Chronic idiopathic intestinal pseudo-obstruction syndrome is a clinical condition induced by an impaired function of intestinal motility. Although its clinical symptoms are those of intestinal obstruction, mechanical obstruction of the intestine cannot be found by vigorous studies, even by operative exploration. We have experienced nine cases of chronic idiopathic intestinal pseudo-obstruction syndrome. It will help in diagnosis and treatment of the pseudo-obstruction to present the patients with these syndromes. In all cases severely delayed intestinal transit times were noted in radiological gastrointestinal studies without evidence of mechanical obstruction. The most frequent tentative diagnoses were congenital megacolons. But all of the patients showed the presence of ganglion cells in the intestinal wall. Histological abnormalities of the muscle layer were found in three patients. We could not resolve the symptoms by the surgical decompression of the intestine (such as colostomy, ileostomy etc.). But the symptoms were improved after total colectomy in a patient whose main affected site was confined to the colon. The megacystis was the most frequent combined anomaly. All patients had been improved with NPO and total parenteral nutrition therapies were inevitable. The drugs which facilitate the intestinal activities such as cisapride could not resolve the symptoms of obstruction. Three patients were died with the complications of total parenteral nutrition, and four patients improved clinically. Early diagnosis and proper management will reduce the mortality and morbidity.
Child ; Cisapride ; Colectomy ; Colon ; Colostomy ; Decompression, Surgical ; Diagnosis ; Early Diagnosis ; Ganglion Cysts ; Gastrointestinal Motility ; Hirschsprung Disease ; Humans ; Ileostomy ; Intestinal Obstruction ; Intestinal Pseudo-Obstruction* ; Intestines ; Mortality ; Parenteral Nutrition, Total

No abstract available.
Lymphangioma*

No abstract available.
Female ; Humans ; Virilism*

OBJECTIVETo investigate the effect of RNA interference by targeting human telomerase reverse transcriptase (hTERT) mRNA in the larynx cancer cell line, Hep-2.

METHODSThe primary structures of hTERT cDNA were found in GenBank. Then the structure analysis were done according to RNAi strategy which determined the specific base sequences to design shRNA plasmid. Two types of plasmid, pshRNA1 and pshRNA2, involved in fluorescein gene were synthesized based on the specific base sequences. Control pshRNA3, a random sequence, and control pshRNA4, without additional specific sequence were also constructed. Cells were treated daily with pshRNA1-4 or normal culture medium respectively. The pshRNA1-3 was identified by electrophoresis. After administration of pshRNA1-4, fluorescence expression was detected by confocal microscopy, the expression of hTERT of the transfected cells was determined by Western blotting, telomerase activity was measured by TRAP-PCR ELISA, cell viability was determined by MTT assay, morphological changes and apoptosis were examined by inverted microscope and TUNEL respectively.

RESULTSThere was a 400 bp balteum in pshRNA1-3 after cut by SalI, which was identical with the size of the objective gene. Many cells presented green fluorescence after being treated by pshRNA1-4, but there are much more dead green fluorescent cells in the pshRNA1 and pshRNA2 group. hTERT protein and telomerase activity was significantly decreased after treated by pshRNA1 or pshRNA2. It was observed that treatment with pshRNA1 or pshRNA2 in the presence of a valid transfection reagent could reduce cell viability of Hep-2 cells within 96 h (P < 0.01). Under the same culture conditions, cells grew more sparsely and the number of apoptotic cell increased significantly.

CONCLUSIONSshRNA plasmid directed against human telomerase reverse transcriptase can effectively transfect Hep-2 cells. shRNA targeted hTERT gene can significantly inhibit the growth and proliferation of Hep-2 cells, which results in apoptotic cell death. RNA interference may be a promising strategy for the treatment of laryngeal cancer.

Apoptosis ; Cell Line, Tumor ; Cell Proliferation ; Humans ; Laryngeal Neoplasms ; metabolism ; pathology ; Plasmids ; RNA Interference ; RNA, Messenger ; biosynthesis ; genetics ; RNA, Small Interfering ; Telomerase ; biosynthesis ; genetics ; Transfection

OBJECTIVETo evaluate the initial clinical characteristics, the response to treatment, and the outcome in adult patients with Evans syndrome.

METHODSThe clinical data of 84 adult patients (20 males, 64 females) with Evans syndrome diagnosed at our center between 1984 and 2007 were retrospectively analyzed.

RESULTSThe patients were followed up for a median duration of 17.5 (0.03 - 140) months. All the patients initially received intravenous steroids with or without intravenous immunoglobulin (IVIG). Forty-seven patients were treated with corticosteroids alone initially. Complete remission (CR) and partial remission (PR) were achieved in 38 of the patients, but 92.1% of them relapsed during a median follow-up of 12 months. Twenty-eight patients who were resistant to corticosteroids therapy or with severe bleeding were subsequently administered immunosupressive agents. CR and PR were obtained in 89.3% of them. Within a median follow-up of 8 months, 84% of these patients relapsed.

CONCLUSIONSEvans syndrome is a chronic and easy to recurrent disease, which is often refractory to conventional therapy. Treatment with combination agents might be a useful therapeutic approach to the patients.

Adrenal Cortex Hormones ; Adult ; Anemia, Hemolytic, Autoimmune ; Follow-Up Studies ; Humans ; Remission Induction ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo promote the awareness of common variable immunodeficiency (CVID).

METHODSReport two cases of CVID and review related literature.

RESULTSThe two CVID patients were manifested recurrent infections of respiratory and digestive tract, splenomegaly, significantly reduced serum immunoglobulin, since adolescence. They were treated with intravenous gammaglobulin, anti-infection and symptomatic therapies and the diseases were well controlled. Case 1 was diagnosed as with spleen peripheral T cell lymphoma after splenectomy. Case 2 developed nutritional megaloblastic anemia resulting from repeated digestive tract infections.

CONCLUSIONCVID is a heterogeneous group of diseases with later-onset, characterized by hypogammaglobulinemia and recurrent bacterial infections. Early diagnosis and regular treatment may improve the prognosis.

Common Variable Immunodeficiency ; drug therapy ; physiopathology ; Female ; Humans ; Male ; Middle Aged ; Young Adult

Objective To explore the clinical characteristics and treatment strategy of chronic low-er limb arterial ischemic. Methods The clinical data of 60 patients with chronic lower limb arterial ische-mic-treated from January 2017 to May 2017 were analyzed retrospectively (63 affected limbs). According to the patient's physical condition, clinical symptoms and pathological features, individualized surgical treat-ment was selected, including autogenous great saphenous vein or artificial blood vessel bypass grafting, ar-tery intima stripped, balloon dilatation, stenting, intracavity volume reduction, or hybrid procedures. The ankle brachial index of preoperative and postoperative 3 months was monitored, and patients were classified according to the Rutherford classification standard. Results Sixty cases were underwent surgery treatment, three patients were treated with bilateral lower limbs in the same period, successfully 58 cases and success rate reached 96. 67％. One case was subjected to amputation, and 1 case died (cardiovascular disease). Postoperative 3 month follow-up, the Rutherford grading of 59 affected limbs was improved markedly, aver-age ankle brachial index of postoperative was higher than preoperative ( P<0. 05 ) . Six cases of patients with ulcer were cured, the ulcer surface of 2 cases were significantly narrowed. Conclusions The inci-dence of chronic lower limb arterial ischemic is increasing year by year, the treatment ideas are constantly updated. It is safe and effective to select individualized treatment according to the patients' physical condi-tion, clinical symptoms and pathological feature. However it still needs to enlarge the sample and extend the time of follow-up to verify this point.

PURPOSE: To perform an economic evaluation of the different treatment methods available for primary open-angle glaucoma in a Korean setting, including medication, selective laser trabeculoplasty, or surgery. METHODS: Three independent Markov chains were constructed for each treatment option to simulate treatment progress and to evaluate the total treatment costs for each initial strategy. The Markov chain consisted of different stages (5, 10, 20 stages), with each stage being one year. Assuming 1000 patients, a Monte Carlo simulation was iterated 1000 times to evaluate the cost of treatment over 5, 10 and 20 years. RESULTS: During the initial five years, medication as the initial treatment was the most expensive, whereas laser trabeculoplasty was the cheapest. After ten years, surgery became the cheapest treatment. In ten years, if the success rate of surgery is greater than 30.1%, it was more economic to choose surgery as the initial treatment. For laser trabeculoplasty, if the success rate was greater than 16.3%, laser treatment was more economical than was medication. Our model shows that only if the annual cost of medication decreases to 60,000 won or 55,000 won, then the cost of choosing medication as the initial treatment strategy will be more economical than that of laser therapy or surgery, respectively. CONCLUSIONS: The economic value of choosing laser therapy as the initial treatment strategy is the greatest over five simulated-years, whereas surgery had the greatest economic value over more than ten years.
Glaucoma ; Glaucoma, Open-Angle ; Health Care Costs ; Humans ; Laser Therapy ; Markov Chains ; Trabeculectomy

Objective:To explore the role of transgelin(TAGLN) in the occurrence and development of papillary thyroid carcinoma (PTC) and its possible signal pathway.Methods:One hundred cases of PTC tissues and corresponding paracancerous normal thyroid tissues were collected. Realtime quantitative PCR (RT-qPCR), Western blotting, and immunohistochemistry were used to analyze the expression of TAGLN in PTC tissues and corresponding paracancerous normal thyroid tissues. PTC cells were transfected with plasmid and shRNA lentivirus vector respectively to up-regulate or down-regulate the expression of TAGLN in order to detect the effects of them on the proliferation, invasion, and migration by cell proliferation assay(cell counting kit-8, CCK-8)and cell invasion and migration assays (Transwell). The effects of TAGLN on mitogen-activated protein kinase (MAPK)/extracellular-signal regulating kinase (ERK) signal pathway was detected with Western blotting.Results:RT-qPCR showed that there was no difference in the expression of TAGLN mRNA between PTC and corresponding paracancerous normal thyroid tissues ( P>0.05); Western blotting demonstrated that the expression of TAGLN protein in PTC tissues was significantly lower than that in corresponding paracancerous normal thyroid tissues ( P<0.01). Immunohistochemical results revealed that the expression of TAGLN in PTC tissues was significantly lower than that in corresponding paracancerous normal thyroid tissues. Overexpression of TAGLN inhibited the proliferation, invasion, and migration of PTC cells ( P<0.01), but knockdown of TAGLN promoted the proliferation, invasion, and migration of PTC cells ( P<0.01). Overexpression of TAGLN decreased the expression of phosphorylated ERK ( P<0.05), whereas silencing TAGLN increased phosphorylated ERK level in PTC cells( P<0.01). Conclusion:The expression of TAGLN in PTC is significantly decreased. It is related to the occurrence and development of PTC, and its mechanism may be related to MAPK/ERK signal pathway.

OBJECTIVETo report 7 cases of acquired hemoglobin H in myelodysplastic syndromes.

CASE DATA AND DISCUSSIONClinical materials of the 7 cases were retrospectively presented. Clinical features of the similar cases in literatures were reviewed. The criteria for diagnosis of this entity by Steensma and its pathogenesis were discussed.

CONCLUSIONThis entity is a new subtype of MDS with unique clinical features and pathogenesis, and might be a proper model in the study of MDS transformation.

Adult ; Female ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes ; complications ; alpha-Thalassemia ; complications