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Biomedical subjects

E Ring

Publications and source records attributed to E Ring.

At least 37 records · Page 2Linked to original sources

Doppler sonography in congenital ureteropelvic junction obstruction and multicystic dysplastic kidneys.

The accurate diagnosis of severe congenital ureteropelvic junction obstruction (UPJO) and the indications for surgical intervention are a matter of debate. Differential diagnosis from, for example, congenital cystic kidney malformations is important. In the present study we analysed the value of Doppler sonography (DS) for assessment of obstruction in congenital UPJO and its usefulness for differential diagnosis. A total of 138 infants and children were examined. Forty-three healthy infants and 24 children who had had a pyeloplasty at least 1 year previously served as control groups. Seventy-one children with UPJO and cystic kidney malformations underwent DS in addition to the usual examinations of intravenous urography and scintigraphy. Resistive Index (RI) was measured in both kidneys. Twenty infants showed decompensated UPJO and had needed an operation. Preoperatively all showed significantly increased RI (RI = 74%), which returned to normal after operation (RI = 68.3%). Thirty-nine children with UPJO could be managed conservatively, showing compensated obstruction on diuretic renography. On DS they had normal and symmetrical RI values (RI = 67%). Eleven children had multicystic dysplastic kidneys; 4 underwent nephrectomy. On DS all showed either absence of perfusion signals or very low flow velocities with very high RI values (RI = 90-100%) in the renal vessels. One child was found to be suffering from a cystic nephroma. DS was able to reveal quite normal vessel distribution in renal parenchyma with slightly elevated RI values (RI = 80%). Our results indicate that DS a non-invasive, non-ionising method of investigation, may serve as an additional functional parameter for defining accurately the degree of obstruction in UPJO.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Ultrasound and MRI findings in a case of childhood amyloid goiter.

Goiter secondary to amyloidosis is rare in clinical practice and only a few descriptions of its radiologic features have been reported. We present the ultrasound and MRI findings of thyroid amyloidosis in a 7-year-old Turkish boy with familial Mediterranean fever.

Amyloidosis↗

Scintigraphy and sonography in reflux nephropathy: a comparison.

The evaluation of reflux nephropathy (RNP) is a major task in the following up of children with vesicoureteral reflux (VUR). Adequate monitoring of RNP development depends on the frequent use of sonographical and scintigraphical examinations. In order to evaluate the accuracy of sonography in monitoring RNP 63 patients suffering from VUR (mean age: 8.8 years, range: newborn to 18 years, male:female = 19:44) were examined prospectively. We calculated renal size sonographically and compared our data to static and dynamic renographic findings. Our results showed a good correlation between relative DMSA uptake and sonographically estimated relative renal size (r = 0.95, P < 0.001), whereas not unexpectedly the correlation between relative kidney function and sonographically estimated renal size was poor (r = 0.83, P < 0.01). We therefore believe, that in monitoring children with VUR, sonography can offer accurate information on questions about renal size and renal parenchymal structure as well as pelvic distention. With very small kidneys as well as with kidneys showing severe dilatation and above all for evaluating renal function, scintigraphy proved to be the better method.

Adolescent↗

Storage material from urine and tissues in the nephropathic phenotype of infantile sialic acid storage disease.

We analysed urine and tissue specimens from two nephrotic infantile sialic acid storage disease patients (nISSD) for free and bound sialic acids in comparison to non-nephrotic ISSD patients (ISSD), patients with minimal change nephrosis (nControl) and normal controls (Control). No differences in the excretion of urinary free sialic acid could be detected between ISSD and nISSD urines. Sialyloligosaccharide fractions were only slightly elevated and of apparently normal composition. Owing to glomerular dysfunction, measurable quantities of protein-bound sialic acids were present in nISSD and nControl. In nISSD tissues, free sialic acid was elevated 18-100-fold above control and 3-12-fold above Niemann-Pick A (NPA) samples. The storage of membrane-bound sialic acid was slightly increased compared to control tissues, but equal to those from NPA, thus reflecting an unspecific increase of membranes due to lysosomal storage. According to these results no major biochemical differences were detectable between ISSD and nISSD. The nephrotic syndrome in nISSD could not be related to a general deficit in the sialylation of glycoproteins. Nevertheless, a cell membrane-specific alteration in sialoglycoproteins of glomerular cells might still be possible.

Glycosaminoglycans↗

The role of sonography in the evaluation of gastro-oesophageal reflux--correlation to pH-metry.

Sonography was compared to pH-metry and/or oesophagomanometry to evaluate the accuracy of sonography in the early diagnosis of gastro-oesophageal reflux. Thirty children with a mean age of 72 days (21-252 days) were studied. The results showed that specificity of sonographic diagnosis was 87.5% and sensitivity was 100% (with P less than 0.001). Sonography also proved helpful in providing both functional and morphological data in addition to pH-metric results. This study therefore suggests that sonography is useful as the first approach in the diagnosis of vomiting babies as it is non-invasive and provides sufficient diagnostic accuracy.

Female↗

[Hemofiltration in acute neonatal metabolic crisis].

Continuous arteriovenous hemofiltration (CAVH) and venovenous hemofiltration (CVVH) were successfully used in the treatment of two neonates, one with maple syrup urine disease and the other with ornithine transcarbamylase deficiency. The plasma concentrations of branched-chain amino acids as well as of ammonium decreased to non-toxic values within a few hours and the clinical improvement in the encephalopathy was striking. Our results and a review of the literature show that hemofiltration is more effective than peritoneal dialysis in removing toxic metabolites. The best clearance rates are achieved by intermittent hemodialysis, but continuous hemofiltration is a simple, safe and effective alternative and is sometimes tolerated better by critically ill infants.

Amino Acids, Branched-Chain↗

[Acute interstitial nephritis in childhood].

Acute tubulointerstitial nephritis (TIN) was diagnosed during a 10-year period in 6 previously healthy children (4 boys) with a mean age of 10 years (range 2.5-14 years). All presented with a non-specific history. First laboratory findings were nearly identical, with a raised erythrocyte sedimentation rate and non-oliguric renal failure; the urine contained protein, glucose, and leucocytes, but no bacteria. A serologically proven infection (streptococcal infection 2x; mononucleosis 1x) or drug-related TIN (penicillin) was present in 4 children; in one, both conditions could have been responsible. One girl suffered from idiopathic TIN with uveitis. Rapid progression of renal failure occurred in 3 children, but the response to steroid therapy was satisfactory. No dialysis was necessary and all patients showed a complete recovery. We think that acute TIN occurs more frequently than suggested previously. Many episodes are probably mild or subclinical and the rate of spontaneous remission is high. The results of initial laboratory evaluation in conjunction with acute renal failure and predominantly proximal tubular dysfunction, are sufficiently typical to arouse suspicion of acute TIN at an early stage, although they do not permit any conclusion as to the aetiology.

Acute Kidney Injury↗

Urinary excretion of N-acetyl-beta-D-glucosaminidase in proteinuric states.

Urinary excretion of N-acetyl-beta-D-glucosaminidase (NAG) was studied in 22 children with proteinuria and a normal glomerular filtration rate. Twelve patients had minimal change nephrotic syndrome (MCNS), 10 children suffered from different types of glomerulonephritis (GN) without tubulointerstitial renal disease. Sixty-six random urine samples were analyzed for protein, creatinine and NAG. There was a strong positive correlation between proteinuria and urinary NAG activity (r = 0.79, p = 0.0001). The raised NAG activities in proteinuric states turned to normal during remission of the disease. Patients with MCNS and GN did not differ significantly and had nearly identical correlation curves. Our results seem to indicate that functional changes in the renal tubular cells probably caused by protein resorption are responsible for this association. Raised NAG activities in proteinuria reflect the activity of the disease but cannot distinguish patients with MCNS and GN.

Acetylglucosaminidase↗

Five years experience with continuous extracorporeal renal support in paediatric intensive care.

Continuous arterio-venous haemofiltration (CAVH) and continuous veno-venous haemofiltration (CVVH) were used as renal support in 52 critically ill infants and children with acute renal failure. The majority of the patients were on mechanical ventilation (90%) and needed vasopressor support (85%). Uraemia was satisfactorily controlled with both treatment modes. Post-treatment serum urea levels were not different between survivors (94 +/- 8.8 mg/dl) and non-survivors (99.5 +/- 8.8 mg/dl). There were significant differences between survivors and non-survivors in the mean arterial pressure (64.7 +/- 3.8 vs 48.0 +/- 2.2 mmHg, p less than 0.001), the number of organ system failures (2.9 +/- 0.16 vs 3.8 +/- 0.21, p less than 0.025), and the severity of illness assessed by the acute physiologic score for children (APSC 19.4 +/- 1.9 vs 26.3 +/- 1.9, p less than 0.01). The overall mortality was 48%. The mortality in the CVVH group (65%) was higher than in the CAVH group (40%). Death was significantly related to sepsis (p less than 0.005) and multiple system organ failure (p less than 0.005). A major complication during CAVH was one femoral artery thrombosis after 12 days of treatment. Technical problems were only observed during CVVH. CAVH and CVVH are safe and effective methods of continuous renal support for critically ill paediatric patients with multiple system organ failure. CAVH is simpler, needs no specially trained staff and seems to the ideal renal replacement system for critically ill infants.

Acute Kidney Injury↗

Amniotic fluid N-acetyl-beta-D-glucosaminidase activity and renal abnormalities.

Amniotic fluid N-acetyl-beta-D-glucosaminidase (NAG) activity was measured in 21 normal pregnancies to determine reference values, and in 10 pregnancies with fetuses who had prenatally recognised renal and urinary tract malformations. The normal values obtained at 17-19 and 28-31 gestational weeks did not differ, and an activity of 105 units NAG/mmol creatinine was the upper limit of the normal range. Raised NAG activities were found in four fetuses with oligohydramnios and severe bilateral renal disease, and also in two infants with a normal amount of amniotic fluid, only unilateral renal lesions, and a normal renal function after birth. Three of four cases with normal NAG activities had malformations without kidney damage, but one baby with prune belly syndrome was subsequently found to have dysplastic kidneys and renal failure. Our preliminary results suggest that a high level of NAG activity in the amniotic fluid indicates unilateral or bilateral kidney damage but is not proof of intrauterine renal failure.

Acetylglucosaminidase↗

Nitrogen and amino acid balance during total parenteral nutrition and continuous arteriovenous hemofiltration in critically ill anuric children.

Amino acid balance and nitrogen balance during total parenteral nutrition (TPN) and continuous arteriovenous hemofiltration (CAVH) were investigated in 11 critically ill anuric patients during the first 7 days after onset of anuria. Nitrogen intake ranged from 0.115 +/- 0.013 (SEM) g/kg/day on day 1 to 0.291 +/- 0.029 (SEM) g/kg/day on day 7. After 7 days of TPN, 9 patients had a positive cumulative protein-N balance of 287.52 +/- 68.52 (SEM) mg/kg, 2 patients had a negative balance of 781.8 and 1,103.2 mg/kg, respectively. Mean amino acid loss in ultrafiltrate was 0.159 +/- 0.008 (SEM) g/kg/day. Four patients died without recovery of renal function.

Acute Kidney Injury↗

[Therapy and prognosis of children with prenatally diagnosed urinary tract abnormalities].

Urinary tract malformations are recognized already prenatally to an increasing extent, but the indications for, and the time of surgical intervention are still a matter of debate. The present study is an analysis of the data collected over an 80-month period of 76 patients whose urinary tract malformation was suspected prenatally and confirmed after birth. Patients with lethal malformations whose disease led to abortion or intrauterine death were excluded. Ureteropelvic junction obstruction was present in 47% of the patients and obstructive uropathy accounted for 67% of all malformations. In contrast, vesicoureteric reflux was found only in eight patients. Thirty seven infants (49%) required surgical intervention and 61% of all operations were performed during the first month of life. 57% of these early interventions were temporary diversions. The indication for surgical treatment was based on sonographic and radiological assessment of the malformation and on the results of functional assessment. In many instances surgery was delayed until indicated on the basis of the findings of the follow-up studies. All reconstruction operations were successful. Prenatal diagnosis benefitted most children, especially those with the most severe malformations. No patient died as a result of the renal condition and the prognosis is grave in only one child, who is likely to go into terminal renal failure in childhood.

Congenital Abnormalities↗