RESUMEN
In order to assess the possible effects of sleep states on cerebral haemodynamics in healthy term infants, we measured cerebral oxyhaemoglobin, deoxyhaemoglobin and total haemoglobin concentration using near infrared spectroscopy. Thirty-seven sleep state changes in seventeen infants (gestational age: 37 to 41 4/7 weeks), aged between two and eight days were continuously registrated during 1-3 h. Transcutaneous PaO2, PaCO2, arterial O2 saturation and heart rate were simultaneously recorded and sleep states were clinically defined. There was a close relationship between sleep state changes and changes in total cerebral haemoglobin concentration, which increased from active to quiet sleep and decreased from quiet to active sleep. Changes in total cerebral haemoglobin were due, in the most part, to changes in the cerebral oxyhaemoglobin concentration. In conclusion, sleep states influence the cerebral haemoglobin concentration. Studies on cerebral haemodynamics should take sleep state into account in term newborn infants.
Asunto(s)
Circulación Cerebrovascular/fisiología , Fases del Sueño/fisiología , Volumen Sanguíneo , Humanos , Recién Nacido , Espectroscopía Infrarroja CortaRESUMEN
We report on a patient with EEC/EECUT syndrome and concomitant hypoplasia of the thymus and reduction of T cells in secondary lymphatic organs. The patient was born prematurely at 35 weeks of gestational age and exhibited ectodermal dysplasia, ectrodactyly, cleft palate and urinary tract abnormalities. On the left side, a large ureterocele was present. On the right side, an atretic ureter was found. Both conditions had led to intrauterine hydronephrosis, renal dysplasia, oligohydramnios, pulmonary hypoplasia, and death of the child. Ureteral malformations are thought to be of epithelial origin. Autopsy showed only small rudiments of thymic tissue containing single epithelial cells, but were completely devoid of Hassall corpuscules. Again, this clearly points to an ectodermal defect. Although there was severe reduction of T cells in secondary lymphatic organs, the thymic defect would not have necessarily led to immunological deficiency; perhaps this is the reason that an epithelial defect in the thymus of patients with EEC syndrome has not yet been reported. With regard to an updating of the diagnosis of the EEC/EECUT syndrome, an "EEC/EECUT plus" syndrome is suggested.
Asunto(s)
Anomalías Múltiples , Labio Leporino , Fisura del Paladar , Displasia Ectodérmica , Linfocitos T/citología , Timo/anomalías , Enfermedades Urológicas/patología , Anomalías Múltiples/patología , Recuento de Células , Displasia Ectodérmica/patología , Humanos , Recién Nacido , Ganglios Linfáticos , Masculino , Bazo , SíndromeRESUMEN
A 44-year-old man was admitted with symptoms compatible with Addison crisis. Abdominal computer tomography revealed extensive bilateral adrenal abscesses. Histoplasma capsulatum was cultured from a needle aspirate. The patient was HIV-seronegative and had no underlying malignancy. He may have acquired the infection during several stays in endemic areas in the United States, South America and Asia. The case was also remarkable for moderate brain atrophy, thrombosis of the portal and splenic veins and liver cirrhosis caused by alpha-1-antitrypsin deficiency (phenotype MZ). The patient recovered fully under substitution of adrenal hormones and antifungal treatment. He received intravenous amphotericin B (75 mg q24h) for 10 days, followed subsequently by oral treatment with itraconazole (400 mg q24h) over several months. Radiologic follow-up 9 and 18 months later showed a pronounced decrease of the inflammatory adrenal lesions.