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[Late congenital glaucoma and pigmentary retinopathy].

Two clinical observations of pigmentary retinopathy associated with late congenital glaucoma are presented. This associations is rarely emphasized in the literature, the most frequent association being of the pigmentary retinopathy and open angle glaucoma. In the former observation a genetical filiation could not been established. In the latter the heredity was autosomal recessive, with two existent distinct pathological genes. The genetic defect in pigmentary retinopathy with dominant autosomal transmission is supposed to be localized on the codon 216 Ser-->Pro or at the level of the codon 296 by the substitution Lys-->Glu and Lys-->Met for the some determination, but done in vitro. In the recessive form the abnormal alleles are localized in the interval D11 5861 and D11 5899.

Adult↗

[Pathogenesis of cholesteatoma: contribution of otoscopic photography].

The filiation between otitis media with effusion, retraction pockets and cholesteatoma appears for most otologist to be very probable and logical but remains controversial. Some experts argue rigourously and they say fairly that it has not been so far strictly proven. Long term otoscopic observation supported by photography could bring indisputable evidences. During a 12 years period we have taken routinely otoscopic photography of every patient with chronic otitis media. We have been able to observe the appearance of cholesteatoma in ear apparently normal at the origin or which presented so-called simple chronic middle ear disease. We report some of the most characteristic observations.

Adolescent↗

[Bacteremia in 13 general hospitals of the province of Barcelona. Prospective study of 1,674 episodes. Group of Microbiologists of the County Hospitals of Catalonia].

BACKGROUND: Clinical and microbiological description of bacteremias seen in 13 regional hospitals of Barcelona. METHODS: From January to December of 1991 a multicenter prospective study of bacteremia in regional hospitals was performed. The filiation, clinical findings and microbiological results were registered according to a common protocol. RESULTS: We studied 1674 episodes of bacteremia (13.8 per 1000 admissions). Patients were distributed into 3 groups according to age: under 14: 204 patients, between 14 to 65: 759 and over 65: 711 patients. Men account for 55% and women for 45% of episodes. The bacteremia was hospital-acquired in 24.4% of cases. The most common foci of infection were: urinary tract (32%), respiratory tract (14%), intestinal tract (8%), intravascular catheter (7%), biliary tract (7%) and unknown (10%). The most commonly isolated strains were: Escherichia coli 544 cases, Streptococcus pneumoniae 212, Staphylococcus aureus 139, coagulase negative staphylococi 85, Non-typhoid Salmonella 66, Neisseria meningitidis 63, Pseudomonas sp. 59, Enterococcus sp. 59 and Bacteroides sp. 58. Polimicrobial bacteremia was detected in 83 episodes (5%). Related mortality rate was 9.6% (161 patients).

Adolescent↗

[Malignant lymphoma with medium-sized macronucleolated cells in the dog: involvement of an original cell from the marginal zone of the reactive lymph node].

Among the non-Hodgkin's malignant lymphomas of the dog, which are largely dominated by the centroblastic heterogeneous type, there is an original form of malignant lymphoma which is homogeneous and diffuse, with macronucleolated medium-sized cells. These cells seem to be morphologically very similar to those which constitute the majority population in the marginal zone of the secondary follicle of the lymph node in the dog, and which appear in the course of certain conditions: systemic lupus erythematosus, leishmaniasis, satellite lymph nodes in benign or malignant tumors. The aim of this study was twofold: on the one hand to establish, in the canine species, the identity of the lymphomatous cells and the reactive cells that make up the marginal zone, i.e. the filiation between the hyperplastic marginal zones and the macronucleolated malignant lymphoma with medium-sized cells, and, on the other hand, to compare this type of malignant lymphoma with those which are reputed to originate in the marginal zone in humans, for example the malignant lymphoma of the lymphoid tissue associated with the mucous membranes, and the monocytoid malignant B-cell lymphomas. Ninety four malignant lymphomas were observed between 1989 and 1994 at the Veterinary School in Lyon; these consisted of 71 cases showing medium or high-grade malignancy, 17 cases with small cells, of low-grade malignancy, and 6 cases of mycosis fungoides. Among the 71 cases of medium and high-grade malignancy, 8 were immunoblastic, 5 centroblastic homogeneous, 50 centroblastic heterogeneous, and 8 homogeneous with macronucleolated medium-sized cells. The methods used in these 94 cases were of a morphological type: cytology, histology, transmission microscopy and immunohistochemistry. The cytohistological, ultrastructural and immuno-phenotypical characteristics (CD3-, CIg-, Ki-67- phenotype) of the lymphomatous cells and the cells of the marginal zone were found to be identical, in the dog; this strongly suggests B-lineage cells which do not secrete cytoplasmic immunoglobulins and are not involved in the cell cycle. Finally, these cells seem to us to be morphologically very similar to the minority population described by Van den Oord in the marginal zone of the secondary follicles in the lymph node in humans, in certain reactive situations.

Animals↗

[The solitary pulmonary nodule. An analysis of the experience over 6 years in a medical service].

We conducted a retrospective analysis of the epidemiological, etiological, clinical and radiological characteristics, and mainly of those related to the diagnosis and treatment, of all the patients with radiological criteria of solitary pulmonary nodule (SPN) studied in our environment during a period of six years (1984-1989). The incidence of SPN was 3.1% (123/3953). Among the 117 cases of filiated etiology, 83 (71%) were malignant and 34 (29%), benign thoracotomy was used as diagnostic method in 11 (32%) benign cases and in 8 (9.6%) malignant cases. Due to several causes, only 31 out of the 75 (41.3%) malignant nodules diagnosed prior to the thoracotomy underwent resection surgery. According to the selection criteria applied in our medium, a high number of the SPN were malignant; in most of these cases, the diagnosis was established without the need of thoracotomy and in less that half of them, curative surgery was attempted.

Aged↗

[Malignant granuloma of the face and angiocentric T-cell lymphoma. A review of the literature apropos of a case].

The authors report the observation of a lethal midline granuloma in a 43 years old man, at first seen with repeated left sinusitis disease, the histo-pathological study of a biopsy of the external middle third of the left side of the nose made the diagnosis of midline lethal granuloma, classically called Stewart granuloma by french authors. General Medical Investigations to find some peripheral extension, done in specialized services were negative, Epstein Barr DNA was not found by the immuno-histochemical investigations and so don't prove the filiation of this case with angiocentric T cell immunoproliferative lesion. By the light of this observation and of a literature review, the authors debate about the difficulty of diagnosis and about the relations with the other granulomatosis lesions of the face, non Hodgkins' lymphoma and angiocentric T cell immuno-proliferative lesions.

Adult↗

Ethical and legal concerns: reproductive technologies 1990-1993.

After the rapid increase in reports, bills, and regulations on assisted reproductive technologies (ART) in the 1980s, the first 3 years of the 1990s reveal a continuation of this trend notably in three major aspects. First, a certain consistency has developed in terms of the conditions of accessibility to ART, the definition of infertility, the terms of donation, and the primacy of social filiation, so that anonymity remains controversial. Second, the importance of protection of genetic material has been reaffirmed with regard to gamete and embryo conservation, embryo research, and, in particular, the acceptability of preimplantation diagnosis. Finally, the framework of practices concerning accreditation and control, organization of national data, and management of nominative information has been increasingly refined.

Accreditation↗

[Psychological approach of the infertile male].

Male infertility is usually anxiogenic, and it is often responsible for sexual dysfunctions due to the cultural indissociability of the virility and fertility concepts on the one hand, and to the timing of sexual intercourse in the ovulation period on the other hand. The intrusion of medicine in the life of the infertile couple frequently amplifies the existing psychological problems and discloses other problems by questioning the representation systems of identity, procreation and filiation.

Humans↗

[Diffuse subcortical heterotopias of the gray matter].

Three cases of diffuse subcortical gray matter heterotopias in children are reported. Generalised seizures and mild mental retardation were the most frequent signs. No specific electroencephalographic pattern was recognized. Magnetic resonance imaging scans showed the thick diffuse layer of heterotopic gray matter which was surrounded by normal white matter. Gyration was normal, and no associated malformation was observed. This neuronal migrational disorder happens between the 10th and 16th gestational week. Nineteen observations (17 girls) are reported in the literature. The filiation with agyria-pachygyria and the possible genetic transmission are discussed.

Adolescent↗

[Unexpected clinical diagnosis of myotonia].

We studied all the electromyographic studies carried out in our Service until 1995, in order to quantify the find of myotonia and to compare it with the previous clinical diagnosis that originated the exploration. 30% of the findings of myotonia were unexpected. Non-filiated myopathy and weakness on study, were the principal clinical correlates in those unexpected findings. It must be noted the importance of misdiagnosed myotonia on the epidemiological data of myotonic dystrophy.

Electromyography↗

Phenotype and function of human hematopoietic cells engrafting immune-deficient CB17-severe combined immunodeficiency mice and nonobese diabetic-severe combined immunodeficiency mice after transplantation of human cord blood mononuclear cells.

In an attempt to understand better the regulation of stem cell function in chimeric immunodeficient mice transplanted with human cells, and the filiation between progenitor cells identified in vitro and in vivo, we assessed the different compartments of hematopoietic progenitors found in the marrow of CB17-severe combined immunodeficiency (SCID) mice (34 mice, 9 experiments) after intravenous injection of 2 to 3 x 10(7) cord blood mononuclear cells. On average 6.3 +/- 4 x 10(5) human cells were detected per four long bones 4 to 6 weeks after the transplant predominantly represented by granulomonocytic (CD11b+) and B lymphoid (CD19+) cells. Twenty five percent of these human cells expressed the CD34 antigen, of which 90% coexpressed the CD38 antigen and 50% the CD19 antigen. Functional assessment of progenitor cells (both clonogenic and long-term culture-initiating cells [LTC-IC]) was performed after human CD34+ cells and CD34+/CD38- cells have been sorted from chimeric CB17-SCID marrow 3 to 10 weeks after intravenous (IV) injection of human cells. The frequency of both colony-forming cells and LTC-IC was low (4% and 0.4%, respectively in the CD34+ fraction) when compared with the frequencies of cells with similar function in CD34+ cells from the starting cord blood mononuclear cells (26% +/- 7% and 7.2% +/- 5%, respectively). More surprisingly, the frequency of LTC-IC was also low in the human CD34+ CD38- fraction sorted from chimeric mice. This observation might be partly accounted for by the expansion of the CD34+ CD19+ B-cell precursor compartment. Despite their decreased frequency and absolute numbers, the differentiation capability of these LTC-IC, assessed by their clonogenic progeny output after 5 weeks in coculture with murine stromal cells was intact when compared with that of input LTC-IC. Furthermore the ratio between clonogenic progenitor cells and LTC-IC was similar in severe combined immunodeficiency (SCID) mice studied 4 weeks after transplant and in adult marrow or cord blood suspensions. Results generated in experiments where nonobese diabetic (NOD)-SCID mice were used as recipients indicate a higher level of engraftment but no change in the distribution of clonogenic cells or LTC-IC. These results suggest that the hierarchy of hematopoietic differentiation classically defined in human hematopoietic tissues can be reconstituted in immunodeficient SCID or NOD-SCID mice.

ADP-ribosyl Cyclase↗

Sexual determination of the femur using discriminant functions. Analysis of a Spanish population of known sex and age.

Until present, functions for sexual discrimination of a Spanish population from series of known sex and age were not available. In this work, we present a sample of 132 femora (72 females and 60 males) belonging to a Spanish population of adult individuals of known filiation deposited in the Complutense University of Madrid. The ten mathematical functions which yield a higher sexual discrimination in each part of the femur, were selected. The resulting percentages of correspondence varied between 84 and 97% when each variable was considered independently, and a 99% is obtained with two variables of the epiphyses combined.

Adult↗

[Anxiety and vertigo].

AIM OF THE STUDY: To determinate the prevalence of anxiety associated with vertigo of vestibular origin. DESIGN: A retrospective study. SETTING: Patients from Salamanca's University Hospital (Neuro-Otology). PATIENTS: 138 patients with asymptomatic vestibular dysfunction (group A: with filiated disease and group B: with unfiliated disease). INSTRUMENTATION: Structured anamnese and STAI questionnary (State-Trait-Anxiety-Scale) were realized in the whole collective. RESULTS: Anxiety (elevated score in STAI-scale) was detected in 48.6% patients and more differences between groups A and B were not significant. With logistic regression anxiety is related with previous symptomatological experiences and subjective global incapacity. Anxiety is clearly connected with the symptoms in the intercritic periods. CONCLUSION: Anxiety is associated with vestibular disease and it follows a parallel course.

Anxiety Disorders↗

Association of hemoglobin Saint Etienne (alpha2beta295F8 His replaced by G1n) with hemoglobins A and F. Synthesis and subunit exchange in vitro.

The unstable hemoglobin (Hb) Saint Etienne (alpha2beta295F8 His replaced by G1n) (betaSE) was found in the red blood cells of an 8-year-old boy. The composition of this hemoglobin was 26% Saint Etienne, 52% A, 3% A2 and 19% HbF. Studies of hemoglobin synthesis indicate: a) a balanced synthesis of alpha and non-alpha chains (alpha=betaA + betaSE + gamma), b) an increased pool of free alpha hemoglobin chains, and c) a rapid exchange of alpha chains between this pool and HbSE. The alpha chain pool resulted from the dissociation of HbSE and the greater instability of betaSE chains than alpha chains upon heating. Hemoglobin F is of the fetal type and is heterogeneously distributed among the red cells. Furthermore, two populations of red blood cells could be separated according to their i antigen content. Analysis of the hemoglobins revealed a heterogeneous distribution. Thus, F hemoglobin was preferentially associated with cells having low i antigen level, while Saint Etienne hemoglobin was increased in cells having a high i antigen level. HbF and HbSE were not present in the parents of the propositus. Study of the genetic markers confirmed the filiation. The parents were normal upon clinical and hematological examination; they exhibited a normal pattern and synthesis of hemoglobin. The Hb Saint Etienne case is compared with Hb Istanbul, which in spite of the same amino acid substitution is not associated with increased HbF level.

Adult↗

[The role of carbapenems in the treatment of nosocomial infection].

Carbapenems are active beta-lactam antibiotics versus most of the gram positive and gram negative microorganisms and anaerobes although their activity is lacking in the case of Staphylococcus sp. resistant to methicillin, Enterococcus faecium and Streptococcus pneumoniae with high resistance to penicillin and some gram negative bacilli which naturally produce an methaloenzyme able to hydrolyze them such as Stenotrophomonas maltophilia. Imipenem, the first synthetized carbapenem requires administration with cilastatin to avoid inactivation by renal dehydropeptidase 1. Meropenem does not require being taken with the renal enzyme inhibitor, with its activity being similar to that of imipenem. In abdominal infection the carbapenems have shown to be the authentic monotherapy in this type of infections being as effective as the different schedules of antibiotic associations normally used. Treatment with carbapenems in bacterial meningitis should be currently limited to the cases produced by gram negative bacilli producers of wide spectrum beta-lactamases (WSBL), cases of meningitis by Pseudomonas aeruginosa or gram negative bacilli producers of inducible cephalosporinase. Meropenem is the carbapenem of choice probably in these cases because the carbapenems are often the only active antibiotics and meropenem, specifically, does not have the risk of convulsions observed with imipenem-cilastatin. The carbapenems have shown to be useful in skin and soft tissue infections as well as in obstetric and gynecologic infections as monotherapy similar to the schedules of the currently used antibiotic associations. In the case of nosocomial pneumonias, all the studies have evaluated the carbapenems in monotherapy as useful and effective, specially in the case of pneumonia by gram negative bacilli. Finally, in non filiated nosocomial sepsis and specially in the case of neutropenic patients, the use of carbapenems is particularly attractive in gram negative sepsis in intensive care units. The appearance in the last few years of strains of gram negative bacilli, producers of wide spectrum beta-lactamase or stable repressed hyperproducers of class I chromosomic cephalosporinase, as well as other multiresistant gram negative bacilli, such as Acinetobacter baumanii make the carbapenems, in many cases, the only effective antibiotic in this type of infections.

Anti-Bacterial Agents↗

[Retroperitoneal phlegmon in the left mesenteric-colonic space. A discussion of a case].

It is presented the case of the child with an altered general health situation and with an abdominal suffering; paraclinical investigations were irrelevant and only the surgical exploration had established the diagnosis: a phlegmon with unusual location retroperitoneal, in the left mesenterico-colonic space, next to the mesenteric basis, it is discussed the novel of this location, differential diagnosis with mesenteric adenolimphite, an eventual filiation with a previous thigh phlegmon and it points cut the value of the therapeutical solution.

Abdomen, Acute↗

Chronicle of an incest foretold.

In this paper the author discusses the situation of children handed over to grandparents or to other relatives of the natural parents to be brought up. She notes that such children are faced with the riddle of their own filiation and postulates that this scenario often conceals an oedipal fantasy to the effect that the child concerned is the fruit of an incestuous relationship between a grandparent and the relevant parent. Following the example of Freud, the author adduces literary models for illustration. As with the Oedipus of Sophocles, the author shows how efforts to thwart the workings of fate actually bring about the consummation of the tragedy in the form of incest, which is favoured by the confused oedipal configuration in the families of handed-over children. The main argument is based on the characters and situations of two novels by Gabriel García Márquez, written at different times in his life. With reference to the psychoanalytic literature on artistic creativity, the author shows the importance of the mid-life crisis in determining how García Márquez came to terms with the fact of having himself been entrusted to grandparents as a child and how this situation is reflected in the works concerned.

Humans↗

[Importance of early management of puerperial psychosis. Report of a case].

Recent reports have demonstrated that puerperal psychosis is preceded by prodromic signs. These signs must be recognized in a multidisciplinary context, opening the way to improved diagnosis, therapy and prognosis. On the basis of our clinical experience, we stress the important of early management in women who present prodromic signs of puerperal psychosis. Specific management relies on intensive psychotherapy with daily sessions centered on filiation. With this approach, the emergence of delusions can generally be prevented, usually allowing an uninterrupted mother-infant relationship. We hypothesize that this approach has a favorable influence on later mother-child interactions and cause profound changes in the mother's psychic organization, resulting in a better long-term prognosis. We illustrate our point with a case report.

Female↗