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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 12 (1997), S. 595-598 
    ISSN: 1437-9813
    Keywords: Key word Pheochromocytoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fourteen children (10 boys and 4 girls, aged 8 to 17 years) had 20 pheochromocytomas treated over a 36-year period from 1959 to 1995 inclusive. Nine patients had 11 tumors before 1980; 5 children had 9 tumors up to 1987. There were no new children with pheochromocytomas at our hospital from 1988 to 1995. Hypertension, sweating, headache, and visual blurring were the most common symptoms and signs (average 5 months). The most reliable biochemical investigations were the urinary catecholamines and norepinephrine. Before 1980, intravenous pyelography and angiography were most successful in localizing the tumor, but since then ultrasonography and computerized tomography have been the radiological investigations of choice. Early involvement of the anesthesiologist in the preoperative control of the hypertension is essential; blood pressure (BP) control was achieved with phenoxybenzamine. The main anesthetic drugs used were: sodium thiopental, fentanyl, methoxyflurane, isoflurane, nitrous oxide, and metocurine. Sixteen tumors were adrenal and 4 were extra-adrenal (1 intrathoracic and 1 extradural). All except 2 tumors were completely resected; they ranged in size from 1.3 to 14 cm. Ligation of the tumor's venous drainage was usually associated with a sudden, temporary fall in systemic BP. There were 2 children with malignant tumors. Four patients had five recurrences (second pheochromocytoma) within 6 years, and all were heralded by a return of their original symptoms and signs. One girl was left with no adrenal tissue. The only complication was in a boy with a large, partly-resected malignant right adrenal tumor who had a subphrenic abscess drained and was left with a temporary bile fistula, cirrhosis, and chronic pain. All children were normotensive when discharged from hospital and remain alive and well with a follow-up of 7 to 36 years. There were no deaths. Long-term follow-up is essential.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 12 (1997), S. 595-598 
    ISSN: 1437-9813
    Keywords: Pheochromocytoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fourteen children (10 boys and 4 girls, aged 8 to 17 years) had 20 pheochromocytomas treated over a 36-year period from 1959 to 1995 inclusive. Nine patients had 11 tumors before 1980; 5 children had 9 tumors up to 1987. There were no new children with pheochromocytomas at our hospital from 1988 to 1995. Hypertension, sweating, headache, and visual blurring were the most common symptoms and signs (average 5 months). The most reliable biochemical investigations were the urinary catecholamines and norepinephrine. Before 1980, intravenous pyelography and angiography were most successful in localizing the tumor, but since then ultrasonography and computerized tomography have been the radiological investigations of choice. Early involvement of the anesthesiologist in the preoperative control of the hypertension is essential; blood pressure (BP) control was achieved with phenoxybenzamine. The main anesthetic drugs used were: sodium thiopental, fentanyl, methoxyflurane, isoflurane, nitrous oxide, and metocurine. Sixteen tumors were adrenal and 4 were extra-adrenal (1 intrathoracic and 1 extradural). All except 2 tumors were completely resected; they ranged in size from 1.3 to 14 cm. Ligation of the tumor's venous drainage was usually associated with a sudden, temporary fall in systemic BP. There were 2 children with malignant tumors. Four patients had five recurrences (second pheochromocytoma) within 6 years, and all were heralded by a return of their original symptoms and signs. One girl was left with no adrenal tissue. The only complication was in a boy with a large, partly-resected malignant right adrenal tumor who had a subphrenic abscess drained and was left with a temporary bile fistula, cirrhosis, and chronic pain. All children were normotensive when discharged from hospital and remain alive and well with a follow-up of 7 to 36 years. There were no deaths. Long-term follow-up is essential.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-198X
    Keywords: Key words Membranoproliferative glomerulonephritis ; Cutaneous telangiectasias ; Sparse hair ; Atrial septal defect ; Ectodermal dysplasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We report the unusual association of normocomplementemic type I membranoproliferative glomerulonephritis in a 10-year-old girl with sparse red hair, absent eyebrows and eyelashes, cutaneous telangiectasias, and an atrial septal defect.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 4 (1990), S. 152-155 
    ISSN: 1432-198X
    Keywords: Hypercoagulability ; Coagulation ; Peritoneal dialysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To determine whether children treated with chronic peritoneal dialysis have a hypercoagulable state, various coagulation and fibrinolytic factor concentrations or activities were measured in 17 children undergoing chronic peritoneal dialysis. The patients had significantly increased activities of factors VII and VIII and increased concentrations of von Willebrand factor (vWF), fibrinogen, factor XIIIA and factor XIIIS compared to reference values (P〈0.001 in each case). The activated partial thromboplastin time was prolonged (P〈0.001) and the thrombin clotting time was decreased (P〈0.05) in these children. The prothrombin time and activities of factors XII, XI, IX, X, V and II were not significantly different from control values. Protein C concentrations were similar to normal, but antithrombin III concentrations were increased (P〈0.05). Within the fibrinolytic pathway, decreased concentrations of plasminogen were found (P〈0.001) and the concentrations of alpha-2-antiplasmin were increased (P〈0.001). The plasma albumin concentration was below 33 g/l in 13 of the 17 children. The duration of treatment with peritoneal dialysis was directly correlated with vWF concentrations (P〈0.001) and inversely correlated with factor VII concentrations (P〈0.01). Of these patients 2 have since had clinical thrombotic episodes. The coagulation abnormalities found may have a role in the occurrence of thrombosis complicating renal transplantation.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-198X
    Keywords: Key words: Renal tubular acidosis ; Osteopetrosis ; Carbonic anhydrase II deficiency ; Bicarbonate reabsorption ; Ammonium excretion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Renal tubular acidosis with osteopetrosis is an autosomal recessive disorder due to deficiency of carbonic anhydrase II (CAII). A 3.5-year-old Egyptian boy with osteopetrosis and cerebral calcification had a persistent normal anion gap type of metabolic acidosis (plasma pH 7.26) and a mild degree of hypokalemia. A baseline urine pH was 7.0; ammonium (NH4 +) excretion was low at 11 μmol/min per 1.73 m2; fractional excretion of bicarbonate HCO3 (FEHCO3) was high at 9%, when plasma HCO3 was 20 mmol/l; citrate excretion rate was high for the degree of acidosis at 0.35 mmol/mmol creatinine. Intravenous administration of sodium bicarbonate led to a urine pH of 7.6, a FEHCO3 of 14%, a urine-blood PCO2 difference of 7 mmHg, NH4 + excretion fell to close to nil, and citrate excretion remained at 0.38 mmol/mmol creatinine. Intravenous administration of arginine hydrochloride caused the urine pH to fall to 5.8, the FEHCO3 to fall to 0, the NH4 + excretion rate to rise to 43 μmol/min per 1.73 m2, and citrate excretion to fall to 〈0.01 mmol/mmol creatinine. These results show that our patient had a low rate of NH4 + excretion, a low urine minus blood PCO2 difference in alkaline urine, and a low urinary citrate excretion, but only when he was severely acidotic. He failed to achieve a maximally low urine pH. These findings indicate that his renal acidification mechanisms were impaired in both the proximal and distal tubule, the result of his CAII deficiency.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 6 (1992), S. 33-37 
    ISSN: 1432-198X
    Keywords: Reflux nephropathy ; Renal scarring ; Captopril test ; Angiotensin converting enzyme inhibitors ; Hypertension ; Blood pressure ; Plasma renin activity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We studied the plasma renin activity (PRA) and blood pressure (BP) responses to a single oral dose of captopril in children with renal scarring due to vesicoureteral reflux. The test was performed on 19 children, of whom 13 were normotensive and 6 hypertensive. Basal PRA was within the normal range for age for all except 1 patient. Mean basal PRA values for normotensive and hypertensive patients were not significantly different. At 90 min post captopril, the mean PRA increase was 3.61 ng/l per second (SD=7.07) in the normotensive group but only 0.77 ng/l per second (SD=0.83) in the hypertensive group. In 11 patients the average systolic BP 60–90 min post captopril was 0.9%–12.4% lower than the basal value. Diastolic BP decreased after captopril administration in 10 patients and increased in 9. The changes in mean arterial pressure and PRA between 0 and 90 min post captopril were inversely correlated (r=0.605,P〈0.01). Because of the PRA responsiveness in the normotensive patients with renal scarring, we suggest that there was activation of the renin-angiotensin axis in this group. However, it is not apparent whether such patients are more at risk of developing hypertension at a later time.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 8 (1994), S. 492-493 
    ISSN: 1432-198X
    Keywords: Cyclosporin ; Ketoconazole ; Renal transplant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Ketoconazole is known to inhibit the metabolism of cyclosporin through inhibition of cytochrome P-450. This pharmacological interaction was used in an 8-year-old renal transplant patient to successfully achieve therapeutic cyclosporin blood concentrations. The addition of ketoconazole to the cyclosporin regimen should be considered when difficulties are encountered in attaining satisfactory cyclosporin levels.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of clinical pharmacology 50 (1996), S. 147-148 
    ISSN: 1432-1041
    Keywords: Key words Felodipine ; Children; pharmacokinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Type of Medium: Electronic Resource
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