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.

Crisis adrenal asociada al uso de modafinilo / Adrenal crisis associated with modafinil use

Resumen La crisis adrenal es la forma más extrema de presentación de la insuficiencia adrenal y representa una urgencia endocrinológica que llega a poner en riesgo la vida. Esta situación puede ser des encadenada por diferentes causas, entre las cuales se incluye el uso de fármacos inductores del CYP3A4, que aceleran la depuración de la hidrocortisona. Describimos el caso de una mujer de 85 años, con antecedentes de insuficiencia adrenal secundaria y enfermedad renal crónica, que presentó síntomas compatibles con crisis adrenal (astenia, adinamia, hiponatremia grave con síntomas neurológicos e hipotensión arterial) luego de nueve días del inicio de tratamiento con modafinilo. El cuadro clínico mejoró rápidamente con la suspensión del modafinilo y la administración de hidrocortisona endovenosa. Luego de descartar las posibles causas desencadenantes (infecciosas, isquémicas, tromboembolismo pulmonar y omisión en la toma de hidrocortisona), se interpretó que el modafinilo precipitó los síntomas de insuficiencia adrenal al aumentar la depuración del corticoide. El modafinilo tiene la capacidad de inducir la actividad del CYP3A4 y, en consecuencia, disminuir la biodisponibilidad de la hidrocortisona. Recalcamos la necesidad de ajustar la dosis de reemplazo de corticoides en sujetos que reciben fármacos inductores del metabolismo.

Abstract Adrenal crisis is the most extreme presentation form of adrenal insufficiency and represents a life-threatening endocrinological emergency. This situation can be triggered by different causes including the use of CYP3A4-inducing drugs, which accelerate hydrocortisone clearance. We describe the case of an 85-year-old woman with secondary adrenal insufficiency and chronic renal disease, who presented symptoms compatible with adrenal crisis (asthenia, adynamia, severe hyponatremia associated with neurological symptoms and hypotension) nine days after the start of modafinil treat ment. The clinical picture improved rapidly with the suspension of modafinil and the administration of intravenous hydrocortisone. After ruling out the possible triggering causes (infectious, ischemic, pulmonary thromboembo lism and failure to take hydrocortisone), it was interpreted that modafinil precipitated the symptoms of adrenal insufficiency by increasing the steroid clearance. Modafinil has the ability to induce the activity of CYP3A4 and consequently decrease the bioavailability of hydrocortisone. We emphasize the need to adjust steroid dose re placement in subjects receiving metabolism-inducing drugs.

Sheehan's syndrome with central diabetes insipidus / Síndrome de Sheehan e diabetes insípido central

Sheehan's syndrome refers to the occurrence of hypopituitarism after delivery, usually preceded by postpartum hemorrhage. The condition still continues to be a common cause of hypopituitarism in developing countries like India. The disorder usually presents with anterior pituitary failure with preservation of posterior pituitary functions. Posterior pituitary dysfunction in the form of central diabetes insipidus is rare in patients with Sheehan's syndrome. We describe the clinical course of a young lady who after her sixth childbirth developed severe postpartum hemorrhage followed by development of panhypopituitarism which was confirmed by hormonal investigation and demonstration of empty sella on imaging. In addition, she developed Polyuria. The water deprivation test and response to vasopressin test results indicated central diabetes insipidus. She needed oral desmopressin on a continuous basis to control polyuria.

A síndrome de Sheehan está relacionada à ocorrência de hipopituitarismo pós-parto, geralmente precedido por hemorragia pós-parto. Essa condição clínica ainda constitui causa comum do hipopituitarismo observado em países em desenvolvimento como a Índia. Essa síndrome se caracteriza pela insuficiência da glândula hipofisária anterior, porém com a conservação das funções da glândula hipofisária posterior. A disfunção da hipófise posterior, sob a forma de diabetes insipidus central, é algo raramente observado em pacientes que apresentam a síndrome de Sheehan. Neste artigo, descrevemos o caso de uma jovem que, após o sexto parto, apresentou hemorragia pós-parto grave, seguida pela evolução de pan-hipopituitarismo que foi confirmado por pesquisa hormonal e exames de imagem que evidenciaram sela vazia. A jovem também apresentou poliúria. Os resultados do teste de privação de água e exame de resposta à vasopressina indicaram diabetes insípido central. A paciente fazia uso contínuo de desmopressina para controlar a poliúria.

Síndrome de Sheehan: descripción de un caso clínico y revisión de la literatura / Sheehan’s syndrome: a case report and literature review

Introducción: la necrosis hipofisaria posparto o síndrome de Sheehan se presenta secundaria a una hemorragia obstétrica, ocasionando un colapso circulatorio intenso el cual, a su vez, predispone a la isquemia de la hipófisis aumentada de tamaño. Objetivo: presentar un caso clínico de una paciente quien consultó por manifestaciones tardías de este síndrome y hacer una revisión de la literatura. Metodología: se buscó información en la bases de datos de PubMed/MEDLINE, Cochrane y SciELO; así como en referencias de artículos de revista y textos principalmente de los últimos cinco años. Seguidamente, se tomaron los siete artículos más relevantes según la calificación de la revista en donde fueron publicados. Resultados: la característica clínica del síndrome de Sheehan que se manifiesta con mayor frecuencia es la incapacidad para lactar debido a una producción insuficiente de prolactina. Otras manifestaciones tardías incluyen: secreción inadecuada de la hormona antidiurética y deficiencias en la secreción de cortisol, de hormona tiroidea y de gonadotrofinas. Es importante que el obstetra y el médico general sepan reconocer este síndrome, ya que la pérdida aguda del funcionamiento de la adenohipófisis puede ser de mal pronóstico si no se realiza un reemplazo hormonal adecuado. Además, el buen control hormonal evita o disminuye las complicaciones metabólicas y cardiovasculares. Conclusión: el síndrome de Sheehan es una enfermedad poco frecuente, la cual es difícil de diagnosticar tempranamente. Asimismo, requiere una terapia de reemplazo hormonal con controles permanentes clínicos y paraclínicos.

Introduction: postpartum pituitary necrosis, or Sheehan’s syndrome, occurs following obstetric haemorrhage which causes intense circulatory collapse, thereby predisposing pituitary ischemia leading to this organ becoming enlarged (the posterior pituitary is generally affected). This document presents a clinical case of a patient who consulted due to late manifestations of this syndrome. A literature review is also made. Objective: presenting the case of a patient who consulted for late manifestations of Sheehan’s syndrome and reviewing the pertinent literature. Methodology: information was sought in the PubMed / Medline, Cochrane, SciELO databases and references from articles in journals and texts (mainly from the last five years) were also taken into account. The seven most relevant articles were taken according to the impact of the journal in which they were published. Results: the most frequently found clinical characteristic was an inability to lactate due to insufficient prolactin production. The most common clinical feature found was an inability to breastfeed due to insufficient prolactin production. Other manifestations included inappropriate antidiuretic hormone secretion and cortisol, thyroid hormone and gonadotropin secretion deficiencies. Obstetricians and GPs should be able to recognise this syndrome and know about its management as the acute loss of adenohypophysis functioning may have a poor prognosis without timely and suitable hormone replacement. Good hormone control prevents or decreases metabolic and cardiovascular complications. Conclusion: this is a rare syndrome in which early diagnosis is difficult and requires hormone replacement therapy with ongoing clinical and laboratory controls.

Aspectos diagnósticos e terapêuticos da síndrome de Sheehan: [revisão] / Clinical, laboratorial and therapeutics aspects of Sheehan's syndrome: [review]

A síndrome de Sheehan se caracteriza pelo hipopituitarismo pós-parto secundário à necrose hipofisária decorrente de hipotensão ou choque em virtude de hemorragia maciça durante ou logo após o parto. Sua freqüência vem caindo em todo o mundo, principalmente em países e regiões mais desenvolvidas em razão da melhora nos cuidados obstétricos, contudo, ainda é freqüente em países em desenvolvimento onde os cuidados obstétricos são mais precários. A síndrome de Sheehan pode evoluir de maneira lenta com diagnóstico muitas vezes tardio e, ainda que alguns sinais de insuficiência hipofisária ocorram, logo após o parto, são pouco valorizados. Seu diagnóstico precoce e o tratamento adequado são importantes para redução da morbimortalidade das pacientes. Esta revisão tem por objetivo descrever aspectos clínicos, laboratoriais e terapêuticos da síndrome de Sheehan, incluindo a nossa experiência pessoal na reposição com GH recombinante neste grupo de pacientes.

Sheehan's syndrome is characterized by hypopituitarism that occurs as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. Nowadays it is not usually seen in developed countries because of the improvements in obstetric care. However, in developing countries it is still frequent and probably one of the most common causes of hypopituitarism. Most patients usually present it months to years later, with a history of failure of postpartum lactation, failure to resume menses and other signs of panhypopituitarism. In mild forms of the disease, patients may remain undetected and do not receive treatment for many years. Early diagnosis and appropriate treatment are important to reduce the morbimortality of the patients with Sheehan's syndrome. The aim of this review is to describe clinical, laboratorial and therapeutic aspects of Sheehan's syndrome, including our experience in the replacement of recombinant GH in these patients.

Diabetes insípida central poshemorragia masiva posparto / Central diabetes insipidus postpartum massive post-hemorrhage

La diabetes insípida central (DIC) transitoria o permanente puede ser debida a causas hereditarias y adquiridas. Entre las últimas, el compromiso de la irrigación pituitaria o hipoxia por hemorragia severa es una situación infrecuente. La manifestación clínica del hipopituitarismo es heterogéneo e imposible de predecir y la DIC acompaña habitualmente a la insuficiencia adenopituitaria, siendo las formas puras o selectivas excepcionales. Presentamos un caso de hipopituitarismo debido a hemorragia masiva postpartum en donde los signos guías fueron ausencia de secreción láctea en el puerperio y poliuria.(AU)

Transient or permanent diabetes insipidus (DI) may be hereditary or acquired. Impairment of the irrigation of the pituitary or hipoxia for severe hemorrhage is a uncommon situation. Symptoms of hypopituitarism are heterogeneous and impossible to predict, but DI frequently accompanies the insufficiency of anterior pituitary, being the selective or pure forms exceptional. We present a case of insufficiency pituitary due to massive hemorrhage postpartum in which the guide - signs were failure to lactate after parturition and poliuria(AU)

Panhipopituitarismo: presentación de un caso clínico / Panhypopituitarism: clinical case

El Panhipopituitarismo es el déficit de las hormonas producidas en la adenohipofisis, clínicamente evidente cuando se ha destruido el 70 a 75 de la glándula. Las manifestaciones dependen del tipo de hormona afectada, edad, causa y rapidez de inicio. El déficit hormonal es progresivo: comienza con disminución de la GH, luego de la FSH /LH, TSH y finalmente la ACTH. Una mujer de 63 años de edad ingresa a UCI del hospital Abel Gilbert, con cuadro clínico de estupor, sudoración profusa, despigmentación de la piel, pérdida del vello axilar y pubiano. Antecedentes obstétricos: histerectomía por sangrado profuso y sepsis posparto. Por los datos clínicos y de laboratorio concluimos que se trataba de un Síndrome de Sheehan.

The panhypopituitarism is a deficit of the hormones produced by the adenohypophysis this is clinically evident when the 75 to 70 of the gland is destroyed. The symptoms depend on the type of hormone affected, age, cause and onset. The hormonal deficiency is progressive: it starts with a decrease of GH then of FSH/LH, TSH and finally ACTH. We have a clinical case of 63 year old woman admitted to ICU of Able Gilbert Hospital with a clinical case of stupor, hyperhidrosis, depigmentation of the skin, loss of pubic and axillary hair. Has an obstetric history of hysterectomy post-operative hemorrhaging and sepsis. Due the symptoms and lab findings we conclude that the diagnosis is Sheehan Syndrome.