α-Glutathione-s-transferase as a new sensitive marker of hepatocellular damage in biliary atresia

 Early identification of patients likely to deteriorate post-hepatic portoenterostomy for biliary atresia (BA) would be beneficial. α-Glutathione-s-transferase (α-GST) is a serologic marker of reactive hepatocellular damage because of its low molecular weight, uniform hepatic distribution, high cytosol concentration, and short half-life. We evaluated whether serum α-GST in post-surgical BA patients correlates with liver function (LF) and investigated its potential as a medium- to long-term marker of prognosis. Postoperative BA patients (n = 30; mean age: 11.8 ± 3.7 years) were divided into three groups based on average LF over the 3 months prior to this study. Group I (n = 8) were jaundice-free and had normal LF. Group II (n = 12) had moderate liver dysfunction, and group III (n = 10) had severe liver dysfunction. Serum α-GST was determined using a specific ELISA. Tissue α-GST was determined immunohistochemically, using liver needle-biopsy specimens. Bile lakes were found in 5 group II patients and 5 group III patients. Serum α-GST was significantly higher in group II (20.7 ± 8.4 ng/ml) than in groups I (4.7 ± 1.3 ng/ml) and III (8.0 ± 1.2 ng/ml) (P < 0.0001) and was highest in group II subjects with bile lakes. In control liver specimens α-GST distribution was weak but uniform throughout normal liver lobule hepatocytes. In group II there was strong staining in centrilobular hepatocytes, and in group III α-GST was only found in regenerative nodules. We conclude that α-GST may be a more sensitive indicator of hepatocellular damage in BA because its distribution is correlated to the proportion of functioning liver tissue present. This is the first report of this relationship, which has great implications for group II subjects because a sudden shift in concentration of α-GST may be a better predictor of impending hepatic dysfunction than conventional LF tests.     

Final height, gonadal function and bone mineral density of adolescent males with central precocious puberty after therapy with gonadotropin-releasing hormone analogues

Few data are available on the outcome of boys with central precocious puberty (CPP) treated with gonadotropin-releasing hormone (GnRH) analogues. We report on final height, endocrine and exocrine testicular function, and bone mineral density (BMD) in nine males (age 16.7 ± 1.5 years) treated with GnRH analogues from the age 6.0 ± 1.8 years for a mean period of 5.6 ± 2.4 years. The following parameters were evaluated: final height, serum gonadotropin and gonadal steroid levels, spermarche, semen analysis, area and volumetric BMD. Final height (−0.4 ± 1.1 SDS) was significantly higher than pre-treatment predicted adult height (−2.0 ± 1.2 SDS) and not significantly different than midparental height (−0.1 ± 0.8 SDS). Pubertal response of gonadotropins to GnRH test occurred within 1.5 years (mean 0.7 ± 0.4 years) and spermarche (n=7) from 0.7 to 3 years (1.8 ± 0.9 years) after the discontinuation of GnRH analogue therapy. No alteration in semen analysis was found (n=6, sperm count, 10⁶/ml: 52.0 ± 18.7; normal motility (%): 49.5 ± 18.7; atypical morphology (%): 44.5 ± 11.4). Area and volumetric BMD were not reduced (0.2 ± 1.0 SDS and −0.1 ± 0.9 SDS, respectively). Conclusion Long-term treatment with gonadotropin-releasing hormone analogues improves final height in boys with central precocious puberty. Post-therapy data demonstrating normal endocrine and exocrine testicular function support the safety of gonadotropin-releasing hormone analogues on reproductive function. Long-term pharmacological suppression of testicular function in childhood does not impair bone mineral density in late adolescence. Received: 4 May 1999 / Accepted: 30 November 1999     

Evaluation of tumour necrosis during chemotherapy with diffusion-weighted MR imaging: preliminary results in osteosarcomas

Background During successful chemotherapy of osteosarcomas tumour size does not diminish significantly because the therapy has limited impact on the mineralized matrix of the tumour. Treatment response is considered successful if, histologically, more than 90% of tumour cells show necrosis. Objective To determine if osteosarcomas change their water diffusion during preoperative chemotherapy in relation to the amount of tumour necrosis. Materials and Methods Eight patients (age 11–19 years) with histologically proven limb osteosarcoma underwent T1-weighted, fat-suppressed T2-weighted and contrast-enhanced T1-weighted spin-echo imaging together with diffusion-weighted EPI sequences (b = 700) at 1.5 T before and after five cycles of standard chemotherapy. Tumour volume and apparent diffusion coefficient (ADC) maps were calculated before and after chemotherapy. The degree of tumour necrosis after chemotherapy was assessed using the histological Salzer-Kuntschik classification (grades 1–6). Results During chemotherapy, the ADC values of osteosarcomas changed significantly. The ADC of untreated tumour was 2.1 ± 0.4 × 10⁻³ mm²/s (mean ± SD) (95% CI 1.6–2.0). The ADC of chemotherapy-treated sarcomas was 2.5 ± 0.4 × 10⁻³ mm²/s (95% CI 1.8–2.2). Necrotic areas, which were confirmed by macroscopic examination, showed ADC values up to 2.7 × 10⁻³ mm²/s. Four patients with little viable tumour tissue within the neoplasm (Salzer-Kuntschik grades 1–2) had an increase in ADC of 0.4 up to 0.7 × 10⁻³ mm²/s. Four patients with larger areas of viable tumour (Salzer-Kuntschik grade 4) showed a lesser increase in ADC of 0.0 up to 0.3 × 10⁻³ mm²/s. The differences in ADC values in tumour tissue before and after chemotherapy were highly significant (P = 0.01). Conclusion During chemotherapy of osteosarcomas, tumour ADC changes are related to the degree of tumour necrosis. Supported by Grant DFG# u103     

Carnitine administration ameliorates the changes in energy metabolism caused by short-term pivampicillin medication

Ten children receiving pivampicillin for 8 days were studied. On the first 4 days the drug was given alone (4 × 500 mg/day), and on the last 4 days in combination with carnitine (4 × 1 g/day). Pivampicillin treatment was associated with formation and urinary excretion of pivaloylcarnitine and administration of carnitine aided the elimination of pivalate as its carnitine ester. The resting respiratory quotient increased from 0.86 ± 0.01 to 0.96 ± 0.01 on the 4th day of pivampicillin treatment. A shift was observed in the metabolic fuel consumption: a significant decrease was found in the amount of fats oxidized (0.31 ± 0.17 vs 1.27 ± 0.17 g · kg⁻¹ · 24 h⁻¹), while the utilization of carbohydrates increased (6.20 ± 0.51 vs 4.00 ± 0.50 g kg⁻¹ · 24 h⁻¹). Administration of carnitine decreased the respiratory quotient to 0.90 ± 0.01 on the 8th day of treatment, consumption of fats increased, and the oxidation of carbohydrates decreased. The resting energy expenditure was not affected by the treatment. Conclusion Pivampicillin treatment results in inhibited oxidation of fats as metabolic fuel. This drug effect was partially reversed by carnitine which promotes the elimination of the pivaloyl moiety from the body. Received: 6 January 1997 / Accepted: 18 March 1997     

Are stable postoperative biliary atresia patients really stable?

 Transforming growth factor-beta 1 (TGF β-1) is an important mediator of liver-cell proliferation and replication that is implicated in hepatic fibrosis (HF). Hepatic stellate cells (HSC) are activated by TGF β-1 and are the main precursor cells involved in fibrogenesis. The correlation between serum TGF β-1, activated HSC in liver-biopsy specimens, and liver biochemistry was investigated to determine the value of TGF β-1 as an indicator of clinical status in postoperative biliary atresia (BA) patients. Thirty-two postoperative BA patients (mean age 11.2 ± 2.8 years) and 13 normal controls (mean age 10.3 ± 3.7 years) were studied. Based on average liver function test (LFT) results over a 3-month period immediately prior to this study, the BA patients were divided into group I (anicteric, normal LFT; n = 10); group II (anicteric, elevated liver transaminases; n = 12), and group III (jaundiced end-stage liver fibrosis awaiting liver transplantation; n = 10). Serum TGF β-1 was determined using ELISA. Liver-biopsy specimens were examined with antibody against TGF β-1 and α-smooth muscle actin (SMA) antibody for detection of activated HSC. Serum TGF β-1 was significantly higher in groups I (11.4 ± 3.7 ng/ml; P < 0.01) and II (23.3 ± 11.3 ng/ml; P < 0.001) than in group III (3.0 ± 1.5 ng/ml) and controls (4.5 ± 2.5 ng/ml) despite normal LFT in group I. The 3 subjects with the highest serum TGF β-1 in group II had bile lakes. Biopsies from groups I and II were strongly positive for TGF β-1 in hepatocytes and Kupffer cells and for activated HSC detected by SMA compared with group III and controls. Because serum TGF β-1 and activated HSC are only present during active fibrosis, we conclude that there is progressive fibrogenesis even in seemingly normal postoperative BA patients. In particular, bile lakes should be regarded as a key sign of progressive HF, the presence of which should be regarded with extreme caution. We suggest that serum TGF β-1 could be used as an accurate indicator of progressive fibrogenesis in postoperative BA patients. Accepted: 14 April 2000     

Serum phenylalanine in preterm newborns fed different diets of human milk

ObjectiveTo evaluate phenylalanine plasma profile in preterm newborns fed different human milk diets.MethodsTwenty-four very-low weight preterm newborns were distributed randomly in three groups with different feeding types: Group I: banked human milk plus 5% commercial fortifier with bovine protein, Group II: banked human milk plus evaporated fortifier derived from modified human milk, Group III: banked human milk plus lyophilized fortifier derived from modified human milk. The newborns received the group diet when full diet was attained at 15 ± 2 days. Plasma amino acid analysis was performedon the first and last day of feeding. Comparison among groups was performed by statistical tests: one way ANOVA with Tukey's post-test using SPSS software, version 20.0 (IBM Corp, NY, USA), considering a significance level of 5%.ResultsPhenylalanine levels in the first and second analysis were, respectively, in Group I: 11.9 ± 1.22 and 29.72 ± 0.73; in Group II: 11.72 ± 1.04 and 13.44 ± 0.61; and in Group III: 11.3 ± 1.18 and 15.42 ± 0.83 μmol/L.ConclusionThe observed results demonstrated that human milk with fortifiers derived from human milk acted as a good substratum for preterm infant feeding both in the evaporated or the lyophilized form, without significant increases in plasma phenylalanine levels in comparison to human milk with commercial fortifier.     

Clinical significance of c-kit expression in biliary atresia

The aim of the present study was to examine the clinical significance of c-kit expression in biliary atresia (BA) using formalin-fixed, paraffin-embedded sections from 21 patients with BA. Patients were divided into group I (n = 8) with good liver function; group II (n = 8) with moderate liver dysfunction; and group III (n = 5) with severe liver dysfunction. Choledochal cysts (CDC, n = 5) and normal liver samples (NL, n = 4) served as controls. The results were analyzed and compared among the groups. Most c-kit ⁺ cells were present in the portal tracts, and their numbers in BA were significantly higher than in the controls (11.12 ± 1.64 vs 2.15 ± 0.15 [mean ± standard error], P = 0.02, BA vs CDC; 11.12 ± 1.64 vs 1.66 ± 0.52, P = 0.03, BA vs NL). Clinical correlation revealed a significantly higher number of c-kit ⁺ cells in group III versus group I (18.10 ± 3.62 vs 8.86 ± 2.51, P = 0.02). These results suggest that c-kit overexpression is associated with an adverse clinical outcome in BA. Accepted: 1 November 2000     

Thyroid function in fullterm and preterm newborns

The development of the hypothalamic-pituitary-thyroid system in the fetus occurs through three phases: thyroid pituitary embryogenesis, maturation of hypothalamus, maturation of thyroid system, neuroendocrine control and T₄ tissue deiodination. Defects in early phases I and II lead to permanent disorders whereas abnormalities in phase III result in transient functional immaturities especially in preterms. Cord serum TSH, T₄ and T₃ were estimated in 450 newborns (390 full term>36 wk gestation and 60 preterms<36 wk). The mean cord TSH of 5·069±7·4 μ U/ml in full term was lower than 7·88±3·77 μ U/ml in preterms (P<0·01). The mean cord T₄ and T₃ were significantly higher (P<0·01) 9·716±6·44 μg/dl and 0·425±0·17 ng/ml in full term as compared to preterms 6·46±3·4 and 0·355±0·16 respectively. There was significant negative correlation of serum TSH (r=−0·84 and P<0·05) and positive correlation of serum T₄ (r=0·97, P<0·001) and T₃ (r=0·89, P<0·05) with gestational age. The relationship of these hormones to weight irrespective of gestational age was more significant when compared in newborns >3 kg and <2 kg rather than in all intermediate groups. No significant differences in these hormones were evident amongst the AGA and SGA infants above and below 36 weeks gestation. Transient hypothyroxinemia, and hyper-thyrotropinemia, transient primary hypothyroidism and low T₃ syndromes are some of the transient abnormalities of thyroid function and are more commonly encountered in preterms.     

Serial diffusion-weighted MRI correlates with clinical course and treatment response in children with intracranial pus collections

Background Accurate assessment of treatment response in children with intracranial pus collections is vital to guide appropriate therapy and reduce morbidity and mortality. Objective To correlate serial MR-measurable changes in diffusion-weighted imaging (DWI) with clinical response to treatment. Materials and methods We retrospectively reviewed clinical notes, conventional MR sequences and DWI in eight children with intracranial pus collections. Trace DWI signal intensity and apparent diffusion coefficient (ADC) values were compared at three time points: at initial diagnosis (eight children, 13 collections), at follow-up during continued clinical infection (three children, sp collections), and at follow-up when clinical infection had resolved (seven children, 12 collections). Results At initial diagnosis all patients were septic and collections showed restricted diffusion (mean ADC 0.61±0.15×10⁻³mm²/s). Patients with persistent clinical sepsis at follow-up DWI had collections with persistent low ADC values (0.66±0.21×10⁻³mm²/s), significantly (P<0.001) below normal cortical gray matter values. Successful resolution of the infection was associated with a significant rise in ADC values (1.57±0.57×10⁻³mm²/s, P<0.01) compared both to patients with signs of continued sepsis and to normal gray matter values. Conclusion Persistent restricted diffusion in pus collections correlates with continued sepsis. Treatment response is associated with clinical resolution of sepsis and ADC value elevation significantly above normal gray matter values. Presented at ESPR 2005, Dublin, Ireland     

Prognostic factors indicating survival with native liver after Kasai procedure for biliary atresia

Background

The number of the bile ducts in the portal canal/measured surface area of the portal canal (BDP ratio) indicates prognosis in biliary atresia (BA), as does an elevated cytokeratin 7 positivity percentage (PCK7). We compared these two markers.

Methods

We reviewed 32 BA cases undergoing Kasai operation from 1976 to 2016 with >5 portal canals in biopsy samples. Group I required liver transplantation or died within a year of operation (n = 8). Group II survived with their native liver (n = 24). We determined the BDP ratio (10²/mm²) and PCK7 (%), subdividing patients into three groups by their age at operation: Group A ≤60 days (n = 6, 1 Group I), 60< Group B ≤90days (n = 16, 5 Group I), Group C >90 days (n = 10, 2 Group I).

Results

PCK7 (%) was 2.71 ± 1.87 in Group I and 4.25 ± 2.56 in Group II (p = 0.13). BDP ratio (10²/mm²) was 1.19 ± 0.424 in Group I and 1.64 ± 0.534 in Group II (p = 0.04). Both markers were higher in Group C than in Group A or B (p < 0.01).

Conclusion

The BDP ratio is a better prognostic indicator than PCK7 in BA.

     

Postnatal management of fetal and neonatal alloimmune thrombocytopenia: the role of matched platelet transfusion and IVIG

We evaluated the effects of platelet transfusions and intravenous immunoglobulin (IVIG) in neonates with fetal and neonatal alloimmune thrombocytopenia (FNAIT) with and without antenatal treatment with IVIG. Records of neonates with FNAIT admitted between January 2000 and November 2005 were reviewed. The patients were divided into group I, treated antenatally with IVIG for known FNAIT, and group II, postnatally diagnosed with FNAIT. The primary outcome was the time interval to reach a platelet level above 100 × 10⁹/L in relation to the type of treatment. Nineteen neonates with FNAIT were identified, 13 in group I and 6 in group II. In group I, four children were born with a platelet count above 100 × 10⁹/L and never needed treatment, and four received a single matched platelet transfusion at birth with a maintained response. Five neonates received IVIG and one matched transfusion, with all but one rapidly responding. In antenatally treated cases, postnatal IVIG had no apparent effect on the platelet count. In group II, two neonates died on day 1 with severe intracranial hemorrhage. Two of the four other patients responded to a number of unmatched platelet transfusions, with one neonate rapidly responding after one matched transfusion, while another needed nine matched transfusions before a persistent adequate platelet count was reached after 9 weeks. Postnatal IVIG had no apparent effect on the platelet count in any of our cases. In neonates with FNAIT treated antenatally with IVIG, neonatal management using a single matched platelet transfusion was adequate in all cases. In neonatally diagnosed cases not treated before birth, multiple matched platelet transfusions may be required. We found no evidence to support the use of IVIG in neonates with FNAIT.     

Rupture of membranes,gastric aspirate cytology and neonatal sepsis

Gastric aspirates were examined for polymorphs in 117 infants, born after prolonged rupture of membranes of more than 12 hours duration. Of 42 infants with gastric aspirate polymorph count of more than 5 per high power field (HPF), 19 (45. 2 per cent) infants developed pneumonia and bronchopneumonia and 11 (26.2 per cent) developed septicemia. Of 75 infants with cell count of less than 5 per HPF, two infants developed pneumonia (2.5 per cent) and one developed septicemia (1.3 per cent). A polymorph count of over 65 per cent was associated with pneumonia or bronchopneumonia in all cases and a count of 30–65 per cent was associated with 20% possibility of infection. Supported in part by the Indian Council of Medical Research.     

Early growth of term SFD infants in relation to caloric intake

Growth and development of 100 term SFD infants divided into 3 groups with body weight of 1·5 kg or less (I), 1·51–1·75 kg (II), 1·76–2·25 kg (III) and 100 AFD term infants was determined longitudinally. Group I and II infants remained smaller and had delayed milestones of development throughout the 1st year of life, with limited catch up only in body weight in the first 3 months. Their milk intake was low (132 ml/kg). Group III infants, who had comparatively better growth parameters at birth, showed effective catch up growth in all the parameters to reach the level of those of AFD infants within 3–10 months by increased consumption of milk (186 ml/kg). Their milestones of development were at par with that of AFD infants who consumed 160 ml of milk/kg/day in the first 2–4 months. The low consumption of milk by group I and II infants with severe intrauterine malnutrition is possibly related to the reduced appetite geared to a small body size.     

High dose methylprednisolone therapy in nephrotic syndrome

This study was done to determine the efficacy of oral high dose methylprednisolone (HDMP) therapy in the treatment of childhood nephrotic syndrome (NS). Fifteen patients were enrolled in the study. Patients were arbitrarily divided into two groups. Group I received prednisolone (daily 60 mg/m² for 4 weeks, 45, 30, 20, 10, 5 mg/m² on alternate days for 4 weeks) and group II received HDMP (30 mg/kg/d for 3 days, 20 mg/kg/d for 4 days, 10 mg/kg/for a week, before 9 am, orally). The patients were followed-up for a duration of 38.0±5.5 months (range 24–68 months) in group I and 42.1±5.5 months (range 16–72 months) in group II. No significant difference was obtained in the duration of remission between both groups (p>0.05), while HDMP induced early remission than prednisolone (p<0.05). The mean relapse rate was 0.8/year in group I and 0.8/year in group II (p>0.05). Although, the number of the patients were limited in the study it can be recommended that patients with NS can be treated with oral HDMP therapy as an alternative to standard oral prednisolone therapy.     

On-Line Automated Border Detection for Echocardiographic Quantification of Right Ventricular Size and Function in Children

Rapid, accurate assessment of right ventricular (RV) size is important for the management of children with congenital heart disease. The usefulness of the Acoustic Quantification system of automated border detection (ABD) and on-line quantification (AQ) for assessment of RV size was tested in 36 children. AQ data were compared to ``corrected AQ' measurements (after correction for cavity areas erroneously included in the region of interest) required for AQ. Furthermore, the influence of necessary changes to gain settings was tested in ``lateral gain control' (LGC) images obtained by removal of ABD overlays. All results were compared to conventional echocardiography (echo), and agreement with magnetic resonance imaging (MRI) RV areas was assessed. Systematic differences (±) limits of agreement with MRI (transverse plane) for conventional echo and AQ (apical four-chamber view) were as follows: end-diastolic −0.8 ± 3.8 (conventional echo) versus −1.7 ± 4.6 (AQ) cm²/m² (p < 0.001); end-systolic −1.3 ± 3.2 versus −4.9 ± 5.8 (AQ) cm²/m² (p < 0.001); fractional area change 7.8 ± 17.0% versus 26.9 ± 31.4% (AQ) (p < 0.001). Differences between conventional echo, LGC, and corrected AQ areas were not statistically significant. The best agreement between MRI and echocardiography was with conventional echo. We conclude that automated border detection of the RV can be performed successfully with the AQ system at a fixed point in the cardiac cycle. For adequate assessment of RV function manual corrections of online AQ results are still required, which results in an important reduction of the time gain of on-line quantification.     

Impaired phagocytosis and opsonisation towards group B streptococci in preterm neonates

AIMS—To study the chemiluminescence response in polymorphonuclear leucocytes (PMNL) at different stages of maturity and the opsonic capacity of sera with defined titres of anti-capsular type III antibodies, after exposure to serotype III group B streptococci (GBS). The influence of GBS type III capsule expression on PMNL chemiluminescence response was also investigated.METHODS—Two clinical isolates of serotype III GBS and two serotype III reference strains which form isogenic variants with high and low amounts of capsule substance, respectively, were used. PMNL and sera were obtained from adult healthy blood donors, full term neonates, and preterm neonates.RESULTS—PMNL from premature infants showed a significantly lower chemiluminescence response (p<0.0001) than the PMNL from adults and neonates, while the chemiluminescence response with adult, neonatal, and preterm sera gradually diminished. In the presence of a serum pool with a standardised complement value, raised (>10 mg/l), rather than low (<1.0 mg/l) anti-III antibody titres induced a higher chemiluminescence response to the capsule expressing variant. When GBS were cultured at pH 5.0, the bacteria had a higher buoyant density, reflecting decreased expression of capsule substance compared with bacteria grown at pH 7.4. Concomitantly, there was a substantial increase in chemiluminescence response for all isolates cultured at the lower pH, except for the capsule deficient mutant.CONCLUSIONS—PMNL function and opsonic capacity are significantly impaired in neonates and correlate with maturation of the newborn child. The combined defect in cellular and humoral defences in preterm neonates may contribute to their increased susceptibility to GBS infection. Growth conditions for GBS, simulating different in vivo environments, greatly affect capsule expression and resistance to phagocytosis.     

Cardiac Troponin T and Cardiac Dysfunction in Extremely Low-Birth-Weight Infants

Extremely low-birth-weight (ELBW) infants frequently manifest signs of cardiac dysfunction requiring inotropic support. It is not clear if this is due to cardiac injury, which can be monitored by measuring cardiac troponin T (cTnT). We performed a nested prospective cohort study at a university level III neonatal intensive care unit. The study included 27 infants weighing between 500 and 999 g. Exclusion criteria included evidence of sepsis, use of postnatal steroids, and cardiac anomalies. Measurements included serum cTnT and echocardiogram in the first 48 hours of life. The mean serum cTnT level of the study population was 0.52 ± 0.38 ng/ml. It was higher in those with lower Apgar scores (0.89 ± 0.37 if 5-minute Apgar < 4 vs 0.36 ± 0.26 ng/ml, p < 0.001) and correlated to initial base deficit (r = −0.37, p < 0.05). Infants who required inotropic support had higher cTnT levels than those who did not (0.73 ± 0.43 vs 0.39 ± 0.29 ng/ml, p < 0.03). cTnT concentrations did not relate to simultaneous echocardiographic measures of cardiac function. In ELBW infants, serum cTnT levels are higher than normally seen in term infants and adults, and they are higher in infants with greater perinatal stress as well as those who show evidence of cardiac dysfunction requiring pressor support.     

Assessment of Ventricular Function and Dyssynchrony Before and After Stage 2 Palliation of Hypoplastic Left Heart Syndrome Using Two-Dimensional Speckle Tracking

Two-dimensional (2D) speckle tracking (2DST) is a new technique independent of ventricular geometry but not independent of preload and afterload. Using 2DST, this study aimed to investigate differences in right ventricular (RV) function and intraventricular dyssynchrony in patients with hypoplastic left heart syndrome (HLHS) before and after preload-reducing stage 2 palliation. For 31 HLHS patients, this study compared global longitudinal strain (S) and strain rate (SR) as well as regional peak systolic longitudinal S, SR, and velocity (V) in six RV segments on echocardiograms before and after stage 2 surgery. Intraventricular dyssynchrony was assessed by calculating the standard deviation of the intervals from the beginning of systole to peak S, SR, and V. Global S (−16.7 ± 5.0 vs −15.6 ± 5.5%) and global SR (−1.2 ± 0.3 vs −1.2 ± 0.3 s⁻¹) did not change after surgery. After surgery, V decreased in the mid lateral segment (2.3 ± 1.3 vs 1.7 ± 0.9 cm/s; p = 0.01) and the basal lateral segment (3.6 ± 1.1 vs 2.8 ± 1.0 cm/s; p = 0.001), whereas S was lower in both of these segments (−19.9% ± 6.0% vs −17.4% ± 6.3%; p = 0.01 and 20.0 ± 5.1 vs 15.8 ± 7.1%; p = 0.002, respectively). Segmental SR and dyssynchrony did not change. Decreased V and S in the RV free wall could be explained by reduced preload of the systemic RV after stage 2 palliation.     

Correlation of Plasma Adrenomedullin to Myocardial Preservation During Open-Heart Surgery

Adrenomedullin (ADM) is a vasoactive peptide with potent dilatory effects. We studied whether perioperative myocardial injury could be altered by the presence of ADM. Blood samples from 19 children with congenital heart disease undergoing surgical repair were collected at six time points: preoperative, on cardiopulmonary bypass (CPB), and 0, 3, 6, and 12 hours after CPB. Blood levels of ADM (pg/ml) and troponin-I (Tn-I; ng/ml), a specific marker of myocardial injury, were measured. Patients were divided into three groups based on their 12-hour Tn-I levels (I, < 10, n= 6; II, 10–25, n= 6; III, >25, n= 7). Preoperative Tn-I levels were within the normal range for all patients. Preoperative ADM levels in group I (with little or no evidence of myocardial injury) were significantly greater than those of either group II or III (242.7 ± 15.4 vs 83.8 ± 18 and 85.2 ± 5.5, respectively; p≤ 0.0001 for each). The 12-hour ADM levels in group I remained significantly lower than preoperative levels (242.7 ± 15.4 vs 197.4 ± 11.6, p≤ 0.03) but higher than in the other groups. In group III, ADM increased at the 12-hour time point (159.2 ± 6.5, p≤ 0.0001 vs baseline). Higher preoperative ADM levels are associated with lower levels of myocardial injury (as assessed by troponin-I release) during congenital heart surgery.     

Improved body mass index after mesenterico-portal bypass

Extrahepatic portal hypertension in children secondary to portal vein obstruction is frequently associated with impaired somatic growth. The aim of this study was to assess growth and nutritional status, as reflected by the body mass index (BMI), before and after mesenterico-portal bypass (Rex shunt). Eleven children with a portal vein cavernoma underwent mesenterico-portal bypass using autologous jugular vein. All shunts have remained patent during follow-up periods of 7 months to 5 years. All except one child, who had a normal BMI prior to surgery, demonstrated an increase in their BMI standard deviation scores after surgery. Mean BMI standard deviation scores increased from −0.44 ± 1.28 (95% CI −1.30 to 0.42) to 0.46 ± 1.08 (95% CI −0.27 to 1.19), a highly statistically significant increase (P = 0.003). Restoration of hepatopetal portal blood flow by mesenterico-portal bypass surgery improves nutrition and growth in children with extrahepatic portal hypertension.