Angiographic evaluation of conjoined twins

Two sets of conjoined twins were studied by angiocardiography and cerebral angiography. Conjoined heart was demonstrated in the thoracopagus twins and surgical separation was impossible. Cerebral angiography disclosed the separate circulations in craniopagus twins and surgical separation was performed.Presented at 14th Meeting of the European Society of Pediatric Radiology, Luzern, May 11–14, 1977     

The computer as an aid in the daily practice of pediatric radiology

The use of a relatively small computer limited to a Department of Radiology has considerable benefit in report generation and information retrieval. In addition, the arithmetical tasks of pediatric radiological reporting procedures such as bone age and scanograms have been automated, and other helpful features have arisen.This paper was presented at the 14th Meeting of the European Society of Pediatric Radiology, Luzern, Switzerland, May 12–14, 1977     

The relationship of juvenile lumbar disc disease and Scheuermann's disease

Between 1969 and 1979 five children were found to have lumbar disc disease and were evaluated for clinically unsuspected thoracic spine abnormalities. Of these five children, two had Scheuermann's disease and one had disc space narrowing associated with Schmorl's nodes. One other had narrowed disc spaces without bony defects, and one had a normal thoracic spine. A unitary concept of childhood lumbar disc disease, Schmorl's nodes and Scheuermann's disease is suggested.     

Complement in cystic fibrosis

Complement components C3, C4, and C3A were estimated in 30 patients with cystic fibrosis aged 1 to 21 years (MF=1614) and were compared with results in 40 healthy, age-matched subjects. The influences of the clinical score, sputum microbiology, and the patients' sex were also investigated. In contrast to most previous communications, this paper shows that, compared to the control group, a significant decrease of C3 (P<0.001) and C4 (P<0.02) was observed whereas C3A levels were not altered. There were no increases in complement. Shwachman-scores above or below 70 did not influence the complement levels, nor did exacerbations of the disease change the levels. No influence of the patients' sex could be shown. Pseudomonas aer. in the sputum was clearly associated with complement defects (14/18). Alternative-pathway involvement of complement activation could be demonstrated in 32%. The results make complement activation due to pulmonary infection most likely. The defects observed probably represent secondary changes.Presented at the VIIth Annual Meeting of the European Working Group for Cystic Fibrosis, Dresden, June 20–21, 1977Dedicated to Professor E. Zweymüller on the occasion of his 60th birtday     

Practical interest in the detection of functional abnormalities in infants and children with lung disease

There are new techniques which have been developed in order to assess objective functional data concerning the severity and type of abnormalities in infants and children with lung disease. In the present review some applications of the various techniques are given in connection with deductions, which can be based on such functional findings. Insight into the mechanisms leading to lung disease and the institution of appropriate therapeutic guidelines largely depend on whether the degree of pulmonary hyperinflation or restriction, of bronchial obstruction, of bronchial hyperreactivity, of ventilation inequalities and the function of gas exchange can be objectively evaluated. Recent advances in the non-invasive assessment of lung function in infants and children promises progress in this task.In part presented at the Annual Meeting of the European Society for Pediatric Research, Zürich, September 1–4th, 1991 and at the Postgraduate course on infant lung function testing of the American Thoracic Society, Miami, May 16–21th 1992     

Water retention in the acute phase of Kawasaki disease: relationship between oedema and the development of coronary arterial lesions

Despite intravenous immunoglobulin therapy, a certain percentage of patients with Kawasaki disease (KD) still develop coronary arterial lesions (CAL). In an effort to find new combined therapies to reduce the incidence of CAL, we focused on the oedema which can be an important sign of the increased vascular permeability in KD. A total of 127 patients with KD were included in the retrospective study. Serial weekly changes in serum sodium and albumin levels from the 1st to the 4th week of illness were examined. In addition, the maximum rate of increase in body weight from admission to the 14th day of illness was evaluated. Serum sodium levels (mEq/l) in only the 2nd week of illness were significantly lower in patients with CAL than in those without CAL (mean ± SD, 135.5±4.5 versus 138.0±2.4, P <0.05). Serum albumin levels in all 4 weeks were significantly lower in patients with CAL than in those without CAL ( P <0.001). The maximum rate (%) of increase in body weight from admission to the 14th day of illness was significantly higher in patients with CAL than in those without CAL (ranges and median values, 0–12.3 (7.0) versus 0–10.3 (3.2), P <0.001). Conclusion:these results suggest that water retention in the acute phase of Kawasaki disease may be a risk factor for CAL, and water intake of both infusion and oral intake should be kept to a minimum in order to avoid progressive oedema.Abbreviations ASA acetylsalicylic acid - CAL coronary arterial lesions - HGF hepatocyte growth factor - IVIG intravenous immunoglobulin - KD Kawasaki disease - MBW maximum rate of increase in body weight - VEGF vascular endothelial growth factor     

Lung disease in the very immature neonate: radiographic and microscopic correlation

Radiographic-pathologic correlation of pulmonary patterns has not been performed in very small preterm infants below 28 weeks of gestation. The radiologic findings of linear intersititial densities or generalized airspace opacity coincided with histologic changes of edema and hemorrhage and indicate that this is the most frequent abnormality producing radiographic pulmonary opacification in infants of 23–27 weeks gestation. On occasion, parenchymal immaturity alone results in lung opacification, reflecting the established interpretation of diffuse atelecatasis as the histologic-radiographic finding in respiratory distress syndrome.This work was supported in part by HHS grant HL-07496, from the National Institutes of HealthPresented at the 28th Annual Meeting of the Society for Pediatric Radiology, April 18–21, 1985, Boston, Massachusetts, USA     

Radiological assessment of fifty patients with juvenile rheumatoid arthritis: Correlation with clinical and laboratory abnormalities

The radiological status of fifty patients with rheumatoid arthritis was re-evaluated in a follow-up study ranging from 1–30 years. A skeletal survey was compared with the radiographs taken at the onset of the disease and changes were graded according to severity (0-IV). — A significant correlation was found between those showing moderate or severe radiological changes and those in whom the age of onset was less than 6 years. A similar correlation was seen with increasing duration of disease activity, intermittent or continuous course (as opposed to monocyclic. Goel and Shanks [4]) poor functional status, physical deformity, splenomegaly, lymphadenopathy and serological abnormalities. — Patients in the group treated by steroids showed a higher incidence of moderate or severe rediological changes. — No association, either positive or negative, was observed between the radiological grade and rheumatoid rash, increased B₁C/B₁A globulins or the sex of the patient.     

Puberty in Laron type dwarfism

The onset and progress of puberty was followed in 18 patients (7 males and 11 females) with Laron-type dwarfism (LTD). The boys had delayed puberty, testicular enlargement occurring between 12–14 years being the first sign. The first conscious ejaculation occurred between 17–21 years and full maturity was reached after the age of 22. In girls menarche occurred between 13–14 years and full maturity was reached between 16–19 years. Two patients—one male and one female—have children.Presented at the Symposium on Pathophysiology of Puberty, Bologna, October, 1979     

Colorectal resection and anal anastomosis with an intraluminal stapler in Hirschsprung's disease

Twenty patients aged 5 months — 4 years (mean 14 months) with Hirschsprung's disease were operated upon. In all cases a pull-through resection and stapled circular coloanal anastomosis was performed, in 11 with a single-stapling technique and in 9 with double-stapling. Isopaque rectography 4–10 days postoperatively showed an intact anastomosis in all 20 patients, i. e., there were no clinical or subclinical leaks. On palpation 4–6 weeks post-operatively, there were signs of stenosis in 4 patients. However, no. 9–11 Hegar dilators passed easily and there was no residual stenosis at follow-up 3 months after surgery. The technique can be recommended in children over 6 months of age.     

Mandibular and para-mandibular tumors in children

Sixteen cases of mandibular tumors or paramandibular soft tissue tumors with mandibular involvement are reported. These include such rare mandibular tumors or tumor-like conditions as melanotic progonoma, intraosseous haematoma secondary to von Willebrand's disease, post-irradiation osteosarcoma, monostotic eosinophilic granuloma, aneurysmal bone cyst and osseous hemangiopericytoma. Three cases of cherubism, one of fibrous dysplasia or aggressive fibromatosis and one of central giant cell reparative granuloma are also reported. The soft tissue tumors comprise round cell sarcoma, parotid adeno-carcinoma with generalised metastases, embryonal rhabdo-myo-sarcoma, neuro-fibro-sarcoma and congenital cystic hygroma. In all the cases the disease was well advanced when the patient presented for X-ray examination. The specific X-ray diagnosis of mandibular and paramandibular tumors in childhood is more difficult than that of similar tumors in other parts of the body.Presented at the 24-th Annual Meeting of the American Society for Paediatric Radiology, San Francisco, California, USA, March 22–27, 1981; Presented at the 18-th Annual Meeting of the European Society of Paediatric Radiology, Oslo, Norway, May 20–22, 1981 and at the 32-nd Annual General Meeting of the Royal Australasian College of Radiologists, Christchurch, New Zealand, September 20–25, 1981     

Faecal excretion in infants

Data on normal defaecation patterns in relation to diet during the first months of infancy are very limited. We therefore investigated in a prospective study faecal weight and gastro-intestinal passage time of breast fed (n=12) and formula fed (n=14) male infants. These were studied in 72 h collecting periods at the age of 17, 35, 57, 87 and 113 (±4) days. Breast fed infants had a significantly lower daily dry faecal weight than formula fed infants in all periods investigated (median at the age of 113 days: 0.28 (0.17–0.75) g/kg and 0.81 (0.22–1.2) g/kg, respectively). Breast fed infants showed a large variation of gastro-intestinal passage time (6.79 h [range: 1.79–13.38 h] at the age of 17 days, 21.84 h [range: 5.41–75 h] at the age of 113 days). Comparable values of formula red infants were 13.75 h (range: 7.13–35.25 h) and 17.42 h (range: 5.38–36.5 h). Despite the efforts of approximation of infant formula to breast milk, differences of defaecation patterns in relation to diet are still relevant at this age and have to be considered in clinical practice.These data have been presented in part at the 26th Scientific Meeting of the German Society of Nutrition, Bonn, 6–7 April 1989.     

Commentary: Sonography in the evaluation of children following blunt trauma: Is it to be or not be?

Over the past decade CT scanning has become generally accepted in North America as the diagnostic modality of choice for the evaluation of abdominal injury in children following blunt trauma [1–5]. Recently, there has been increasing interest in the use of sonography as the primary screening examination in this area. Initial studies utilizing sonography in the evaluation of trauma patients focused primarily on identifying hemoperitoneum in adults [6–8]. More recent studies have also attempted to evaluate the accuracy of sonography for the diagnosis of solid viscus injury [9–14]. Filiatraut and colleagues recently reported a long and successful experience using sonography for the investigation of blunt abdominal trauma in children [12]. Their work in this area should be applauded. However, whether wide-spread application of this modality can be successful remains uncertain. In the space below a critical evaluation of sonography and CT in the assessment of injured children is presented.     

Congenital eventration of the diaphragm — 45 years' perspective

Fifty-five children with congenital eventration of the diaphragm were operated upon in a single tertiary-care children's hospital during the 45-year period from 1948 to 1992. The study was divided into three periods: 1948–1962, 1963–1977, 1978–1992. There were 14 patients in the first group, 26 in the second, and 15 in the third. Children later in the series presented earlier and with more acute symptoms. Mortality was related to severe pulmonary hypoplasia and associated anomalies, especially chromosomal defects: 14% during the first period, 27% during the second, and 7% during the third.     

Involvement of the central nervous system in renal hypertension

Involvement of the central nervous system (CNS) is a major complication of renal hypertension and is usually due to hypertensive encehalopathy. During a 17-year period we observed 25 children with renal hypertension associated with CNS manifestations in the absence of (group A) and 33 in the presence of advanced renal failure (group B). Convulsions were the most important symptom (65% in both groups combined). Other manifestations were reduced consciousness (69%), visual disturbances (28%), hemiplegia (14%), and cranial nerve palsy (10%). In 56% of children in group A, hypertension was recognised only after appearance of CNS symptoms. Three patients in group A and 19 in group B died. Intracranial haemorrhage associated with hemiplegia was found in 3 cases. From 1970–1977 to 1978–1986 the number of patients and the mortality in group B declined dramatically, probably as a result of improved antihypertensive and renal replacement therapy. In contrast, in group A the number of patients and of CNS symptoms remained similar. The study underlines the importance of frequent blood pressure monitoring in presence of acute or chronic renal disease.     

Radiologic diagnosis of subglottic hemangioma in infants

Recent experience indicates that the most accurate and least dangerous method of diagnosing subglottic hemangioma is soft tissue radiography of the neck. We suggest, that the radiologic appearance of subglottic hemangioma is pathognomonic in most cases in young infants. Multiple instrumentation under general anaesthesia be it for diagnostic or follow-up investigation, must be avoided. With conservative approach and in view of the benign natural history of this disease fewer infants should develop severe morbidity and mortality.Presented at The Canadian Association of Radiologists 36th Annual Meeting, March 12–16, 1973.     

Determinants of hemoglobin concentration in cyanotic heart disease

Summary Children with cyanotic heart disease were studied to determine the effects of chronic hypoxemia, iron stores, and age on hemoglobin. Red cell mean corpuscular volume >90th percentile was found in 72% of children and hemoglobin was higher in this group than in patients with a mean corpuscular volume in the 10–90th percentile or <10th percentile (p<0.05). In children presumed iron sufficient and with oxygen saturation >75%, multiple regression analysis showed that age (p<0.0001) and oxygen saturation (p<0.0001) were associated with hemoglobin. Significant correlations between hemoglobin and oxygen saturation were present for children <2 years (r=–0.50) and 2–11 years (–0.48), but not for patients>11 years. We conclude that variables other than oxygen saturation, including age and iron stores, are important in determining hemoglobin concentration in cyanotic heart disease.     

Upper clayx reno-vascular obstruction in children: Fraley's syndrome

Intrarenal vascular obstruction of the upper calyceal infundibulum has been distinguished by Fraley as an entity separate from an asymptomatic vascular impression. Clinically, this form of intrarenal vascular obstruction presents as nephralgia or hematuria. This infundibular obstruction is caused by normal renal vessels, usually the upper segmental artery: radiologically, the vessels produce a constant well-defined filling defect in the infundibulum at IVP, and, the upper calyx group is distended and shows delay in emptying. We found this condition in 14 children from 1968–1975. Control studies showed no progression in the radiological findings, even over a long period. In one of our cases angiography was indicated to exclude tumour or cyst; in another case, surgery was necessary. Complications are urinary tract infection or stone formation. Therefore, children with Fraley's syndrome require regular clinical examination.Presented at the 12th Meeting of the European Society of Pediatric Radiology, Stockholm, Sweden, May 19–22, 1976     

Radiographic features of fucosidosis

Fucosidosis is an uncommonly recognized lysosomal storage disease which presents with psychomotor deterioration and progressing in some cases to death. The roentgenographic findings in five boys from three families are described. Skeletal abnormalities of a dysostosis multiplex nature are present and though not diagnostic should direct the clinician to perform appropriate biochemical and tissue assay studies to establish the diagnosis.This paper was presented in part at the 13th Meeting of the European Society of Pediatric Radiology, Stockholm, Sweden, May 19–22, 1976