A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

Occurrence of the t(2;5)(p23;q35) in non-Hodgkin's lymphoma

Primary CD30(Ki-1)-positive anaplastic large-cell lymphoma (ALCL) is considered by some to be a distinct clinicopathologic entity associated with the t(2;5) (p23;q35). However, the specificity of t(2;5) for ALCL has not been carefully studied. Therefore, we performed a detailed analysis of all cases of ALCL with abnormal cytogenetics results in the Nebraska Lymphoma Study Group registry, as well as all other cases of non-Hodgkin's lymphoma with t(2;5) in the registry. We found the t(2;5) in only five of 10 cases of ALCL, four of whom were young patients. However, we also found the t(2;5) in 11 other cases of nonanaplastic lymphoma, including eight children with typical peripheral T-cell lymphomas of various types. The t(2;5) was also found in three older adults with B-cell lymphomas of various types. Thus, the t(2;5) was not specific for CD30+ ALCL. However, t(2;5) may define a clinicopathologic entity in children and young adults characterized by variable morphologies with a T-cell or indeterminate phenotype, CD30-positivity, nodal disease with frequent extranodal involvement, advanced stage, and an excellent response to therapy, including bone marrow transplantation for relapsed disease. The clinical relevance of the t(2;5) in older patients requires further study.     

INVOLVEMENT OF NON-NMDA AND NMDA RECEPTORS IN GLUTAMATE-INDUCED PRESSOR OR DEPRESSOR RESPONSES OF THE PONS AND MEDULLA

1. Fifty-five intact and six baroreceptor denervated and vagotomized cats of either sex were anaesthetized intraperito-neally with urethane (400 mg/kg) and a-chloralose (40 mg/kg). Responses of the systemic arterial pressure (SAP), mean SAP (MSAP) and sympathetic vertebral nerve (VNA) and renal nerve activities (RNA) were recorded. 2. In intact animals, monosodium L-glutamate (Glu, 0.1 mol/L, 50 nL) was microinjected into pressor areas of the locus coeruleus (LC), gigantocellular tegmental field (GTF), rostral ventrolateral medulla (RVLM) and dorsomedial medulla (DM), and the depressor areas of caudal ventrolateral medulla (CVLM). The induced actions were compared before and after microinjection of either glutamate antagonists, glutamate diethylester (GDEE, 0.5 mol/L, 50–100nL), a competitive AMPA receptor blocker, or 2-amino-5-phosphonovaleric acid (D-AP5, 0.025 mol/L, 50–100 nL), a competitive N-methyl-D-aspartate (NMDA) receptor blocker. GDEE completely blocked the increases of SAP and VNA elicited from all pressor areas. D-AP5 only partially blocked the pressor but slightly blocked VNA and RNA responses from LC, GTF and DM, particularly those from RVLM. Neither GDEE nor D-AP5 blocked the depressor responses of SAP and two nerve activities elicited from CVLM. 3. In baroreceptor denervated animals, NMDA (2 mmol/L, 50–100 nL) and AMPA (0.2 mmol/L, 50–100 nL) were micro-injected into the same pressor areas of GTF, RVLM and DM and the depressor area of CVLM responsive to Glu activation (0.1 mol/L, 30 nL). In RVLM, DM and CVLM, the results of either NMDA or AMPA were similar to those induced by Glu. However, in GTF, microinjection of either NMDA or AMPA did not induce similar responses to Glu. This suggests that the nature of GTF may differ from RVLM and DM. 4. The above results suggest that the Glu-induced pressor responses from LC, GTF, DM and especially RVLM, are primarily mediated through AMPA receptors. The Glu-induced depressor responses from CVLM may not be predominantly mediated by either AMPA or NMDA receptors. 5. In both baroreceptor-intact and -denervated cats stimulation of the pressor areas often produced an increase of VNA and a decrease of RNA, while in the depressor CVLM decreased both VNA and RNA. The VNA, but not RNA were positively correlated with the pressor responses, while both VNA and RNA were positively correlated with the depressor responses. This may suggest that neurons of the sympathetic vertebral and renal nerves are topographically organized in the brain.     

Induced luteolysis in the primate: rapid loss of luteinizing hormone receptors

The molecular mechanisms involved in luteolysis are still unclear in the primate. This study aimed to investigate the effect of induced luteolysis on the ovarian luteinizing hormone (LH) receptor and the steroidogenic enzyme, 3beta-hydroxysteroid dehydrogenase (3beta-HSD) in the marmoset monkey. Luteolysis was induced in the mid-luteal phase either directly by systemic prostaglandin F2alpha (PGF2alpha), or indirectly by LH withdrawal using systemic gonadotrophin releasing hormone antagonist (GnRHant) treatment. The LH receptor was studied by isotopic mRNA in-situ hybridization and in-situ ligand binding and 3beta-HSD expression was studied using isotopic mRNA in-situ hybridization and immunohistochemistry. Induced luteolysis was associated with a reduction in the expression of LH receptor (P < 0.0001) and 3beta-HSD mRNA, closely followed by a reduction in the LH receptor (P < 0.05) and 3beta-HSD protein concentrations within 24 h. There were no differences in the findings whether luteolysis was induced with PGF2alpha or GnRHant. This study shows that disparate mechanisms to induce luteolysis in the primate result in an identical rapid loss of the LH receptor and 3beta-HSD. In conclusion, induced luteolysis leads to rapid loss of the steroidogenic pathway in luteal cells.      

Transdifferentiation of endothelial and renal tubular epithelial cells into myofibroblast-like cells under in vitro conditions: a morphological analysis

Renal fibrosis is a hallmark of progressive kidney disease and is characterized by an accumulation of extracellular-matrix-synthesizing cells in the glomerulus and tubulointerstitium. This population of myofibroblast-like cells (MFLC) is heterogeneous. It has been experimentally shown, for example, that tubular epithelial cells could change their phenotype into MFLC under certain circumstances, a process called epithelial-mesenchymal transdifferentiation. However, MFLC may also originate from other sources. Therefore, we examined whether endothelial cells (EDC) are able to transdifferentiate into MFLC in vitro. We compared potential differences between syngeneic tubular epithelial cells (EPC) and EDC during transdifferentiation into MFLC using bovine and porcine EDC isolated from pulmonary arteries, and glomerular capillaries. Renal tubular EPC were prepared from bovine renal cortical tissue by collagenase digestion and isolation from homogeneous cell monolayers. Bovine renal tubular EPC stained positive for cytokeratin. Furthermore, tubular EPC selectively incorporated labeled bovine serum albumin, a typical property of differentiated renal tubular cells. EDC were characterized by the absence of epithelial markers (e.g. cytokeratin), but stained positive for vWF. The transdifferentiation of EDC into MFLC occurs sequentially in two steps: First, by a rapid reversible transformation in postconfluent or clonal cultures without the need of cytokine stimulation and second, by a prolonged secondary step in the presence of the transformation-accelerating cytokines and the absence of adherently growing EDC. Thus, EDC that are able to sprout can also irreversibly transdifferentiate into MFLC. On the other hand, prolonged incubation of EPC in the presence of cytokines such as transforming growth factor-beta1 and tumor necrosis factor-alpha leads only to a very small number of MFLC without the ability to further proliferate. Our in vitro data suggest that EDC can more easily transdifferentiate into MFLC than syngeneic renal tubular EPC.     

The unfavorable effects of concomitant asthma and sleeplessness due to the atopic eczema/dermatitis syndrome (AEDS) on quality of life in subjects allergic to house-dust mites

BACKGROUND: Allergic rhinitis, asthma or the atopic eczema/dermatitis syndrome (AEDS) may independently impair quality of life in patients. However, although many allergic patients may suffer from more than one disorder, the effect of concomitant disease -- in particular, the impact of AEDS -- is largely unknown. As part of a large multicenter clinical trial on the efficacy of mattress casings in house-dust mite (HDM) allergy, generic quality of life in a mixed population of 224 subjects with rhinitis (n = 198) and/or asthma (n = 111) and/or AEDS (n = 64) was studied. The study aimed to estimate quality of life impairment in these atopic patients and to address the question/issue of whether one atopic disorder goes beyond other existing allergic diseases, thereby causing further impairment to quality of life. METHODS: Generic quality of life was assessed by SF-36. Quality of life in the atopic group was compared with a Dutch norm population. Multiple linear regression was used to determine the effects of disease (i.e. the presence of allergic rhinitis, asthma or AEDS) or disease severity, as assessed by visual analog scores (VAS) for asthma, rhinitis, VAS sleeplessness and VAS itching being considered as major symptoms in AEDS on SF-36 domains. RESULTS: Compared to the norm group, atopic patients were impaired in: physical functioning; role physical functioning; general health; vitality; and social functioning. The diagnosis of asthma was negatively associated with the SF-36 subscales for physical functioning (P = 0.02), and general health (P < 0.01). In line with these findings, asthma severity (VAS asthma) was negatively associated with physical functioning (P < 0.01), role physical functioning (P < 0.01), general health (P < 0.0.1), social functioning (P = 0.01), emotional functioning (P = 0.01), and vitality (P = 0.01). VAS sleeplessness had significant negative effect on role physical functioning (P < 0.01), bodily pain (P < 0.01), General health (P = 0.01), mental health (P < 0.01), social functioning (P < 0.01), and vitality (P < 0.01). In contrast, neither the diagnosis of allergic rhinitis or AEDS, nor VAS itching as an outcome parameter of AEDS, exerted additional effects on the SF-36 domains. CONCLUSIONS: Patients with atopic disease based on HDM allergy may have impaired quality of life. The majority of these patients have allergic rhinitis. The (co)existence of asthma, expressed in terms of diagnostic criteria or symptom severity, or the presence of sleep disorders as a consequence of AEDS, may further impair quality of life.     

The effect of encasings on quality of life in adult house dust mite allergic patients with rhinitis, asthma and/or atopic dermatitis

BACKGROUND: Environmental control has been put forward as an integral part of the management of house dust mite (HDM) allergy in sensitized patients. To validate this statement allergic disorders involved in HDM allergy--allergic asthma, rhinitis and atopic eczema/dermatitis syndrome (AEDS)--should be taken together and studied in terms of the efficacy of environmental control. Because a generic quality of life questionnaire exceeds the border of disease, this may be used as major outcome parameter. RESEARCH OBJECTIVE: To study the effects of bedding encasings in HDM allergic patients with asthma, rhinitis and AEDS. MATERIAL AND METHODS: A total of 224 adult HDM allergic patients with rhinitis and/or asthma and/or dermatitis were randomly allocated impermeable or nonimpermeable encasings for mattress, pillow and duvet. Short form 36 (SF-36) was filled in at baseline and after 12 months. Results: Lower physical (P = 0.01) and emotional (P < 0.001) sumscores were seen in females. Also, the presence of asthma resulted in lower physical sumscore (P = 0.01). However, no effect was seen of encasings on either sumscore. CONCLUSION: Bedding encasings do not improve quality of life in a mixed population of subjects with combinations with rhinitis, asthma and atopic dermatitis and sensitized to HDMs.