The patient is an 11-year old boy, who was born with universe alopecia as well as dry and coarse skin. When he was 3 months old, photophobia was noticed, and since then, upper respiratory tract infection had occurred twice a month complicated by frequent diarrhea. He had short stature with slight conjunc- rival congestion, corneal vascularization, opacity, coarseness and poor vision. No abnormality was found in the teeth, sweating ability, or hearing. He had universal alopecia; his skin was dry and rough with generalized rhombus- or polygon-shaped scaly patches. Particularly thick brown scales were observed on the upper limbs. Moreover, there were spiny follicular papules on the abdomen and axillae, hyperkeratosis of palm and sole, and dystrophic nails. Hyperextensibility of proximal interphalangeal joints of the third, fourth and fifth fingers was noticed. He also suffered from mental retardation, the verbal intelligence quotient being 52, performance intelligence quotient lower than 40, full intelligence quotient lower than 40, but no abnormality was found in the heart, lung, liver or spleen. Histopathology of skin on the abdomen suggested a change characteristic of ichthyosis. Chromosome analysis revealed a karyotype of 46, XY. This is the first diagnosed case of ichthyosis follicularis with atrichia and photophobia syndrome in China.

Objectlve To study the significance of injury and posthumous changes to the axonal in brain stem and provide evidence to the forensic discrimination of brainstem injury. Methods Select the cases that died of the primary brainstem injury or other disease randomly, All cases were divided into control group and experimental group, control group and experimental group that were autopsied at the postmortem interval less than 24h or 48h, or longer than 24h or 48h. The brainstem tissue were cut and stained by silver; Two hundred four samples were observed in microscope and selected 9～10 widest axonal to measure its diameter, then analysis the data. Results There is significant difference in axonal diameter of midbrain and mudella between control group (not including those cases died of cardiovascular diseases) and experiment group (P0.05). Conclusion The axonal swelling of the brainstem is closely related with the traumatic injury, so axonal swelling is a finding useful for identification of traumatic brainstem lesions, but death from cardiovascular diseases should be excluded.

OBJECTIVETo investigate the clinicopathological features and the differential diagnosis of poroma and porocarcinoma.

METHODSHistopathological characteristics and clinical data of 35 cases of poroma and 10 cases of porocarcinoma were analyzed retrospectively.

RESULTSThe average age of 35 patients of poroma was 48 years. The average age of 10 patients of porocarcinoma was 65 years. Both poroma and porocarcinoma occured most frequently on the scalp and face,as well as the extremities. Histologically, cases of poroma were divided into three subtypes, including classic poroma (23 cases), hidroacanthoma simplex (3 cases) and dermal duct tumor (9 cases). Residual foci of benign poroma were found in all cases of porocarcinoma, most of which were classic poroma. The malignant components showed severe dysplasia and/or stromal infiltration.

CONCLUSIONThe diagnosis of poroma and porocarcinoma is mainly based on the microscopic characteristics. An invasive architectural pattern and/or significant cytologic pleomorphism are the most important clues for the diagnosis of porocarcinoma. Neither focal mitotic activity nor the presence of necrosis was the diagnostic feature of porocarcinoma. Malignant transformation can occur in some cases of long existing poroma with recent, rapid tumor enlargement.

Aged ; Cell Transformation, Neoplastic ; Diagnosis, Differential ; Eccrine Porocarcinoma ; pathology ; Extremities ; Head and Neck Neoplasms ; pathology ; Humans ; Middle Aged ; Poroma ; pathology ; Retrospective Studies ; Scalp ; Skin Neoplasms ; pathology ; Sweat Gland Neoplasms ; pathology

Objective To investigate any anti-inflammatory and curative effect of hyperbaric oxygen (HBO) therapy on mice with acute lung injury (ALI) induced by lipopolysaccaride endotoxin. Methods Fortysix Sprague-Dawley rats, half male and half female, were randomly divided into a normal saline group with 10 rats,an LPS group with 12 rats, and an HBO group with 24 rats. LPS was injected via the tail vein at 5 mg/kg weight to establish the ALI model. The HBO group rats were treated with HBO. Then leucocyte counts, arterial blood gas analysis, interleukin-8 (IL-8) in the blood and lung wet/dry weight (W/D) were compared among the 3 groups at the 3rd and 6th hours. Pathological changes were examined under a light microscope. Results Compared with the LPS group, blood oxygen and W/D were higher in the HBO group, the white blood cell, neutrophil and IL-8 all were lower, and inflammatory cell infiltration was less in lung slides from the HBO group. Conclusions HBO therapy can improve oxygen deficiency and has an anti-inflammatory effect. As a result it might have protective effect against ALI.

Objective To investigate the real situation of the burden of the caregivers who take care of patients with spinal cord injury.Methods Using phenomenological research methods of qualitative study,information of 15 family caregivers of patients with spinal cord injury were collected through semi-structured interviews in Jiangxi province,and data were analyzed with Colaizzi 7-step analysis method.Results Four themes were summarized:weak social support system,heavy economic burden,insufficient care ability,mental and physical exhaustion; mental and physical exhaustion concluded three sub-themes:burnout,lifestyle changes,sad/helplessness.Conclusions Care burden of caregivers of patients with spinal cord injury includes four aspects:social,physical,psychological,and less caring capacity.Care workers should be concerned about the health status of the caregivers,give targeted care intervention,so that caregivers can be adapted to the caregivers' role,maintain and promote a high level of health of the patients and their families.

Objective To investigate the level of care burden and self efficacy of caregivers of patients with spinal cord injury,and analyze their correlation.Methods By convenience sampling method,150 cases of spinal cord injury patients and their caregivers were investigated with Zarit Caregiver Burden Scale and general self-efficacy scale.Results The score of care burden of caregivers with spinal cord injury was (52.91 ± 11.56) points,self-efficacy score of caregivers was (19.63 ± 4.85) points.The score of care burden of caregivers and self-efficacy was highly negatively correlated.Conclusions We should pay more attention to improve the self-efficacy of caregivers of patients with spinal cord injury (SCI),in order to mitigate the care burden,improve care ability and health level of the patients' families.

Objective Investigate the clinicopathological characteristics of h?rthle cell tumors of the thyroid. Methods Sixteen cases of HCT were studied by the clinicopathological study and immunohistochemical and review of the literature. Results There were 13 cases of h?rthle cell adenomas, 5 h?rthle cell follicular carcinomas. Immunostains showed that almost all benign and malignant HCT expressed TG, vimentin, S-100 protein. Conclusions HCT is a potential malignant tumor with a defined pathologic feature. It should be follow up for a longer time to their biological behaviors. To distinguish between the benign and the malignant depends on traditional encapsulation, and whether there is a blood vessel invasion or metastasis.

Objective To investigate the effects of Ginaton on acute lung injury (ALI) induced by lipopolysaccaride (LPS).Methods The acute lung injury rat model was induced by receiving 5 mg/kg LPS injection in tail vein.A total of 63 Wistar rats were divide into 3 groups without caring sex.Saline control group,LPS treated group and Ginaton treated group.The samples of different groups were collected 2 h,6 h,and 10 h after tail vein administration.Serum soluble cell ashesion molecule-1 (sICAM-1) was measured by ELISA,immunohistochemical staining and Western blotting of NF-kB were performed on sections of lung specimens.Results The acute lung injury rat model was successfully induced by receiving LPS injection in tail vein.The sICAM-1 levels increased more in Ginaton treated group than those in Saline control group (P＜0.01),and the increase in LPS treated group were the highest.By immunohistochemical staining,the positive NF-kB cells in Ginaton treated group were much less than those in LPS treated group (P＜0.01),and in Saline control group were least (P＜0.01).The results of the Western-blot method were consistent with immunohistochemical method.Conclusion ALI could be induced by LPS,Ginaton showed a protective effect through probably inhibiting activation of NF-kB on LPS-induced-ALI in this animal model.

Objective To investigate the safety and efficiency of China-made stent in patients with acute myocardial infarction(AMI).Methods Thirty-six patients with AMI were enrolled in our study,with mean age(57?9.7)years old.China-made stent were implanted successfully.Eighteen patients had acute anterior myocardial infarction,14 had acute inferior myocardial infarction,2 had acute lateral myocardial infarction,2 had acute posterior accompanied with right ventricular myocardial infarction.There were 12 patients with diabetes,20 patients with hypertension,18 patients with smoking.Results Thirty-seven China-made stents were implanted in the 36 patients.There were no complications during the procedure.All have acquired TIMI 3 flow.There were no thrombosis in the stent in 20 patients at the 7-day following up.Conclusion It is safe and efficient with the China-made stent implanted in patients with acute myocardial infarction.

Objective To develop allele specific oligonucleotide(ASO) -PCR assay based on TCR βgene rearrangements and provide a screening method for minimal residual disease (MRD) in adult patients with T-lineage acute lymphoblastic leukemia (T-ALL).Methods DNA samples from newly diagnosed 20 adult T-ALL patients were obtained.The TCR β gene rearrangements were detected by multiplex PCR,which included 38 paired of primers in 3 reaction tubes.Gel electrophoresis and two-color Gene Scanning was also applied for clonality analysis of TCR β followed by sequencing and subsequent blasting for monoclonal PCR products in four patients.ASO primers were designed based on the sequence of junction regions.MRD were detected in the bone marrow by RQ-PCR with ASO upstream primers, consensus Jβprobes and downstream primers.Results The detection rate of the clonal TCR β rearrangements was 85.0% (17/20).At least one complete Vβ-Jβ rearrangement could be detected at the time of diagnosis in 16 out of 17 patients(94.1%, 16/17).Incomplete Dβ-Jβ rearrangement could be detected in 7 patients (41.2% ,7/17).The positivitity rate of Vβ-Jβ to Dβ-Jβ was 2∶1 (94.1% versus 41.2% ).Two-color Gene Scanning analysis showed the Jβ2 family was used more frequently than the Jβ1 family (73% versus 27% ).The slopes of the standard curves ranged from - 3.60 to - 3.27.The correlation coefficients of all four standard curves were more than 0.99.The detection sensitivity of ASO-PCR was 4 × 10 -5 μg/μl.The fluorescence background were detected at a low level.Quantitative MRD values of TCR β rearrangement in sequential BM specimens of 4 adult T-ALL patients were monitored during the treatment, including complete remission after induction and after consolidation therapy. RQ-PCR showed the MRD values of TCR β rearrangement were gradually decreased in response to the treatment.Conclusions The quantification of TCR β rearrangement by ASO-PCR approach is sensitive, specific and reliable for the accurate evaluation of malignant clones.It is suitable for the monitoring of minimal residual disease of adult T-ALL patients.