Chiung-Hui Chen

Response to supplementary vaccination with recombinant or plasma hepatitis B vaccine in healthy non-responding children.

Fifty-three children who failed to respond to four doses of plasma hepatitis B (HB) vaccine (anti-HBs titre < 10 IU l-1) were divided into two groups and received revaccination with either three doses of recombinant HB vaccine (10 micrograms/dose, by 0, 1, 6 month schedule; group A) or two additional doses of plasma HB vaccine (5 micrograms/dose, by 0, 1 month schedule; group B) respectively. Thirty-two vaccinees in group A had a response rate (with anti-HBs > 10 IU l-1) of 53.1% (17/32), 87.5% (28/32), and 100% (32/32) after first, second and third doses of the vaccine respectively. Twenty-one vaccinees in group B had a response rate of 61.9% (13/21) after two additional doses of plasma vaccine. High anti-HBs titres (> 1000 IU l-1) were noted in 50% of the vaccinees in group A after three doses of vaccine. Comparing anti-HBs response between group A and group B after two additional doses of HB vaccine, group A had a higher anti-HBs titre (geometric mean titre 104.7 IU l-1 versus 75.9 IU l-1) along with a better seroconversion rate (87.5 versus 61.9%). However, the differences in vaccine dose between the two groups may also be a contributory factor. Our findings indicate that three doses of recombinant HB vaccine were invariably effective in eliciting a good immune response in previous non-responders to the four doses of plasma HB vaccine. Therefore, it is speculated that these young vaccinees who did not respond to four doses of plasma HB vaccine may not be real non-responders, but hyporesponders.

Hypovolemia and hypovolemic shock in children with nephrotic syndrome.

Hypovolemic shock is not an uncommon presentation in nephrotic syndrome, yet it is seldom mentioned in the literature. This study was performed to investigate the prevalence of hypovolemia and hypovolemic shock in the acute nephrotic stage, and the association of hemoconcentration and abdominal pain with hypovolemic status. Two hundred and twenty-five patients with a total of 328 admissions to the pediatric ward of our hospital during 1983 to 1996 were retrospectively reviewed for hypovolemic episodes. Clinical presentation and laboratory data including hemoglobin, serum sodium, albumin, cholesterol, and triglyceride levels were investigated. Thirteen patients with 19 episodes (5.8%) of hypovolemic shock were found, and had more severe hemoconcentration (hemoglobin 19.6 +/- 2.2 g/dL) and hyponatremia (127.3 +/- 7.2 mEq/L). Another 33 patients with 41 symptomatic hypovolemic episodes without hypotension (12.5%) were found, and their hemoglobin levels were higher compared to patients without hypovolemic symptoms. Among 61 episodes of abdominal pain and hemoconcentration, 58 were responsive to albumin infusion. This suggested the presence of hypovolemia. Twenty patients had abdominal pain without hemoconcentration, and 18 of them had primary peritonitis. Hypovolemia was found in patients at the acute nephrotic stage, and was usually associated with hemoconcentration and abdominal pain. A combined examination of hemoglobin and serum sodium is the best indicator of hypovolemic status. Both primary peritonitis and hypovolemic episodes should be taken into consideration when managing abdominal pain in children with nephrotic syndrome.

Decreased Urinary Epidermal Growth Factor in Children with Acute Renal Failure: Epidermal Growth Factor/Creatinine Ratio Not a Reliable Parameter for Urinary Epidermal Growth Factor Excretion

To verify some animal experimental results in humans, we have studied urinary epidermal growth factor (EGF) excretion in normal children as well as children with acute renal failure (ARF). Urinary EGF excretion was expressed as a ratio of urinary EGF to urinary creatinine concentration (EGF/Cr) for random and 24-h urine, and a daily total urinary EGF for 24-h urine. The highest urinary EGF/Cr in children was found at 1 mo to 3 y of age. There was a highly significant correlation between random urine EGF/Cr and 24-h urine EGF/Cr (r = 0.92, p < 0.001), whereas no correlation of urinary EGF/Cr with daily total urine EGF was found. During the course of ARF, a decline in urinary EGF/Cr from the period before peak serum creatinine to the period after the declination of serum creatinine was noted (p = 0.013, n = 13, by repeated measure analysis), with a constant low daily total urine EGF (p value not significant). However, a rise in both urinary EGF/Cr and daily total urine EGF was found between the period of serum creatinine decline and the period of completely normal serum creatinine(p < 0.001). Serum EGF remained unchanged throughout the course of ARF. These results suggest 1) the possible role of EGF in renal growth or maturation during the first 2 or 3 y of life, 2) the possible renal origin of human urinary EGF, and 3) decreased urinary EGF excretion in children with ARF. In particular, EGF/Cr is not a reliable indicator for the expression of actual urinary EGF excretion in ARF. Instead of urinary EGF/Cr, urinary EGF concentration may be used to predict the daily total urinary EGF excretion during ARF. These results provide the pattern of urinary EGF excretion during ARF in children and may be of help for further clinical studies.

Urinary Epidermal Growth Factor Excretion in Children with Chronic Renal Failure

To investigate the excretion of urinary epidermal growth factor (EGF) in children with chronic renal failure (CRF), we have measured the urinary EGF/creatinine ratio (EGF/Cr) and the 24-hour urine EGF concentration in 19 children with CRF, 11 children with kidney disease and normal creatinine clearance, and 12 healthy children. Children with CRF had a significantly lower daily urine EGF concentration as well as urinary EGF/Cr. In contrast, children with kidney disease and normal renal function had normal daily urine EGF levels and urinary EGF/Cr. Accompanied by no difference in serum EGF between these two groups of patients, these data provide indirect evidence of the kidney as a source of human urinary EGF. There was a positive correlation of urinary EGF/Cr with creatinine clearance in all renal patients (r = 0.608, n = 30, p < 0.001). A much better correlation was found between daily urine EGF and creatinine clearance (r = 0.855, n = 30, p < 0.001). Our results implicate that there is a functional relationship between glomerular filtration and urinary EGF excretion, and that the urinary EGF/Cr may be a reliable indicator of urinary EGF excretion in children with CRF.

Nephromegaly and elevated hepatocyte growth factor in children with biliary atresia

Nephromegaly, a rarely mentioned but probably common situation, was studied in children with biliary atresia. We evaluated the length and the cross-sectional diameters of the kidney by ultrasound in 21 children with biliary atresia as well as in 50 healthy children. The ages ranged from 1 month to 10 years. Plasma hepatocyte growth factor (HGF) was measured in 18 children with biliary atresia and also in 18 age- and sex-matched normal controls. There was a significant nephromegaly (increase in the renal length and the kidney volume) in children with biliary atresia as compared with normal children (P < 0.001 by analysis of covariance). Plasma HGF levels were elevated in these patients (2.13 +/- 1.06 v 0.76 +/- 0.19 ng/mL in controls, P < 0.001) and had a positive correlation with the renal size after considering the effect of body height by multiple regression analysis (P = 0.0022 for renal length, and P < 0.001 for kidney volume). These results confirm the presence of large kidneys in biliary atresia and implicate the possible pathogenic role of HGF in such a condition. Nephromegaly in biliary atresia may provide a new in vivo model to study the mechanism of renal growth.

Renal calcification in very low birth weight infants.

Between January 1990 and December 1991, serial real-time ultrasound examinations and analyses of urine were performed on a total of 50 infants with birth weights less than 1,500 g to assess the incidence of renal calcification. Five infants (10%) developed renal calcification at a mean age of 48.8 +/- 14.1 days. These 5 infants with renal calcification had significantly shorter gestations (28.2 +/- 0.8 vs. 30.1 +/- 1.7 weeks, p < 0.0005) and lower birth weights (934 +/- 45 vs. 1,311 +/- 188 g, p < 0.0005) when compared with infants without renal calcification. None of the affected infants were treated with furosemide. Affected infants had a mean urine volume of 85.8 +/- 11.3 ml/kg/24 h, mean urine calcium level of 5.07 +/- 1.18 mg/kg/24 h, mean urine calcium to creatinine (mg/mg) ratio of 0.67 +/- 0.09, and a mean urine N-acetyl-beta-D-glucosaminidase (NAG) to creatinine (U/g) ratio of 259 +/- 133. Urinalyses showed that affected infants had significantly higher urine pH values and hematuria. Alkaline phosphatase concentrations and initial parathyroid hormone levels were not different among the two groups. In summary, renal calcification occurred in 10% of very low birth weight infants and multiple risk factors seem to be contributory. The smaller, sicker and more immature infants appear to have increased risk for developing renal calcification.(ABSTRACT TRUNCATED AT 250 WORDS)

Autosomal Dominant Polycystic Kidney Disease: An Unusual Presentation as Unilateral Renal Mass in the Infant

The onset of autosomal dominant polycystic kidney disease in infants and children is unusual, and renal involvement is typically bilateral. The presentation of a unilateral renal mass in such a disorder is extremely rare. We report a 2-month-old infant with autosomal dominant polycystic kidney disease presenting with unilateral renal involvement; the literature concerning this entity is reviewed.

Renal Function in Neonatal Hyperbilirubinemia

A total of 75 jaundiced infants with gestational ages ranging from 37 to 42 weeks were studied during the first 10 days of age to evaluate renal function by measuring endogenous creatinine clearance (Ccr), fractional excretion of N-acetyl-beta-D-glucosaminidase (NAG) to creatinine, fractional excretion of sodium (FENa) and urine osmolality. All jaundiced infants were divided into two groups. Group 1 infants (n = 35) had total serum bilirubin levels ranging between 21 and 39.6 mg/dl (mean 27.2). Exchange transfusions were performed in all group 1 infants at the time of the initial study. Group 2 infants (n = 40), whose total serum bilirubin levels ranged between 12.3 and 20 mg/dl (mean 16.4), received phototherapy, except for 2. Conjugated bilirubin levels were less than 1.0 mg/dl in all these infants. Results were compared with 25 untreated control infants with corresponding gestational and postnatal ages. Follow-up studies were done in 27 of the 35 group 1 infants and in 32 of the 40 group 2 infants prior to hospital discharge, when total serum bilirubin levels had decreased to less than 10 mg/dl. Ccr, fractional excretion of NAG to creatinine, FENa and urine osmolality of group 1 infants were statistically significantly different when compared to those of group 2 and the control infants. The measured parameters in the post-treatment follow-up study of group 1 infants returned to near-normal levels when total serum bilirubin levels became normal. However, no significant differences were seen between group 2 and the control infants in any of the measured parameters.(ABSTRACT TRUNCATED AT 250 WORDS)

Correlation between Urinary Epidermal Growth Factor Excretion and Serum Thyroid Hormone in Premature and Term Neonates

Eighty neonates, including 14 full-term, 31 premature, 27 twin or triplet, 6 small-for-gestational-age, and 2 infants with hyperthyroidism, were evaluated. The urinary epidermal growth factor/creatinine ratio (EGF/Cr) on the 1st postnatal day was not statistically different among full-term, premature, multiple-pregnancy, and small-for-gestational-age infants (F = 1.06, p = 0.6). There was no difference in urinary EGF/Cr between the 1st postnatal day and the 7th day (p = 0.4 by paired t test). The urinary EGF/Cr was not correlated with the serum thyroid-stimulating hormone level (r = –0.162, n = 60, p = 0.21), but showed a positive correlation with serum total T3 (r = 0.526, n = 60, p < 0.001) and with serum total T4 (r = 0.460, n = 60, p < 0.001). The correlation between urinary EGF/Cr and serum free T4 was even much better (r = 0.727, n = 25, p < 0.001). These results implicate that thyroid hormone may play a role in regulating urinary EGF excretion.

Symptomatic Hypomagnesemia in Children

Between January 1996 and June 1998, 24 children with symptomatic hypomagnesemia were enrolled for analysis of their symptomatology, causes, and treatment outcome. Hypocalcemia and hyperphosphatemia suggesting impaired parathyroid function were the most common electrolyte disorders. Hypokalemia was also frequently noted. The related symptoms including seizure, tetany, and weakness were common. Drug-induced renal magnesium wasting was the most common cause of symptomatic hypomagnesemia, and tended to occur in older children using aminoglycoside, furosemide, and amphotericin-B. The associated gastrointestinal causes might add a minor contribution to the development of hypomagnesemia. Analyses of PTH levels in 13 children suggested that inhibition of PTH synthesis or secretion was responsible for hypomagnesemic hypocalcemia in most patients. However, peripheral PTH resistance might also account for the mechanism in a few patients. In most patients, symptomatic hypomagnesemia was transient, and improved after magnesium provision. Only one child with congenital renal magnesium wasting and two with primary hypomagnesemia needed long-term magnesium treatment.