Standard value of ultrasound elastography using acoustic radiation force impulse imaging (ARFI) in healthy liver tissue of children and adolescents.

PURPOSE: Ultrasound elastography by acoustic radiation force impulse imaging (ARFI) is used in adults for non invasive measurement of liver stiffness, indicating liver diseases like fibrosis. To establish ARFI in children and adolescents we determined standard values of healthy liver tissue and analysed potentially influencing factors. MATERIALS AND METHODS: 132 patients between 0 and 17 years old were measured using ARFI. None of them had any liver disease or any other disease that could affect the liver secondarily. All patients had a normal ultrasound scan, a normal BMI and normal liver function tests. The mean value of all ARFI measurements was calculated and potentially influencing factors were analysed. RESULTS: The mean value of all ARFI elastography measurements was 1.16 m/sec (SD ±â€Š0.14 m/sec). Neither age (p = 0.533) nor depth of measurement (p = 0.066) had no significant influence on ARFI values, whereas a significant effect of gender was found with lower ARFI values in females (p = 0.025), however, there was no significant interaction between age groups (before or after puberty) and gender (p = 0.276). There was an interlobar difference with lower values in the right liver lobe compared to the left (p = 0.036) and with a significantly lower variance (p < 0.001). Consistend values were measured by different examiners (p = 0.108), however, the inter examiner variance deviated significantly (p < 0.001). CONCLUSION: ARFI elastography is a reliable method to measure liver stiffness in children and adolescents. In relation to studies which analyse liver diseases, the standard value of 1.16 m/sec (±â€Š0.14 m/sec) allows a differentiation of healthy versus pathological liver tissue.

Partial splenic embolization as an alternative to splenectomy in hypersplenism--single center experience in 16 years.

PROBLEM: In young patients with hypersplenism splenectomy implies a lifelong increased risk for post-splenectomy infection. Especially in children, whose immune system is not yet completely matured, the risk for some bacterial infection may increase after splenectomy because the spleen helps to defend against encapsulated bacteria like pneumococci, meningococci and haemophilus influenzae. We present partial splenic embolization as an alternative to surgical splenectomy. METHOD: Partial splenic embolization was performed in 17 patients from 1-31 years with hypersplenism of various etiologies and was achieved by selective catheterization of splenic arteries and injection of 150-355 µm polyvinyl alcohol particles (Ivalon (®)). After the intervention the patients received an intensified analgesic regimen and antibiotics to avoid concurrent infectious complications. RESULTS: Partial splenic embolization represented between 30-60% of the splenic volume and was followed in general by an immediate increase of all blood cells and symptoms of hypersplenism were reduced. In 2 patients the procedure was repeated because the result of the first embolization was insufficient in one patient and became necessary in another in the long run. Post-procedural side effects included fever, abdominal pain, ascites and pleural effusions. There were no acute infections in any patient. CONCLUSION: Our monoinstitutional experiences over 16 years offer, partial splenic embolization in patients with hypersplenism from miscellaneous reasons as a low-risk alternative to surgical splenectomy. The procedure can be repeated as necessary, but it is always a temporary palliation depending on the underlying disease which often leads to liver transplantation. Using intensive analgesia and antibiotics side effects were tolerable, and patients could be discharged after a few days.

Fenestration in extracardiac conduits in children after modified Fontan operation by implantation of stent grafts.

Three patients (ages 3, 5, and 8 years) with various forms of functionally univentricular heart lesions received a total cavopulmonary connection with an extracardiac conduit as a final reconstructive procedure. Failure of the Fontan circulation occurred immediately after surgery because of spontaneous closure of surgical fenestrations in two children and absent fenestration in one. As an emergency procedure, in all patients the conduit was perforated by transcatheter intervention in order to create a connection to the anatomical right atrium. Following balloon dilatation of the perforated conduit, in all three patients covered stent grafts were placed in the newly created defect to attain a reliable communication. Patency of the fenestration was demonstrated by angiogram and any leakage was ruled out. Cardiac output improved and severe pleural effusion and ascites subsided. Right-to-left shunt could be demonstrated by echocardiography at follow-up after 7 months (median) in all three patients. Oxygen saturation remained stable between 85 and 90%. These preliminary results suggest that stent graft fenestration can serve as a valuable tool in failing Fontan circulation, particularly in patients with an extracardiac conduit. Covered stents have the potential to reduce the acute risk of bleeding and they help to prevent early spontaneous closure of the newly created fenestration.

Human lactate dehydrogenase-B processed pseudogene: nucleotide sequence analysis and assignment to the X-chromosome.

Two human genomic clones containing the lactate dehydrogenase-B processed pseudogene were isolated from two patients deficient in lactate dehydrogenase-B isozyme. The sequences of 3,287 nucleotides, including the pseudogenes and its flanking regions, from both clones were found to be identical except for three differences in the pseudogenes. The sequences of 1,286 nucleotides from these two pseudogenes exhibited 93% homology with the cDNA sequence of the lactate dehydrogenase-B functional gene, and the pseudogene contained 75/76 base substitutions, 11/12 single-base deletions, and 5 single-base insertions. This pseudogene was mapped to the x-chromosome by dot-blot analysis using a probe for the pseudogene or its 5' flanking sequence.

The clinical usefulness of chromosome analysis by flow cytometry.

Historical and clinical aspects of chromosome analysis by flow cytometric methods are reviewed. A new method of preparing small samples (1.0 mL of blood) of peripheral lymphocytes for flow karyotype analysis using phytohemagglutinin and interleukin-2 is presented in detail. Figures of flow karyotypes, partial banded karyotypes, and idiograms of patients with inv(8), rec dup(8), rob t(14;21), and t(1;22) are presented, as well as examples of univariate and bivariate flow histograms from other researchers' published works. The clinical utility of these techniques is explored, with specific reference to recent work in chromosome polymorphisms and cultured amniocyte lines. We conclude that, although flow karyotyping is not a replacement for classical banded chromosome analysis, used as an adjunct, this new technique has some clinical usefulness relating to its capability to resolve small differences in chromosomal DNA content.

Assignment of the gene for the E1 alpha subunit of branched chain alpha-ketoacid dehydrogenase to chromosome 19.

The gene encoding the E1 alpha subunit of branched chain alpha-ketoacid dehydrogenase was mapped to human chromosome 19. 32P-labeled human E1 alpha cDNA was hybridized with DNA derived from flow-sorted human chromosomes; it hybridized exclusively with that from chromosome 19.

Mapping of human lactate dehydrogenase-A, -B, and -C genes and their related sequences: the gene for LDHC is located with that for LDHA on chromosome 11.

Human testis-specific lactate dehydrogenase-C (LDHC) gene-related sequences are located with the LDHA gene on chromosome 11. The LDHB gene is on chromosome 12. Chromosomes 1, 2, 4, 9, and 10 appear to contain LDHA gene-related sequences, whereas the X chromosome and chromosome 13 possess LDHB gene-related sequences.