Biomedical subjects
Publications and source records attributed to O Eklöf.
Available films of 21 cases of erroneously diagnosed Wilms' tumor in the European Wilms' material have been studied. The diagnosis has been reassessed and the reasons for agreement in 6 cases and disagreement in the remaining 15 are discussed. General diagnostic recommendations are given to help secure optimal diagnostic information.
In three male neonates, successfully treated for posterior urethral valves, unilateral "pseudotumour" deformity of the collecting system ensued. Two of them had, preoperatively, a marked ipsilateral perirenal urinary extravasation (urinoma), one in addition gross contralateral renal backflow with moderate leakage to the renal capsule. The other one had slight contralateral backflow to the kidney parenchyma but also urinary ascites. The third patient had considerable unilateral renal backflow on the side of developing deformity but no perirenal extravasation. Out of the three patients, two had bilateral Grade IV-V vesico-ureteral reflux, in the third patient reflux of the same degree was present and this only on the side opposite to the deformity. In all three patients the kidney with the "pseudotumour" became taller and more slender than the normally developing mate. There was either slight or no loss of renal parenchyma. At the last follow-up, 1-3 years after diagnosis of the valves, kidney function was normal.
Although blunt abdominal trauma is common in children, the G-I tract is involved only rarely. However, this paper deals with a series of 24 cases in whom a hollow viscus was injured. We present the radiological and operative findings as well as the results of subsequent radiological workup in patients who had no surgery. Contrary to the recent trend which stresses early abdominal CT as critically important for guiding treatment we have retained a more conservative attitude. Our present protocol includes plain radiography frequently combined with sonographic screening of the abdomen. In patients with stable vital signs and when necessary, we follow up with scintigraphy. CT is reserved for complicated cases with obscure clinical manifestations which do not fit the plain radiological findings and also cases running an unexpected course.
This report deals with an unusual case of spondyloepiphyseal dysplasia, where the diagnosis was suspected clinically and radiologically and where cartilage and osseocartilaginous specimens were taken for ultrastructural and biochemical analyses. Unlike cases described previously the electron microscopic studies of chondrocytes showed dilatation of the rough endoplasmic reticulum cisternae. The dilated cisternae contained an amorphous substance, and the picture is suggestive of a metabolic disturbance. The results of the biochemical analyses were within the normal range and did not indicate storage of glycosaminoglycans.
A simple and exact method for evaluation of suspected or overt slip of the femoral capital epiphysis is revived. This procedure gives an estimate of all degrees of abnormality from the early stages to those with gross deformity. In our experience slip of the femoral capital epiphysis is always posterior and in approximately 80% of cases bilateral. Therefore, both hips should be examined whenever this condition is suspected. The method provides for examination of each hip separately. Strict adherence to the procedure also provides a precise way of assessing the femoral anteversion angle, significant in many orthopedic disorders. For the latter purpose access to a particular nomogram is mandatory.
A case of epidermolysis bullosa dystrophica ( EBD ), neonatally corrected atresia of the pylorus and early infantile development of urinary tract manifestations is reported. Owing to misinterpretation of radiological findings, instrumental treatment of the urethra was employed. It resulted in complete and permanent obliteration of the lumen. While the skin disorder has remained slight to moderate in severity, recurrent keratitis has resulted in impaired vision.
A rare case of bone dysplasia with moderate modelling anomalies of the long bones, joint dislocation, osteolysis, a congenital calvarial defect, a patent ductus arteriosus, a mild degree of hypospadias and hypertensive renal cystic dysplasia is presented. In spite of many similarities with the Hajdu - Cheney Syndrome (HCS), important dissimilarities indicate that this case may be either an extreme variant of HCS, or a closely related, separate entity for which the term Arthro-Osteo-Renal Dysplasia is proposed.
The renal growth pattern following uninephrectomy for Wilms' tumour was analysed in 18 boys and 18 girls by means of a comparison between renal parenchymal and body surface area. No straightforward correlation was noted between age at nephrectomy, chemo- and radiation therapy applied, and subsequent renoprival dimensions. Five years on average after operation, a mean renal size corresponding to approximately 125 per cent of the normal for one of two healthy kidneys was recorded. However, wide variations in size were observed, but only 10 patients had a compensatory growth of the remaining kidney equivalent to that seen in patients nephrectomized for unilateral, non-malignant renal disease.
The value of glucagon to facilitate hydrostatic reduction of childhood ileo-colic intussusception was tested in a series of 188 consecutive cases. Sixty-nine patients selected at random received 0.05 mg glucagon/kg body weight intramuscularly before attempts at reduction were started. The rate of successfully accomplished reductions was 84 per cent in the test group and 76 per cent in the control group (not significant). The time for accomplished reduction was approximately equal in the two groups. After 3 unsuccessful attempts at hydrostatic reduction patients in the control group received glucagon. The subsequent attempt at reduction proved successful in slightly more than every second case. No complications occurred. In general, glucagon was not found to improve the rate of employed reductions nor on the time necessary for reduction to take place. The investigation indicated, however, that there may be a limited benefit of adjuvant medication of glucagon. After two unsuccessful barium enemas glucagon administration is therefore recommended before a third attempt at reduction is performed.
The experiences gained with conventional radiographic examinations as a means of excluding childhood ileo-caecal intussusception are presented. According to these, a supplementary barium enema is indicated a) in patients under 6 months of age, b) in patients presenting with scant intestinal gas, c) in patients where the caecum cannot be outlined, d) in patients with a possible mass lesion of the colon, e) in patients with obstruction presumably due to intussusception, and f) in patients with discrepancy between the clinical and radiologic findings (1, 4). In all other patients the barium examination is not used. The key to a successful application of this protocol is a thorough familiarity with the limitations and potentials of the conventional abdominal examination.
The full variety of secondary and associated upper tract abnormalities concurrent with obstructive valves seems not to be generally appreciated. While upper urinary dilatation and impaired renal function are features regarded as almost obligatory, there are cases at the other end of the spectrum with surprisingly slight or no abnormalities of the bladder, ureters or collecting systems. Therefore a negative IVP does not exclude posterior urethral valves in any boy with symptoms pertaining to the urinary tract. A MCU should invariably be included in the radiological work-up.
In an attempt to evaluate the long-term renoprival growth in patients uni-nephrectomized for Wilms' tumour, renal length was related to the length of the lumbar segment L1-L3. The results of this study indicated that spinal growth retardation, secondary to applied adjuvant treatment, invalidated employment of the method for this particular purpose. A computerized analysis showed no straight-forward correlation between age at operation, chemotherapy utilized, irradiation and the post-treatment renoprival growth as established by substitution of the observed L1-L3 length with a mathematically corrected value. Emergent figures exclude significant compensatory growth of the remaining kidney.
Barium peritonitis secondary to perforation of the gut is an infrequent complication of radiological G-I tract examinations. It is thought to imply a high mortality rate. However, this conception seems to be based on data obtained during the early 1950's. More recent case reports and small series indicate a more favourable prognosis depending on effective antibiotics and successful maintenance of severely ill patients. The scant information concerning the fate of pediatric cases warranted presentation of the experience of 5 patients, who all survived. Our approach to treatment and the suggested use of barium studies, in particular barium enema, in obscure abdominal emergency cases are offered.
The results of light and electron microscopic examination and of biochemical proteoglycan studies of costochondral and iliac crest biopsies from a recently diagnosed case of Dyggve-Melchior-Clausen dysplasia are reported. At light microscopy of resting cartilage large lacunae containing clusters of five or more chondrocytes were seen in some areas. In the hyaline cartilage there were scattered fibrous foci but no mineralized areas. Electron microscopy revealed chondrocytes containing widened cisternae of rough endoplasmic reticulum and vesicles coated with a smooth single-layered membrane. The content of the cisternae and of the vesicles was amorphous. Throughout the cartilage a considerable proportion of the chondrocytes displayed more or less pronounced necrobiotic changes. The biochemical analysis showed an increased amount of glucosaminoglycans in the cartilage and indicated that the ability of proteoglycan monomers to reaggregate to hyaluronic acid chains was decreased. Our findings support the suggestion that Dyggve-Melchior-Clausen dysplasia is due to a disturbance in proteoglycan metabolism.
Neuroblastoma presenting with wide spread osseous metastases (stage IV) is generally considered incurable. However, rare cases of spontaneous regression and cure are on record. This report deals with such a case in which an infant received steroids as sole treatment for developing skeletal pain. Established metastases to the brain, scalp and skeleton disappeared. Today 4 1/2 years later the patient is free from symptoms and signs of tumour.
Explore the source record for details and available documents.
In infants and children the association of paravertebral widening and abdominal tumour has been regarded as typical of neuroblastoma. However, a paraspinal mass may develop in other malignant tumours too, but seems to be extremely rare in nephroblastoma. The fourth case so far on record is reported. Microscopically the tumour was of the sarcomatous clear cell variant.
Pulmonary consolidation, rarefaction of adjacent ribs and pleural effusion, a triad frequently considered characteristic of actinomycosis, seemed in the present case to support this serologically entertained diagnosis. However, the subsequent course of disease disclosed the most uncommon association of actinomycosis an metastatic Ewing's sarcoma of the lung. The possibility of combined occurrence of an opportunistic infection by actinomyces and malignancy, calls for open biopsy in cases not responding to penicillin treatment or with an unexpected evolution of the clinical manifestations.