Adrenal and pituitary incidentalomas in a case of Cushing's syndrome.

Cushing's syndrome is a pathological condition where surgery may be lifesaving. The proper diagnosis depends upon the hormonal pattern of the patient, various dynamic tests and imagistic investigations. We report a case of a patient with Cushing's syndrome, with bilateral adrenal tumors and a pituitary microadenoma. She presented increased levels of basal cortisol, unsuppressed during a low and a high dose Dexamethasone test. She underwent right laparoscopic adrenalectomy and developed acute adrenal insufficiency. Two years after the intervention, she still requires adrenal substitution therapy. Acute adrenal crisis is a serious complication of adrenal surgery, with high mortality if unrecognized.

Pheochromocytoma--from endocrinologist to surgeon.

We report the case an asymptomatic patient who underwent left adrenalectomy for a incidentally discovered large, cystic adrenal tumor. The patient presented no hypertension before or during the intervention. The hystopatological exam revealed a pheochromocytoma, with signs suggesting malignancy. One year after the surgery, the patient is still asymptomatic, the metanephrine levels are normal, and the 1231-metaiodobenzylguanidine scintigraphy showed no metastases. The indications and limits of laparoscopic adrenalectomy are presented.

Retinal involvement in a case of nephronophthisis associated with liver fibrosis Senior-Boichis syndrome.

Electroretinographic and electroencephalographic studies were conducted in a 12-year-old boy with nephronophthisis, chronic hepatic fibrosis, mental retardation and tapetoretinal degeneration (Senior-Boichis syndrome). Markedly reduced ERG amplitudes and flat oscillatory potentials were found in the proband. Delayed scotopic implicit time and reduced amplitudes of the beta-wave were found in the mother's ERG. ERG may identify the carrier state of the Senior-Boichio syndrome.

Electroretinography and temporal aspects in macular dystrophy.

In a family with cone-rod dystrophy of autosomal recess inheritance, the diagnosis was established by characteristic abnormalities of the ERG, which demonstrates cone responses reduced in amplitude and delayed in implicit time, and rod delayed implicit time in two sisters with macular degeneration. The abnormalities of the implicit time are seen also in two other sisters of the propositi, who are clinically normal. The hypothesis that they are only capable to transmit the gene defect to their offspring is advanced.

The effects of acetazolamide on the human electroretinogram.

The effects of acetazolamied on the cerebral circulation and metabolism was previously shown to be due to a raised PCO2 in brain tissue. The effects of the drug on the human electroretinogram (ERG) are investigated in fifteen human volunteer subjects. A significant increase in the b-wave was found. A similitude with the mechanism of acetazolamide administration in brain and retinal tissue seems to be a possible explanation.

Temporal aspects of electroretinography in patients with myotonic dystrophy.

Electroretinographic records of eight patients with myotonic dystrophy (Steinert's) in ganzfeld and single-flash stimulation showed a decrease of the b-wave amplitude and a delayed scotopic implicit time.

["Implicit time". Practical and theoretical aspects]. / Le "temps implicite". Aspects théoriques et pratiques

52 normal subjects were studied as control for our "Ganzfeld" method of E.R.G. Separately 132 patients with different retinopathies; inflammatory and degenerative (or dystrophic) were studied and results compared and discussed. It seems that the amplitude is involved - diminished - in some retinopathies, but implicit time only when widespead alteration of neuro-epithelim accured.