Primordial odontogenic tumour of the maxilla: case report and review of the literature.

Primordial odontogenic tumour (POT) is a relatively newly described entity with well-defined clinicopathological features. Since its initial description in 2014, 22 cases of POT have been reported in the literature. Only five cases of POT have arisen in the maxilla. This article describes an additional patient with a POT of the maxilla and provides a review of the literature on POT.

[Appendicular actinomycosis as a cause of ileocecal mass in children: Case report]. / Actinomicosis apendicular como causa de masa ileocecal en la edad pediátrica: Caso clínico.

INTRODUCTION: Appendicular actinomycosis is a rare disease produced by cecal appendix invasion by Actinomyces gender bacteria after a predisposing cause that disrupts mucosal barrier, such as acute appendicitis. The highest frequency appears in adults; however, there are cases in paediatric age described in literature. The aim of this article is to introduce a case of appendicular actinomycosis in a child with atypical presentation and to review the literature. CLINICAL CASE: 10-year-old boy transferred to our hospital due to intraoperative finding at a different centre of a right flank tumor, without identification of cecal appendix. After completing the study with image tests, the patient was re-intervened with the diagnosis of complicated acute appendicitis. Intraoperatively, an organized plastron lacking of acute inflammatory signs was found. Post-surgical evolution was positive with conventional antibiotic treatment. Histological study of the specimen revealed an appendicular actinomycosis, so antibiotic treatment was widened in an outpatient basis and predisposing diseases were ruled out. COMMENTS: Appendicular actinomycosis is an infrequent cause of appendicitis that can simulate an intra-abdominal tumor in children and adults. It is necessary to rule out predisposing diseases and to administer an adequate antibiotic treatment.

INTRODUCCION: La actinomicosis apendicular es una patología rara producida por la invasión del apéndice cecal por bacterias del género Actinomyces al alterarse la barrera mucosa después de una causa predisponente, como podría ser una apendicitis aguda. Se presenta con mayor frecuencia en adultos, aunque en la literatura se han descrito algunos casos en edad pediátrica. En este artículo se presenta un nuevo caso pediátrico de actinomicosis apendicular de presentación atípica y se revisa la literatura al respecto. CASO CLINICO: Paciente varón de 10 años de edad derivado a nuestro hospital por el hallazgo intraoperatorio en otro centro de una tumoración en flanco derecho, sin identificación del apéndice cecal. Tras completar el estudio con pruebas de imagen, el paciente fue reintervenido bajo la orientación diagnóstica de apendicitis aguda complicada. Intraoperatoriamente, se identifica un plastrón organizado y con escasos signos inflamatorios agudos. La evolución postquirúrgica fue favorable con tratamiento antibiótico convencional. El estudio anatomopatológico de la pieza reveló una actinomicosis apendicular, por lo que se amplió el tratamiento antibiótico de forma ambulatoria y se descartó patología predisponente. COMENTARIOS: La actinomicosis apendicular es una causa infrecuente de apendicitis que puede simular una tumoración intraabdominal en niños y adultos. Es necesario descartar patología predisponente y realizar un tratamiento antibiótico adecuado.

Alveolar capillary dysplasia with misalignment of pulmonary [corrected] veins: concordance between pathological and molecular diagnosis.

We report the case of a newborn with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV), a rare condition of unknown etiology presenting in the neonatal period with significant persistent pulmonary hypertension. The diagnosis was made by lung biopsy and confirmed at autopsy. Specific genetic analysis demonstrated defects in the FOXF1 gene. The diagnosis of ACD/MPV requires a high level of suspicion and is made by lung biopsy or necropsy examination by a pediatric pathologist with experience in this condition. The availability of genetic testing has led to increasing diagnosis of patients with this lethal disorder and can influence their management, specifically by indicating the need for lung biopsy in a critically ill newborn.

Novel histologic scoring system for long-term allograft fibrosis after liver transplantation in children.

The existing systems for scoring fibrosis were not developed to evaluate transplanted livers. Our aim was to design and validate a novel fibrosis scoring system specifically adapted to assess liver allograft fibrosis (LAF). Clinical data, histology, transient elastography (TE) and AST/platelet ratio index (APRI) were reviewed in 38 pediatric liver transplant (LT) recipients. Protocol liver biopsies performed at 6 months and 7 years post-LT were reviewed by three pathologists who assessed LAF using the METAVIR and Ishak systems. LAF was also scored separately in portal (0-3), sinusoidal (0-3) and centrolobular areas (0-3). Scoring evaluations were correlated with fibrosis quantification using morphometry, and also with TE and APRI. Statistical correlations between morphometry and METAVIR were 0.571 (p < 0.000) and 0.566 (p < 0.000) for the Ishak system. The novel score (0-9) for separate assessment of portal, sinusoidal and centrolobular fibrosis showed a better correlation with morphometry (0.731; p < 0.000) and high intra-/interobserver agreement (0.966; p < 0.000 and 0.794; p < 0.000, respectively). No correlation was found between TE or APRI and morphometry or the three histologic scores. In conclusion, this novel semiquantitative fibrosis scoring system seems to more accurately reflect LAF than the existing scoring system and may become a practical tool for staging fibrosis in LT.

Selection of Arabidopsis mutants overexpressing genes driven by the promoter of an auxin-inducible glutathione S-transferase gene.

Transgenic arabidopsis plants were isolated that contained a T-DNA construct in which the promoter of an auxin-inducible glutathione S-transferase (GST) gene from tobacco was fused to the kanamycin resistance (nptII) as well as to the beta-glucuronidase (gusA) reporter gene. Subsequently, seeds were treated with EMS to obtain mutants in which both reporter gene fusions were up-regulated. Northern analysis showed that the mRNA level of a related, endogenous auxin-inducible GST gene of Arabidopsis was increased in some of these mutants as well. Two of the gup (GST up-regulated) mutants were characterized in more detail and roughly mapped. Both had epinastic cotyledons and leaves, a phenotype that turned out to be linked to the gup mutation.

Overexpression of a novel Arabidopsis gene related to putative zinc-transporter genes from animals can lead to enhanced zinc resistance and accumulation.

We describe the isolation of an Arabidopsis gene that is closely related to the animal ZnT genes (Zn transporter). The protein encoded by the ZAT (Zn transporter of Arabidopsis thaliana) gene has 398 amino acid residues and is predicted to have six membrane-spanning domains. To obtain evidence for the postulated function of the Arabidopsis gene, transgenic plants with the ZAT coding sequence under control of the cauliflower mosaic virus 35S promoter were analyzed. Plants obtained with ZAT in the sense orientation exhibited enhanced Zn resistance and strongly increased Zn content in the roots under high Zn exposure. Antisense mRNA-producing plants were viable, with a wild-type level of Zn resistance and content, like plants expressing a truncated coding sequence lacking the C-terminal cytoplasmic domain of the protein. The availability of ZAT can lead to a better understanding of the mechanism of Zn homeostasis and resistance in plants.

[Nitric oxide synthase activity in nasal mucosa]. / Actividad óxido nítrico sintasa en la mucosa nasal.

Nitric oxide (NO) plays an important regulatory role in airway function and seems to be implicated in the pathophysiology of several airway diseases. We studied the presence of NO synthase activity in human nasal mucosa and nasal polyp tissues obtained from patients undergoing septoplasty or polypectomy, respectively. NO synthase activity was quantified in tissue homogenates using citrulline release assay and was located in tissue sections using NADPH-diaphorase histochemistry. The results indicated that nasal polyps contain higher levels of total NO synthase activity than nasal mucosa tissue. In addition, nasal polyps contained mainly inducible NO synthase activity whereas all NO synthase activity detected in the nasal mucosa was in constitutive form. In both cases, NO synthase activity was localized in epithelial cells. In view of these results, we conclude that NO may be an important inflammatory mediator in the respiratory system and that the epithelium may be a source of NO production.

[Recurrent peripheral facial paralysis. Our case load from 1995]. / Parálisis facial periférica recidivante. Nuestra casuística del año 1995.

Of 117 cases of peripheral facial palsy seen in our emergency room in 1995, 10 (8.5%) were relapses. Seven were males and 3, females. Mean age at the first appearance was 23.6 years. The mean interval between the first facial palsy and the first recurrence was 10.1 years, and between the first and second recurrence, 4.6 years. Five of the 10 patients (50%) had a second recurrence. Two of the 10 patients (20%) had a family history of facial palsy. Only 1 (10%) was diabetic. Seven of 10 patients achieved a complete functional recovery and 3 had sequelae. Computed tomography was normal in every patient. In our series, a family history of facial palsy and low ENoG results were related with a poorer prognosis. ENoG scan was used as a prognostic and follow-up factor.

[Omeprazole: a new treatment for paranasal sinus polyps in Widal syndrome. Preliminary study]. / Omeprazol: un nuevo tratamiento de la poliposis nasosinusal en el síndrome de Widal. Estudio preliminar.

A preliminary report is made of the potential therapeutic effect of omeprazol in reducing nasosinusal polyps. This study is based on the empirical observation of nasal airflow improvement in patients suffering from nasosinusal polyposis after administering omeprazol. Different phases of the study suggested that patients with Widal's syndrome benefited the most. Based on the results of this study, we have undertaken a randomized, parallel, double-blind, placebo-controlled clinical trial.

[Submucous urethral angioma]. / Angioma uretral submucoso.

OBJECTIVES: Urethral hemangioma is a rare benign vascular tumor. The clinical features of this tumor type and the therapeutic options are discussed. METHODS/RESULTS: We report on a 55-year-old male with urethral hemangioma who consulted for urethral hemorrhage. CONCLUSIONS: To our knowledge, approximately 40 cases of this rare tumor type have been reported in the literature. Urethroscopy is the best diagnostic procedure. Treatment depends on tumor site, size and number. Transurethral resection of the tumor, urethrectomy, arterial embolization, radiotherapy or ablation with Nd:YAG laser can be utilized.

[An etiopathogenetic and prognostic study of sudden sensorineural hypoacusis]. / Aportación al estudio etiopatogénico y pronóstico de la hipoacusia neurosensorial brusca.

From October 1987 till November 1989 the AA. have studied 40 cases with Sensorineural sudden hypoacusis. The control term varied between 3 months and 2 years. The whole group followed an scheduled prospective protocol with the aim of find out the more significative factors regarding the etiology and prognostic of the process. On the other hand, minimize the cost of explorations, spare nuisances to the patients and select the most reliable methods were the purposes of the AA.

Studies of cell-surface glorin receptors, glorin degradation, and glorin-induced cellular responses during development of Polysphondylium violaceum.

The chemoattractant mediating cell aggregation in the slime mold Polysphondylium violaceum is N-propionyl-gamma-L-glutamyl-L-ornithine-delta-lactam ethylester (glorin). Here we examine the binding properties of tritiated glorin to intact P. violaceum cells. Scatchard analysis of binding data yielded slightly curvilinear plots with Kd values in the range of 20 and 100 nM. The number of glorin receptors increased from 35,000 in the vegetative stage to 45,000 per cell during aggregation. Later, during culmination receptor numbers decreased to undetectable levels (less than 1000). The receptor binding kinetics show binding equilibrium within 30 s at 0 degrees C, and ligand dissociation occurs from two kinetically distinct receptors whose half-times were 2 s for 72% of the bound glorin and 28 s for the remainder. The enzymatic degradation of glorin did not affect binding data during incubations of up to 1 min at 0 degrees C. Two glorinase activities were observed. An ornithine delta-lactam cleaving activity with a Km of ca. 10(-4) M and a propionic acid removing activity (Km 10(-5) M), both of which were detected mainly on the cell surface. Cleavage of the lactam occurred at a higher rate than removal of propionic acid. Lactam-cleaved glorin showed no chemotactic activity nor did it bind to cell-surface glorin receptors. Cell-surface-bound glorinase activity and glorin-induced cGMP synthesis were developmentally regulated, peaking at aggregation. In the most sensitive stage half-maximal responses (cGMP synthesis, chemotaxis, light-scattering) were elicited in the 10-100 nM range. Neither cAMP synthesis nor glorin-induced glorin synthesis was observed. Guanine nucleotides specifically modulated glorin receptor binding on isolated membranes, and, conversely, glorin modulated GTP gamma S binding to membrane preparations. Our results support the notion that glorin mediates chemotactic cell aggregation in P. violaceum acting via cell-surface receptors, G-proteins, and cGMP accumulation.

Postaggregative differentiation induction by cyclic AMP in Dictyostelium: intracellular transduction pathway and requirement for additional stimuli.

Cyclic AMP induces postaggregative differentiation in aggregation competent cells of Dictyostelium by interacting with cell surface cAMP receptors. We investigated the transduction pathway of this response and additional requirements for the induction of postaggregative differentiation. Optimal induction of postaggregative gene expression requires that vegetative cells are first exposed to 2-4 hr of nanomolar cAMP pulses, and subsequently for 4-6 hr to steady-state cAMP concentrations in the micromolar range. Cyclic AMP pulses, which are endogenously produced before and during aggregation, induce full responsiveness to cAMP as a morphogen. The transduction pathway from the cell surface cAMP receptor to postaggregative gene expression may involve Ca2+ ions as intracellular messengers. A cAMP-induced increase in intracellular cAMP or cGMP levels is not involved in the transduction pathway.

Analogs of cyclic AMP as chemoattractants and inhibitors of Dictyostelium chemotaxis.

Aggregative amoebae of Dictyostelium discoideum, D. mucoroides, D. purpureum, and D. rosarium react chemotactically to cyclic AMP (cAMP). We measured the chemotactic activity of 14 cAMP analogs and found that these four species have a similar sensitivity to chemical modifications of cAMP; this suggests that the cAMP receptor is identical in all of these species. Besides the induction of a chemotactic response, cAMP analogs also may delay or prevent cell aggregation. cAMP analogs like N1-O-cAMP, 2'-H-cAMP, and 5'NH-cAMP are chemotactically nearly as active as cAMP and induced no, or only a short, delay of cell aggregation. Other cAMP derivatives, such as 6-Cl-cPMP and 8-Br-cAMP, are chemotactically active only at high concentrations and delayed cell aggregation for several hours. Still other cAMP analogs, which do not induce a chemotactic reaction in D. mucoroides, D. purpureum, and D. rosarium, either prevented cell aggregation [cAMPS(S), cAMPS(R), and 3'-NH-cAMP[ or had no effect on cell aggregation [cAMPN(CH3)2(S) and cAMPN(CH3)2(R)]. cAMP analog 3'-NH-cAMP prevented cell aggregation by the inhibition of chemotaxis, whereas cell locomotion was not affected. Although we cannot provide a satisfactory explantation for these observations, our data suggest that occupation and activation of the cAMP receptors do not always induced a chemotactic response.