Desmoplastic Fibroma Recurrence Associated with Tuberous Sclerosis in a Young Patient.

Case Report. A nine-year-old patient with a diagnosis of tuberous sclerosis (with no pathological record) that showed calcifications at the brain level. Besides, the case showed the Vogt triad (epilepsy, mental retardation, and sebaceous adenoma). The patient clinically showed a volume increase of hard consistency, without suppuration and no sessile that included the following teeth 73, 74, and 75. Cone beam computed tomography (CBCT) was obtained, and it displayed a delimited unilocular lesion. After surgical excision, the histopathological report was desmoplastic fibroma (DF). It was observed that the patient had an aggressive recurrence of DF at four months after surgery treatment. Due to these clinical findings, resective osseous surgery and curettage were carried out. It is uncommon to find these two pathologies together (DF and tuberous sclerosis). Since DF is a benign pathology but very invasive and destructive, it is necessary a constant follow-up examination due to a high recurrence frequency.

The H.U.G.E. Formula (Hematocrit, Urea, Sex) for Screening Chronic Kidney Disease (CKD) in an Age-Stratified General Population.

AIM: To evaluate the screening power of the HUGE formula for the detection of chronic kidney disease (CKD) in a Spanish population sample obtained from the HERMEX study, a survey of cardiovascular risk factors carried out in the region of Extremadura, stratified by age. DESIGN AND METHODS: This was an observational, cross-sectional, population-based study. The final sample included 2,813 subjects selected from Health Care System records. Anthropometric data and cardiovascular risk factors were recorded. Hematocrit, urea, creatinine and microalbuminuria were analyzed, after which the HUGE formula was applied. Renal function, assessed as eGFR based on serum creatinine, was estimated following the MDRD-4 formula. RESULTS: Using the HUGE formula, the estimated prevalence of CKD was 2.2% (men 2.2%, women 2.1%). The prevalence of CKD increased with age (5.0% in persons aged 60- 70 years and 9.6% in individuals over 70 years of age, p < 0.001) whereas with the MDRD formula the prevalence values were 9.8% and 15.5% respectively. The HUGE formula was seen to be highly specific (0.99). CKD was more common in persons >70 years, obese subjects, hypertensive patients, dyslipidemic subjects and those with microalbuminuria. Multivariate analysis revealed an independent negative association of CKD as the dependent variable with SBP, serum triglyceride levels and microalbuminuria. CONCLUSIONS: The HUGE formula allows the prediction of CKD in the general population to be honed without relying on serum creatinine levels. This method was found to have a higher specificity than the MDRD-4 formula. Moreover, it could reduce the excessively extensive diagnostic suspicion of CKD in women.

[Changes in emergency admission times after the introduction of direct admission from emergency services]. / Tiempo de demora para la hospitalización tras la implantación del ingreso directo a cargo del Servicio de Urgencias.

OBJECTIVE: To measure the differences of waiting time for the emergency admission depending on which professional is in charge ie. the emergency doctor or the specialist of a specific area. PATIENTS AND METHODS: Once the Hospital Emergency Service was permitted to admit patients directly to the specialities, two periods of study where taken: Period A (before the direct admission) and Period B (after the implementation of it). The following was analysed: the number of patients taken, the complexity of their problem, the number of admissions and length of their stay. RESULTS: During Period A 41,917 patients were seen (228.07 patients/day) and during Period B 41,948 (230.48 patients/day). The average of the stay for those patients that were admitted directly from Emergencies had decreased by 1h and 42 min (p=0.001). The admissions in the Internal Medicine Service kept the same waiting time in both periods. CONCLUSIONS: The transfer of the responsibility of the admissions to emergency doctors has decreased the average waiting time of the patients in this area. The total number of admissions has not increased and has reduced the amount of work the different specialist.

Symmetrical ambulatory arterial stiffness index: relationship with microalbuminuria and renal function.

OBJECTIVE: The aim of this study was to investigate the arterial stiffness parameters derived from the proposed linear relationship between SBP and DBP obtained by ABPM, regarding its relationships with two markers of renal disease, microalbuminuria and renal function. DESIGN AND METHODS: One hundred and sixty six patients were studied: 73 males and 93 females mean age 55.2+/-15.5 years. 36.2% were receiving antihypertensive drug treatment. Microalbuminuria was measured in 24-h urine collection as well as albumin to creatinine ratio (ACR) in first morning urine. The ambulatory BP was measured non-invasively for 24 h by the Spacelab devices. RESULTS: Correlation test showed a significant relationship of Sym-AASI with age (p<0.001), serum creatinine (p=0.038), creatinine clearance (-0.423, p<0.001) and GFR (-0.263, p<0.001). On the other hand AASI was also correlated with age (p<0.001) and creatinine clearance (p=0.012), but not with the other parameters studied. 24-h albumin excretion rate was not correlated with Sym-AASI or AASI. Contrariwise, the albumin to creatinine ratio was correlated with Sym-AASI (p=0.013). As expected, AASI and Sym-AASI increase as severity of renal diseases grows. The patients in the highest quartile of Sym-AASI distribution showed an older age (p<0.001) and worse parameters of renal function (GFR, p<0.001; and creatinine clearance, p<0.008). CONCLUSIONS: Sym-AASI, an improved method for detecting arterial stiffness, seems to get an independent relationship with these parameters of renal disease which could not be detected with AASI.

[Acute mushrooms poisoning]. / Intoxicaciones agudas por setas.

The collection and consumption of wild mushrooms by individuals with no mycological knowledge and their accidental ingestion by minors are responsible for a growing increase of poisonings. It is not usual for emergency care of a patient affected by mushroom poisoning to be based on recognition of the responsible species of fungus or access to a sample of the mushroom consumed. It is this reason knowledge of toxidromes is crucial. In the majority of cases, the symptoms are weak. Mortality is usually related to the development of potentially fatal liver necrosis after consumption of the fungus Amanita phalloides and others which contain amatoxins. Treatment is based on support measures but no specific treatments exist that are based on scientifically proven studies. In the following article the clues used in diagnosing which make it possible to carry out a syndromic diagnosis based on a period of latency will be analyzed. The mushrooms that are responsible for the most common syndromes, therapeutic options, as well as varieties of toxic fungus which may produce peculiar and exceptional symptoms are also reported.

Beneficial effect of verapamil added to chronic ACE inhibitor treatment on renal function in hypertensive elderly patients.

This study analysed the effect of low doses of verapamil added to chronic treatment with angiotensin-converting enzyme (ACE) inhibitors on blood pressure and serum creatinine levels in eight elderly hypertensive patients who had a steady increase of serum creatinine while on ACE inhibitors. The study was performed in eight elderly hypertensive subjects, five men and three women (mean age 70+/-2 years; systolic blood pressure 173+/-4 mm Hg; diastolic blood pressure 99+/-1 mm Hg) and serum creatinine of 1.60+/-0.27 mg/dl before treatment. During an average of 25 weeks, ACE inhibitors significantly reduced both systolic and diastolic blood pressures, but serum creatinine levels were increased over basal levels (0,68+/-0,20 mg/dl, p < 0.05). During an average of 10 weeks, the addition of verapamil did not decrease blood pressure further, but serum creatinine levels were reduced to baseline. Our study suggests that the addition of verapamil to ACE inhibitors can reverse ACE-induced increase in creatinine levels in elderly hypertensive patients in whom this side effect is observed.

Cardiovascular effects of elgodipine and nifedipine compared in anaesthetized rats.

The cardiovascular effects of elgodipine were studied and compared with those of nifedipine in the presence or absence of ganglion blockade. A bolus of elgodipine (5-25 microg/kg) or nifedipine (60-120 microg/kg) was given and sequential cardiovascular effects in rats were recorded. Both dihydropyridines induced a dose-dependent decrease in mean arterial pressure but, whereas nifedipine induced reflex tachycardia, elgodipine induced a dose-dependent bradycardia. Both substances induced decreases in left ventricular d P/dt(max) without significant changes in central venous pressure. Good linear correlation was observed between the elgodipine-induced decrease in mean arterial pressure and those of heart rate and left ventricular dP/dt(max). The profile of the decrease in mean arterial pressure in animals pretreated with hexametonium chloride (20 mg/kg) was the same but the nifedipine-induced tachycardia was abolished without changes in elgodipine-induced bradycardia. These characteristics of elgodipine makes this dihydropyridine a potentially beneficial therapeutic agent in the case of severe hypertension accompanied by obstructive coronopathy.

Different experimental conditions which regulate type II 5'-deiodinase mRNA in rat Harderian gland.

In the present study, we describe the modifications in the expression of type II 5'deiodinase activity (5'D) in Xenopus laevis oocytes by injection of polyadenylated (poly A) mRNA from hypothyroid rat Harderian gland. The time-course study showed that the expression of the enzyme was dependent on time. Thus, enzyme activity was observed in oocytes 6 and 12 hours after the injection with poly A mRNA, reaching a maximal value at 24 hours. The activity was partially inhibited by 6-n-propyl-thiouracil, completely inhibited by iopanoic acid and exhibited a higher affinity for the T4 (Km=1.5 nM) than rT3 (Km=20 nM). The expression of the enzyme was modified in different experimental conditions: (a) exhibited diurnal variations with maximal peak values at night, (b) was inhibited by light at night and, (c) was activated by isoproterenol. On the other hand, we have also identified, for the first time, the size of mRNA capable of inducing 5'D in rats.

Characterization of binding sites for beta-adrenergic agonists and vasoactive intestinal peptide in the rat harderian gland.

Vasoactive intestinal peptide (VIP) receptors and beta-adrenergic receptors were investigated in rat Harderian gland membranes using 125I-VIP and 125I-cyanopindolol (125I-CYP), respectively, as ligands. The receptor bindings were rapid, reversible, saturable, specific, and dependent on time, temperature, and membrane concentration. The stoichiometric data suggested the presence of two classes of VIP receptors with Kd values of 0.36 and 65.37 nM and binding capacities of 323 and 39,537 fmol VIP/mg protein, respectively. The interaction showed a high degree of specificity, as suggested by competitive displacement experiments with several peptides structurally or not structurally related to VIP as follows: VIP > helodermin > rGRF > PHI > > secretin. Glucagon, somatostatin, insulin, and pancreastatin were ineffective at concentrations up to 1 microM. However, the stoichiometric data suggest the presence of one class of binding sites for 125I-CYP. The Kd for the single site was 290 pM with a binding capacity of 32 pmol/L. The pharmacological characterization of 125I-CYP binding to membranes showed that only isoproterenol, a beta-adrenergic agonist, and norepinephrine, an alpha beta-adrenergic agonist, was as effective as propranolol in inhibiting 125I-CYP binding to Harderian gland membranes. However, alpha 1- and alpha 2-adrenergic agonists and blockers such as methoxamine, prazosin, clonidine, and yohimbine were shown to be ineffective. These results demonstrate the presence of specific VIP and beta-adrenergic receptors in the Harderian gland and suggest a role for VIP and beta-adrenergic agonists in the physiology of this gland.

Inhibition by nitrendipine of 86Rb+ fluxes in subconfluent MDCK cells.

Part of the natriuretic mechanism of dihydropyridine Ca2+ channel antagonists involves the inhibition of renal tubular sodium reabsorption. To identify the membrane ion transport system involved in this natriuretic action, we tested nitrendipine on unidirectional 86Rb+ fluxes in Madin-Darby canine kidney (MDCK) cells. To dissect between direct and indirect effects (via cytosolic Ca2+) of nitrendipine, the compound was re-examined on ion fluxes in human erythrocytes. In MDCK cells, external Ca2+ (3 mM), adrenalin (100 microM) and the Ca2+ ionophore A23187 (20 microM) strongly and transiently stimulated 86Rb+ efflux. All these stimulatory actions were fully inhibited by quinine (1 mM) suggesting that they reflect the opening of Ca(2+)-sensitive K+ channels. Nitrendipine was able to inhibit these Ca(2+)-sensitive K+ channels, bit this inhibitory action required concentrations of the compound (approximately 100 microM). Regarding 86Rb+ influx, the most significant result with nitrendipine was a partial inhibition of bumetanide-sensitive 86Rb+ influx. This effect represented a maximal flux inhibition of about 70% and required very low nitrendipine concentrations (IC50 approximately 1 nM). The Ca2+ ionophore A 23187 strongly stimulated bumetanide-sensitive 86Rb+ influx in MDCK cells. Conversely, a very important reduction (approximately 79%) of this influx component was found in Ca2+ depleted cells. In human red blood cells, Na+, K+, Cl- cotransport fluxes were resistant to nitrendipine, even at high concentrations of the compound (100-500 microM). Conversely, Ca(2+)-sensitive K+ channels were inhibited by nitrendipine with IC50 = 6 +/- 3 microM (mean +/- S.E.M., n = 3).(ABSTRACT TRUNCATED AT 250 WORDS)

Expression of type II thyroxine 5'-deiodinase from rat harderian gland in Xenopus laevis oocytes.

The presence of isoenzymes mediating the conversion of thyroxine to 3,5,3'-triiodothyronine has been studied according to characteristic kinetics and physiological regulation. In this paper, we report the expression of type II 5'-deiodinase (5'D) activity in oocytes of Xenopus laevis. Oocytes injected with total RNA extracted from rat Harderian gland, and then incubated up to five days demonstrated a progressive increase in 5'D activity, reaching a maximal value at 24 h; then, 5'D activity remained almost stable for an additional period of four days. Characteristics of the enzyme activity expressed by oocytes included its inhibition by iopanoic acid, but not by propylthiouracil, and its increase during beta-adrenergic agonist treatment and hypothyroidism. The expressed activity manifests characteristics typical of the type II isoenzyme. Deiodinating activity in oocytes also exhibited diurnal variations. In this study, 5'D activity expressed in oocytes exhibited low values when animals were killed during the day, and high values when animals were killed at night. Maximal values were reached 3-4 h before the nocturnal peak of 5'D activity in Harderian gland crude homogenates. Results suggest that the in vivo activation of 5'D by isoproterenol, hypothyroidism, or dark exposure may be caused by an increase in the synthesis and/or maturation of the RNA expressing the enzyme.

Mechanism of low serum sodium levels in Mediterranean spotted fever.

Prominent degrees of hyponatremia are detected in the severe forms of Mediterranean spotted fever and the intensity of this abnormality parallels the severity of the infectious process. In order to determine the incidence, degree and evolution of hyponatremia in 110 patients with Mediterranean spotted fever and to explore the feasible renal mechanism that could lead to this phenomenon, serum and urinary osmolality and levels of urea, creatinine and electrolytes were measured in samples obtained at selected points (up to the fifth week) in the course of the disease, and parameters of renal function were calculated. Mean serum sodium levels of 135.6 +/- 5.5 mEq/l were detected during the acute phase of the infection. At this point, 42 patients (38.2%) had sodium concentrations less than or equal to 135 mEq/l. After recovery, mean serum sodium values were 142.5 +/- 2.5. The analysis of the parameters of renal function indirectly rules out an inappropriate antidiuretic hormone secretion or renal failure as the cause of hyponatremia. As tubular incompetence to reabsorb sodium is also rejected in these patients, a shifting of sodium to the interstitial or intracellular space may account for the phenomenon.

Autonomic nervous system in haemodialysis.

Fifty-two patients with terminal chronic renal failure on haemodialysis were assessed for the existence of autonomic neuropathy using the Valsava index. The values in the patients were lower than in the controls. Only a few symptoms could be related to the autonomic neuropathy. No correlation was found between Valsalva index and orthostatic or intradialysis hypotension, motor-nerve conduction velocity or plasma intact parathyroid hormone. Having demonstrated the existence of autonomic neuropathy, eight healthy subjects and ten patients on haemodialysis were studied to locate the segment of the autonomic reflex are affected. Heart rate and intraarterial pressure were monitored during the following tests: phenylephrine; hyperventilation; cold pressor; atropine; tyramine and adrenaline. An alteration was observed in baroreceptors, together with adrenergic hyperexcitability (reflected in the hypersensitivity of the sympathetic efferent pathway) and a greater response to the stimulus for the release of extra-adrenal adrenaline. The lower response to atropine is indicative of vagal alteration.

Changes of contact phase proteins during haemodialysis.

Functional and immunological activities of factors XII and XI, Prekallikrein, high molecular weight kininogen and C-1-esterase inhibitor were studied in eight patients before, during and after haemodialysis. During and after dialysis sessions a significant decrease in the functional activities of Prekallikrein and high molecular weight kininogen (p less than 0.05) could be detected, whereas their immunological levels did not change. Furthermore C-1-esterase inhibitor increased after dialysis (p less than 0.05). These results indicate activation of the kinin system during haemodialysis.

Focal segmental glomerular sclerosis in three generations of a single family.

We studied members of three generations of a family presenting a nephropathy characterized by proteinuria, occasional microscopic hematuria, progressive deterioration of renal function and an autosomic dominant hereditary pattern. In seven percutaneous needle biopsies, examination by light microscopy showed findings compatible with Focal Segmental Glomerulosclerosis (FSGS) in six patients and Focal Global Glomerulosclerosis (FGG) in one case. Deposits of immunoglobulins IgM, IgA and C3 following mesangial and peripheral distribution were observed. According to electron microscopy, the basal membrane was unchanged though electron dense deposits were found at subendothelial, subepithelial and mesangial locations.

Plasma renin activity, plasma aldosterone and distal urinary acidification in diabetics with chronic renal failure.

The renin-angiotensin-aldosterone system and the acidification capacity of the renal tubule were studied in 13 diabetic patients with chronic renal failure. As a whole, the group showed hyporeninaemic hypoaldosteronism (HH). Studied alone, 12 of the 13 patients presented the requirements for HH. This group showed hypercholaemic hyperkalaemic metabolic acidosis with a disturbance in renal acidification which may be classified as Type IV renal tubular acidosis. The results of this group were compared to those of another two groups; one of diabetic patients without chronic renal failure and the other with chronic renal failure (C Cr less than 40ml/min); both were seen to show different behaviour to that of the group affected by the two processes.