RESUMO
A 36-year-old female was admitted to the emergency department with a hypoglycemic shock of unknown origin. Initial diagnostic procedures could exclude diabetes mellitus, postprandial hypoglycemia, insulinoma, tumor, drug side effects, and exogenous insulin application. Detailed evaluation of the patient's history revealed that the patient had not been able to lactate after an uncomplicated pregnancy and delivery 3 years ago. Endocrinological evaluation showed a complete adrenocorticotropic insufficiency caused by a Sheehan syndrome. Concomitantly, an empty sella syndrome was visible in MRI. Even after an uncomplicated delivery and even with normal laboratory values, Sheehan-Syndrome can become manifest in a hypoglycemic shock due to hypopituitarism with insufficiency of the adrenocorticotropic axis.
Assuntos
Coma/diagnóstico , Coma/etiologia , Síndrome da Sela Vazia/complicações , Síndrome da Sela Vazia/diagnóstico , Hipoglicemia/diagnóstico , Hipoglicemia/etiologia , Hipopituitarismo/complicações , Hipopituitarismo/diagnóstico , Adulto , Coma/prevenção & controle , Diagnóstico Diferencial , Síndrome da Sela Vazia/terapia , Feminino , Humanos , Hipoglicemia/prevenção & controle , Hipopituitarismo/terapiaRESUMO
Hereditary haemorrhagic telangiectasia (HHT) is a genetic disorder characterized by epistaxis, telangiectasia and visceral vascular manifestations. It is associated with migraine with aura due to pulmonary arteriovenous malformations (pAVMs). The aim of the study was to evaluate headache prevalence in 106 consecutive HHT patients (67 female, 39 male, age 53.5 +/- 14.5 years) and age- and gender-matched controls. An extensive clinical work-up was performed and headache prevalence was determined. Lifetime prevalence of migraine was higher in HHT patients (39.6%) than in controls (19.8%) [P < 0.001, chi(2) = 12.17, odds ratio (OR) 3.0; 95% confidence interval 1.6 < OR < 5.7]. A positive association was confirmed between HHT patients with pAVMs and migraine with aura (38.5% vs. 10%). Furthermore, HHT patients without pAVMs had a higher prevalence of migraine without aura (11.5% vs. 26.3%; chi(2) = 11.85; d.f. = 2; P = 0.003). We speculate that pathophysiological mechanisms, including dysfunction of the transforming growth factor-beta pathways and resulting vascular changes, contribute to the higher prevalence of migraine without aura in HHT patients without pAVMs.
Assuntos
Malformações Arteriovenosas/complicações , Transtornos de Enxaqueca/complicações , Artéria Pulmonar/anormalidades , Veias Pulmonares/anormalidades , Telangiectasia Hemorrágica Hereditária/complicações , Malformações Arteriovenosas/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos de Enxaqueca/epidemiologia , Prevalência , Telangiectasia Hemorrágica Hereditária/epidemiologiaRESUMO
HISTORY AND CLINICAL FINDINGS: A 52-year-old man with liver cirrhosis and ascites, hepatorenal syndrome and severe pancytopenia was admitted to hospital for treatment and consideration for future liver transplantation. INVESTIGATIONS: Laboratory tests revealed severe leukocytopenia and thrombocytopenia, which had persisted after otherwise successful treatment of spontaneous bacterial peritonitis and a hepatorenal syndrome. There was no evidence of a toxic cause such as drugs or alcohol nor of an underlying haematologic disease. The most likely diagnosis was hypersplenism. DIAGNOSIS, THERAPY AND CLINICAL COURSE: Because of the high risk of a splenectomy it was decided to perform a partial splenic arterial embolization. Both leukocyte and thrombocyte counts rose after the intervention and continued to stay within normal range. This made it possible to discharge the patient from inpatient treatment until he was admitted again for a subsequently successful liver transplantation. CONCLUSIONS: Partial splenic artery embolization can be successfully used to treat severe leukocytopenia and thrombocytopenia in patients with liver cirrhosis and hypersplenism.