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Biomedical subjects

P N Rieu

Publications and source records attributed to P N Rieu.

At least 19 recordsLinked to original sources
PubMed2001

Quality of life in patients with anorectal malformation or Hirschsprung's disease: development of a disease-specific questionnaire.

PURPOSE: Hirschsprung's disease and anorectal malformation are congenital diseases of the digestive tract with sequelae into adulthood. The quality of life of patients with these diseases is largely unknown. The aim of the study was 1) to construct a self-report disease-specific instrument to assess the quality of life in these patients and 2) to evaluate its psychometric performance. METHODS: An age-specific (6 and 7 years, 8-11 years, 12-16 years, and >17 years) questionnaire called the Hirschsprung's disease/anorectal malformation quality-of-life instrument was constructed. This questionnaire consists of 39 to 42 items, grouped into 10 to 11 scales that cover physical, emotional, and social functions as well as disease-related symptoms. Generic quality-of-life data were obtained in addition. A national sample of 715 patients aged six years and older completed the questionnaire (response rate, 61.9 percent). RESULTS: Multitrait scaling analyses confirmed the hypothesized scale structure with exception of the scales related to diet for the two youngest groups. Cronbach's alpha ranged (with exception of the diet scales) from 0.62 to 0.91 for children (8-11 years), from 0.69 to 0.82 for adolescents (12-16 years) and from 0.57 to 0.91 for adults. Selective scales were able to discriminate between subgroups of adult patients known to differ in disease and disease severity. Relevant scales of the adult version showed substantial correlations (> 0.40) with comparable scales of the SF-36. In the two youngest age groups the differences between subgroups of patients were less significant, but in the expected direction. CONCLUSIONS: With the exception of the scales related to diet, the Hirschsprung's disease/anorectal malformation quality-of-life instrument is an instrument with promising reliability and validity, to measure the disease-specific quality of life of patients with anorectal malformation or Hirschsprung's disease.

Adolescent↗
PubMed2000

Residual lesions after Kasabach-Merritt phenomenon in 41 patients.

BACKGROUND: Kasabach-Merritt phenomenon (KMP) is the association of a vascular tumor and thrombocytopenic coagulopathy. Vascular tumors are either kaposiform hemangioendothelioma or tufted angioma but not "true" common hemangioma of infancy. There is a conspicuous absence in the literature regarding the late outcome and possible residual lesions after apparent clinical cure of KMP. OBJECTIVE: The purpose of the study was to analyze these residua in a large number of patients. METHODS: Clinical data on 41 patients who had KMP were accrued in an international cooperative study. The emphasis was on the residual lesions after resolution of the thrombocytopenia and other coagulation abnormalities. Imaging studies (follow-up magnetic resonance imaging studies available for 10 patients) and histologic specimens (30 specimens available for 26 patients, 18 biopsies done during the KMP and 12 concerning the sequelae) were reviewed. RESULTS: Residual lesions after "cure" of KMP were common. They exhibited 3 clinical patterns: type I lesions (n = 28) showed a cutaneous red stain, with or without associated red papules. The stain might overlap a minor fibrotic infiltration or a significant poorly delineated diffuse fibrotic infiltration. These cutaneous vascular lesions varied in size and appearance over time and were occasionally painful. Type II lesions were telangiectatic streaks and swelling (n = 5), and type III lesions showed a minor, firm, irregular, subcutaneous mass assessed by palpation or deep infiltration evidenced by computed tomography or magnetic resonance imaging (n = 8). A fourth feature was sequelae in muscles and/or joints. Histologically, tufted angioma was more common in the specimens from residual lesions, whereas kaposiform hemangioendothelioma was more common during the active phase of KMP. Imaging findings were remarkably reproducible and revealed a persistent vascular tumor. CONCLUSION: Residua of tumors associated with KMP are common after the resolution of thrombocytopenia and coagulopathy. They are (more or less) prominent dormant vascular tumors, not "scars" and, clinically as well as histologically, they differ markedly from involuted hemangioma.

Adolescent↗
PubMed1998

Parenting children with anorectal malformations: implications and experiences.

Parents play a crucial role in the life of a child suffering from an anorectal malformation (ARM), since their guidance contributes to the degree to which the child learns to cope with his or her disability. We investigated whether they experience stress in parenting such a child and also attempted to identify somatic or behavioral characteristics in the child that influence the stress of parenting. The parents of 109 children (69 males, 40 females; median age 5.9 years, range 1-18 years) with an ARM (58 low, 10 intermediate, 41 high) were studied. The Nijmegen Questionnaire on Child-rearing Situations (NQCS) was used to investigate the existing parenting situation. Behavioral characteristics of the children were studied by means of the Child Behaviour Checklist (CBCL) and the Teacher Report Form (TRF). In a semi-structured interview, we investigated how parents experienced the implications of the disability in everyday life with their child. Our study showed that as far as the perception of parenting stress is concerned, parents of children with an ARM do not differ from those with healthy primary-school children. Within the group of parents with ARM-afflicted children, the parents of older, incontinent children experienced relatively more stress, especially when the child concerned was male. With regard to the children's behavior, the parents and teachers under investigation did not report a higher than normal incidence of deviant behavior. However, when individual parents observed difficult behavior in their child, they found it harder to deal with than the incontinence for feces. Regarding the implications of the disorder for their everyday lives, parents were concerned and indicated a need for specific counselling. We conclude that having a child with a somatic affliction, in this case an ARM, does not automatically imply that the parents experience child-rearing problems. However, certain groups of parents are more at risk, i.e., parents with older, incontinent sons and parents with children exhibiting behavioral problems. In addition, our study shows that parents do have difficulties in coping with the implications of the disorder and express a need for support. We feel that patient care can be improved if aid is tailored to these specific problems.

Abnormalities, Multiple↗
PubMed1997

Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas.

OBJECTIVE: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion "capillary hemangioma." Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood. Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a "true," classic, involuting type of hemangioma of infancy and childhood. STUDY DESIGN: We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them. RESULTS: Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm3) and consumption of fibrinogen. Histologically, none had the typical "capillary," involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients. The infants exhibited a heterogeneous response to a number of therapeutic regimens, as noted in other reports. Severe morbidity was present; three of our patients died, and one had leg amputation. "Residua" were, in fact, residual vascular neoplasia, variable in duration, and not a stable fibrofatty residuum, as in classic involuted hemangioma; only the hematologic phenomenon was "cured" after a period of years. CONCLUSIONS: KMS is a distinctive disease of infancy, but the underlying vascular lesion is not a "true," classic, involuting type of hemangioma of infancy. This is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels. Whether the underlying lesion in KMS is a single anatomic entity or heterogeneous cannot be definitely concluded from this study. We need a better understanding of the pathogenesis of KMS to improve our therapeutic management.

Biopsy↗
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↗
PubMed1996

Intestinal pseudo-obstruction syndrome in a child with myotonic dystrophy.

Gastrointestinal symptoms in myotonic dystrophy are increasingly observed, but major intestinal movement disorders such as intestinal pseudo-obstruction appear to be an infrequent complication. We describe a 13-year-old boy who, after appendectomy, developed intestinal pseudo-obstruction syndrome as the first clinical manifestation of myotonic dystrophy. He developed several similar episodes thereafter, which responded to conservative measures. When a child with myotonic dystrophy presents with an ileus, the diagnosis of intestinal pseudo-obstruction should be considered and therapy should then be conservative.

Adolescent↗
PubMed1994

Quality of life after operatively corrected high anorectal malformation: a long-term follow-up study of patients aged 18 years and older.

Fifty-eight patients (median age, 26.0 years; range, 18.1 to 56.9 years) with an operatively corrected high anorectal malformation were evaluated by questionnaire. No patient had normal continence for feces; however, 84% had a socially acceptable defecation pattern. The quality of life (QOL) and general and mental health perception of these patients were evaluated. For social functioning and health perception, items from the medical outcome study (MOS) were used. QOL and health perception were compared with those of the general population. Most aspects of QOL (corrected for age and gender) and mental health did not differ from those of the general population. However, the patient population had lower educational and general health levels (P < .01). Twelve percent felt restricted socially by their handicap, and 24% never had a lasting relationship. Of the patients who had a lasting relationship, 43% noted that the handicap had been disturbing in the relationship. Associated anomalies had no influence on QOL and health perception. QOL, education level, and relationships were affected by fecal incontinence. It is possible that more appropriate psychosocial support, eg, addressing the implications of the handicap on everyday life, would have a positive influence.

Adolescent↗
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↗
PubMed1992

Esophageal stricture as a complication in Henoch-Schönlein purpura.

We report on a three-year-old boy with Henoch-Schönlein purpura developing an esophageal stenosis during severe clinical course of this disease. There are to date no reports on the development of such a complication. The possible pathogenesis in relation to stricture formation of ileum and ureter in Henoch-Schönlein's purpura is discussed.

Child, Preschool↗
PubMed1992

A complicated forearm greenstick fracture: case report.

Closed reduction of a greenstick fracture of the forearm in a child of 9 years was complicated by interposition of the deep flexor tendon of the third, fourth, and fifth fingers in the fracture gap. Undelayed release of the tendon resulted in a complete functional recovery.

Child↗
PubMed1992

Short-term results of gastrectomy with Roux-en-Y or Billroth II anastomosis for peptic ulcer. A prospective comparative study.

Since the Roux-en-Y anastomosis prevents the sequela of postoperative enterogastric reflux after gastrectomy, this approach has been advocated as the primary procedure in patients undergoing gastrectomy for peptic ulcer. We have prospectively followed for 2 years 22 patients, in whom gastrectomy was performed with, at random, either Roux-en-Y (n = 11) or Billroth II (n = 11) anastomosis. Two of the 11 patients who had received the Roux-en-Y procedure had anastomotic ulcers, leading to reresection in one of them. These two patients were found to have the highest values for basal and pentagastrin stimulated gastric acid output. After the Billroth II procedure a single patient had a small anastomotic ulcerative lesion. Apart from differences in intragastric bile acids (p less than 0.0001) and the gastritis activity score (p less than 0.01), no significant differences were found between the patients with Roux-en-Y and Billroth II anastomosis with respect to basal and pentagastrin-stimulated gastric acid secretion, basal, postprandial and bombesin-stimulated serum gastrin secretion, serum pepsinogen A and C concentrations, the serum pepsinogen A/C ratio, postprandial glucose, and for a modified Visick grading. From this small series we conclude that, as compared with the Billroth II-anastomosis, the Roux-en-Y procedure effectively prevents enterogastric reflux, and is associated with a higher gastritis activity score, but not with differences in gastric acid, gastrin, pepsinogens, or Visick grading. Furthermore, inadequate reduction of acid secretion in some patients after the Roux-en-Y procedure may lead to recurrent peptic ulcers.

Anastomosis, Roux-en-Y↗
PubMed1991

Effect of partial gastrectomy on serum anti-Helicobacter pylori immunoglobulins in peptic ulcer patients.

Since biliary enterogastric reflux is suggested to eradicate gastric infection with Helicobacter pylori (HP), we have investigated in a prospective randomized study the effect of partial gastrectomy with either Billroth II or Roux-en-Y anastomosis on infection with HP as assessed by the titers of IgG and IgA antibodies against HP in serum. These antibodies were measured by ELISA in serum of 22 patients before and at 10 days and 6, 15, and 24 months after either Billroth II (N = 11) or Roux-en-Y (N = 11) gastrectomy for peptic ulcer. All patients had HP demonstrated in their preoperative endoscopic gastric biopsies. The preoperative serum IgA antibodies against HP (anti-HP IgA) were increased in 20 of the 22 patients (range 0.21-1.69) while the IgG antibodies (anti-HP IgG) were increased in all 22 patients (range 0.38-1.31). Four of the Billroth II patients had clearance of HP from gastric biopsies accompanied by rapid and pronounced decrease of anti-HP IgA. In contrast, the patients with Roux-en-Y gastrectomy and the Billroth II patients with persistent HP infection had no change in anti-HP IgA after surgery. Anti-HP IgG showed variable results in the four patients without gastric HP infection and was not affected by gastrectomy in the patients with persistent HP infection. We concluded that serum anti-HP IgA, but not anti-HP IgG, is helpful in identifying those patients in whom HP is no longer demonstrable after Billroth II gastrectomy. Gastrectomy with Roux-en-Y anastomosis had no effect on gastric HP infection.

Antibodies, Bacterial↗
PubMed1991

Long-gap esophageal atresia: experience with reconstruction in 25 patients.

In a series of 183 patients with esophageal atresia in a period of 20 years, 25 had a "long-gap" esophageal atresia. In most of these cases we succeeded in saving the patients' own esophagus but at the cost of one to two years of intensive treatment. The overall survival was 80%. The result compares favorably with the results of esophageal replacement reported in the literature.

Anastomosis, Surgical↗
PubMed1991

[Vesico-intestinal fissure: a multidisciplinary problem].

Vesico-intestinal fissure or exstrophy of the cloaca is a rare but serious birth defect of the urogenital tract and distal part of the digestive tract. The most important hallmarks are: bladder exstrophy, fusion between bladder and the exstrophied iliocoecal region, short blind-ending colon and imperforate anus. In addition the lower part of the body shows defects of the abdominal wall, symphysis, upper urinary tract, the internal and external genitals, lumbosacral spine, spinal cord and the lower extremities. It is just after 1960 that corrective surgery started to become successful. Because of the complexity of the anomaly it seems that only management by a team of specialists in a centre for pediatric surgery guarantees optimal treatment. The quality of live of these children can still be improved by application of the newest surgical techniques and excellent cooperation between the different involved specialists. The defects, surgical treatment and results in eight patients with vesico-intestinal fissure, seen at the Universitair Centrum voor Chirurgie bij Kinderen Nijmegen (UCCKN) from 1974 till 1989 inclusive, are presented.

Abnormalities, Multiple↗
PubMed1990

Effect of partial gastrectomy with Billroth II or Roux-en-Y anastomosis on postprandial and cholecystokinin-stimulated gallbladder contraction and secretion of cholecystokinin and pancreatic polypeptide.

This prospective study was undertaken to determine the effect of partial gastrectomy without vagotomy on postprandial gallbladder contraction and secretion of cholecystokinin (CCK) and pancreatic polypeptide (PP) in 22 peptic ulcer patients randomly assigned to either Billroth II (N = 11) or Roux-en-Y (N = 11) anastomosis. The patients were studied within two weeks before surgery and at six months postoperatively. After surgery basal gallbladder volumes were larger than preoperatively (P less than 0.02). Integrated postprandial gallbladder contraction was not significantly affected by gastrectomy, either in the patients with Billroth II anastomosis (2276 +/- 268 vs 1985 +/- 362%/60 min) or in those with Roux-en-Y anastomosis (2045 +/- 327 vs 2445 +/- 352%/60 min) when studied pre- and postoperatively, respectively. Similarly, integrated postprandial plasma CCK secretion was not significantly changed by either Billroth II gastrectomy (200 +/- 31 vs 166 +/- 21 pM/60 min) or Roux-en-Y gastrectomy (146 +/- 26 vs 147 +/- 12 pM/60 min). However, integrated postprandial PP secretion was significantly (P less than 0.05) lower after Billroth II gastrectomy (6.8 +/- 2.4 vs 2.2 +/- 1.0 nM/60 min), while the reduction in plasma PP after Roux-en-Y gastrectomy just failed to reach statistical significance (6.0 +/- 1.5 vs 3.4 +/- 0.9 nM/60 min). Similarly, the PP response, but not the gallbladder response, to an intravenous bolus injection of 1 IDU CCK/kg body weight was significantly decreased after gastrectomy independent of the type of anastomosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Roux-en-Y↗