Embolia de líquido amniótico asociada a paro cardiorrespiratorio recuperado y síndrome de Sheehan / Amniotic fluid embolism associated with recovered cardiac arrest and postpartum Sheehan's syndrome

Resumen La embolia de líquido amniótico es una condición catastrófica propia del embarazo que ocurre típicamente durante el parto o justo posterior a este, cuyo sustrato fisiopatológico no ha sido aclarado por completo. Se ha estimado, según cifras de los Estados Unidos, que su incidencia rondaría 1 por cada 12.953 partos, y en el Reino Unido 1 por cada 50.000 partos; sin embargo, estas cifras pueden ser imprecisas debido a que no existen una referencia ni un consenso respecto a los criterios diagnósticos, además de que el cuadro clínico se puede confundir con otras emergencias obstétricas. Se presenta el caso de una paciente sin antecedentes mórbidos que presenta un cuadro de embolia de líquido amniótico no fatal, caracterizado por un estado fetal no tranquilizador durante la inducción del trabajo de parto, seguido de un paro cardiorrespiratorio durante la cesárea de urgencia y la rápida y catastrófica aparición de signos clínicos de una coagulopatía de consumo grave. Se describen además las complicaciones posoperatorias y su manejo, entre ellas un síndrome de Sheehan y la aparición de convulsiones tónico-clónicas generalizadas con alteración de neuroimágenes.

Abstract Amniotic fluid embolism is a catastrophic pregnancy condition that typically occurs during or inmediately after delivery, and whose pathophysiological background has not been fully clarified. According to US records the incidence of amniotic fluid embolism could been around 1 for every 12,953 births and in the United Kingdom 1 for every 50,000 births, however these numbers may be imprecise because there is no gold standard as well as no consensus regarding the diagnostic criteria, in addition that the clinical presentation can be misdiagnosis with other obstetric emergencies. We present the clinical case of a patient without a morbid history who presents with a non-fatal amniotic fluid embolism, characterized by an non-reassuring fetal status during labor induction, followed by cardiorespiratory arrest during emergency cesarean section and the rapid and catastrophic appearance of clinical signs of a severe consumptive coagulopathy. Postoperative complications and their management are also described, including Sheehans syndrome and the appearance of generalized tonic-clonic seizures with impaired neuroimaging.

Adenohipopituitarismo de rápida instalación, sin correlato imagenológico / Acute onset adenohypopituitarism without an image diagnosis

We report two cases of acute onset of adenohypopituitarism without a sellar MRI finding. The first case is a postmenopause woman complaining of fatigue, weakness, nausea, vomiting, diarrhea and mild weight loss. She was extensively studied with upper gastrointestinal endoscopy, colonoscopy and abdominal CT. An incidental possible pituitary enlargement on a Brain CT opened a pituitary function study, revealing adenohypopituitarism. The sellar MRI was perfectly normal, without anatomical explanation. The second case is a postmenopause woman complaining of fatigue and weakness, who had an episode of syncope and concomitant hyponatremia. Her study revealed adenohypopituitarism and a primary empty sella image in the MRI. The clinical problem of adenohypopituitarism without an image diagnosis brings the ethical dilemma to make a “blind” transsphenoidal biopsy or just treat them without a certain diagnosis. Patients with Empty Sella in the MRI show frequently normal pituitary function and it is not considered as a cause of so extensive hypopituitarism. Sometimes the clinical and image evolution can suggest the etiology and require of histological sample, so it is rational to keep an active surveillance and repeat the functional tests and Sellar MRI within the follow up...

Sheehan's syndrome presenting as psychosis: a rare clinical presentation

Sheehan's syndrome [SS] refers to the occurrence of varying degree of hypopituitarism after parturition [1]. It is a rare cause of hypopituitarism in developed countries owing to advances in obstetric care and its frequency is decreasing worldwide. However, it is still frequent in underdeveloped and developing countries. Sheehan's syndrome is often diagnosed late as it evolves slowly [2, 3]. Reports of psychoses in patients with Sheehan's syndrome are rare. Herein, a case report of psychosis in a 31 year old woman who developed Sheehan's syndrome preceded by postpartum haemorrhage is presented. Treatment with thyroxine and glucocorticoids resulted in complete remission after attaining euthyroid and eucortisolemic state

Hiponatremia sintomática como forma de presentación clínica de un macroadenoma hipofisiario: Comunicación de tres casos / Hyponatremia associated to pituitary adenomas: Report of three patients

Severe hyponatremia occurring as the presenting feature of hypopituitarism secondary to pituitary adenomas is rare. We report three patients with this condition: Two elderly males (74 and 78 year-old) presenting with impaired consciousness and low plasma sodium after an episode of diarrhea and a 56-year-old male presenting with impaired consciousness after an episode of vomiting. All had clinical features of hypopituitarism and pituitary adenomas were found on imaging studies. Two were subjected to a trans sphenoidal resection of the adenoma.

La corrección de la sustitución excesiva de cortisol mejora la morbilidad en pacientes con hipopituitarismo: un estudio piloto / Correction of Cortisol Overreplacement Ameliorates Morbidities in Patients with Hypopituitarism: A pilot study

Context: Hyporituitarism in adults is known to be associated with deleterious effects on body composition, lipid profile and quality of life (QoL). This was attributed to GH deficiency. The potential role of glucocorticoid overreplacement had never been investigated. Objective: To investigate whether reduction in glucocorticoid replacement dose to more physiological one could ameliorate the "AO-GHD"-attributed symptomatology in patients with hypopituitarism. Design: Eleven patients with panhypopituitarism taking 20-30 mg/day of hydrocortisone, but on no GH replacement were switched to 10 to 15 mg of hydrocortisone daily. Both basally and 6-12 months later, their body mass index, body composition by dual-energy x-ray absorptiometry, lipid profile, and the score of quality of life, QOL-AGHDA were measured. Results: Within 6-12 months of lower hydrocortisone dose, subjects lost an average of 7.1 kg of total body fat and 4.1 kg of abdominal fat. No changes were seen in lean body mass, bone mineral content and HOMA-IR Plasma total cholesterol and triglyceride concentrations decreased significantly (<0.05) and the QoL improved (p=0.018). Conclusions: Our pilot study suggests that decreasing the glucocorticoid replacement dose to ~ 15 mg/ day is beneficial in terms of patients' body composition, lipid profile and quality of life.

Avaliação do crescimento craniofacial e das extremidades de pacientes com deficiência de hormônio de crescimento ou síndrome de Turner em tratamento prolongado com hormônio de crescimento / Craniofacial and extremities growth evaluation of patients with GH deficiency or Turner syndrome during long-term growth hormone treatment

Objetivo: estudo prospectivo para avaliar atuação do hormônio de crescimento (GH) sobre o crescimento craniofacial de pacientes com deficiência de GH ou síndrome de Turner e o possível desenvolvimento de traços acromegálicos. Avaliamos 30 pacientes agrupados de acordo com diagnóstico e uso de GH. Realizamos medidas antropométricas (altura, pés e mãos) e avaliação ortodôntica (clínica, facial, radiográfica e cefalométrica) por no mínimo 3 anos. Observamos atuação significante do GH na base posterior do crânio e mandíbula. Todos os pacientes mantiveram o mesmo padrão de crescimento facial. Ocorreu melhora da harmonia facial em 28 por cento dos pacientes retrognatas; nenhum paciente desenvolveu desproporções ou desarmonia facial, no entanto, observamos aumento das extremidades, principalmente dos pés.

Our objective was to carry out a prospective study to evaluate the action of growth hormone (GH) on the craniofacial growth of patients with GH deficiency or Turner syndrome and the possible development of acromegalic features. We evaluated 30 patients classified in 3 groups based on diagnose and GH use. We realized anthropometrical (height, hands and feet) measurements and orthodontics analyses (clinical, facial, radiographic and cephalometric). We observed a greater GH effect on the posterior skull base and lower jaw; all patients kept their craniofacial growth pattern during the follow-up; there was an improvement in the facial harmony in 28 per cent of the retrognathic patients, no patient developed facial disharmony, however we observed an increase of the extremities, mainly of the feet.

[Pituitary deficiency of elderly subject: analysis of 14 cases]

The pituitary deficiency [PD] of the old subject is rare. To study the clinical biological etiologic and therapeutic characteristicsof PD in old subject. We report in this work a retrospective study of 14 patients older than 65 years presenting pituitary deficiency. This PD is characterized by a female preponderance [78, 5%of cases] and insidious initial symptomatology dominated by signs of thyreotrope deficiency and with a less degree of the signs of corticotrope sufficiency Anaemia [57, 5%] hypoglycaemia [28, 5%] and hyponatremia [21, 4%] are the most frequent biological signs. Hormonal exploration showed a dissociated pituitaiy deficiency in 11 cases [782 and global in 3 cases [21, 5%] The axis more touched is the gonadotrope axis [85, 7%] followed by the corticotrope [71, 4%] and thyreotrope axis [71, 4%]. The aetiologies are dominated by the Sheehan syndrome in 5 cases [35, 7%] followed by the hypophysis adenoma m 4 case [21, 4%]. The pituitary deficiency was idiopathic probably of vascular ongin in 2 cases [14, 28%] and secondary to primitive turcic saddle in 1 cases to a cyst of the pocket of Rathke in I case and to prolonged corticotherapy in 1 case. The hormonal substitutive treatment hare per-miffed a spectacular improvement in all the cases. It is necessary to think about the PD in the old subjects in front of minor signs and at the vascular ongin of this one witch remains the most frequent aetiology at the old subject especially those presenting atherogens factors

[Sheehan syndrom [of 40 cases]]

We proposed to undertake a study on the epidemiology, clinical, hormonal and evaluative profiles of 40 observations of antehypophysary failure of Sheehan postpotrum-syndrom assessed between 1980 and 1994. The occurance of hemorrhagic delivery is relatively early [29 years average], but the diagnosis was done 7years and half later [ranges going from 2 to 20 years]. Child delivery took place at home in 92% cases. The abondant hemorrhagy was found in 38 patients. The absence of this hemorrhagy in 2 of our patients leads to the hypothesis of lymphocytary hypophysite of poetpaetum. In case of the immediate post-partum the agalactia and the absence of recouvering are constant signs. In the mouths following the child-delivery, the clinical report of SHEEHAN syndrome did not show any particularities, compared to the classic form. In the absence of a dynamic hormonal explorations, we can not discuss neither the importance of hormonal deficiency, norther their repartition. The short term development under substitutive treatment, in good with ponderal advantage and with resumption of normal physical activities. At long term, an adherence failure to treatment was noticed in 6 patients

Hipoglicemia secundaria a hipopituitarismo congénito / Hypoglycemia secundary a congenital hipopituitarism

La hipoglicemia es la manifestación inicial de los pacientes con hipopituitarismo congénito, especialmente en el período neonatal, en la mayoría de los casos se asocia a defectos en la línea media, sin déficid pondoestatural al momento del nacimiento, ni en los primeros meses de vida. Se presenta el caso de lactante mayor con hipoglicemia y convulsiones generalizadas, sin alteraciones al examen físico. El perfil endocrinológico reportó insuficiencia hipofisiaria

Reposiçäo de hormônio de crescimento recombinante em adultos com déficit após cirurgia hipofisária / Recombinant growth hormone replacement in adults with deficiency after pituitary surgery

a deficiência hormonal mais comumente identificada em associaçäo com doenças da regiäo hipotálamo-hipofisária ou de sua terapia é a do hormônio do crescimento (GH), já tendo sido reconhecida uma síndrome clínica específica no adulto. A deficiência de GH está associada com um número de mudanças na composiçäo corporal, diminuiçäo na densidade mineral óssea (BMD), hipercolesterolemia, reduçäo na massa miocárdica ventricular, diminuiçäo na funçäo sistólica, distúrbios psicológicos, e diminuiçäo na taxa de filtraçäo glomerular e fluxo plasmático renal. O diagnóstico é realizado através de dosagens basais de insulin like growth factor (IGF-I) e proteína ligadora de IGF-I tipo 3 9IGFBP-3), além de testes de estímulo de liberaçäo de GH, sendo o da insulina e clonidina os mais utilizados. Com relaçäo ao tratamento, a dose utilizada varia entre 0,012 e 0,036 UI/Kg/dia, observando-se acentuada melhora dos sintomas e sinais; os efeitos adversos mais comuns säo a retençäo hídrica (edema, síndrome do túnel do carpo), astralgias e mialgias. Estudos prospectivos realizados em grupos maiores de pacientes säo necessários para confirmar se a reposiçäo de GH melhora a mortalidade e morbidade em adultos com deficiência de GH.

Hipopituitarismo originado por metastasis de carcinoma de colon asociado con aspergilosis hipofisaria / Hypopituitarism originating to colonic carcinoma metastasis associated with hypophysial aspergillosis

Las metástasis hipofisiarias constituyen el 1 al 8,3 por ciento del total de tumores metastásicos cerebrales. La localización más frecuente es en el lóbulo posterior y la diabetes insípida puede ser el único síntoma de disfunción. La aspergilosis cerebral es una enfermedad inusual y ha sido descripta complicando una malignidad preexistente, o posteriormente a una cirugía intracraneal. Describimos un hipopituitarismo e hiperprolactinemia en una paciente quien presentó metástasis hipofisarias de un carcinoma de colon y aspergilosis coexistente. Dos años antes, se le había resecado un adenocarcinoma de colon (clase Cl de Duke). Ningún signo clínico de hipopituitarismo o galactorrea estaban presentes. Los hallazgos de laboratorio reflejan déficit de corticotrofina (ACTH), hormona luteinizante (LH), hormona folículo estimulante (FSH), y una leve hiperprolactinemia (PRL). Una resonancia magnética cerebral reveló una massa intra y supraselar la cual se extendía a hipotálamo. La radiografía de tórax y la tomografía computada confirmó una massa macronodular en el segmento apical del lóbulo pulmonar inferior izquierdo con nódulos hipertróficos meidastinales. Fue diagnosticado un tumor hipofisario no funcionante realizándose cirugía transfenoidal. El examen microscópico confirmó una proliferación maligna que sugería diferenciación colónica. Fragmentos de tejido ipofisário tumoral mostraron hifas de aspergilus formando abscesos. La aspergilosis como complicación de enfermedades neoplásicas, es mayor en leucemias y linfomas que en tumores sólidos. El diagnóstico de aspergilosis del SNC es dificultoso, siendo éste generalmente confirmado durante la necropsia. Esta localización puede aparecer como parte de una aspergilosis diseminada, o como una infección única del SNC, aunque la mayoría de los casos está asociada a una aspergilosis pulmonar. Hasta la fecha, no hemos encontrado publicada la asociación de hipopituitarismo y moderada hiperprolactinemia cuya etiología fue metástasis hipofisaria de carcinoma de colon y aspergilosis coexistente