Beneficial effects of continuous overnight catheter drainage in children with polyuric renal failure.

OBJECTIVE: To determine the possible beneficial effect of providing decompression of the collecting system by continuous overnight catheter drainage (COCD) in children with progressive renal disease and dysfunctional bladder syndrome, commonly associated with polyuria which may overwhelm bladder capacity. PATIENTS AND METHODS: COCD was used in seven patients (four boys) with progressive polyuric kidney failure associated with dysfunctional bladders (current age 18.7 years, SD 5; age at COCD 12 years, SD 6). Five children had surgical bladder augmentation and all were prescribed daytime intermittent catheterization (IC) for a mean (SD) of 4.7 (3.4) years before COCD. All had significant polyuria, with a mean (SD) urine output of 2370 (971) mL/m2 per day. RESULTS: The mean (SD) glomerular filtration rate at the start of COCD was 48 (21) mL/min/1.73 m2, which is currently stable in the five patients continuing treatment. The mean (SD) duration of COCD was 4.9 (2) years. One patient showed no improvement and had a pre-emptive transplant within 1.2 years; another was transplanted after 5.5 years. Six patients showed evidence of benefit from COCD, with significant attenuation in the slope of renal functional decay (P = 0.02) and a mean (sd) prolongation of the predicted time to end-stage renal disease of 12.2 (5.6) years (P < 0.002). Hospitalization for febrile urinary tract infections was decreased from a mean (sd) of 1.7 (1.4) to 0.4 (0.7) times (P = 0.03) in the first year of COCD and eliminated by the second year (P < 0.01). CONCLUSION: COCD of the dysfunctional bladder in patients with progressive polyuric renal failure appears to offer the potential for preserving kidney function in selected patients. It does not replace surgical bladder augmentation or daytime IC in the core management.

Results of transvenous occlusion of secundum atrial septal defects with the fourth generation buttoned device: comparison with first, second and third generation devices. International Buttoned Device Trial Group.

OBJECTIVES: The purpose of this study was to assess safety and effectiveness of the fourth generation buttoned device in dosing atrial septal defects (ASDs) and to test the hypothesis that introduction of double button reduces unbuttoning rate without reducing effectiveness. BACKGROUND: Because of the high unbuttoning rate (7.2%) with first, second and third generation buttoned devices, the device was modified (fourth generation) so that there were two radiopaque spring buttons 4 mm apart on the button loop attached to the occluder. METHODS: During a four-year period ending in September 1997, 423 patients, ages 1.5 to 80 years (median 16 years), underwent closure of ASD at 40 medical centers around the world. RESULTS: The ASD size varied between 5 and 30 mm (median 17 mm). The device size varied between 25 and 60 mm. Unbuttoning occurred in 4 (0.9%) of 423 patients. Effective occlusion, defined as no (n = 343) or trivial (n = 34) residual shunt on echo-Doppler studies performed within 24 h of the procedure, was demonstrated in 377 patients (90%). Thus, the unbuttoning rate (0.9 vs. 7.2%) decreased (p<0.01) while effective occlusion rate (90 vs. 92%) remained unchanged (p>0.1) with this device, compared with earlier generation devices. During follow-up from one month to five years (23+/-15 months), 21 (5%) of 417 patients required surgical (n = 12) or transcatheter (n = 9) reintervention, mostly to treat significant residual shunt. In the remaining patients there was gradual reduction and disappearance of the residual shunt. No wire integrity problems were observed. CONCLUSIONS: These data suggest that the fourth generation buttoned device is as effective as earlier generation devices, but without significant unbuttoning. Follow-up results remained good, with a reintervention-free rate of 89% at five years.

ACE inhibition scintigraphy in the management of hypertension in children.

Angiotensin converting enzyme (ACE) inhibition scintirenography was performed to help establish the diagnosis and plan treatment of renovascular hypertension (RVH) in 57 hypertensive pediatric patients, 33 infants and 24 children older than 1 year. In 16 of 33 hypertensive infants, ACE inhibition scintirenography established the diagnosis of RVH from renal ischemia (due to aortic or renal arterial thrombi). Two scintigraphic criteria were used for the diagnosis of RVH: criterion I, ischemic and damaged kidney (a non-functioning kidney on or off ACE inhibition) and criterion II, ischemic but not damaged kidney (ACE inhibition induced deterioration of function of the kidney). When criterion I was present and the contralateral kidney was normal, ACE inhibitors could be used for treatment of hypertension without deterioration of renal function; kidneys satisfying criterion I eventually involuted or manifested growth arrest and frequently caused persistent RVH, even after resolution of the thrombus, requiring nephrectomy. When criterion II was present bilaterally, or it was associated with criterion I contralaterally, the use of antihypertensive drugs other than ACE inhibitors was necessary in order to prevent renal insufficiency or failure from ACE inhibitors. However, kidneys with criterion II showed normal growth and, following retraction or dissolution of the aortic thrombus, hypertension resolved. In 2 of 24 hypertensive children older than 1 year, the test was diagnostic of branch renal artery stenosis; RVH was cured by selective angioplasty. ACE inhibition scintirenography is useful in the evaluation and planning of treatment in children with hypertension and may predict the outcome of therapy and ultimate renal function.

Abnormal urinary acidification in infants with hydronephrosis.

Distal renal tubular abnormalities have been observed in patients with dilated urinary tract disorders. The present study was undertaken to look for patterns in urinary acidification in infants with varying degrees of hydronephrosis due to either reflux or obstruction and occurring as unilateral or bilateral disease. Three groups of infants (mean age 3.7+/-3.8 months) were studied prospectively. Groups IA and IB included patients with hydronephrosis who were acidotic and non-acidotic, respectively. Group II served as controls and consisted of patients with diarrhea and secondary metabolic acidosis with no known renal disease. Serum electrolytes, creatinine, and urine pH were measured in all patients. Urinary titratable acidity, ammonium (NH4), and net acid excretion (NAE) were measured by the titrimetric method. Infants with hydronephrosis demonstrated lower urinary buffering capacity, reflected in low NAE in the face of acidosis. Deficiencies were noted in both titratable acid and NH4 excretion compared with control infants. Acidosis was as common in unilateral as in bilateral disease, regardless of severity score. These data confirm a defect in distal urinary acidification in infants with hydronephrosis, whether unilateral or bilateral. Immaturity and endogenous acid load may play a significant role in the manifestation of metabolic acidosis with unilateral disease.

Role of inverted buttoned device in transcatheter occlusion of atrial septal defects or patent foramen ovale with right-to-left shunting associated with previously operated complex congenital cardiac anomalies.

Feasibility, safety, and effectiveness of transcatheter occlusion of atrial septal defect (ASD) with the buttoned device has been demonstrated. Despite its effectiveness in occluding secundum ASD with left-to-right shunt and patent foramina ovalia, presumably responsible for paradoxic embolism, it has limitations in effectively occluding ASD with evident right-to-left shunts. Therefore, the device was modified so that the square-shaped occluder component of the device is on the right atrial side, the inverted buttoned device. This study determines the feasibility, safety, and effectiveness of inverted buttoned device occlusion of atrial defects with right-to-left shunts. During a 17-month period ending August 1996, 12 patients, aged 1.6 to 39.0 years, underwent occlusion of residual ASD after repair of pulmonary atresia/stenosis with intact ventricular septum (n = 5), modified or fenestrated Fontan for tricuspid or pulmonary atresia (n = 5), and double-inlet left ventricle (n = 2). The systemic arterial oxygen saturation increased (p <0.001) from 82 +/- 7% (range 72% to 90%) to 94 +/- 3% (range 88% to 98%). There was no change in heart rate or cardiac index measured by Fick. Right atrial pressure increased by 1 to 4 mm in 5 of 12 patients (42%). There was trivial (n = 5) or no (n = 7) residual shunt by color Doppler study. Six- to 18-month (median 12) follow-up in all 12 patients revealed stable oxygen saturations (92 +/- 3%) by pulse oximetry. Color Doppler studies revealed small (n = 1), trivial (n = 1), or no (n = 10) residual shunt at follow-up. Based on these data, it is concluded that transcatheter occlusion of ASD with right-to-left shunts with inverted buttoned device is feasible in relieving arterial hypoxemia. Further clinical trials in a larger number of patients and careful evaluation of follow-up results are indicated to examine its safety and longer term effectiveness.

Usefulness of tilt table test with normal saline infusion in management of neurocardiac syncope in children.

Head-up tilt test has been useful in evaluating children with neurocardiac syncope. In this study patients with positive baseline and isoproterenol tests had repeat tilt testing done after normal saline infusion. If the symptoms persisted, the test was then repeated with phenylephrine infusion. Of the 101 patients studied, 58 (57%) had a positive tilt sign. Normal saline infusion was given to 53 patients. Three patients were excluded because of structural heart disease. Fifty patients (aged 14 +/- 4 years) comprised the study group. Forty-two (84%) of 50 patients had a negative repeat tilt sign after normal saline infusion, and these patients were treated with 0.5 to 1 gm of salt three times a day and/or fludrocortisone (20 patients). Of the 8 (16%) patients who did not respond to normal saline infusion, 5 had negative results when given phenylephrine and were treated with pseudoephedrine. Follow-up data on 42 patients (range 4 to 40 months, median 18 months) showed that all 35 patients who responded to normal saline were either asymptomatic or had improved. Two patients were successfully treated with pseudoephedrine; however, two patients in this group required pacemaker therapy. We conclude that (1) normal saline infusion mitigates the hemodynamic effects of neurocardiac syncope, (2) high-salt diet treatment in these patients was economical and effective, and (3) failure to respond to normal saline test may indicate a less favorable prognosis.

Influence of infection on patent ductus arteriosus and chronic lung disease in premature infants weighing 1000 grams or less.

OBJECTIVES: To test the hypotheses that (1) infection increases ductal dilatory prostaglandins and inflammatory mediators that may influence the closure of a patent ductus arteriosus (PDA), increasing the incidence of late episodes of PDA (after 7 days) and the rate of closure failures, and (2) the concurrence of PDA and infection increases the risk of chronic lung disease (CLD). METHODS: One hundred fourteen premature infants (birth weight, 500 to 1000 gm) were prospectively assessed for PDA and infection. Serum levels of 6-ketoprostaglandin F1 alpha and tumor necrosis factor alpha were measured routinely in all infants and when PDA or infection was present. Multivariate assessment of risk factors for PDA closure failure and for CLD was done by logistic regression, and expressed as an odds ratio and as 95% confidence intervals. RESULTS: Late PDA episodes were more frequent in infants with infection than in those without infection. A temporally related infection (<5 days between both diagnoses) was associated with an increased risk of PDA closure failure (odds ratio, 19.1 (confidence interval, 4 to 90)). In addition to birth weight and the severity of initial respiratory failure, PDA and infection increased the risk of CLD (odds ratio, 11.7 (confidence interval, 1.7 to 81) for PDA; odds ration, 3.1 (confidence interval, 1 to 11) for infection). Furthermore, when both factors were temporally related, they further increased the risk of CLD (odds ratio, 29.6 (confidence interval, 4.5 to >100)). Infants with infection and those with PDA had higher levels of 6-ketoprostaglandin F1 alpha than did control subjects. Levels of tumor necrosis factor alpha were also elevated in infants with infection and in those with late PDA. CONCLUSIONS: Infection adversely influences PDA outcome by increasing the risk of late ductal reopening and PDA closure failures. Increased levels of prostaglandins and tumor necrosis factor alpha in infants with infection may explain the poor PDA outcome. The concurrence of PDA and infection potentiates their negative effects on the risk of CLD.

Renal tubular abnormalities in infants with hydronephrosis.

PURPOSE: Abnormalities in renal tubular function have been observed in hydronephrotic urinary tract disease, resulting in metabolic acidosis, hyperkalemia and excessive free water diuresis. The frequency of these abnormalities, particularly in our infant population, was the impetus for our study. MATERIALS AND METHODS: We studied 50 infants selected from 199 patients followed for hydronephrosis before any surgical intervention during a 5-year period. Mean patient age was 1.5 +/- 1.0 months at the time of diagnosis by ultrasound, voiding cystourethrography and a radionuclide renal scan. Lesions were classified as unilateral or bilateral and graded according to severity of renal pelvic dilatation or grade of vesicoureteral reflux. RESULTS: At least 1 abnormality of tubular function was present in 29 patients (58%) of whom the predominant abnormality was renal tubular acidosis in 23 (79%, 46% of the total study group). Renal tubular acidosis was diagnosed on the basis of a serum total carbon dioxide of 19 mM./l. or less with urinary pH 5.5 or greater. The defect appeared to be distal in most cases. Other abnormalities included defects in urinary concentrating ability in 10 patients (4 with unilateral urinary tract dilatation). Distal tubular aldosterone resistance in 6 patients (3 with unilateral dilatation) was demonstrated by hyperkalemia with a low transtubular potassium gradient of 3 or less and low fractional excretion of potassium. Although common in unilateral lesions, renal tubular dysfunction became more prevalent with an increase in severity score and bilaterality. CONCLUSIONS: Renal tubular dysfunction is frequent in hydronephrotic infants with unilateral or bilateral disease. Although rarely life threatening and usually self-limiting, the metabolic consequences of these abnormalities require investigation to allow for appropriate medical management.

Displacement of preexisting thrombus by umbilical vein catheterization.

Balloon atrial septostomy is recommended as a standard palliative procedure in neonates with congenital heart defects who are dependent on intracardiac shunting. We describe an unusual and unreported complication associated with "bedside" balloon atrial septostomy. The umbilical venous catheterization resulted in displacement of a thrombus from the ductus venosus or the hepatic vein. We recommend careful two-dimensional echocardiographic monitoring during such procedures.

Ventricular arrhythmias in postoperative tetralogy of Fallot.

Ventricular arrhythmias in patients after total surgical repair of tetralogy of Fallot have been associated with late sudden death. In this large multicenter retrospective study of 359 patients with postoperative tetralogy of Fallot, spontaneous ventricular premature complexes (VPCs) on 24-hour ambulatory electrocardiographic monitoring and laboratory-induced ventricular tachycardia (VT) by electrophysiologic stimulation were analyzed. The mean age at surgical repair was 5 years and the mean follow-up duration after repair was 7 years. Spontaneous VPCs on ambulatory monitoring were found in 48% and induced VT on electrophysiologic stimulation was found in 17% of patients. Both spontaneous VPCs and induced VT were significantly related to delayed age at repair, longer follow-up interval, symptoms of syncope or presyncope and right ventricular systolic hypertension (greater than 60 mm Hg) (p less than 0.05), but not to right ventricular diastolic pressure greater than 8 mm Hg. The VPCs on ambulatory monitoring were more complex with increasing age at repair and follow-up duration. Induction of VT on electrophysiologic stimulation correlated with spontaneous VPCs including VT on 24-hour ambulatory electrocardiographic monitoring. The electrophysiologic stimulation protocol varied and the induction of VT increased with a more aggressive stimulation protocol. While induced sustained monomorphic VT was related to all forms of spontaneous VPCs, induced nonsustained polymorphic VT was related to more complex forms of VPCs on ambulatory monitoring. VT was not induced in asymptomatic patients who had normal 24-hour ambulatory electrocardiographic monitoring and normal right ventricular systolic pressure. (ABSTRACT TRUNCATED AT 250 WORDS)