[Wolfram's or Didmoad's syndrome].
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Biomedical subjects
Publications and source records attributed to C Bernard.
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Measurement of glutamate decarboxylase (GAD) activity in the intermediate lobe of the frog pituitary and brain showed that neurointermediate lobe extracts represented 12% of the GAD activity detected in the whole brain. No significant activity was measured in distal lobe extracts. Immunocytochemical studies revealed GAD-containing fibers among the parenchymal cells of the pars intermedia. The localization of GAD-like material in the intermediate lobe of the frog pituitary suggested a possible role of gamma-aminobutyric acid (GABA) in the regulation of melanotropic cell secretion. Administration of GABA (10(-6) to 10(-4) M), to perifused neurointermediate lobes caused a brief stimulation of alpha-melanocyte stimulating hormone (alpha-MSH) release followed by an inhibition. Picrotoxin (10(-4) M), a Cl- channel blocker, abolished only the stimulatory effect of GABA (10(-4) M), whereas bicuculline (10(-4) M), a specific antagonist of GABAA receptors, totally inhibited the effects of GABA (both stimulatory and inhibitory phases). Bicuculline induced by itself a slight stimulation of alpha-MSH release, suggesting that GABA-ergic nerve fibers present in the intermediate lobe are functionally active in vitro. The GABAA agonist muscimol (10(-7) to 10(-4) M) mimicked the biphasic effect of GABA on alpha-MSH release. Administration of baclofen, a specific GABAB agonist (10(-7) to 10(-4) M) induced a dose-dependent inhibition of alpha-MSH secretion. In contrast to GABA or muscimol, baclofen did not cause any stimulatory effect whatever the dose. Taken together these result suggested that GABAA and GABAB receptors were present on frog melanotrophs.(ABSTRACT TRUNCATED AT 250 WORDS)
The vertebral artery and vein are mostly involved, but other sites exist, especially between the inferior thyroid artery and the internal jugular vein. Five cases were treated in our department. All the fistulas were closed by percutaneous transarterial occlusion with a detachable balloon. The mechanisms of fistula formation are discussed. The different therapeutic methods (surgical ligature and endovascular occlusion) are discussed. It would appear the endovascular method gives the best results.
After 1,748 translumbar aortograms three cases of intervertebral disk space infection were observed over a five-year period, for an incidence of 0.15%. Cultures suggested that the intervertebral disk had been inoculated with digestive tract organisms by the needle used to puncture the aorta. Diagnosis of this complication can be made early by retrieval of the responsible organisms from the intervertebral disk under CT control. Treatment consists of prolonged immobilization associated with appropriate antibiotic therapy for at least three months.
The effect of a milk substitute diet containing concentrated soya protein on secretory functions of the abomasum and pancreas and on plasma concentrations of gut hormones and soya antibodies was studied. Sixteen calves aged 12-19 weeks were given a milk substitute in which a major part of the protein source was either soya concentrate (soya diet) or skim milk (control diet). The soya diet was prepared by hot aqueous ethanol extraction of soya bean meal to remove oligosaccharides and inactivate antigenic constituents. Circulatory IgG antibodies against soya proteins were found in all of the calves when they were 16 weeks of age. Their titres increased slightly between 16 and 19 weeks, irrespective of the diet. It seems unlikely that the presence of these antibodies was related specifically to the feeding of the soya concentrate. At slaughter the weight of the gastric mucosa and pancreas and quantities of pancreatic protein together with specific activities of trypsin and chymotrypsin were significantly lower (17, 20, 16, 30 and 36%, respectively) with the soya diet. The quantities of enzymes in the gastric mucosa or the specific activity of pancreatic amylase were not affected, whereas that of lipase increased by 26%. Total enzyme activities as well as units per kg live weight gave significant differences only for trypsin and chymotrypsin which were reduced by 43 and 38%, respectively. With the soya diet, fasting concentrations of gastric inhibitory peptide (GIP) and secretin in plasma samples were significantly lower (49 and 34%, respectively). Values of GIP were also lower (54%) 1 h after feeding. In contrast, postprandial values of cholecystokinin (CCK) were 1.4 times greater. No significant differences were found between the two diets for gastrin, vasoactive intestinal peptide (VIP), bovine pancreatic polypeptide (BPP), somatostatine and motilin. In general these observations could be explained, in part, by the more rapid passage of protein and fat from the abomasum to the duodenum following feeds containing soya concentrate. However, these differences in concentrations of gut hormones did not seem to be related to variations in the weights of gastric mucosa and pancreas or activities of pancreatic enzymes.
Pulmonary sequestrations are congenital abnormalities where nonfunctioning lung tissue receives its vascular supply from the systemic circulation (thoracic or abdominal aorta). It is necessary to establish the diagnosis in childhood when the lesions are uncomplicated. The authors present three cases of sequestration of the apex (2 extralobar and 1 atypical) with the main clinical and radiological features. Sequestrations in the upper lobe are rare, and the usual site is the left lower lobe. Plain x-rays show a dense opacity, sometimes air-filled and sometimes with an air-fluid level: angiography is currently the best mean for definitive diagnosis; however, computed tomography will probably be very useful in the future. Differential diagnosis includes tumours of the superior mediastinum (neurogenic tumours, digestive duplication, bronchogenic cysts, pheochromocytoma and hydatid cysts).
The mechanisms of secretion of endolymph were studied in vitro in the isolated inner ear of the frog. Prior to in vitro experiments, the composition of perilymph was evaluated in vivo and compared to that of plasma. Composition of perilymph resembled that of an extracellular fluid, although Na and Cl concentrations were higher and K concentration was lower in perilymph than in plasma water. No difference in Ca and Mg concentrations was observed between these two fluids. Osmolality averaged 227 mosmol/kg H2O in perilymph and 183 mosmol/kg H2O in plasma. Endolymph in frog inner ear corresponded in chemical pattern to mammalian endolymph. K and Na concentrations in endolymph collected from the ampulla of the posterior vertical semicircular canal averaged 121.1 mM and 2.5 mM, respectively. Osmolality of endolymph was 237 mosmol/kg H2O. K and Na concentrations were unaltered when inner ears were incubated for 24 h either at 15 degrees C or at 4 degrees C. Addition of ouabain (10(-4) M) to the perilymph-like bathing solution altered greatly Na and K composition of endolymph after incubation for 3 h at 15 degrees C. The Na and K concentration gradients between endolymph and the bath were abolished after incubation for 24 h. Ligatures of the posterior vertical semicircular canal were performed at different sites to isolate some parts of the canal, i.e. the ampulla and the non-ampullar duct. K concentration in the ampulla after incubation for 24 h remained as high as 20 times that in the bath. This K gradient was abolished in the presence of ouabain (10(-4) M). High K concentration could be maintained in the non-ampullar part of the semicircular canal only if the latter communicated with the ampulla. It is concluded that endolymph is actively secreted into the ampulla of the semicircular canal. Na+-K+-activated ATPase in the ampullar dark cells may energize the ouabain sensitive ionic transports that are involved in the production of endolymph. Endolymph secreted into the ampulla would spread intraluminally to account for the high K and low Na concentrations of the fluid which fills the non-secretory part of the semicircular canal.
Intralobar pulmonary sequestration in an upper lobe is uncommon. The radiologic manifestations are similar to lower lobe lesions. A case is presented in which there are features of both intralobar and extralobar sequestration.
In amphibians, alpha-MSH secretion is stimulated by thyrotropin-releasing hormone (TRH). In the present study we show that extracellular calcium influences directly the spontaneous secretion of alpha-MSH and that it participates in the mechanism of action of TRH on frog melanotrophs.
Twenty two patients presenting a typical sonographic pattern of tumefactive biliary sludge are studied. In all cases was observed a polypoid mobile, rounded mass, without acoustic shadow. The lesion stayed in the gallbladder in 15 cases and in distended main bile duct in 3 others. There was a double localisation in 4 cases. Seven verified cases proved to be constituted of very thick biliary sludge.
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A boy of two years has an hematuria which was first related with an hypertensive acute glomerulonephritis. However because of recurrency of the hematuria radiological investigations were performed and a solid tumor of the right kidney was discovered. The arterial hypertension regressed after the right nephrectomy. Pathology demonstrated a tumor of Grawitz which is rare in children and which is seldom associated with an arterial hypertension. We found only five similar cases in the literature. Arterial hypertension can be related either with a compression of the renal artery of with an inadequate secretion of renin by the tumor.
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Substance P-, somatostatin-, vasoactive intestinal polypeptide- and cholecystokinin-like levels were measured in lumbar dorsal and ventral cord of polyarthritic rats and compared with those obtained in vehicle-treated rats taken as controls. Polyarthritis decreased substance P concentration in lumbar ventral cord and increased cholecystokinin level in lumbar dorsal cord, while the other two peptides did not show any change. The results are discussed in relation to immunohistochemical data found in the literature.
Spontaneous activity of semicircular canal afferents in the isolated labyrinth of the frog is altered by bath application of cholinergic agonists. Muscarinic agonists can produce an increase in action potential frequency of individual afferents. This increase develops slowly and is prolonged in the time course of its action. Nicotinic agonists can either increase (most cases) or decrease afferent activity. These effects occur rapidly and decay during the period of activation, suggesting desensitization. Muscarinic effects are blocked by prior administration of atropine and nicotinic effects (both increases and decreases in action potential frequency) by curare. Intracellular recordings reveal that the nicotinic effects on afferent action potential frequency are the result of alterations in the frequency of spontaneous synaptic potentials, indicating a presynaptic site of action on the hair cells for these compounds. This conclusion is supported by the fact that in the presence of high Mg2+/low Ca2+, which blocks hair cell release of transmitter, cholinergic agonists do not affect the resting membrane potential of the vestibular afferent. Electrical stimulation of the VIIIth cranial nerve can result in either an increase or a decrease in spontaneous synaptic potential and action potential frequency of an afferent. These effects are blocked by prior administration of curare or of nicotinic agonists. Repetitive or continuous stimulation of the VIIIth nerve results in a reversible reduction of the evoked response, suggesting desensitization. Transection of the VIIIth cranial nerve two weeks prior to recording eliminates these actions of electrical stimulation, but not the responses to cholinergic agonists, indicating that the effects of electrical stimulation are mediated by centrally arising efferents. These findings confirm that acetylcholine is probably the transmitter released from centrally arising vestibular efferents, and, in addition, demonstrate that efferent-mediated effects are predominantly expressed through nicotinic receptors. Studies comparing the effects of isolation of the semicircular canal alone versus the intact labyrinth suggest that the method of isolation may be an important factor in determining whether efferent activity results in a predominant increase or decrease in afferent activity.
Adult frogs (Rana esculenta) were given subcutaneous injections of 10, 20, 30, 50 and 100 mg/kg capsaicin in sequential order over 5 days, or the vehicle only. The nociceptive thresholds to electrical, thermal and chemical stimuli were measured before, and 1, 5 and 24 h after each injection. Capsaicin was followed by a dose-related reduction of nociceptive responses to all stimuli, but these effects lasted for only 1-5 h after the given injection. Water/acetic extracts of undivided brains and spinal cords were prepared at the corresponding time periods for the radioimmunoassay of peptides. Spinal cord concentrations of immunoreactive substance P were essentially unaffected by capsaicin, while those of immunoreactive somatostatin were significantly increased after the second for fourth injections (20, 30 and 50 mg/kg) of capsaicin. Brain extracts showed an increase of somatostatin and substance P concentrations after the dose of 50 mg/kg. In an additional experiment, immunoreactive substance P, somatostatin and cholecystokinin were measured in tissue samples taken at 2 and 10 min, and 1, 5 and 24 h after a single dose of either 50 mg/kg capsaicin or the vehicle. The only significant effect of capsaicin was an increase of immunoreactive somatostatin concentration in brain homogenates at 5 h, while the vehicle in itself elicited major variations of all three peptides in spinal cord and/or brain. These results indicate that capsaicin reduces the nociceptive responses to cutaneous stimuli in adult frogs.(ABSTRACT TRUNCATED AT 250 WORDS)