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Biomedical subjects

E Ring

Publications and source records attributed to E Ring.

At least 55 records · Page 3Linked to original sources
PubMed1990

[Familial hypophosphatemia].

The main features of X-linked dominant renal hypophosphatemic rickets are illustrated in this study of two patients who presented with rickets, deformities of the lower limbs, and small stature. Hypophosphatemia secondary to a reduction in renal tubular reabsorption of phosphate and a defect of vitamin D hydroxylation are the hallmarks of the disease. The best measure of renal handling of phosphate is to determine the tubular maximum of phosphate transport normalized for glomerular filtration rate (TmPO4/GFR). Determination of the calcium/creatinine ratio of a random urine sample proved to be a good additional parameter to control supplementation of phosphate and calcitriol. Diagnosis of the disease in our two patients enabled us to recognize renal hypophosphatemic rickets in both mothers and in two further ancestors of one patient. Both mothers suffered from early arthrosis.

Calcitriol↗
PubMed1990

Continuous arteriovenous hemodialysis in critically ill infants.

Continuous arteriovenous hemofiltration (CAVH) and continuous arteriovenous hemodialysis (CAVHD) were performed in 6 critically ill infants with hypervolemia and anuria using the Amicon Minifilter and Minifilter plus. The ultrafiltration rates produced during CAVH and CAVHD were 2.1 +/- 0.3 and 1.7 +/- 0.4 ml/min/m2 and the urea clearances were 2.0 +/- 0.4 and 6.6 +/- 1.4 ml/min/m2. Serum creatinine and urea levels increased during CAVH and decreased during CAVHD. Both treatment modes were well tolerated by all infants. CAVHD is superior to CAVH in terms of urea clearance and maintains all advantages of CAVH such as simplicity and safety.

Acute Kidney Injury↗
PubMed1990

Retrospective diagnosis of Jeune's syndrome in two patients with chronic renal failure.

Two patients are reported who presented at the age of 12 1/2 years with advanced chronic renal failure. Clinical findings and renal histology were consistent with nephronophthisis. The retrospective diagnosis of Jeune's syndrome was possible in both. Cone-shaped phalangeal epiphyses of hands and feet and metaphyseal chondrodysplasia of femoral heads and necks were the prominent findings on skeletal survey. One patient had a small bell-shaped thorax; in the other this aspect was present on reevaluation of neonatal X-rays. Our patients support the view that nephronophthisis is the principal cause of renal failure in Jeune's syndrome and that a wider awareness of this association could lead to detection of more so far unrecognized patients.

Asphyxia Neonatorum↗
PubMed1990

Clinical scoring systems in children with continuous extracorporeal renal support.

Three physiologic scoring systems, the Physiologic Stability Index (PSI), the Pediatric Risk of Mortality (PRISM), the Acute Physiologic Score for Children (APSC), and the Therapeutic Intervention Scoring System (TISS) were applied to 32 critically ill infants and children with acute renal failure or multiple organ system failure undergoing continuous extracorporeal renal support. APSC was developed from the Apache II score. It describes 6 organ systems with 14 variables. PSI describes 7 organ systems with 34 variables, PRISM 5 organ systems with 14 variables. Simultaneously, the TISS was recorded at admission, 24, 48, 96 and 144 h later. All physiologic scores showed significant differences between survivors (S) (n = 18) and nonsurvivors (NS) (n = 14) from admission on. The highest significance was obtained with the APSC (admission score: S: 17.8 +/- 7.4 vs. 27.1 +/- 11.4 NS; p = 0.01; day 4: S: 10.3 +/- 6.1 vs. 26.1 +/- 10.8 NS; p = 0.0001). No significant differences in TISS and in the number of organ system failure were observed during the first 4 days of intensive care.

Acute Kidney Injury↗
PubMed1989

[The value of prenatal ultrasound screening exemplified by abnormalities of the urogenital system. Data from the Styrian malformation register 1985 to 1987].

Over a three-year period (1985 to 1987), the number of fetal malformations was entered into Styrian Malformation Register (SMR). The data were compared with those of the Austrian Ministry of Health. The SMR collected 137 cases of urinary tract malformations, but only six appeared in the Health Ministry statistics. This discrepancy resulted from the different data collection methods. Presently only a small percentage of fetal abnormalities is officially registered. Using the example of urinary tract malformations this paper shows the value of prenatal ultrasound screening. Such infants must be treated soon after delivery to avoid progression of parenchymal damage. Ultrasound examinations should not be restricted to pregnant women at defined risk. Registration of all prenatally diagnosed--or missed--malformations is a quality-control measurement and improves prenatal diagnosis.

Austria↗
PubMed1989

Continuous arteriovenous renal replacement systems for critically ill children.

Five different arteriovenous renal replacement systems were used to treat 23 critically ill oliguric or anuric children. Slow continuous ultrafiltration (SCU) was carried out for 8 patient days, continuous arteriovenous haemofiltration (CAVH) for 40, suction-supported CAVH for 56, continuous or intermittent arteriovenous haemodiafiltration (AVHDF) for 3, and continuous arteriovenous haemodialysis (CAVHD) for 24 days. SCU allowed excellent control of fluid overload in 4 patients within 47 +/- 17 h. Urea clearances ranged from 5.6 +/- 2.1 ml/min per m2 (spontaneous CAVH) to 15.3 +/- 3.7 ml/min per m2 (CAVHD) and enabled good control of azotaemia. Ultrafiltration rates of the different filters ranged from 1.6 +/- 0.3 to 11.5 +/- 2.4 ml/min per m2. The only serious complication was a femoral artery thrombosis in a 1.5-year-old boy. Minor side-effects were local bleeding at the entrance site of the arterial catheter and transient hypotension during suction-supported CAVH. Of 23 patients, 8 died because of progressive multiple organ system failure, a mortality of 35%.

Acute Kidney Injury↗
PubMed1989

Bone disease of primary hyperoxaluria in infancy.

A patient with primary hyperoxaluria type I in infancy is reported. He had renal insufficiency, but urolithiasis was absent. Demonstration of diffuse nephrocalcinosis by renal ultrasound contributed to early diagnosis. Prolonged survival leads to extensive extrarenal oxalate deposition. Repeated skeletal surveys showed the development and the progression of severe hyperoxaluria-related bone disease. Translucent metaphyseal bands with sclerotic margins, wide areas of rarefaction at the ends of the long bones, and translucent rims around the epiphyses and the tarsal bones were signs of disordered bone growth. Bone density generally increased with time indicating progressive sclerosis due to oxalate deposition in the previously normal bone structure.

Bone Diseases, Metabolic↗
PubMed1989

Continuous arteriovenous hemofiltration in premature infants.

Five critically ill premature infants with acute renal failure (ARF) and hypervolemia were treated by continuous arteriovenous hemofiltration (CAVH). Prostacyclin was used to prevent hemofilter clotting. Mean treatment duration was 53.6 +/- 14 h. Mean blood flow rates of 1.6 +/- 0.22 ml/min and filtration fractions of 17.2 +/- 3.7% produced mean ultrafiltration rates of 8.3 +/- 3.1 ml/kg.h. Fluid overload was easily corrected by means of CAVH. The mean prehemofiltration serum creatinine and urea levels were 2.3 +/- 0.4 and 77 +/- 29.7 mg/dl; the mean posthemofiltration levels were 2.38 +/- 0.43 and 92 +/- 34.4 mg/dl, respectively. Hemofilter clotting occurred every 14 h. Urinary output was restored in three infants. Three of the five infants died, but none of the deaths was related to ARF or CAVH. CAVH is a safe and simple method to control fluid and metabolic imbalances in critically ill premature infants.

Acute Kidney Injury↗
PubMed1989

[N-acetyl-beta-D-glucosaminidase (NAG) in the urine. An additional parameter for diagnosing the site of urinary tract infection].

Urinary excretion of NAG was determined in 32 patients aged four months to 15 years with urinary tract infection (UTI). Level diagnosis was made by means of commonly accepted clinical and laboratory criteria. Pathological enzymuria was present in all 15 patients with pyelonephritis while 12 of 14 children with cystitis had a normal urinary NAG excretion. Two patients with cystitis and 3 patients with questionable level diagnosis had elevated urinary NAG levels. According to these data the level diagnosis of UTI was changed in 16% of the patients by including the NAG values. Follow-up studies of nine patients showed a significant decrease of initially elevated urinary NAG levels after a 10-days course of antibiotic treatment and the values were within the normal range in 8 of 9 patients. Determination of urinary NAG excretion seems to be of definite value as an additional parameter for level diagnosis of childhood urinary tract infection.

Acetylglucosaminidase↗
PubMed1988

Vascular access for continuous arteriovenous hemofiltration in infants and young children.

The blood flow through the hemofilter device is the key point for ultrafiltrate production during continuous arteriovenous hemofiltration. It mainly depends on arteriovenous pressure gradient and vascular access, which is the major problem for arteriovenous hemofiltration in infants and small children. In infants, we inserted short 18-20 gauge catheters into the radial or brachial artery and the internal jugular vein and achieved mean blood flow and ultrafiltration rates of 6.5 +/- 2.2 and 1.3 +/- 0.3 ml/min, respectively. In small children, we placed 4 or 5 French catheters into the femoral vessels, and achieved mean blood flow and ultrafiltration rates ranging from 18.5 to 63.6 and 1.9 to 6.9 ml/min, respectively. The only catheter-related complication was a femoral artery thrombosis, which needed surgical revision. These results show that vascular access for arteriovenous hemofiltration in infants and small children provides sufficient blood flow through the device for ultrafiltrate production.

Blood Pressure↗
PubMed1988

Urinary infection and malformations of urinary tract in infancy.

One hundred and forty infants with their first urinary tract infections were studied and pronounced differences in age and sex were found. Two thirds of the patients had their first urinary tract infection during the first three months of life, and boys were significantly younger. There was a predominance of boys from 1-3 months old, but of girls thereafter. Obstructive uropathies occurred more often in boys, and during the first two months of life. The incidence of vesicoureteric reflux was similar for both sexes. Malformations recognised after urinary tract infections were compared with urinary tract malformations recognised prenatally. Fetal urinary tracts were evaluated in just over half of all pregnancies during the study period. Obstructive uropathies and multicystic dysplastic kidneys were more often diagnosed prenatally, and most refluxes were diagnosed after the urinary tract infection. In conclusion age and sex differences are common in urinary tract infection, and even though many urinary tract malformations were diagnosed prenatally this did not influence the high incidence of malformations recognised after urinary tract infection in infancy.

Age Factors↗
PubMed1988

Suction-supported continuous arteriovenous hemofiltration in children.

Spontaneous continuous arteriovenous hemofiltration (CAVH) may fail to control azotemia in small patients with renal failure due to poor blood flow. To produce adequate ultrafiltrate a continuous negative pressure was added to the ultrafiltrate line. Two hemofilter systems of different membrane and surface area were used. Suction support approximately doubled ultrafiltration rate in both hemofilters. No side effects such as severe hypotonia or early hemofilter clotting occurred. Added suction can serve as a useful support of spontaneous CAVH in children with renal failure and too poor a blood flow to control azotemia.

Acute Kidney Injury↗
PubMed1988

Anticoagulation for continuous arteriovenous hemofiltration in children.

Continuous arteriovenous hemofiltration requires continuous anticoagulation to prevent early hemofilter clotting. We used heparin given continuously in the arterial line of the extracorporeal circuit as anticoagulant in children with initially normal coagulation status, and heparin and/or prostacyclin in high-risk bleeding patients with preexisting coagulopathy. Heparin infusion enabled a mean running time of 22.2 +/- 9.6 h, with the 0.1-m2 hemofilter and of 26.6 +/- 4.7 h with the 0.25-m2 hemofilter. The mean filter running time with combined heparin/prostacyclin infusion was 31 +/- 8.8 h. Prostacyclin as the sole antithrombotic agent provided good filter function only in 1 patient with preexisting coagulopathy. No adverse effects such as bleeding thrombosis, or hypotension were observed.

Anticoagulants↗
PubMed1988

[The initial presentation of renal tubular syndrome].

History, clinical presentation and first laboratory results were analyzed in three patients with cystinosis and in three patients with distal renal tubular acidosis. Growth failure, gastrointestinal tract disturbances, polydipsia, and polyuria were the most constant findings on history. Patients with distal renal tubular acidosis had a shorter period of clinical symptoms. All patients presented with dystrophy and dehydration. First laboratory investigation uniformly showed metabolic acidosis, high urinary-pH, and in spite of dehydration a low osmolarity of the first voided urine specimen. There were lower values of serum potassium and of urine osmolarity in cystinosis. In addition, metabolic acidosis was not so pronounced. Our study shows that history, clinical presentation and the results of a few simple laboratory investigations can indicate the presence of a renal tubular disorder even on admission of the patients.

Acidosis, Renal Tubular↗
PubMed1987

Takayasu's arteritis associated with crescentic glomerulonephritis.

We describe the initial course and followup of a 16-year-old white female patient who presented in 1983 with aortitis and rapidly progressive renal failure. Renal biopsy revealed crescentic glomerulonephritis. Segmental occlusions of the distal aorta and both common iliac arteries were demonstrated on aortography. Initially, her renal function and arterial occlusions markedly improved with pulse prednisolone therapy, but 34 months later, the glomerulonephritis and aortitis worsened. This case report, the first of its kind, documents both the association of Takayasu's arteritis with crescentic glomerulonephritis and a marked, though incomplete, response to pulse therapy.

Adolescent↗
PubMed1987

Continuous arteriovenous hemofiltration in critically ill children with acute renal failure.

Last year, five critically ill children with acute renal failure were treated by continuous arteriovenous hemofiltration. Mean treatment duration was 326 +/- 89 (SD)h, for a total of 1632 h. Mean ultrafiltration rates of 5.4 +/- 1.7 ml/min X m2 achieved mean serum urea levels of 150 +/- 25 mg/dl and a decline of mean prehemofiltration serum creatinine level of 3.5 +/- 3.6 to 2.9 +/- 2.0 mg/dl posthemofiltration. Continuous arteriovenous hemofiltration allowed adequate parenteral nutrition with a mean caloric intake of 79.6 +/- 9.2 kcal/kg X day. In the four surviving patients, urinary output started between 12 and 42 days after the onset of acute renal failure. Continuous arteriovenous hemofiltration is a very effective extracorporeal therapeutic system to control azotemia, fluid, and electrolyte balance in critically ill children with acute renal failure and hemodynamic instability.

Acute Kidney Injury↗