Kinesin family member 12-related hepatopathy: A generally indolent disorder with elevated gamma-glutamyl-transferase activity.

Exome sequencing (ES) has identified biallelic kinesin family member 12 (KIF12) mutations as underlying neonatal cholestatic liver disease. We collected information on onset and progression of this entity. Among consecutively referred pediatric patients at our centers, diagnostic ES identified 4 patients with novel, biallelic KIF12 variants using the human GRCh38 reference sequence, as KIF12 remains incompletely annotated in the older reference sequence GRCh37. A review of these and of 21 reported patients with KIF12 variants found that presentation with elevated serum transaminase activity in the context of trivial respiratory infection, without clinical features of liver disease, was more common (n = 18) than manifest cholestatic disease progressing rapidly to liver transplantation (LT; n = 7). Onset of liver disease was at age <1 year in 15 patients; LT was more common in this group. Serum gamma-glutamyl transpeptidase activity (GGT) was elevated in all patients, and total bilirubin was elevated in 15 patients. Liver fibrosis or cirrhosis was present in 14 of 18 patients who were biopsied. The 16 different pathogenic variants and 11 different KIF12 genotypes found were not correlated with age of onset or progression to LT. Identification of biallelic pathogenic KIF12 variants distinguishes KIF12-related disease from other entities with elevated GGT.

Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis-Case Compilation and Review of the Literature.

(1) Background: Progressive familial intrahepatic cholestasis (PFIC) is a rare cause of liver failure. Surgical biliary diversion (SBD) and ileal bile salt inhibitors (IBAT) can delay or prevent liver transplantation (LTX). A comparison of the two methodologies in the literature is lacking. The combination has not been investigated. (2) Methods: We performed a literature survey on medical and surgical treatments for PFIC and reviewed the charts of our patients with PFIC of a tertiary hospital. The end points of our analysis were a decrease in serum bile acid (sBA) levels, reduction of pruritus and delay or avoidance of (LTX). (3) Results: We included 17 case series on SBD with more than 5 patients and a total of 536 patients. External or internal SBD, either conventional or minimally invasive, can reduce pruritus and sBA, but not all PFIC types are suitable for SBD. Six publications described the use of two types of IBAT in PFIC with a total of 118 patients. Treatment response was dependent on genetic type and subtype. Patients with PFIC 2 (nt-BSEP) showed the best response to treatment. Four out of eleven PFIC patients underwent SBD at our centre, with two currently receiving IBAT. (4) Conclusions: Limited data on IBAT in selected patients with PFIC show safety and effectiveness, although surgical methods should still be considered as a successful bridging procedure. Further studies to evaluate a possible combination of IBAT and SBD in PFIC are warranted and treatment decision should be discussed in an interdisciplinary board.

Golimumab in adolescents with Crohn's disease refractory to previous tumour necrosis factor antibody.

AIM: Anti-tumour necrosis factor (TNF)-α drugs are effective treatments for the management of moderate/severe Crohn's disease (CD), but treatment failure is common. In the treatment of paediatric CD, there are no data about the use of a third introduced subcutaneous TNF antibody golimumab. METHODS: We evaluated the efficacy of golimumab for adolescents with moderate/severe CD. Retrospective analyses were done in all 7 (5 girls) adolescents who received golimumab at a median age of 17 years for a median of 7.2 months. Paediatric Crohn's disease activity index (PCDAI), full blood count, inflammatory markers, use of corticosteroids and adverse events were recorded. RESULTS: With golimumab, 5 of the 7 children were PCDAI responders and 2 entered remission (PCDAI <10). Faecal calprotectin was significantly reduced after 4 weeks compared to baseline. Out of five children, steroid withdrawal was possible in one and steroid reduction in two cases. There were no serious side effects. CONCLUSION: In moderate/severe CD, golimumab induced clinical remission with PCDAI response. Golimumab may be an effective rescue therapy in refractory CD.

Vedolizumab use after failure of TNF-α antagonists in children and adolescents with inflammatory bowel disease.

BACKGROUND: Vedolizumab is safe and effective in adult patients with Crohn's disease (CD) and ulcerative colitis (UC); however, data in children with inflammatory bowel disease (IBD) are scarce. Therefore, we evaluated vedolizumab use in a cohort of Austrian paediatric patients with IBD. METHODS: Twelve patients (7 female; 7 CD; 5 UC), aged 8-17 years (median, 15 years), with severe IBD who received vedolizumab after tumour necrosis factor α antagonist treatment were retrospectively analysed. Clinical activity scores, relevant laboratory parameters, and auxological measures were obtained at infusion visits. RESULTS: In the CD group, 1/7 patient discontinued therapy due to a severe systemic allergic reaction; 1/7 and 2/7 patients achieved complete and partial response, respectively, at week 14; and 3/7 patients discontinued therapy due to a primary non-response or loss of response. In the UC group, complete clinical remission was achieved at weeks 2, 6, and 14 in 2/5, 1/5 and 1/5 patients respectively; partial response was observed in one patient at week 2. CD activity scores did not significantly change from baseline to week 38 (median 47.5 vs. 40 points, p = 1,0), while median UC activity scores changed from 70 to 5 points (p < 0,001). Substantial weight gain and increased albumin and haemoglobin levels were observed in both groups. CONCLUSION: These results demonstrate that vedolizumab can be an effective treatment for individual paediatric patients with IBD who are unresponsive, intolerant, or experience a loss of efficacy in other therapies. However, vedolizumab appears to be more effective in paediatric UC than in paediatric CD.

Reduced risk of pulmonary emboli in children treated with long-term parenteral nutrition.

BACKGROUND & AIMS: Pulmonary embolism (PE) is a complication of parenteral nutrition (PN) with a prevalence of 35% in children. In 2003 new intravenous lipid emulsions (ILEs) with MCT, olive and/or fish oil in addition to soybean oil were introduced. The aim was to compare the incidence of PE before and after introduction. METHODS: 327 surveillance ventilation-perfusion (V/Q) scintigraphies from 68 children aged 0.3-15 years, treated with PN from 1993 to 2010, were retrospectively reviewed. Rate of PE/1000 central venous catheter (CVC) days, number of children with PE pre- and post-introduction of ILEs were compared. Multivariate analyses were performed for risk factors. RESULTS: Twenty-two (32%) children (19/42 before 2003 and 3/26 after 2003, p = 0.007) had at least one episode of PE. Thirty seven (11%) episodes of PE were detected accounting for a mean of 0.2/1000 CVC days prior to 2003 and 0.05/1000 CVC days after 2003, p = 0.04. Regression analysis indicated that higher content of ILE/infusion (p = 0.045) and frequency of ILE of >3 nights/week were associated with more PE (p = 0.001). New ILEs were associated with lower risk (p = 0.003). CONCLUSION: With a four-fold fall in incidence with new ILE, PE remains a complication. We recommend 12-18 monthly surveillance with lung perfusion scan and anticoagulants if PE is diagnosed.

Histopathological features of gastrointestinal mucosal biopsies in children with juvenile idiopathic arthritis.

BACKGROUND: The association between inflammatory bowel disease and joint involvement is well established. There is a paucity of data describing histopathological features of the gut in relation to juvenile idiopathic arthritis (JIA). METHODS: We retrospectively identified 33 (21 male) children aged 3-16 y with JIA (11 with oligoarthritis, 5 with polyarthritis, 8 with systemic onset arthritis, 8 with enthesitis-related arthritis (ERA), and 1 with psoriatic arthritis) with significant gastrointestinal (GI) symptoms who underwent upper and/or lower endoscopy. The histopathological findings were reviewed in addition to presence of autoantibodies and concomitant treatment. RESULTS: The most common GI indications for endoscopy were persistent abdominal pain (14/33 (42%)) and diarrhea (10/33 (30%)). Of the 33 children, 28 (85%) had gut mucosal inflammation, mostly affecting the colon (80%). Active inflammation of the gut was found in 5 of 28 (17%) children, and 15 of 28 (53%) children showed mild nonspecific inflammation. Eight patients (27%) had predominantly an eosinophilic infiltrate. Twenty-six patients had previously received treatment for JIA. There was a negative association with the use of immunomodulators and the presence of eosinophil inflammation. CONCLUSION: The majority of children with JIA and GI symptoms have histological evidence of mild nonspecific inflammation, but some having active colitis and prominent eosinophil infiltrate.

Growth and bone health in paediatric patients with Crohn's disease receiving subcutaneous tumor necrosis factor antibody.

AIM: To study whether adalimumab (ADA) was associated with improvement in growth, bone mineral density (BMD) and bone metabolism. METHODS: In children with Crohn's disease (CD) there is a high prevalence of growth failure and reduced BMD. Treatment with infliximab is associated with an improvement in growth. Anthropometry, paediatric CD activity index (PCDAI), bone markers and BMD was measured in 18 patients (72% females) one year before and after start of ADA with a median age of 14.4 years (range: 5-19 years) at treatment start. Outcomes were indicators of growth with treatment as well as interval growth. RESULTS: Eleven (61%) children experienced catch-up growth after ADA. PCDAI significantly decreased from 52.1 ± 16 to 30.4 ± 23 (P ≤ 0.001). Post ADA, body mass index (BMI) standard deviation score (SDS) 0.1[range: 2.7-(-0.8)] vs -1 [range: 0.1-(-3.6)], P = 0.04 and ∆BMI SDS in children 0.3 [range: 0.7-(-0.2)] vs -1.1 [range: 1.2-(-2.3)], P = 0.01 in remission were significantly higher compared to those with moderate to severe inflammation. The main predictors for growth were 25-hydroxycholecalciferol and for bone mineralisation weight and height SDS. ADA had no significant influence on bone markers and BMD. CONCLUSION: Next to improvement of PCDAI, half of the children achieved a positive catch-up growth. A better nutritional status with improvement in BMI and weight is positive predictor for improved growth and bone mineralisation.

Overexpression of Hsp70 confers cytoprotection during gliadin exposure in Caco-2 cells.

BACKGROUND: In Celiac disease (CD), cytoskeletal integrity of intestinal cells is disrupted by gliadin exposure. This study investigates the role of heat shock protein (Hsp)70 during cytoskeletal recovery in CD by assessing its induction and effects on junctional proteins. METHODS: Using an in-vitro model of CD, cytoskeletal injury and recovery was assessed in gliadin-exposed Caco-2 cells by measuring cellular distribution of ezrin, E-cadherin, and Hsp70 by differential centrifugation. Effects of Hsp70 were tested by an in-vitro repair assay, based on the incubation of injured or recovered cytoskeletal cellular fractions in noncytoskeletal supernatants containing low or high levels of Hsp70, or by transient transfection of Caco-2 cells with Hsp70. RESULTS: Cytoskeletal disruption of ezrin and E-cadherin was demonstrated in gliadin-exposed Caco-2 cells by their significant shift from the cytoskeletal pellet into the noncytoskeletal supernatant fraction. Recovery from gliadin exposure was associated with induction and cytoskeletal redistribution of Hsp70. The in-vitro repair assay delineated direct evidence for HSP-mediated repair by stabilization of junctional proteins by Hsp70. Overexpression of Hsp70 resulted in significantly increased cytoskeletal integrity. CONCLUSION: Our results establish an essential role of HSP-mediated cytoskeletal repair in Caco-2 cells during recovery from in-vitro gliadin exposure.

Nutritional status in children with Shwachman-diamond syndrome.

OBJECTIVE: In Shwachman-Diamond syndrome (SDS), pancreatic insufficiency can lead to malabsorption of fat-soluble vitamins and trace elements. The aim of this study was to assess the serum concentrations of vitamins A and E, zinc, copper, and selenium and their deficiencies. METHODS: This retrospective review was performed in 21 children (12 were male; median age, 7.8 years) with genetically confirmed SDS at a tertiary pediatric hospital. Pancreatic enzyme replacement therapy (PERT) and vitamin or trace elements supplements were documented. RESULTS: Twenty patients (95%) had pancreatic insufficiency receiving PERT, 10 (47%) had a combined vitamin and trace element deficiency, 6 (29%) had an isolated vitamin deficiency, and 4 (19%) had an isolated trace element deficiency. Vitamins A and E deficiency occurred in 16 (76%) and 4 (19%) of 21, respectively. Low serum selenium was found in 10 (47%), zinc deficiency in 7 (33%), and copper deficiency in 5 (24%). Eleven patients (52%) were on multivitamin supplementation, and 2 (10%) on zinc and selenium supplements. No statistical differences were found between repeated measurements for all micronutrients. CONCLUSIONS: More than 50% of the children had vitamin A and selenium deficiencies despite adequate supplementation of PERT and supplements. Micronutrients should be routinely measured in SDS patients to prevent significant complications.

Prevalence of Gallstones Compared in Children With Different Intravenous Lipids.

OBJECTIVE: The aetiology of biliary liver disease in children with intestinal failure treated with long-term parenteral nutrition (PN) is multifactorial. Risks include the lipid component of PN. The aim of the study was to compare prevalence and outcome of gallstones with different types of intravenous lipids. METHODS: Liver and biliary tract imaging and relevant clinical details were reviewed in 71 patients (37, 52% boys) treated with PN for >3 months. Types of lipid infused were compared with regard to hepatobiliary abnormalities. RESULTS: In total 369 abdominal ultrasounds were performed in 71 patients of age between 3 months and 17 years. Underlying diagnoses were short bowel syndrome in 20 (28%), small intestinal enteropathy in 34 (48%), and motility disorder in 17 (24%). A total of 67 (94%) children had 362/369 scans on lipid-containing PN. Of the total, 15 (21%) patients had gallstones, 8 (11%) had sludge, and both were detected in 7 (10%) children. The gallstones/sludge resolved in 7 patients (10%) and persisted in 10 (13%). In 6 patients, sludge progressed to form discrete gallstones, and in 9 children, gallstones led to biliary duct dilatation. Four (6%) patients underwent cholecystectomy. Fewer children had abnormalities with the newer mixed lipid emulsion (P = 0.005). There was a higher prevalence of sludge (P = 0.01) on pure soya lipid. Predictors for sludge were young age at PN (P = 0.001), lack of enteral feed (P < 0.001), and motility disorder with stoma (P = 0.002). CONCLUSIONS: Hepatobiliary pathology is common in children on PN. The use of mixed lipid was associated with less biliary complications and should be the first choice of treatment in children.

Reduction of blood stream infections in children following a change to chlorhexidine disinfection of parenteral nutrition catheter connectors.

BACKGROUND & AIMS: Catheter-related-blood-stream-infection (CRBSI) might be prevented by optimal catheter connector antisepsis in children with intestinal failure on parenteral nutrition (PN). We changed the disinfectant used from isopropanol 70% to chlorhexidine 2% in 70% isopropanol, which leaves a residue of chlorhexidine on the connector. METHODS: We conducted this before/after study in children treated with PN for >28 days. Episodes of CRBSI were recorded for all 42 children treated for >28 days during May-November 2006 before introducing chlorhexidine and for all 50 children treated in May-November 2007 after chlorhexidine was introduced. The number of hospital-acquired CRBSI and number of PN days was counted for each period. The rate of CRBSI/1000 catheter days and the proportion of patients that experienced at least one CRBSI during the two periods were compared. RESULTS: There were 3.1 CRBSI/1000 catheter days prior to using chlorhexidine and 0.4 CRBSI/1000 catheter days after it was introduced, p = 0.03. Prior to chlorhexidine 10/42 (24%) patients experienced at least one episode of CRBSI, compared to 3/50, (6%) after introducing it (p = 0.02). The survival rate in both periods was similar, but after chlorhexidine significantly more children made a full recovery and a lower proportion of children had irreversible intestinal failure (p = 0.01). CONCLUSIONS: Our results support the use of 2% chlorhexidine not only to reduce risk of sepsis for central venous catheter connector antisepsis in catheters used for intravenous nutrition, but also to improve the patients' chances of recovering intestinal function.

Paediatric patients with inflammatory bowel disease who received infliximab experienced improved growth and bone health.

AIM: Children with inflammatory bowel disease (IBD) have a high prevalence of growth retardation and low bone mineral density (BMD). This retrospective study investigated whether the start of infliximab treatment (IFX) was associated with improvement of growth and bone health. METHODS: Anthropometry, BMD and bone markers were measured 1 year before and after the start of IFX treatment in 33 patients (51% males), with a median age of 13.5 years at baseline. Outcomes were growth with treatment and indicators of improved bone health. RESULTS: Twenty-one children (64%) experienced a positive catch-up growth after IFX. Height standard deviation scores (SDS) were significantly higher in children in remission. Treatment with IFX was associated with a statistically significant increase in 25-hydroxycholecalciferol (25-OHD, p = 0.01). IFX had no influence on BMD. Children with low BMD < -2 had significantly higher inflammation scores, lower body mass index, weight, height SDS and 25-ODH after IFX. CONCLUSION: After treatment with IFX, children with IBD improved significantly in weight, with the majority achieving positive catch-up growth. Bone mass tended to remain static with time of treatment with IFX, despite a significant increase in 25-OHD. Improved nutritional status positively predicts improved bone mineralisation.

Body composition in paediatric intestinal failure patients receiving long-term parenteral nutrition.

OBJECTIVE: Outcome of children with intestinal failure (IF) has improved on treatment with parenteral nutrition (PN). The effects of PN and IF on body composition (BC) are unknown. The aim was to review BC in PN-treated children and those weaned off and to compare with reference data. DESIGN: Children on long-term/home PN underwent measurement of regional fat mass (FM) and lean mass (LM) using dual energy X-ray absorptiometry. Underlying diseases were intestinal enteropathy, n=15, short bowel syndrome (SBS), n=8 and intestinal dysmotility, n=11. PN duration was median 10 years. Fat Mass Index (FMI) and Lean Mass Index (LMI) were compared in children with and without intestinal inflammation, steroid treatment and according to PN dependency. RESULTS: 34 children aged 5-20 years were studied. They were short, mean height SD score (SDS) -1.8 (p<0.001) and light (mean weight SDS -0.86, p<0.001) with high body mass index (BMI) SDS: mean 0.4 (p=0.04) and low Limb LMI SDS -0.9 (p<0.001). Children with SBS had low FMI SDS -0.8 (p=0.01). BC did not significantly differ between diagnostic groups or with steroid treatment. Patients with intestinal inflammation (n=20) had higher BMI SDS than those without, p=0.007. Totally, PN-dependent children, n=11 had higher BMI SDS, p=0.004, total body FMI SDS, p=0.008 and trunk FMI SDS, p=0.001 compared with patients partially dependent and off PN. CONCLUSIONS: Significantly low limb LM was seen in all patient groups with high FM in children on total PN. Children with IF requiring PN treatment >27 days may benefit from BC monitoring and PN adjustment according to results in order to maximise linear growth and health in later life.

Growth and bone health in pediatric intestinal failure patients receiving long-term parenteral nutrition.

BACKGROUND: Children with chronic intestinal failure (IF) treated with long-term parenteral nutrition (PN) may present with low bone mineral density (BMD). The cause may reflect small body size or suboptimal bone mineralization. OBJECTIVE: We assessed growth and bone health in children with severe IF. DESIGN: Height, weight, and fracture history were recorded. The lumbar spine bone mass was measured in 45 consecutive patients (24 male subjects) aged 5-17 y receiving PN for a median of 5 y. BMD and bone mineral apparent density (BMAD) [ie, adjusted-for-height SD scores (SDSs)] were calculated. RESULTS: Diagnoses were short bowel syndrome in 12 patients (27%), intestinal enteropathy in 20 patients (44%), and motility disorder in 13 patients (29%). Mean (±SD) weight, height, and body mass index SDSs were -0.8 ± 1.3, -1.80 ± 1.5, and 0.4 ± 1.3, respectively. The height SDS was less than -2 in 23 children (50%). Patients with enteropathy or intestinal mucosal inflammation (associated with dysmotility or short bowel) were significantly shorter than patients without enteropathy (P = 0.007). The BMD SDS was -1.7 ± 1.6, and the BMAD SDS was -1.4 ± 1.5, independent of primary diagnosis or mucosal inflammation. Nineteen patients (42%) had low BMD (SDS less than -2.0), and 14 patients (31%) had low BMAD. In 25 patients studied at 1-2-y intervals, the BMD SDS fell significantly with time, whereas BMAD declined less, which suggested that a poor bone mineral accretion reflected poor growth. A total of 11 of 37 patients (24%) had nonpathologic fractures (P = 0.3 compared with the general population). CONCLUSIONS: Approximately 50% of children were short, and one-third of children had low BMD and BMAD. Children with enteropathy or intestinal mucosal inflammation are at greatest risk of growth failure. Close nutritional monitoring and bespoke PN should maximize the potential for growth and bone mass.

Intestinal failure-associated liver disease in surgical infants requiring long-term parenteral nutrition.

PURPOSE: Our aim was to determine incidence, severity, and outcome, as well as predisposing factors and underlying diagnoses, of intestinal failure-associated liver disease (IFALD) in surgical infants requiring long-term parenteral nutrition (PN). METHODS: We retrospectively studied surgical infants receiving PN for at least 28 days for congenital or acquired intestinal anomalies over a 5-year period (January 2006 to December 2010). Intestinal failure-associated liver disease was defined as type 1 (early)--persistent elevation of alkaline phosphatase for 6 weeks or longer; type 2 (established)--additional elevated total bilirubin (≥ 50 µmol/L); and type 3 (late)--additional clinical signs of end-stage liver disease. RESULTS: Eighty-seven infants required PN for at least 28 days. Intestinal failure-associated liver disease occurred in 29 infants (33%). Intestinal failure-associated liver disease was managed medically in all but 2 patients who underwent intestinal elongation. None were referred for intestinal or liver transplant. Intestinal failure-associated liver disease has been reversed in 17 (59%) of cases to date. Sixty-one children receiving long-term PN (70%) have achieved enteral autonomy, whereas 12 (14%) require home PN. Severity of IFALD was significantly associated with duration of PN and female sex. CONCLUSION: Intestinal failure-associated liver disease remains a fairly common but rarely life-threatening complication of intestinal failure in surgical infants. Intestinal failure-associated liver disease can be reversed in more than half of these children, and enteral autonomy was achieved in more than two thirds, even with minimal use of intestinal elongation. This is the first study to demonstrate an association between the severity of IFALD in surgical infants and female sex.

Intestinal failure-associated liver disease in hospitalised children.

OBJECTIVE AND AIM: Liver disease is a potentially life-threatening complication of intravenous/parenteral nutrition (PN). Our aim was to determine the incidence, aetiology and outcome of intestinal failure-associated liver disease (IFALD) in hospitalised children treated with long-term PN (>27 days). METHODS: Over 4 years all long-term intestinal failure (IF) patients were reviewed for the possible predisposing factors of age, diagnosis, PN lipid, sepsis, length of PN treatment and length of hospitalisation. Outcome measures were IFALD incidence, severity and prognosis. RESULTS: Of 60/279 (22%) children aged 0-18 years who developed IFALD, 13 (5%) progressed to type 3/end stage disease. IFALD was associated with younger age (p=0.03), longer treatment (p<0.001), longer hospitalisation (p=0.01), surgical diagnosis (p=0.005) and prematurity (p=0.03). IFALD was not associated with sepsis. Intestinal surgery was associated with IFALD independently of age (p=0.03). Survival was 86%, with three deaths attributed to IFALD (1% of all cases), all of which were surgical. CONCLUSION: IFALD incidence was lower than previously reported in paediatric patients, with surgical neonates at greatest risk.

Feasibility of a finger prick-based self-testing kit in first- and second-degree relatives of children with coeliac disease.

AIM: To assess feasibility of a finger prick-based kit as method for self-testing of first and second-degree relatives of coeliac disease (CD) patients. METHODS: A total number of 379 subjects were invited to participate in this study, consisting of 197 first-degree and 182 second-degree relatives of CD patients. The self-testing kit (Biocard™) was sent out with included instructions for use. Completed tests were sent back to the study coordinator for assessment. RESULTS: One hundred and ninety-six invited relatives carried out the Biocard™ test at home. Amongst these, 70% were children. In 97% of the cases the test was performed correctly. Three tests revealed a positive result, all of which were later confirmed by serology and histology as coeliac disease. CONCLUSION: Our study indicates that Biocard™ test is a reliable, easy to use and well-accepted tool for home testing of first- and second-degree relatives of CD patients.

Changes of blood pressure and left ventricular mass in pediatric renal transplantation.

Cardiovascular events are among the most frequent causes for long-term morbidity and mortality in children after renal transplantation. The aim of this study was to analyze the effects of post-transplant changes in arterial hypertension, as assessed by 24-h ambulatory blood pressure measurement (ABPM), on myocardial architecture, as assessed by echocardiography. In a retrospective chart review analysis, 39 children were identified in whom 24-h ABPM and echocardiography had been assessed within a 3-month interval after a mean of 4 years post transplantation; 20 repeated pairs of measurements after a mean of 2 years of follow-up were available to analyze the longitudinal effects of post-transplant changes of blood pressure control on left ventricular mass index (LVMI). Arterial hypertension (59%) and left ventricular hypertrophy (50%) were highly prevalent in children after renal transplantation. Renal allograft function and number of antihypertensive medications, but not ABPM variables, were correlated with LVMI at the initial observation. However, at repeat assessment, a significant correlation between ABPM and LVMI was found. In the longitudinal assessment, left ventricular remodeling was dependent on change of dosage of cyclosporine and interval changes of blood pressure levels. Hence, control of blood pressure correlates with changes of LVMI in children with renal allografts. These results clearly underline the importance of blood pressure control for the maintenance of the myocardial architecture.