The efficacy and safety of valganciclovir vs. oral ganciclovir in the prevention of symptomatic CMV infection in children after solid organ transplantation.

Routine prophylaxis for CMV with valganciclovir is common in adult recipients but data to support its use in children are scarce. The aim of this study was to compare the efficacy and safety of valganciclovir vs. ganciclovir in a pediatric cohort. We performed a retrospective analysis of 92 children after KTx and/or LTx. All children have received IV ganciclovir for two wk, and then oral ganciclovir (TID; n = 41) before 2004, or valganciclovir (OD; n = 51) thereafter. Treatment was given for three months in R+/D+ or R+/D- recipients and for six months in R-/D+. Patients were followed for one yr post transplant. Both groups were comparable in their demographic and transplant-related history. Symptomatic CMV infection/disease developed in 13.7% vs. 19.5% of valganciclovir and ganciclovir groups, respectively (P-NS). Time-to-onset of CMV infection was comparable in both groups (P-NS); rates of acute allograft rejection were similar in both groups (3.9% vs. 9.8%). Risk factors for CMV infection included young age, serostatus of R-/D+, and allograft from cadaver donor. No significant side effects were noted in both groups. As in adults, valganciclovir appears to be as efficacious and safe as oral ganciclovir. Valganciclovir should be considered as a possible prophylactic treatment for CMV in pediatric recipients of KTx or LTx.

Outcomes of prenatally diagnosed solitary functioning kidney during early life.

OBJECTIVE: To evaluate outcomes of congenital solitary functioning kidney (SFK) in early childhood. STUDY DESIGN: A retrospective study of 32 children diagnosed in utero with SFK owing to unilateral renal agenesis or multicystic dysplastic kidney and followed for 1 to 11.5 years. RESULTS: SFK length was in the compensatory hypertrophy range in 45% of fetal sonographic evaluations from mid-pregnancy, and in 85% on postnatal follow-up. Glomerular filtration rate was below normal range in 44.4%, 12.5% and 0% at <1 year, age 1 to 3 years and thereafter, respectively. Hyperfiltration was detected in 18.5% and 82.6% at <1 year and >3 years, respectively. Hypertension was documented in 35% at age 1 to 3 years but in none at an older age. Proteinuria was absent in all children. CONCLUSION: Congenital SFK is apparently associated with little or no renal damage in infancy or childhood. Compensatory enlargement of the functioning kidney begins in utero and might serve as a prognostic indicator for normal renal function after birth.

Growth hormone treatment increases circulating lipoprotein(a) in children with chronic renal failure.

Cardiovascular disease is the major cause of death in chronic renal failure (CRF) patients managed by dialysis or kidney transplantation. Whilst the use of human growth hormone (hGH) is of established benefit in CRF children particularly in those with short stature, in the present study we assessed in CRF children the effect of hGH treatment on circulating lipoprotein(a) [Lp(a)], a genetically determined cardiovascular risk factor. We studied 15 CRF children treated by dialysis or conventional therapy and after kidney transplantation. Overnight fasting blood samples were collected immediately before and after 6 months hGH treatment. In all but one of the children there was a significant increase in serum Lp(a) over the 6 month treatment period -(+)66.7% over the basal levels (range 14 to 180%). After the hGH treatment, in six children Lp(a) levels were elevated to above 300 mg/l, the cut-off level for increased coronary artery disease (CAD) risk. Concomitantly/children also had an increase in serum levels of IGF-I (+96.4%) and insulin (+85.8%). All children had an accelerated growth velocity during the treatment; there was no effect on serum creatinine. Our study shows that hGH treatment in CRF children, though beneficial in its growth promoting effects, increases the already characteristically high levels of serum Lp(a), a risk factor for CAD, and that serum Lp(a) monitoring during treatment with hGH may be useful in evaluating future cardiovascular risk.

Genu valgum in children who received renal transplant.

In 19 children who received renal transplants, 6 developed genu valgum one to two years after the transplantation. Except for one patient, biochemical blood and urine parameters were normal and bone radiograph showed no signs of renal osteodystrophy during this period. Two patients had bilateral varus supracondylar osteotomy, and in one patient medial hemistapling of the distal femur was performed.

Fetal hyperechogenic kidney with normal amniotic fluid volume: a diagnostic dilemma.

OBJECTIVE: To determine the prognostic value of sonographically detected fetal hyperechogenic kidneys with normal amniotic fluid volume. METHODS: Seven cases of hyperechogenic fetal kidneys were identified by sonography over a 7-year period (1996--2002). Increased renal echogenicity was diagnosed when the renal parenchyma was of greater echogenicity than adjacent liver tissue. Amniotic fluid volume was measured by the semiquantitative sonographic technique known as the amniotic fluid index (AFI). RESULTS: Three of the live-born infants had autosomal dominant polycystic kidney disease and one had autosomal recessive polycystic kidney. In the remainder, autopsy study revealed multifocal renal dysplasia in two cases and normal kidneys in one. CONCLUSIONS: Increased renal echogenicity with normal amniotic fluid volume in a fetus without other anomalies is a difficult diagnostic dilemma. Although it is usually indicative of renal parenchymal disease with possible renal failure after birth or in early childhood, in some cases, it represents a normal variant. .

Parameters of evaluation and correlation of renal phosphate handling and of parathyroid function in children.

The aim of the study was to establish normal values for parameters of parathyroid (PT) function in childhood and to examine whether a correlation could be found between these parameters and tubular phosphate (P) reabsorption. Values for PTH (carboxyl terminal) and urinary cAMP (UcAMP) throughout childhood were found to be significantly lower than in adults, with mean values of 0.3 ng/ml (30 pmol/l) and 1.96 nmol/dl glomerular filtrate (GF) respectively. A significant correlation was found between UcAMP and tubular P reabsorption (TP), expressed by the equation: TP = 5.5-(0.5 x UcAMP) mg/dl GF (= 1.78-(0.16 x UcAMP) mmol/dl GF, with a scatter of 10.6 mg/dl around the mean (+/- 1 SD). These data appear to permit the evaluation of primary tubular components in patients with defects of renal P handling, as opposed to those related to PT activity.

Calcium-deficiency rickets in a four-year-old boy with milk allergy.

A 4-year-old boy was found to have rickets associated with normal serum levels of 25-hydroxyvitamin D and high serum levels of 1,25-dihydroxyvitamin D. These findings were thought to be the result of dietary calcium deficiency caused by the prolonged elimination from his diet of cow milk and milk products because of allergy. Adequate intake of calcium resulted in rapid improvement.

Hypercalciuria as a cause of persistent or recurrent haematuria.

Of 91 children with idiopathic persistent or recurrent haematuria, eight also had hypercalciuria. This was transient in three and persistent in five. Persistent hypercalciuria was a rare cause of microscopic haematuria (two of 80), but common in association with gross haematuria (three of 11).

Maxillomandibular brown tumor--a rare complication of chronic renal failure.

We report a 17-year-old hemodialysis patient with a rapidly growing maxillary mass diagnosed as a brown tumor. Although successful control of the parathyroid hormone (PTH) levels was achieved by treatment with vitamin D pulse therapy, the lesion progressed, invaded the maxillary sinus, and caused severe eating and speech disabilities. No recurrence was observed following surgical excision. The differential diagnosis and considerations regarding the causes of the disease in a child, therapy options, and review of the literature are presented.

Low serum C3, leukopenia, and thrombocytopenia: unusual features of henoch-schonlein purpura.

Henoch-Schonlein purpura (HSP) affects predominantly the skin, joints, gastrointestinal tract and kidney. Although the pathogenesis is probably of immune origin and complement activation is thought to play a role, laboratory findings including the serum level of the complement components are usually normal. We present a patient with a severe form of HSP nephritis who had unusual laboratory findings of a low level of C3, mild leukopenia and thrombocytopenia. These findings may further support the importance of complement activation in the pathogenesis of HSP.

Inferior vena cava diameter: a useful method for estimation of fluid status in children on haemodialysis.

BACKGROUND: An accurate assessment of fluid status in haemodialysis patients presents a significant challenge especially in growing children. Clinical parameters of hydration are not always reliable, and invasive methods such as measurement of central venous pressure cannot be used routinely. We evaluated the usefulness of inferior vena cava diameter (IVCD) measured by echocardiography in the estimation of hydration in children on haemodialysis. METHODS: Fifteen haemodialysis patients (mean age 14 years) were evaluated. Clinical assessment included patients' symptoms, weight, blood pressure, heart rate, presence of oedema and vascular congestion, before and after dialysis session. Dry weight was assessed based on the above parameters. Fifty-two echocardiographic studies immediately prior and 30-60 min following dialysis were performed. The anteroposterior IVCD was measured 1.5 cm below the diaphragm in the hepatic segment in supine position during normal inspiration and expiration. IVCD was expressed per body surface area. RESULTS: Following haemodialysis mean IVCD (average of expiration and inspiration) decreased from 1.12+/-0.38 to 0.75+/-0.26 cm/m(2) (P<0.0001). Changes in IVCD were significantly correlated with alterations in body weight following dialysis (P<0.0001). The collapse index (per cent of change in IVCD in expiration vs inspiration) increased significantly after dialysis (P=0.035). IVCD clearly reflected alterations in fluid status. It did not vary significantly with changes in dry weight in a given patient. CONCLUSIONS: Our findings support the applicability of VCD measurement in the estimation of hydration status in paediatric haemodialysis patients. The combination of clinical parameters and measurement of IVCD may enable more accurate evaluation of hydration of children on haemodialysis.