Pratique anesthésique pour adénomectomies hypophysaires par voie endonasale à Abidjan, Côte d'Ivoire / Anesthesia for endonasal pituitary adenomectomies in Abidjan, Ivory Coast

L'objectif de l'étude est d'évaluer la pratique anesthésique au cours des adénomectomies hypophysaires par voie endo nasale à Abidjan. Méthode : Il s'agissait d'une étude rétrospective à visée analytique et descriptive, menée entre le 01 janvier 2010 et le 31 décembre 2020 et portant sur les patients admis au bloc opératoire pour une adénomectomie hypophysaire. Les caractéristiques sociodémographiques, cliniques, anesthésiques, évolutives et pronostiques ont été étudiées. Résultats : Nous avons recensé 102 patients. L'âge moyen était de 44,72±12,14 ans. Le sex ratio était de 1. Le délai de diagnostic était d'un an pour 46% (n = 47) des patients. L'hypertension artérielle était l'antécédent le plus retrouvé. Les principaux motifs de consultation étaient les céphalées et les troubles de l'acuité visuelle. Le syndrome d'hypertension intracrânien a été observé chez 67,6% (n= 69) des patients. Tous les patients ont bénéficié d'une consultation pré anesthésique. Cinquante-huit pourcent (n=59) des patients ont été classés ASA I selon la société américaine d'anesthésie. L'anesthésie générale était le seul schéma anesthésique. La durée de l'anesthésie était de plus de trois heures chez 57% (n=58) des patients et le réveil sur table a concerné 88,2% (n=90) des patients. La mortalité était de 3,9%. La durée de l'anesthésie supérieure à 6 heures (p= 0,0012 ; OR= 55,8 [4,88-637,33]) et la perte sanguine supérieure à 1000 ml (p = 0,0228 ; OR=18,6 2,152- 160,747]) ont constitué des facteurs de mauvais pronostic (p<0,05). Conclusion: La réduction de la létalité au cours de l'anesthésie pour adénomes hypophysaires passe par la lutte contre les facteurs de mauvais pronostics

Nariz en silla de montar poscirugía transesfenoidal de hipófisis. Reconstrucción con cartílago costal / Saddle nose after transsphenoidal pituitary surgery. Reconstruction with rib cartilage

Durante los últimos años, la cirugía endoscópica transesfenoidal se ha convertido en el procedimiento quirúrgico de elección para el tratamiento de tumores hipofisarios. La técnica de abordaje actual es el resultado de una evolución histórica de vía por craneotomía a vía endonasal con ingreso a través del seno esfenoidal. Aunque la cirugía de tumores hipofisarios vía transesfenoidal endoscópica ha permitido disminuir las complicaciones graves asociadas a los abordajes externos, no está exenta de complicaciones, como la fístula de líquido cefalorraquídeo. A nivel del abordaje nasal, hay escasas descripciones de complicaciones y el compromiso del cartílago septal con deformidad en silla de montar no es una complicación documentada. Se presenta un caso de rinodeformidad en silla de montar poscirugía transesfenoidal de hipófisis en una paciente de 32 años a quien se le practicó una reconstrucción nasal con cartílago costal autólogo. En nuestra búsqueda bibliográfica es el primer caso registrado en Iberolatinoamérica

In recent years, transsphenoidal endoscopic surgery has become the surgical procedure of choice for the treatment of pituitary tumors. The current approach technique is the result of an historical evolution from craniotomy surgery to an endonasal procedure with entry through the sphenoid sinus. Although endoscopic transsphenoidal surgery has made it possible to reduce serious complications associated with external approaches, it is not without complications, such as cerebrospinal fluid fistula. At the level of the nasal approach, there are few descriptions of complications and involvement of the septal cartilage with saddle deformity is not a documented complication. We present a case of saddle rhinodeformity after transsphenoidal pituitary surgery in a 32-year-old patient who underwent nasal reconstruction with autologous costal cartilage. In our bibliographic search, it is the first case registered in IberoLatin America.

Proteína moduladora de la actividad del receptor de aril hidrocarburos (AIP): genética, bioquímica e impacto clínico / Aryl hydrocarbon receptor interacting protein (AIP): genetics, biochemistry and clinical impact

El gen AIP (proteína moduladora de la actividad del receptor de aril hidrocarburos) se localiza en la región 11q13.2 y codifica para una proteína de 330 aminoácidos que interactúa con el factor de transcripción AhR (receptor para aril hidrocarburos). Las mutaciones en este gen se han asociado con adenomas pituitarios aislados de tipo familiar (APAF). Se caracterizan por una presentación temprana (alrededor de 20 años), por lo regular producen hormona de crecimiento y/o prolactina, tienen un comportamiento clínico agresivo y poca respuesta a análogos de somatostatina.

The AIP gene (aryl hydrocarbon receptor interacting protein) is located on chromosome 11q13.2 and encodes a 330 amino acid protein which interacts with the aryl hydrocarbon receptor (AHR) transcription factor. Mutations in the AIP gene have been associated with familial isolated pituitary adenomas (FIPA). They characterize by an early-onset (around the age of 20 years old) and for being aggressive, growth hormone and/or prolactin-secreting tumors, with poor response to somatostatin analogues.

Two Cases of Hypovascular Pituitary Metastases and Literature Review / 中国医学科学院学报

Pituitary metastasis,a rare kind of intracranial malignant tumor,is characterized by metastasis from all parts of the body to the pituitary.The common tumors metastatic to pituitary are abundant with blood supply,located in the posterior pituitary lobe and/or the pituitary stalk.The lesion shows infiltrative growth,and mainly demonstrates low signal on T1 weighted imaging and high signal on T2 weighted imaging.It is usually enhanced significantly after iodinated contrast administration.The metastatic tumor with poor blood supply is similar to pituitary macroadenoma on magnetic resonance image,which makes it difficult to be differentiated.We reported two cases of pathologically diagnosed pituitary metastasis with poor blood supply and reviewed related papers,aiming to provide the imaging differentiation points of hypovascular pituitary metastasis.

Cryptorchidism is a useful clue for Idiopathic Hypogonadotropic Hypogonadism in Pituitary Stalk Thickening / Journal of the ASEAN Federation of Endocrine Societies

@#Pituitary stalk lesions can represent a wide range of pathologies. The exact cause is often unknown due to hesitancy to proceed with biopsy. We present a 16-year-old adolescent who presented with delayed puberty, short stature and bilateral cryptorchidism. He was found to have a thickened pituitary stalk of uncertain etiology with partial hypopituitarism (gonadotrophin and growth hormone deficiency) on further assessment. The presence of bilateral cryptorchidism and micropenis represents lack of “mini puberty,” a phenomenon of activation of the hypothalamic-pituitary-gonadal (HPG) axis in-utero or within the first few months of life.1 These key clinical features have been useful to establish an early temporal relationship and suggest a congenital origin of disease. This enabled a more conservative approach of surveillance to be employed as opposed to invasive pathological examination with pituitary stalk biopsy.

Sampling of the petrosal sinuses. Indications, results and usefulness / Muestreo de senos petrosos. Indicaciones, resultados y utilidad

Abstract Objectives: to analyze, evaluate and describe the usefulness of petrosal sinus sampling for diagnosing central Cushing's syndrome. Materials and methods: the technical aspects and results of bilateral venous sampling of the petrosal sinuses at the Hospital Universitario San Vicente de Paul in Medellín, Colombia, from January 1, 2012 to December 31, 2018, were analyzed. Results: the average age was 43.3 years, with a range from 19 to 69 years. Laterality could be shown in 68.2% of cases, with a tendency to be located on the left in 53.3%. The central source of ACTH production could be shown in 95.4% of cases, with a basal average central/peripheral ratio of 21.7, and 70.8 after stimulation. All samples at 3, 5 and 10 minutes were confirmatory following stimulation. Conclusion: in our retrospective study, petrosal sinus catheterization provided laboratory confirmation of the central source of ACTH production in a high percentage of patients, with no immediate complications.

Resumen Objetivos: analizar, evaluar y describir la utilidad del muestreo de senos petrosos para diagnóstico del síndrome de Cushing de origen central. Material y métodos: se analizaron los aspectos técnicos y resultados del muestreo bilateral venoso de senos petrosos, desde el 1° de enero de 2012 a 31 de diciembre de 2018 en el Hospital Universitario San Vicente de Paúl en Medellín, Colombia. Resultados: el promedio de edad fue 43.3 años con un rango de edad desde los 19 hasta los 69 años. La lateralidad pudo ser demostrada en 68.2% de los casos con una tendencia a la localización en el lado izquierdo en 53.3%. El origen central de producción de ACTH logró ser demostrado en 95.4% de los casos, con una relación central/periferia basal promedio de 21.7 y postestimulación de 70.8. Todas las muestras a los 3, 5 y 10 minutos fueron confirmatorias tras la estimulación. Conclusión: en nuestro estudio retrospectivo el cateterismo de senos petrosos confirmó la fuente central de producción de ACTH por laboratorio en un alto porcentaje de pacientes sin ninguna complicación inmediata.

Hipopituitarismo postraumatismo encefalocraneano: revisión de la literatura y algoritmo de estudio y abordaje terapéutico

Hypopituitarism after moderate or severe traumatic brain injury (TBI) is usually underdiagnosed and therefore undertreated. Its course can be divided in an acute phase during the first 14 days after TBI with 50 to 80% risk of hypopituitarism, and a chronic phase, beginning three months after the event, with a prevalence of hypopituitarism that ranges from 2 to 70%. Its pathophysiology has been addressed in several studies, suggesting that a vascular injury to the pituitary tissue is the most important mechanism during the acute phase, and an autoimmune one during chronic stages. In the acute phase, there are difficulties to correctly interpret pituitary axes. Hence, we propose a simple and cost-effective algorithm to detect and treat a potential hypothalamic-pituitary-adrenal axis impairment and alterations of sodium homeostasis, both of which can be life-threatening. In the chronic phase, post-concussion syndrome is the most important differential diagnosis. Given the high prevalence of hypopituitarism, we suggest that all pituitary axes should be assessed in all patients with moderate to severe TBI, between 3 to 6 months after the event, and then repeated at 12 months after trauma by a specialized team in pituitary disease.

Comparison of a combination test (1 µg ACTH test plus glucagon test) versus 1 µg ACTH test and glucagon test in the evaluation of the hypothalamic-pituitary-adrenal axis in patients with pituitary disorders

ABSTRACT Objective To investigate whether a combination of the low-dose (1 µg) adrenocorticotropin (ACTH) stimulation test and glucagon stimulation test (GST) could overcome the problem of equivocal results with the GST or ACTH test alone in patients with pituitary disorders. Subjects and methods The study included 41 adult patients with pituitary disorders and 20 healthy subjects who underwent evaluation of cortisol response to ACTH, GST, and a combination of both tests. Blood samples for cortisol measurement were obtained at baseline and 30, 60, 90, and 120 minutes after intravenous administration of ACTH 1 μg and 90, 120, 150, 180, 210, and 240 minutes after subcutaneous injection of glucagon 1 mg. The combination test was performed by injecting ACTH 1 µg at the 180-minute time point of the GST, with blood samples for cortisol measurement obtained at 210 and 240 minutes. Results Overall, 28 patients with normal cortisol response to both tests also had a normal cortisol response to the combination test. Ten patients with adrenal insufficiency in both tests also had adrenal insufficiency in the combination test, including a patient who had a peak cortisol value of 12.4 µg/dL (which is the cutoff value for the combination test). Two patients with adrenal insufficiency in the ACTH stimulation test and one patient with adrenal insufficiency in the GST had normal cortisol responses to the combination test. Conclusion By using an appropriate cutoff value, the combination test may offer additional information in patients with equivocal results in the GST and ACTH stimulation test.

Hipofisitis linfocitaria primaria. Caso clínico / Primary lymphocytic hypophysitis. Report of one case

Primary lymphocytic hypophysitis is an autoimmune disease characterized by lymphocytic infiltration of the pituitary gland, with a higher incidence during late pregnancy and the postpartum period. It causes glandular destruction, mass effect and symptoms such headache, visual field defects, ophthalmoplegia and symptoms of hypopituitarism. We report a 38-year-old postpartum woman who, after giving birth presented decreased left visual acuity associated with a non ictal headache. Magnetic resonance imaging demonstrated a sellar mass associated with decreased free thyroxine and cortisol levels. Suspecting a primary lymphocytic hypophysitis, she was treated with prednisone 60 mg/day and hormonal replacement therapy. One month later, size of the pituitary gland decreased, and the visual field defect improved. Steroidal treatment was maintained for 36 months and progressively tapered. After two years of follow-up, the imaging studies show a normal sized pituitary gland.

Quiste de la bolsa de Rathke / Rathke pouch cyst

Es una lesión quística que surge del remanente epitelial de la bolsa de Rathke, casi siempre su diagnóstico es un hallazgo incidental ya que en la mayoría de los casos es asintomático. Cuando se manifiesta se debe a que ha aumentado lo suficiente su volumen hasta comprimir estructuras vecinas causando cefalea, alteraciones visuales y disfunción pituitaria. En su mayoría ocurre en adultos entre la cuarta y quinta década de vida. Presentamos el caso de una paciente femenina de 9 años de edad que consultó por talla baja al servicio de endocrinología, por lo cual se inició tratamiento con hormona de crecimiento y se solicitó una resonancia magnética nuclear (RMN) la cual reportó quiste de la bolsa de Rathke versus adenoma hipofisario.

Rathke pouch cysts are epithelium-lined cysts arising from the embryological remnants of Rathke ́s pouch. They are usually incidentally identified since the majority are asymptomatic. They become symptomatic when they enlarge enough to compress neighbor structures causing headache, visual disturbances and pituitary dysfunction. They occur mostly in adults in the fourth to fifth decades of life. A case is presented in a 9-year-old female patient who consulted for growth retardation to the endocrinology service. She was treated with growth hormone and a magnetic resonance imaging (MRI) scan reported Rathke ́s pouch cyst versus pituitary adenoma.

Dome-shaped pituitary enlargement in primary hypothyroidism / Journal of the ASEAN Federation of Endocrine Societies

@#We describe three cases of primary hypothyroidism which presented initially to neurosurgery department with pituitary hyperplasia. We have found a novel pattern of ‘dome-shaped’ enlargement of pituitary in MRI of these patients. Out of these 3 patients, in two of them, the planned surgery was deferred when endocrinologists were consulted and the pituitary hyperplasia completely resolved with levothyroxine treatment. In the third case, pituitary surgery was already performed before endocrinology consultation and histopathology revealed thyrotroph hyperplasia. The hyperplastic lesions described typically have a homogenous symmetrical ‘dome’ shaped architecture unlike the non-functioning pituitary adenoma (NFPA), which usually might often be of varying shapes and homogeneity. Analysis of pituitary images from similar case reports published in literature, also showed this typical ‘dome’ shaped pituitary enlargement. This imaging characteristic can be a clue to look for underlying hormone deficiency, especially in primary hypothyroidism. Therefore, a thorough endocrine evaluation especially looking for primary hypothyroidism in such dome-shaped pituitary lesions are mandatory to prevent unwarranted neuro-surgical intervention as treatment of primary hypothyroidism may result in resolution of the abnormal enlargement.

Quiste de la bolsa de Rathke / Rathke pouch cyst

Es una lesión quística que surge del remanente epitelial de la bolsa de Rathke, casi siempre su diagnóstico es un hallazgo incidental ya que en la mayoría de los casos es asintomático. Cuando se manifiesta se debe a que ha aumentado lo suficiente su volumen hasta comprimir estructuras vecinas causando cefalea, alteraciones visuales y disfunción pituitaria. En su mayoría ocurre en adultos entre la cuarta y quinta década de vida. Presentamos el caso de una paciente femenina de 9 años de edad que consultó por talla baja al servicio de endocrinología, por lo cual se inició tratamiento con hormona de crecimiento y se solicitó una resonancia magnética nuclear (RMN) la cual reportó quiste de la bolsa de Rathke versus adenoma hipofisario.

Rathke pouch cysts are epithelium-lined cysts arising from the embryological remnants of Rathke ́s pouch. They are usually incidentally identified since the majority are asymptomatic. They become symptomatic when they enlarge enough to compress neighbor structures causing headache, visual disturbances and pituitary dysfunction. They occur mostly in adults in the fourth to fifth decades of life. A case is presented in a 9-year-old female patient who consulted for growth retardation to the endocrinology service. She was treated with growth hormone and a magnetic resonance imaging (MRI) scan reported Rathke ́s pouch cyst versus pituitary adenoma.

Lista de chequeo preoperatorio para la cirugía endoscópica de hipófisis / Preoperative checklist for endoscopic pituitary surgery

Introducción: las listas de chequeo son herramientas que buscan evitar errores y complicaciones al momento de realizar un procedimiento. El adenoma hipofisiario es la primera causa de cirugía endoscópica transnasal de la región selar, y se estima que ocurren hasta un 20 % de complicaciones derivadas de esta cirugía. Objetivo: desarrollar una lista de chequeo preoperatoria que sirva como una guía para la evaluación prequirúrgica de los pacientes a quienes se les realicen cirugías de la región selar. Metodología: se realizó una revisión de la literatura para desarrollar una lista de chequeo preliminar y una serie de declaraciones que resumían los puntos importantes de la lista. Se construyó un comité de expertos en cirugía endoscópica de la base de cráneo conformado por neurocirujanos y otorrinolaringólogos de hospitales de Bogotá. Utilizando la metodología Delphi modificada, se llegó a un consenso para la realización de la lista de chequeo final. Resultados: se describieron 15 declaraciones; de estas, hubo una discrepancia en 6 declaraciones. Por esta razón, se realizaron modificaciones en 5 declaraciones, y se llegó a un consenso entre los expertos participantes. La lista de chequeo final aprobada está compuesta por 4 ítems. Discusión/conclusiones: la cirugía para el manejo de la patología de la región selar es compleja y requiere de un equipo quirúrgico entrenado y disciplinado para lograr los mejores desenlaces posibles. Consideramos que nuestra lista de chequeo es una herramienta que permitirá a los equipos que realizan estas cirugías en Colombia tener una visión más completa del paciente y, eventualmente, ayudar a evitar errores y posibles complicaciones.

Introduction: checklists that seek to avoid errors are very useful tools for any surgical practice. Pituitary adenomas are the leading indication to perform a transnasal endoscopic surgery in the sellar region and up to 20 % complications have been reported related to this procedure. Objetive: develop a preoperative checklist, based on an expert consensus, to serve as a guide for the skull base teams to consistently evaluate preoperatively all patients with pathologies in the sellar region. Methods: a literature review was conducted to develop a preliminary checklist and a series of statements summarizing the most important items on the list. A committee of experts in endoscopic skull base surgery was summoned, made up of neurosurgeons and otolaryngologists from hospitals in Bogotá. Using the modified Delphi methodology, a consensus was reached for the completion of the final checklist. Results: fifteen statements were developed. A discrepancy was seen in six statements. Modifications were made for five statements, thus reaching a consensus among the participating experts. The final approved checklist is made up of 4 items. Conclusion: surgery for treating different pathologies in the sellar region is complex and requires a welltrained and disciplined surgical team to achieve the best possible outcomes. We consider that our preoperative checklist is a valuable resource for skull base surgical teams performing transnasal endoscopic surgery of the sellar region. This tool will allow skull base surgical teams in Colombia to have a more comprehensive view of the patient and eventually help to avoid errors and possible complications.

Síndromes de neoplasia endocrina múltiple: revisión radiológica / Multiple Endocrine Neoplasia Syndromes: Radiological Review

Los síndromes de neoplasia endocrina múltiple (MEN), incluyen una serie de enfermedades con alteraciones genéticas que se caracterizan por la presencia de tumores que afectan a dos o más glándulas endocrinas. Son síndromes con una herencia autosómica dominante e incluyen tres patrones: MEN 1 (síndrome de Wermer), MEN 2 (que incluye MEN 2A o síndrome de Sipple y MEN 2B o síndrome de Wagenmann-Froboese) y MEN 4. Los adenomas paratiroideos y el carcinoma medular tiroideo, son los tumores más frecuentes del MEN tipo 1 y 2 respectivamente. Esos síndromes son más comunes en pacientes jóvenes, con patología de afectación bilateral, múltiple o multifocal y, sobre todo, en pacientes con antecedentes familiares. Es necesario el trabajo en equipo de endocrinólogos, cirujanos, oncólogos y radiólogos para optimizar el tratamiento de esos pacientes.

Multiple endocrine neoplasia (MEN) encompasses a serial of familial genetically disorders in wich tumors simultaneusly occur in two or more endocrine organs. MEN síndromes are autosomal-dominant disorders categorized into three main patterns: MEN 1 (Wermer syndrome), MEN 2 (includes MEN 2A o Sipple syndrome and MEN 2B o Wagenmann-Froboese syndrome) and MEN 4. Parathyroid adenomas and medullary thyroid carcinoma are the most frecuent tumors in MEN 1 and MEN 2 respectively. These entities will be suspected in younger patients, bilateral, multiple or multifocal disease and, specially, in patients with family background. Cooperation between endocrinologist, surgeons, oncologists and radiologists is pivotal for optimizing patient treatment.

Infundibuloneurohipofisitis: caso clínico y revisión de la literatura / Infundibuloneurohypophysitis: clinical report and literature review

Infundibuloneurohypophysitis is a rare condition, which is part of the group of hypophysitis, of relatively recent description (1993). The main clinical manifestation is diabetes insipidus, whose natural evolution is towards chronicity. The differential diagnosis with other thickening of the hypophysial stem is very important, where the clinic, imaging, laboratory and eventually biopsy are a main support for a correct diagnosis. We present a clinical case that shows the usual picture of infundibuloneurohypophysitis, and illustrates the imaging evolution in a female patient, with diabetes insipidus as the main clinical manifestation

Histopathological features of post-mortem pituitaries: A retrospective analysis / Características histopatológicas de hipófises post mortem: análise retrospectiva

SUMMARY Objective: As a result of the use of neuroimaging techniques, silent pituitary lesions are diagnosed more and more frequently; however, there are few published post-mortem studies about this gland. Incidence data of pituitary lesions are rare and in Portugal they are outdated or even non-existent. The aim of this study is to determine the prevalence of normal patterns and incidental post-mortem pituitary pathology at Centro Hospitalar Lisboa Norte, analyzing the associations with clinical data and assessing the clinical relevance of the findings. Method: We reviewed retrospectively and histologically 167 pituitaries of a consecutive series of autopsies from the Department of Pathology of this centre. They were done between 2012 and 2014, and in all cases medical records were reviewed. The morphological patterns observed, were classified into three major groups: 1) Normal histological patterns and variants; 2) Infectious-inflammatory pathology, metabolic and vascular disorders; 3) Incidental primary proliferation and secondary to systemic diseases. Results: The subjects included in this study were of all age groups (from 1 day to 91 years old), 71 were female and 96 male. Fifty-seven of these glands didn’t show any alteration; 51 showed colloid cysts arising from Rathke cleft; 44 presented hyperplasia in adenohypophysis and we identified 20 adenomas in 19 glands (immunohistochemically, eight PRL-producing and five ACTH-producing tumors), ten of which associated with obesity, 11 to hypertension and six to diabetes mellitus. There were two cases with metastasis. Conclusion: Subclinical pathology in our country is similar to that seen in other parts of the world, but at older ages.

RESUMO Objetivo: como resultado da utilização de técnicas de neuroimagem, cada vez se diagnosticam mais lesões hipofisárias silentes; porém, há poucos estudos post mortem publicados sobre essa glândula. Os dados de incidência existentes sobre lesões hipofisárias são raros, sendo em Portugal desatualizados ou inexistentes. O objetivo é determinar a prevalência dos padrões normais e da patologia hipofisária incidental post mortem no Centro Hospitalar Lisboa Norte, analisando as associações com dados clínicos e avaliando a relevância clínica dos achados. Método: revisaram-se histologicamente de forma retrospectiva 167 hipófises de uma série consecutiva de autópsias do Serviço de Anatomia Patológica desse centro, realizadas entre 2012 e 2014, sendo revisadas em todos os casos as histórias clínicas. Os padrões morfológicos observados classificaram-se em três grandes grupos: 1) padrões histológicos de normalidade e variantes; 2) patologia infeccioso-inflamatória, distúrbios metabólicos e transtornos vasculares; 3) proliferação primária incidental e secundária a doenças sistêmicas. Resultados: os doentes incluíam todas as faixas etárias (de 1 dia a 91 anos), sendo 71 do sexo feminino e 96 do masculino. Cinquenta e sete das glândulas não apresentaram qualquer alteração; 51 mostraram cistos coloides derivados da fissura de Rathke; em 44, observou-se hiperplasia da adeno-hipófise e identificaram-se 20 adenomas em 19 glândulas (oito imuno-histoquimicamente produtores de PRL e cinco de ACTH), dos quais dez associados à obesidade, 11 à hipertensão arterial e seis a diabetes mellitus. Houve dois casos com metástases. Conclusão: a patologia subclínica em nosso meio é similar à observada em outras partes do mundo, mas em idades mais avançadas.

Correlation between Pituitary Stalk Interruption Syndrome and Prokineticin Receptor 2 and Prokineticin 2 Mutations / 中国医学科学院学报

<p><b>OBJECTIVE</b>To analyze the correlation between pituitary stalk interruption syndrome (PSIS) and prokineticin receptor 2 (PROKR2) and prokineticin 2 (RROK2) mutations.</p><p><b>METHODS</b>PROKR2 and RROK2 genotypes were identified by multiplex polymerase chain reaction analysis with exon-flanking primers and by automated sequencing techniques with peripheral blood DNA samples from 59 patients with PSIS.</p><p><b>RESULTS</b>Of these 59 PSIS patients, 6 showed intragenic deletions at the PROKR2 locus. Of them, 5 patients exhibited intragenic subsititution of exon 2 (c.991G>A), and the remaining one patient exhibited intragenic subsititution of exon 2 (c.1057C>T). No PROK2 mutation was found in these PSIS patients.</p><p><b>CONCLUSION</b>PROKR2 may be the susceptibility gene of PSIS.</p>

Clinical Features of Pituitary Stalk Interruption Syndrome in 114 Cases / 中国医学科学院学报

Objective To analyze the clinical characteristics of pituitary stalk interruption syndrome(PSIS). Methods The clinical data including clinical manifestations,laboratory tests,and imaging findings of 114 PSIS patients in our hospital were retrospectively analyzed. Results Of these 114 PSIS patients,102 cases (89.4%) were male. The average age was 21.1?6.1 years. A history of breech delivery was documented in 91 cases (91.9%). Short stature was found in 89 cases (71.8%) and bone age delayed (6.1?5.1) years. Secondary sex characteristics were poor or undeveloped in most patients. The prevalence of deficiencies in growth hormone,gonadotropins,corticotropin,and thyrotropin were 100.0%,94.0%,84.2%,and 74.6%,respectively. Hyperprolactinemia was found in 28.1% of patients. Three or more pituitary hormone abnormalities were found in 105 cases(92.1%). Compared with the 5 cases with history of cephalic delivery,no difference were found in the aspects of height(t=0.297,P=0.634),penile length(t=1.205,P=0.882),testicular volume (U=99.000,P=0.348),growth hormone peak (U=89.000,P=0.186),adrenocorticotropic hormone peak(U=131.000,P=0.967),luteinizing hormone peak(U=98.500,P=0.582),thyroid-stimulating hormone (U=82.000,P=0.162),and the height of anterior pituitary (t=1.676,P=0.107) in the 53 cases with history of breech delivery. Conclusions The clinical manifestations,symptoms,hormone deficiencies were severe in our series. The condition severities were not remarkably different in patients with different delivery ways.

Unusual presentation of Sheehan's syndrome with severe hyponatremia and recurrent symptomatic hypoglycemia: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#<p style="text-align: justify;">Sheehan's syndrome (SS) is postpartum hypopituitarism caused by necrosis of the pituitary gland. The onset in most cases is several months or even years after the inciting delivery, so it is often unrecognized and not adequately treated. Because SS often evolves slowly, it is usually diagnosed late. We report a 47-year old woman with loss of consciousness. Fourteen years ago, she had postpartum hemorrhage with subsequent amenorrhea and failure to lactate. Laboratory investigation showed low blood sugar and serum sodium levels, amid normal cortisol and thyroid function tests. Magnetic resonance imaging (MRI) of the pituitary revealed an empty sella consistent with SS. The presentation of hypoglycemia and hyponatremia are less known complications of Sheehan's syndrome with only a few documented in case reports.</p>