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Biomedical subjects

R S Severijnen

Publications and source records attributed to R S Severijnen.

At least 19 recordsLinked to original sources
PubMed2001

Multidisciplinary behavioral treatment of defecation problems: a controlled study in children with anorectal malformations.

BACKGROUND/PURPOSE: The most frequent consequences of being born with an anorectal malformation (ARM) are problems with fecal continence and constipation, which can have various negative implications. In this prospective, controlled study the effect of multidisciplinary behavioral treatment dealing with these problems is evaluated. METHODS: The effect of multidisciplinary behavioral treatment was studied in 24 children (15 boys, 9 girls; mean age 5.8 years). Thirteen children were allocated to the treatment condition. The 11 children allocated to the waiting list control group also were treated after a waiting period of 6 months. Children underwent follow-up after treatment. RESULTS: Compared with a waiting list control group, the experimental treatment group scores significantly better on 2 important measures ("Templeton," "Percentage of feces in toilet"). Although young children had poorer scores than older children before treatment, no significant differences in the favorable outcome of treatment were found between both groups after treatment. No effect of type of ARM on treatment was found either. The results of multidisciplinary behavioral treatment remain stable over a mean follow-up period of 7 months. CONCLUSION: Multidisciplinary behavioral treatment is an important and valuable supplement to the standard medical treatment of children born with ARM suffering from chronic defecation problems.

Anal Canal↗
PubMed2001

Defecation problems in children with Hirschsprung's disease: a prospective controlled study of a multidisciplinary behavioural treatment.

UNLABELLED: Constipation, faecal incontinence, soiling and difficult toilet training remain significant problems in children with Hirschsprung's disease after corrective surgery. Chronic defecation problems can have various negative implications. At the University Medical Centre Nijmegen, a multidisciplinary behavioural treatment was developed to treat defecation problems. In this paper, a prospective controlled study is presented concerning the effect of this treatment upon children suffering from chronic defecation problems following corrective surgery for Hirschsprung's disease. The effect of treatment was studied in 27 children (21M, 6F, mean age 5.2y, range 2-11 y). Fourteen children were allocated to the experimental treatment group. The 13 children allocated to the waiting-list control group were also treated following a waiting period of 6 mo. On all outcome variables, children in the experimental treatment group had significantly better results after treatment than children in the waiting-list control group after the waiting period. No effect of age upon treatment was found. The effect of treatment remained significant on all outcome variables at a mean follow-up of 7 mo after the end of treatment. CONCLUSION: Multidisciplinary behavioural treatment is successful in decreasing chronic defecation problems in children with Hirschsprung's disease.

Behavior Therapy↗
PubMed2000

RET and GDNF gene scanning in Hirschsprung patients using two dual denaturing gel systems.

Hirschsprung disease (HSCR) is a congenital disorder characterised by intestinal obstruction due to an absence of intramural ganglia along variable lengths of the intestine. RET is the major gene involved in HSCR. Mutations in the GDNF gene, and encoding one of the RET ligands, either alone or in combination with RET mutations, can also cause HSCR, as can mutations in four other genes (EDN3, EDNRB, ECE1, and SOX10). The rare mutations in the latter four genes, however, are more or less restricted to HSCR associated with specific phenotypes. We have developed a novel comprehensive mutation detection system to analyse all but three amplicons of the RET and GDNF genes, based on denaturing gradient gel electrophoresis. We make use of two urea-formamide gradients on top of each other, allowing mutation detection over a broad range of melting temperatures. For the three remaining (GC-rich) PCR fragments we use a combination of DGGE and constant denaturing gel electrophoresis (CDGE). These two dual gel systems substantially facilitate mutation scanning of RET and GDNF, and may also serve as a model to develop mutation detection systems for other disease genes. In a screening of 95 HSCR patients, RET mutations were found in nine out of 17 familial cases (53%), all containing long segment HSCR. In 11 of 78 sporadic cases (14%), none had long segment HSCR. Only one GDNF mutation was found, in a sporadic case.

Amino Acid Substitution↗
PubMed2000

Defecation problems in children with Hirschsprung's disease: a biopsychosocial approach.

Although most patients with operated Hirschsprung's disease (HD) have good continence in adulthood, a majority have postoperative defection problems during school age. Persistence of chronic constipation and/or incontinence may have considerable consequences for psychosocial development, parent-child interactions, quality of life, and the child's general condition. Considering these consequences, it is important to treat these problems as early as possible. From a biopsychosocial view, we developed a multidisciplinary treatment aimed at resolving defecation problems by teaching the child bowel self-control, primarily by training optimal defecation skills and subsequently toilet behavior. This treatment, carried out by a child psychologist, a pediatric physiotherapist, and a pediatric surgeon, consists of five steps: explanation; extinction of fear and avoidance behavior; learning new defecation behavior; learning an adequate straining technique; and generalization toward daily life. The effect of the treatment was investigated retrospectively in 16 boys with operated HD. The children improved significantly in all aspects during treatment, suggesting that multidisciplinary treatment can significantly reduce the postoperative chronic bowel problems of most children with operated HD. The treatment was as effective in young children (2-5 years) as in older children (5-14 years).

Adolescent↗
PubMed2000

Biopsychosocial treatment of defecation problems in children with anal atresia: a retrospective study.

In a retrospective study, we examined whether multidisciplinary treatment based on a biopsychosocial approach and carried out by a pediatric surgeon, a child psychologist, and a pediatric physiotherapist is successful in reducing defecation problems (incontinence and/or constipation) in children with operated anal atresia (AA) (mean age 6.9 +/- 4.01 years). A second question was whether this treatment is successful in young children aged 2-5 years. The multidisciplinary approach consisted of standard medical treatment and a behavioral program to teach children and their parents adequate defecation behavior including an adequate straining technique. Forty-three children aged 2-16 years were included: 27 boys and 16 girls with AA, of whom 26 had high or intermediate and 17 low AA. Besides continence and constipation, defecation behavior and straining technique were evaluated. The children improved significantly during treatment in all aspects of defecation. No differences in effect of treatment were found between young children (2-5 years) and older ones, so this treatment seems to be equally effective in both age groups. This study demonstrates that both somatic and behavioral factors contribute to the persistence of chronic defecation problems. It is concluded that treatment of these problems in patients with operated AA should include behavioral modification techniques.

Adolescent↗
PubMed2000

Clinical letter: epidural analgesia in a newborn with Hirschsprung's disease, associated with congenital central hypoventilation syndrome.

A case is presented of a neonate with Hirschsprung's disease, associated with congenital central hypoventilation syndrome. After an ileostomy (at 2 days) and a stoma revision (at 10 days), postoperative pain management was established by continuous intravenous infusion of morphine, which caused severe postoperative respiratory depression. At 6 weeks a re-exploration and stoma revision was performed using postoperative epidural analgesia with bupivacaine. This caused no respiratory depression. A colectomy under epidural analgesia at 8 months was also uneventful. Respiratory difficulties in children with congenital central hypoventilation syndrome associated with Hirschsprung's disease are discussed in relation to the technique of choice for postoperative pain management.

Analgesia, Epidural↗
PubMed1999

Neurological complications in children with supracondylar fractures of the humerus.

OBJECTIVE: To study the incidence of primary and secondary nerve injury in supracondylar fractures of the humerus, and to find out which factors may be responsible for iatrogenic nerve injuries. DESIGN: Retrospective study. SETTING: University Hospital, The Netherlands. SUBJECTS: 32 children admitted with type II or type III supracondylar fracture of the humerus. MAIN OUTCOME MEASURES: Incidence of primary and secondary nerve injury and their relation to type of fracture and treatment. RESULTS: 3 patients developed primary nerve injuries (9%). 4 further children developed nerve injury during treatment. All these children had several closed reductions attempted before definitive treatment was given (either pinning with a Kirschner wire or overhead traction). CONCLUSION: Several attempts at closed reduction may cause iatrogenic nerve injury in unstable supracondylar fractures of the humerus.

Adolescent↗
PubMed1997

Role of bacteria in the pathogenesis of short bowel syndrome-associated D-lactic acidemia.

Previously, we have demonstrated that short bowel syndrome (SBS) patients suffer daily from D-lactic acidemia; in these patients rather high amounts of (bacterial) D-lactate emerge in blood and urine with a circadian rhythm. The aim of this study was to establish the microbial basis of D-lactic acidemia in SBS. Therefore, faecal flora of (young and adult) SBS-patients was analysed qualitatively and quantitatively, and compared to that of controls. The isolated bacterial species were screened for massive D- and/or L-lactate production after in vitro growth. After introduction of oral feeding in SBS-infants shortly after the resection, lactobacilli increased from < or = 1% up to 60 +/- 5% of the faecal flora within 2-3 weeks. In the faeces of patients with oral feeding the lactate producers Lactobacillus acidophilus and Lactobacillus fermentum were the major resident bacteria (each with 10(10)-10(12) cfu/g faeces). During active growth in vitro these lactobacilli produced massive amounts of D- and L-lactic acid from glucose. Use of oral antibiotics in two SBS-children did not reduce the total numbers of lactobacilli, but caused shifts within the intestinal populations of at least lactobacilli. It is concluded that the strongly reduced intestinal capacity for carbohydrate absorption and the oral consumption of easily fermentable carbohydrates form the physiological basis for D-lactic acidemia in SBS, and that the fermentative D-lactate production by intestinal bacteria, especially the abundant, resident lactobacilli, forms its microbial basis. In these patients the antimicrobial and therapeutic effects of antibiotics are unpredictable.

Acidosis, Lactic↗
PubMed1996

Additional congenital defects in anorectal malformations.

UNLABELLED: From 1974 until 1995 a total of 264 (141 male, 123 female) patients born with an anorectal malformation (ARM) were referred to the University Hospital Nijmegen in the Netherlands. All additional congenital defects (ACDs) were registered. Special attention was paid to whether the ACDs take part in associations, syndromes, or sequences. One or more ACDs were observed in 67% of the patients. In decreasing order the defects concerned the uro-genital tract (43%), skeleton (38%), gastrointestinal tract (24%), circulation (21%), extremities (16%), face (16%), central nervous system (15%), respiratory tract (5%), and remaining defects (5%). Associations were observed in 49% of the patients mostly (in 44%) the Vertebral, Anorectal, Cardial, Tracheo-Esophageal, Renal and Limb association. In 5% of the patients syndromes were recognized. Sequences were seen in 2% of the patients. Remarkable is the combination of trisomy 21 and ARM without a fistula. The combination of ARM and the Zellweger syndrome has not been reported before. CONCLUSION: Almost all combinations of ARM and ACDs can be classified as an association, syndrome or sequence. ARM-causing agents affect males and females in equal numbers but lead to different expression in the sexes. The origin of the Omphalocele, Extrophia of the bladder, Imperforate anus, Sacral anomalies complex probably differs from that of other forms of ARM.

Abnormalities, Multiple↗
PubMed1996

Adults born with high anorectal atresia--how do they manage?

PURPOSE: We are interested in the way patients, who underwent surgery for high anorectal atresia, control their defecation. Considering that some patients, despite newer operative techniques, always will suffer from minor or major soiling we attempted to find some guidelines for postoperative support for future patients. METHOD: Fifty-eight patients (median age, 26 (range, 18.1-56.9) years) were personally interviewed. RESULTS: Regulating defecation is done in five different modes: 16 patients have stools after urge, 15 control their stools mainly by going to the toilet at regular times, 18 perform bowel-irrigations or use enemas, 2 have loss of feces continuously, and 7 patients have an ileostomy or colostomy. More than one-half of patients influence their defecation by diet. Of the patients with anal defecation, 6 never soil, 39 sometimes soil small amounts, and 6 often soil seriously. Eighteen patients occasionally suffer from constipation. There is no mode of defecation regulation outstanding in preventing soiling or constipation. However, patients who do not regulate defecation somehow suffer from serious soiling. Most patients are content with their level of cleanliness. CONCLUSION: Irrespective of the mode of defecation regulation, many patients soil sometimes small amounts and a few often soil seriously. In view of the fact that most patients had to find the current control of defecation regulation by themselves rather late and lacked professional support, it is questionable whether the chosen mode of defecation regulation is the most optimal mode for each patient. We assume that a stepwise protocol under professional support, starting by the most natural mode of defecation, will improve defecation regulation in a more efficient way (earlier and better).

Activities of Daily Living↗
PubMed1993

Are adults content or continent after repair for high anal atresia? A long-term follow-up study in patients 18 years of age and older.

OBJECTIVE: This study investigated the current state of fecal and urinary continence in an extensive group of adults after operative correction for high anorectal malformations and how they cope with their incontinence. SUMMARY BACKGROUND DATA: Normal fecal continence is hardly to be expected after correction for high anorectal malformation; despite this, it is commonly accepted that for most patients fecal continence improves with growing age and that most adult patients have no problems. Until now, however, few long-term follow-up studies in small groups of adults have been performed to assess continence after operative repair for high anorectal malformation. METHODS: Fifty-eight adult patients (median age, 26.0 years; range, 18.1 to 56.9 years) with an operatively corrected high anorectal malformation were evaluated by questionnaire with respect to their current state of fecal and urinary continence and mode of control of defecation. RESULTS: Seven patients have a permanent ileostoma or colostoma. Of the 51 patients with anal defecation, 61% control defecation by themselves, whereas 35% control defecation by using enemas or bowel irrigations, and 4% do not have any control at all. Besides medical therapy, 65% take dietary measures to influence defecation. According to existing scoring methods, 41% reached good and 49% fair control of defecation, whereas only 10% had poor control. Current control of defecation was reached from a median age of 15.0 years (range, 5 to 31 years). CONCLUSION: Conclusively, the authors can say that after correction for high anorectal malformation nobody reached normal fecal continence. Most patients with anal defecation reached good and fair control of defecation, however. Of all 58 patients, 84% are satisfied with their level of cleanliness.

Adolescent↗
PubMed1993

Chronic tunnel infections in children: removal and replacement of the continuous ambulatory peritoneal dialysis catheter in a single operation.

OBJECTIVE: Chronic tunnel infections often necessitate the removal of the continuous ambulatory peritoneal dialysis (CAPD) catheter. Most published studies advocate postponing the insertion of a new catheter for several weeks. For young children it will be particularly difficult to wait this length of time, since vascular access may be cumbersome, and hemodialysis may not be well tolerated. The present study describes the results of the simultaneous removal and replacement of the CAPD catheter. DESIGN: Twenty-three Toronto Western Hospital II catheters were inserted in 17 children because of infectious complications (21 chronic tunnel infections; 2 recurrent peritonitis) in a single operation under appropriate antibiotic prophylaxis. The new catheter was inserted at the contralateral side of the abdomen with the deep cuff in the midline, using the same entrance to the peritoneal cavity. Dialysis was resumed immediately after the operation. SETTING: A university pediatric dialysis unit. PATIENTS: Seventeen children (mean age 3.7 years; range 1.0-8.5 years) were studied. In this group 23 catheters were replaced. RESULTS: In four cases a relapse of the tunnel infection was observed within 3 months. All other cases remained free of infection for a period of at least 6 months. The main causative microorganism was Staphylococcus aureus (15 occurrences). CONCLUSION: It is not necessary to interrupt peritoneal dialysis for the replacement of a CAPD catheter because of infectious complications.

Bacterial Infections↗