Isolated herpes simplex keratoconjunctivitis in a neonate born by cesarean delivery.

Herpes simplex virus (HSV) infection can be devastating in the neonate. The disease most commonly presents as 1 of 3 clinical manifestations: disseminated visceral infection (with and without central nervous system involvement), isolated meningoencephalitis, and infection limited to the skin, eyes, and/or mucous membranes (SEM). Exposure leading to neonatal infection typically occurs as peripartum vertical transmission, most typically by direct contact with urogenital lesions or infected genital secretions, or as an ascending infection exploiting disrupted chorioamniotic membranes. We present a novel case of a newborn girl who developed HSV-2 keratoconjunctivitis despite being delivered via an elective, uncomplicated, repeat cesarean over intact chorioamniotic membranes in the absence of active clinical maternal HSV infection and despite having a negative medical history of previous orolabial or genital herpetic infection.     

Expression of late cell cycle genes and an increased proliferative capacity characterize very early relapse of childhood acute lymphoblastic leukemia.

PURPOSE: In childhood acute lymphoblastic leukemia (ALL), approximately 25% of patients suffer from relapse. In recurrent disease, despite intensified therapy, overall cure rates of 40% remain unsatisfactory and survival rates are particularly poor in certain subgroups. The probability of long-term survival after relapse is predicted from well-established prognostic factors (i.e., time and site of relapse, immunophenotype, and minimal residual disease). However, the underlying biological determinants of these prognostic factors remain poorly understood. EXPERIMENTAL DESIGN: Aiming at identifying molecular pathways associated with these clinically well-defined prognostic factors, we did gene expression profiling on 60 prospectively collected samples of first relapse patients enrolled on the relapse trial ALL-REZ BFM 2002 of the Berlin-Frankfurt-Münster study group. RESULTS: We show here that patients with very early relapse of ALL are characterized by a distinctive gene expression pattern. We identified a set of 83 genes differentially expressed in very early relapsed ALL compared with late relapsed disease. The vast majority of genes were up-regulated and many were late cell cycle genes with a function in mitosis. In addition, samples from patients with very early relapse showed a significant increase in the percentage of S and G(2)-M phase cells and this correlated well with the expression level of cell cycle genes. CONCLUSIONS: Very early relapse of ALL is characterized by an increased proliferative capacity of leukemic blasts and up-regulated mitotic genes. The latter suggests that novel drugs, targeting late cell cycle proteins, might be beneficial for these patients that typically face a dismal prognosis.     

眼眶硬化性骨髓外造血细胞瘤

骨髓外造血细胞瘤可发生于包括骨髓纤维化、慢性骨髓性门血病和非常少见的红细胞增多症等骨髓增殖紊乱时。当纤维巨细胞增值伴有坚硬肿块形成时，该区域被称为硬化性骨髓外造血细胞瘤（SEMHTs），据我们所知．我们描述的是第一例发生于双侧眼眶SEMHTs的多发性骨髓纤维变中性。     

Psoriasin expression in mammary epithelial cells in vitro and in vivo.

We determined, by serial analysis of gene expression (SAGE) analysis of normal and DCIS (ductal carcinoma in situ) mammary epithelial cells, that psoriasin and several other genes implicated in psoriasis are aberrantly expressed in high-grade, comedo DCIS. Real-time PCR, mRNA in situ hybridization, and immunohistochemical analysis of breast carcinomas confirmed that psoriasin is frequently overexpressed in estrogen receptor-negative tumors. To gain insight into regulatory pathways that control psoriasin expression, we developed polyclonal and monoclonal antibodies and investigated mechanisms that may account for elevated levels of psoriasin in DCIS. Here, we report that loss of attachment to extracellular matrix, growth factor deprivation, and confluent conditions dramatically up-regulate psoriasin expression in MCF10A mammary epithelial cells. All of these conditions are characteristic of high-grade DCIS and psoriatic skin lesions; therefore, the same mechanisms may be responsible for increased expression of psoriasin in vitro and in vivo.     

CT与磁共振成像在儿童人工耳蜗植入术前研究

目的　评价CT与磁共振成像 (magneticresonanceimaging ,MRI)在儿童人工耳蜗植入术前的诊断作用 ,以及对手术选择的影响。方法　 71例 ( 142耳 )双耳重度耳聋、拟行人工耳蜗植入的患儿进行术前CT与MRI检查。结果　CT与MRI均发现 ,12例 ( 2 2耳 )患者有前庭水管扩大 ( 15 5 % ) ;14例 ( 2 5耳 )患儿有Mondini畸形 ( 17 6% ) ;3例 ( 5耳 )患儿有内耳道扩大 ( 3 5 % ) ;2例 ( 4耳 )患者有可疑内耳道底骨质缺损 ( 2 8% )。MRI发现有 5例 ( 5耳 )患者单侧耳蜗纤维化 ( 3 5 % ) ,而CT未见异常。1例 ( 2耳 )患儿的CT显示面神经裸露 ( 1 4% ) ,MRI正常。结论　人工耳蜗植入术前应该进行CT与MRI检查。对于发现前庭水管综合征、Mondini畸形、内耳道扩张及内耳道底骨质缺损有重要意义。这二种影像学检查结果可以相互补充诊断耳蜗纤维化与面神经裸露。对手术适应证的选择以及保证手术正常进行有重要意义     

Spatial Orientation Adjustments in Children with Autism in Hong Kong

Abnormal spatial orientation and body postures in children with autism often interfere with visual abilities to attend tasks and social interactions. Twenty-four children diagnosed with autism from Kowloon, Hong Kong were assessed for spatial orientation and spatial management abilities. Positive changes in spatial orientation were evident when the children wore ambient prism lenses and included changes in posture from slanted to erect. Adjustments in spatial management were evident in improved ball catching ability, a task requiring visual tracking and eye-hand coordination. The findings suggest that alterations to the sensory systems may lead to behavioral change in some children.     

Diagnosis and management of increased intracranial pressure

Increased intracranial pressure (ICP) is a pathological state common to a variety of neurological diseases, all of which are characterized by the addition of volume to the skull contents. Elevated ICP may lead to brain damage or death by two principle mechanisms: 1) global hypoxic-ischemic injury, as a consequence of reduced cerebral perfusion pressure (CPP) and cerebral blood flow; and 2) mechanical distortion and compression of brain tissue as a result of intracranial mass effect and ICP compartmentalization. All ICP therapies have as a goal, reduction of intracranial volume. In unmonitored patients with acute neurological deterioration, head elevation, hyperventilation, and mannitol (1g/kg) can rapidly lower ICP. Fluid-coupled ventricular catheters and fiberoptic transducers are the most accurate and reliable instruments for measuring ICP. In monitored patients, the treatment of critically raised ICP should proceed in an orderly step-wise fashion: 1) consideration of neuroimaging to exclude a new surgically operable lesion; 2) intravenous sedation to attain a quiet motionless state; 3) manipulation of blood pressure to keep CPP >70 and <120; 4) mannitol infusion; 5) moderate hyperventilation (P(CO2) 26 to 30 mmHg); and 6) high-dose pentobarbital therapy. Application of moderate hypothermia (32 to 33 degrees C) shows promise as a newer method for treating refractory ICP. Placement of an ICP monitor is the critical first step in management of ICP. Treatment is best done using a stepwise protocol, with careful attention to sedation and CPP control prior to using mannitol and hyperventilation.     

MRI of spinal hardware: comparison of conventional T1-weighted sequence with a new metal artifact reduction sequence

Objective. This study was designed to compare diagnostic quality of MR images of patients with spinal hardware acquired using a conventional T1-weighted spin-echo sequence and a new metal artifact reduction sequence (MARS). Conclusion. The new MARS sequence effectively reduces the degree of tissue-obscuring artifact produced by spinal fixation hardware and subjectively improves image quality compared with the conventional T1-weighted spin-echo sequence. Received: 30 May 2000 Revision requested: 12 September 2000 Revision received: 27 September 2000 Accepted: 27 November 2000