[Neonatal hearing loss screening: our results three years after starting the program]. / Despistaje de la hipoacusia neonatal: resultados después de 3 años de iniciar nuestro programa.

Since the 90s there increasing interest in early detection of neonatal hearing loss due to the feasibility of universal neonatal screening as well as to the possibility of early intervention. This has led to set up neonatal screening programs based upon otoacoustic emissions and to a great increase in the number of publications in the field. Our hospital started a neonatal hearing loss screening program using otoacoustic emission in March 2000. We used a Capella Cochlear Emissions (MADSEN electronics) equipment. Until May 2003 we have performed the test in 6372 children with a median age of 71 days. In our study, the sensibility of the test was 90.9% and the specificity 99.7%. Our results are similar to those reported in the literature although the age of the children was higher and we have found an incidence of hearing loss lower than other studies.

Effects of parenteral nutrition supplemented with glutamine or glutamine dipeptides on liver antioxidant and detoxication systems in rats.

Our aim was to determine the effects of glutamine or alanyl glutamine parenteral supplementation on the liver oxidant/antioxidant balance and on cytochrome-P450-mediated detoxication in rats. Animals were infused for 5 d with standard total parenteral nutrition (TPN), glutamine-enriched TPN, or alanyl glutamine-enriched TPN. The hepatic concentration of glutathione was reduced, and the levels of thiobarbituric-acid-reactive substances (TBARS) were increased in animals receiving standard TPN. Both glutamine and alanyl glutamine supplementation normalized glutathione, but thiobarbituric-acid-reactive substance concentration was only decreased by ananyl glutamine. This effect was parallel to a partial recovery of the activity of antioxidant enzymes. Cytochrome-P450 liver content, cytochrome-P450-dependent monooxygenases, and antipyrine clearance were not modified by glutamine or alanyl glutamine. Our data suggest a better protection against free radicals by alanyl glutamine supplementation and an absence of effects of both glutamine and alanyl glutamine on liver oxidative metabolism.

The effects of total parenteral nutrition on the hepatic handling of bilirubin in the rat.

Disturbances of bilirubin metabolism such as jaundice or pigment gallstone formation occur during total parenteral nutrition (TPN). We have studied the effects of TPN on bile flow and bile acid secretion and on the hepatobiliary transport of bilirubin in rats. Animals on parenteral nutrition for 5 days received 4.8 g of amino acids and 6.9 g of glucose daily. Controls were orally fed animals. Bile flow and bile acid secretion were not significantly modified by TPN. Serum bile acid and alkaline phosphatase levels were significantly increased in TPN animals when compared with the controls (+98% and +38%, respectively), which points to a relative cholestasis in the TPN rats. The biliary excretion of bilirubin monoconjugates and bilirubin diconjugates was significantly increased (+72% and +78%, respectively). This provides evidence for enhanced production of the pigment. Serum concentration of total bilirubin was enhanced in the TPN rats (+240%). The esterified/total bilirubin ratio in serum increased, whereas the bilirubin diconjugates/bilirubin monoconjugates ratio decreased. These facts, together with the minor reduction of hepatic bilirubin UDP glucuronosyltransferase activity (-12%), suggest that hyperbilirubinemia would be a consequence of both cholestasis and increased bilirubin production. The alterations reported here could contribute to the explanation of hyperbilirubinemia and pigment gallstone formation in patients maintained on parenteral nutrition.

[Incrusted cystitis with isolation of Corynebacterium group D2]. / Cistitis incrustante con aislamiento de Corynebacterium grupo D2.

Three cases of encrusted cystitis caused by Corynebacterium group D2 are described. The vesical damage previous to the establishment of this bacteria is noteworthy and the very rapid increase in urease activity explains the pathogenesis of the situation. Thus allowing for its identification and is relevant to treatment. Cloudy urine with a strong smell of ammonium, alkaline pH and crystals of ammonium magnesium phosphate in the sediment will bring this microorganism and its characteristic growth pattern to mind thus avoiding a falsely negative report. Treatment combining an antimicrobial agent and cystoscopic resection of the encrusted stones, where Corynebacterium group D2 has lodged, has proved efficacious. Vancomycin and teicoplanin have always been active and are eliminated through the kidneys.

[A histopathological study of the hepatic changes induced by parenteral nutrition in the rat]. / Estudio histopatológico de las alteraciones hepáticas inducidas por la nutrición parenteral en la rata.

Total parenteral nutrition is associated with hepatic metabolic and morphologic changes. Histological alterations induced by administration of glucose plus amino acids (group A) or glucose plus amino acids and lipids (group B) have been investigated in the rat. Parenteral nutrition provided 187 kcal/kg/day in both groups of animals. Rats in group B received 30% of nonprotein calories as lipids (Intralipid). In group A the most frequent histopathological findings were inflammation, portal edema and microesteatosis. Those alterations did not disappear by lipid addition, being frequent the existence of congestive phenomenons and esteatosis together with occasional pigment deposits in Kupffer cells. Those data suggest that supplementation of solutions with LCT-base lipid emulsions does not compensate for the histopathological alterations induced by parenteral nutrition.

[Hepatic metabolism of cholesterol]. / Metabolismo hepático del colesterol.

Cholesterol is an essential component of all tissues, as it is a part of the structure of cell membranes, and it is an immediate precursor of a series of essential substances such as vitamins, steroid hormones, and bile acids. Under physiologic conditions, the intake and output of cholesterol in the organism is coordinated and balanced with the aim of guaranteeing the availability of adequate amounts of cholesterol to satisfy the needs of the different tissues (fig. 1). Under pathological conditions there is an imbalance between these mechanism, which leads to an increase in the circulating levels of cholesterol, leading to pathological processes such as hyperlipemias, atherosclerosis and bile stones. The liver plays a central role in the regulation of the homeostasis of cholesterol. The molecule enters the liver in the form of chylomicrons and low density lipoproteins (LDL), through lipoprotein receptors, and this is also the most important organ for the de novo biosynthesis of cholesterol from acetyl coenzyme A, by means of a cascade enzyme reaction in which the enzyme 3-hydroxy-3 methyl glutaryl CoA reductase (HMG-CoA) is the key of the entire process. Cholesterol is found in the liver in the form of cholesterol esters or as free cholesterol. The two most effective ways of eliminating body cholesterol are found in the liver, with the degradation of the compound to bile acids and the biliary secretion of cholesterol. The conversion to bile acids takes place through a series of enzymatic steps in which the formation of 7-alpha-hydroxycholesterol by the enzyme cholesterol 7-alpha-hydroxylase is the key of the process. The biliary secretion of cholesterol is 600 mg/day. Both the abundance and the universality of cholesterol in living things as its clinical implications emphasize the importance and interest of this compound.

[Amino acid pattern variation in animals after the administration of hypocaloric peripheral parenteral nutrition]. / Variación del patrón de aminoácidos en animales tras la administración de nutrición parenteral periférica hipocalórica.

The pattern of intracellular aminoacids may be reproduced individually and is different for each illness. This study seeks to measure alterations in this pattern in rats after the administration of hypocaloric peripheral parenteral nutrition (HPPN), group B, for five days, by comparing them with rats subjected to conventional fluidtherapy, group A, for the same period. HPPN tolerance was good in all cases. Our results show a higher tendency to glyconeogenesis in group A, measured by a reduction in the glycogenic aminoacids especially alanine. An increase in branched aminoacids was observed in group A due to an increase in proteolisis. The animals in group B showed a lesser reduction in the intracellular aminoacids necessary for glyconeogenesis, especially glycine. HPPN, was found, to alter the aminoacids pattern in fasting rats. The results might be corroborated in humans studies.

[An experimental model of continuous biliary and vascular access in the free rat: a study of the changes in biliary function under the influence of the parenteral administration of amino acids]. / Modelo experimental de acceso biliar y vascular continuo en rata libre: estudio de las alteraciones de la función biliar por efecto de la administración de aminoácidos por vía parenteral.

Total parenteral nutrition is associated with different hepatic alterations such as cholestasis, steatosis or cholelithiasis. To study such alterations anaesthetised rats, in which the enterohepatic circulation of bile acids is interrupted, have been widely used. A model of free moving rat with venous and biliary catheterization has been developed in this work. This overcomes the interferences induced by anaesthesia as well the alterations in hydrostatic pressure or restraint-induced stress present in other models. The use of our animal model has confirmed previous data from anesthetised rats indicating that parenteral solutions of amino acids induce a cholestasis effect, with decreases in bile flow but not significant change in bile acid secretion.

[Segond's fracture at the knee level]. / La fracture de Segond au niveau du genou.

Ten patients out of twelve with Segond's fracture were treated surgically, checking the existence of an injury to the anterior cruciate ligament and in the remaining two were confirmed clinically and by radiology. Some controversy has existed regarding just what type of injury was being treated, and if it was associated or not with a ligament injury. In that series the injury was defined exactly, and we confirm the opinion of authors such as Insall, who consider it to be a pathognomonic sign of injury of the anterior cruciate ligament.

[Rheumatic manifestations associated with infection by the hepatitis C virus]. / Manifestaciones reumáticas asociadas a la infección por el virus de la hepatitis C.

The development in the past few years of laboratory test for hepatitis C virus allow us to associate it with a broad range of autoimmune manifestations such as cryoglobulinemia and Sjögren syndrome. As in other virus' infections, rheumatic manifestations have been described during VHC infection, but there are no large studies enough to know their true frequency and characteristic. The three reported patients in this issue presented and HCV related arthropathy once clinical picture, laboratory test and following, allowed us to exclude other diagnostics. Clinical manifestations ranged from arthralgias and intermittent arthritis to symmetric polyarthritis without any kind of join damage.

[Vesical schistosomiasis: an uncommon cause of bladder wall thickening]. / La esquistosomiasis vesical: una causa poco frecuente de engrosamiento de la pared vesical.

Schistosomiasis is typically a tropical disease; however, given the large influx of immigrants to our country and the boom in tourism to exotic locations, schistosomiasis should be taken into account in the differential diagnosis of certain clinical entities. The flatworm Schistosoma haematobium can cause a large variety of lesions in the urinary tract. The most common sites of involvement are the bladder and the lower third of the ureters, where it can cause lesions ranging from lithiasis and ureteral stenosis to the formation of masses inside the bladder. Ultrasonography is the imaging technique of choice for evaluating these alterations in the bladder; in addition to being fast, noninvasive, and painless, it is very useful for monitoring the response to antihelmintic treatment. We believe that it is essential to know the alterations in the urinary tract caused by this trematode, as this infectious disease that can be cured with Praziquantel might be confused with tumor diseases, such as vesical masses.

[Inflammatory aorto-iliac aneurysm. A cause of extrinsic ureteral obstruction]. / Aneurismas aorto-ilíacos inflamatorios. Una causa de obstrucción ureteral extrínseca.

OBJECTIVE: A ureteral pathology arising from inflammatory aneurysm of the abdominal aorta is described. METHODS/RESULTS: A case of inflammatory abdominal aortic aneurysm causing entrapment of both ureters, destruction of a renal unit and ureterohydronephrosis of the contralateral kidney is described. CONCLUSIONS: Treatment of inflammatory aneurysm of the abdominal aorta is by surgical repair with a prosthesis. Endourological urinary drainage is warranted prior to surgical repair, but ureterolysis to preserve renal function is unnecessary.

Pectin feeding influences fecal bile acid excretion, hepatic bile acid and cholesterol synthesis and serum cholesterol in rats.

This study was designed to investigate the effects of pectin on cholesterol and bile acid metabolism and to elucidate the mechanisms involved in its hypolipidemic effect in rats. The key regulatory enzymes in cholesterol and bile acid metabolism, 3-hydroxy-3-methylglutaryl-CoA reductase (HMG-CoA reductase) and cholesterol 7alpha-hydroxylase, were determined. Circulating, hepatic, and biliary lipid concentrations and fecal bile acid excretion were also measured. Male Wistar rats were fed a fiber-free or a pectin-supplemented (7 g/100 g) diet for 4 wk. Bile flow and the biliary secretion of both bile acids and cholesterol were not significantly different than controls in pectin-fed rats. The addition of pectin to the diet resulted in lower serum and liver cholesterol concentrations (-27 and -17%, respectively). Fecal bile acid excretion (+168%) and the hepatic activity of cholesterol 7alpha-hydroxylase (+70%) were significantly higher in pectin-fed animals. HMG-CoA reductase activity was also significantly greater (+11%) in the presence of dietary pectin. Results of our study indicate that pectin, by enhancing fecal bile acid excretion, may cause increased hepatic synthesis of bile acids and liver depletion of cholesterol in rats, which results in a higher rate of cholesterol synthesis and reduced serum cholesterol concentrations.

[Substitution and augmentation enterocystoplasty: long-term follow up]. / Enterocistoplatias de sustitución y ampliación: seguimiento a largo plazo.

From 1957 to 1981, nine patients (7 males and 2 females) were submitted to enterocystoplasty or bladder augmentation procedure. Patient age ranged from 19-67 years (mean = 40 yrs). Patient follow-up ranged from 9 to 31 years (mean = 19 yrs). The terminal ileum was utilized in 6 and the sigmoid in 3. Five patients had tuberculous conditions, 2 had transitional cell bladder tumor, 1 had interstitial cystitis, and 1 patient had bladder fibrosis. The ureters were directly reinserted in 2 cases. Two patients required TUR of the bladder neck; 1 patient had nocturnal incontinence. Two presented severe and 1 moderate chronic renal failure. In our view, augmentation and substitution plasty utilizing a segment of the intestine achieve good results. However, patients must be followed closely. Two patients who had been lost to follow-up presented with chronic renal failure. These patients might have benefitted from TUR of the bladder neck to correct voiding problems and avoid deterioration of the upper urinary tract.

[Corynebacterium D2 as a ureolytic organism: report of 5 cases]. / Corynebacterium D2 como germen ureolítico: a propósito de cinco casos.

Corynebacterium D2, a saprophytic microorganism of skin, causes alkaline encrusted cystitis in patients with a previous bladder injury. In 5 patients that had presented this nosological entity, these gram-positive rods were isolated in urine cultures and calculi. Four patients had undergone urological instrumentation maneuvers and one patient (female) had a history of recurrent cystitis from gram-negative bacteria. Corynebacterium D2 grows slowly and under certain conditions, as those described above, must be considered pathological despite counts of less than 100,000/ufc-cc. It is ureolytic and highly resistant to antibiotics. The synergistic effects of antimicrobials, acetohydroxamic acid and transurethral resection of the lithiasic plaques achieve satisfactory treatment of alkaline encrusted cystitis from Corynebacterium D2.

[Renal angiomyolipoma: a cause of massive retroperitoneal hemorrhage]. / Angiomiolipoma renal: una causa de hemorragia retroperitoneal masiva.

Five patients were diagnosed as having angiomyolipoma. Evidence or signs of tuberous sclerosis were observed in no patient. In 2 patients the presenting symptoms was massive retroperitoneal hemorrhage which required emergency surgery. The kidney could be preserved in 4 cases. One underwent nephrectomy for synchronous clear cell renal tumor. CT and US afford a diagnostic accuracy of nearly 100 percent. According to the data reported in the literature, tumors larger than 4 cm are amenable to treatment by conservative surgery. Smaller tumors can be followed carefully. However, surgical exploration is warranted not only in patients with pain and hemorrhage, but also in those cases with complex lesions less than 4 cm.

[Epidermoid carcinoma of the urinary tract. Immunohistochemical study]. / Carcinoma epidermoide de vías urinarias. Estudio inmunohistoquímico.

Six patients diagnosed as having urothelial carcinoma with varying degrees of squamous features are described. Four had pure squamous carcinoma. Two had transitional cell carcinoma (1 with squamous areas and the other had carcinoma in situ). The histological analyses of specimens were performed using immunohistochemical techniques to detect epithelial markers with low molecular weight monoclonal antibodies to cytokeratin. The tests were positive for pure squamous carcinoma and transitional cell carcinoma with squamous areas, and were negative for all areas of transitional cell carcinoma. We underscore the importance of the squamous component in the prognosis of urothelial carcinomas as well as the usefulness of this type of markers, particularly low molecular weight antibodies to cytokeratin, in differentiating squamous areas.