Descripción angiográfica de los aspectos anatómicos y clínicos de la arteria interventricular anterior en un grupo de personas colombianas

Introducción. La arteria interventricular anterior se origina en la coronaria izquierda, irriga la cara anterior de los ventrículos, el ápex y el tabique interventricular; es la segunda arteria más relevante del corazón. Objetivo. Describir las características anatómicas y clínicas de la arteria interventricular anterior mediante angiografía. Materiales y métodos. Se realizó un estudio descriptivo con 200 reportes angiográficos de personas colombianas; se valoraron el origen, el trayecto y la permeabilidad de la arteria interventricular anterior, así como la dominancia coronaria. Se incluyeron datos relacionados con dolor precordial, infarto agudo de miocardio, dislipidemia y alteración electrocardiográfica. No fue posible hacer pruebas estadísticas, debido a la escasa prevalencia de variaciones anatómicas de dicha arteria. Resultados. Se encontró una arteria interventricular anterior con su origen en el seno aórtico izquierdo, sin puente miocárdico, sin alteración de la permeabilidad y con dominancia izquierda. La frecuencia de los puentes fue del 2 % y la dominancia más frecuente fue la derecha en el 86 %. Se presentaron alteraciones de permeabilidad en el 43 % de los casos, las cuales afectaron principalmente al S13. El 25 % de los pacientes presentó dolor precordial; el 40 %, alteraciones ecocardiográficas; el 5 %, cardiopatía isquémica, y el 59 %, alguna alteración electrocardiográfica. Conclusiones. Las variaciones en el origen de la arteria interventricular anterior son poco prevalentes, según reportes de Chile, Colombia y España. Los puentes miocárdicos de esta arteria fueron escasos respecto a otros estudios, lo cual sugiere mejor especificidad de los hallazgos de la angiotomografía o de la disección directa. La permeabilidad coronaria se valora con la escala TIMI (Thrombolysis in Myocardial Infarction); puntajes de 0 y 1 indican una lesión oclusiva asociada con cardiopatía isquémica. La dominancia coronaria más frecuente, según diversas técnicas, es la derecha, seguida de la izquierda en hombres y de una circulación balanceada en mujeres.

Introduction. The anterior interventricular artery originates from the left coronary artery and irrigates the anterior surface of the ventricles, apex, and interventricular septum, making it the second most relevant artery of the heart. Objective. To describe the anatomical and clinical aspects of the anterior interventricular artery through angiography. Materials and methods. A descriptive study was conducted using 200 angiographic reports of Colombian individuals. The anterior interventricular artery's origin, course, patency, and coronary dominance were evaluated. Data related to chest pain, acute myocardial infarction, dyslipidemia, and electrocardiographic abnormalities were included. Statistical tests could not be performed due to this artery's low prevalence of anatomical variations. Results. One anterior interventricular artery was found to have originated from the left coronary sinus without a myocardial bridge, with no alteration in permeability, and with left dominance. The frequency of bridges was 2%, and the most frequent dominance was right in 86; permeability alterations occurred in 43% mainly affecting S13. Twenty-five per cent presented chest pain; 40%, echocardiographic alterations; 5%, ischemic heart disease, and 59%, electrocardiographic alterations. Conclusions. Variations of origin of the anterior interventricular artery have a low prevalence according to reports from Chile, Colombia, and Spain. anterior interventricular artery myocardial bridges were scarce compared to other studies, suggesting better specificity of computed tomography angiography or direct dissection for these findings. The assessment of coronary permeability is graded with the thrombolysis in myocardial infarction scale; values 0 and 1 indicate occlusive lesion associated with ischemic heart disease. According to various techniques, the most frequent coronary dominance the right, followed by the left in men and balanced circulation in women.

Manejo de la hemorragia posparto en una paciente con útero bicorne con la sutura B-Lynch. Reporte de caso y revisión de la literatura / Management of postpartum hemorrhage in a patient with bicornuate uterus using the B-Lynch suture. Case report and review of the literature

Objetivos: Describir un caso de manejo con sutura de B-Lynch en una mujer con útero bicorne que presenta hemorragia posparto (HPP), y realizar una revisión de la literatura acerca de las estrategias de control quirúrgico de la HPP en pacientes con malformaciones müllerianas, los resultados maternos en términos de control de la hemorragia, así como las complicaciones tempranas y tardías. Materiales y métodos: Se reporta el caso de una paciente con útero bicorne, atendida en un hopsital de referencia regional, que, posterior a parto por cesárea, presenta hemorragia posparto, la cual es controlada con éxito empleando la sutura de B-Lynch. Se realizó una búsqueda en las siguientes bases de datos: PubMed, Embase, Medline, Google Scholar y LILACS. Se emplearon los términos MeSh: "Uterine Atony", "Postpartum Hemorrhage", "Immediate Postpartum Hemorrhage", "Bicornuate Uterus", "Mullerian Anomalies", "Mullerian Ducts Abnormalities", "Uterine Compression Suture". Se incluyeron reportes y series de casos que hubieran incluido pacientes con malformaciones müllerianas con HPP que no respondieran al manejo farmacológico inicial, con uso de procedimientos quirúrgicos conservadores para el control de la hemorragia. Se hace análisis narrativo de los hallazgos respecto a las características de los estudios, técnicas utilizadas y complicaciones. Resultados: Se seleccionaron 5 estudios, 4 de tipo reporte de caso y 1 serie de casos, que corresponden a 12 mujeres con malformación uterina que desarrollaron HPP, en las cuales se empleó manejo quirúrgico (suturas de compresión uterina), y con dispositivo (tipo balón intrauterino) para el control de la hemorragia, logrando controlar el sangrado en 11 casos, solo una (8,3 %) requirió histerectomía. No se reportan complicaciones a largo plazo. Conclusiones: El manejo de la HPP en mujeres con malformaciones uterinas müllerianas cuenta con una literatura limitada a reportes de casos, con uso tanto de suturas compresivas como dispositivos médicos tipo balones intrauterinos. La sutura compresiva tipo B-Lynch parece ser una buena alternativa en el control de la HPP en estas mujeres con el fin de preservar la fertilidad, con baja tasa de complicaciones. Se requiere continuar documentando este tipo de casos con el fin de consolidar evidencia de la utilidad de esta técnica en el control del sangrado uterino posparto en esta población.

Objectives: To describe the use of the B-Lynch suture in a case of postpartum hemorrhage of a woman with bicornuate uterus, and to carry out a review of the literature on PPH control strategies in patients with müllerian anomalies, maternal outcomes in terms of hemorrhage control, as well as early and late complications. Material and methods: Case report of a patient with bicornuate uterus who presented to a regional referral hospital with postpartum hemorrhage following a cesarean section, which was successfully controlled using the B-Lynch suture. A search was conducted in the PubMed, Embase, Medline, Google Scholar and LILACS databases. The MeSh terms used were: "Uterine Atony," "Postpartum Hemorrhage," "Immediate Postpartum Hemorrhage," "Bicornuate Uterus," "Müllerian Anomalies," "Müllerian Duct Abnormalities". Case reports and case series of patients with müllerian malformations and PPH not responding to initial pharmacological management in whom conservative surgical procedures were used to control bleeding were included. A narrative analysis of the findings was carried out based on study characteristics, techniques used and complications. Results: Five studies were selected, 4 case reports and 1 case series which included 12 women with uterine malformations who developed PPH and in whom surgical management (uterine compression sutures) or devices (intrauterine balloon) were used for hemorrhage control. Bleeding was successfully controlled in 11 cases, with hysterectomy required only in one case (8.3 %). No long-term complications were reported. Conclusions: The literature on PPH management in women with müllerian uterine malformations is limited to case reports in which either compression sutures or medical devices such as intrauterine balloons were used. The B-Lynch-type compression suture appears to be a good option for controlling PPH in these women in order to preserve fertility, with a low rate of complications. Further documentation of these types of cases is needed in order to build the evidence regarding the usefulness of this technique for controlling postpartum uterine bleeding in this population.

Riñón pélvico fusionado con uréter único, hidrometrocolpos y ano imperforado: conjunción de malformaciones infrecuentes en una adolescente / Pancake kidney with a single ureter, hydrometrocolpos, and imperforate anus: a set of uncommon malformations in a female adolescent

Una niña de 11 años de edad con antecedentes de ano imperforado, infección urinaria y episodios de constipación intermitentes se presentó a la consulta con cólicos abdominales y náuseas de una semana de evolución. Estudios radiológicos revelaron hidrometrocolpos y fusión renal pélvica con uréter único hidronefrótico. El examen vaginal evidenció un tabique transverso no permeable. Se evacuó temporalmente la colección con resolución de los síntomas. La paciente fue programada para cirugía vaginal reconstructiva definitiva. Se destaca en este caso no solo la asociación de malformaciones infrecuentes, sino una sintomatología muy común en la práctica pediátrica a causa de una patología rara vez considerada en el diagnóstico diferencial, y la importancia de una evaluación precoz y completa de este tipo de malformaciones para un tratamiento oportuno.

An 11-year-old girl with a history of imperforate anus, urinary tract infection, and intermittent episodes of constipation presented with abdominal pain and nausea for 1 week. The x-rays revealed hydrometrocolpos and fused pelvic kidney with a single hydronephrotic ureter. The vaginal examination revealed a non-permeable transverse vaginal septum. The collection was temporarily drained and symptoms resolved. The patient was scheduled for definitive vaginal reconstructive surgery. In this case, it is worth noting the association of infrequent malformations and also the signs and symptoms very common in pediatric practice due to a pathology rarely considered in the differential diagnosis, and the importance of an early and complete assessment of this type of malformations for a timely treatment.

Radial peripapillary capillary network in optic disc anomalies with abnormal tissues on disc surface

Background: Optic disc anomalies with abnormal tissue on the disc surface includes, myelinated nerve fiber, optic disc drusen, and Bergmeister papillae. Imaging the radial peripapillary capillary (RPC) network in optic disc anomalies with optical coherence tomography–angiography (OCTA) can give information on the RPC network in these conditions. Purpose: This video describes the OCTA of optic nerve head and RPC network using the angio disc mode in cases of optic disc anomalies with abnormal tissue on the disc surface. Synopsis: This video presents characteristic features of RPC network in one eye each of myelinated nerve fiber, optic disc drusen, and Bergmeister papillae. Highlights: OCTA in optic disc anomalies with abnormal tissue on the disc surface show a dense RPC microvascular network. OCTA is an effective imaging modality to study vascular plexus/RPC and their alteration in these disc anomalies

Need of optical aids for schools for blind students in Pune and Nasik districts and compliance of their use

Purpose: To assess the need for spectacles and low?vision aids (LVA) in students attending schools for the blind and study their compliance with use. Methods: Comprehensive ocular evaluation was performed using a hand?held slit lamp and ophthalmoscope. Vision acuity was measured using a logarithm of the minimum angle of resolution (logMAR) chart for distance, as well as for near. Spectacles and LVAs were dispensed after refraction and LVA trial. Follow?up was performed to assess the vision using the LV Prasad Functional Vision Questionnaire (LVP?FVQ) along with compliance with use after 6 months. Results: Of the 456 students examined from six schools, 188 (41.2%) were female; 147 (32.2%) were <10 years of age. In all, 362 (79.4%) were blind since birth. The students dispensed only LVAs were 25 (5.5%), only spectacles were 55 (12.1%), and both spectacle and LVAs were 10 (2.2%). The vision improved using LVAs in 26 (5.7%) and using spectacles in 64 (9.6%). There was a significant improvement in LVP?FVQ scores (P < 0.001). Also, 68/90 students were available for follow?up, of whom 43 (63.2%) were compliant to use. Causes of not wearing spectacles or LVA in 25 were left somewhere or lost 13 (52%), broken 3 (12%), uncomfortable to use 6 (24%), not interested to use 2 (8%), and got operated 1 (4%). Conclusion: Although the dispensing of LVA and spectacles improved the visual acuity and vision function of 90/456 (19.7%) students, nearly a third were not using them after 6 months. Efforts need to be taken to improve the compliance of use

Etiology of Hemoptysis in Children and Adolescents – Multicentric Retrospective Data (2012-2021) From Chennai

Objective: To analyze the etiology of hemoptysis in children and to correlate the severity of bleed with the etiology. Methods: This retrospective multicentre study reviewed data from inpatient units of four tertiary care public and private sector pediatric hospitals in Tamil Nadu. Methods: Inpatient case records of children (aged 2 month-15 years) treated for hemoptysis at the four institutions between April, 2012 and March, 2021 were identified, after ethical clearance from respective institutions. Data of children with underlying known bleeding disorders like hemophilia or platelet abnormality were excluded from the study. Hemoptysis was categorized as mild, moderate and severe Results: Of the 73 children who had presented with hemoptysis during the study period, 60 (82.2%) children had mild, 9 (12.3%) had moderate and rest had severe hemoptysis. Idiopathic pulmonary hemorrhage was the most common cause of hemoptysis. The common causes of mild hemoptysis in children were idiopathic pulmonary hemorrhage (n=15, 25%), pulmonary tuberculosis (n=12, 20%) and pneumonia (n=8, 13.3%). Congenital airway anomalies and vascular anomalies were more likely to present with moderate to severe bleed. Conclusion: Etiology of hemoptysis is broad and categorizing them into mild, moderate and severe may give a clue about the possible etiology, there by restricting to the required investigations.

Nexus of ocular motility and dry eye

Purpose: To evaluate the effectiveness of vision therapy (VT) in patients with chronic presumed refractory dry eye disease (DED) and concurrent nonstrabismic binocular vision anomalies (NSBVAs). To propose an algorithmic approach to manage patients with refractory DED. Methods: Thirty?two patients with chronic (>1 year) presumed refractory DED and NSBVA were prospectively evaluated. The baseline dry eye evaluation and comprehensive orthoptic evaluation were done. VT was administered by a trained orthoptist for 2 weeks. The binocular vision (BV) parameters and percentage subjective improvement were assessed after the VT. Results: On evaluation, 12 patients (37.5%) had both DED and NSBVA, and 20 patients (62.5%) had only NSBVA. Twenty?nine patients (90.62%) showed significant improvement in BV parameters following VT. Binocular near point of accommodation (median, range) improved from 17 (8–40) to 12 (5–26) mm (P value < 0.0001), and near point of convergence (median, range) improved from 6 (3–33) to 6 (5– 14) (P value 0.004) with VT. Thirty?one patients (96.87%) reported symptomatic improvement after VT, and 62.5% of these showed more than 50% improvement in symptoms. Conclusion: The present study confirms the beneficial role of VT in the treatment of patients with DED with concurrent NSBVA. It is essential to diagnose and treat NSBVA in patients with DED to ensure complete relief of symptoms and patient satisfaction. As there is a significant overlap between symptoms of dry eye disease and that of NSBVA, a complete orthoptic evaluation is recommended in all patients presenting with refractory dry eye disease related symptoms

Tendência temporal da prevalência e mortalidade infantil das anomalias congênitas no Brasil, de 2001 a 2018 / Temporal trends in prevalence and infant mortality of birth defects in Brazil, from 2001 to 2018

Resumo As anomalias congênitas (AC) configuram um relevante problema para a saúde pública global, afetando em média de 3% a 6% dos recém-nascidos em todo o mundo. No Brasil, ocupam a segunda posição entre os principais grupos de causas de óbito infantil. Assim, estudos amplos são necessários para mostrar o impacto das AC na saúde infantil. O presente estudo descreve a tendência temporal da prevalência e da mortalidade infantil por AC entre nascidos vivos (NV) no Brasil e em suas cinco regiões de 2001 a 2018, utilizando dados vinculados entre as bases de dados do Sistema de Informações sobre Nascidos Vivos (SINASC) e do Sistema de Informações sobre Mortalidade (SIM). A prevalência e mortalidade infantil por AC mostrou-se crescente no Brasil na maioria das regiões, principalmente no Norte e no Nordeste. Aquelas do aparelho osteomuscular foram as mais prevalentes ao nascimento (29,8/10.000 NV); as do aparelho circulatório passaram para a segunda posição (12,7/10.000 NV) após a vinculação das bases e representam a primeira causa de morte desse grupo. A técnica de vinculação de dados aplicada corrigiu a prevalência nacional das AC em 17,9% no período analisado, após serem recuperadas as AC notificadas no SIM, mostrando ser uma boa ferramenta para melhorar a qualidade das informações das AC.

Abstract Congenital anomalies (CA) are a relevant problem for global public health, affecting about 3% to 6% of newborns worldwide. In Brazil, these are the second main cause of infant mortality. Thus, extensive studies are needed to demonstrate the impact of these anomalies on births and deaths. The present study describes the temporal trends of prevalence and infant mortality due to CA among live births in Brazil and regions, from 2001 to 2018, using the related data between the Live Birth Information System (SINASC, acronym in Portuguese) and the Mortality Information System (SIM, acronym in Portuguese). The prevalence and infant mortality due to CA has increased in Brazil and in most regions, especially in the Northeast and North. CAs in the musculoskeletal system were the most frequent at birth (29.8/10,000 live births), followed by those in the circulatory system (12.7/10,000 live births), which represented the primary cause of death in this group. The applied linkage technique made it possible to correct the national prevalence of CA by 17.9% during the analyzed period, after retrieving the anomalies reported in SIM, thereby proving to be a good tool to improve the quality of information on anomalies in Brazil.

Anomalous aortic origin of the left coronary artery / Origen aórtico anómalo de la arteria coronaria izquierda

Abstract We present an unusual case of an anomalous left coronary artery arising from the contralateral sinus of Valsalva: a 63-year-old male patient who consulted to our emergency department with 1-week history of progressive dyspnea on exertion with clinical signs of heart failure, associated with lung congestion on the chest X ray and elevated NT-proBNP levels (2000 pg/ml; normal value <150). Doppler echocardiography showed severe dilation of both left atrium and left ventricle, with severe deterioration of LV systolic function (Ejection fraction of 26%), global hypokinesia and a moderate mitral regurgitation with central jet. A cardiac cath eterization was performed, which evidenced an anomalous origin of the left main coronary artery from the right coronary sinus with a proximal lesion of nearly 50%. A coronary computed tomographic angiography confirmed the diagnosis of an anomalous origin with an intramyocardial path at the level of the interventricular septum, as sociated with moderate extrinsic compression. To determine the degree of functional ischemia presented by the left main coronary artery lesion we performed a fractional flow reserve evaluation, resulting in 0.75, which was ranked as significant. An angioplasty with implantation of a drug-eluting stent (with Everolimus) was performed successfully to the target lesion. The patient evolved favorably during hospitalization and was discharged from the medical center to continue outpatient follow-up. Patient remained asymptomatic at 1-month and 6 months, during clinical evaluation, without evidence of ischemia on noninvasive functional assessment.

Resumen Presentamos un raro caso de nacimiento anómalo de arteria coronaria izquierda en el seno de Valsalva contralateral. Se trata de un hombre de 63 años que consultó al servicio de emergencias de nuestro centro por disnea progresiva de una semana de evolución, con signos clínicos de insuficiencia cardíaca, aso ciado a signos de congestión en la radiografía de tórax, y valores de NT-proBNP elevados (2000 pg/ml; valor normal <150). El ecocardiograma Doppler evidenció dilatación grave de la aurícula y del ventrículo izquierdo, con deterioro grave de la función sistólica (fracción de eyección de 26%), hipoquinesia global e insuficiencia mitral moderada con jet central. Se realizó una cinecoronariografía que evidenció el nacimiento anómalo del tronco de arteria coronaria izquierda desde el seno coronario derecho, con una lesión cercana al 50%. Una angiotomografía coronaria confirmó el origen anómalo del vaso coronario, con trayecto intramiocárdico a nivel del septum interventricular asociado a compresión extrínseca moderada. Para determinar el grado de isquemia funcional que presentaba la lesión del tronco coronario izquierdo se evaluó la reserva de flujo fraccional, que arrojó un resultado de 0.75 el cual se consideró significativo, prosiguiendo a angioplastia con implante de stent liberador de droga (con Everolimus) a dicha lesión. El paciente evolucionó favorablemente durante la internación en el hospital, egresando de la institución para continuar seguimiento ambulatorio. Persistió asintomático en los controles realizados al mes y a los 6 meses, sin evidencia de isquemia en la evaluación funcional no invasiva.

Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies and management

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Radial peripapillary capillary network in congenital cavitary optic disk anomalies

Background and Purpose: "Congenital cavitary optic disk anomalies" is a term used to include optic disk pit (ODP), optic disk coloboma, and morning glory disk anomaly (MGDA). Imaging the radial peripapillary capillary (RPC) network in congenital optic disk anomalies with optical coherence tomography?angiography (OCTA) can shed light on its pathogenesis. This video describes the OCTA findings of optic nerve head and RPC network using the angio?disk mode in five cases of congenital cavitary optic disk anomalies. Synopsis: The video presents characteristic RPC network alterations in two eyes of ODP, one eye of optic disk coloboma, and two eyes of noncontractile MGDA. Highlights: OCTA in ODP and coloboma shows absence of RPC microvascular network and a region of capillary dropout. This finding is in contrast to MGDA, where the microvascular network is dense. OCTA is an effective imaging modality to study vascular plexus and RPC and their alteration in congenital disk anomalies, which could provide information about the structural differences among them.

Testicular Volume of Boys Aged 5-17 Years in Relation to Sexual Maturity Rating and Clinical Onset of Puberty in an Urban Setting in Gujarat, India

Background : Assessment of Sexual Maturity Rating and Testicular Volume are indispensable in the routine assessment of puberty in boys. There is paucity of data in Indian population for Testicular Volume particularly in early adolescence. Aims : The aims of the study were to collect data for testicular volume,correlate testicular volume with Sexual Maturity Rating (SMR) and the clinical onset of puberty; and to identify Testicular abnormalities in boys aged 5 to 17 years in an Urban setting in Gujarat, India. Materials and Methods : A prospective observational study was undertaken in boys aged5 to 17years of age from Gujarat from April, 2019 to August, 2019. Mean Testicular Volume was measured with a Prader抯 orchidometer. Parameters like Age, Weight and Height were also measured and Body Mass Index (BMI) was calculated. Pubertal stage was categorized using Tanner staging. Data was statistically analyzed using Microsoft Excel and SPSS software. Results : 977 boys were included in the study. Mean age at SMR stage 2 was 11.22 years. SMR stage 2 was earliest seen at 6 years and latest at 15 years of age. 15% of boys in pre-adolescence, 60% in early adolescence and 94% in middle adolescence showed changes of Puberty. Precocious puberty was detected in 33 boys (3.38%). Delayed Puberty was detected in 4 boys (0.4%) and Undescended Testes in 4 boys (0.4%). Testicular Volume showed positive correlation with Weight, Height and BMI.

Does Postpartum Hypopituitarism Influence the Craniofacial Pattern? A Case-Control Study with Sheehan's Syndrome Patients

This study aimed to assess the craniofacial morphologic aspects of Sheehan's syndrome (SHS) patients.An observational study was performed with 19 women diagnosed with SHS and 19 controls matched by age and sex. Lateral cephalometric radiographs were obtained, and 30 linear and angular measurements were analyzed using the Radiocef Studio 2 software. The mean age of patients was 65.47 ± 10.19 years. The main findings were propositioned maxilla (52.63 %) and mandible (52.63 %) relative to the cranial base, mandibular prognathism in 73.68 %, deep growth pattern in 42.1 %, increased mandibular plane in 36.84 %, and reduction in anterior facial height. The SHS group showed statistically significant differences in SNB (p=0.026), N-Me (p=0.006), soft palate length (p=0.011), and Ena-Me (p<0.001) in comparison with controls. The standard deviation score analysis revealed altered values in relation to total maxillary and mandibular lengths. SHS showed altered craniofacial morphology, characterized by maxillo- mandibular prognathism, brachyfacial type, increased mandibular plane, and reduction in soft palate length. This study reports novel findings in SHS.

Este estudio tuvo como objetivo evaluar los aspectos morfológicos craneofaciales de los pacientes con síndrome de Sheehan (SHS). Se realizó un estudio observacional con 19 mujeres diagnosticadas con SHS y 19 controles asociados por edad y sexo. Se obtuvieron radiografías cefalométricas laterales y se analizaron 30 medidas lineales y angulares mediante el software Radiocef Studio 2. La edad media de los pacientes fue de 65,47 ± 10,19 años. Los principales hallazgos fueron proposición maxilar (52,63 %) y mandíbula (52,63 %) con respecto a la base del cráneo, prognatismo mandibular en 73,68 %, patrón de crecimiento profundo en 42,1 %, aumento del plano mandibular en 36,84 % y reducción de la altura facial anterior. El grupo SHS mostró diferencias estadísticamente significativas en SNB (p=0,026), N-Me (p=0,006), longitud del paladar blando (p=0,011) y Ena-Me (p<0,001) en comparación con los controles. El análisis de la puntuación de la desviación estándar reveló valores alterados en relación con las longitudes maxilares y mandibulares totales. El SHS mostró una morfología craneofacial alterada, caracterizada por prognatismo maxilomandibular, tipo braquifacial, aumento del plano mandibular y reducción de la longitud del velo del paladar. Este estudio informa hallazgos novedosos en SHS.

Research progress on ankylosis of primary molars / 口腔疾病防治

@#Ankylosis of primary molars is a kind of eruption abnormality of the teeth, where the periodontal membrane disappears, owing to a bony union between bone and root. Studies have shown that the common proportion of ankylosed primary molars is 1.3%~8.9% with an equal occurrence. In the primary dentition, the mandibular first primary molar is the most commonly affected tooth, while in the middle mixed dentition stage of development, the second primary molar is more affected. Its etiology may be related to genetics, signaling pathways of mineralization metabolism of local alveolar bone or cementum, cytokines secreted by epithelial rest cells of Malassez, and enhanced inflammatory reactions during physiological absorption of roots. Ankylosis of primary molars can be diagnosed by clinical symptoms and imaging and is classified as mild, moderate and severe according to the degree of infraocclusion. As it may cause a series of complications, such as occlusal disturbances, delayed exfoliation and incomplete alveolar process development, multidisciplinary treatment, including in the departments of pediatric dentistry, orthodontics, periodontics and prosthodontics, should be adopted, and long-term treatment is determined based on the patient's age, severity of infraocclusion, and presence of permanent teeth. This review summarizes the etiology, diagnosis, complications and treatment of ankylosed primary molars to provide a reference for the clinical diagnosis and treatment of decidual molar fixation.

Advance in the immunodeficiency-centromeric instability-facial anomalies syndrome / 中华实用儿科临床杂志

The immunodeficiency-centromeric instability-facial anomalies (ICF) syndrome is the only known human genetic disease involving DNA methylation defects.About 50% of the cases are caused by the compound heterozygous mutation of DNMT3B gene.About a hundred cases were reported in the world, but only a few cases came from China.There may be a misdiagnosis and missed diagnosis.To the best of our knowledge, no Chinese article systematically discusses the ICF syndrome.This paper aims to review the possible mechanisms, clinical manifestations, genetic characteristics, treatment, and prognosis of the ICF syndrome, and to improve Chinese doctors′ knowledge about this disease.

Research Progress on the Application of Goel Technique in Craniovertebral Junction Anomalies / 中国医学科学院学报

Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding soft tissue,and nervous system,which are caused by a variety of factors.Chiari malformation is a common type of craniovertebral junction anomalies,the conventional surgical therapy of which is posterior fossa decompression.Currently,scholars represented by Goel have proposed a new theory on the classification,pathogenesis,and treatment of Chiari malformation based on posterior atlantoaxial fixation (Goel technique).This article introduces the progress in Goel technique,aiming to provide reference for the clinical work.

Consensus recommendations on the evaluation and treatment of laryngotracheal anomalies in infants and young children / 临床耳鼻咽喉头颈外科杂志

Infants with laryngotracheal anomalies are clinically manifested as stridor or noisy breathing, choking, hoarseness, feeding difficulties, and cyanotic spells, followed by developmental and growth retardation and other health issues; in severe cases, patients may present with severe dyspnea, which is associated with high mortality. A timely diagnosis as well as appropriate strategy for laryngotracheal anomalies is still challenging for pediatric otolaryngologists. This consensus statement, evolved from expert opinion by the members of the Pediatric Otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance for otolaryngologists who manage infants and young children with laryngotracheal anomalies in evaluation and treatment based on symptomatology, physical and laboratory examinations.

Prevalence of congenital malformations from 2015 up to 2019, in Rondônia (RO), Amazon, Brazil / Prevalência de malformações congênitas entre 2015 e 2019 em Rondônia (RO), Amazônia, Brasil

Abstract Objectives: to determine and characterize the prevalence of congenital malformations among live births in Rondônia, from 2015 to 2019. Methods: an ecological study was conducted using publicly available data at Sistema de Informações sobre Nascidos Vivos collected in January 2021. The prevalence of congenital malformation and possible associations were calculated. The strength of the association between variables was measured using the odds ratio. Variables that were statistically significant at the 5% level were selected for adjusted odds ratio calculation using logistic regression models. The R programming language was used for all analyses and interactions with the database. A 95% confidence interval was considered. Results: a total of 1,110 records were analyzed. The overall prevalence of congenital malformation was 8.36, with higher rates observed among mothers over 35 years, those with low educational levels, unmarried status, and preterm birth. Infants with low birth weight and low Apgar scores also had higher prevalence rates of congenital malformations. Conclusion: the prevalence of congenital malformations in the state was similar to the national average, but regional disparities were observed and warrant further investigation.

Resumo Objetivos: determinar e caracterizar a prevalência de malformações congênitas entre nascidos vivos em Rondônia, no período de 2015 a 2019. Métodos: estudo ecológico, com dados públicos disponíveis no Sistema de Informações sobre Nascidos Vivos coletados em janeiro de 2021. Foi calculada a prevalência das malformações e as possíveis associações. A força de associação entre as variáveis foi medida pela razão de odds. Aquelas variáveis consideradas estatisticamente significante ao nível de 5% foram selecionadas para o cálculo de razão de odds ajustada, usando modelo de regressão logística. A linguagem de programação R foi usada para todas as análises e interação com o banco de dados. O Intervalo de Confiança considerado foi de 95%. Resultados: foram analisados 1.110 registros. A prevalência geral de malformação congênita foi de 8,36, sendo maior entre mães com idade acima de 35 anos, baixa escolaridade, solteiras, com partos prematuros; crianças com baixo peso ao nascer e baixos escores de Apgar. Conclusão: a prevalência de malformação congênita no estado foi próxima da nacional, mas houve discrepâncias entre as regiões de saúde que merecem uma investigação futura.

Embarazo gemelar espontáneo en útero bicorpóreo: una contribución a la experiencia / Spontaneous twin pregnancy in a bicorporeal uterus, a contribution to the experience

Resumen ANTECEDENTES: Las anomalías uterinas congénitas son consecuencia de la formación anormal, fusión o reabsorción de los conductos de Müller durante el desarrollo embriológico. El útero bicorne, unicorne y didelfo (U1, U2, U3 ESHRE-ESGE) son defectos en la unificación con una baja prevalencia en la población general y en la que tiene antecedente de aborto e infertilidad; se estima en alrededor de 0.4 a 1.1%, respectivamente. La presentación del caso puede aportar información útil relacionada con la evolución del embarazo y los desenlaces perinatales cuando aparece esta anomalía. CASO CLÍNICO: Paciente de 35 años, con amenorrea secundaria y prueba inmunológica de embarazo positiva, con antecedentes de dismenorrea primaria severa y anomalía uterina congénita compatible con útero bicorpóreo (U3 completo ESHRE-ESGE), bicorne completo (4a-AFS) y didelfo. El embarazo trascurrió sin complicaciones hasta la semana 37, cuando finalizó mediante cesárea, con hipotonía uterina que no ameritó transfusión de hemoderivados. CONCLUSIÓN: Los embarazos gemelares con útero bicorne son excepcionales y casi todos se logran espontáneamente; los que llegan a término finalizan mediante cesárea electiva.

Abstract BACKGROUND: Congenital uterine anomalies are the result of abnormal formation, fusion, or resorption of the Müllerian ducts during embryological development. The bicornuate, unicornuate and didelphic uterus (U1, U2, U3 ESHRE/ESGE), are unification defects with a low prevalence both in the general population and in the population with a history of abortion and infertility, is estimated at around 0.4 to 1.1%, respectively. The presentation of the case can provide useful information related to with the evolution of the pregnancy and the perinatal outcomes when this anomaly appears. CLINICAL CASE: A 35-year-old patient with secondary amenorrhea and a positive immunological pregnancy test with a history of severe primary dysmenorrhea and previous magnetic resonance imaging with a congenital uterine anomaly compatible with a bicorporeal uterus (U3 complete ESHRE-ESGE), complete bicornuate (4a /AFS) , uterus didelphys (ASRM). The pregnancy was uncomplicated until 37 weeks, when it was terminated by cesarean section, with uterine hypotonia that did not require transfusion of blood products. CONCLUSION: Twin pregnancy with bicornuate uterus is a rare entity, most of them have been achieved spontaneously and those that reach term have been resolved by elective cesarean section.