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Biomedical subjects

J Broto

Publications and source records attributed to J Broto.

At least 19 recordsLinked to original sources

[Hirschsprung-neurointestinal dyspasia: diferential diagnosis and reliability of diagnostic procedures].

The suspicion of Hirschsprung's disease (HD) or Neurointestinal Dysplasia (NID) arises with the appearance of a common symtomatology : delay in meconium evacuation, abdominal distention, vomiting, intestinal occlusion and ultimately, chronic constipation. The need to perform a correct differential diagnosis between both pathologies is essential, given that the treatment of HD is surgical while NID is expectant. The objective of this paper is to define the necessity of using or not all the diagnostic tests simultaneously to obtain a precise diagnosis. The authors performed a complete clinical review of a series of 42 HD and 18 NID analysing the results of diagnostic procedures (Radiology / transition zone, anorrectal manometry / absence of anal inhibitory reflex, and suction biopsy / AcHE study). The authors concluded that is necessary to perform all the three diagnostic procedures simultaneously in all patients with symtomatology given that not one test has the capacity to provide a diagnosis alone. Anorectal manometry has proven to be the most reliable test to diagnose HD. The authors agree with the guidelines published by Meier Ruge in 2004 to diagnose NID.

Constipation↗

[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↗

[Nineteen years experience with posterior sagittal anorectoplasty as a treatment of anorectal malformation].

UNLABELLED: Posterior sagittal anorectoplasty (PSARP) is considered today the best surgical technique for the treatment of the anorectal malformations. With the aim of evaluating the results of the PSARP in our patients, the charts of 39 children (27 male, 12 female) were reviewed. Mean age was 11 years (3 to 20 years) and the mean follow-up period from the closure of the colostomy was 9.4 years (1 to 18 years). Patients with any alteration of the extrinsic innervation or those with rare malformations were excluded of the study. According to the Wingspread classification, 14 patients had a high defect, 13 had an intermediate type and 12 had a low one. One patient had a PSARP in the newborn period whereas the rest of them had a descending loop colostomy. Definitive repair consisted in PSARP in low and intermediate forms and in six of the high type patients. The rest (8) of the high type patients had a Rehbein abdominoperineal pull-through (DA) plus a PSARP. Evaluation for the surgical results was made by means of an anorectal manometry (existence or not of anal inhibitory reflex [RIA], the symmetry and pressure of the canal anal, and the existence of extrinsic innervation) and a clinical questionnaire. Patients or parents were asked for fecal continence, age at which the continence was achieved and the existence of severe constipation. RESULTS: Low defects: Eleven patients were continent between 3 and 5 years old. Seven patients had RIA. Intermediate defects: Seven patients were continent. Five (all with RIA) achieved continence between 2 and 8 years old; the other 2 were continent at the age of 10. The six incontinent patients had absence of RIA and/or low-pressure anal canal. High defects: Five patients were continent, between 11 and 16 years old. All had an DA plus PSARP. None of them had RIA. All the incontinent patients had an anomalous anal canal. CONCLUSIONS: The lowest the type of an anorectal malformation, the better the prognoses. In these patients, there is a relationship between the achievement of continence, the presence of RIA and the symmetry and high pressures at the anal canal. Among the patients with high defects, the results are better when they had an abdominoperineal pull-through plus PSARP.

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↗

Conservative treatment of caustic esophageal injuries in children: 20 years of experience.

The authors present their experience in the medical treatment of 1, 296 caustic esophageal injuries in children over the last 20 years in two study groups, one comprising the period up to 1989 and the other 1990 to 1996, comparing the different treatments used in each group. The treatment was based fundamentally on dilatations with anterograde mercury bougies, Savary bougies, or retrograde thread-guided bougies with gastrostomy. Pneumatic balloons or stenting procedures have also been employed in the last 3 years. Early fiberendoscopy was used systematically in the second group, which provides a more accurate evaluation of the esophageal lesions. Antibiotic coverage was done systematically during the first 10 days in all serious cases, while steroids were employed routinely only in the last 3 years. The results were similar in both groups, with a dilatation average of 32 in the first and 30 in the second group and an initial dilatation interval of 3 to 4 weeks in both. Using updated exploration and dilatation techniques, we drastically reduced the number of gastrostomies needed for retrograde thread-guided dilatations from 51 in the first group to 5 in the second, consequently improving the patients' life quality. There was no mortality and only five esophageal perforations, which did not require surgical treatment.

Anti-Bacterial Agents↗

[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↗

Changes in growth, growth hormone, and insulin-like growth factor-I (IGF-I) after orthotopic liver transplantation.

Growth failure is an important consequence of chronic liver disease in childhood. Insulin-like growth factor-I (IGF-I), which is synthesized and released by the liver, plays an important role as a growth regulator in humans. We examined the growth hormone (GH)/IGF-I axis before and after orthotopic liver transplantation (LT) in 14 children aged between 2 and 11 years (mean 5.6 +/- 1.1 years). Pre-transplantation serum GH levels (7.5 +/- 1.2 ng/ml) were significantly higher (P < 0.001) compared with controls (5 +/- 0.5 ng/ml). However, post-transplantation levels (1.8 +/- 0.8 ng/ml) did not differ from those in the control group. Serum IGF-I levels showed a statistically significant increase after LT (20.1 +/- 9.4 vs 190 +/- 66.2 ng/ml; P < 0.001) and became indistinguishable from the levels in the control group (180 +/- 96 ng/ml). In comparison with pre-transplantation data (z - 2.70), there was an increase in height 4 years postoperatively (z - 1.68). Catch-up growth was highly significant, in particular during the 1st year after LT (z -1. 58 +/- 1.63 vs 2.59 +/- 5.29; P < 0.01). We conclude that a GH resistance state found in patients with severe chronic liver disease reverted following LT. Given that IGF-1 depends upon liver function, this could be one of the main factors in the significant catch-up growth in pediatric LT recipients.

Child↗

[Laparoscopy for the undescended testis].

The authors show their experience in the use of the laparoscopic approach in 18 cases of unilateral exploratory anorchia. They highlight the advantages that this method offers in efficiency, speed, security and minimal aggression compared with conventional exploration of the inguinal channel in the search of the undetectable testis. In the 18 cases studied through the insertion of 5 mm lense in the umbilical area, it was established that a testicular hypoplasia was present in 8 cases. In another 5 cases testicular agenesia was diagnosed, and in the remaining 5, intraabdominal testis were found. In four of these testicles, a laparoscopic approach helped in their correct placement and also in the removal of the fifth in a patient with recurrent leukemia.

Child↗

[Pulmonary biopsy by thoracoscopy: techniques and results in 9 patients].

Video-assisted endoscopic techniques have decreased the surgical aggression in abdominal and thoracic surgery. In our country, pediatric laparoscopy has been developing slowly, but this is not the case for thoracoscopy. The aim of this paper is to present the techniques and results of thoracoscopy pulmonary biopsy in our first patients. Pulmonary biopsies with this approach had been done in 9 patients (5 males, 3 females). Their age ranged between 30 months and 16 years. In all cases this was the last resort for the diagnosis of pulmonary condensation of unknown etiology. The biopsies were done with the pretied knot in 5 cases, stappler in 1 case and with biopsy forceps in 2 cases. Thoracotomy was necessary in one patient, due to intraoperative haemorrhage. Enough tissue for bacterial and pathological diagnosis was obtained. There was not mortality nor important morbidity related with the technique. Postoperative recovery is better when compared with conventional thoracotomy. Thoracoscopy is an adequate approach to perform pulmonary biopsies in children. The advantages if we compare with open thoracotomy are: 1. The possibility to choose the are to perform a minimally invasive biopsy. 2. To take samples of different pulmonary lobes. 3. Less postoperative pain and shorter hospital stay (36-48 hours).

Adolescent↗

[The usefulness of manometry in the determination of the morphology of the anal canal].

The appearance of the new lecture systems for the manometry studies by computer, like the Polygram by Synectics (vector volume), offer the possibility to see the circumferential pressure forces, that even in rest conditions as in voluntary contraction, they keep coaptation of the anal canal, and this will act as a continent closure system in the most distal part of the G.I. tract. The study is with the normal parameters obtained in 14 individuals, considered as normal, getting the mean +/- sd pressure of the anal canal convey in mm Hg, from de anal canal profile in rest as in voluntary contraction, and the maximum variability that could exist between the six profile waves, that are obtained in the same individual to develop an image of the anal canal. This valves will allow the author's to get to the bottom of fecal incontinence derivative from anorectal malformations, defining the pressure valves of muscular hypoplasia or surgical outcomes of the malformations.

Adolescent↗

[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↗

[Otoneurologic examination of patients with AIDS].

The influence of the HIV virus on the central nervous system tracts has been definitively shown. Over the past year we have affected otoneurologic tests based on elicited auditive potentials and tests of oculomotility on a total of 30 patients affected by stages II and III of the disease. The results show that around 27 percent of cases present alterations in the provoked auditory potentials and 20 percent in the oculomotility tests. The results are compared with those of previous studies.

Acquired Immunodeficiency Syndrome↗

[Considerations regarding the treatment of non-aganglionic congenital intestinal neuropathies].

Hyperganglionosis or neuronal intestinal dysplasias (NID) and hypoganglionosis (HO) are intestinal diseases of difficult diagnosis and treatment and diverse evolution, despite identical histologic findings. The aim of this study was to discuss the therapeutic problems derived from the patients differing clinical course. Retrospective review of 14 patients with regard to diagnosis, manometry and histology (hematoxylin-eosin, acetylcholinesterase activity, immunohistochemistry and Smith's silver stain) was done. Six patients presented intestinal occlusion or sub-occlusion from the first months of life with impeded oral feeding. Ileostomy was performed in 5 and total colectomy with anastomosis in 1. All patients required parenteral nutrition; cisapride was added in 2. Three died from sepsis (3 NID). Of the 3 survivors, 2 have ileostomies (2 NID) and the other ileo-rectal anastomosis (NID). Of the remaining patients, two presented aganglionism and the finding of proximal hyperganglionism occurred post-surgery. Surgery was repeated in one patient. The remaining 6 (1 HO, 5 NID) were diagnosed between 3 and 10 years of age because of constipation. Four are under treatment with cisapride and 2 required partial colic resection. No relationship can be established between histologic findings and clinical manifestations. In chronic clinical courses, treatment with cisapride and cleaning enemas should be tried first. Acute clinical pictures (occlusion-sub occlusion) should be treated by decompressive ileostomy. Partial colic resection may lead to new intestinal failure.

Anastomosis, Surgical↗

[Surgical technique in reduced hepatic transplantation in children].

The lack of availability of pediatric donors for liver transplant has resulted in a high mortality rate among children awaiting transplantation, especially in patients weighing less than 10 kg. Knowledge of techniques of liver reduction allows liver transplantation in low-weight children with an adult liver graft, although with increased risk. The authors studied 70 pediatric liver transplants, 14.3% of whom received a reduced-size organ. Maximal difference between donor and recipient weight was 7.7. Segments II, III and IV (right hepatectomy) were transplanted in eight cases while segments II and III were transplanted in two cases. Transfusional requirements during the 48 hour postoperative period were not significantly different between patients with reduced-size liver and patients who received the full-size organ. There was not morbi-mortality secondary to surgical technique in reduced liver transplant group of patients.

Body Weight↗

Paediatric liver transplantation: life after portoenterostomy in biliary atresia.

Seventy-five percent to 80% of patients with biliary atresia (BA) will be candidates for paediatric liver transplantation (PLTx) throughout the first 14 years of life. They form the main group of recipients in our Paediatric Liver Transplant Unit. Of 48 children transplanted, 21 (44%) had BA. These patients present particular features, average weight of 12 kg, mean age of 3 years, and severe malnutrition prior to PLTx, which distinguish them from other paediatric candidates. With the advent of PLTx, portoenterostomy (PE) has ceased to be the only recourse for treating the majority of patients with BA. Different factors converge in these patients: some, including haemorrhage and adhesions, may present technical difficulties, and others, such as infections (19% in this series) due to severe malnutrition and malabsorption and consequent chronic rejection (14% in this series), often lead to death in the postoperative period (33% in this series). BA is treated by all paediatric surgeons, but the overall success rate now depends not only on PE but also on PLTx. In an attempt to facilitate possible later PLTx in pts with BA, the authors as paediatric surgeons performing PE or PLTx present surgical modifications and emphasize the most important medical aspects conducive to the improved general status of these pts. Our aim was to establish guidelines for taking full advantage of PE while preventing posterior problems and facilitating future transplant surgery.

Biliary Atresia↗