Renal bicarbonate reabsorption and hydrogen ion excretion in normal infants.

After acute administration of ammonium chloride, infants 1 to 16 months of age were similar to older children in their capacity to acidify their urine. The infants had a higher rate of excretion of titratable acid and a lower rate of excretion of ammonium but were similar in their rate of excretion of total hydrogen ion.Bicarbonate titrations performed in infants during the first year of life demonstrated a threshold ranging from 21.5 to 22.5 mmoles per L, maximal rate of reabsorption from 2.6 to 2.9 mmoles per 100 ml glomerular filtrate, and marked titration splay. A nephronic frequency distribution curve of the ratio of glomerular filtration rate to tubular reabsorptive capacity demonstrated both heterogeneity and skewing to the right, suggesting the presence of significant numbers of nephrons with low tubular transport capacity relative to filtration rate.It is suggested that the "physiologic acidosis" of the infant is due neither to a limited renal capacity to excrete hydrogen ion nor to a reduced capacity for reabsorption of bicarbonate, but rather to a low renal plasma bicarbonate threshold. Although the level of the threshold may relate to the kinetics of bicarbonate reabsorption during this period, it appears to be due at least in part to functional and morphologic heterogeneity of nephrons.

Minoxidil-induced bullous eruption.

A previously unreported side effect--serosanguineous bullae--was found in one patient receiving minoxidil. The eruption disappeared with cessation of the drug and reappeared on rechallenge. The question arises whether continuation of minoxidil treatment could have led to vasculitis.

Clinical use of captopril in Takayasu's disease.

Three patients with Takayasu's disease (TD), initially observed with hypertension and/or congestive heart failure, were treated with captopril, an orally active inhibitor of converting enzyme. As arteritis in this disease is diffuse and extensive, surgical correction of renovascular hypertension is often delayed or impossible. Our experience enables us to recommend captopril for conservative treatment in TD.

Metabolic responses to the administration of angiotensin II, K and ACTH in two salt-wasting syndromes.

Metabolic responses to the administration of Angiotensin II, K and ACTH are described in two salt-wasting syndromes: hypoaldosteronism in Jews from Iran, which is characterized by an enzymic block in the conversion of 18-hydroxycorticosterone to aldosteron; and pseudohypoaldosteronism, a disorder in which aldosterone secretion is high in association with renal tubular unresponsiveness to mineralocorticoids. The response of plasma and urinary aldosterone to K and ACTH is qualitatively normal in hypoaldosteronism; however, infusion of Angiotensin II, in a dose that was pressor and elevated aldosterone levels threefold in control subjects, was only pressor in hypoaldosteronism. In pseudohypoaldosteronism, plasma and urinary aldosterone respond to Angiotensin II, K and ACTH, notwithstanding very high basal hormonal levels.

The conservative management of vesicoureteric reflux: a review of 121 children.

A group of 121 children with vesicoureteric reflux (VUR) grades 1 to 3 managed conservatively were followed-up for a period of six to ten years. In the majority of patients VUR grades 1 and 2 disappeared spontaneously. Prognosis was less favorable in those who were seen initially with grade 3 VUR. A statistically significant higher incidence of reflux disappearance was observed in children who were seen before 4 years of age. In the majority of patients with paraureteric-vesicle diverticulum, reflux persisted. Reflux was more likely to disappear in children with lower incidence of urinary tract infection than in those with multiple infections.

Steady state serum digoxin concentration in relation to digitalis toxicity in neonates and infants.

Steady state serum digoxin concentrations were determined in 34 neonates and infants receiving standard maintenance doses of the drug. Digitalis intoxication, diagnosed by ECG criteria, occurred in four of 13 patients with a serum concentration above 2 ng/ml and not in any of 21 subjects with a serum digoxin concentration below this level. This association was found to be significant. It seems that the concept of increased tolerance to digoxin hitherto ascribed to infants is not tenable and that the monitoring of serum digoxin concentration is essential to treatment in this age group.

Progressive spastic paraparesis, vitiligo, premature graying, and distinct facial appearance: a new genetic syndrome in 3 sibs.

We describe progressive spastic paraparesis of the lower limbs in the presence of generalized vitiligo, premature graying of body hair, and distinct facial appearance in 3 sibs whose parents are first cousins. The condition is considered an autosomal recessive trait. The pathogenesis of this neurocutaneous disorder is unknown.

Variables affecting outcome from severe brain injury in children.

This study evaluates the outcome of 56 severely brain injured children (mean age 6.2 +/- 2.1 years) and relates the Initial Glasgow Coma Scale (IGCS), initial intracranial pressure (ICP int), maximal intracranial pressure (ICP max) and minimal cerebral perfusion pressure (CPP min) to quality of survival. Forty-one children sustained head trauma, five severe central nervous system infections and 10 were of miscellaneous etiology. Therapy consisted of mechanical hyperventilation, moderate fluid restriction, dexamethasone and diagnosis specific measures when indicated. Outcome was categorized according to the Glasgow outcome scale at discharge from the hospital. An IGCS of 3 was associated with 100% mortality, 7 and above resulted in 72% good recovery, 28% poor outcome and no mortality. ICP int of less than 20 torr was noted in (67%) of the patients, and did not correlate with ICP max or outcome. Conversely, ICP int in excess of 40 torr correlated well with ICP max and outcome. ICP max of less than 20 torr resulted in 57% good recovery, 36% poor outcome and 7% mortality. ICP max greater than 40 torr resulted in 7% poor outcome and 93% mortality (p less than 0.001). In head trauma, 32 patients (78%) were alive with mean ICP max 16.9 +/- 3.1 and CPP min 65.5 +/- 8.5 torr compared to 9 patients (22%) who died with mean ICP max 53.7 +/- 10.8 and CPP min 6 +/- 3.9 torr, (p less than 0.01). In children with infectious etiology 60% survived with mean ICP max 16 +/- 3 and CPP min 96 +/- 16 torr.(ABSTRACT TRUNCATED AT 250 WORDS)

Vesicoureteral reflux in boys with hypospadias.

Hypospadias in a congenital anomaly which in most cases has to be corrected surgically. We include micturition cystourethrography (MCU) in the preoperative workup of all these children as well as three months postoperatively. Meatal stenosis and müllerian duct remnants are known to occur in this condition, but vesicoureteral reflux (VUR) was an unexpected finding in many boys who were asymptomatic and had no evidence of urinary tract infection. Fifty-eight of 305 hypospadiacs, examined by MCU, proved to have reflux, most of them grade II. In 37, VUR was found before surgery and in the remaining 21 after surgical correction, many of them with meatal stenosis. In 10 patients reflux appeared in the postoperative period where there had been none in the preoperative study; 8 of the 10 had a postoperative stricture of the distal urethra, and VUR subsided in 4 after adequate dilatation. We conclude that vesicoureteral reflux is not uncommonly found in patients with hypospadias, even in those who are completely asymptomatic. We believe this is an additional incentive to include an MCU in the pre- and postoperative evaluation of patients with this anomaly.

Minoxidil in the treatment of refractory hypertension.

Minoxidil, a powerful vasodilator, is a very effective antihypertensive drug. It was used to treat 22 patients, most of whom were refractory to conventional antihypertensive medication. There was a swift and definite drop of blood pressure in all cases. In 7 patients with renal disease, renal function did not deteriorate during the administration of Minoxidil, and it improved dramatically in the eighth patient. Side effects of the drug were hirsutism, fluid retention, and in 1 patients a pruritic bullous eruption which disappeared when the drug was discontinued.

Minoxidil in the treatment of refractory hypertension.

Minoxidil, a powerful vasodilator, is a very effective antihypertensive drug. Twenty-two patients, who were mostly refractory to conventional anti-hypertensive medication, were treated with this drug. There was a swift and definite drop of blood pressure in all cases. In 7 patients with renal disease, renal function did not deteriorate during the administration of minoxidil, and it improved dramatically in the eighth patient. Side effects of the drug were hirsutism, fluid retention, and in 1 patient a pruritic bullous erruption which disappeared when the drug was discontinued.

Anuria in childhood due to bilateral urolithiasis. A report of four cases.

Four children with anuria due to occlusion of both ureters by calculi are presented. A plain film of the abdomen revealed faintly radiopaque urinary calculi bilaterally in three patients. In the fourth case, one calculus was visualized in the right ureter, but further contralateral exploration showed an obstructing ureteral calculus in the left ureter as well. It is concluded that in children with acute anuria, the possibility of bilateral ureteral obstruction due to calculi should be the physician's first consideration.