregnancy outcomes monitored during the study, 1795 children with a single congenital anomaly and 288 with multiple congenital anomalies detectable at birth were assessed. MEASUREMENTS AND MAIN RESULTS--The percentage of pregnancy terminations was higher in the case of multiple anomalies (16%) than with single ones (7.5%). Leaving aside the lethal birth defects, this percentage became 7.9% in the case of multiple anomalies and 4.3% with isolated ones. A significant increase (p < 0.001) occurred over the seven year study period in the total percentage of terminations because of isolated anomalies but not in that involving multiple ones. The increase observed in the former case was found to be mainly attributable to an increase in the number of terminations of pregnancy undertaken because of anomalies which were either lethal or associated with very low survival rates (p < 0.001). CONCLUSIONS--Termination of pregnancy after prenatal ultrasound examination was found to have a definite impact on the prevalence at birth of lethal and congenital anomalies with a low survival rate, and this impact tended to increase over time. No such impact was observed in the case of congenital anomalies associated with high survival rates.

Local density functional calculations on a series of small, neutrals radicals demonstrate that the unrestricted Kohn-Sham wave-function is much less contaminated by higher spin states than its unrestricted Hartree—Fock couterpart, even for radicals which exhibit major contamination in their standard UHF wavefunction. A study of the dissociation curves for the OH and CN radicals shows that the UKS wavefunction remains essentially spin pure out to about 1.5 times the equilibrium bond length. Some general observations regarding spin contamination in unrestricted SCF wavefunctions are presented.

Twenty-two patients with metaphyseal primary malignant bone tumors (17 osteosarcomas, 5 Ewing's tumors) occurring before closure of the growth plate were examined with plain radiographs and MRI in order to determine the physeal or epiphyseal extent of the tumor. Results were correlated with the pathologic examination. Transphyseal spread was pathologically proven in 13 cases (59%): 12 cases of osteosarcoma and 1 case of Ewing's tumor (70% and 20%, respectively). There was no significant relation between epiphyseal invasion, age of patient, length of tumor or, in the cases of osteosarcoma, response to chemotherapy. Plain radiographs showed epiphyseal involvement in 4 cases and there were 10 false negatives. MRI revealed epiphyseal involvement in all cases; there were no false positives or false negatives. T1-weighted images in coronal or sagittal planes appeared to be sufficient. These findings are very useful in planning surgical limb salvage procedures and stress the ineffectiveness of the "barrier effect" of the growth plate against tumor spread.

Ab initio molecular quantum mechanics has been applied to the cyclopentadienylideneketene molecule, a C 7 H 4 O isomer. Until very recently, it was thought that this molecule had not been observed spectroscopically. However, recent experiments appear to show that an infrared feature near 2085 cm -1 (and often attributed to benzyne) is due to cyclopentadienylideneketene. Here the structure of the latter molecule has been optimized using three theoretical methods, including second-order perturbation theory with a triple-ζ plus double-polarization (TZ2P) basis set

Splenic hemangioma is a very rare neoplasm in children. We report three cases of splenic hemangioma, two of solitary hemangioma and one of hemangiomatosis. Two patients had hematologic disorders related to hemangioma. Imaging findings of this entity are presented and discussed with emphasis on pathologic correlation.

Three children displaying hypotonia, cardiac involvement and defects of the mitochondrial respiratory chain complexes are reported. The first case showed severe neonatal hypotonia, failure to thrive, hepatomegaly, dilation of the right cardiac cavities, profound lactic acidosis and amino aciduria. The boy died at the age of 7 weeks. In the second case hypotonia, severe cardiomyopathy, cyclic neutropenia, lactic acidosis and 3-methylglutaconic aciduria occurred. The boy died at the age of 27 months. The third case presented at the age of 16 months as an acute hypokinetic hypertrophic cardiomyopathy with transient hypotonia and mild lactic acidosis. Spontaneous clinical remission occurred. In all cases muscle biopsy was performed. Morphological studies failed to show ragged-red fibers but there was lipid storage myopathy and decreased cytochrome c oxidase activity. Biochemical studies confirmed the cytochrome c oxidase deficiency in muscle in all cases. It was associated with complex I III deficiency in case 1 and with severe deficits of all respiratory chain complexes in case 2. Post-mortem studies in case 1 indicated that complex IV was reduced in the liver but not in the heart and quantitative analysis of mtDNA revealed a depletion in muscle. Cases 1 and 2 shared some clinical features with fatal infantile myopathy associated with cytochrome c oxidase deficiency, while case 3 displayed a very unusual clinical presentation. The histochemical enzyme reaction of cytochrome c oxidase is useful for the diagnosis of mitochondrial myopathy because ragged-red fibers may be lacking. Finally, biochemical measurement of the different mitochondrial respiratory chain complexes is required because multiple defects are frequent and occasionally related to mtDNA depletion.

The infrared spectrum of ortho-benzyne has been a challenge to theory and experiment alike for some time. Ab initio molecular quantum mechanics has been applied to this problem at a level of theory significantly higher than previously possible. Second-order perturbation theory (MP2) using a triple-zeta-plus-double-polarization (TZ + 2P) basis set predicts the CC harmonic vibrational frequency to be 1943 cm − . With the anticipated small downward correction for anharmonicity, this result is in close agreement with two recent experiments, but in poor agreement with four earlier laboratory studies. Theory unequivocally predicts a weak triple bond for benzyne.

The idiopathic hypereosinophilic syndrome (IHS) is extremely rare in childhood and relationships of this syndrome with my-eloproliferative diseases are controversial. We reported the observation of a 7-year-old girl suffering from an IHS with myelofibrosis. A clonal cytogenetic abnormality, trisomy 8, was detected in the bone marrow cells of this child. This is the decisive proof of a myeloproliferative disorder. IHS with myelofibrosis is usually considered as unresponsive to corticotherapy. In our case, corticotherapy resulted in a rapid, complete, and lasting disappearance of myelofibrosis. Complete remission of the disease, however, was not achieved and the trisomy 8 persisted after treatment.

We have studied the ground states of BH 2 PH 2 , BH 2 PH − , and BH 2 PHLi by the methods of ab initio molecular electronic structure theory using a basis set of high quality. For each moleculem we have predicted its molecular geometry, energy, dipole moment, net atomic charges, bond orders, normal vibrational modes, and harmonic vibrational frequencies. Our results for these molecules are compared with experimental data for their derivatives with alkyl and aryl substituents and for the BH 2 NH 2 molecule

Four siblings with hepatic fibrosis are described. The liver damage in these patients was secondary to chronic ingestion of massive doses of vitamin A for congenital ichthyosis. Although the extrahepatic manifestations were helpful in the diagnosis of hypervitaminosis A, the distinctive features of hepatic histopathology were confirmatory. The plasma concentrations of vitamin A and retinol-binding protein were misleading. The recovery from the liver damage in these patients was slow despite a complete withdrawal of the vitamin A intake. These cases show the importance of hepatic vitamin A assessment in the diagnosis of hepatic fibrosis.

Resume La tumeur melanotique neuroectodermique est une tumeur exceptionnelle de l'enfance qui a une predilection pour le maxillaire et derive des creles neurales. Observation. - Une tumeur a debute chez une enfant âgee de 2 mois, sous la forme d'un kyste d'allure odontogenique. L'enucleation de cette tumeur, localisee au premaxillaire, a permis d'en confirmer la nature neuroectodermique. L'evolution a ete marquee par l'apparition rapide d'une recidive csseuse de contiguite. Il n'y avait pas d'excretion urinaire accrue de catecholamines, pas d'image d'hyperfixation a la scintigraphie (methyl iodo benzyl guanidine), et l'examen histoloque on microscopie electronique a montre diffentes variete, de melanocytes. Une maxillectomie partielle, par voie endobuccale, a ete effectuee. Quatre ans et demi apres, le bilan est satisfaisant. Conclusion. - Cette observation confirme le potentiel de recidive de cette tumeur et l'interet esthetique de son abord chirurgical endobuccal.

Virtual organisations within and across enterprise structures are becoming mature as a potentially effective means for goal-oriented business teamwork. Among the many support functions enabling efficient cooperation in teams, team decision-making is a very crucial one, but it does not find adequate support in contemporary team software solutions. Even the most knowledgeable results from collective choice theory are not reflected in the respective virtual team environments. This paper makes a systematic attempt to provide virtual teams with the power of voting procedures developed in the context of the theory of collective choice. A toolset called TEAMVOTE, for electronic decision-making, is presented, implementing a blend of the most important collective choice strategies, like the Dodgson rule, the majority rule, plurality voting, Borda counting, approval voting, the Nanson rule, the Hare rule, the Coomb rule, etc., based on standard Internet technology. The services of TEAMVOTE, which are targeted to support goal-oriented business teams in group decision matters, raise the perspective of its use to enable democratic processes on a societal scale.

We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.

A three-year-old female child was referred to the medical clinic for abdominal pain and distension. The patient had no significant past medical history. Physical examination revealed a huge abdominal mass, predominant in the right flank, pallor and weight loss. Plain radiography showed a mass in the right upper quadrant of the abdomen, without any calcification. Ultrasonography showed this mass, involving the right kidney and crossing the midline (Fig. 1). Only the lower pole of the right kidney was recognizable. The mass was heterogeneous and hyperechogenic compared to the liver parenchyma. The inferior vena cava was thrombosed and the thrombus involved the right atrium and was lying in the right ventricle during the diastol. There was an hepatic nodule, hypoechogenic compared to the liver parenchyma with an hyperechogenic rim. CT-scan showed the abdominal mass crossing midline, displacing the aorta to the left side, involving the right

We report the case of a patient with infrarenal abdominal aortic aneurysm with mural thrombus covering the ostium of a patent inferior mesenteric artery (IMA). The IMA was supplied via flow from an accessory aneurysmal lumen within the mural thrombus that filled retrogradely from the aorta. This unusual pattern, associated with calcifications within the thrombus, raised the possibility of chronic aortic dissection.

Pulmonary blastoma is a rare malignant tumor. A new case is reported in a 3 years 6 month-old girl. An apparent clinical remission was first obtained after a surgical treatment followed by a conventional chemotherapy during six months. Afterwards the persistence of a microscopic residual pulmonary disease lead us to deliver successfully an intensive chemotherapy followed by autologous peripheral blood stem cells reinjection. The child remains disease free 12 months after graft. Problems set by the histogenesis of this tumor, its unspecific clinical and paraclinical features and the role of conventional and intensive chemotherapy followed by autologous bone marrow transplantation are discussed on the basis of a review of 50 cases in the literature.

Marantic or non-bacterial thrombotic endocarditis is a common complication of terminal malignancy, usually discovered at autopsy. The authors report a case of marantic endocarditis as a presenting sign of pancreatic carcinoma in which the diagnosis was made ante-mortem by transoesophageal echocardiography.

• Background: The fetal face is clearly seen by ultrasonography: we considered measurement of certain orbitofacial parameters of interest in the human fetus in order to establish norms for facial development. • Methods: We included 108 “normal” fetuses ranging in age from 16.5 to 41 weeks of amenorrhea. The orbitofacial parameters studied were outer canthal distance, inner canthal distance, oropalpebral distance right side and left side, and palpebral fissure length rside and left side. The ocular parameters studied were corneal horizontal diameter and axial length. The traditional anthropometric parameters of the fetus were determined by pathological examination: age, weight, head circumference and height. A statistical study analyzed the different correlations and established linear regression equations for orbitofacial parameters as a function of age. Polynomial regression models were tested to the third degree as a function of age and the head circumference/17 ratio. • Results: Results are given in six different age groups. We find excellent correlation between the different parameters. Statistically valid linear regression equations were established for orbitofacial parameters. Polynomial regression equations were compared to linear equations their correlation coefficient and standard error, but showed no greater validity. Skull growth is more rapid than facial growth, which itself is more rapid vertically than horizontally. • Conclusion: This study establishes norms for the different orbitofacial parameters, in particular the oropalpebral distance, for which we found no bibliographic references. The general interest of these measures lies in the description of malformation syndromes.

Melanotic neuroectodermal tumor is a mostly benign tumor, rare in childhood, essentially located in the head and neck region.A two-month-old girl was seen for a rapidly increasing odontogenic tumor which appeared cystic at the CT scan. After enucleation, this premaxillar tumor recurred one month later with an osteogenic aspect at the CT scan. Urine catecholamine excretion was normal; Methyl Iodo Benzyl Guanidin scintigraphy failed to show any fixation and electron microscopy examination of the biopsy showed several varieties of melanocytes. A partial maxillectomy was performed by oral approach. The patient is well 4 1/2 years later.This observation confirms the recurrence potential of this tumor and the cosmetic interest of the oral surgical approach.

In this geographically based study the findings on 158 abnormal fetuses, primarily diagnosed by routine antenatal ultrasound, are correlated with the results of the examinations subsequently carried out by a fetopathologist and a clinical geneticist. Ninety fetuses (57%) had a single malformation, 66 were polymalformed (42%) and 2 had no malformations. In 90% of all these cases, the prenatally and postnatally detected anomalies were identical; in 3% the defect established at necropsy was different from that diagnosed prenatally, and in 7% the predicted anomaly was absent. These values did not depend on whether single or multiple malformations were involved. In 57% of the polymalformed cases, however, the ultrasound examination missed at least one other diagnosable anomaly. On the basis of pathological and clinical genetic expertise, a risk of recurrence of the anomaly was revised in 13% of the single malformed cases and in 53% of the multiple ones, i.e., in 30% of all the cases of malformation on average. This study confirms the need for the fetus to be examined by a pathologist and a clinical geneticist after termination of a not 'at risk' pregnancy in order to check the accuracy of the sonographic procedure, to confirm the reasons for terminating the pregnancy to the parents, and to be able to monitor the next pregnancy based on an accurate assessment of the risk of recurrence.

Calcifications in lymphoma are rare findings prior to therapy. We report our experience with a 13-year-old boy who presented with a primary calcified duodenal Burkitt's lymphoma. US and CT findings are described. Two differential diagnoses are discussed.

IgA nephropathy may occur in adults with alcoholic cirrhosis. It has also been reported in children with alpha-1-antitrypsin deficiency. A case of IgA nephropathy associated with cirrhosis due to Wilson's disease is reported.A 10 year-old girl, was admitted for cirrhosis. She had suffered from ascitis and hematuria since the age of 6 years and vascular purpura since the age of 9 yr 6 mo. At admission, she had atrophic liver cirrhosis, ascitis splenomegaly and petechiae on her legs. There was microcytic anemia due to iron deficiency. The Coombs test was positive. There were hypoalbuminemia (2 g/dl) and polyclonal hyperglobulinemia (IgA 750 mg/dl, IgG 1670 mg/dl and IgM 250 mg/dl). Her serum concentrations of complement factors were normal and no immune complexes were detected in serum. She also showed biological changes due to kidney failure, hematuria and proteinuria. A liver biopsy showed micronodular cirrhosis with numerous plasma cells containing IgA. The search for an etiology showed ceruloplasmin levels of 5 mg/dl, elevated urinary copper excretion (150 micrograms/day) and above normal liver copper (255 micrograms/g dry weight); Kayser-Fleischer ring was also seen. The renal biopsy showed membranoproliferative glomerulonephritis, and immunofluorescent microscopy revealed C3 and IgA in the mesangial and subendothelial regions. The patient was given spironolactone and furosemide followed by triethylenetetramine dihydrochloride.The IgA nephropathy, vascular purpura and elevated serum IgA levels could be due to the liver changes. If so, they should be improved by a liver transplant, although our patient would be a better candidate for combined liver and kidney transplantation.

A prospective registration of incident cancers in childhood in two south-east regions of France since 1 January 1984 allows us to collect pertinent data on 875 cases throughout a period of 8 years. World age-standardised overall incidence rate is 137.63 cases/million/year. It is close to that reported in other white European. North American and Oceanian populations. The age-adjusted (age-standardised) relative frequency of each pathological group is: leukaemias 29.71%; central nervous system tumours 20.61%; lymphomas 12.75%; sympathetic tumours 9.03%; soft tissues tumours 7.37%; bone tumours 5.89%; kidney tumours 4.82%; epithelial tumours 3.83%; germinal and gonadal tumours 3.24%; retinoblastomas 2.11%; liver tumours 0.45% and others 0.14%. The comparison of these results with international available data shows that we record the world highest adjusted incidence rates for neuroblastomas (15.46) and rhabdomyosarcomas (7.04) and a high rate for Ewing's sarcomas (3.30); this fact will need to be confirmed by a longer period of observation, but even now the total number of cases (particularly for neuroblastoma) is high when compared with the data of other children registries which give rates for longer periods and for similar or larger populations.

Advances in liver preservation had been slow, until Jamieson et al. (1) described a successful long-term cold-preservation of the dog liver based on the use of a lactobionatecontaining solution. However, additional progress has remained limited for two reasons. First, the mechanisms responsible for cellular injury induced by cold-ischemic preservation are not fully understood due to the complexity of events occurring during storage and reperfusion. Second, the evaluation of the metabolic status of the transplant during ischemia and reperfusion has lacked adequate invasive and non-invasive methodologies.

The authors have analysed a series of aneurysmal bone cysts (A.B.C.) in children and adolescents which were reviewed at an average follow-up of 4 years 2 months (6 months-15 years). The average age at diagnosis was 8 years (3-19 years). Pathological fractures were the main reason for consultation, probably due to the predominance of central lesions. If conventional radiology remains indispensable to diagnosis, magnetic resonance imaging is nevertheless the most important examination in determining the extent of the affection. The diagnosis remains anatomopathological, even if this may be difficult due to associated lesions (A.B.C. illness and A.B.C. symptoms). In long bones lesions, we observed recurrence after curettage in 3 out of 7 cases. For this reason the recommend simple resection or reconstructive resection rather than curettage whenever possible. 5 lesions in contact with growth plate were observed. In such cases we recommend its conservation by careful curettage, especially in young children. We feel that subsequent recurrence is easier to treat than an epiphysiodesis bridge. The surgical techniques employed to conserve the plate are described along with methods of bone reconstruction after surgery.

A case of acute eosinophilic myocarditis without hypereosinophilia, presenting as hypokinetic dilated cardiomyopathy in a 24-year old man is reported. Sudden worsening of subacute cardiac failure required heart transplantation 3 months after the onset of the disease. Only pathological examination provided the diagnosis of acute necrotizing eosinophilic myocarditis of undetermined origin. Two years after transplantation, the patient had no clinical or histological sign of recurrence. Seldom described in the literature, acute eosinophilic myocarditis is a dangerous form of eosinophilic heart disease which often follows a fulminant course beyond all therapeutic resources. This case, which is particular in its clinical presentation, in the lack of hypereosinophilia and above all in its cure after heart transplantation, enables the authors to discuss the mechanisms and various manifestations of the cardiotoxicity of eosinophils.

The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.

Three pediatric cases of malignant rhabdoid tumor of the neck are described. Clinical data and imaging findings (US, CT and MRI) are stressed. The mass was well defined, containing punctate calcifications in two cases and encasing the vessels in two other cases. Two patients were treated with a chemotherapy regimen according to MMT 89 SIOP protocol, one had chemotherapy and radiotherapy. Two children died of progressive disease; the remaining child who had complete surgical removal of the tumor is on remission 17 months after diagnosis.

We are yielding a case of recurring melanotic neuroectodermal tumor of infancy situated in the premaxilla, with a very quick evolution, haemorrhage, and which has precociously recurred after surgical removal. This tumor is studied on the paraclinical (CT scan, M.I.R.) and on the histological hand (immunohistochemical and ultra-microscopy). This observation is then compared with those of literature in which we find about 200 cases. The neurocristopathic histogenesis is actually doing the unanimity. The prognosis must now be quite reserved because of the recurrences (one case for six) and the possibility of malignant forms, some with metastasis.

Using our recently implemented closed-shell coupled cluster singles and doubles (CCSD) model, the equilibrium structure and vibrational harmonic frequency of N 2 , CO, HF and OH − have been determined. This set ofdiatomics was specially chosen so as to represent the range of bonding characteristics found in small molecules. The CCSD results are compared to analogous configuration interaction (CI) predictions and are found to be most similar to CISDQ or CISDTQ. That is, CI including all singles + doubles + quadruples (CISDQ) or CISDQ+all triple excitations (CISDTQ). The agreement of CCSD with CISDQ/CISDTQ is found to be much better for the singly bonded species with the agreement for neutral HF somewhat better than for anionic OH − . We arrive at two major conclusions. First, higher-order effects not present in the CCSD model are less negligible for multiply bonded species and, to a smaller degree, for anionic species as well. Secondly, even for the two types of molecules discussed above, the CCSD results are closer to the CISDQ/CISDTQ predictions than are CISD values. Since CCSD is less computationally time consuming than CISDQ/CISDTQ, we suggest that the CCSD model is one of the best methods available when highly accurate ab initio predictions for chemical species strongly dominated by a single-determinant wavefunction are desired.

Recently developed analytic CCSD gradient methods have been used to predict the harmonic vibrational frequencies of six molecules: CH4, NH+4, HCN, C2H2, HNC, and CO2. In every case a double zeta plus polarization (DZ+P) basis set of size C,N,O(9s5p1d/4s2p1d), H(4s1p/2s1p) was used. Previous analogous studies of H2O, H2CO, and NH3 are extended to form a statistical base of nine molecules. For these molecules 28 harmonic vibrational frequencies (out of total of 35 fundamentals) are thought to be known from experiment. The average errors with respect to experiment were found to be 9.1% (DZ+P self‐consistent field), 3.7% (DZ+P configuration interaction including single and double excitations), and 2.2% (DZ+PCCSD). These statistics should provide guidance for the use of the CCSD method in situations where experimental vibrational frequencies are not available. Infrared intensities are also compared with available experimental data.

The theory for the analytic evaluation of energy gradients for coupled cluster (CC) wave functions is presented. In particular, explicit expressions for the analytic energy gradient of the CC singles and doubles (CCSD) wave function for a closed‐shell restricted Hartree–Fock reference determinant are presented and shown to scale as N6 where N is the one‐electron number of atomic basis functions for the molecular system. Thus analytic CCSD gradients are found to be of the same magnitude in computational cost as is the evaluation of analytic gradients for the configuration interaction singles and doubles (CISD) wave function. Applications of this method are presented for the water molecule and the formaldehyde molecule using a double‐ζ plus polarization (DZ+P) basis set. The CCSD equilibrium geometries, dipole moments, and, via finite differences of gradients, CCSD harmonic vibrational frequencies and infrared intensities are reported. For H2O these results are compared to analogous CISD, CISDT, CISDTQ, and...

The analytic energy gradient method that we have recently implemented for CCSD wave functions is applied to fully optimize the pyramidal C3v and planar D3h structures of ammonia. Using a double-zeta plus polarization basis set, results for harmonic vibrational frequencies, infrared intensities, and dipole moments have been obtained. Comparison with different levels of truncated configuration interaction suggests that CCSD values are of better than CISD quality and generally closer to CISDTQ results.