A Network Pharmacological Approach to Explore the Mechanisms of TongXieYaoFang in Inflammatory Bowel Disease

Abstract Inflammatory bowel disease (IBD) is a chronic inflammatory disorder of the intestine, demonstrating an increasing incidence every year. TongXieYaoFang (TXYF) has been used widely in China as a complementary therapy to relieve the symptoms of IBD for hundreds of years. In the present research, a network pharmacology-based approach was used to systematically explore the intrinsic mechanisms of TXYF in IBD at the molecular level. Network pharmacology-based methods, which mainly included database mining, screening of bioactive compounds, target prediction, collection of IBD-related targets, gene enrichment analysis, network construction, and molecular docking, were employed in the present study. Network analysis revealed a total of 108 potential targets derived from 22 component compounds of TXYF, among which 34 targets were common with the IBD-related targets. In the protein-protein interaction (PPI) network, 10 key targets were identified. The gene enrichment analysis suggested that anti-inflammatory processes, such as NF-kappa B signaling pathway and Toll-like receptor signaling pathway, could be the core processes involved in the action of TXYF in IBD. Molecular docking results revealed that three compounds present in TXYF exhibited strong binding affinity for PTGS2. The present study provides novel insights into the molecular mechanisms and network approaches of TXYF action in IBD from a systemic perspective. The potential targets and pathways identified in the present study would assist in further research on the clinical application of TXYF in IBD therapy.

Enhancement of dissolution rate of racecadotril by liquisolid compact technology

Abstract The current investigation was used to improve the rate of dissolution of an anti-diarrheal drug i.e., racecadotril (RT) at low pH conditions (i.e., in the stomach) by reducing the water secretion and electrolyte in to the intestine by liquisolid tablets. Different formulations (liquisolid) were prepared using Avicel PH 102 as a carrier. Aerosil 200 as a coating material and sodium starch glycolate used as a disintegrant. Polyethylene glycol 200 was used as a non-volatile vehicle to dissolve the drug. FTIR, DSC, XRD and dissolution studies were conducted to characterise liquisolid tablets. Characterisation studies indicated that no interactions between carrier and drug. Solid state characterization had shown a reduction in crystallinity that further supports increment in solubility and dissolution. The optimised formulation showed a significant increase in dissolution i.e., 99.54±0.62% in 30 min compared to directly compressible tablets (38.47±0.26%). The % dissolution efficiency of racecadotril liquisolid tablets 76.86% compared to marketed tablet (27.56%) and conventional direct compression tablet (17.11%). Significant reduction in mean dissolution time of racecadotril from liquisolid tablets (6.84 min) compared to direct compression tablet (44.57 min), indicating faster release of drug and faster onset of action. Formulation of liquisolid tablets could enhance solubility, dissolution and bioavailability of racecadotril

Vólvulo de intestino medio: a propósito de dos casos / Middle intestine volvulus: on the purpose of two cases / Volvulus do intestino médio: com propósito de dois casos

La malrotación intestinal es un espectro de malformaciones, que incluye una gran variedad de alteraciones en el proceso de rotación y fijación del intestino. La ausencia completa de rotación intestinal, uno de los tipos de malrotación intestinal más frecuentes, puede presentarse como hallazgo asintomático o manifestarse clínicamente por un vólvulo del intestino medio. Sin embargo, incluso entre aquellos que se presentan con vólvulo del intestino medio, la clínica puede ser muy diferente, según el grado de isquemia y eventual necrosis intestinal. Se presentan dos casos clínicos de malrotación intestinal con vólvulo del intestino medio con presentaciones, imágenes, tratamientos y evoluciones muy disímiles. Se analizan los mismos a la luz de una revisión bibliográfica relevante al tema tratado, se sacan aprendizajes del manejo realizado y la evolución que presentaron, y se enfatizan los elementos de mayor jerarquía para optimizar el manejo de estos pacientes.

Intestinal malrotation is a spectrum of malformations that includes a great variety of alterations in the rotation and fixation process of the intestines. The total absence of intestinal rotation, one of the most frequents types of intestinal malrotation could present as an asymptomatic find or appear clinically as a midgut volvulus. However, even those that appear as midgut volvulus, could show quite different signs and symptoms according to the degree of ischemic insult and possible intestinal necrosis. Two clinical cases of intestinal malrotation with midgut volvulus with quite different presentations, images, treatments, and evolutions, are informed. The two cases are analyzed under a relevant bibliographic revision, knowledge is derived from the carried-out management and evolution, and elements for the future optimization of management are underlined.

A má rotação intestinal é um espectro de malformações, que inclui uma grande variedade de alterações no processo de rotação e fixação do intestino. A ausência completa de rotação intestinal, um dos tipos mais comuns de má rotação intestinal, pode se apresentar como um achado assintomático ou manifestar-se clinicamente como um volvo de intestino médio. Porém, mesmo entre aqueles que apresentam volvo de intestino médio, os sintomas podem ser muito diferentes, dependendo do grau de isquemia e eventual necrose intestinal. Dois casos clínicos de má rotação intestinal com volvo de intestino médio são apresentados com apresentações, imagens, tratamentos e evoluções muito diferentes. São analisados ​​à luz de uma revisão bibliográfica pertinente ao tema em questão, lições aprendidas com o manejo realizado e a evolução que apresentaram, e os elementos de maior hierarquia são enfatizados para otimizar o manejo desses pacientes.

Obstrucción duodenal congénita de etiología múltiple: reporte de un caso con malrotación intestinal, bandas de Ladd y membrana duodenal intrínseca como causantes de obstrucción intestinal en el neonato / Congenital duodenal obstruction of multiple etiology: case report with intestinal malrotation, Ladd bands and intrinsic duodenal membrane as causes of intestinal obstruction in the neonate

Resumen: La obstrucción duodenal congénita agrupa un amplio espectro de anomalías en el desarrollo del intestino delgado; se clasifica según su etiología tanto en extrínseca como intrínseca. Su presentación es relativamente común, teniendo una incidencia de 1: 2.500-10.000 nacidos vivos y normalmente presenta asociación con aneuploidías (como la trisomía 21) y malformaciones cardíacas. Las imágenes tienen un papel importante en la aproximación diagnóstica y la radiografía de abdomen simple, es el primer escalón en la aproximación de la obstrucción intestinal. El tratamiento involucra una exploración quirúrgica del abdomen con corrección de los defectos encontrados. Este es un reporte de caso de un neonato que presentó obstrucción duodenal congénita de etiología tanto extrínseca (malrotacion intestinal y bandas de Ladd) como obstrucción intrínseca (membranas duodenales).

Abstract: Congenital duodenal obstruction groups together a broad spectrum of abnormalities in the development of the small intestine; it is classified according to its etiology as both extrinsic and intrinsic. Its presentation is relatively common, having an incidence of 1: 2.500-10.000 live births and it is normally associated with aneuploidy (such as trisomy 21) and cardiac malformations. Images play an important role in the diagnostic approach, with simple abdominal radiography being the first step in the approximation of intestinal obstruction. The treatment corresponds to a surgical exploration of the abdomen with resolution of the defects found. This is a case report of a newborn who presented congenital duodenal obstruction of both extrinsic etiology (intestinal malrotation and Ladd bands) and intrinsic obstruction (duodenal membranes).

Presence of anisakid larvae in commercial fishes landed in the Pacific coast of Ecuador and Colombia / Presencia de larvas de anisakidos en peces comercializados y desembarcados en la costa del Pacífico de Ecuador y Colombia

Introduction: Anisakidosis is a zoonotic disease caused by the consumption of raw or undercooked fish or crustaceans parasitized by nematode larvae of the Anisakidae family. In this study, the presence of anisakid larvae was identified in fish species of consumer of the Pacific coast in Ecuador and Colombia. Methods: We obtained 438 samples grouped into twenty species of fish caught in the fishing ports of Manta, Santa Rosa, Buenaventura and Tumaco. The morphological identification of the larvae was made by taxonomy and the percentage of infection, were calculated. For the identification of species, a multiplex PCR was carried. Results: The taxonomic review identified eight species of fish as hosts of the genders Anisakis and Pseudoterranova. The larvae were isolated mainly from the intestine with a percentage of infection between 18 and 100%. The percentage of infection and identification of anisakids in these fish will aid in the prevention and control of anisakiasis as a possible emerging disease for this area of the Pacific. With the multiplex PCR, A. pegreffii, A. physeteris, and P. decipiens were identified. Conclusion: The identification of these species is reported for the first time in this geographical area, providing the basis for future research into the Anisakidae family.

Introducción: La anisakidosis es una enfermedad zoonótica causada por el consumo de pescado o crustáceos crudos o poco cocinados parasitados por las larvas de nematodos de la familia Anisakidae. En este estudio, se identificó la presencia de larvas de anisakidos en especies de peces de consumo de la costa del Pacífico en Ecuador y Colombia. Métodos: Obtuvimos 438 muestras agrupadas en veinte especies de peces capturados en los puertos pesqueros de Manta, Santa Rosa, Buenaventura y Tumaco. La identificación morfológica de las larvas se realizó por taxonomía y se calculó el porcentaje de infección. Para la identificación de las especies, se llevó a cabo una PCR múltiplex. Resultados: La revisión taxonómica identificó ocho especies de peces como huéspedes de los géneros Anisakis y Pseudoterranova. Las larvas se aislaron principalmente del intestino con un porcentaje de infección entre 18 y 100%. El porcentaje de infección e identificación de anisakidos en estos peces ayudará a prevenir y controlar la anisakiasis como una posible enfermedad emergente en esta área del Pacífico. Con la PCR múltiplex, se identifico A. pegreffii, A. physeteris y P. decipiens. Conclusión: La identificación de estas especies se informa por primera vez en esta área geográfica, proporcionando la base para futuras investigaciones sobre la familia Anisakidae.

Duplicación quística intestinal: Caso clínico / Intestinal cystic duplication: Case report

Las duplicaciones intestinales quísticas son anomalías congénitas infrecuentes, con incidencia de 1:4500 autopsias y etiopatogenia incierta. Son estructuras quísticas, tubulares o diverticulares revestidas de mucosa gastrointestinal con pared de músculo liso, adheridas al tracto gastrointestinal, pero no suelen comunicar con la luz intestinal. Los quistes de duplicación gástrica representan el 7-9% de los quistes de duplicación intestinal. Pueden diagnosticarse intraútero mediante ecografía y la resonancia magnética fetal permite caracterizar el quiste y descartar patologías concomitantes. La ecografía posnatal revela una imagen quística de "doble pared". Los neonatos suelen permanecer asintomáticos, pero pueden presentar desde síntomas gastrointestinales inespecíficos hasta síntomas de obstrucción por efecto masa, torsión o infección. En pacientes asintomáticos, se aconseja el seguimiento clínico y estudios de imagen periódicos. El tratamiento es la resección quirúrgica electiva, de manera preferente, con técnicas mínimamente invasivas. Se presenta un caso con sospecha prenatal de quiste de duplicación enteral.

Intestinal cystic duplications are rare congenital anomalies, with an estimated incidence of approximately 1:4500 autopsies. The etiopathogenesis is uncertain. These duplications are cystic, tubular or diverticular structures lined with gastrointestinal mucosa. They share a common smooth muscle wall with the gastrointestinal tract but usually their lumens do not communicate with each other. Gastric duplication cysts represent 7-9% of the gastrointestinal tract duplication. They can be diagnosed prenatally by fetal ultrasound; magnetic resonance imaging characterizes the cyst and excludes other malformations. Postnatal ultrasound shows a characteristic double walled cyst. Newborns are usually asymptomatic, although nonspecific gastrointestinal symptoms, intestinal obstruction due to mass effect, volvulus or infection are described. In asymptomatic patients, clinical follow-up and periodic image controls are recommended. Elective surgical resection is the treatment of choice, using minimally invasive technique whenever possible. A case of prenatally suspected intestinal cystic duplication is presented.

Ausencia congénita de la vena porta hepática asociada a malrotación intestinal y a vena cava inferior izquierda: reporte de caso / Congenital absence of the hepatic portal vein associated to intestinal malrotation and left-sided inferior vena cava: a case report

La ausencia congénita de la vena porta hepática es una malformación en extremo rara, fue descrita por primera vez en 1793 por John Abernethy y a la fecha se han reportado sólo 101 casos. Afecta con mayor frecuencia a mujeres y determina que el drenaje venoso intestinal sea derivado hacia el territorio de las venas sistémicas. Es también conocida como derivación porto-sistémica extra hepática congénita (CEPS), por su sigla en inglés, y se asocia a otras alteraciones congénitas,incluyendo anomalías cardíacas, de las vías biliares, enfermedades metabólicas y retardo mental. En este trabajo presentamos el hallazgo de esta malformación en el cadáver de un niño de dos años de edad, donde la vena porta seguía un trayecto anómalo y se unía a la vena renal derecha. El confluente venoso "mesentérico-esplénico-renal" así formado presentaba un trayecto descendente, recibía a la vena gonadal derecha, y desembocando en la confluencia de las venas ilíacas comunes. Esto se asociaba a mal rotación intestinal, arteria hepática aberrante y a vena cava inferior izquierda, situación descrita sólo una vez en la literatura. El hallazgo de estas anomalías anatómicas en los cadáveres disecados con fines docentes en nuestro Departamento de Anatomía, tiene un valor formativo indiscutible para nuestros alumnos de pre y postgrado, quienes pueden valorar las implicancias de este conocimiento anatómico en la clínica diaria.

Congenital absence of the hepatic portal vein is an extremely rare malformation that was first described by John Abernethy in 1793. Only 101 cases had been described in the literature until 2015 and most affected females. In this anomaly, also known as congenital extrahepatic porto-systemic shunt (CEPS), intestinal venous drainage is derived towards the territory of the systemic veins and could be associated with other birth defects, including heart and biliary tract anomalies, metabolic diseases, mental retardation. We present the case of a 2-year-old boy who died because of multifocal pneumonia and necropsy showed anatomical findings consistent with this malformation as an incidental finding. The portal vein followed an anomalous course and joined the right renal vein, forming the "mesenteric-splenic-renal" venous collector, which then received the right gonadal vein and ended at the confluence of the common iliac veins. In our case this anomaly was associated to intestinal malrotation, aberrant hepatic artery and persistent left inferior vena cava, situation described once in the literature. The finding of these anatomical abnormalities in cadavers has a great teaching value for our undergraduate and graduate students who are learning anatomy and they can also assess the associated clinical.

Left-sided appendicitis: diagnosis and minimally invasive treatment / Left-sided appendicitis: diagnosis and minimally invasive treatment.

Intestinal malrotation and situs inversus can have important repercussions if acute abdominal pain develops. Intraabdominal structures can have inverted position and thus may easily mislead the surgeon during physical examination. Fortunately, radiological exams have improved the preoperative diagnosis of these patients. However, in difficult cases when an underlying surgical disease is suspected, laparoscopy remains the gold standard in order to diagnose and treat if possible the suspected disorder. We present a case of acute left-side appendicitis in a child with unknown congenital intestinal malrotation. In addition, this case stressed the value of laparoscopy in daily practice to evaluate patients with atypical abdominal pain.

Cloacal Exstrophy: a complex disease

Introduction Cloacal exstrophy is a rare occurrence with an incidence of 1:200,000 to 1:400,000 live births. It represents one of the most challenging reconstructive endeavors faced by pediatric surgeons and urologists. Aside from the genitourinary defects, there are other associated anomalies of the gastrointestinal, musculoskeletal and neurological systems that require a multidisciplinary approach when counseling anxious parents. Material and Methods We present a video of a patient with cloacal exstrophy treated with 21 days of life. Surgery consisted of separation and tubularization of the cecal plate from the exstrophied bladder halves and colostomy construction. The bladder was closed primarily and umbilical scar reconstructed and used for ureteral and cistostomy drainage. A urethral catheter was used to guide bladder neck tubularization. A final epispadic penis was obtained and planned for further repair in a second step. Results The patient had an initial uneventful postoperative course and immediate outcome was excellent. The bladder healed nicely but patient presented with abdominal distension in the 5th day of postoperative setting requiring parenteral nutrition. The distal colon persisted with lower diameter although non obstructive, but causing difficulty for fecal progression. Continuous colostomy dilatation and irrigation were required. Conclusions Approximating the bladder halves in the midline at birth and primary bladder closure is a viable option, intestinal transit may be a issue of concern in the early postoperative follow-up. .

Rare clinical presentation of intestinal fixation anomaly with traumatic cecal perforation

Intestinal fixation anomalies are a part of intestinal malrotation. It is a rare occurrence for an unexpected malrotation of the gut to be revealed on laparotomy for other reasons in adults. There are no reports of intestinal fixation anomalies with traumatic perforation in the world literature. We hereby present a case of a 15-year-old girl with traumatic cecal perforation, who on laparotomy showed an intestinal fixation anomaly. We describe the challenges faced in the management of this patient

Cecal volvulus associated with intestinal malrotation presenting as postoperative intestinal obstruction

To report a case of intestinal malrotation predisposing to cecal volvulus following nonabdominal surgery in an adult. A 23-year-old male with known anorectal malformation developed a postoperative intestinal obstruction following percutaneous nephrolithotomy for left renal calculus. Computed tomography of the abdomen revealed a grossly dilated cecum [9 cm] with ileocecal junction on the left side and small bowel loops on the right side of the abdomen. The patient underwent emergency exploratory laparotomy and retroperitonealization cecopexy. Bowel functions were normal at the follow-ups. This case highlights the importance of suspecting cecal volvulus even following nonabdominal surgery in the presence of predisposing factors

Malrotación intestinal con vólvulo, asociada a hiperglucemia grave por estrés / Intestinal malrotation with volvulus associated with severe stress hyperglycemia

Se presenta un niño de 2 años internado con diagnóstico de debut diabético, con glucemia de 500 mg% al ingreso, sin cetosis ni acidosis metabólica. Presenta también vómitos biliosos y amarronados, con deposiciones sanguinolentas. Se opera con diagnóstico presuntivo de obstrucción intestinal aguda, hallándose vólvulo intestinal secundario a malrotación intestinal congénita. Luego de la cirugía normaliza la glucemia. Se asume el cuadro como hiperglucemia sin cetosis, de característica graves, por estrés, secundaria a vólvulo por malrotación intestinal. Esta asociación, aún no ha sido descripta.

A 2-year-old boy was admitted with diagnosis of diabetes debut, with blood glucose of 500 mg% on admission, without ketosis or metabolic acidosis. He also presented bilious vomiting and brownish bloody stools. He was operated with a presumptive diagnosis of acute intestinal obstruction. The fnal diagnosis was volvulus, secondary to congenital malrotation. After surgery, he normalized blood sugar levels. The clinical setting was assumed as hyperglycemia without ketosis, with characteristic of severity caused by stress, secondary to volvulus in malrotation. This association has not yet been described.

Outcomes of surgical treatment of malrotation in children

Background: Urethral mucosal prolapse is rare. This condition may be confused with tumour or sexual abuse in girls. This study aims at reporting the pathology presentation and therapeutic options of urethral prolapse in girls. Materials and Methods: A retrospective study was undertaken from January 2000 to December 2008. Authors analysed the clinical features and the treatment options. Results: There were nine cases of urethral prolapse. The ages ranged from 2.5 to 10 years (mean age: 5.08 years). The main presentation was vaginal bleeding (five cases). Physical examination revealed a soft; non-tender mass that bleeds on touch (six cases); with a length ranging from 0.75 to 1 cm. Urine culture in four patients revealed urinary infection that yielded Escherichia coli in three cases and the Staphylococcus aureus in one case. Six patients had surgical treatment while three had medical treatment. In those who had surgery; one had acute urine retention and one had recurrence that was treated successfully without operation. All the nine girls are cured. Conclusion: Urethral prolapse is a disease of the prepubertal girls of low socio-economic group. Diagnosis is clinical. The treatment of choice is surgical

Pentalogía de Cantrell en un recién nacido expuesto en útero a misoprostol / Pentalogy of Cantrell in a newborn associated with prenatal exposure to misoprostol

El misoprostol es un análogo sintético de la prostaglandina E1 y es usada por sus efectos utero-tónicos como abortivo. En los países donde se utiliza como abortivo sin éxito durante el primer trimestre de gestación, se han informado un espectro de malformaciones congenitas asociadas con la exposición prenatal, que incluyen el síndrome de Moebius, defectos de la pared abdominal, artrogriposis y anomalías por reducción de los miembros, entre otros. La pentalogía de Cantrell (PC), con una prevalencia de 1 en 65.000 nacidos vivos, es un síndrome polimalformativo que consta de 5 anomalías: un defecto de la pared abdominal, defecto esternal inferior, defecto del diafragma anterior, defecto pericárdico diafragmático y anormalidades congenitas del corazón. Se reporta un caso de pentalogía de Cantrell asociado al uso de misoprostol en el primer trimestre de la gestación, siendo este reporte el primer registro de esta asociación.

Misoprostol is a synthetic analogue of prostaglandin E1 and is used for effects such as uterotonic aborti-facient. In countries where abortion is used as unsuccessful in the first trimester of gestation, reported a spectrum of congenital malformations associated with prenatal exposure, which include Moebius syndrome, abdominal wall defects, and anomalies as artrogriposis reduction members, among others. The pentalogy of Cantrell with a prevalence of 1 in 65,000 live births, is a syndrome consisting of 5 abnormalities: a defect in the abdominal wall, lower sternal defect, defect above the diaphragm, diaphragmatic pericardial defect, and congenital abnormalities of the heart. We report a case of Pentalogy of Cantrell associated with the use of misoprostol in the first trimester of pregnancy, this report is the first record of this association.

Interstitial cells of Cajal and intestinal function in Trinidadian children / Células intersticiales de Cajal y función intestinal en niños trinitenses

OBJECTIVE: This study was carried out to compare the density of the interstitial cells of Cajal (ICCs) in the bowel wall of children with Hirschsprung's disease (HD), anorectal malformations (ARM) and normal controls in Trinidad and Tobago. SUBJECTS AND METHOD: Segments of bowel wall excised from eight children with HD, three controls and two children with ARM were immunostained with c-Kit primary antibody. Cells with features of ICCs were counted. RESULTS: All three controls and the two children with ARM had dense distribution of ICCs. Most children (6/8;75%) with HD had markedly reduced counts in aganglionic bowel. Two (25%) also had a decrease in ganglionic bowel. Possible influences were patient age and gender and the level of bowel sectioned. CONCLUSION: Analysis of this sample suggests that immunostaining for c-Kit positive cells might be a useful screening test in the assessment of bowel motility disorders. The possible effects of age, gender and the level of bowel sampled await determination.

OBJETIVO: Este estudio se llevó a cabo con el propósito de comparar la densidad de las células intersticiales de Cajal (CIC) en las paredes intestinales de niños con la enfermedad de Hirschprung (EH), y malformaciones anorectales (MAR), frente a controles normales en Trinidad Tobago. SUJETOS Y MÉTODOS: Segmentos de las paredes intestinales les fueron extirpados a ocho niños con EH; tres controles y dos niños con MAR fueron inmunoteñidos con anticuerpo primario c-kit. Se contaron las células con características de CIC. RESULTADOS: Los tres controles y los dos niños con MAR presentaban una distribución densa de CICs. La mayor parte de los niños (6/8; 75%) con EH tuvieron conteos marcadamente reducidos de intestino agangliónico. Dos niños (25%) también tuvieron una disminución de intestino gangliónico. Entre las influencias posibles se cuentan la edad y el género del paciente así como el nivel de intestino seccionado. CONCLUSIÓN: El análisis de esta muestra sugiere que la inmunotinción para células c-kit positivas podría ser un útil test de pesquisaje a la hora de evaluar desórdenes en la motilidad intestinal. Los efectos posibles de la edad, el género y el nivel de intestino muestreado, están pendientes de determinación.

A Systematic Classification of the Congenital Bronchopulmonary Vascular Malformations: Dysmorphogeneses of the Primitive Foregut System and the Primitive Aortic Arch System

PURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). MATERIALS AND METHODS: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. RESULTS: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. CONCLUSION: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.

Duplicação intestinal e teratoma retroperitoneal na infância: relato de caso / Intestinal duplication and retroperitoneal teratoma in childhoof: a case report

Os autores apresentam um caso de duplicação intestinal e teratoma retroperitoneal em uma paciente de sete anos de idade com massa palpável e dor abdominal a esclarecer, que foi submetida a estudo radiológico convencional, ultra-sonografia, tomografia computadorizada e posterior laparotomia exploradora. O estudo anatomopatológico constatou duplicação intestinal e teratoma maduro. Na literatura médica existente não foi encontrado nenhum caso semelhante.

The authors present a case of intestinal duplication and retroperitonealteratoma in a 7-year-old patient with evident mass and abdominal pain to explain; that it was submitted to study conventional X-ray, ultrasonography, computed tomography and subsequentexploiting laparotomia. The anatomopathological study verified intestinal duplication and ripe teratoma. In the existent medical literature it was not found any similar case.

Bilateral eventration of the diaphragm with malrotation and unilateral renal agenesis.

Bilateral eventration of the diaphragm associated with malrotation of gut and unilateral renal agenesis is a very rare entity. The authors treated a seven mth old male child who presented to them with respiratory distress and gastrointestinal problem. The child was anemic and was in type II respiratory failure. His X-ray chest showed bilateral eventration of diaphragm and ultrasonography abdomen was consistent with left renal agenesis which was confirmed by radionuclide scan. Bilateral plication of the diaphragm done with correction of malrotation which was diagnosed peroperatively. The association of these three anomalies are rare and to be kept in mind whenever treating a patient with bilateral eventration of the diaphragm.