Ewing-like sarcoma bladder primary tumour: A case report and literature review.

Bladder primitive neuroectodermal tumors are extremely rare but are most frequent in older adult. 59-year-old man that complained of hematuria for the previous 24 h, urethral syndrome, and pain in the right renal fossa over the previous two weeks. No definitive management or treatment guidelines have been established. Hematuria is the most frequent symptom. Advanced age, metastasis, and incomplete tumor resection are determinants of a poor prognosis. Ewing-like bladder primary tumor is a rare entity with a poor prognosis, hence an aggressive treatment combining surgery and chemotherapy must be considered from the beginning.

Priapismo de alto flujo en población pediátrica: serie de casos y revisión de la literatura / High-flow Priapism in Pediatric Population: Case Series and Review of the Literature

Introducción: El priapismo consiste en una erección mantenida durante más de cuatro horas. Es una patología infrecuente en la población pediátrica, estimada en 0,3 a 1,5 por cada 100.000 niños al año. La secuencia diagnóstica incluye anamnesis, exploración física y ecografía doppler peneana (EcoDP). No siempre es necesaria la punción de cuerpos cavernosos para establecer el diagnóstico diferencial entre priapismo de alto y bajo flujo. El tratamiento de elección en la edad pediátrica no está bien definido.Material y métodosEstudio multicéntrico, retrospectivo, descriptivo de pacientes menores de 14 años con priapismo de alto flujo, entre los años 2010 y 2020. Revisión de la literatura.ResultadosUn total de siete pacientes fueron diagnosticados de priapismo de alto flujo. Ninguno requirió punción de cuerpos cavernosos. Se realizó un manejo conservador en todos ellos, dos pacientes necesitaron embolización arterial superselectiva por persistencia de la clínica.ConclusionesEl priapismo de alto flujo es una entidad muy infrecuente en la edad pediátrica por lo que es importante conocer el diagnóstico y manejo adecuados. Actualmente, la ecografía doppler suele ser suficiente para el diagnóstico, obviando el uso de la gasometría. El manejo inicial en niños es conservador, reservando la embolización para los casos refractarios. (AU)

Introduction: Priapism is a prolonged erection that lasts longer than four hours. It is a rare pathology in the pediatric population, with an estimation of 0.3-1.5 per 100,000 children per year. The diagnostic sequence includes clinical history, physical examination and penile Doppler ultrasound (PDUS). Puncture of corpora cavernosa is not always necessary to establish the differential diagnosis between high-flow and low-flow priapism. The treatment of choice in pediatric age is not well defined.Patients and methodsMulticentric, retrospective and descriptive study including patients under 14 years with high-flow priapism between 2010 and 2020. Literature review.ResultsA total of seven patients were diagnosed with high-flow priapism. None of them required puncture of the corpora cavernosa. Patients were treated with a conservative management, two patients required superselective arterial embolization due to persistent symptoms.ConclusionsHigh-flow priapism is a very rare entity in pediatric age; therefore, knowing the proper diagnosis and management is crucial. Currently, penile doppler ultrasound is enough for diagnosis in most cases and allows obviating the use of blood gas analysis. Children should be initially treated with a conservative management, reserving embolization for refractory cases. (AU)

High-flow priapism in pediatric population: Case series and review of the literature.

INTRODUCTION: Priapism is a prolonged erection that lasts longer than four hours. It is a rare pathology in the pediatric population, with an estimation of 0.3-1.5 per 100,000 children per year. The diagnostic sequence includes clinical history, physical examination and penile Doppler ultrasound (PDUS). Puncture of corpora cavernosa is not always necessary to establish the differential diagnosis between high-flow and low-flow priapism. The treatment of choice in pediatric age is not well defined. PATIENTS AND METHODS: Multicentric, retrospective and descriptive study including patients under 14 years with high-flow priapism between 2010 and 2020. RESULTS: A total of seven patients were diagnosed with high-flow priapism. None of them required puncture of the corpora cavernosa. Patients were treated with a conservative management, two patients required superselective arterial embolization due to persistent symptoms. CONCLUSIONS: High-flow priapism is a very rare entity in pediatric age; therefore, knowing the proper diagnosis and management is crucial. Currently, penile doppler ultrasound is enough for diagnosis in most cases and allows obviating the use of blood gas analysis. Children should be initially treated with a conservative management, reserving embolization for refractory cases.

High-flow Priapism in Pediatric Population: Case Series and Review of the Literature. / Priapismo de alto flujo en población pediátrica: serie de casos y revisión de la literatura.

INTRODUCTION: Priapism is a prolonged erection that lasts longer than four hours. It is a rare pathology in the pediatric population, with an estimation of 0.3-1.5 per 100,000 children per year. The diagnostic sequence includes clinical history, physical examination and penile Doppler ultrasound (PDUS). Puncture of corpora cavernosa is not always necessary to establish the differential diagnosis between high-flow and low-flow priapism. The treatment of choice in pediatric age is not well defined. PATIENTS AND METHODS: Multicentric, retrospective and descriptive study including patients under 14 years with high-flow priapism between 2010 and 2020. RESULTS: A total of seven patients were diagnosed with high-flow priapism. None of them required puncture of the corpora cavernosa. Patients were treated with a conservative management, two patients required superselective arterial embolization due to persistent symptoms. CONCLUSIONS: High-flow priapism is a very rare entity in pediatric age; therefore, knowing the proper diagnosis and management is crucial. Currently, penile doppler ultrasound is enough for diagnosis in most cases and allows obviating the use of blood gas analysis. Children should be initially treated with a conservative management, reserving embolization for refractory cases.

Toxicity of phospholipases A2 D49 (6-1 and 6-2) and K49 (Bj-VII) from Bothrops jararacussu venom.

Purified phospholipase A2 (PLA2) enzymes from Bothrops jararacussu snake venom were examined to evaluate NIH 3T3 and COS7 fibroblast cytotoxicity, as well as muscle myotoxic and inflammatory activities. Separation of fractions Bj-VII (from BthTX-I; a Lys49 PLA2 homolog) and 6-1 and 6-2 (from BthTX-II; an Asp49 PLA2) from B. jararacussu snake venom by SDS-PAGE in tricine buffer in the absence and presence of dithiothreitol revealed a homodimer with an estimated molecular mass of approximately 30 kDa (monomer mass approximately 15 kDa). This finding indicates that these toxins form dimeric complexes-a previously reported tendency among PLA2s. These toxins were assayed for viability with the MTT assay, which is used to examine the effects of phospholipases on the mitochondrial viability of cells. The toxins were also assayed for cytolysis of the fibroblast cell lines NIH 3T3 and COS7 by quantification of lactate dehydrogenase released into the medium. The results indicate that the PLA2s 6-1, 6-2 and the Bj-VII PLA2 homolog studied here induce moderate footpad edema and local myotoxicity. Moreover, exposure to these phospholipases led to a reduction in fibroblast viability; at the 1 muM dose of PLA2 tested, a reduction of 50% in cell viability was observed. The present findings indicate that the inflammatory activity observed in envenomation may be correlated with the cytotoxicity observed in fibroblasts.

Purification and n-terminal sequencing of two presynaptic neurotoxic PLA2, neuwieditoxin-I and neuwieditoxin-II, from Bothrops neuwiedi pauloensis (jararaca pintada) venom

Two presynaptic phospholipases A2 (PLA2), neuwieditoxin-I (NeuTX-I) and neuwieditoxin-II (NeuTX-II), were isolated from the venom of Bothrops neuwiedi pauloensis (BNP). The venom was fractionated using molecular exclusion HPLC (Protein-Pak 300SW column), followed by reverse phase HPLC (æBondapak C18 column). Tricine-SDS-PAGE in the presence or absence of dithiothreitol showed that NeuTX-I and NeuTX-II had a molecular mass of approximately 14 kDa and 28kDa, respectively. At 10æg/ml, both toxins produced complete neuromuscular blockade in indirectly stimulated chick biventer cervicis isolated preparation without inhibiting the response to acetylcholine, but NeuTX-II reduced the response to KCl by 67.0±8.0 percent (n=3; p<0.05). NeuTX-I and NeuTX-II are probably responsible for the presynaptic neurotoxicity of BNP venom in vitro. In fact, using loose patch clamp technique for mouse phrenic nerve-diaphragm preparation, NeuTX-I produced a calcium-dependent blockade of acetylcholine release and caused appearance of giant miniature end-plate potentials (mepps), indicating a pure presynaptic action. The N-terminal sequence of NeuTX-I was DLVQFGQMILKVAGRSLPKSYGAYGCYCGWGGRGK (71 percent homology with bothropstoxin-II and 54 percent homology with caudoxin) and that of NeuTX-II was SLFEFAKMILEETKRLPFPYYGAYGCYCGWGGQGQPKDAT (92 percent homology with Basp-III and 62 percent homology with crotoxin PLA2). The fact that NeuTX-I has Q-4 (Gln-4) and both toxins have F-5 (Phe-5) and Y-28 (Tyr-28) strongly suggests that NeuTX-I and NeuTX-II are Asp49 PLA2.

[Prenatal diagnosis of congenital diaphragmatic hernia: evaluation of the prognosis]. / Diagnostic prénatal de la hernie des coupoles diaphragmatiques: évaluation du pronostic.

OBJECTIVE: The aim of this study was to evaluate prognostic prenatal factors of congenital diaphragmatic hernia. MATERIAL AND METHODS: We designed a retrospective study of 34 patients with congenital diaphragmatic hernia. The infants were delivered at the Edouard Herriot Hospital between September 1, 1994 and June 30, 1998. We excluded cases of pregnancy termination. After eliminating 4 cases, we studied 30. The factors studied were: polyhydramnios, transverse abdominal diameter, hepatic and umbilical vein deviation, mediastinal deviation, intrathoracic location of the stomach. Prenatal karyotype and echocardiography were systematically obtained. RESULTS: Total perinatal mortality was 53% and was 48% in case of unique diaphragmatic hernia. Prognosis was poorer if the diaphragmatic hernia was associated with another malformation (perinatal mortality: 80%, 4/5 cases), or in case of preterm delivery (83%, 5/6 cases). Factors associated with poor prognosis were: polyhydramnios, transverse abdominal diameter below the 5(th) percentile and major hepatic deviation. Diagnosis before 25 weeks was not associated with poor prognosis. Intrathoracic stomach was a good diagnostic sign, but did not allow an assessment of prognosis. CONCLUSION: Diagnosis of congenital diaphragmatic hernia was made before 25 weeks in 77% of the cases. It was not a factor of poor prognosis. As other authors, we found that prenatal association with another malformation (especially cardiac malformation) polyhydramnios, deviation of the liver, and abdominal transverse diameter below the 5(th) percentile were factors of poor prognosis. But it was difficult to determine the prenatal prognosis. Improvement is needed.

[The migrating intrauterine device. Case report and review of the literature]. / Le stérilet migrateur. A propos d'un cas et revue de la littérature.

We report a case of migration of an intrauterine contraceptive device (IUCD) detected during pregnancy one year after insertion. The advanced gestation of the pregnancy and the absence of symptoms lead to expectant management. There were no complications during the pregnancy or delivery. A CT Scan in the post-partum period suggested that the IUCD was located in the region of the small bowel. Laparoscopy confirmed that the IUCD was superficially embedded in the intestinal wall but with no mucosal lesion. The device was removed with the aid of a mini-laparotomy. The migration of an IUCD into the abdominal cavity is rare. A literature review spanning the past 18 years revealed 165 reported cases. The IUCD was located in the following sites: omentum 45, rectosigmoid 44, peritoneum 41, bladder 23, appendix eight, small bowel two, adnexa one, iliac vein one. The majority of the authors recommend removal of copper containing devices, because of the potential for inflammatory reactions that can cause bowel obstruction and perforation. Initial laparoscopy has mostly superceded laparotomy but conversion may be necessary if difficulties arise.

A computerized measurement of dental relapse following orthognathic surgery.

A computer-programmed osteotomy package was used to measure agreed values from lateral cephalograms preoperatively, postoperatively, and during the follow-up period. The variable representing the postoperative cephalometric analysis predicted by the program was included for further comparisons. The results were used to measure the degree of dental and skeletal relapse during the follow-up period, but only the dental relapse is discussed in the present study. There was a statistically significant proclination of the maxillary anterior teeth postoperatively that continued during the mean follow-up period of 16 months.