Hemimegalencephaly: 2D, 3D Ultrasound and MRI Correlation.

BACKGROUND: Hemimegalencephaly (HMC) is a disorder associated with enlarged and dysplastic hamartomatous overgrowth of all or part of the one cerebral hemisphere that can be isolated or associated with other syndromes. In the normal development of the brain it is important to bear in mind that there are two main processes: firstly the development of the hemispheres and the corpus callosum, and secondly the cortical formation with proliferation, migration and organization of the cortex, which occurs mostly between 12 and 20 weeks of gestation. CASE REPORT: We present a 22-week-old fetus with macrocephaly depending on HMC and emphasize the possibility of an early ultrasound diagnosis, the correlation in the diagnosis between 2D and 3D ultrasound, and the use of magnetic resonance imaging as an imaging method for a more precise diagnosis of neuronal migration anomalies. CONCLUSION: The diagnosis of HMC is possible at the time of the anomaly scan. The use and correlation with other diagnostic tools provide essential information for parent counseling in these complex cases.

Hypertensive disorders in pregnancy: screening by uterine artery Doppler imaging and blood pressure at 11-13 weeks.

OBJECTIVES: To examine the performance of screening for hypertensive disorders in pregnancy at 11-13 weeks by a combination of the maternal history, uterine artery Doppler imaging and blood pressure. METHODS: This was a prospective screening study for pre-eclampsia (PE) requiring delivery before 34 weeks (early PE), late PE and gestational hypertension (GH) in women attending for their routine first hospital visit in pregnancy at 11 + 0 to 13 + 6 weeks of gestation. Maternal history was recorded, color flow Doppler imaging was used to identify the uterine artery with the lowest pulsatility index (L-PI) and automated devices were used to measure the mean arterial pressure (MAP). The performance of screening for PE and GH by a combination of the maternal factor-derived a-priori risk, the uterine artery L-PI and MAP was determined. RESULTS: There were 8061 (96.4%) cases unaffected by PE or GH, 165 (2.0%) that developed PE including 37 that required delivery before 34 weeks (early PE) and 128 with late PE, and 140 (1.7%) that developed GH. The MAP was higher in early PE, late PE and GH than in the unaffected group (P < 0.0001), and in early PE than in GH (P = 0.002). The uterine artery L-PI was significantly higher in early PE and late PE than in the unaffected group (P < 0.0001), in early PE than late PE or GH (P < 0.0001), and in GH than in the unaffected group (P = 0.014). In screening by a combination of the maternal factor-derived a-priori risk, uterine artery L-PI and MAP, the estimated detection rate at a 10% false-positive rate was 89.2% (95% CI, 74.6-96.9%) for early PE, 57.0% (95% CI, 48.0-65.7%) for late PE and 50.0% (95% CI, 41.4-58.6%) for GH. CONCLUSIONS: Effective screening for hypertensive disorders in pregnancy is provided by a combination of maternal history, uterine artery Doppler imaging and blood pressure at 11-13 weeks.

Characterization of mouse CD229 (Ly9), a leukocyte cell surface molecule of the CD150 (SLAM) family.

Mouse CD229 (Ly9) is a cell surface molecule of the CD150 (signaling lymphocyte activation molecule) family. This family consists of nine leukocyte receptors of the immunoglobulin superfamily that are involved in leukocyte activation. CD229 binds to SAP, a protein encoded by the gene for X-linked lymphoproliferative disease. In this study, mouse CD229 expression was assessed with a new CD229-specific monoclonal antibody (mAb) (Ly9.ab3), raised using CD229-transfected cells. CD229 was expressed on Sca-1+c-kit+Lin- hematopoietic stem cells, and this expression increased during lymphocyte maturation. Virtually, all T and B cells expressed high levels of CD229. CD229 was absent on granulocytes, bone marrow-derived dendritic cells, platelets, and red blood cells (RBCs). However, it was expressed at significant levels on monocytes, indicating that it is also expressed on mouse myeloid cells. We also show that natural killer cells, natural killer T cells, and B1 cells express very high levels of this molecule. In vitro functional experiments showed that ligation of CD229 inhibited the expression of the activation markers CD69 and CD25 on T lymphocytes in response to anti-CD3 stimulation. Moreover, this reduced activation was concurrent with a reduction in cytokine production. Our results show that CD229 is a pan-lymphocyte marker and indicate that mAbs against CD229 are able to down-modulate T-cell activation.

Differential expression of SAP and EAT-2-binding leukocyte cell-surface molecules CD84, CD150 (SLAM), CD229 (Ly9) and CD244 (2B4).

The CD150 (SLAM) family consists of nine leukocyte cell-surface proteins involved in lymphocyte activation that belong to the immunoglobulin (Ig) superfamily. Six members of this family--CD84, CD150 (SLAM), CD229 (Ly9), CD244 (2B4), NTB-A, and CS1--associate with adapter proteins--SLAM-associated protein (SAP) and EAT-2. SAP is a short intracellular molecule that is mutated in humans with X-linked lymphoproliferative disease. Flow cytometric analysis of the expression of CD84, CD150, CD229, and CD244 cell-surface receptors on several leukocyte and lymphocyte subsets was performed. CD84 and CD150 were present on thymocytes, mature T cells and antigen-presenting cells. The expression of CD84 and CD150 was high on memory T cells. CD150 expression was strongly up-regulated after cell activation. In contrast to CD84, CD150 was absent on resting monocytes and immature dendritic cells (DCs). CD229 presented a pattern of expression restricted to lymphocytes. CD244 was preferentially expressed on natural killer cells, CD8(+) effector cells, resting monocytes, basophils, and eosinophils. We describe a broader distribution of CD84, CD150, CD229, and CD244 than previously reported and show that they are differentially expressed on hematopoietic cells. The heterogeneous expression of these receptors indicates that these molecules may play non-redundant functions in the regulation of both innate and adaptive immune responses.

CD84 functions as a homophilic adhesion molecule and enhances IFN-gamma secretion: adhesion is mediated by Ig-like domain 1.

CD84 is a member of the CD2 subset of the Ig superfamily of cell surface molecules. Its cytoplasmic tail binds to Src homology 2 domain-containing protein 1A (signaling lymphocytic activation molecule-associated protein), a protein encoded by the X-linked lymphoproliferative disease gene. It is preferentially expressed on B lymphocytes, monocytes, and platelets. We show that it is also expressed on thymocytes and T cells. CD84 was positive on CD4-CD8- thymocytes, and its expression decreased with cell maturation. It is expressed on mature T cells preferentially on CD45RO+. To identify the CD84 ligand, we generated a soluble Ig fusion protein containing the human CD84 extracellular domains (CD84-Ig). Because receptor-ligand interactions occur between several members of this subfamily, we assayed CD84-Ig binding with all members of the CD2 family. CD84-Ig bound to CD84-transfected cells, whereas no binding was detected with cells expressing other CD2 subfamily receptors, showing that CD84 binds to itself. Anti-CD84 mAbs recognizing epitopes wholly within domain 1 of CD84 blocked the binding of the CD84-Ig fusion protein to CD84-transfected cells and platelets. Data from CD84 domain human/mouse chimeras further revealed that only the first extracellular domain of the molecule is involved in the ligand receptor recognition. The CD84-CD84 interaction was independent of its cytoplasmic tail. Finally, concurrent ligation of human CD84 with mAbs or CD84-Ig and CD3 enhanced IFN-gamma secretion in human lymphocytes. Thus, CD84 is its own ligand and acts as a costimulatory molecule.

Ultrastructure and cytopathology of a rickettsia-like organism causing systemic infection in the redclaw crayfish, Cherax quadricarinatus (Crustacea: decapoda), in Ecuador.

A study of the ultrastructural characteristics of an intracellular bacterium infecting the redclaw crayfish, Cherax quadricarinatus, a pathogen referred to previously as a rickettsia-like organism (RLO), revealed the presence of different developmental stages. These included a rod-shaped and uniformly electron-dense elementary body (EB) and an intermediate body (IB). The length of the EB varied between 0.48 and 0.6 microm, and the diameter was 0.3 microm. The IB was 0.75 to 1.1 microm long by 0.36 to 0.44 microm in diameter. Although the EB of this bacterium has ultrastructural characteristics similar to those of Rickettsiella, no information is available regarding its genetic relationship to this genus, and the intracellular bacterium should continue to be referred to as a rickettsia-like organism. The hemocytes had different levels of infection, and the RLO proliferated inside these cells. The EB appeared to be free in the cytoplasm of infected hemocytes and other cells; however, this might be a fixation artifact. The EB was also contained in membrane-bound vacuoles along with the IB. RLO colonies were observed inside small granular cells. No large granular cells were observed in the sections examined; therefore, no data were obtained regarding infection of this type of hemocyte. The fixed phagocytes on the external side of the terminal hepatic arterioles had an activated interrupted layer containing RLO bacteria. Stem cells in the hematopoietic tissue were also infected, and some cells were apparently being released into circulation.