La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris最新文献

The authors report two cases of chiasmatic tuberculomas. They emphasize that diagnosis is still difficult despite CT scan if a picture of tuberculous meningitis is lacking. Surgical approach may be justified when diagnosis remains uncertain, especially as tuberculoma is sometimes responsible for recurrent meningitis.

A case of true cutaneous histiocytosarcoma --or Crosti's reticulosis--is reported, and provides the basis for a discussion of the techniques at present used in defining the histiocytic origin of lymphomas, criteria for assessing the malignancy of histiocytic infiltrates, and clinical data and course of histiocytic sarcomas. Particular emphasis is laid on the characteristics of Crosti's reticulosis, which the authors consider to be a form of histiocytic sarcoma, usually with a favorable outcome. They also attempt to isolate pathological criteria for assessing the prognosis of the disease, which appears to vary substantially from case to case. Setting aside cases which had not been studied by both ultrastructural and enzymo-immunological techniques, the authors found only the twenty-two cases of primary cutaneous histiocytosarcoma in dermatological literature, including their own.

I.V. arterio-nephro- urotomogram turns out to be very useful in the work-up of renal traumas mainly by studying the cortical phase and the arterial phase. This technique allows to limit the indications for selective renal arteriogram.

Serum parathyroid hormone was assayed in 16 elderly patients with primary, roentgenographically proved, articular chondrocalcinosis. Mean value, i.e. 4.10 + 1.16 mUI/ml, was compared to that of a control group of 16 subjects matched for sex and age without chondrocalcinosis, living in the same geriatric institution, i.e. 3.94 + 1.43 mUI/ml. The difference is not significant (t = 0,35). Latent hyperparathyroidism is therefore not demonstrated in primary proved articular chondrocalcinosis in the elderly.

Porphyria cutanea tarda (PCT) is the most common porphyria. In contrast to the other porphyrias, which are undisputably hereditary, PCT was long considered to be acquired. However, the recent demonstration of a defect in uroporphyrinogen decarboxylase suggests that PCT is supported by a genetic factor. Though the hereditary or acquired nature of the enzymatic defect is still under debate, it seems that the disease has a polygenic origin involving an impairment of iron metabolism and a specific genetic background.

Three new cases of myelopathy without compression associated with paraproteinemia are reported. The neurologic picture was that of spinal muscular atrophy in one case and chronic spinal combined sclerosis in two. The nature of the paraproteinemia is discussed: all three patients seem to have had benign monoclonal dysglobulinemia. One patient died from cardiovascular disease five years after onset, and another from complications related to decubitus seven years after onset. Postmortem examination was not possible in either case. Spinal muscular atrophy has been reported in certain carcinomas (lung, stomach, breast) and, less frequently, in macroglobulinemia. As benign dysglobulinemia is common after sixty, coincidental association cannot be outruled. However, data from the literature and the response to cancer chemotherapy in two patients suggest an original pathologic association. Recent demonstration of demyelinating neuropathies associated with benign paraproteinemia provide further evidence in support of such an association.

The authors report the case of a 60-year-old man presenting with an esophagobronchial fistula. The congenital origin of the lesion was demonstrated by the history of the clinical symptomatology and by radiological, endoscopic and anatomical arguments. While it is a rare complaint in adults, it is worth considering in the diagnosis of a chronic cough, because it can be cured surgically without any problems.

A new familial observation of Cohen syndrome in two sisters is reported. Both children exhibited the typical features of Cohen syndrome, i.e. obesity, hypotonia, mental deficiency and a dysmorphic syndrome mainly involving the facies and extremities. More accurate delineation of the nosologic limits of this syndrome is ensured by a critical review of the 17 observations published in the medical literature, which include 9 familial cases: the contribution of subsequent observations to the initial description is pointed out. Attention is drawn to the unusual course of the obesity which typically occurs after five years of age, in contrast to common obesities. Furthermore, Cohen syndrome is remarkable by the morphologic abnormalities, particularly those of the facies, which should suggest the diagnosis. As in our observation, data from the literature is in support of autosomic recessive inheritance which implies restrictive genetic counseling.

The diagnosis of temporal arteritis is made after a mean delay of four months. This delay is explained by the absence, in 50% of cases, of any clinical sign in the temporal area, the possible presence of alterations in liver function tests, and the frequency of anemia and loss of weight. These signs often suggest a mistaken diagnosis of hepatobiliary disease or cancer. The authors evaluate the cost of this delay in a personal series of 33 cases confirmed histologically. They recommend biopsy of the temporal artery in each patient aged more than 70 with a marked rise in erythrocyte sedimentation rate unexplained by an infectious disease or dysglobulinemia.

Prostaglandins and thromboxanes are 20-carbon-atom-chain fatty acids which contain a cyclopentane or oxane nucleus respectively. They belong to a family of icosanoids whose main intracellular precursor is arachidonic acid. These icosanoids are synthetized in all tissues (except red cells). Enzymatic hydrolysis of the arachidonic acid of lipids is the first step of their biosynthesis. This step, which is limitative, is usually catalyzed by phospholipase A2 and may be indirectly inhibited by glucocorticoids. Once released, arachidonic acid is promptly transformed into an endoperoxide intermediary through the action of an enzymatic complex called "cyclooxygenase". This intermediary is the precursor of both prostaglandins and thromboxane. Non-steroidal antiinflammatory agents such as acetylsalicylic acid and indomethacin have an inhibitory action on cyclooxygenase. The mechanisms of action of prostaglandins and thromboxane are still poorly understood. In certain tissues (platelets, adipose cells) their effect seems to involve stimulation of adenyl-cyclase and/or guanyl-cyclase. In other tissues (smooth muscle) their biological action is associated with changes in transcellular flows of calcium. Prostaglandins and thromboxane have many biological properties. Their effects are local, as they are produced in small quantities and promptly metabolized. They act more often as cell function modulators than as essential mediators. The often conflicting biological actions of these icosanoids involve: a) smooth muscles (stimulation or relaxation of blood vessel, respiratory tract, digestive tract and genitourinary tract musculatures); b) platelets (induction or inhibition of aggregation); c) the inflammatory reaction; d) the cellular fluid and electrolyte transports; e) and certain metabolic functions (lipolysis, insulin release, renin release, mobilization of bone calcium...). The advances achieved over the last decade now provide some insight into the significance of the physiologic role of icosanoids. Several prostaglandins, as well as some of their analogues, are already being used as therapeutic agents. In the future, rational manipulation of dietary fatty acids and development of selective inhibitors can be expected to provide the necessary tools for better controlling the icosanoid system in medicine.