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Biomedical subjects

A Queizán

Publications and source records attributed to A Queizán.

At least 19 recordsLinked to original sources
PubMed2006

Congenital segmental fibromuscular hypertrophy of the esophagus and esophageal atresia: an uncommon case.

Although a rare entity, congenital esophageal stenosis due to segmental hypertrophy of the muscularis and submucosal layers with diffuse fibrosis should be considered a possibility in patients with esophageal stricture associated to congenital esophageal atresia. The efficacy of dilatation seems to be limited, and may even result in severe complications such as an esophageal rupture. Surgical repair for congenital esophageal stenosis is the authors' preferred treatment, although initial dilatation may be effective for some patients.

Esophageal Atresia↗
PubMed2006

Prenatal diagnosis of gastric triplication.

Duplications of the intestinal tract are rare malformations, and triplications are even less common; only two cases are found in the literature. The authors describe a case of prenatal diagnosis of a gastric triplication and the surgical treatment.

Adult↗
PubMed2005

Body wall dysplasia: a rare case of abdominal hernia.

Malformations of the fetal abdominal wall include a broad spectrum of anomalies, and prenatal sonography provides the possibility of detecting most of them. Omphalocele and gastroschisis are the most common conditions, but there are other rare forms. We describe here a rare case of body wall dysplasia that we called abdominal hernia that appeared upon prenatal sonography as an omphalocele-like defect.

Abdominal Wall↗
PubMed2004

[Aggressive conservative treatment remains the best option for oesophageal perforation in children].

UNLABELLED: Oesophageal perforation (OP) requires prompt and vigorous treatment. In contrast with adult patients in whom surgical closure of perforation is preferred, non-operative treatment has been the usual approach in children. The present report aims at assessing whether this strategy stands the passage of time. We studied retrospectively the charts of patients treated at our institution for OP between 1991 and 2001. Between these years, we treated 19 episodes of OP in 17 patients aged 5.3 +/- 0,94 years. In 9 cases (4 lye burns, 3 oesophageal atresias, 1 bullous epidermolysis and 1 mucocutaneous candidiasis) OP occurred during dilatation of strictures. Foreign body extraction was the cause in 3 cases, and blunt trauma and sclerosis of varices were the causes in 2 cases each. The last child had multiple gastrointestinal perforation during treatment for leukaemia. Subcutaneous emphysema was seen in 7 instances, pneumomediastinum/pneumothorax in 14, pleural effusion in 9, dyspnoea in 9, severe thoracic pain in 1 and pericardial effusion in 1. The diagnosis was intraoperative in only 2 children but the symptoms and imaging signs prompted vigorous treatment within the first 24 hours in 15 instances. One or more pleural tubes were inserted in 11 cases and pericardial drainage was required once. Perforations closed without direct surgery in 18/19 episodes (16/17 children). Five gastrotomies and 2 jejunostomies were performed and several major abdominal operations were necessary to repair concurrent lesions in a child who sustained severe blunt abdominal trauma and in the one with leukaemic perforations. All these patients survive and all recovered oesophageal function although 2 with intractable lye structures ultimately required oesophageal replacement 6 and 10 months after OP. The only patient in whom direct approach for esophageal necrosis after variceal endosclerosis was unavoidable lost her organ and had a replacement after a successful porto-systemic shunt. CONCLUSIONS: Prompt and aggressive non-operative approach of oesophageal perforations in children allows survival and conservation of the organ and its function in most cases and should remain the first therapeutic choice at this age.

Adolescent↗
PubMed2004

[Congenital neuroblastomas].

BACKGROUND: We consider congenital neuroblastomas (CN) those detected in pregnancy or at the very first hours of life. Due to perinatal sonography, its incidence has increased in the last years. We present herein our experience in the treatment of this condition and we try to find out any different clinical pattern from those neuroblastomas diagnosed later in life. METHODS: We review the CN treated in our hospital from 1990 to 2003, analyzing diagnosis, localization, tumor staging, N-myc amplification, treatment and evolution. RESULTS: Among the 107 neural tumors managed during this period (89 neuroblastomas, 18 ganglioneuromas), 8 were congenital neuroblastomas (7 girls, 1 boy). Two patients had prenatal diagnosis and 6 tumours were detected in routine exploration or casual findings upon neonatal examination. Six were abdominal, 1 thoracoabdominal and 1 abdominopelvic with dumbbell invasion. Three tumours were classified like stage 1, 1 stage 2, 1 stage 3, 1 stage 4 and 2 stage 4S. Although most of them had unfavorable histology, we didn't find N-myc amplification in any tumor. All patients were operated upon, with preoperative chemotherapy in 2 of them. Resection was complete in 7 out of the 8 tumors. The patient who presented neurological symptoms at birth recovered neither motility nor bladder function after resection. All of them survive after 60+/-53 months of follow-up. CONCLUSIONS: The outcome in this group of neuroblastomas is better that expected, probably because of its abdominal location. On the contrary in dumbbell neuroblastomas, neurological damage at birth seems to be irreversible.

Female↗
PubMed2003

Aggressive conservative treatment of esophageal perforations in children.

BACKGROUND/PURPOSE: In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has been the usual approach for esophageal perforation (EP) in children. This report aims to assess whether this strategy stands the passage of time. METHODS: We reviewed retrospectively the charts of 17 patients aged 5.3 +/- 0.9 years (mean +/- SD) treated at our institution for EP between 1991 and 2001. RESULTS: Nineteen episodes of EP were caused by stricture dilation in 9 cases, foreign body extraction in 3, and blunt trauma and sclerosis of varices in 2 cases each. The remaining child had multiple gastrointestinal perforations in the course of chemotherapy for leukemia. Vigorous treatment, consisting of nasopharyngeal aspiration, wide spectrum antibiotics, prompt drainage of effusions and either parenteral or infraesophageal nutritition, was implemented immediately after diagnosis. Perforations were closed without direct surgery in 18 of 19 episodes (16 of 17 children). One or more pleural drains were inserted in 12 cases, and pericardial drainage was required once. Seven gastrostomies, 2 jejunostomies, and one esophagostomy were performed. Several major abdominal operations were necessary to repair concomitant lesions in a child who sustained severe blunt abdominal trauma and in the patient with leukemic perforations. All patients survived, and all recovered esophageal function. However, 2 with intractable lye strictures ultimately required esophageal replacement. The only patient in whom a direct approach for esophageal necrosis due to variceal endosclerosis was unavoidable, lost her organ and had a retrosternal colonic interposition after a successful portosystemic shunt. Excluding patients with other concomitant lesions and the patient who underwent surgery, median length of stay was 11 days (range, 6 to 47). CONCLUSIONS: Prompt and aggressive nonoperative treatment of esophageal perforations in children allows survival with conservation of the organ in most cases and remains, in the authors' hands, the first therapeutic choice at this age.

Abdominal Injuries↗
PubMed2000

[Impact of complete resection on survival of patients with large neuroblastoma].

UNLABELLED: The aim of our study is to assess the role of complete resection after chemotherapy in stage 3 and 4 (INSS) neuroblastoma. MATERIAL AND METHODS: We treated in the period 1990-1997 a group of 71 infants and children with neural tumors. There were 63 neuroblastomas (median age: 24.8 +/- 25 months, median 18). 47 were abdominal, 13 thoracic, 2 pelvic and 1 cervical. Survival rate (Kaplan-Meier) in patients with or without complete resection of the tumor were assessed (Mantel Cox). RESULTS: 14 of 17 patients with stage 3 tumors and only 8 of 23 with stage 4 survive. All patients with stage 3 undergoing complete resection are alive, whereas only 4 of 7 with incomplete resection survive (p < 0.01). In contrast, the effort and risk of resection do not appear to be worth in stage 4. Two kidneys, one spleen and a portion of the pancreas were removed to perform complete tumor removal. CONCLUSIONS: Complete resection in stage 3 neuroblastoma after chemotherapy improves survival, and radical surgery seems justified even if neighboring structures have to be removed. Radical surgery does not seem to be useful in stage 4 neuroblastoma.

Child, Preschool↗
PubMed1997

Mutational analysis of the p16 gene in human neuroblastomas.

Neuroblastoma is one of the most frequent tumors in infancy. We analyzed 26 neuroblastomas, two ganglioneuromas, and a neuroblastoma metastasis for mutations and homozygous deletions of the p16 (or MTS1 or CDKN2) gene by means of the polymerase chain reaction (PCR) in combination with the single-strand conformation polymorphism (SSCP) technique and by multiplex PCR analysis. We detected mobility shifts in the SSCP gels in seven cases in the 3 half of exon 2 (named exon 2C) of the p16 gene. By PCR amplification of this particular region and SacII restriction enzyme digestion, we confirmed that those cases had a known polymorphism at codon 140 of the p16 gene. Neither mutations nor homozygous deletions were detected. Our results confirm those of Beltinger et al. (Cancer Res 55:2053-2055, 1995), which showed no p16 mutations or homozygous deletions in 18 primary neuroblastomas and nine tumor-derived cell lines. We conclude that the common pattern of p16 inactivation by homozygous deletion or mutation does not seem to be relevant to the development of neuroblastomas.

Carrier Proteins↗
PubMed1997

[Adrenocortical tumors in children].

Adrenocortical tumors are uncommon in childhood. The incidence of these tumors is about 0.3-0.4% of all the solid tumors in childhood. Because of its low incidence, there are no established protocols or uniform histological classification. Most of these endocrine tumors are hormone producing, causing virilization, Cushing's syndrome, feminization, hiperaldosteronism or hipoglicemy. Only a few of these do not produce hormones. The treatment is always surgical. The efficacy of another treatments (chemotherapy or radiotherapy) has not been demonstrated in infancy. It prognosis depends on the age and the resectability.

Adrenal Cortex Neoplasms↗
PubMed1995

[Sensibility and specificity of N-myc oncogene with respect to other prognostic factors in 15 neuroblastomas].

Several biologic features of tumor cells correlate closely with a favorable or unfavorable outcome. To aid in assessing correlation in the various number of prognostic factors including the age, stage, VMA/HVA ratios, and the serum levels of NSE and ferritin, the histopathological features, ploidy, partial monosomy for the short arm of chromosome 1, and the tumor N-myc gene copy number, are examined. We determined the sensitivity and specificity of classical markers above the amplification of the N-myc oncogene. A striking new observation is the positive correlation between genomic amplification and some prognostic factors (stage, ferritin, NSE, pathologic anatomy and 1p deletion.

Brain↗
PubMed1994

No TP53 mutations in neuroblastomas detected by PCR-SSCP analysis.

We have analysed 29 neuroblastomas for TP53 mutations in exons 5 to 8 by means of the polymerase chain reaction in combination with the single-strand conformation polymorphism technique. We could not detect any mutation. These results indicate that, in contrast to the majority of tumors so far studied, TP53 mutations do not seem to be important for the development of neuroblastomas.

DNA, Neoplasm↗
PubMed1994

[Early or late orchidopexy? An evaluation of germ cell proliferation by PCNA immune expression].

A immunocytochemical study for detection of proliferating cell nuclear antigen (PCNA) in order to quantify the number of PCNA-positive spermatogonia, and cytophotometric determination of spermatogonial DNA were performed in cryptorchid and control testes. The number of PCNA-positive spermatogonia, and the average DNA content of spermatogonia in the cryptorchid testes were altered from first years of age. These precocious spermatogonial alterations suggest that the early surgical testicular descent doesn't prevent lesions of germ cells.

Aging↗
PubMed1993

Cytophotometric DNA quantification in human spermatogonia of cryptorchid testes.

The DNA content of spermatogonia was studied by cytophotometric quantification in the testes of cryptorchid children and adults, as well as in age-matched control males. In most cases, the average DNA content of spermatogonia was significantly increased in the cryptorchid testes of children with uni- or bilateral cryptorchidism, as well as in the contralateral scrotal testes of children with unilateral cryptorchidism. In the group of adult men the average DNA content of spermatogonia in the testes was even more increased than in children. There were not significant differences between 4 and 14 years of age, between unilateral and bilateral cryptorchidism, or between cryptorchid testes and contralateral normally descended testes. The DNA content of spermatogonia in the surgically descended testes of 3 children who were re-biopsied 3-4 years after orchidopexy was similar before and after orchidopexy. These findings suggest that the spermatogonia of many cryptorchid males bear a congenital lesion.

Adolescent↗
PubMed1990

[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↗
PubMed1986

[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↗
PubMed1985

Histologic lesions in undescended ectopic obstructed testes.

Testicular biopsies and hormone studies have been carried out on 229 children with unilateral (181) or bilateral (48) undescended ectopic obstructed testes not associated with other pathologic conditions. With regard to the histologic lesions, the obstructed testes may be classified into four types: (1) testes with minimal lesions (40.1%), showing slight reduction in both mean tubular diameter (MTD) and tubular fertility index (TFI); (2) testes with marked germinal hypoplasia (33.6%), showing slight or marked reduction in MTD and marked reduction in TFI; (3) testes with diffuse tubular hypoplasia (19.1%), showing severe reduction in MTD, marked or severe germinal hypoplasia, and normal or decreased Sertoli cell number per transverse tubular section (SCI); and (4) testes with Sertoli cell hyperplasia (7.2%), showing slightly decreased MTD, marked or severe germinal hypoplasia, and marked increased in SCI. These lesions are similar to those found in cryptorchid testes, although the proportion of testes with type III and IV lesions (the most severe) is lower than in cryptorchid testes. Hormone assays revealed normal basal gonadotropin and testosterone levels. The response of gonadotropins to gonadotropin-releasing hormone stimulation and the response of testosterone to human chorionic gonadotropin stimulation were normal or slightly reduced.

Child↗