Hernias inguinales y atípicas en la edad pediátrica / Inguinal and atypical hernias in the pediatric age

Las hernias inguinales y cuadros relacionados, como el quiste de cordón o el hidrocele, son la patología quirúrgica más frecuente en la edad pediátrica. No obstante, tienen diferentes características e historia natural lo que condiciona su manejo. por otra parte, hernias crurales, directas o en localizaciones atípicas, son extremadamente infrecuentes en niños, por lo que es importante saber reconocerlas y diferenciarlas a fin de hacer un correcto diagnóstico. En este artículo revisaremos los cuadros más importantes con especial hincapié en su diagnóstico, manejo y tratamiento definitivo

Inguinal hernias and related anomalies, such as spermatic cord cyst or hydrocele, are the most frequent surgical conditions in the pediatric age. However, they have distinct characteristics and natural history which differentiates their management. on the other hand, crural, inguinal direct hernias or those in atypical locations are extremely infrequent in children, therefore it is important to recognize and differentiate them in order to make a correct diagnosis. in this article we will review their most important features with special interest on their diagnosis, management and definitive treatment

Genetic influence of the nonclassical major histocompatibility complex class I molecule MICB in multiple sclerosis susceptibility.

It has been widely reported that the major histocompatibility complex (MHC) class II region provides the main genetic contribution to multiple sclerosis (MS) susceptibility. However, recent studies have suggested that the MHC class I region may also contribute to the development of MS. In this study, we investigated the possible association of the human leukocyte antigen (HLA)-B, MHC class I chain-related gene B (MICB) and MHC class I chain-related gene A (MICA) genes, located in the MHC class I region, with MS susceptibility. For this purpose, we analyzed the distribution of HLA-DR, HLA-B, MICB and MICA alleles in 121 MS patients and 156 healthy controls. Neither HLA-B nor MICA alleles were found to be associated with MS susceptibility, and only the frequency of HLA-DRB1*01 allele was found to be increased in controls (31% vs 14%, P(c) = 0.011). However, MICB*004 allele frequency was significantly increased in MS patients (46.3% vs 23.3%, P(c) < 0.001, odds ratio = 2.82, 95% confidence interval = 1.68-4.73). Although, MICB*004 and HLA-DRB1*15 belong to the AH 7.1 ancestral haplotype, the association of MICB*004 to MS susceptibility was found to be independent of HLA-DRB1*15 in our population. This and previous studies clearly suggest that the MHC class I, in addition to class II, could be involved in MS susceptibility.

Clinical behavior of multiple sclerosis is modulated by the MHC class I-chain-related gene A.

It is well known that certain HLA class II alleles confer an increased risk for developing multiple sclerosis (MS). Recent studies have suggested HLA class I as a region that may also contribute to the development of MS. In this study, we investigated the association between HLA-DR, HLA-B alleles, and major histocompatibility complex (MHC) class I-chain-related gene A (MICA) transmembrane (MICA-TM) polymorphisms and disease progression in 104 MS patients and 116 healthy controls. DR1 was found to be decreased in patients when compared with controls (p(c) = 0.012). Neither HLA-B nor HLA-DR alleles were found to be associated with MS susceptibility. Furthermore, the prevalence of MICA-A5 in patients with relapsing MS was 9% while the prevalence in progressive forms was 42% (p(c) = 0.0015). The extended haplotypes related to MICA-TM5 that were found in our population were DR7-MICA5-B64 (EH 64.1, delta(s) = 0.38), DR4-MICA5-B62 (EH 62.1, delta(s) = 0.28), and DR11-MICA5-B35 (EH35.1, delta(s) = 0.10), but none of them were found to be associated to MS susceptibility or disease progression. Our data could indicate a possible role of MICA-TM in MS prognosis.

[Charles Bonnet's syndrome]. / Síndrome de Charles Bonnet.

Charles Bonnet's syndrome is characterized by the existence of visual hallucinations without psychiatric manifestations or cognitive disorder. Most patients are elderly people with severe visual problems. The objective of this paper is to describe the cases of three patients with this syndrome. The first is an 87 year old woman with bilateral cataract who had visual hallucinations seeing women and faces. The second is another 87 year old woman with advanced myopia and visions of people, animals and objects. The third is a 52 year old woman with atypical pigmentary retinopathy who suffered visual hallucinations of objects and animals in color. The neuroimaging and neurophysiological studies were not contributory. Treatment with neuroleptics or antiepileptics was effective only in one case. We conclude that it is important to know the syndrome and to differentiate it from psychiatric semiology. Deafferentation of the visual cortex could be the decisive factor in the occurrence of visual hallucinations.

Long-term effect of IFNbeta1b treatment on the spontaneous and induced expression of IL-10 and TGFbeta1 in MS patients.

Interferon-beta (IFNbeta) is an effective treatment that lessens the frequency and severity of exacerbations in relapsing-remitting multiple sclerosis (RRMS). The mechanism of action of IFNbeta1b may be by upregulating antiinflammatory cytokines levels. We studied the effect of IFNbeta1b treatment on the in vivo gene expression and protein synthesis of two immunosuppressive cytokines, IL-10 and TGFbeta1, and its persistence with chronic therapy. Peripheral blood samples were obtained from 16 patients before and after 3, 6 and 12 months of IFNbeta1b treatment. Eleven patients did not have any clinical relapse, whereas the other five each had one clinical exacerbation during the study. We employed a highly sensitive RT-PCR technique to study the spontaneous gene expression of IL-10 and TGFbeta1. Protein concentration in serum and in culture supernatants from mitogen-stimulated cells were measured by ELISA. In the group of patients who remained clinically stable during the study, IL-10 mRNA levels decreased significantly after 6 months of treatment to normalize at 1 year of therapy as compare with the initial values. In the five patients who relapsed, mRNA IL-10 levels were significantly diminished at 3, 6, and 12 months of therapy. IL-10 serum levels did not vary significantly in any group of patients during the study. Treatment did not modulate mRNA or serum levels of TGFbeta1 at any time period in the group of stable patients. However, in the five patients who relapsed, TGFbeta1 mRNA significantly decreased at 6 and 12 months of therapy. IFNbeta1b treatment was unable to restore the initial low mitogen-induced production of IL-10; only after 1 year of therapy was a slight increase observed. Cytokine therapy did not affect the mitogen-induced production of TGFbeta1. We can conclude that chronic administration of IFNbeta1b does not result in an upregulation of IL-10 and TGFbeta1.

Interferon beta-1b treatment modulates TNFalpha and IFNgamma spontaneous gene expression in MS.

BACKGROUND: Interferon beta (IFNbeta) lessens the overall frequency of acute attacks in patients with the relapsing-remitting form of multiple sclerosis (RRMS). IFNbeta may act by decreasing the synthesis of inflammatory cytokines. OBJECTIVES: To determine whether IFNbeta-1b treatment had an initial and sustained effect on the in vivo synthesis and secretion of tumor necrosis factor alpha (TNFalpha) and IFNgamma. METHODS: A highly sensitive reverse-transcriptase PCR technique was used to measure baseline levels of mRNA in freshly isolated cells from patients before therapy and at 3, 6, and 12 months of treatment. Also, protein concentration was measured in serum and in culture supernatants from mitogen-stimulated cells. The authors studied 16 patients, of whom 11 did not have clinical exacerbations, whereas 5 had one clinical relapse each during the study. RESULTS: Mean values of TNFalpha mRNA levels in the 11 stable patients decreased significantly at 3 and 6 months of treatment in comparison with initial data. After 6 months of therapy, IFNbeta-1b downmodulated TNFalpha transcripts in the 5 patients who experienced relapse. In this group of patients, TNFalpha levels rose sharply to reach pretreated values at 1 year of IFNbeta-1b treatment. At the beginning of therapy, 6 patients had high concentrations of serum TNFalpha, which decreased to normal values following IFNbeta-1b therapy. IFNgamma mRNA expression also diminished after 6 and 12 months of IFNbeta-1b therapy in the group of stable patients, whereas nonrelevant variations were observed in patients who had one relapse. Initially, patients' peripheral mononuclear cells secreted diminished amounts of TNFalpha and IFNgamma on PHA + PMA mitogen stimulation in comparison with normal control subjects. After 1 year of therapy, IFNbeta-1b restored the normal production of TNFalpha, whereas therapy did not restore IFNgamma secretion to control values. CONCLUSION: IFNbeta-1b decreases the spontaneous expression of two proinflammatory cytokines.

Influence of age and parity on the distribution of cells expressing major histocompatibility complex class II, CD4, or CD8 molecules in the endometrium of mares during estrus.

OBJECTIVE: To evaluate effect of age and parity on distribution and number of cells expressing major histocompatibility complex (MHC) class II, CD4, or CD8 molecules in the endometrium of mares during estrus. ANIMALS: 32 gynecologically healthy mares, categorized as young (3 to 8 years; n = 17) or old (9 to 16 years; 15) and nulliparous (n = 6), nulliparous embryo donors (16), or parous (10). PROCEDURES: Endometrial specimens collected from the uterine body and horns during estrus were stained by use of the avidin-biotin-peroxidase method, using monoclonal antibodies against equine MHC class II, CD4, and CD8 molecules. Labeled cells in the stratum compactum within 5 randomly selected fields at 400x magnification (total area = 0.31 mm2) were counted, and numbers were compared among groups and between locations. RESULTS: Age did not affect cell numbers within the 3 cell subsets examined. Numbers in each subset were higher in the uterine body than in the horns, although the difference was not significant for cells expressing MHC class II. Significantly more cells expressing MHC class II molecules were detected in the uterine body of nulliparous and parous mares than in embryo donors, whereas in the horns, these cells were significantly higher in number only in parous mares. Parity did not affect number of CD4+ or CD8+ cells. CONCLUSIONS AND CLINICAL RELEVANCE: The increased likelihood for endometritis to develop in mares as they age cannot be explained by a decrease in number of cells expressing MHC class II, CD4, or CD8 molecules within the endometrium. However, greater number of cells within these 3 subsets detected in the uterine body, compared with the horns, during estrus suggests a local readiness to act against microorganisms or semen introduced during mating or insemination.

Glucocorticoids increase IL-10 expression in multiple sclerosis patients with acute relapse.

High doses of glucocorticoids (GCs) are widely employed to treat acute attacks in relapsing-remitting multiple sclerosis (MS) patients. Their beneficial effects are partially due to their capacity to regulate the cytokine network. In the present work, we have examined the effect of GCs on the production of the immunosuppressor cytokine IL-10. Blood samples from MS patients suffering an acute relapse were obtained immediately before initiating therapy and after receiving a daily dose of 1 g intravenous methylprednisolone (MP) for four days. Levels of IL-10 mRNA in PBMC were semiquantified by RT-PCR, whereas protein concentration in serum and in cell culture supernatant was measured by ELISA. Our results show that 7 out of the 9 patients studied displayed increased IL-10 mRNA expression as well as higher serum IL-10 concentration following steroid treatment. In contrast, mRNA expression of two inflammatory cytokines, TNFalpha and IFNgamma, decreased following steroid therapy. In vitro experiments employing normal PBMC showed that methylprednisolone (MP) upregulated IL-10 expression as determined by measuring mRNA levels, flow cytometry of intracytoplasmic protein concentration, and the amount of secreted protein. Peak responses of secreted IL-10 by PBMC cultured cells treated with MP were obtained at 48 h. The effect was steroid-specific as IL-10 expression reversed to baseline levels in the presence of the glucocorticoid receptor antagonist RU486. Contrary to the effect of MP on the spontaneous expression of IL-10, this drug downregulated LPS-induced IL-10 synthesis. In fact, the concentration of IL-10 in LPS-induced IL-10 secretion from normal PBMC decreased upon addition of MP to cell cultures. Thus, it seems that MP exerts an opposite effect on the spontaneous and LPS-induced IL-10 production. Our studies indicate that GCs may control inflammatory responses by upregulating production of the immunosuppressor cytokine IL-10.

Concentrations of total protein, albumin and immunoglobulins in undiluted uterine fluid of gynecologically healthy mares.

Undiluted uterine fluid from 20 Warmblood/Standardbred mares (5 to 14 yr old) was recovered by absorption to an intrauterine tampon. The mares were considered gynecologically healthy based on a clinical examination including uterine swabs for cytology and bacteriology as well as endometrial biopsy examinations. The protein profiles (SDS-PAGE) and concentrations of total protein, albumin, and immunoglobulins (Ig) A and G in the uterine fluid were examined and compared with the same proteins in serum. Major peaks were identified on the obtained protein profiles, and there was a clear similarity between the serum profiles and uterine fluid profiles. Variability in protein concentrations among mares was considerably larger in uterine fluid than in serum. Concentrations of the various proteins in uterine fluid were 44 to 56% of those in serum, except for IgA, which had a similar concentration in both serum and uterine fluid. Concentration of the proteins corresponding to peak No. 3 (molecular weight 60 to 71 kDa) in uterine fluid was higher (P < 0.05) in younger mares than in older ones. Parity had no effect on the recorded protein concentrations. The present study of gynecologically healthy mares showed that there is a large individual variation in the protein composition of uterine fluid. The results suggest that age, but not parity, may affect this composition, and indicate further that there is considerable transudation to the uterine cavity.

Base anatómica del colgajo fronto-nasal de Marchac / Anatomical basis of Marchacs frontonasal flap

Se presenta la experiencia de 17 casos de cáncer de nariz cuya reconstrucción se realizó con el Colgajo Fronto-nasal Pediculado de Marchac. Las operaciones se hicieron entre 1988 y 1996 en 9 mujeres y 8 hombres, con edades de 52 a 79 años. Se describen con detalle las características de la arteria Dorsal de la nariz, base nutricia de este colgajo, y diferentes aspectos anatómicos de la técnica para que sea realizada con mayor seguridad quirúrgica. Se evalúan las ventajas del procedimiento como método de elección para reconstruir parcialmente el dorso de las paredes laterales de la nariz

Base anatómica del colgajo fronto-nasal de Marchac / Anatomical basis of Marchac's frontonasal flap

Se presenta la experiencia de 17 casos de cáncer de nariz cuya reconstrucción se realizó con el Colgajo Fronto-nasal Pediculado de Marchac. Las operaciones se hicieron entre 1988 y 1996 en 9 mujeres y 8 hombres, con edades de 52 a 79 años. Se describen con detalle las características de la arteria Dorsal de la nariz, base nutricia de este colgajo, y diferentes aspectos anatómicos de la técnica para que sea realizada con mayor seguridad quirúrgica. Se evalúan las ventajas del procedimiento como método de elección para reconstruir parcialmente el dorso de las paredes laterales de la nariz

Ultrastructure of the secretory endometrium during oestrus in young maiden and foaled mares.

Cyclical accumulation of uterine fluid occurs during oestrus and is often seen in excessive volumes in mares considered susceptible to endometritis. Since the mechanisms behind the formation of free uterine fluid remain to be clarified, the fine structure of the secretory equine endometrium was studied in biopsies collect during videoendoscopy from 14 endometritis-free, 4-9-year-old mares during oestrus. A distinct oedema of the tunica mucosa was evident. The surface epithelium had both ciliated and nonciliated cells and, particularly at the uterine body, often presented intra-epithelial macrophages. The epithelial cells of the gland duct were similar to the surface epithelium, except that the nonciliated cells lacked secretory vesicles in the non ciliated cells. This glandular epithelium presented clear signs of secretory activity with conspicuous secretory vesicles holding electron-dense granula in the adluminal cytoplasm and a well developed supranuclear Golgi apparatus. Secretory products as well as cell debris were commonly found in the lumen of the glands. No clear signs of apocrine secretion were found and it seemed therefore, that the mechanism of secretion is merocrine, i.e. by exocytosis. The endometrial oedema and intense secretory activity, both under oestrogenic influence, contribute to the building up of the uterine fluid during oestrus. No differences in morphology of the secretory endometrium could be noticed between nulliparous mares and mares that had had 1 or 2 foals.

[Prognostic factors of supratentorial cerebrovascular accidents]. / Factores pronósticos en los accidentes cerebrovasculares supratentoriales.

Our aim was to delimit prognostic factors in supratentorial stroke based on data obtained upon hospitalization. We studied two series of patients, the first being 150 with brain infarct and the second 135 having intracerebral haemorrhage. We analyzed: age, Glasgow and Canadian scales, glucose and urgence haemogram and the size of the lesion across its greatest diameter using computerized tomography (CT). Follow-up time was until death or one year after the stroke. Those who lived longer than one year after were subclassified according to the Rankin scale as < 3 and > or = 3. There was a significant difference between those who survived for less than one month and those surviving more than one year: their age (p < 0.01), average score on the scale (p < 0.001) and size of infarct (p < 0.05) or haematoma (p < 0.001). The Rankin subgroups < 3 and > or = 3 also differed significantly with regard to age. Noteworthy were the unfavourable data: Glasgow < 10 points and Canadian < 5 points, in infarcts > 6 cm and haematomas > 4 cm in diameter. We comment on other evolutionary variables which may influence prognostic assessment such as clinical deterioration or CT sensitivity of the infarct depending on the carry-out time.

[Recurrent confusional states and periodic lateralized epileptiform discharges: a new type of non-convulsive status epilepticus?]. / Síndrome confusional recurrente y complejos periódicos: un nuevo tipo de status no convulsivo?

Periodic lateralized epileptiform discharges (PLEDs) in an EEG usually indicate the presence of an underlying structural lesion (of vascular origin in most cases). PLEDs are also sometimes observed in certain types of infections (mainly viral), in which they may even constitute a characteristic finding useful for diagnostic purposes. In recent years cases have been reported in which PLEDs are linked to recurring confusional states that do not fit in with established classifications and that may be epileptic in nature. We discuss the cases of 2 patients who were repeatedly admitted to our hospital in confusional states, in whom PLEDs were observed in EEG readings. Clinical evolution in both cases paralleled EEG alterations. We were able to perform both critical and intercritical single proton emission tomography on 1 patient, finding, respectively, hyper- and hypoabsorption foci. Symptoms resolved with antiepileptic treatment.

[Janz's juvenile myoclonic epilepsy: a little-known frequent syndrome. A study of 85 patients]. / La epilepsia mioclónica juvenil de Janz: un síndrome frecuente poco conocido. Estudio de 85 pacientes.

BACKGROUND: Juvenile myoclonic epilepsy (JME) constitutes 10% of all epilepsies. Despite this syndrome being well defined, its diagnosis is usually delayed. The aim of this study was to analyze the clinical and electroencephalographic characteristics to facilitate guidelines to contribute to its recognition. METHODS: From January 1986 to July 1993 the clinical and EEG data of 85 patients with JME were prospectively studied. In 68 cases (80%) the polygraphic study of sleep was also analyzed during a nap period. RESULTS: The series included 44 males and 41 females of a mean age of 28 years (range: 13-63). Fifty-six percent of the cases showed family history of epilepsy and/or febrile convulsions. All the patients had myoclonic crisis with the age of 15 being the mean age of initiation (range: 8-27). Eighty-seven percent also had generalized tonic-clonic crisis and 18% typical absences. Myoclonias were presented daily up the administration of adequate treatment in 60% of the cases with 21% having myoclonic status. The mean interval from the initiation of the myoclonic crisis to diagnosis of JME was of 10.6 years. On monotherapy with valproic acid and following a mean follow up period of 23.8 months, 86% of the patients remained free of crisis. Nonetheless, the rate of recurrence was 100% in the 19 patients who discontinued the treatment. Surveillance EEG was normal on some occasion in 88% of the cases. The most characteristic paroxysms were the following: wave-point at 4-5 Hz and generalized rapid wave-polypoint. Light stimulation provoked a paroxysmal response in one third of the cases. Sleep EEG was abnormal in all the patients. An activation of the paroxysms during non-REM sleep in 78% of the cases and on waking up in 25%. CONCLUSIONS: Juvenile myoclonic epilepsy is a well defined syndrome. Its diagnosis is based on directed anamnesis allowing myoclonic jerks to be collected which often remain unperceived, and EEG exploration with sleep tracing in which the characteristic outbreaks of wave-point or generalized rapid wave-polypoints may be discovered.