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Biomedical subjects

G Zobel

Publications and source records attributed to G Zobel.

At least 73 records · Page 4Linked to original sources

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↗

[A clinical score system for children with ARDS].

In a retrospective study we developed a simple acute respiratory distress syndrome (ARDS) scoring system in order to analyze the severity of ARDS as precisely as possible. From March 1983 to May 1990, 17 children with ARDS were admitted and treated at the pediatric intensive care unit of the University Hospital of Graz. The ARDS score was evaluated as a predictor of outcome. The score is based on four variables: mean airway pressure, oxygenation index, additional number of organ system failure and a radiological score, each of which was assigned a score between 0 and 3 (Table 3). The score was recorded on admission or immediately after respiratory failure and after 24, 48 and 72 h. The patients were divided into survivors and non-survivors. After 24 h of mechanical ventilation the ARDS score was 7.16 +/- 0.79 in survivors and 10.4 +/- 0.4 points in non-survivors (P less than 0.0006). Similar differences were found after 48 and 72 h of therapy. In addition, the predictive power of the ARDS score after 24 h was tested at a level of 8 points. The sensitivity and the positive predictive value were 90%, while specificity and negative predictive value were 85.7%. The correct prediction was 88.2%. We conclude that this simple ARDS score can be an useful prognostic factor in patients with ARDS.

Carbon Dioxide↗

Continuous extracorporeal fluid removal in children with low cardiac output after cardiac operations.

Eleven hypervolemic and oliguric children with low cardiac output after cardiac operations were treated by slow continuous ultrafiltration or continuous arteriovenous hemofiltration. A mean negative fluid balance of 1.63 +/- 0.37 ml/kg/hr (standard error of the mean [SEM]) significantly improved the hemodynamic status within 59 +/- 6.1 hours (SEM). Although the central venous pressure decreased significantly from 15.2 +/- 0.84 to 8.8 +/- 0.92 mm Hg (p less than 0.0001), the mean arterial pressure increased significantly from 41.5 +/- 2.54 to 53.5 +/- 2.21 mm Hg (p less than 0.001). In addition, pH increased significantly from 7.31 +/- 0.01 (SEM) to 7.43 +/- 0.001 (SEM) (p less than 0.001) and oxygenation index (arterial oxygen tension/inspired oxygen fraction) from 119 +/- 15.2 (SEM) to 214 +/- 27.0 (SEM) (p less than 0.001). Hemodynamic improvement during slow continuous extracorporeal fluid removal allowed a significant decrease of the catecholamine infusion rate. After normovolemia had been achieved, continuous arteriovenous hemofiltration had to be continued in four children because of persistent anuria. Eight patients could be weaned from artificial ventilation and vasopressor support. Two patients died without recovery of renal function and one with restored renal function. Slow continuous ultrafiltration and continuous arteriovenous hemofiltration improve the cardiovascular function in children with low cardiac output by optimizing the preload conditions of the failing heart. In addition, they improve acid-base balance and pulmonary gas exchange.

Blood Pressure↗

[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↗

[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↗

Ceftriaxone monotherapy for bacterial meningitis in children.

A total of 33 patients with bacterial meningitis were treated with single daily doses of ceftriaxone (CTR 100 mg/kg/day i.v.) for a median duration of 13 days. Pathogens isolated by culture and/or determined by latex agglutination were 15 Haemophilus influenzae b, 7 Neisseria meningitidis, 2 Streptococcus pneumoniae, 1 group B streptococcus, 2 Streptococcus viridans and 2 Staphylococcus epidermidis. In 4 cases a diagnosis of purulent meningitis could only be made by means of the inflammatory liquor parameters. All cerebrospinal fluid (CSF) drug levels even at the end of the dosing interval were at least 10-fold higher than the MICs of the respective bacterial isolates. The average penetration of CTR into the CSF was 6.6%. Within 12-46 h after the first dose, control spinal taps were performed. Cultures were sterile in all cases. Side effects encountered were diarrhea, exanthema, neutropenia and transient elevation of glutamic oxaloacetic transaminase, but none caused a change of therapy. One patient developed a biliary concrement. No patient died; 5 patients had prolonged fever (greater than 5 days), and 2 were left with persistent hearing deficiencies. CTR can be recommended as a safe and effective antibiotic agent for once daily treatment of bacterial meningitis in children.

Adolescent↗

Evaluation of clinical scoring systems in critically ill infants and children.

Four scoring systems, the Acute Physiologic Score for Children (APSC), the Physiologic Stability Index (PSI), the Paediatric Risk of Mortality (PRISM) and the Therapeutic Intervention Scoring System (TISS), were evaluated for 103 critically ill infants and children according to the Clinical Classification System (CCS) class IV. The admission scores were higher for children who died than those who lived (APSC, PSI, PRISM p less than 0.001, TISS p <0.025). In addition, the mean APSC and PSI showed significant differences (p less than 0.01) between survivors (S) and nonsurvivors (NS) in all patients, mean PRISM showed significant differences (p less than 0.01) between S and NS in all but renal failure patients and the mean TISS showed only significant differences (p less than 0.01) between S and NS with primary cardiovascular and respiratory diseases. The mortality rate was 30%. Using the 0.5 predicted risk rate, total correct prediction of admission APSC, PSI, and PRISM was 80%, 80.5% and 80% respectively. Receiver Operating Characteristic (ROC) curves drawn for each severity index were in a discriminating position. There were no significant differences between the areas under the ROC curves of the physiological scores. However, there was a significant difference between the physiologic scores and TISS (p less than 0.001). Admission APSC, PSI and PRISM excellently describe severity of illness and give prognostic information in critically ill paediatric patients. In addition, TISS gives information about the therapeutic support needed.

Child, Preschool↗

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↗

Extracorporeal CO2 removal in a lung lavage induced respiratory distress syndrome.

Ten pigs with experimental respiratory distress syndrome were treated by extracorporeal CO2 removal (ECCO2-R) combined with low frequency positive pressure ventilation (LPPV). After lung damage had been induced by repeated lung lavages a PEEP trial was conducted in order to find the appropriate PEEP for the damaged lungs. This PEEP was then applied during the ECCO2-R/LPPV period. Blood gas values improved significantly on extracorporeal bypass within a short time (pre-bypass paO2: 54.2 +/- 3.7 vs 168.5 +/- 31.6 mmHg after 15 min on bypass, p less than 0.001) and were kept constant during the next 4 hours. Minute ventilation (MV) was reduced from 4.01 +/- 0.31 to 0.74 +/- 0.07 l/min (p less than 0.0001), FiO2 of the ventilator from 1.0 to 0.46 +/- 0.08 (p less than 0.0001) whereas FiO2 of the membrane lung (ML) was not changed significantly (FIO2ML 0.59 +/- 0.07 vs 0.53 +/- 0.06). During controlled mechanical ventilation (CMV), comparable adequate gas exchange was only achieved at a significantly higher mean airway pressure (Paw 14.1 +/- 0.08 vs 21.2 +/- 0.47 cmH20, p less than 0.0001). Hemodynamic variables did not change significantly during bypass time. ECCO2-R/LPPV driven by a simple renal perfusion system allows adequate gas exchange in experimental respiratory failure.

Animals↗

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↗

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↗

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↗

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↗

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↗