PubMed Health⌕ Search

Biomedical subjects

J M Mariño

Publications and source records attributed to J M Mariño.

11 recordsLinked to original sources

[Extensive aganglionosis. Treatment and long term results].

INTRODUCTION: Extensive aganglionosis (EA) that affects the total colon (including or not part of the small intestine), shows more diagnostic problems and it is associated to higher morbidity and mortality rates than the classic cases of Hirschsprung disease (ED). This study takes into account last years cases and their results in the medium and long term. MATERIAL AND METHODS: Between 1983 and 2000, 232 patients suffering ED have been treated. 15 out of those 232 patients showed EA. We took into consideration the diagnosis, surgical procedures, enterocolitis before and after the treatment, the surgical technique and the complications. We analyze the nutritional state and the long term result according to clinical bowel function scoring system continency Scale, considering the surgical possibilities depending on each case. RESULTS: Two patients died due to septics complications, one of them during the neonatal period and the other one at the age of 3. Two other cases became possible candidates to an intestinal transplantation due to the extension of their disease. Five out of the 11 left patients had more than 30 cm of small intestine involved, and another five had involved less than 30 cm from ileocecal valve. The last case showed a total colonic aganglionosis with associated intestinal neuronal displasia. One case was associated with S. Ondine and another one with a 21 triosomia. Two of the patients were brothers and another patient has got a brother suffering from rectosigmoid HD. The surgical techniques used with 13 were: 8 cases using Lester Martin modification (one of them needed redo procedure because of anastomosis leak and perineal fistula), 4 Swenson procedure and one patient was treated by Ziegler's miectomy with prolongated miotomy. Nine of the patients mentioned before, lead a quite life (five L, Martin, 4 Swenson); 2 patients suffering frequent fecal retention crisis. 5 out of the 11 reviewed cases, show a weight and height p > or = 50, 2 below p50, 2 below p25, and 2 under p3. CONCLUSION: EA represents a small percentage of Hirschsprung disease. Although Martin modification is the most widely employed technique in cases of distal ileal involvement, Swenson procedure has provided good results in the long term follow-up with few complications. Intestinal transplantation may be the only choice for long term survival in patients with near total intestinal aganglionosis. Continence improves in the follow-up but few times is normal. Nutritional state should be-controlled by a specialized team.

Child, Preschool↗

Pre- and post-natal growth of the human ductus epididymidis. A morphometric study.

A histometric study of the development of the human epididymis from the fetal period to adulthood has been carried out in males without testicular or related pathology, distributed into the following groups: (I) fetuses (between the 28th and 37th week of pregnancy); (II) newborns (1-30 days of age); (III) infants (2-4 months of age); (IV) infants (5-12 months of age); (V) infants (1-4 years of age); (VI) children (5-14 years [prepubertal]); and (VII) adults (15-60 years of age). For each age group and each epididymal portion (efferent ducts, caput, corpus and cauda epididymidis) the parameters measured were (1) total surface (epithelium + muscular layer + lumen); (2) the surface occupied by the lumen; (3) the surface occupied by the muscular layer; (4) total diameter of the duct; (5) total diameter of the lumen; and (6) the height of the epithelium. The results of the present study revealed that the development of the efferent ducts and ductus epididymidis follows a biphasic pattern. A progressive development occurs from the fetal period to infants 2-4-months of age. However, this development is transient and regresses during infancy (groups IV and V). At childhood (group VI), a definitive development is initiated and completed at puberty (group VII). These changes seem to be related to the androgen-dependence of the epididymis, the different stages of testicular maturation, and the steroidogenic activity of Leydig cells.

Adolescent↗

Neural tube defects: an experimental model in the foetal rat.

We report on our experience in the experimental induction of Neural Tube Defects (NTD) in the foetal rat by maternal administration of retinoic acid. The teratogen diluted in olive oil was administered in a single intragastric dose (125 mg/kg body weight) to pregnant rats (n = 31) on the 10th day of gestation. Pure olive oil was given to control rats (n = 9). The foetuses were recovered by caesarian section on the 20th day and prepared for morphological investigation. We have studied 201 experimental and 82 control animals. There were NTD in 36.3% of experimental foetuses and none in the control ones. Sacral dysraphism was the most frequent defect but we also observed Arnold Chiari malformations and crowding of the bony limits by an enlarged neural axis. Other associated malformations found were: craneofacial (78.1%), caudal (80%), anorectal (31.4%), and limb defects (89.5%). This simple and inexpensive model may allow us to gain a better knowledge of the biology in the foetus with NTD.

Abnormalities, Drug-Induced↗

Residual cavities after surgery for hepatic hydatid cysts--an ultrasonographic evaluation.

Ultrasonography has made it easier to diagnose precisely hepatic hydatid cysts, but the interpretation of images of residual cavities persisting long after the operation can be difficult. We reviewed the clinical histories of 22 children treated surgically for hepatic hydatid cysts at our hospital (1985-1992) to find clues to the proper attitude toward residual cavities. The children's mean age was 7.4 +/- 2.5 years. Fourteen children had single cysts (11 right and 3 left) and 8 children had multiple cysts, one of them 30 cysts (16 right and 14 left). The mean ultrasonographic diameter of the 44 cysts was 6.1 +/- 3 cm. Cystectomy with partial pericystectomy was performed in every case. Six months after operation, 29 cavities had disappeared, became calcified, or were substituted with fibrous scar tissue. The remaining 15 residual cavities had a mean diameter of 3 +/- 1 cm. The residual cavities exhibited little change at 12 months (2.8 +/- 1 cm), but at 18 months 9 had disappeared, 2 that increased in size and 4 remained the same size. Of these 4 stable lesions, surgery performed in 2 found no cysts and 2 have been followed-up ultrasonographically for 5 years. This short series shows that ultrasonography is useful in the diagnosis and follow-up of residual cavities after surgery for hepatic hydatid cysts. Most residual cavities disappeared by 18 months. In the cavities that persisted, our findings suggest that surgical treatment is justified only when the cyst becomes larger on ultrasonography.

Child↗

[Gastroschisis: study of teratogenic factors].

The medical and surgical treatment of gastroschisis has improved in the last decades leading to better survival rates. Since in our institution there were the same cyclic variations in the incidence as in other large series in the literature, we have reviewed the epidemiological factors that can be at the origin of such variations. We studied 20 different variables in our patients in a case-control basis. Only five of them were significantly different between cases and controls. (Birth weight, gestational age, infection, drugs during the first three months of gestation and maternal age). The results point to the role of environmental factors more relevant in the etiology of gastroschisis than genetic ones.

Abdominal Muscles↗

[Surgical treatment of differentiated carcinoma of the thyroid. Retrospective study].

This is a review of the surgical treatment in six patients with papillary carcinoma of the thyroid. The mean age at the time of diagnosis was eight years (range four-twelve years). Clinical presentation was: all patients had a palpable thyroid tumor, palpable lymph nodes in four and one with lung metastases. The initial surgical treatment was lobectomy and modified regional lymph nodes resection. Four patients underwent a second operation for multifocal disease, palpable lymph node or thyroid tumor. The interval from initial surgical management to the detection of recurrence ranging from one month to four years. In three patients the remaining thyroid gland was removed. Radioiodine, external irradiation and chemotherapy were given during the postoperative. The median follow-up was eight years (range one-fifteen years). All patients are alive with thyroid hormone therapy by hypothyroidism. There were no hypoparathyroidism or injuries to the recurrent laryngeal nerve. The results of this study suggest that the surgical approach in papillary carcinoma of the thyroid is total thyroidectomy because reoperation were performed in four patients.

Antineoplastic Agents↗

[Traumatic diaphragmatic hernia in children].

We have treated three children with traumatic diaphragmatic hernia appearing after blunt abdominal trauma. The hernia was on the right side in two patients and was accompanied by liver and lung injuries. The most common site of diaphragmatic rupture was the postero-lateral area in both sides. Diagnosis was suspected in all of them with chest radiograph before operative management. The transabdominal approach has proven to be efficacious in repairing the defects and often require emergency care. The diaphragmatic repair was performed using non absorbable interrupted mattress sutures. No prosthetic materials were required.

Child, Preschool↗

[Digestive system duplications].

Fifteen digestive duplications, corresponding to fourteen patients, have been surgically treated in "La Paz" Pediatric Hospital between 1966 and 1984. The most common localization (40%) was the large bowel. The first choice treatment was the complete excision of lesions, with led to cure of all patients. Embryologic theories and clinical forms are also reviewed.

Esophagus↗

[Colonic atresia].

Nine cases of colonic atresia, treated in the Departament of Pediatric Surgery of "La Paz" Pediatric Hospital, are reported. They account for 11% of all our intestinal atresias. We review the different etiologic and pathologic theories and classificate our group according to the LOUW and HAYS method. In two cases their digestive anomalies and in one renal anomalies were found to be associated mortality was 11%.

Colon↗

Epididymal growth and differentiation are altered in human cryptorchidism.

Despite the knowledge and histological classification of testicular lesions, epididymal lesions associated with cryptorchidism are not well defined and only macroscopic alterations have been reported. We have evaluated the alterations in the growth of both the epithelium and muscular wall of efferent ducts and epididymis in human patients with cryptorchidism from infancy to adulthood. In addition, by cytokeratin immunostaining we have also evaluated the stage of differentiation of each segment along the human postnatal life in these patients. A decrease is shown in the size of efferent and epididymal ducts in cryptorchid children compared with normal, age-matched controls. The height of the epithelium, muscular wall, and lumen of the cryptorchid epididymis were reduced at every age studied. This decrease in all regions was seen even in the testicular quiescent period (1 to 4 years of age). In addition, the cryptorchid epididymis grows more slowly during the transition to the pubertal period. The smaller size of the cryptorchid epididymis in pubertal and adult men compared with that of normal men is due primarily to underdevelopment of the muscular wall and a reduction in epithelial height. The pattern of growth of cryptorchid efferent ducts and ductus epididymides parallels that in normal men, except that development of the lumen and muscular layer in the cauda epididymis region are delayed. Epithelial differentiation, monitored by cytokeratin expression, is minimal in efferent ducts and throughout the epididymis of the cryptorchid male, and this is already seen in children. In conclusion, our immunohistochemical and morphometric results show a reduced development of the human cryptorchid epididymis that is already evident in childhood. They indicate that cryptorchidism is a primary congenital illness of the testis and spermatic ducts, with evident lesions from the first years of life, and suggest that surgical descent would probably not be able to completely reverse these alterations.

Adult↗