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Biomedical subjects

J M Gil Vernet

Publications and source records attributed to J M Gil Vernet.

At least 19 recordsLinked to original sources

[Biliary atresias operated with favourable results: predictable outcome].

Since 1975, our experience in the treatment of biliary atresia with Kasai's technique has improved little by little, achieving 65% favourable outcome in the last five years. We define "good results" as the complete restoration of biliary flow and normalization of bilirrubin levels. The long-term evolution of these good results can be diverse. The objective of the present work is to analyze the outcome of patients in our series in whom a favourable initial response was achieved, as well as evaluating their present situation and future perspectives. The authors present a total of 17 patients operated by Kasai's technique since 1985, that constitutes the group with good results in our series. The controls were based on general analysis, liver function and periodic ultrasound explorations. All received a standardized medical treatment consisting of vitamin supplements (A, D3, E, K) minerals (zinc, calcium, phosphate, iron) ursodexoxicolic acid, luminal,as well as close control of calorie intake. In two patients the levels of bilirrubine were progressively increased with time, stabilizing at between 5/6 mgs/100 ml, with progressive hepatic hardening, appearance of splenomegalia, indirect signs of portal hypertension and a slight deterioration of hepatic function. One received a transplant at age 12 with Quick levels below 50%. The other, aged 16, continues with an acceptable hepatic function and good quality of life under recommendation of transplant. Eleven patients with ages ranging from fourteen months to seventeen years presented slight and firm hepatomegalia, moderate portal hypertension, GOT 71 +/- 8 mg/100 ml, GPT 97 +/- 11 mg/100 ml and normal bilirrubine levels. From this group, 3 patients, all under five years of age, experienced bleeding from esophageal varices which were controlled by sclerosis and medical treatment (propanolol and isosorbide dinitrate). Recently, one three year-old patient developed a hepatocarcinoma of rapid, mortal evolution. Since then, the determination of alfa-feto protein in follow-up controls has been introduced. Four other patients of 5, 6, 14, 16, years of age are completely assymptomatic with an excellent clinical evolution. In our experience,the patients that overcome the third year after surgery without serious complications seem destined to reach puberty with a good quality of life. However, some cases show signs of hepatic fibrosis and portal hypertension, 77% in our series. Only 23% of patients with a favorable initial evolution appear to present a complete normalization of their hepatic lesion in the long term.

Adolescent↗

[Endoscopic treatment of tracheo-esophageal fistulas: fact or fiction?].

Tracheo-esophageal fistulas as a sequelae of esophageal atresia surgery are usually a severe complication whose correction requires an important surgical aggression, often with uncertain results. The possibility of treating this problem through the use of non-aggressive endoscopic methods has been described in medical literature over the last years, applying several products for occlusion with conflicting results in different publications. We present three cases of tracheo-esophageal fistulas, describing the technique of trans-tracheal endoscopic approach based on the novelty of employing a laryngeal mask which facilitates ventilation during the procedure, as well as the possibility to use endoscopic material of greater diameter. We comment on the different occlusion materials employed (Tissucol & Histoacryl) and the difficulties of their management. In one case two attempts of occlusion were made, and three in the other two, varying the application method and product. In two cases the fistulas initially reopened and in the other an occlusion was demonstrated by esophagogram and remission of respiratory symptoms during eight months was achieved. In a posterior control, a thread-like fistula reappeared but did not affect clinical improvement. The possibility to employ endoscopic techniques always seemed very promising for this type of pathology. The appearance on the market of various substances with capacity of occlusion has allowed us to employ them in comparative studies and simultaneously test the efficacy of the laryngeal mask in this kind of situations. The results show the complete ineffectiveness of some of these products and the relative possibilities of others, although in our experience we have yet to achieve any definitive occlusion.

Child↗

[Treatment of caustic stenosis of the esophagus with self-expanding devices].

Caustic stenosis is a serious problem in children due to its complicated resolution and implications in important areas like nutrition, as well as the child's tolerance to the measures taken to correct them. After dealing extensively with this problem over the last twenty five years, always from a conservative approach using traditional methods like dilatations, we believe we have found a technique that brings together all the necessary conditions to achieve a favorable and definitive solution. Our ideal goal to achieve a device that provides a well-tolerated permanent esophageal expansion during the scarring process, also permitting normal swallowing, seems to have been reached through the use of the new generation of silicone stents. The authors present their experience in the first seven cases of caustic stenosis treatment through the placement of silicone stents, describing a precise placement technique while establishing a standard protocol for the use of these devices.

Adolescent↗

[Bile duct atresia: outline for a solution].

UNLABELLED: Biliary atresia continues to be a serious and relatively rare disease (1/50,000 newborns) and whose long-term prognosis has changed drastically since the appearance of liver transplant (LT) as a therapeutic weapon. The combination of two factors, early diagnosis and correct application of Kasai's surgical technique, is essential to obtain acceptable results and sufficient biliary drainage allowing the children to overcome the critical 7 kg barrier and place them in the lesser morbi-mortality range in relation to a possible LT. But we must keep in mind that despite its critics, Kasai's technique can guarantee, both in our own experience and in the literature, ten years survival percentages over 50% with correct hepatic function, as well as clinical normality and a quality of life clearly superior to first years post-LT. We present the evolution of a group of 20 patients affected with biliary atresia, diagnosed in our center since 1985, the year when pediatric LT began to be used as a therapeutic procedure in this country. We valued the age of intervention, technique, immediate and long-term results and the evolution and necessity of LT. All 20 patients were analyzed individually, and they currently have an age range from 2-14 years and were all operated by Kasai's technique. We classified the patients as having good, regular or poor results with regards to biliary flow, normalization of billirubin levels and clinical evolution. Sixteen patients presented biliary flow of such an extent that 14 of them, classified as good, completely normalized the billirubin levels. Two others, presently aged 14 and 8 years respectively, present average levels of 2.5-5.5 mg/100 ml and are classified as regular in a situation of advisable transplant, although with an acceptable hepatic function. Only one case, the first in the poor group, did not initially present biliary elimination and died at age six months while on the waiting list. Three other cases in the same group presented insufficient biliary elimination and were transplanted with 7, 11 and 12.5 kg, respectively. The second died in the first year post-transplant. CONCLUSIONS: In our opinion, action in biliary atresia must be early and based on the correct application of Kasai's technique, seeking to achieve a biliar flow that eliminates or distances the patient as far as possible from the necessity of a future LT. Three lines come together to obtain this target: an early diagnosis, a correct application of Kasai's technique, and an implication in the follow-up and treatment of these children by the hepatic transplant groups. All this advises us, as is done in other countries, to create reference centers for the study of neonatal cholestasis where an accumulated experience of a relatively rare pathology can be taken advantage of.

Adolescent↗

[Intraesophageal stent in the prevention of stenosis caused by caustic ingestion].

Esophageal stenosis is a frequent consequence of lye ingestion, and their treatment is of longstanding and complex matter. The search of new solutions for this problem is challenging and always attractive if its associated with less aggressiveness to the patient. The use of intraesophageal stents is not new in the treatment of stenosis, but it was anecdotal, until histocompatible material came out in the market. The stent is designed in the OR, having the same length as the stenosis, previously observed by endoscopy, its made out of a silicone tube 30 or 36 French, mounted over an ng tube 16, all this is fixed in the ends of the silicone tube, that way won't slip over the ng tube. It is placed with a laryngoscope assistant, the proximal end of the ng tube comes out the nasal nares. Once the location of the tube is checked, the stent is left in place for six weeks. Our experience, with seven patients, has shown excellent tolerance to the stent; at the third post procedure day the patients were eating soft diet by mouth, we believe that the esophageal spasm had resolved by this day. Once the stent was withdraw, five cases were free of esophageal lesions, in the other two small areas of bleeding were visualized. In the follow-up the five cases that were free of lesions, had a normal esophageal diameter, in the other two, one had a moderate restenosis and the other case was severe.

Age Factors↗

[Transjugular portosystemic shunt. An alternative in recurrent hemorrhage in portal hypertension in children].

The transjugular portosystemic bypass is a new technique for treating portal hypertension of an intrahepatic nature by inserting a multipurpose catheter through the jugular vein and vena cava as far as the suprahepatic vein, with the aid of a puncture device it is fed via the hepatic parenchyma into a portal branch. The passageway thus created is distended with a balloon to permit the insertion of an extensible metal mesh prosthesis or "stent" to maintain a permeable connection. Our experiment was conducted on a paediatric patient suffering from mucoviscidosis with severe pulmonary and hepatic involvement and recurrent bleeding in an uncontrollable position. After inserting the bypass, portal pressure dropped sharply and the bleeding stopped, the patient being discharged on the sixth day. Two months after the bypass, the coronary vein of the stomach, the seat of the gastric varicose veins, was selectively occluded through the stent via the femoral vein. Six months after follow-up, the digestive bleeding has not recurred.

Adolescent↗

[Our experience in the management of portal cavernoma in children].

The authors present 14 patients affected with portal cavernoma, diagnosed and controlled over the last 12 years. The splenomegaly (60%) and the digestive hemorrhages (40%) were the initial study causes. The cavernoma appeared idiopathic in 10 patients or secondary either to a catheterization of umbilical vein (3 cases) or onfalitis (1 case). The first clinical manifestation oscillated between the ages of 3 months to nine years. The diagnosis was performed in the initial cases (4 cases) using splenoportography (3 cases) and superior mesenteric arteriography (SMA) in the other. In the remaining cases the ultrasonography together with the endoscopic evidence of the varies backed up the diagnosis. Before the intervention, a SMA was performed in all cases. The surgical decision was taken with regards to the persistence of the digestive bleeding after the start of medical treatment with propranolol, cimetidine, and sucralfate, along with the existence of dangerous varices. Eight patients were operated on, two by the Warren technique, four by mesocaval jugular graft interposition (MJI) and two by azigo portal disconnection (APD). The shunts initially controlled the bleeding in all cases. There was one rebleeding instance at eleven months in a patient operated on by MJI, and in another by APD. Four patients continue medical treatment without problems, and two are free of treatment. Our experience with varies sclerosis is limited to two cases, although since the beginning of the treatment, one year ago, neither of them has bled.

Angiography↗

[Anorectal manometry in Hirschsprung disease].

To show the absence of inhibitory reflex of the anus (R.I.A.) by ano-rectal manometry is an important sign in the diagnosis of Hirschsprung's disease. Between 1989 and 1991, a series of 199 patients was studied. The patients were divided into two groups: 0-1 month and 1 month to 50 years. Both groups were subdivided into cases with or without clinical and radiological suspicion of aganglionism. In this series, R.I.A. was absent in 88 patients and present in 111. Histologic study and follow-up confirmed the existence of aganglionism in 86 patients, hyperganglionism in 1, anal stenosis in 8, cystic fibrosis in 4 and 1 coeliac disease; the rest were megarecta to a greater or lesser degree. There was 1 false negative, signifying a percentage of error of 0.5%.

Adolescent↗

[Intestinal dysmotility-pseudo-obstruction].

Chronic intestinal pseudo-obstruction is defined as a syndrome related to any process which affects intestinal regulation and propulsion. Its origin may be muscular, neurogenic or hormonal, excluding Hirschsprung's disease or any known mechanical obstruction. Between 1989 and 1991, 11 patients with intestinal pseudo-obstruction were studied at our centre, and included nine hyperganglionisms B, and two hypoganglionisms) and two visceral myopathies (Berdon's syndrome). Diagnosis was established in all cases by histologic study. The techniques of haematoxylin-eosin, acetylcholinesterase, enolase, protein S-100 and Smith were used in neuropathies and haematoxylin-eosin and Masson's trichromic in myopathies. Intestinal motility was studied by ano-rectal and gastrointestinal manometry in seven and three cases respectively. Gastrointestinal manometry and radiology permitted differentiation of localized and diffuse forms of involvement. Medical treatment consisted of total parenteral nutrition when oral feeding was impossible, and in five cases, cisapride was given, with good results in four. Derivative surgery was performed in cases of diffuse involvement, and resection with anastomosis in those of localized forms. We conclude that: 1. Diagnosis is established according to histologic criteria. 2. Complementary examinations should be directed towards distinguishing localized from diffuse involvement. 3. Cisapride was effective in the treatment of neuropathies in the majority of cases.

Female↗

[The usefulness and evaluation of postanorectoplasty sagittal posterior biofeedback].

Posterior sagittal anorectoplasty (ARPSP) was described at first of the 80-90 decade like a new surgical technique for the anorectal malformation treatment and derived fecal incontinence. We have evaluated 35 of 67 ARPSP using biofeedback techniques (BFB) before surgery as restore system to striate muscle. Manometric values of the canal anal profile (PCA), postoperative external sphincter pressure and postoperative, first and last BFB voluntary contraction pressure shows clearly the differences between surgical intervention and BFB outcome. The author's opinion is that BFB is the indispensable therapy in the ARPSP postoperative to lend the patients get optimal fecal continence.

Adolescent↗

[Extrahepatic cholestasis of nonatretic origin. New diagnostic and therapeutic possibilities].

Nonatretic cholestatic disorders exhibit differential features in the pediatric age group, at times not too obvious but always discernible. These characteristic differences, with the aid of increasingly more precise exploratory techniques, make a rapid and sound diagnosis possible. At present, echography, transparietohepatic cholangiography and endoscopic retrograde cholangiography allow us, within a short time, to reach a diagnosis of disorders that not long ago were only roughly profiled and on occasion were left undiagnosed. At the Children's Hospital Valle de Hebrón, treatment has been afforded, in the last seven years, to seven patients with a choledochal cyst, four with a long common biliopancreatic duct, one with a choleperitoneum due to spontaneous perforation of a choledochal cyst and one patient with congenital stenosis of the common hepatic duct. In all cases where echography was performed, an intra- or extrahepatic bile duct dilatation could be demonstrated, or otherwise the diagnosis of a choledochal cyst was established. Furthermore, four transhepatic and three retrograde cholangiographic examinations were carried out, via a fiber duodenoscopy, in those cases that failed to show, on echography, the existence of a choledochal cyst.

Biliary Atresia↗

[Rhabdomyosarcoma in children (author's transl)].

48 cases of embryonal sarcoma are reported. 41.6% of them are currently surviving, three for longer than five years, two longer than four, five longer than three, five longer than two, and five longer than twelve months. Treatment is based on surgery, chemotherapy and radiotherapy.

Age Factors↗