Full-thickness gastric prolapse following percutaneous endoscopic gastrostomy.
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Aged↗
Biomedical subjects
S Refsum
Publications and source records attributed to S Refsum.
Monitoring urinary bladder volume and detecting post-operative urinary retention in children with an ultrasound scanner.
BACKGROUND: Post-operative urinary retention (PUR) is associated with a risk of over-distension and permanent detrusor damage. The prevention of PUR by routine catheterization may increase the risk of urinary tract infection. Post-operative monitoring of the bladder volume is a reliable method in adults, but has not been evaluated for reliability in children. METHODS: Forty-eight patients who, according to current clinical guidelines, required urinary bladder emptying were scanned before catheterization using the Bladderscan. Volumes estimated with ultrasound were compared with volumes measured after emptying the bladder with a catheter. Bladder emptying was controlled by concomitant fluoroscopy in 20 children. RESULTS: The mean difference between the ultrasound estimates and the catheter-emptied urine volumes was 4 ml [standard deviation (SD) = 25 ml] in 26 children above the age of 3 years and -18 ml (SD = 19 ml) in 22 younger children. In the subpopulation in whom complete bladder emptying could be confirmed by fluoroscopy (14 children; median age, 3 years; range, 1-11 years), the mean difference between the ultrasound estimates and the catheter-emptied volumes was -11 ml (SD = 24 ml). CONCLUSIONS: This study confirms agreement between the ultrasound scanner estimates of urinary bladder volume and the urine volume measured by emptying the bladder. Reliability was good in children above the age of 3 years. The volume was underestimated in younger children. Thus, routine monitoring of urinary bladder volume with an ultrasound scanner is a non-invasive, pain-free and reliable way of preventing over-distension of the urinary bladder in children after surgery and other procedures under general anaesthesia.
Adolescent↗
Solitary caecal diverticulitis--a rare cause of right iliac fossa pain.
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Abdominal Pain↗
[Crohn disease of the oral cavity, illustrated by some cases].
BACKGROUND: We have several times experienced that Crohn's disease has not been diagnosed until several years after the start of oral symptoms. This article presents some patients with oral manifestations of Crohn's disease, as a contribution to more awareness of this possibility. MATERIAL AND METHODS: Clinical and histological data from four patients are presented. RESULTS: All patients had granulomatous inflammation of the oral mucosa. In three patients, the disease started in the mouth. The diagnosis of intestinal Crohn's disease was made from some months up to seven years later. In one patient, no granuloma was found in the intestinal tract, but the patient had intestinal symptoms. Two patients experienced disappointments in their contacts with the local health care system, with considerable delay in diagnosis. INTERPRETATION: We interpret these cases as evidence that a greater awareness of the possibility of Crohn's disease in the oral cavity may lead to a quicker diagnosis, earlier correct treatment, and less patient worry caused by uncertainty.
Adolescent↗
[Enuresis nocturna--practical advice for examination and treatment].
Children suffering from nocturnal enuresis have better self-esteem when they stay dry. A medical examination is necessary in order to exclude specific causes. The choice of treatment should be based on: motivation and age of the child, nocturnal polyuria versus bladder capacity, possibilities to borrow an alarm device, and follow-up. Informed choice treatment should be offered. Treatment with an alarm device and minimum standards are discussed. Desmopressin for short-term treatment or for three month periods are recommended for patient who respond to desmopressin. If organic causes are suspected, the child has diurnal incontinence symptoms, or does not stay dry in spite of the recommended treatment, referral to a paediatrician or a paediatric department is recommended. A better program for managing these patients at paediatric departments should be developed in collaboration with other specialists.
Child↗
Prenatal diagnosis of urinary tract anomalies. The value of two ultrasound examinations.
OBJECTIVE: To determine when fetal urinary tract anomalies were detected by ultrasound screening during pregnancy and to discuss the possible consequences if only one early ultrasound examination is performed. DESIGN: A retrospective study of 47 cases where fetal urinary tract malformations were diagnosed in a two-stage screening program (17th and 32nd week of gestation) covering 22,310 women over ten years, 1982-91. SETTING: Ullevål University Hospital in Oslo which serves as a referral center for obstetric and neonatal diseases. RESULTS: Urinary tract anomalies were diagnosed in 0.18% of the pregnancies. Of these, 61.7% were found at the second routine ultrasound screening. The most difficult differential diagnoses were those of hydronephrosis without megaureter and a multicystic kidney. Hydronephrosis was found in 51% and a multicystic kidney in 21% of the cases. The tentative prenatal diagnoses were confirmed postnatally in 83% of the cases. Six of the fetuses had anomalies regarded as incompatible with postnatal life. These pregnancies were terminated, and the diagnoses verified by autopsy. Two other infants died perinatally, one of them as a result of the urinary tract anomalies. CONCLUSION: If only one ultrasound scanning had been performed in the 17th week, approximately two-thirds of the cases would not have been detected. Early intervention and follow-up after delivery would only have been performed if the infants had developed symptoms or complications related to the urinary tract anomalies.
Abortion, Induced↗
A prospective clinical five year follow up study after open synovectomy of the knee joint in patients with chronic inflammatory joint disease. The prognostic power of clinical, arthroscopic, histologic and immunohistologic variables.
Arthroscopy and clinical examination was performed on the knee joint of 26 patients with chronic inflammatory joint disease, before and at 6 and 12 months after open synovectomy. Biopsies were examined by histologic and immunohistologic methods. Clinically the patients improved until 6 months after synovectomy, and the improvement was maintained for 5 years. Patients with the best clinical function prior to synovectomy also had the best long term results (p = 0.024). The state of the cartilage was the best predicting variable with a significant prognostic power (p = 0.01). Thus patients with normal cartilage at the time of synovectomy had the best clinical score five years later. The patients with most resynovitis 12 months after surgery did less well at five year clinical follow up (p = 0.032) than those with little or no resynovitis. Absence or low number of IgA-positive plasma cells in the cellular infiltrate at 12 months after surgery correlated with a good clinical score five years after surgery (p = 0.036). This suggests that a high number of IgA-positive plasma cells may be indicative of a more aggressive, destructive disease.
Adolescent↗
Histopathologic changes in arthroscopic synovial biopsies before and after open synovectomy in patients with chronic inflammatory joint diseases.
Synovial biopsies were taken from five preselected areas of knee joints in patients with chronic inflammatory joint diseases immediately prior to synovectomy as well as 6 and 12 months later. The histologic changes were assessed by evaluating 16 different parameters in each individual biopsy. When the 16 parameters were treated as a group there was a significant reduction (p less than 0.001) in inflammatory changes at 6 months, but a slight increase again after 12 months. This was expressed by a significant reduction in lymphocytes (p less than 0.025) and plasma cells (p less than 0.01). The reduction seen in macrophages was not significant (p greater than 0.05). The cellular infiltrates in the interstitial stroma were dominated by lymphocytes, plasma cells and macrophages preoperatively and by lymphocytes and macrophages following synovectomy. Fibrosis was a dominating feature before synovectomy, and it increased (p less than 0.025) following the operation, probably due to the surgical intervention. Before synovectomy the severity of inflammatory changes within each single joint varied, but none of the preselected areas was more prone to these changes than any other. Postoperatively at both 6 and 12 months the inflammatory parameters were more pronounced in the intercondylar area (p less than 0.025).
Arthritis↗
[Esophageal strictures in children].
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Dilatation↗
Clinical and biochemical heterogeneity in conditions with phytanic acid accumulation.
Phytanic acid accumulation has for more than 20 years been used as a diagnostic criterion of Refsum's disease. Recently, however, phytanic acid has also been found in peroxisomal disorders (Zellweger's syndrome, neonatal adrenoleukodystrophy, infantile Refsum's syndrome, rhizomelic chondrodysplasia punctata). The 17 patients with Refsum's disease in the present study had serum phytanic acid values differing from 73 to less than 0.5 mg/dl (normal). alpha-Oxidation of phytanic acid in skin fibroblast cultures showed a defective capacity in all, with only small differences in residual activity. Phytanic acid determinations in serum from 3 of the 7 patients with peroxisomal disorders showed slightly elevated levels in 2. The alpha-oxidation capacity in the fibroblasts was defective in all, with a residual activity similar to that of Refsum's disease. An assay of the alpha-oxidation capacity may be useful in the diagnosis of both Refsum's disease and the peroxisomal disorders. The distinction between Refsum's disease and the peroxisomal disorders can easily be done on a clinical basis.
Adrenoleukodystrophy↗
Ehlers-Danlos syndrome and diverticula of the bladder.
Cases of two boys, 6 and 4 years old respectively, with Ehlers-Danlos syndrome (EDS) and large bladder diverticula, are reported. Diverticulectomies were performed, but large diverticula reappeared in both patients. A connection between the increased extensibility of the connective tissue in EDS and the development of bladder diverticula is suggested.
Child↗
Phytanic acid storage disease: hearing maintained after 15 years of dietary treatment.
Heredopathia atactica polyneuritiformis is a biochemically defined disease with a specific dietary treatment. It is an autosomal inborn error of metabolism. The phytanic acid is of exogenous origin and stems mainly from preformed phytanic acid in foods. In two Norwegian patients, serum phytanic acid has been brought down to normal levels and one of them has been followed for 15 years. During this period of dietary treatment there was no worsening of hearing.
Eicosanoic Acids↗
Benign familial myoclonus-like movements, partly of early onset.
A family with benign, myoclonus-like involuntary movements of early onset has previously been described by us (Refsum and Sjaastad, 1972, 1973; Sjaastad, 1981). In the present communication electrographical investigations carried out in the proband and his sister will be reported. Additional information will also be reported regarding onset of the abnormal movements.
Adult↗
Refsum's disease and metabolism of phytanic acid.
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Eicosanoic Acids↗
Anorectal anomalies--international classification.
80 patients with anorectal anomalies were admitted to the Paediatric Surgical Service of the National Hospital of Norway during the five year period 1970--74. The anomalies were classified according to the International Classification which was found to be of obvious value in comparing material and results. Intermediate and high deformities were generally treated with an initial colostomy and a subsequent sacroperineal pullthrough operation. The low anomalies were generally treated with an anoplasty in two procedures. Patients with poor functional results and incontinence can be improved with free muscle transplantation.
Abnormalities, Multiple↗
Heredopathia atactica polyneuritiformis phytanic-acid storage disease, Refsum's disease:" a biochemically well-defined disease with a specific dietary treatment.
Heredopathia atactica polyneuritiformis (phytanic-acid storage disease, Refsum's disease) is an inborn error of metabolism, in which the body accumulates exogenous phytanic acid. The severe manifestations of this disease, which include pigmentary retinal degeneration, chronic polyneuropathy, ataxia, impaired hearing, and cardiopathy, can be either kept from worsening or reversed by elimination of foods rich in phytanic acid from patients' diets. This is borne out by a 20-year experience with two patients, whose conditions improved markedly once they stopped eating butter, animal fat, and other foods rich in phytanic acid.
Humans↗
Late presentation of left-sided congenital diaphragmatic (Bochdalek) hernia.
Two patient with late presentation of Bochdalek hernia are reported. The intraoperative findings strongly suggested a "plugging effect" of the spleen in the diaphragmatic defect, thus protecting for some time against herniation. The initial absence of respiratory symptoms indicates a normal development of the lungs in these cases of late herniation.
Hernia, Diaphragmatic↗
Recurrent bladder papilloma in a child with Cushing's syndrome. Case report.
A case of bladder papilloma in a 12-year-old boy with Cushing's syndrome was reported by us in 1975. A walnut-sized papilloma was removed by transvesical extirpation, and bilateral adrenalectomy was performed. Two years later he was reoperated because of recurrent Cushing syndrome, and at the same time he had a recurrence of multiple bladder papillomas. Since removal of residual adrenocortical tissue and hormone substitution therapy his Cushing appearance has disappeared completely. The recurrent papillomas were removed by transurethral electrocoagulation and control cystoscopies have shown no sign tumour recurrence. We suggest a possible neoplastic effect of some tryptophan metabolites(s) in combination with immunosuppression as the cause of the bladder papillomas.
Adrenalectomy↗