Skin punch biopsies and peripheral blood lymphocyte preparations from two siblings with early infantile (Santavuori) neuronal ceroid lipofuscinosis have been examined by electron microscopy. In both cases characteristic osmiophilic inclusion bodies were found in various cells in the skin and in lymphocytes. In one case, lymphocyte inclusions were detected before the onset of any symptoms as a result of a family study. This indicates the possibility of screening lymphocytes of siblings of affected patients as a result of a family study. This indicates the possibility of screening lymphocytes of siblings of affected patients with a view to early detection of the disorder.
THE PROBLEM of increased arterial blood pressure has assumed vast importance in recent years. As a cause of death, it is preeminent since, directly or indirectly, hypertension causes four times as many deaths as does cancer; moreover, its victims fall into a lower age-group than do those of malignant disease. The resistance of hypertension to effective medical treatment has attracted the surgeon to the problem and his attack has centered particularly upon the nervous system (splanchtlic resection, anterior nerve root section, etc.), the objective being the elimination of vasomotor stimuli to the vascular tree. In general, the results of these operations have not been up to expectations.The classic work of Goldblatt and his associates' on the production of hypertension in animals (dogs and monkeys) by constricting the main renal arteries stimulated the present studies. These workers found that constriction of only one renal artery was followed by hypertension lasting for days or weeks, the blood pressure eventually returning to the normal level; chronic persistent hypertension could be produced only by constricting both main renal arteries (bilateral renal ischemia) or by constricting the main renal artery of one kidney and removing the other kidney. They observed that in animals with experimental hypertension, whenever the blood pressure fell to a lower value it was due either to inadequate clamping of the renal arteries or to the development of an adequate collateral circulation by the kidney through the ureteral and capsular vessels.It follows naturally that if human essential hypertension is the counterpart of hypertension produced in animals by renal ischemia, an adequate collateral circulation brought to one or both kidneys would be followed by a return of the blood pressure to lower levels. However, there is a fundamental difference between the two forms of hypertension. In human essential hypertension renal ischemia is rarely due to obstruction of the main renal arteries (such cases, however, have been described) but to a diffuse narrowing of the preglomerular arterioles. It seemed important, nevertheless, to determine whether an improvement of the vascular supply to the human kidney by increasing the accessory circulation would result in a fall in blood pressure
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