Pentalogy of cantrell. a stillbirth case report

ABSTRACT Introduction: Pentalogy of Cantrell is a rare congenital disorder characterized by midline birth anomalies. Its embryological origins are related to anomalies of the abdominal wall that prevent the closure of the thorax. Its etiology is not yet clear, but it has been associated with a failure of migration of the lateral plate mesoderm to the midline. Case description: A stillbirth at 25.2 weeks of gestation diagnosed with pentalogy of Cantrell. The mother was a 14-year-old teenager with no relevant history of disease. An obstetric ultrasound was performed at 19 weeks of gestation and revealed findings suggestive of pentalogy of Cantrell. The mother was informed of the potential risks and future complications for the fetus, yet she chose to continue with the pregnancy. At 25.2 weeks of gestation, the mother went to the emergency department due to pain in the hypogastrium accompanied by moderate vaginal bleeding and absence of fetal activity. Once fetal death was confirmed by ultrasound, labor was induced, resulting in stillbirth with anencephaly, thoracic hypoplasia, gastroschisis, and eventration of the liver. Conclusions: Adequate antenatal care, including strict ultrasound follow-up, is essential to detect future complications in the fetus; to provide advice on possible malformations incompatible with life, such as pentalogy of Cantrell; and to determine the best therapeutic approach.

RESUMEN Introducción. La pentalogía de Cantrell es una anomalía congénita rara que se caracteriza por malformaciones en la línea media del cuerpo, y cuyos orígenes embriológicos están relacionados con anormalidades de la pared abdominal que impiden el cierre del tórax. Su etiología aun no es clara, pero se ha asociado con una falla de la migración de los pliegues del mesodermo lateral a la línea media. Presentación del caso. Mortinato de 25.2 semanas de gestación diagnosticado con pentalogía de Cantrell. La madre era una menor de 14 años sin antecedentes patológicos. A las 19 semanas de gestación se realizó ecografía obstétrica que mostró imágenes sugestivas de pentalogía de Cantrell, y se le explicó a la gestante los riesgos y complicaciones futuras que presentaría el feto, pero esta decidió continuar con el embarazo. A las 25.2 semanas de gestación, la joven acudió al servicio de urgencias por dolor en hipogastrio acompañado de sangrado vaginal moderado y ausencia de actividad fetal. Una vez confirmada la muerte fetal mediante ecografía, se indujo el trabajo de parto, obteniéndose mortinato con anencefalia, hipoplasia de caja torácica, gastrosquisis y eventración del hígado. Conclusiones. La realización de adecuados controles prenatales, en los cuales se realice un seguimiento ecográfico estricto, es fundamental para detectar futuras complicaciones en el feto; brindar asesoría sobre posibles malformaciones que sean incompatibles con la vida, como la pentalogía de Cantrell, y establecer las mejores alternativas de manejo.

Mesotelioma en paciente colombiano con sobrevida mayor a tres años: presentación de caso / Mesothelioma in a Colombian Patient over three years: Case Presentation / Mesotelioma em paciente colombiano com sobrevivência superior a três anos: apresentação de caso

Resumen Introducción: el mesotelioma epitelioide es un tumor que se desarrolla en las capas embrionarias mesoteliales; es de etiología desconocida, pero se relaciona con la exposición al asbesto, con una presentación clínica inespecífica y con un pronóstico de sobrevida corto después del diagnóstico. Presentación del caso: hombre de profesión mecánico automotor, con tos sin expectoración, disnea, hipertermia y emaciación posterior a la extracción quirúrgica de lipomas que afectaban el tórax, quien posteriormente fue diagnosticado con mesotelioma epitelioide maligno con ubicación en la pleura del hemitórax derecho y fue tratado con toracotomía, quimioterapia con los medicamentos pemetrexed y cisplatino y sesiones de radioterapia, que mostraron un aumento en la sobrevida 3 tres años. Conclusión: este caso permitió identificar que el uso de la pleurodesis química con quimioterapia como tratamiento podría ser responsable del aumento de la esperanza de vida y la calidad de esta en los pacientes que padecen este tipo de tumor.

Abstract Introduction: Epithelioid mesothelioma is a tumor that develops in the mesothelial embryonic layers; it is of an unknown etiology, but it is related to asbestos exposure with a nonspecific clinical presentation and a short survival prognosis after diagnosis. Case presentation: An automotive mechanic patient presents with cough without expectoration, dyspnea, hyperthermia, and emaciation following surgical removal of lipomas. This affected the chest and the patient was subsequently diagnosed with malignant epithelioid mesothelioma located in the pleura of the right hemithorax. The patient was treated with thoracotomy, chemotherapy with the drugs pemetrexed and cisplatin, and radiation therapy sessions which resulted in an increased survival rate at 4 years. Conclusion: This case report identifies the use of chemical pleurodesis in combination with chemotherapy as an effective treatment for increasing the life expectancy and quality of life in patients suffering from this type of tumor.

Resumo Introdução: o mesotelioma epitelióide é um tumor que se desenvolve nas camadas embrionárias mesote-liais; é de causa desconhecida, mas está relacionado com a exposição ao amianto e possui uma manifestação clínica inespecífica e com prognóstico de sobrevivência curto após o diagnóstico. Apresentação do caso: o paciente é um mecânico automotivo, que apresentou tosse seca, dispneia, hipertermia e emagrecimento posterior a extração cirúrgica de lipomas que afetavam o tórax sendo posteriormente diagnosticado com mesotelioma epitelióide maligno localizado na pleura do hemitórax direito e foi tratado com toracotomia, quimioterapia com os medicamentos pemetrexed e cisplatino além de sessões de radioterapia, mostrando um aumento de expectativa de vida para 4 anos. Conclusão: este estudo de caso permite identificar que o uso da pleurodese química com quimioterapia como tratamento poderia ser a responsável pelo aumento da expectativa e qualidade de vida em pacientes acometidos por este tipo de tumor.

Uso del lexema δερµα (derma) en terminologia embryologica / Use of the lexeme δερµα (derm) in terminologia embryologica

Al lexema δερµα (derma) que proviene de las raíces griegas δέρ-µα/µατος se lo define como piel, pellejo, cuero, odre (Cortez, 2011). Lo encontramos en los términos ectodermo, mesodermo y endodermo, utilizados para describir las estructuras durante la tercera semana del desarrollo embriológico humano. Se consultó el significado y sus raíces en el diccionario Manuel Griego clásico-Español Vox (Pabón, 1967) y Diccionario Médico-Biológico, Histórico y Etimológico (DICCIOMED) de la Universidad de Salamanca (Cortez); de igual manera se investigó la utilización de los términos ectodermo, mesodermo y endodermo en la Terminologia Embryologica (FIPAT, 2013) y en su última versión (FIPAT, 2017). La búsqueda reportó que estos términos están compuestos por dos raíces griegas el sufijo δέρµα (derma) presente en los tres términos; más los prefijos ἐκτός que significa externo; µÎ­σος definido como medio y ἐνδο cuyo significado es dentro. Estos tres tejidos se derivan a la vez del epiblasto que viene de dos raíces griegas ἐπί- ep(í) que significa sobre + ßλαστός - blast(o) que se traduce como germen, retoño, forma celular inmadura; y del hipoblasto que cuyo término se forma de las raíces griegas ὑπό (hypó) que significa 'debajo de' + ßλαστός - blast(o). Podemos señalar que el mejor término para denominar a estas tres estructuras debiera ser ßλαστός (blasto); y por lo tanto, se deberían denominar a estas tres estructuras como ectoblasto, mesoblasto y endoblasto; debido a que son células o tejidos inmaduros, transitorios y no tejidos definitivos como es la piel; lo cual a su vez se corresponde con los objetivos determinados por la FIPAT.

The lexeme δέρ-µα (derma) that comes from the Greek δέρ-µα/µατος is defined as skin, hide, leather, wineskin (Cortez, 2011). We find it in the term ectoderm, mesoderm and endoderm, used to describe the structures during the third week of human embryological development. The meaning and its roots were consulted in the Manuel Greek-Spanish Vox dictionary (Pabón, 1967) and Medical-Biological, Historical and Etymological Dictionary (DICCIOMED) of the University of Salamanca (Cortez); the same way, the use of the terms ectoderm, mesoderm and endoderm was investigated in Terminologia Embryologica (FIPAT, 2013) and in its latest version Terminologia Embryologica (FIPAT, 2017). The search reported that these terms are composed of two Greek roots, the suffix δέρµα (derma) present in the three terms; plus the prefixes ἐκτός which means external; µÎ­σος defined as medium and ἐνδο whose meaning is within. These three tissues are derived in turn from the epiblast that comes from two Greek roots ἐπί- ep (í) which means over + ßλαστός - blast (o) which translates as germ, shoot, immature cell form; and from the hypoblast whose term is formed from the Greek roots ὑπό (hypó) meaning 'under' + ßλαστός - blast (o). We can say that the best term to name these three structures should be ßλαστός (blast); and therefore, these three structures should be named as ectoblast, mesoblast and endoblast; because they are immature, transitory cells or tissues and definitive non-tissues such as the skin; which in turn corresponds to the objectives determined by FIPAT.

Derivation of endothelial cells from porcine induced pluripotent stem cells by optimized single layer culture system

Embryological Consideration of Dural AVFs in Relation to the Neural Crest and the Mesoderm / 신경중재치료의학

Intracranial and spinal dural arteriovenous fistulas (DAVFs) are vascular pathologies of the dural membrane with arteriovenous shunts. They are abnormal communications between arteries and veins or dural venous sinuses that sit between the two sheets of the dura mater. The dura propria faces the surface of brain, and the osteal dura faces the bone. The location of the shunt points is not distributed homogeneously on the surface of the dural membrane, but there are certain areas susceptible to DAVFs. The dura mater of the olfactory groove, falx cerebri, inferior sagittal sinus, tentorium cerebelli, and falx cerebelli, and the dura mater at the level of the spinal cord are composed only of dura propria, and these areas are derived from neural crest cells. The dura mater of the cavernous sinus, transverse sinus, sigmoid sinus, and anterior condylar confluence surrounding the hypoglossal canal are composed of both dura propria and osteal dura; this group is derived from mesoderm. Although the cause of this heterogeneity has not yet been determined, there are some specific characteristics and tendencies in terms of the embryological features. The possible reasons for the segmental susceptibility to DAVFs are summarized based on the embryology of the dura mater.

Induced Intermediate Mesoderm Combined with Decellularized Kidney Scaffolds for Functional Engineering Kidney

BACKGROUND: Chronic kidney disease is a severe threat to human health with no ideal treatment strategy. Mature mammalian kidneys have a fixed number of nephrons, and regeneration is difficult once they are damaged. For this reason, developing an efficient approach to achieve kidney regeneration is necessary. The technology of the combination of decellularized kidney scaffolds with stem cells has emerged as a new strategy; however, in previous studies, the differentiation of stem cells in decellularized scaffolds was insufficient for functional kidney regeneration, and many problems remain. METHODS: We used 0.5% sodium dodecyl sulfate (SDS) to produce rat kidney decellularized scaffolds, and induce adipose-derived stem cells (ADSCs) into intermediate mesoderm by adding Wnt agonist CHIR99021 and FGF9 in vitro. The characteristics of decellularized scaffolds and intermediate mesoderm induced from adipose–derived stem cells were identified. The scaffolds were recellularized with ADSCs and intermediate mesoderm cells through the renal artery and ureter. After cocultured for 10 days, cells adhesion and differentiation was evaluated. RESULTS: Intermediate mesoderm cells were successfully induced from ADSCs and identified by immunofluorescence and Western blotting assays (OSR1 + , PAX2 +). Immunofluorescence showed that intermediate mesoderm cells differentiated into tubular-like (E-CAD + , GATA3 +) and podocyte-like (WT1 +) cells with higher differentiation efficiency than ADSCs in the decellularized scaffolds. Comparatively, this phenomenon was not observed in induced intermediate mesoderm cells cultured in vitro. CONCLUSION: In this study, we demonstrated that intermediate mesoderm cells could be induced from ADSCs and that they could differentiate well after cocultured with decellularized scaffolds.

Placental mesenchymal dysplasia: a case report and literature review / 中华围产医学杂志

Objective@#To summarize the clinical, sonographic and pathological characteristics, and the perinatal outcomes of pregnancies complicated by placental mesenchymal dysplasia (PMD).@*Methods@#We reported the diagnosis and treatment of a case of PMD in Tianjin Union Medical Center. Pertinent Chinese literatures on PMD were reviewed to analyze the clinical features and the outcomes for mothers and infants.@*Results@#(1) Case report: The patient was admitted to our hospital with elevated blood pressure in July 2011 and underwent caesarean section at 30+1 gestational weeks indicated by severe preeclampsia and fetal distress. PMD was diagnosed by placental pathological examination. The patient was discharged from the hospital after oral antihypertensives treatment with stabilized blood pressure. Both the mother and the child were healthy during follow-ups until August 2018. (2) Literature review: A total of 14 cases of PMD were reported domestically including the index case. The average maternal age was 27.9(23-42). Of all the 14 fetuses, half were born alive and half were died in the uterus; two were male, 10 were female and two of unknown sex. Sonographic findings of 10 cases showed thickened placenta with hypoechoic areas. Human chorionic gonadotropin (hCG) was tested in five cases, and the results were all normal. One case was tested for maternal serum alpha fetoprotein (AFP), which was increased. Among the 14 cases, there were two of preeclampsia, six of intrauterine death, three of fetal distress and one of the premature rupture of fetal membranes. Four out of the seven live births were born prematurely. According to the medical record, the average weight of placentas of seven gravidas was 665 g, and eight placentas were larger than the gestational age in size. Pathological examinations showed 11 placentas were covered with grape-like cystic vesicles. No trophoblastic proliferation or stromal trophoblastic inclusion was observed in 12 cases under the microscope.@*Conclusions@#PMD is mainly characterized by enlarged and cystic placenta with hypoechoic areas in sonographic findings as well as elevated AFP and normal hCG concentrations in serum. It is more likely to occur in female fetuses with normal karyotype. Placentas with significantly increased size and weight and grape-like cystic vesicles are typical features of PMD that can be detected by pathological examinations. Some gravidas may develop hypertensive disorders of pregnancy and deliver prematurely due to fetal distress, but the maternal and neonatal outcomes are usually good. Close monitoring of the gravidas and fetuses with PMD may help to improve pregnancy outcomes.

Placental mesenchymal dysplasia: a case report and literature review / 中华围产医学杂志

Objective To summarize the clinical, sonographic and pathological characteristics, and the perinatal outcomes of pregnancies complicated by placental mesenchymal dysplasia (PMD). Methods We reported the diagnosis and treatment of a case of PMD in Tianjin Union Medical Center. Pertinent Chinese literatures on PMD were reviewed to analyze the clinical features and the outcomes for mothers and infants. Results (1) Case report: The patient was admitted to our hospital with elevated blood pressure in July 2011 and underwent caesarean section at 30+1 gestational weeks indicated by severe preeclampsia and fetal distress. PMD was diagnosed by placental pathological examination. The patient was discharged from the hospital after oral antihypertensives treatment with stabilized blood pressure. Both the mother and the child were healthy during follow-ups until August 2018. (2) Literature review: A total of 14 cases of PMD were reported domestically including the index case. The average maternal age was 27.9(23-42). Of all the 14 fetuses, half were born alive and half were died in the uterus; two were male, 10 were female and two of unknown sex. Sonographic findings of 10 cases showed thickened placenta with hypoechoic areas. Human chorionic gonadotropin (hCG) was tested in five cases, and the results were all normal. One case was tested for maternal serum alpha fetoprotein (AFP), which was increased. Among the 14 cases, there were two of preeclampsia, six of intrauterine death, three of fetal distress and one of the premature rupture of fetal membranes. Four out of the seven live births were born prematurely. According to the medical record, the average weight of placentas of seven gravidas was 665 g, and eight placentas were larger than the gestational age in size. Pathological examinations showed 11 placentas were covered with grape-like cystic vesicles. No trophoblastic proliferation or stromal trophoblastic inclusion was observed in 12 cases under the microscope. Conclusions PMD is mainly characterized by enlarged and cystic placenta with hypoechoic areas in sonographic findings as well as elevated AFP and normal hCG concentrations in serum. It is more likely to occur in female fetuses with normal karyotype. Placentas with significantly increased size and weight and grape-like cystic vesicles are typical features of PMD that can be detected by pathological examinations. Some gravidas may develop hypertensive disorders of pregnancy and deliver prematurely due to fetal distress, but the maternal and neonatal outcomes are usually good. Close monitoring of the gravidas and fetuses with PMD may help to improve pregnancy outcomes.

The use of human mature cystic ovarian teratoma as a model to study the development of human lymphatics / 대한해부학회지

The angiogenic theory to the development of human lymphatics is not clear. The objective of this study was to investigate the development of human lymphatics. Semi-thin and thin paraffin sections from human mature cystic ovarian teratoma tissues were studied using light and electron microscopy. Lymphatics were formed by the differentiation of mesenchymal cells that gradually acquired morphological features of endothelial cells. It is suggested that in human mature cystic ovarian teratoma the lymphatic endothelium develops from mesenchymal cells, and not from cells derived from mature endothelium of a preexisting vein or lymphatic.

Embolização de artérias renais para o tratamento da hipertensão arterial não controlada / Embolización de arterias renales para hipertensión arterial no controlada

Resumo A causa mais comum e muitas vezes ignorado de hipertensão secundária e resistente é a doença renal parenquimatosa, cuja prevalência está crescendo. Quando se tenha esgotado a terapia anti-hipertensiva oral, podem ser usadas outras medidas não farmacológicas como a embolização de artérias renais, que visa diminuir o estímulo simpático, o estado hiperreninêmico e ativação do sistema renina angiotensina aldosterona que estão aumentados na doença renal crônica. Reportamos o caso de uma mulher com doença renal crônica e em terapia de substituição renal, com mínimo de diurese residual e hipertensão arterial resistente, submetida à embolização de artérias renais com a finalidade de conseguir um melhor controle dos valores da pressão arterial e diminuir o nómero de medicamentos anti-hipertensivos. Com este procedimento, consegue-se os objetivos propostos, além de uma melhor adesão ao manejo médico e menor probabilidade de internações hospitalares por crise hipertensiva.Pouco existe na literatura sobre a embolização de artérias renais em pacientes em terapia dialítica; no entanto, é possível que uma função renal residual mínima possa levar a estados de hiperatividade simpática e altos níveis de renina circulantes, que pode gerar hipertensão arterial resistente; é aqui onde a embolização de artérias renais teria sua maior utilidade.

Resumen La causa médica más común y con frecuencia ignorada de hipertensión arterial secundaria y resistente es la enfermedad renal parenquimatosa, cuya prevalencia viene en aumento. Cuando se ha agotado la terapia antihipertensiva oral se pueden utilizar otras medidas no farmacológicas que buscan frenar la contribución del riñón a la hipertensión, esto es mediante la disminución del estímulo simpático, el estado hiperreninémico y la activación del eje renina angiotensina aldosterona que se encuentran aumentados en la enfermedad renal crónica, mediante herramientas poco usadas pero útiles para este fin como lo es la embolización de las arterias renales. Realizamos el reporte de un caso de embolización de arterias renales en una mujer con enfermedad renal crónica y en terapia de reemplazo renal, con mínima diuresis residual e hipertensión arterial resistente, en quien se buscaba como objetivo primario un mejor control en las cifras de presión arterial y como objetivo secundario la disminución del número de medicamentos antihipertensivos a utilizar. Se logró no solo la mejoría en las cifras tensionales y la disminución de los medicamentos antihipertensivos, sino también una mejor posibilidad de adherencia al manejo médico a futuro, con menor nómero de recaídas por crisis hipertensiva. Poco existe en la literatura actual acerca de la embolización de arterias renales en pacientes en terapia dialítica; sin embargo, es factible que una mínima función renal residual pueda llevar a estados de hiperactividad simpática y altos niveles de renina circulante, que puedan generar escenarios de hipertensión arterial resistente; es aquí en donde la embolización de arterias renales tendría su mayor utilidad.

Hiperplasia virginal mamaria asociado a hiperplasia pseudoangiomatosa del estroma mamario difuso / Virginal breast hypertrophy associated to difuse pseudoangiomatous stromal hyperplasia of the breast

Se presenta el caso de una mujer de 20 años de edad con crecimiento mamario severo bilateral tipo gigantomastia, de tres meses de evolución, quien fue valorada inicialmente en hospital de primer nivel de complejidad, donde se le hizo diagnóstico de mastitis aguda, la cual no tuvo respuesta a los antibióticos y cuya biopsia de mama fue sospechosa para angiosarcoma mamario, por lo cual remiten a un tercer nivel. Allí fue valorada por el grupo de mastología que reorienta el diagnóstico luego de exámenes complementarios, documentándose así una poco frecuente asociación entre hipertrofia virginal mamaria e hiperplasia pseudoangiomatosa del estroma mamario difusa. Se revisa la presentación clínica de ambas entidades y su diagnóstico diferencial.

Presents a woman of 20 years old with severe bilateral mammary growth rate type gigantomastia with three months of duration. Initially valued and diagnosed at first level as an acute mastitis, which didn’t respond to antibiotics. A mammary biopsy was suspicious for breast angiosarcoma, and refered, valued by mastology that reorients the diagnosis after further examination and biopsy, and documenting a rare association between virginal breast hypertrophy and difuse pseudoangiomatous hyperplasia of the mammary stroma, which together with a good diagnosis could receive appropriate treatment. The clinical presentation of the diseases and their differential diagnosis is reviewed.

Distribution of CD10-positive epithelial and mesenchymal cells in human mid-term fetuses: a comparison with CD34 expression / 대한해부학회지

CD10, a marker of immature B lymphocytes, is expressed in the developing epithelium of mammary glands, hair follicles, and renal tubules of human fetuses. To assess mesenchymal and stromal expression of CD10, we performed immunohistochemical assays in whole body sections from eight fetuses of gestational ages 15-20 weeks. In addition to expression in urinary tract and intestinal epithelium, CD10 was strongly expressed at both gestational ages in fibrous tissues surrounding the airways from the larynx to lung alveoli, in the periosteum and ossification center, and in the glans of external genitalia. CD10 was not expressed, however, in other cavernous tissues. These findings suggest that mesenchymal, in addition to epithelial cells at specific sites, are likely to express CD10. The glomeruli, alveoli, and glans are all end products of budding or outgrowth processes in the epithelium or skin. However, in contrast to the CD34 marker of stromal stem cells, CD10 was not expressed in vascular progenitor cells and in differentiated vascular endothelium. The alternating pattern of CD10 and CD34 expression suggests that these factors play different roles in cellular differentiation and proliferation of the kidneys, airway and external genitalia.

Mest Attenuates CCl4-Induced Liver Fibrosis in Rats by Inhibiting the Wnt/beta-Catenin Signaling Pathway

BACKGROUND/AIMS: The Wnt/beta-catenin signaling pathway has been reported to play an important role in liver fibrosis. This study was designed to investigate whether mesoderm-specific transcript homologue (Mest), a strong negative regulator of Wnt/beta-catenin signaling, could inhibit liver fibrosis. METHODS: pcDNA-Mest was transfected into hepatic stellate cells (HSCs) and rats. Rats were randomly divided into four groups: normal group (normal saline), treatment group (pcDNA-Mest+CCl4), control group (pcDNA-neo+CCl4), and model group (normal saline+CCl4). Changes in liver pathology were evaluated by hematoxylin and eosin and Masson's trichrome staining. The levels of alanine transaminase, aspartate transaminase, lactic dehygrogenase, hyaluronic acid, and laminin in the serum and hydroxyproline in the liver were detected by biochemical examination and radioimmunoassay, respectively. The expression and distribution of beta-catenin, alpha-smooth muscle actin (alpha-SMA), Smad3, and tissue inhibitor of metalloproteinase type I were determined, and the viability of the HSCs was tested. RESULTS: Our data demonstrate that Mest alleviated CCl4-induced collagen deposition in liver tissue and improved the condition of the liver in rats. Mest also significantly reduced the expression and distribution of beta-catenin, alpha-SMA and Smad3 both in vivo and in vitro, in addition to the viability of HSCs in vitro. CONCLUSIONS: We found that Mest attenuates liver fibrosis by repressing beta-catenin expression, which provides a new therapeutic approach for treating liver fibrosis.

Desarrollo del Aparato Digestivo / Digestive System Development

El aparato digestivo deriva del endodermo y el mesodermo, que forman su epitelio y la musculatura lisa respectivamente. Al igual que en el resto de los sistemas, existe un interacción epitelio-mesenquimática mediada por moléculas como Hedgehog, BMP y FoxF1 que determinan el crecimiento intestinal en sus ejes principales. Los genes Hox, junto con el resto de las moléculas, participan en la regionalización del sistema digestivo. En sus inicios lo denominaremos intestino primitivo, formado por un tubo endodérmico que deriva del saco vitelino; dividiéndose en intestino anterior, medio y posterior. En esta revisión veremos cómo estos 3 segmentos darán origen a las diferentes estructuras del sistema digestivo en los vertebrados.

The digestive system is derived from the endoderm and mesoderm, which form its epithelium and smooth muscle, respectively. As in the other systems, there is an epithelial-mesenchymal interactions mediated by molecules such as Hedgehog, BMP and FoxF1, determining intestinal growth in the main axes. The Hox genes, together the rest of the molecules, involved in the regionalization of the digestive system. In the beginning we call it primitive gut, consisting of a tube derived of endodermal yolk sac, divided into foregut, midgut and hindgut. In this review we will see how these 3 segments give rise to different structures of the digestive system in vertebrates.

Anomalias ósseas congênitas em sotalia guianensis (mammalia, cetacea, delphinidae) da costa centro-norte do estado do Rio de Janeiro / Congenital bone defects in guiana dolphin, sotalia guianensis (mammalia, cetacea, delphinidae) in the northcentral coast of Rio de Janeiro state, Brazil

Carcaças de botos-cinza Sotalia guianensis (van Bénéden, 1864) foram recuperadas entre 20 de agosto de 2001 e 13 de fevereiro de 2006, na costa centro-norte fluminense. A maior parte delas (85 por cento) entre Barra de São João (22º35'S 41º59'W) e Quissamã (22º06'S 41º28'W). Foram estudadas as colunas vertebrais dos 20 exemplares que apresentavam mais de 60 por cento das vértebras presentes. A coleção é formada por 75 por cento de animais imaturos, e todos os esqueletos mostram alterações tafonômicas mínimas. Duas categorias de anomalias congênitas foram diagnosticadas, ambas relacionadas ao desenvolvimento do mesoderma paraxial. A sétima vértebra cervical (C7) foi a única afetada, as costelas cervicais e o não fechamento do arco neural atingiram 15 (75 por cento) dos indivíduos analisados, dos quais três (15 por cento) apresentam ambas as anomalias. Nove (45 por cento) indivíduos apresentaram costelas cervicais uni ou bilateral, e nove (45 por cento) indivíduos apresentaram não fechamento do arco neural; em todos os casos as vértebras contíguas eram normais. A ocorrência de anomalias nesta série do Rio de Janeiro é maior do que as referidas na literatura brasileira para outras séries de Sotalia do Amazonas, Ceará e Santa Catarina. A série de Sotalia descrita vem de uma região do litoral muito limitada e provavelmente representa uma população local. As costelas cervicais são geneticamente determinadas e podem estar concentradas por uma condição de grande proximidade biológica entre os animais; o não fechamento do arco pode ter também um componente ambiental, a ser investigado futuramente.

Carcasses of Guiana dolphins, Sotalia guianensis (van Bénéden, 1864) were recovered from August 20, 2001 to February 13, 2006 along the north-central coast of Rio de Janeiro state. Most of the animals (85 percent) were found between Barra de São João (22º35'S 41º59'W) and Quissamã (22º06'S 41º28'W). We studied the vertebral columns of all the 20 specimens that had more than 60 percent of the vertebrae preserved. The series has 75 percent of immature animals and all skeletons displayed a minimum of taphonomic changes. Two categories of congenital anomalies were diagnosed, both related to the development of the paraxial mesoderm. The seventh cervical vertebra (C7) was the only affected; the cervical ribs and the cleft neural arches were present in 15 (75 percent) of the individuals, three (15 percent) of which had both anomalies. Nine (45 percent) individuals had unilateral or bilateral cervical ribs, and nine (45 percent) individuals had cleft neural arches; the contiguous vertebrae were normal in every case. The frequency of these anomalies was higher in that Rio de Janeiro series than in other Sotalia series previously reported in the Brazilian literature for Amazonas, Ceará and Santa Catarina States. The present Sotalia series here described comes from a very limited coastal region probably representing a local population. The cervical ribs are genetically defined and may be concentrated because of a condition of close biological proximity among the animals; the cleft arch could also be determined by environmental factors, to be investigated in the future.

The origin of endoderm: Transmission electron microscopic point of view.

Objective: To show the transmission electron microscopic (TEM) evidence to confirm that the endoderm originates from the epiblast of the primitive streak or from other sources. Methods: 60 fertilized Leghorn hen’s eggs were used in this study by incubating the eggs for about 18-27 hours at 38oC, then the chick embryos of the primitive streak stage to 7-somite stage were further processed for routine TEM study at the region of the primitive streak. Results: The epiblast proliferates and accumulates to form the primitive streak at the midcaudal of the embryonic disc from 18-27 hours incubation which corresponds with the early third week of the human embryo. TEM evidence shows that the epiblast at the primitive streak is the stratified columnar type of epithelium while the hypoblast is the simple squamous and the mesoderm cells are irregular in shape. The process of gastrulation begins with the formation of the filopodia of the epiblast by numerous protrusions of the plasma membrane from lateral side of the cell. These structures initiate the separation of the contacted cells. The deepest epiblast cells separate first while the superficial epiblast cells exhibit the desmosome between the adjacent cells. The separated epiblast cells are bottle-shaped with numerous filopodia and gradually change the shape into round or oval cells which migrate in the space between the epiblast and hypoblast. Some of these migrate to the hypoblast and contact with the hypoblast, the mesoblasts lose the filopodia and gain more close contact to the hypoblasts which become a very thin sheet of cells. The facing cell membrane later gradually disappears and the mesoblast then occupies the region of pre-existing hypoblast. There is no evidence that the mesoblast displaces the pre-existing hypoblast laterally to form the extraembryonic endoderm. Conclusion: These are TEM evidences that the epiblast of the primitive streak separates and migrates to form the mesoblast and some contact with the hypoblast. The later process appeared to reveal that the mesoblast compresses the hypoblast until the facing plasma membrane disappears and occupies the region of the pre-existing hypoblast.

Tumor de células gigantes costal ocupando todo o hemitórax / Giant cell tumor of the rib occupying the entire hemithorax

Os autores relatam o caso de uma paciente de 28 anos de idade portadora de tumor de células gigantes originário da costela. O tumor de grandes dimensões (25 × 17 cm) ocupava todo o hemitórax e causava atelectasia do pulmão esquerdo. Tratava-se de uma neoplasia mesenquimal benigna, a qual raramente acomete as costelas. Foi realizada toracotomia com ressecção em bloco da parede torácica e do tumor. O objetivo deste artigo é enfatizar que, apesar da grande dimensão do tumor, ele pôde ser completamente ressecado, e o pulmão foi reabilitado.

The authors report the case of a 28-year-old female patient with a giant cell tumor originating from the rib. The tumor, measuring 25 × 17 cm, occupied the entire hemithorax and caused atelectasis of the left lung. This tumor was a benign mesenchymal neoplasm, which rarely affects the ribs. A thoracotomy involving en bloc resection of the chest wall and tumor was performed. Despite the large dimensions of the tumor, complete resection was possible, and lung function was restored.

Extraskeletal Chondroma of the Fallopian Tube

Extraskeletal chondroma can occur in the hands, feet, head and neck. This tumor usually presents as a small solitary nodule. The histogenesis of the tumor is controversial, but some have suggested a metaplastic origin. Chondroma of the fallopian tube is very rare. There is only one report in English literature. The origin of this tumor can be subcoelomic mesenchyme of the tubal serosa or mesenchyme of the myosalpinx. We describe a case of chondroma arising from the serosal surface of the fallopian tube with a review of literature. A 30-yr-old woman visited hospital due to left adnexal mass. On operating finding, 2 x 3 cm sized nodular mass was noted on the left tubal serosal area. The excised mass showed multilobulated appearance covered with thin fibrous membrane. The cut surface was solid, grayish yellow, and myxoid with a focal gelatinous area. The microscopic finding showed islands and elongated lobules of mature benign cartilage without cytologic atypia.

Two Cases of Holoprosencephaly / 대한산부인과학회잡지

Holoprosencephaly is a rare malformation complex or development defect including different degrees of incomplete cleavages of the embryonic prosencephalon and varying degrees of the midface defects, resulting from the defect of prechordal mesoderm, migrating forward into the area anterior to the notochord during the third week of fetal development. Early antenatal diagnosis of holoprosencephaly is important to find out its severity, to predict its prognosis, and to determine proper management according to its prognosis and severity. The possibility of early antenatal diagnosis of holoprosencephaly by ultrasound has been suggested, but occasionally missed and rarely confirmed. We present one case of lobar holoprosencephaly, diagnosed postnatally and one case of alobar holoprosencephaly, diagnosed antenatally in our hospitals.

Mesoderm induction in amphibians and chick.

Induction is a process in which the developmental pathway of one cell is controlled by signals emitted from another. Mesoderm induction is the first inductive interaction in the Xenopus enbryo and probably occurs in all vertebrates. It is a very important event as it is implicated in the regulation of morphogenesis. Nieuwkoop first demonstrated the importance of vegetal endoderm in inducing the mesoderm. Slack and co-workers incorporated the information obtained from experimental embryology in a "three signal" model for mesoderm induction in amphibians (signals arising from ventral vegetal hemisphere, dorsal vegetal hemisphere and the organizer). More recent research has resulted in the detection of mesoderm inducing factors which are members of FGF and TGF--β families. Activin, a member of the TGF-ß family, has been shown to induce differential gene expression and cell differentiation in a concentrationdependent manner giving credence to the theory of morphogen gradients. Study of mesoderm induction in the chick embryo is much more difficult due to several reasons. Novel experimental approaches, however, have been used which point to the role of activin and FGF in chick mesoderm induction. The demonstration of mesoderm inducing activity of activin and FGF in other groups of vertebrates, particularly the chick embryo brings out the possibility of a universal mechanism of mesoderm induction being operative in all the vertebrates.