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Biomedical subjects

A Vos

Publications and source records attributed to A Vos.

At least 109 records · Page 6Linked to original sources
PubMed1990

Triad of anorectal stenosis, sacral anomaly and presacral mass: a remediable cause of severe constipation.

Two neonates with intestinal obstruction and two children (aged 1 and 4 years) with severe constipation since birth are reported in whom stenosis of the distal rectum was found. In association with the rectal anomaly, three of them had a presacral tumour (teratoma in two, hamartoma in one) and all had a deformed sacrum. An embryological hypothesis to explain this association has been postulated by Currarino, after whom this triad has been named. Two patients were related (father and daughter). The role of hereditary factors in the occurrence of the syndrome has been reported before. Operative treatment of the rectal stenosis was necessary in all patients. Preoperative diverting colostomy was performed in three cases, followed by a posterior sagittal approach to excise the rectal stenosis and the presacral mass. In one case, persistent cerebrospinal fluid leakage required re-exploration for closure of a tear in a congenitally abnormal dural sac. The fourth patient had undergone a low anterior resection in the past via the abdominal route and needed rectal dilatation afterwards for some time. The final result in all patients appears satisfactory, although follow-up is short. Most cases of this triad have been reported in children but a number of patients have been diagnosed only as adults. Recognition of this triad should imply a careful search for neural crest malformations. Operative treatment to correct all soft tissue anomalies leads to good results.

Child, Preschool↗
PubMed1990

Incidence of forearm and hand ischaemia related to radial artery cannulation in newborn infants.

We report the incidence of transient and permanent ischaemia of the forearm and hand caused by radial artery cannulation in a series of 98 newborn infants. Birth weight ranged from 550 to 3920 g (median 1600 g) and gestational age ranged from 26 to 40 weeks (median 31 weeks). In 4 of the 98 infants transient ischaemia of the forearm and/or hand occurred. In three infants the onset of ischaemia was probably related to thrombo-embolic events and in one infant to inadequate palmar collateral circulation. Permanent ischaemic damage with tissue loss did not occur in any infant. It is concluded that permanent ischaemic damage to the forearm and/or the hand with tissue loss, attributable to radial artery cannulation, is seldom encountered in newborn infants. In minimizing the risk of ischaemia, careful assessment of palmar collateral circulation prior to cannulation and of all factors predisposing to the onset of ischaemia is essential. Immediate removal of the catheter at the earliest signs of ischaemia, is essential to prevent ensuing tissue loss.

Catheterization, Peripheral↗
PubMed1990

Incidence and duration of total occlusion of the radial artery in newborn infants after catheter removal.

The incidence and duration of total occlusion of the radial artery after catheter removal was determined using repeated Doppler flow measurements. Thirty-two newborn infants with birthweights ranging from 945 g to 3890 g (median 1935 g) and gestational age ranging from 26 to 40 weeks (median 32 weeks) were studied. In 20 out of 32 infants (63%), complete occlusion of the radial artery occurred. The number of occlusions were not related to birthweight, gestational age or duration of cannulation. In all infants, blood flow in the radial artery resumed within 1-29 days after catheter removal. The duration of occlusion was directly related to the duration of cannulation and inversely related to birthweight. This study demonstrates a high frequency of total occlusion of the radial artery in newborn infants after percutaneous radial artery cannulation. In the majority of infants with a radial artery catheter, blood flow to the tissue distal to the cannulation site is dependent solely on the existence of an adequate arterial palmar collateral circulation.

Arm↗
PubMed1990

Atresia of jejunum and ileum: is it the same disease?

A retrospective analysis of 21 patients with jejunal atresia and 24 with ileal atresia showed more differences than similarities between the two categories. The incidence of jejunal atresia appeared to be increasing during the period of observation (1978 to 1987). In the past 2 years, many children with jejunal atresia, who belonged to a set of heterozygotic twins, were admitted to this institution. The mean birth weight and gestational age in jejunal atresia were significantly lower than in ileal atresia. The majority of jejunal atresias were multiple, whereas most ileal atresias were single. Antenatal perforation occurred rather frequently (10 cases) in ileal atresia but only twice in jejunal atresia. The postoperative course was more often prolonged in jejunal than in ileal atresia. Mortality was higher in jejunal atresia: three deaths, all in apple peel atresia, versus one death in ileal atresia. An explanation for some of these observations can be found in a difference of compliance of the bowel wall between jejunum and ileum: the more compliant jejunal wall allows for massive dilatation with subsequent loss of peristaltic activity. It is postulated that because of the many differences, jejunal and ileal atresia may be considered as separate diseases.

Diagnosis, Differential↗
PubMed1990

Jejunal atresia in twins.

In the past 2 1/2 years a sudden increase in the frequency of jejunal atresia in discordant, nonidentical twins was observed. Reported are the details of 11 cases and some reflections on possible pathogenetic mechanisms involved.

Diseases in Twins↗
PubMed1990

Cytogenetics of carcinoma in situ of the testis.

Carcinoma in situ (CIS) of the testis is the precursor lesion of most testicular germ cell tumors (TGCTs). Karyotyping of CIS is important for a better understanding of the pathogenesis of TGCTs and the progression to invasive cancer. We karyotyped three cases of CIS. All three cases showed a numerical abnormal chromosomal pattern. In one case, two copies of the germ cell tumor marker i(12p) were found.

Adult↗
PubMed1990

Cytogenetics of thyroid follicular adenomas.

We present the results of a cytogenetic study of three cases of follicular adenoma of the thyroid. All three cases had a numerical strongly abnormal karyotype with most abnormalities (eg, +4 or +5, +7, +9, +12, +16) in common, possibly indicating that this cluster of abnormalities might be specific for follicular adenomas. For benign tumors, the three cases showed a remarkably abnormal karyotype. These cases are another example of benign tumors with chromosomal abnormalities that might be specific for follicular adenomas.

Adenoma↗
PubMed1990

Karyotyping and DNA flow cytometry of metastatic ovarian yolk sac tumor.

We karyotyped a metastasis composed of pure yolk sac tumor derived from a primary ovarian germ cell tumor with two components: a dermoid cyst [DNA index (DI) 1.0] and a pure yolk sac tumor (DI 1.88). The metastatic yolk sac tumor had a hypertriploid karyotype and a DI of 1.78 and lacked the germ cell tumor marker i(12p). The absence of this marker in a metastasis from a tumor with a dermoid cyst component might be indicative for a pathogenesis of the yolk sac tumor similar to that of a dermoid cyst and different from that of dysgerminoma.

Adult↗
PubMed1990

Pathogenesis of adult testicular germ cell tumors. A cytogenetic model.

In essence, two models exist of the pathogenetic relationship between seminomas and nonseminomatous germ cell tumors (NSGCTs). In the first model, the histogenesis of seminomas is assumed to diverge from that of the other testicular germ cell tumors (TGCTs) at an early stage. The neoplastic pathway of seminomas and NSGCTs is different, with limited or no crossover. The second model suggests that seminomas and NSGCTs have a common origin with a single neoplastic pathway on which seminomas are an intermediate stage in development of NSGCTs. Our data on the cytogenetics and ploidy of seminomas, combined tumors, and NSGCTs lend support to the model of pathogenesis of seminomas and NSGCTs in which all TGCTs (with the possible exception of spermatocytic seminoma and infantile yolk sac tumor) have a single origin and neoplastic pathway, with seminomas representing an intermediate stage in development of NSGCT components, as opposed to the model in which seminomas and NSGCTs develop separately. The progression of TGCTs probably proceeds from high to lower numbers of chromosomes and is therefore accompanied by a net loss of chromosomal material. This decrease will be the end result of loss of specific chromosomes, gain of some other chromosomes (or part of chromosomes), and development of structural abnormalities.

Carcinoma in Situ↗
PubMed1990

Diagnosis of congenital cystic adenomatoid malformation of the lung in newborn infants and children.

Seventeen patients were diagnosed as having congenital cystic adenomatoid malformation of the lung during 1970-88. One case was associated with congenital diaphragmatic hernia and the child died before operation. The other 16 children underwent successful surgery. The patients presented in one of three ways--with neonatal respiratory impairment (12 cases) requiring urgent diagnosis and treatment, with recurrent respiratory tract infections (3), and with pneumothorax (1). Diagnostic problems arose with the neonatal presentation. In four of the children laparotomy was performed for presumed diaphragmatic hernia; three of these children had right sided lesions. Congenital diaphragmatic hernia was excluded by contrast studies in a further two patients. Histological examination confirmed the diagnosis in all cases. The postoperative course was uneventful in all 16 patients. No long term impairment of pulmonary function was noted.

Child↗
PubMed1989

[Lung operations in dyspneic newborn infants].

Thirteen newborns with congenital cystic adenomatoid malformation and six with congenital lobar emphysema were operated on between 1970 en 1988. Eighteen children had more or less severe dyspnoea and (or) tachypnoea, one child presented with respiratory tract infection. The diagnosis could be made on the chest X-ray in most instances. However, diagnostic problems arose in the differentiation between congenital cystic adenomatoid malformation and congenital diaphragmatic hernia. Four times a laparotomy was done for presumed diaphragmatic hernia followed by thoracic surgery in the same session. In two children barium contrast studies of the gastrointestinal tract were done to exclude diaphragmatic hernia. Treatment consisted of lobectomy in 15 cases and segmental resection in four. Histological examination confirmed the clinical diagnosis in all instances. The results of the operations were excellent in all patients. No short- or long-term complications occurred.

Cystic Adenomatoid Malformation of Lung, Congenita↗
PubMed1989

[Congenital anomalies of the urogenital tract in patients with a Wilm's tumor].

133 patients in whom a Wilms' tumour had been diagnosed between 1971 and 1986 were evaluated retrospectively for presence of congenital anomalies of the genitourinary tract. These were detected in 51 patients, 38.3%. A comparison was made between the incidence of each detected malformation in the patient group and in the general population. Some anomalies seemed to have a high association with the nephroblastoma; sharing the same basic embryological disorder is suggested as a possible explanation for this association.

Adolescent↗
PubMed1989

Diagnosis and management of neonatal adrenal haemorrhage.

Three patients with neonatal adrenal haemorrhage (NAH), presenting with various signs and symptoms, are reported. The role of ultrasonography in diagnosis and management of this entity is emphasized. Conservative management is advocated, once the diagnosis is confirmed. Surgery is indicated only for cases of uncontrollable haemorrhage.

Adrenal Gland Diseases↗
PubMed1989

A malignant mixed gonadal stromal tumor of the testis with heterologous components and i(12p) in one of its metastases.

A malignant mixed gonadal stromal tumor with mesenchymal heterologous elements of the testis is presented. This entity has been described in the ovary, but not hitherto in the testis. Karyotyping and ploidy measurement was done of the primary tumor and of an inguinal and lung metastases. The DNA ploidy and modal chromosome numbers were in agreement with each other in all samples. The most significant cytogenetic finding was the presence of the metacentric germ cell tumor marker i(12p) in an inguinal metastasis. This marker has been demonstrated in testicular and ovarian germ cell tumors and in a mixed Müllerian tumor, which raises the question of a possible relationship between the pluripotency of these tumors and the presence of i(12p).

Chromosome Aberrations↗
PubMed1989

Prognosis of extrahepatic biliary atresia.

We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After successful hepatic portoenterostomy the development of cholangitis was the most important determinant of long term survival; five year survival was 54% in the 19 patients who had cholangitis and 91% in the 27 who did not. In the whole group of 71 patients the five year survival was 47%. Seventeen patients were at least 5 years of age at the time of writing, three of whom had had liver transplantation. Three patients have cirrhosis and hyperbilirubinaemia, and the other 11 have normal bilirubin concentrations and normal or slightly raised transaminase activities. To improve these results early surgical intervention in all children with extrahepatic biliary atresia is necessary, as are better methods of prophylaxis and treatment of cholangitis.

Anastomosis, Surgical↗
PubMed1989

Health significance of cadmium induced renal dysfunction: a five year follow up.

To assess the health significance of the early renal changes after chronic exposure to cadmium, 23 workers removed from exposure because of the discovery of an increased urinary excretion of beta 2-microglobulin or retinol binding protein, or both, have been examined once a year for five years. Eight of these workers had also an increased albuminuria. These workers had been exposed to cadmium for six to 41.7 years (mean 25 years) and their first follow up examination took place when they had been removed from exposure for six years on average. At that time, their mean age was 58.6 years (range: 45.5-68.1). It has been confirmed that the proteinuria induced by cadmium is irreversible. The most important finding, however, is a significant increase of creatinine and beta 2-microglobulin concentrations in serum with time, indicating a progressive reduction of the glomerular filtration rate despite removal from exposure. It is estimated that on average this rate has decreased by 31 ml/min/1.73 m2 during the five year follow up study. This decrease is significantly greater (about five times) than that accounted for by aging and is not more pronounced in workers with impaired renal function at the start of the study than in those presenting only with subclinical signs of renal damage. Serum alkaline phosphatase activity also increases significantly with time. In conclusion, the present study indicates that the early renal changes induced by cadmium should be regarded as adverse effects; they are predictive of an exacerbation of the age related decline of the glomerular filtration rate.

Aged↗