Immunoglobulin G4-Related Disease, A Diagnosis to Take into Account: About a Case

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated and multisystemic condition associated with developing fibroinflammatory lesions in any organ. The diagnosis is made based on the sum of clinical, serological, radiological, and histopathological criteria; however, this is often difficult due to its similarity to neoplasms, infections, or other immune-mediated diseases. Treatment is based on corticosteroids, in a possible combination with immunomodulators. The present case concerns a 59-year-old man with a history of jaundice syndrome and weight loss, admitted for suspected malignant neoplasia of the bile duct. Imaging revealed stricture with dilation of the intrahepatic bile ducts, prominent pancreas, pancreatic duct stricture, and nodular renal lesions. Due to the history of left submandibulectomy two years before the current disease and histology compatible with Küttner's tumor, plus the high IgG4 serum values, the diagnosis of IgG4-RD was established. He started treatment with corticosteroids and was asymptomatic during follow-up.

La enfermedad relacionada con inmunoglobulina G4 (ER-IgG4) es una condición inmunomediada y multisistémica asociada al desarrollo de lesiones fibroinflamatorias en cualquier órgano. El diagnóstico se realiza bajo la suma de criterios clínicos, serológicos, radiológicos e histopatológicos; sin embargo, este suele ser difícil debido a su similitud con neoplasias, infecciones u otras enfermedades inmunomediadas. El tratamiento se basa en corticosteroides, en una posible combinación con inmunomoduladores. El presente caso trata de un varón de 59 años con historia de síndrome ictérico y baja de peso, admitido por sospecha de neoplasia maligna de la vía biliar. Las imágenes revelaron estenosis con dilatación de las vías biliares intrahepáticas, páncreas prominente, estenosis del conducto pancreático y lesiones nodulares renales. Debido al antecedente de submandibulectomía izquierda 2 años antes de la enfermedad actual e histología compatible con tumor de Küttner, más los valores elevados de IgG4 sérico, se estableció el diagnóstico de ER-IgG4. Inició tratamiento con corticosteroides y cursó asintomático durante el seguimiento.

Enfermedad de Pompe: historia, epidemiología, fisiopatología y manifestaciones clínicas / Pompe's disease: History, epidemiology, pathophysiology and clinical manifestations

Introducción: La enfermedad de Pompe o glucogenosis tipo II pertenece al grupo de las miopatías metabólicas y es producida por la deficiencia parcial o total de la enzima alfa glucosidasa ácida. La ausencia/ déficit de esta enzima genera un almacenamiento de glucógeno en el interior de los lisosomas en diversos tejidos, incluidos el músculo esquelético, el miocardio y las células del músculo liso. Se trata de una enfermedad multisistémica que puede tener un inicio temprano o tardío de los síntomas. Contenidos: En este artículo se describirán los aspectos históricos de la enfermedad, su fisiopatología y sus manifestaciones clínicas, con el énfasis puesto en su inicio temprano o tardío. Conclusiones: Es necesario reconocer la enfermedad de Pompe debido a que esta patología es susceptible de tratamiento.

Introduction: Pompe's disease or glucogenosis type II belongs to the group of metabolic myopathies and is caused by a partial or total deficiency of the acid alpha glucosidase enzyme. The lack/deficiency of this enzyme generates glycogen storage inside the lysosomes in various tissues including skeletal muscle, myocardium and smooth muscle cells. It is a multisystemic disease that can have an early onset or a late onset. Contents: In this article, the historical aspects, the pathophysiology and the clinical manifestations of the disease, will be described. Conclusions: It is necessary to recognize Pompe disease because this pathology is treatable.

[Inflammatory Bowel Disease in elderly patients: Clinical characteristics and management in a tertiary hospital]. / Enfermedad Inflamatoria Intestinal en el adulto mayor: Características clínicas y manejo en un hospital de referencia.

Inflammatory bowel disease (IBD) in elderly patients is characterized by its clinical variability, different differential diagnoses and therapeutic management. The objective of our investigation is to evaluate the clinical characteristics and management of elderly patients with IBD. We developed an observational, descriptive, retrospective study from January 2011 to December 2019 in patients with IBD at the Gastroenterology Service of Guillermo Almenara Irigoyen National Hospital, Lima-Peru. 55 patients with CD and 107 with UC were evaluated; 45.6% of patients with IBD are older adults. Of these, 28 had CD and 46 UC. Older adults with CD presented predominantly an inflammatory phenotype and colonic location, while extensive and left-sided colitis were the most frequent in UC. Elderly patients had a lower CDAI score (279.8 vs 323.2) and a lower Mayo index (7.1 vs 9.2) in relation to the younger, without significant differences. Regarding treatment, a lower use of azathioprine (2 vs 8, p <0.03) and Anti-TNF (9 vs 18, p <0.01) was observed in the elderly with CD. The need for surgery and the frequency of post-surgical complications were similar between both groups. In conclusion, nearly half of IBD patients are older adults. The colonic location was the most frequent in CD, and in UC extensive and left colitis. We observed a lower use of azathioprine and biological therapy in elderly patients, without significant differences in the use of corticosteroids and aminosalicylates compared to younger people.

Multifocal glioblastoma and hormone replacement therapy in a transgender female.

Background: Glioblastoma multiforme represents approximately 60% of all brain tumors in adults. This malignancy shows a high level of biological and genetic heterogeneity associated with exceptional aggressiveness, leading to poor patient survival. One of the less common presentations is the appearance of primary multifocal lesions, which are linked with a worse prognosis. Among the multiple triggering factors in glioma progression, the administration of sex steroids and their analogs has been studied, but their role remains unclear to date. Case Description: A 43-year-old transgender woman who has a personal pathological history of receiving intramuscular (IM) hormone treatment for 27 years based on algestone/estradiol 150 mg/10 mg/mL. Three months ago, the patient suddenly experienced hemiplegia and hemiparesis in her right lower extremity, followed by a myoclonic focal epileptic seizure, vertigo, and a right frontal headache with a visual analog scale of 10/10. Magnetic resonance imaging images revealed an intra-axial mass with poorly defined, heterogeneous borders, and thick borders with perilesional edema in the left parietal lobe, as well as a rounded hypodense image with well-defined walls in the right internal capsule. The tumor was resected, and samples were sent to the pathology department, which confirmed the diagnosis of wild-type glioblastoma. Conclusion: This report identifies prolonged use of steroid-based hormone replacement therapy as the only predisposing factor in the oncogenesis of multifocal glioblastoma. It is an example that highlights the importance for physicians not to consider pathologies related to the human immunodeficiency virus rather than neoplasms in transgender patients in view of progressive neurological deterioration.

Enfermedad Inflamatoria Intestinal en el adulto mayor: Características clínicas y manejo en un hospital de referencia

La enfermedad inflamatoria intestinal (EII) en adultos mayores se caracteriza por su variabilidad clínica, distintos diagnósticos diferenciales y manejo terapéutico. El objetivo de la presente investigación es evaluar las características clínicas y manejo de los pacientes adultos mayores con EII. Se realizó un estudio observacional, descriptivo, retrospectivo de enero del 2011 a diciembre del 2019 en pacientes con EII en el Servicio de Gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen, Lima-Perú. Fueron evaluados 55 pacientes con EC y 107 con CU; 45,6% de pacientes con EII eran adultos mayores. De ellos, 28 tenían EC y 46 CU. Los adultos mayores con EC presentaron fenotipo inflamatorio y localización colónica predominantemente, mientras en CU, la colitis extensa e izquierda fueron las más frecuentes. Asimismo, los ancianos tuvieron menor puntaje CDAI (279,8 vs 323,2) y menor índice de Mayo (7,1 vs 9,2) con relación a los pacientes jóvenes, sin diferencias significativas. Respecto al tratamiento, se observó un menor uso de azatioprina (2 vs 8, p<0,03) y Anti-TNF (9 vs 18, p<0,01) en los adultos mayores con EC. La necesidad de cirugía y la frecuencia de complicaciones post quirúrgicas fueron similares entre ambos grupos. En conclusión, casi la mitad de los pacientes con EII son adultos mayores. La localización colónica fue la más frecuente en EC, y en CU la colitis extensa e izquierda. Observamos un menor uso de azatioprina y terapia biológica en adultos mayores, sin diferencias significativas en el uso de corticoides y aminosalicilatos respecto a los jóvenes.

Inflammatory bowel disease (IBD) in elderly patients is characterized by its clinical variability, different differential diagnoses and therapeutic management. The objective of our investigation is to evaluate the clinical characteristics and management of elderly patients with IBD. We developed an observational, descriptive, retrospective study from January 2011 to December 2019 in patients with IBD at the Gastroenterology Service of Guillermo Almenara Irigoyen National Hospital, Lima-Peru. 55 patients with CD and 107 with UC were evaluated; 45.6% of patients with IBD are older adults. Of these, 28 had CD and 46 UC. Older adults with CD presented predominantly an inflammatory phenotype and colonic location, while extensive and left-sided colitis were the most frequent in UC. Elderly patients had a lower CDAI score (279.8 vs 323.2) and a lower Mayo index (7.1 vs 9.2) in relation to the younger, without significant differences. Regarding treatment, a lower use of azathioprine (2 vs 8, p <0.03) and Anti-TNF (9 vs 18, p <0.01) was observed in the elderly with CD. The need for surgery and the frequency of post-surgical complications were similar between both groups. In conclusion, nearly half of IBD patients are older adults. The colonic location was the most frequent in CD, and in UC extensive and left colitis. We observed a lower use of azathioprine and biological therapy in elderly patients, without significant differences in the use of corticosteroids and aminosalicylates compared to younger people.

Deep DNA sequencing of MGMT, TP53 and AGT in Mexican astrocytoma patients identifies an excess of genetic variants in women and a predictive biomarker.

PURPOSE: Astrocytomas are a type of malignant brain tumor with an unfavorable clinical course. The impact of AGT and MGMT somatic variants in the prognosis of astrocytoma is unknown, and it is controversial for TP53. Moreover, there is a lack of knowledge regarding the molecular characteristics of astrocytomas in Mexican patients. METHODS: We studied 48 Mexican patients, men and women, with astrocytoma (discovery cohort). We performed DNA deep sequencing in tumor samples, targeting AGT, MGMT and TP53, and we studied MGMT gene promoter methylation status. Then we compared our findings to a cohort which included data from patients with astrocytoma from The Cancer Genome Atlas (validation cohort). RESULTS: In the discovery cohort, we found a higher number of somatic variants in AGT and MGMT than in the validation cohort (10.4% vs < 1%, p < 0.001), and, in both cohorts, we observed only women carried variants AGT variants. We also found that the presence of either MGMT variant or promoter methylation was associated to better survival and response to chemotherapy, and, in conjunction with TP53 variants, to progression-free survival. CONCLUSIONS: The occurrence of AGT variants only in women expands our knowledge about the molecular differences in astrocytoma between men and women. The increased prevalence of AGT and MGMT variants in the discovery cohort also points towards possible distinctions in the molecular landscape of astrocytoma among populations. Our findings warrant further study.

Evidence based Latin American Guidelines of clinical practice on prevention, diagnosis, management and treatment of glucocorticoid induced osteoporosis. A 2022 update : This manuscript has been produced under the auspices of the Committee of National Societies (CNS) and the Committee of Scientific Advisors (CSA) of the International Osteoporosis Foundation (IOF).

Guidelines and recommendations developed and endorsed by the International Osteoporosis Foundation (IOF) are intended to provide guidance for particular pattern of practice for physicians who usually prescribe glucocorticoid (GC) therapy, and not to dictate the care of a particular patient. Adherence to the recommendations within this guideline is voluntary and the ultimate determination regarding their application should be made by the physician in light of each patient's circumstances. Guidelines and recommendations are intended to promote a desirable outcome but cannot guarantee any specific outcome. This guideline and its recommendations are not intended to dictate payment, reimbursement or insurance decisions. Guidelines and recommendations are subjected to periodic revisions as a consequence of the evolution of medicine, technology and clinical practice. A panel of Latin American (LATAM) experts specialized in osteoporosis with recognized clinical experience in managing patients with glucocorticoid-induced osteoporosis (GIO) met to produce evidence-based LATAM recommendations for the diagnosis and management of GIO. These guidelines are particularly intended to general practitioners and primary care physicians who prescribe GC treatments in LATAM to guide their daily clinical practice in terms of evaluation, prevention and treatment of GIO. These recommendations were based on systematic literature review using MEDLINE, EMBASE, SCOPUS and COCHRANE Library database during the period from 2012 to 2021. Randomized clinical trials (RCT), systematic reviews of RCT, controlled observational studies, guidelines and consensus were considered. Based on the review and expert opinion the panel members voted recommendations during two successive rounds of voting by panel members. Agreements for each statement were considered if a concordance of at least 70% was achieved following Delphi methodology. Grading of recommendations was made according to the Oxford Centre for the Evidence-based Medicine (EBM) criteria. Among five GIO guidelines and consensus initially identified, two of them (American College of Rheumatology 2017 and the Brazilian Guidelines 2021) were selected for comparison considering the latter as the most current guides in the LATAM region. Based on this methodology fifty statements were issued. All of them but four (1.20, 1.21, 1.23 and 4.2) attained agreement.

Frontal aslant tract: Anatomy and tractography description in the Mexican population.

Background: The aim of the study was to describe the origin, course, and termination of frontal aslant tract (FAT) in the Mexican population of neurosurgical referral centers. Methods: From January 2018 to May 2019, we analyzed 50 magnetic resonance imaging (MRI) studies in diffusion tensor imaging sequences of patients of the National Institute of Neurology and Neurosurgery "Manuel Velasco Suárez." Five brains were fixed by the Klingler method and dissected in the neurosurgery laboratory of the Hospital Civil de Guadalajara to identify the origin, trajectory, and ending of the FAT. Results: FAT was identified in 100% of the MRI and brain dissections. The origin of the FAT was observed in 63% from the supplementary premotor area, 24% from the supplementary motor area, and 13% in both areas. Its ending was observed in the pars opercularis in 81%, pars triangularis in 9%, and in both pars opercularis and ventral premotor area in 10% in the magnetic resonance images, with a left side predominance. In the hemispheres dissections, the origin of FAT was identified in 60% from the supplementary premotor area, 20% from the supplementary motor area, and 20% in both areas. Its ending was observed in the pars opercularis in 80% and the pars triangularis in 20%. It was not identified as an individual fascicle connected with the contralateral FAT. Conclusion: In the Mexican population, FAT has a left predominance; it is originated more frequently in the supplementary premotor area, passes dorsal to the superior longitudinal fascicle II and the superior periinsular sulcus, and ends more commonly in the pars opercularis.

[Medical and surgical management of moderate-to-severe inflamatory bowel disease]. / Manejo médico quirúrgico de la enfermedad inflamatoria intestinal moderada-severa.

The management of inflammatory bowel disease (IBD) is mainly medical, however, more than 70% of patients with Crohn's disease (CD) and 25% with ulcerative colitis (UC) will require surgery during their lifetime. OBJECTIVE: To evaluate medical, surgical management and evolution in patients with moderate-to-severe IBD. MATERIALS AND METHODS: Observational, descriptive, retrospective study from January 2011 to December 2019 in the Gastroenterology Service of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. RESULTS: Twenty two patients with IBD, 17 with CD and 5 with UC were included. Male predominance (59%). Emergency surgery was performed in 35.2% and 60% of patients with CD and UC, respectively. Stenosis and toxic megacolon were the most frequent indications. According to the type of surgery, hemicolectomy (41%) and intestinalresection (41%) were the most frequently performed in CD, while in UC it was total colectomy (60%). Among the postoperative complications, dehiscence/fistula and intra-abdominal collections were the most frequently reported in CD; whereas in UC it was surgical site infection and adynamic ileus. After surgery, biologics and 5-ASA associated with immunomodulator were the most used treatment in CD and UC, respectively. Mortality was 17.6% in CD and 60% in UC. CONCLUSIONS: Surgical treatment is an option in the management of moderate-to-severe IBD. Emergency surgery in IBD continues to have a high morbidity and mortality rate.

Decompressive craniectomy: A salvage treatment for patients with central nervous system tumors.

BACKGROUND: Decompressive craniectomy (DC) has been used for the treatment of refractory increased intracranial pressure (ICP) in patients with brain trauma and stroke; its beneficial role is still a matter of debate. Little has been written on the role of DC in the setting of patients with intracranial tumors. METHODS: We retrospectively reviewed our institutional tumor registry for all adult patients treated with a DC as an emergency treatment between January 2012 and June 2019. RESULTS: A total of 61 patients were taken into surgery for a DC secondary to raised ICP related to a central nervous system tumor. The Kaplan-Meier curves in the study showed that 18.9 months was the mean survival time (MST) of the global population, 40 patients died (65.5%) during the follow-up period. Patients in the group of over 60 years had a worst survival time than younger patients (p = 0.01). Patients with intracerebral hemorrhage had the worst MST compared with the patients with other etiologies (p = 0.04). CONCLUSION: Our data show that in some selected cases DC is a viable option as a salvage treatment for patients with intracranial tumors.

ANTECEDENTES: la craniectomía Descompresiva (CD) se ha utilizado para el tratamiento del aumento de la presión intracraneal en pacientes con traumatismo cerebral y accidente cerebrovascular; su papel beneficioso sigue siendo un tema de debate. Poco se ha escrito sobre el papel de la CD en el contexto de pacientes con tumores intracraneales. MÉTODOS: Revisamos retrospectivamente nuestro registro institucional de tumores para todos los pacientes adultos tratados con craniectomía descompresiva como tratamiento de emergencia entre enero de 2012 y junio de 2019. RESULTADOS: Un total de 61 pacientes fueron llevados a cirugía por una CD secundaria a elevación de ICP secundario a un tumor del sistema nervioso central. Las curvas de Kaplan-Meyer mostraron que 18.9 meses fue el tiempo medio de supervivencia de la población global, 40 pacientes murieron (65.5%) durante el período de seguimiento. Los pacientes del grupo de más de 60 años tuvieron un peor tiempo de supervivencia que los pacientes menores (p = 0,01). Los pacientes con hemorragia intracerebral tuvieron la peor sobrevida en comparación con los pacientes con otras etiologías (p = 0,04). CONCLUSIÓN: Nuestros datos muestran que en algunos casos seleccionados, la CD es una opción viable como tratamiento de rescate para pacientes con tumores intracraneales.

Dolor abdominal y parálisis flácida, una aproximación a las porfirias: serie de casos y revisión de la literatura / Flaccid paralysis and abdominal pain, an approach to porphyrias: a literature review and case series

RESUMEN Las porfirias son trastornos metabólicos hereditarios causados por deficiencias enzimáticas de la biosíntesis del grupo HEM. Con presentación en distintos grupos de edades, más común en infancia y tercera a cuarta década de la vida, se caracterizan por elevación de porfirinas, y manifestaciones variadas cutáneas y neuro viscerales. Describimos una serie de 3 casos de pacientes femeninas en tercera década de la vida con dolor abdominal severo e inespecífico y una amplia gama de manifestaciones clínicas con sus complicaciones a corto y largo plazo en quienes se diagnosticó porfiria aguda intermitente (PAI). Se hará revisión en la literatura para aportar al reconocimiento temprano de estas condiciones e instaurar de forma temprana el manejo específico e impactar en desenlaces irreversibles.

ABSTRACT Porphyrias are inherited metabolic disorders caused by enzymatic deficiencies of HEM group biosynthesis. Most common in childhood at the third and fourth decade of life. They are characterized by increased levels of porphyrins, and various cutaneous, neurological, and visceral manifestations. We describe a series of 3 cases of female patients in the third decade of life with abdominal pain and a wide range of clinical manifestations and short and long-term complications. Our review contributes to the early recognition of these diseases to establish early specific managements to impact on irreversible outcomes.

Manejo de la hemorragia digestiva alta en pacientes con infección por SARS-CoV-2 en un hospital de Lima, Perú / Management of upper gastrointestinal bleeding in patients with SARS-COV-2 infection in a hospital in Lima-Peru

Resumen Introducción: la pandemia por SARS-CoV-2 ha conllevado la reestructuración de las unidades de endoscopía digestiva en el mundo, lo cual ha limitado los procedimientos endoscópicos y priorizado indicaciones de emergencia como la hemorragia digestiva alta (HDA). No obstante, existe escasa evidencia respecto a su impacto en la evolución y resultados. Objetivo: evaluar el manejo de la HDA en el contexto de la pandemia del coronavirus por SARS-CoV-2. Materiales y métodos: estudio observacional, descriptivo, retrospectivo de marzo a agosto de 2020 en pacientes con diagnóstico de HDA e infección por SARS-CoV-2. Resultados: de 4320 pacientes con infección por SARS-CoV-2, 51 presentaron HDA al ingreso. La mediana de edad fue de 70 años. El 58,8 % era de sexo masculino. El 56,9 % tenía una puntuación de Glasgow-Blatchford (SGB) ≥12. El 21,6 % requirió soporte de oxígeno. Solo 34 pacientes (66,7 %) recibieron tratamiento médico; asimismo, 17 (33,3 %) recibieron tratamiento médico más endoscopia digestiva alta (EDA); de estos, a 6 (35,3 %) se les realizó endoscopia terapéutica. La enfermedad ulcerosa péptica fue el hallazgo más frecuente. Al comparar el tipo de tratamiento recibido, no hubo diferencias significativas entre el número de transfusiones de glóbulos rojos, resangrado, reingreso por HDA, estancia hospitalaria ni mortalidad secundaria a la HDA. La mortalidad global fue del 25,4 % (13 pacientes) y se debió, principalmente, al compromiso respiratorio por SARS-CoV-2. Conclusiones: se observa una reducción en el número de EDA de emergencia por HDA en la pandemia actual, así como un tiempo mayor al estándar para su realización. Más del 80 % de los pacientes que recibieron solo tratamiento médico evolucionaron favorablemente, y solo un tercio de los pacientes a quienes se les realizó una EDA requirió terapéutica endoscópica.

Abstract Introduction: The SARS-CoV-2 pandemic has led to the restructuring of digestive endoscopy units around the world, limiting endoscopic procedures and prioritizing emergency indications such as upper gastrointestinal hemorrhage (UGH). However, there is little evidence regarding its impact on evolution and outcomes. Objective: To evaluate the management of UGH in the context of the SARS-CoV-2 coronavirus pandemic. Materials and methods: Observational, descriptive, retrospective study carried out between March and August 2020 in patients with diagnosis of UGH and SARS-CoV-2 infection. Results: Of 4 320 patients with SARS-CoV-2 infection, 51 had UGH on admission. The median age of the population was 70 years and 58.8% were male. Glasgow-Blatchford Bleeding Score (GBS) of ≥12 was obtained in 56.9%. Oxygen support was required by 21.6%. 34 (66.7%) patients received medical treatment only, while 17 (33.3%) received medical treatment plus upper gastrointestinal endoscopy (UGE), of which 6 (35.3%) underwent therapeutic endoscopy. Peptic ulcer disease was the most frequent finding. When comparing the type of treatment received, there were no significant differences between the number of red blood cell transfusions, rebleeding, re-admission due to UGH, hospital stay, or mortality secondary to UGH. Overall mortality was 25.4% (13 patients), mainly due to respiratory failure due to SARS-CoV-2. Conclusions: A reduction in the number of emergency upper gastrointestinal endoscopies for UGH was observed during the current pandemic, as well as a longer than standard time for their performance. More than 80% of patients who received medical treatment alone evolved favorably and only one third of the patients who underwent UGE required endoscopic therapy.

Manejo médico quirúrgico de la enfermedad inflamatoria intestinal moderada-severa

El tratamiento de la enfermedad inflamatoria intestinal (EII) es principalmente médico, sin embargo, más del 70% de pacientes con enfermedad de Crohn (EC) y 25% con colitis ulcerativa (CU) requerirán cirugía. Objetivos: Evaluar el manejo médico- quirúrgico y evolución en pacientes con EII moderada-severa. Materiales y métodos: Estudio observacional, descriptivo, retrospectivo de enero del 2011 a diciembre del 2019 en el Servicio de Gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen, Lima-Perú. Resultados: Se incluyó 22 pacientes con EII, 17 con EC y 5 con CU. El 59% fueron masculinos. Se realizó cirugía de emergencia en el 35,2% y el 60% de los pacientes con EC y CU respectivamente. La estenosis y el megacolon tóxico fueron las indicaciones más frecuentes. Según el tipo de cirugía, la hemicolectomía (41%) y la resección intestinal (41%) fueron las más frecuentemente realizadas en EC, mientras que, en CU fue la colectomía total (60%). Dentro de las complicaciones postquirúrgicas, las dehiscencias/fistulas y colecciones intraabdominales fueron las más frecuentemente reportadas en EC; mientras que, en CU fue la infección del sitio operatorio e íleo adinámico. Luego de cirugía, los biológicos y los 5-ASA asociados a inmunomodulador fueron el tratamiento más utilizados en EC y CU respectivamente. La mortalidad fue del 17,6% en EC y 60% en CU. Conclusiones: El tratamiento quirúrgico es una opción en el manejo de la EII moderada-severa. La cirugía de emergencia en EII continúa presentando una alta morbimortalidad.

The management of inflammatory bowel disease (IBD) is mainly medical, however, more than 70% of patients with Crohn's disease (CD) and 25% with ulcerative colitis (UC) will require surgery during their lifetime. Objective: To evaluate medical, surgical management and evolution in patients with moderate-to-severe IBD. Materials and methods: Observational, descriptive, retrospective study from January 2011 to December 2019 in the Gastroenterology Service of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. Results: Twenty two patients with IBD, 17 with CD and 5 with UC were included. Male predominance (59%). Emergency surgery was performed in 35.2% and 60% of patients with CD and UC, respectively. Stenosis and toxic megacolon were the most frequent indications. According to the type of surgery, hemicolectomy (41%) and intestinal resection (41%) were the most frequently performed in CD, while in UC it was total colectomy (60%). Among the postoperative complications, dehiscence/fistula and intra-abdominal collections were the most frequently reported in CD; whereas in UC it was surgical site infection and adynamic ileus. After surgery, biologics and 5-ASA associated with immunomodulator were the most used treatment in CD and UC, respectively. Mortality was 17.6% in CD and 60% in UC. Conclusions: Surgical treatment is an option in the management of moderate-to-severe IBD. Emergency surgery in IBD continues to have a high morbidity and mortality rate.

Pregnancy and brain tumors; a systematic review of the literature.

The incidence of primary brain tumors during pregnancy is uncommon. The etiology of these can range from different genetic syndromes such as Li Fraumeni, neurofibromatosis type I, and hormonal associated tumors. The number of meningiomas gradually tends to increase during pregnancy, suggesting a relationship between non-malignant meningiomas and hormonal changes. Clinical features are non specific or can be misinterpreted with pregnancy symptoms such as headache, vomiting and dizziness. It is worth mentioning that the symptoms due to intracranial tumors are no different in pregnant compared with non pregnant patients. However, retrospective studies in glioma behavior suggested that both tumor volume and growth, increased during pregnancy. These changes were correlated with clinical worsening and increased frequency of seizures. The diagnosis requires a proper neurologic exploration and the support of imaging studies. Treatment of tumors is very controversial since we look for the preservation of both mother and fetus. In theory, the best therapy for the mother will also be the best therapy for the fetus. During pregnancy, ideally the treatment is symptomatic, to preserve the fetus, and definite treatment may be performed after birth; the latter is not always accomplished since patients may present with impending herniation or a malignant tumor for which immediate management is necessary. We intend to give an updated review in the literature on the adequate treatment of brain tumors during pregnancy and the anesthetic management during the definite treatment. Literature data was obtained from Pubmed using the search terms: "Pregnancy", "Brain", "Tumors". A total of forty-three articles were selected.

Challenges and opportunities for quality densitometry in Latin America.

Latin America and the Caribbean region account for 8% of the world's total population. Experts from 18 countries were invited to contribute to this article to provide the best available data on the number, types, and quality of densitometry, DXA experts, the local/national incidence and prevalence of osteoporosis and fragility fractures, and other uses and information on the challenges and opportunities for quality densitometry with examples of local initiatives. Osteoporosis is the primary reason for densitometry in our region, which is not a priority for public health in most countries. Access and quality are major challenges, and there is a clear trend to concentrate on densitometry services in the largest cities. Urgent action is needed to face the rapidly increasing burden of osteoporosis in our region, including robust and up to date epidemiology, access to health professionals, and quality densitometry. Health professionals require better access to training, courses, and other activities at a more local or regional level.

Landscape of Germline Genetic Variants in AGT, MGMT, and TP53 in Mexican Adult Patients with Astrocytoma.

Astrocytoma is the most common type of primary brain tumor. The risk factors for astrocytoma are poorly understood; however, germline genetic variants account for 25% of the risk of developing gliomas. In this study, we assessed the risk of astrocytoma associated with variants in AGT, known by its role in angiogenesis, TP53, a well-known tumor suppressor and the DNA repair gene MGMT in a Mexican population. A case-control study was performed in 49 adult Mexican patients with grade II-IV astrocytoma. Sequencing of exons and untranslated regions of AGT, MGMT, and TP53 from was carried in an Ion Torrent platform. Individuals with Mexican Ancestry from the 1000 Genomes Project were used as controls. Variants found in our cohort were then assessed in a The Cancer Genome Atlas astrocytoma pan-ethnic validation cohort. Variants rs1926723 located in AGT (OR 2.74, 1.40-5.36 95% CI), rs7896488 in MGMT (OR 3.43, 1.17-10.10 95% CI), and rs4968187 in TP53 (OR 2.48, 1.26-4.88 95% CI) were significantly associated with the risk of astrocytoma after multiple-testing correction. This is the first study where the AGT rs1926723 variant, TP53 rs4968187, and MGMT rs7896488 were found to be associated with the risk of developing an astrocytoma.

[Flaccid paralysis and abdominal pain, an approach to porphyrias: a literature review and case series]. / Dolor abdominal y parálisis flácida, una aproximación a las porfirias: serie de casos y revisión de la literatura.

Porphyrias are inherited metabolic disorders caused by enzymatic deficiencies of HEM group biosynthesis. Most common in childhood at the third and fourth decade of life. They are characterized by increased levels of porphyrins, and various cutaneous, neurological, and visceral manifestations. We describe a series of 3 cases of female patients in the third decade of life with abdominal pain and a wide range of clinical manifestations and short and long-term complications. Our review contributes to the early recognition of these diseases to establish early specific managements to impact on irreversible outcomes.

Discrimination of residents during neurosurgical training in Mexico: Results of a survey prior to SARS-CoV-2.

BACKGROUND: Recent severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic represents an important negative impact on global training of neurosurgery residents. Even before the pandemic, discrimination is a challenge that neurosurgical residents have consistently faced. In the present study, we evaluated discriminatory conditions experienced by residents during their neurosurgical training in Mexico before the SARS-CoV-2 pandemic. METHODS: An electronic survey of 18 questions was sent among residents registered in the Mexican Society of Neurological Surgery (MSNS), between October 2019 and July 2020. Statistical analysis was made in IBM SPSS Statistics 25. The survey focused on demographic characteristics, discrimination, personal satisfaction, and expectations of residents. RESULTS: A response rate of 50% (132 of 264 residents' members of MSNS) was obtained and considered for analysis. Median age was 30.06 ± 2.48 years, 5.3% (n = 7) were female and 16.7% (n = 22) were foreigners undergoing neurosurgical training in Mexico. Approximately 27% of respondents suffered any form of discrimination, mainly by place of origin (9.1%), by gender (8.3%) or by physical appearance (6.1%). About 42.9% (n = 3) of female residents were discriminated by gender versus 6.4% (n = 8) of male residents (P = 0.001); while foreign residents mentioned having suffered 10 times more an event of discrimination by place of origin compared to native Mexican residents (36.4% vs. 3.6%, P < 0.001). CONCLUSION: This manuscript represents the first approximation to determine the impact of discrimination suffered by residents undergoing neurosurgical training in Mexico before the SARS-CoV-2 pandemic.

Reconversion of neurosurgical practice in times of the SARS-CoV-2 pandemic: a narrative review of the literature and guideline implementation in a Mexican neurosurgical referral center.

OBJECTIVE: The coronavirus disease 2019 (COVID-19) pandemic has forced the modification of surgical practice worldwide. Medical centers have been adapted to provide an efficient arrangement of their economic and human resources. Although neurosurgeons are not in the first line of management and treatment of COVID-19 patients, they take care of patients with neurological pathology and potential severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Here, the authors describe their institutional actions against the pandemic and compare these actions with those in peer-reviewed publications. METHODS: The authors conducted a search using the MEDLINE, PubMed, and Google Scholar databases from the beginning of the pandemic until July 11, 2020, using the following terms: "Neurosurgery," "COVID-19/SARS-CoV-2," "reconversion/modification," "practice," "academy," and "teaching." Then, they created operational guidelines tailored for their institution to maximize resource efficiency and minimize risk for the healthcare personnel. RESULTS: According to the reviewed literature, the authors defined the following three changes that have had the greatest impact in neurosurgical practice during the COVID-19 pandemic: 1) changes in clinical practices; 2) changes in the medical care setting, including modifications of perioperative care; and 3) changes in the academic teaching methodology. CONCLUSIONS: The Instituto Nacional de Neurología y Neurocirugía "Manuel Velasco Suárez" is one of the major referral centers for treating highly complex neurosurgical pathologies in Mexico. Its clinical and neurosurgical practices have been modified with the implementation of specific interventions against the spread of COVID-19. These practical and simple actions are remarkably relevant in the context of the pandemic and can be adopted and suited by other healthcare centers according to their available resources to better prepare for the next event.

Síndrome de Sjögren primario que debuta con polimiositis mitocondrial, neuropatía axonal y parálisis hipopotasémica: reporte de caso / Onset of primary Sjögren syndrome with mitochondrial polymyositis, axonal neuropathy, and hypokalaemic paralysis: Case report

RESUMEN El síndrome de Sjögren es una entidad multisistémica de naturaleza autoinmune, clásicamente considerada una exocrinopatía debido a la alta frecuencia de síntomas secos (queratoconjuntivitis seca, xerostomía) como resultado de infiltración poliglandular por linfocitos autorreactivos. Sin embargo, menos del 10% de estos pacientes puede iniciar con manifestaciones extraglandulares severas, traducidas en peores desenlaces a largo plazo. Se presenta el caso de una gestante que inició con síndrome de debilidad aguda proximal relacionada con miositis con enfermedad mitocondrial e hipopotasemia severa, en el contexto de acidosis tubular renal distal, como manifestación extraglandular de síndrome de Sjögren primario. Se discuten brevemente manifestaciones neurológicas de esta entidad, incluyendo aquellas secundarias a trastornos metabólicos precipitados por compromiso autoinmune.

ABSTRACT Sjögren's syndrome is a multisystemic autoimmune disorder. It is classically considered as an exocrine disease, given the high frequency of dry symptoms (keratoconjunctivitis sicca, xerostomia) as a result of poly-glandular infiltration by autoreactive lymphocytes. However, less than 10% of these patients can onset with severe extra-glandular manifestations, resulting in worse long-term outcomes. The case of a pregnant woman is presented, who debuted with acute proximal weakness syndrome related to myositis with mitochondrial pathology and severe hypokalaemia in the context of distal renal tubular acidosis, as an extra-glandular manifestation of primary Sjögren's syndrome. Neurological manifestations of this condition are briefly discussed, including those secondary to metabolic disorders precipitated by autoimmune compromise.