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R Rintala

Publications and source records attributed to R Rintala.

At least 37 records · Page 2Linked to original sources
PubMed1998

Distribution of glial cell line-derived neurotrophic factor mRNA in human colon suggests roles for muscularis mucosae in innervation.

BACKGROUND/PURPOSE: Glial cell line-derived neurotrophic factor (GDNF) is a ligand for the receptor complex of GDNF family receptor alphas (GFRalphas) and Ret receptor tyrosine kinase, the product of a known Hirschsprung's disease gene. The aim of this study was to analyze the mRNA distribution of these genes in the developing human intestine to understand their roles in enteric innervation. METHODS: Cryosections of fetal and newborn stomach, ileum, and colon were hybridized in situ with S35-labeled cRNA probes to GDNF, Ret, GFRalpha-1 or GFRalpha-2. GDNF mRNA levels in fetal ileum and colon were compared by reverse transcription-polymerase chain reaction (PCR). RESULTS: GDNF mRNA expression was abundant in the muscularis mucosae of both fetal and newborn colon but was found neither in the neural plexuses nor in other regions of the intestine. Accordingly, by reverse transcription-PCR, GDNF mRNA level was many times higher in colon than ileum. Ret, GFRalpha-1 and GFRalpha-2 mRNA were expressed in the ganglionic cells of both myenteric and submucosal plexuses throughout the intestine. CONCLUSIONS: The highly restricted distribution of GNDF mRNA suggests an important role for muscularis mucosae in the development of human enteric nervous system. Ret, GFRalpha-1, and GFRalpha-2 most likely act as GDNF receptors in colon but may have alternative ligands in other enteric segments.

Colon↗
PubMed1998

T-cells and HLA-class II expression in the large intestine of infants in the early postnatal period.

BACKGROUND: There is only limited knowledge of the development of the immune responses of the gut in very young infants after exposure to bacterial and food antigens at birth. METHODS: In this study, 49 large intestinal biopsy specimens, which were judged to have normal morphology, were taken from 49 young infants. Eleven patients had Hirschsprung's disease (group 1) and 38 had miscellaneous conditions (group 2). The densities of T cells, their subsets expressing surface antigens CD8 and CD4, and T-cell receptors alpha/beta or gamma/beta were measured, as well as densities of mononuclear and epithelial cells expressing HLA-class II antigens. RESULTS: T-cell densities in groups 1 and 2 were similar. Patients with Hirschsprung's disease had significantly more HLA-DR (p = 0.006) and HLA-DP-expressing cells (p = 0.003) in the lamina propria than did the patients in group 2. In group 1, HLA-DR- (r = 0.58; p = 0.46) and HLA-DP-expressing cells (r = 0.66; p = 0.03) showed a significant positive regression with age in the lamina propria, whereas in group 2, HLA-DR+ cells in the lamina propria showed marked (r = -0.9; p = 0.006) negative regression during the first 1.5 months of life. In contrast to results in previous reports, in the current results, HLA-D region antigens were present in the epithelium in a considerable proportion (up to one fourth) of specimens from the large intestine in both groups. CD3+ (r = -0.59; p = 0.006) and CD4+ (r = -0.64; p = 0.002) cells showed a strong negative regression with age in the lamina propria during the first 2.5 months; and thereafter, there was a weak, insignificant rise in the numbers of these cells. The distribution of CD4+, CD8+, and TCR alpha/beta or gamma/beta T cells of the epithelium of the young infants did not differ significantly from that in the epithelium of adults. CONCLUSIONS: These results show that several significant changes occur in the mucosal immune system during the first few weeks of life.

Biopsy↗
PubMed1998

Endometroid adenocarcinoma of the cervix in a 9-year-old girl.

We present the first reported case of endometroid adenocarcinoma of the uterine cervix in a young girl. The differential diagnosis of a vaginal mass in this age group is usually rhabdomyosarcoma, although other, rarer tumours also occur.

Adenocarcinoma↗
PubMed1997

Long-term anal sphincter performance after surgery for Hirschsprung's disease.

BACKGROUND/PURPOSE: The aim of the study was to assess anal sphincter performance in relation to clinical fecal continence in adult patients who have Hirschsprung's disease. METHODS: Fifty-four adult patients (mean age, 29 +/- 7.2 years; 46 men; 8 women) who had undergone surgery for Hirschsprung's disease during their childhood underwent anorectal manometry and clinical examination. Fecal continence was evaluated with a quantitative scoring method (scoring, 0-14; 14, normal bowel function; 10-13, good continence, no social problems; 5-9, fair continence, marked social limitations; 0-4, total incontinence). Thirty healthy adults were used as controls. RESULTS: Fourteen patients had normal bowel habits according to the quantitative scoring. The median anal resting pressure of these patients was 25 cm H2O (range, 15-37.5). The median resting pressure of patients with good continence (n = 30; median, 20 cm/H2O; range, 5-27.5) and with fair continence (n = 6; median, 15 cm/H2O; range, 5-27.5) was significantly lower (P < .01) than in patients who had normal continence. There was no statistical difference in maximal squeeze pressure between the patient groups (median normal, 52.5; good, 45; fair, 52.5). In the controls, the median resting pressure (61.5 cm H2O; range, 34-105) and maximal squeeze pressure (86 cm H2O; range, 55-148) were significantly higher than in all patient groups (P < .0001). The voluntary sphincter force (maximal squeeze pressure minus resting pressure) was similar in patients and controls (patients median, 27 cm H2O; controls median, 16 cm H2O, NS). CONCLUSIONS: There is a positive correlation between functional outcome and anal resting pressure in adults who have repaired Hirschsprung's disease. The overall low resting pressure reflects internal sphincter dysfunction, which may be caused by operative trauma. Despite this, most patients have a satisfactory functional outcome, which is probably related to normal voluntary sphincter performance.

Adult↗
PubMed1997

Orotracheal fibreoptic intubation in children under general anaesthesia.

Orotracheal fibreoptic intubation under general anaesthesia in children was studied in eleven consecutive patients of three months to eight-years-of-age without anticipated intubation difficulties. One case report is also included. Three fibrescopes with a different diameter were used in the study. The fibrescope used was chosen so that it fitted snugly in the tracheal tube. The fibreoscopy was prolonged in one patient due to mucus and two tries were needed. Resistance to the tracheal tube upon intubation was encountered in five patients, only one of these patients was older than two years. Fibreoptic intubation succeeded in nine patients. Two patients were intubated with the Macintosh laryngoscope. The problems encountered in children during orotracheal fibreoptic intubation under general anaesthesia are the same as with adults: easy fibreoscopy is not always followed by easy tracheal intubation, there may be prolonged fibreoscopy and failed intubations. Manipulation of the tracheal tube can lead to successful tracheal intubation and resistance to the tube is more common in smaller children.

Anesthesia, General↗
PubMed1997

Evidence-based surgery: interventions in a regional paediatric surgical unit.

OBJECTIVES: To determine the proportion of paediatric surgical interventions that are evidence-based and to identify areas where randomised controlled trials (RCTs) or further research are required. DESIGN: Prospective review of paediatric general surgical inpatients. SETTING: A regional paediatric surgical unit. SUBJECTS: All consecutive paediatric general surgical patients admitted in November, 1995. MAIN OUTCOME MEASURES: Each patient on whom a diagnosis had been made was allocated a primary diagnosis and primary intervention (n = 281). On the basis of expert knowledge, Plusnet Medline, and ISI Science Citation database searches, each intervention was categorised according to the level of supporting evidence: category 1, intervention based on RCT evidence; category 2, intervention with convincing non-experimental evidence such that an RCT would be unethical and unjustified; category 3, intervention without substantial supportive evidence. RESULTS: Of 281 patient interventions, 31 (11%) were based on controlled trials and 185 (66%) on convincing non-experimental evidence. Only 23% of interventions were category 3. CONCLUSIONS: In common with other medical specialties, the majority of paediatric surgical interventions are based on sound evidence. However, only 11% of interventions are based on RCT data, perhaps reflecting the nature of surgical practice. Further RCTs or research is indicated in a proportion of category 3 interventions.

Evidence-Based Medicine↗
PubMed1996

Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects.

Between 1948 and 1980, 107 of 164 patients survived after repair of a congenital diaphragmatic defect. Sixty of the survivors (mean age, 29.6 years; SD, 9.0 years) underwent clinical examination, chest radiography, spirometry, and diffusing capacity measurement 11 to 41 years after the diaphragmatic repair. Twenty-seven of the 60 had body plethysmography, xenon 133 radiospirometry, and a test of bronchial hyperreactivity. Subjective physical performance was below average for eight patients (13%), seven patients (12%) had asthma, and four patients (7%) reported increased susceptibility to respiratory infections. Thirty-one patients (52%) had ventilatory impairment, which was obstructive in nine (15%), restrictive in seven (12%), and obstructive and restrictive in 15 patients (25%). Nine (35%) of the 26 patients tested had bronchial hyperreactivity. The presence of ventilatory impairment and bronchial hyperreactivity correlated with the initial clinical severity of the affliction. Chest asymmetry (29 patients; 48%) and scoliosis (16 patients; 27%) were more common among patients with ventilatory impairment than among those with normal spirometric findings. Ventilatory impairment and thoracic deformities are common in adults with repaired diaphragmatic defects. Surveillance should begin in infancy and continue into adulthood.

Adolescent↗
PubMed1996

Chest wall and spinal deformities in adults with congenital diaphragmatic defects.

Between 1948 and 1980, 107 of 164 patients survived after repair of congenital diaphragmatic defects. To study chest wall and spinal deformities among these patients, 60 survivors (mean age, 29.6 +/- 9.0 years) underwent clinical examination, chest and spinal radiography, spirometry, and diffusing capacity measurements. Twenty-seven of these patients also had body pletysmography, xenon 133 radiospirometry, and a test for bronchial hyperreactivity. Chest asymmetry was present in 29 patients (48%). Eleven patients (18%) had pectus excavatum, and one had a pectus carinatum deformity. Anterior asymmetry, pectus deformities, and a flat chest were more common among the patients who initially had a large diaphragmatic defect. Two patients underwent anterior thoracoplasty because of their deformities. Sixteen patients (27%) had significant scoliosis (Cobb angle > or = 10 degrees). Scoliosis was more common in patients with ventilatory impairment than in those with normal lung function. One patient required spinal stabilization, one with severe kyphoscoliosis remains under observation, and a patient with moderate scoliosis was treated with a spinal brace. The results of this study indicate that chest wall deformities and scoliosis are common among adults with repaired congenital diaphragmatic defects. In most patients the deformity is mild, but some will require surgery. Surveillance until adulthood is appropriate.

Adolescent↗
PubMed1995

Long-term complications in cases of isolated esophageal atresia treated with esophageal anastomosis.

Long-term follow-up examination with esophagogastroduodenoscopy was performed on seven patients who had undergone successful delayed anastomosis for isolated esophageal atresia. The follow-up period ranged from 1.2 to 11.3 years (mean, 5.3). All patients had undergone fundoplication because of symptomatic gastroesophageal reflux (GER). Three anastomotic strictures had to be resected. At the time of the last follow-up examination, the subjective results were excellent for five patients and good for two. The last endoscopy showed macroscopic esophagitis in three and normal mucosa in four. The fundoplication was partly disrupted in two patients. In three patients the fundoplication was competent but partly intrathoracic. Histological examination showed moderate esophagitis in one, mild esophagitis in one, and normal mucosa in five patients; however, four patients were on continuous medication for esophagitis. In conclusion, the subjective results of patients with isolated esophageal atresia treated with esophageal anastomosis are good. However, long-term complications caused by GER are common in these patients. Therefore, active search and treatment of reflux is necessary for these patients.

Anastomosis, Surgical↗
PubMed1995

Bowel function in adults who have sustained spinal cord injury in childhood.

The impact of neuropathic bowel dysfunction on bowel habits was studied in 35 adult spinal cord injury (SCI) patients, who had sustained their injury in childhood. The methods used in the study were clinical interview and examination, quantitative scoring of bowel function (BCS) and estimation of bowel transit time with radiopaque markers. Thirty five healthy subjects without previous anorectal disease or surgery and with similar age and sex distribution as the patients served as controls. Most of the SCI patients were content with their bowel function. Nine (26%) out of 35 of the SCI patients were completely satisfied with their bowel function and reported no limitations in social life. The majority (69%) of the patients considered their bowel function to be significantly altered, causing only mild problems in their social life. Two of the patients had major problems of bowel function, which caused severe limitations in their social life. The majority (77%) of the patients declared that they had a low frequency of bowel evacuation. Eight out of 35 patients were using laxatives to promote bowel emptying. The quantitative BCS of the patients was significantly lower than that of the controls (P < 0.001). Moreover, there was a significant difference between the scores of patients with complete high (C2-T6) and complete low (T7-S4-5) lesions. The difference between the BCS and overall satisfaction with bowel function can be explained by good habilitation to SCI and by the prolonged transit times which enables relatively rare and controlled bowel movements and firm consistency of stools.

Adolescent↗
PubMed1995

Gut failure in pediatric and adult patients. Candidates for small-bowel transplantation in southern Finland.

BACKGROUND: This study investigated the need for small-bowel transplantation (SBT) in southern Finland. In addition, we evaluated the effect of colon resection on the time required for intestinal adaptation in patients with gut failure. METHODS: Mortality, morbidity, intestinal adaptation time, and surgical management were evaluated retrospectively in 24 patients (12 adults and 12 children) with jejunoileal exculsion. Of the 24 patients 19 had undergone accompanying colon resection. RESULTS: Intestinal adaptation had occurred in 12 of 18 survivors (67%). Two unadapted survivors were considered to be currently suitable candidates for SBT. The length of the remaining colon was inversely correlated (correlation coefficient r = -0.789; p < 0.0001) with the duration of intestinal adaptation. CONCLUSIONS: Each year one to two patients per 1.27 million people could benefit from SBT in southern Finland. The length of the remaining colon is a major determinant of the duration of intestinal adaptation in patients with massive jejunoileal resection.

Adaptation, Physiological↗
PubMed1994

Does disordered upper gastrointestinal motility predispose to Helicobacter pylori colonization of the stomach in children?

Helicobacter pylori is an important factor in the pathogenesis of chronic gastritis and gastroduodenal ulcer disease. However, the basic causal mechanisms of H pylori colonization on the gastric mucosa are still unclear. The authors evaluated the prevalence of H pylori colonization in 266 children who underwent upper gastrointestinal endoscopy during a 12-month period. The indications for endoscopy were follow-up of esophagitis related to gastroesophageal reflux (n = 17), suspicion of gastroesophageal reflux (n = 51), abdominal pain (n = 28), vomiting (n = 30), follow-up of esophageal atresia (n = 46) and duodenal atresia (n = 28), inflammatory bowel disease (n = 28), and miscellaneous (n = 38). The methods used to detect H pylori colonization were histology and the rapid urease test. H pylori colonization was demonstrated in 31 (11.6%) of the 266 patients. In two patient groups, a high prevalence of colonization was identified. In patients with an operated duodenal atresia, 36% (10 of 28) had H pylori on the gastric mucosa. The organism was demonstrated on the gastric mucosa in 47% (8 of 17) of the patients with gastroesophageal reflux-related esophagitis; five of the eight patients had neurological impairment. In the other patient groups, the prevalence of H pylori infection ranged from 2% to 14%. The present study suggests that, in children, the disturbed esophagogastroduodenal motility, which is commonly associated with gastroesophageal reflux and duodenal atresia, predisposes to H pylori infection.

Adolescent↗
PubMed1994

Fecal continence and quality of life for adult patients with an operated high or intermediate anorectal malformation.

Fecal continence and quality of life were evaluated by a questionnaire completed by 33 patients (mean age, 35 years; 25 males, 8 females) who had undergone anorectal reconstruction for a high or intermediate anorectal anomaly between 1946 and 1962. The malformation was classified as high in 30 cases and as intermediate in three. The primary reconstruction was abdominoperineal in 23 patients and direct perineal in 10. Ten patients had undergone secondary sphincter reconstructions. Fecal-continence was assessed by the scoring system described by Holschneider. Thirty-five healthy people with a similar age and sex distribution were used as controls. Only 6 patients (18%), including all three with an intermediate anomaly, had good fecal continence. None had completely normal bowel habits. Eighteen patients (54%) had a fair continence outcome. Seven patients (21%) had complete fecal incontinence. A permanent colostomy had been performed in two patients. Urinary incontinence was reported by 11 patients (33%). Ten patients (30%) had difficulties with sexual functions. Social problems related to defective continence were found in 28 patients (85%). All controls had good fecal continence; 80% had completely normal bowel habits. The authors conclude that most adults with high anorectal malformations who have undergone abdominoperineal or direct perineal repair suffer from severely defective fecal continence and have poor quality of life.

Adult↗
PubMed1993

Surgical treatment of intractable cholestasis associated with total parenteral nutrition in premature infants.

We report surgical reversal of intractable total parenteral nutrition (TPN)-associated cholestasis refractory to conservative treatment in 9 premature infants. Indications for TPN were poorly tolerated enteral feedings in all patients. Five patients had undergone gastrointestinal operations; in addition, 7 of the 9 patients had had bacterial sepsis. The median duration of TPN was 28 days (range, 20 to 50 days). The median duration of preoperative full enteral nutrition after weaning from TPN was 34 days (range, 16 to 95 days). All patients had progressive conjugated hyperbilirubinemia, no excretion of Tc-labeled HIDA to the biliary tree and duodenum, and markedly elevated liver enzyme values. Intraoperative cholangiography showed normal biliary anatomy in all cases; in addition, 2 patients had gallbladder stones. Bile was hyperviscous in all patients and contained biliary sludge in 4. The biliary tree was irrigated and the liver biopsied in all patients. The gallbladder was removed from 2 patients who had stones in the gallbladder. Liver histology was consistent with TPN-associated cholestasis in all cases, and in addition, 4 cases showed significant destruction of intrahepatic bile ducts. One patient died 2 weeks postoperatively from intracerebral hemorrhage. Jaundice completely resolved in other patients within 2 weeks. HIDA-biligraphy performed 1 to 2 months postoperatively showed normal excretion of the radioactive marker to the biliary tree and duodenum in all cases. The functional abnormality in bile excretion and bile duct motility in TPN-associated cholestasis may be reversed by irrigation of the biliary tree. Surgical intervention should be considered when cholestasis is progressive and refractory to medical management.

Cholestasis↗
PubMed1993

Anorectal function in adults operated for a benign sacrococcygeal teratoma.

Fecal continence and quality of life were evaluated by a questionnaire in 26 adult patients (mean age, 30 years; 6 men, 20 women) who had undergone surgery for a benign sacrococcygeal teratoma in infancy. The fecal continence was assessed by a score described by Holschneider. Twenty-six healthy people with a similar age and sex distribution were used as controls. Good fecal continence was reported by 88% of the patients; however, only 27% had completely normal bowel habits. Some fecal soiling was present in 27% of the patients. No correlation between the severity of aberrations in anorectal functions and the degree of intrapelvic extension of the tumor was found. Social problems related to deficient anorectal function were reported by 27% of the patients. Other health problems including urinary incontinence were reported by 50% of the patients. All the controls had good fecal continence; 77% of them had completely normal bowel habits. The present study shows that at the adult age, a significant proportion of patients who have undergone surgery for sacrococcygeal teratoma suffer from deficient anorectal function and a diminished quality of life.

Adult↗
PubMed1993

Chronic esophagitis and gastric metaplasia are frequent late complications of esophageal atresia.

Long-term follow-up examination with esophagogastroduodenoscopy was performed on 39 esophageal atresia patients 2 to 11 years (mean, 7.6) after the anastomotic operation. Nine of these patients had undergone fundoplication for symptomatic gastroesophageal reflux. The subjective results at the last follow-up were as follows: excellent in 25, good in 10, and fair in 4 patients. The endoscopic findings were normal in 17 patients. Endoscopy of the remaining 22 patients showed esophagitis in 20, 5 associated with Barrett's change. Hiatal hernia was found in 10 patients. The fundoplication was totally or partially disrupted in 3 patients. Esophageal biopsies were obtained from 37 patients. The histological findings were as follows: esophagitis in 21, gastric metaplasia in 3, and normal esophageal mucosa in 13 patients. The endoscopic and histological findings correlated poorly with subjective results as more than half of the patients with excellent subjective results had esophagitis, one of them with gastric metaplasia. Therefore, long-term endoscopic follow-up of all esophageal atresia patients is warranted.

Child↗
PubMed1993

Constipation is a major functional complication after internal sphincter-saving posterior sagittal anorectoplasty for high and intermediate anorectal malformations.

Anorectal function was clinically, manometrically, and radiologically followed-up in 40 patients who underwent an internal sphincter-saving posterior sagittal anorectoplasty in 1984 to 1989. The functioning internal sphincter was manometrically verified in 83% (33/40) of the patients. Soiling related to sphincter insufficiency was found in only 4 of the 33 patients with a functioning internal sphincter; 5 of the 7 patients without an internal sphincter had soiling. Symptomatic constipation was found in 73% (24/33) of the patients with a functioning internal sphincter, but in only 28% (2/7) of the patients without this structure. Constipation was not associated with a stenotic anal outlet in any patient. On anorectal manometry, there was no statistical difference in basal and pressures and internal sphincter reflex threshold values between constipated and nonconstipated patients. Histologically, ganglion cells were found in the proximal anal canal in all cases. The radiological size of the rectal pouch before closure of the protecting colostomy had a positive correlation with the severity of constipation. Medical treatment with diet and bulk-laxatives (13 patients) or with stimulant laxatives and occasional enemas (11 patients) successfully relieved constipation in 24 patients. In 2 patients, the symptoms were refractory to medical treatment and a resection of the megarectum was required. In conclusion, the preservation of the internal sphincter in patients with high or intermediate anorectal anomalies gives a good fecal continence outcome, but is associated with a high incidence of symptomatic constipation.

Anal Canal↗