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Sample records for acquired adamts13 deficiency

  1. Acquired Idiopathic ADAMTS13 Activity Deficient Thrombotic Thrombocytopenic Purpura in a Population from Japan

    Matsumoto, Masanori; Bennett, Charles L.; Isonishi, Ayami; Qureshi, Zaina; Hori, Yuji; Hayakawa, Masaki; Yoshida, Yoko; Yagi, Hideo; Fujimura, Yoshihiro

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai) ADAMTS13-deficient TTP (ADAMTS13 activity <5%) were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01), while older patients were more likely to die during the TTP hospitalization (p<0.05). Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort) and gender composition (60% to 100% female in the other cohort). We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed. PMID:22427934

  2. Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan.

    Masanori Matsumoto

    Full Text Available Thrombotic thrombocytopenic purpura (TTP is a type of thrombotic microangiopathy (TMA. Studies report that the majority of TTP patients present with a deficiency of ADAMTS13 activity. In a database of TMA patients in Japan identified between 1998 and 2008, 186 patients with first onset of acquired idiopathic (ai ADAMTS13-deficient TTP (ADAMTS13 activity <5% were diagnosed. The median age of onset of TTP in this group of patients was 54 years, 54.8% were female, 75.8% had renal involvement, 79.0% had neurologic symptoms, and 97.8% had detectable inhibitors to ADAMTS13 activity. Younger patients were less likely to present with renal or neurologic dysfunction (p<0.01, while older patients were more likely to die during the TTP hospitalization (p<0.05. Findings from this cohort in Japan differ from those reported previously from the United States, Europe, and Korea with respect to age at onset (two decades younger in the other cohort and gender composition (60% to 100% female in the other cohort. We conclude that in one of the largest cohorts of ai-TTP with severe deficiency of ADAMTS13 activity reported to date, demographic characteristics differ in Japanese patients relative to those reported from a large Caucasian registry from Western societies. Additional studies exploring these findings are needed.

  3. Acquired TTP: ADAMTS13 meets the immune system

    N. Sorvillo

    2013-01-01

    Autoantibodies directed against ADAMTS13 prohibit the processing of VWF multimers, initiating a rare and life-threatening disorder called acquired thrombotic thrombocytopenic purpura (TTP). At present it is not clear why previously healthy individuals develop anti-ADAMTS13 antibodies. The studies de

  4. Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura.

    Deforche, Louis; Tersteeg, Claudia; Roose, Elien; Vandenbulcke, Aline; Vandeputte, Nele; Pareyn, Inge; De Cock, Elien; Rottensteiner, Hanspeter; Deckmyn, Hans; De Meyer, Simon F; Vanhoorelbeke, Karen

    2016-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy linked to a deficiency in the metalloprotease ADAMTS13. In the current study, a novel mouse model for acquired TTP was generated to facilitate development and validation of new therapies for this disease. Therefore, a large panel (n = 19) of novel anti-mouse ADAMTS13 (mADAMTS13) monoclonal antibodies (mAbs) of mouse origin was generated. Inhibitory anti-mADAMTS13 mAbs were identified using the FRETS-VWF73 assay. Four mAbs strongly inhibited mADAMTS13 activity in vitro (∼68-90% inhibition). Injecting a combination of 2 inhibitory mAbs (13B4 and 14H7, 1.25 mg/kg each) in Adamts13+/+ mice resulted in full inhibition of plasma ADAMTS13 activity (96 ± 4% inhibition, day 1 post injection), leading to the appearance of ultra-large von Willebrand factor (UL-VWF) multimers. Interestingly, the inhibitory anti-mADAMTS13 mAbs 13B4 and 14H7 were ideally suited to induce long-term ADAMTS13 deficiency in Adamts13+/+ mice. A single bolus injection resulted in full ex vivo inhibition for more than 7 days. As expected, the mice with the acquired ADAMTS13 deficiency did not spontaneously develop TTP, despite the accumulation of UL-VWF multimers. In line with the Adamts13-/- mice, TTP-like symptoms could only be induced when an additional trigger (rVWF) was administered. On the other hand, the availability of our panel of anti-mADAMTS13 mAbs allowed us to further develop a sensitive ELISA to detect ADAMTS13 in mouse plasma. In conclusion, a novel acquired TTP mouse model was generated through the development of inhibitory anti-mADAMTS13 mAbs. Consequently, this model provides new opportunities for the development and validation of novel treatments for patients with TTP. In addition, these newly developed inhibitory anti-mADAMTS13 mAbs are of great value to specifically study the role of ADAMTS13 in mouse models of thrombo-inflammatory disease. PMID:27479501

  5. Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura

    Zheng, X. Long; Wu, Haifeng M.; Shang, Dezhi; Falls, Erica; Skipwith, Christopher G.; Cataland, Spero R.; Bennett, Charles L.; Kwaan, Hau C.

    2010-01-01

    Background Type G immunoglobulins against ADAMTS13 are the primary cause of acquired (idiopathic) thrombotic thrombocytopenic purpura. However, the domains of ADAMTS13 which the type G anti-ADAMT13 immunoglobulins target have not been investigated in a large cohort of patients with thrombotic thrombocytopenic purpura. Design and Methods Sixty-seven patients with acquired idiopathic thrombotic thrombocytopenic purpura were prospectively collected from three major U.S. centers. An enzyme-linked immunosorbent assay determined plasma concentrations of anti-ADAMTS13 type G immunoglobulins, whereas immunoprecipitation plus western blotting determined the binding domains of these type G immunoglobulins. Results Plasma anti-ADAMTS13 type G immunoglobulins from 67 patients all bound full-length ADAMTS13 and a variant truncated after the eighth TSP1 repeat (delCUB). Approximately 97% (65/67) of patients harbored type G immunoglobulins targeted against a variant truncated after the spacer domain (MDTCS). However, only 12% of patients’ samples reacted with a variant lacking the Cys-rich and spacer domains (MDT). In addition, approximately 37%, 31%, and 46% of patients’ type G immunoglobulins interacted with the ADAMTS13 fragment containing TSP1 2-8 repeats (T2-8), CUB domains, and TSP1 5-8 repeats plus CUB domains (T5-8CUB), respectively. The presence of type G immunoglobulins targeted against the T2-8 and/or CUB domains was inversely correlated with the patients’ platelet counts on admission. Conclusions This multicenter study further demonstrated that the multiple domains of ADAMTS13, particularly the Cys-rich and spacer domains, are frequently targeted by anti-ADAMTS13 type G immunoglobulins in patients with acquired (idiopathic) thrombotic thrombocytopenic purpura. Our data shed more light on the pathogenesis of acquired thrombotic thrombocytopenic purpura and provide further rationales for adjunctive immunotherapy. PMID:20378566

  6. Pathogenicity of Anti-ADAMTS13 Autoantibodies in Acquired Thrombotic Thrombocytopenic Purpura

    Mari R. Thomas

    2015-08-01

    Conclusions: Anti-spacer domain autoantibodies are the major inhibitory antibodies in acquired TTP. However, depletion of ADAMTS13 antigen (rather than enzyme inhibition is a dominant pathogenic mechanism. ADAMTS13 antigen levels at presentation have prognostic significance. Taken together, our results provide new insights into the pathophysiology of acquired TTP.

  7. ADAMTS13 Deficiency and Thrombotic Thrombocytopenic Purpura Associated with Trimethoprim-Sulfamethoxazole

    Bapani, Sowjanya; Epperla, Narendranath; Kasirye, Yusuf; Mercier, Richard; Garcia-Montilla, Romel

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a hematological disease characterized by microangiopathic hemolytic anemia and thrombocytopenia. Although the link between ADAMTS13 deficiency and idiopathic TTP has been well-established, the role of trimethoprim-sulfamethoxazole (TMP-SMX) in the pathogenesis of TTP is not yet well elucidated. To the best of our knowledge, there have been only two previous reports linking this medication with the development of TTP. We present the case of a health...

  8. Characterization of human antibodies against ADAMTS13 that develop in patients with acquired TTP

    Pos, W.

    2010-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a thrombotic micro-angiopathy characterized by the absence or dysfunction of the von Willebrand factor cleaving protease ADAMTS13. Functional absence of ADAMTS13 results in impaired cleavage of newly released ultra large von Willebrand factor (UL-VWF) mul

  9. ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura

    Manea, Minola; Kristoffersson, AnnCharlotte; Tsai, Han-Mou; Zhou, Wenhua; Winqvist, Ingemar; Oldaeus, Göran; Billström, Rolf; Björk, Peter; Holmberg, Lars; Karpman, Diana

    2006-01-01

    The activity of ADAMTS13, the von Willebrand factor cleaving protease, is deficient in patients with thrombotic thrombocytopenic purpura (TTP). In the present study, the phenotype of ADAMTS13 in TTP and in normal plasma was demonstrated by immunoblotting. Normal plasma (n = 20) revealed a single band at 190 kD under reducing conditions using a polyclonal antibody, and a single band at 150 kD under non-reducing conditions using a monoclonal antibody. ADAMTS13 was not detected in the plasma fro...

  10. The expression of ADAMTS13 in human microvascular endothelial cells.

    Wang, Anyou; Duan, Qiaohong; Wu, Jingsheng; Liu, Xin; Sun, Zimin

    2016-06-01

    ADAMTS13, as a specific von Willebrand factor (VWF)-cleaving protease, prevents microvascular thrombosis of VWF/platelet thrombi. It has been reported that human vascular endothelial cells could also synthesize and secrete ADAMTS13, and these reports were focused in human umbilical vascular endothelial cells. Considering the particularity of its huge quantity and structure of human microvascular endothelial cells (HMECs) in the body, whether ADAMTS13 is expressed in HMECs also needs to be confirmed. To investigate whether ADAMTS13 is expressed in HMECs. Real-time PCR (RT-PCR) amplification detected ADAMTS13 mRNA in HMEC-1 cell line. The expression and distribution of ADAMTS13 protein and VWF were detected by fluorescence immunoassay and western blot. We observed the expression and distribution of ADAMTS13 in HMECs. We confirmed the expression of ADAMTS13 mRNA in HMEC-1, and found that there were some partly common distributions of ADAMTS13 protein and VWF. This study provides the evidence that HMECs also express ADAMTS13. HMECs might also be a primary source for human plasma ADAMTS13. The overlap region for the distribution of ADAMTS13 and VWF suggests that ADAMTS13 might have a potential regulation role for VWF inside cells. PMID:26366828

  11. [Construction of ADAMTS13-pEGFP-N1 vector and its expression in HeLa cells].

    Ling, Jing; Ma, Zhen-Ni; Su, Jian; Ruan, Chang-Geng

    2013-02-01

    This study was aimed to construct a pEGFP-N1 vector of von Willebrand factor cleaving protease (ADAMTS13, a disintegrin and metalloprotease with a thrombospondin type 1 motifs 13) so as to pave the way for further studying its synthesis and secretion. Human full-length cDNA sequence of ADAMTS13 was acquired by polymerase chain reaction (PCR) with Phusion(®) High-Fidelity (NEB), then the PCR product was double digested with EcoRI and XhoI. After digestion, the ADAMTS13 cDNA sequence was purified and recombined with the pEGFP-N1 vector. The DNA sequence analysis showed that ADAMTS13 was ligated to the pEGFP-N1 vector correctly. After transient expression in HeLa cells, the expression of EGFP could be detected by fluorescent microscopy, and the expression of ADAMTS13 protein could be detected by SDS-PAGE and Western blot. It is concluded that the ADAMTS13-pEGFP-N1 vector is successfully constructed, and it can be widely used in further research on the mechanism of the synthesis and secretion of ADAMTS13. PMID:23484705

  12. Thrombotic thrombocytopenic purpura: The role of ADAMTS13.

    Rogers, Heesun J; Allen, Charles; Lichtin, Alan E

    2016-08-01

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease requiring prompt diagnosis and initiation of therapeutic plasma exchange to improve patient survival. However, diagnosis is often difficult because of atypical presentations and signs and symptoms that resemble other conditions. Measurements of ADAMTS13 activity, ADAMTS13 inhibitor, and ADAMTS13 autoantibody are useful for diagnosing TTP, guiding therapy, and predicting relapse. PMID:27505881

  13. The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura

    Lancellotti, S.

    2015-08-13

    Congenital thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy, inherited with autosomal recessive mode as a dysfunction or severe deficiency of ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin 1 repeats Nr. 13), caused by mutations in the ADAMTS-13 gene. About 100 mutations of the ADAMTS-13 gene were identified so far, although only a few characterised by in vitro expression studies. A new Asp to Gly homozygous mutation at position 173 of ADAMTS-13 sequence was identified in a family of Romanian origin, with some members affected by clinical signs of TTP. In two male sons, this mutation caused a severe (< 3 %) deficiency of ADAMTS-13 activity and antigen level, associated with periodic thrombocytopenia, haemolytic anaemia and mild mental confusion. Both parents, who are cousins, showed the same mutation in heterozygous form. Expression studies of the mutant ADAMTS-13, performed in HEK293 cells, showed a severe decrease of the enzyme’s activity and secretion, although the protease was detected inside the cells. Molecular dynamics found that in the D173G mutant the interface area between the metalloprotease domain and the disintegrin-like domain significantly decreases during the simulations, while the proline-rich 20 residues linker region (LR, 285–304) between them undergoes extensive conformational changes. Inter-domain contacts are also significantly less conserved in the mutant compared to the wild-type. Both a decrease of the inter-domain contacts along with a substantial conformational rearrangement of LR interfere with the proper maturation and folding of the mutant ADAMTS-13, thus impairing its secretion.

  14. A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13.

    Meyer, Sara C; Jeddi, Ramzi; Meddeb, Balkis; Gouider, Emna; Lämmle, Bernhard; Kremer Hovinga, Johanna A

    2008-08-01

    Hereditary thrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by occlusive microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia. Homozygous or compound heterozygous mutations in the ADAMTS13 gene result in a congenital severe ADAMTS13 deficiency and subsequent accumulation of ultra-large von Willebrand factor multimers, which tend to form platelet thrombi in the microcirculation. We report a first case of congenital TTP on the African continent with a new, homozygous mutation in the metalloprotease domain of ADAMTS13. An initially oligo-symptomatic presentation was followed by acute exacerbation with ischemic stroke and acute renal failure highlighting the severity of this syndrome. PMID:18443791

  15. A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

    Risa Yamada

    2011-01-01

    Full Text Available Thrombotic thrombocytopenia purpura (TTP caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA. Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE. In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.

  16. ADAMTS13, lucky to have a hydrophobic pocket

    Zheng, X. Long

    2015-01-01

    In this issue of Blood, de Groot et al identify a hydrophobic pocket in the Cys-rich domain of ADAMTS13 that appears to interact with the hydrophobic pocket in the central A2 domain of von Willebrand factor (VWF) for its proteolysis.1

  17. Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis

    Lin, Jainn-Jim; Chan, Oi-Wa; Hsiao, Hsiang-Ju; Wang, Yu; Hsia, Shao-Hsuan; Chiu, Cheng-Hsun

    2016-01-01

    Abstract Decreased ADAMTS 13 activity has been reported in severe sepsis and in sepsis-induced disseminated intravascular coagulation. This study aimed to investigate the role of ADAMTS 13 in different pediatric sepsis syndromes and evaluate its relationship with disease severity and outcome. We prospectively collected cases of sepsis treated in a pediatric intensive care unit, between July 2012 and June 2014 in Chang Gung Children's Hospital in Taoyuan, Taiwan. Clinical characteristics and ADAMTS-13 activity were analyzed. All sepsis syndromes had decreased ADAMTS 13 activity on days 1 and 3 of admission compared to healthy controls. Patients with septic shock had significantly decreased ADAMTS 13 activity on days 1 and 3 compared to those with sepsis and severe sepsis. There was a significant negative correlation between ADAMTS 13 activity on day 1 and day 1 PRISM-II, PELOD, P-MOD, and DIC scores. Patients with mortality had significantly decreased ADAMTS 13 activity on day 1 than survivors, but not on day 3. Different pediatric sepsis syndromes have varying degrees of decreased ADAMTS 13 activity. ADAMTS 13 activity is strongly negatively correlated with disease severity of pediatric sepsis syndrome, whereas decreased ADAMTS 13 activity on day 1 is associated with increased risk of mortality. PMID:27100422

  18. Association of ADAMTS13 polymorphism with cerebral malaria

    Kraisin Sirima

    2011-12-01

    Full Text Available Abstract Background Cerebral malaria is one of the most severe manifestations of Plasmodium falciparum malaria. The sequestration of parasitized red blood cells (PRBCs to brain microvascular endothelium has been shown to contribute to the pathophysiology of cerebral malaria. Recent studies reported increased levels of von Willebrand factor (VWF and reduced activity of VWF-cleaving protease, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, in patients with cerebral malaria. Methods Association of six single nucleotide polymorphisms (SNPs of the ADAMTS13 gene with cerebral malaria was examined in 708 Thai patients with P. falciparum malaria. Results Among six SNPs, the derived allele of a SNP located in intron 28, rs4962153-A, was significantly associated with protection against cerebral malaria when 115 cerebral malaria patients were compared with 367 mild malaria patients (Fisher's exact P-value = 0.0057; OR = 0.27; 95% CI = 0.096-0.76. Significant association was also detected between 115 cerebral malaria and 593 non-cerebral malaria (226 non-cerebral severe malaria and 367 mild malaria patients (Fisher's exact P-value = 0.012; OR = 0.30; 95% CI = 0.11-0.83. Conclusions Excessive adhesion of PRBCs to the platelet-decorated ultra-large VWF (ULVWF appears to enhance the sequestration of PRBCs to cerebral microvascular endothelium. The genetic association observed in the present study implies that the regulation of platelet-decorated ULVWF strings by ADAMTS13 may play a role in the development of cerebral malaria.

  19. ADAMTS-13 deficiency following Hemiscorpius lepturus scorpion sting

    Ehsan Valavi

    2011-01-01

    Full Text Available Hemiscorpius lepturus is a lethal scorpion with potentially cytotoxic venom. Various degrees of local and systemic toxicity have been observed after its envenomation ranging from local erythema to disseminated intravascular coagulation, renal failure and severe pulmonary hemorrhage. In this case report, we report on a seven-year-old patient who developed the hemolytic uremic syndrome (HUS after being stung by the scorpion H. lepturus. This condition is characterized by microangiopathic hemolytic anemia, thrombocytopenia, increased serum levels of lactate dehydrogenase and uremia. We evaluated the causes of HUS and found that the levels of C3, C4, CH50 and H factors were normal, but the activity of Von Willebrand factor cleaving protease was decreased (less than 5% of the normal activity. The patient improved after administering therapy with plasma exchange.

  20. ADAMTS13-binding IgG are present in patients with thrombotic thrombocytopenic purpura

    Tsai, Han-Mou; Raoufi, Mojgan; Zhou, Wenhua; Guinto, Enriqueta; Grafos, Nickolas; Ranzurmal, Safi; Greenfield, Robert S.; Rand, Jacob H.

    2006-01-01

    Functional assays are commonly used to measure the antibodies of ADAMTS13 found in patients of thrombotic thrombocytopenic purpura (TTP). In this study we used an enzyme-linked immunoassay to analyze the ADAMTS13-binding IgG levels in six groups of individuals: normal, random hospitalized patients, acute TTP,TTP after receiving plasma therapy, TTP in remission, and other types of thrombotic microangiopathy (TMA). The results showed thatADAMTS13-binding IgG levels were elevated in 100% of the ...

  1. Atypical presentations of thrombotic thrombocytopenic purpura: a diagnostic role for ADAMTS13.

    Kalish, Yosef; Rottenstreich, Amihai; Rund, Deborah; Hochberg-Klein, Sarit

    2016-08-01

    Thrombotic thrombocytopenic purpura (TTP) is an acute, life threatening disease. Only a minority of patients expresses the complete clinical presentation and unusual manifestations can occur. Demonstration of low activity levels of ADAMTS13 (importance of having a high clinical suspicion of TTP in cases of thrombosis even without hematological abnormalities in patients with previous attacks of TTP. In this clinical scenario, measurement of ADAMTS13 activity is important for diagnosis and early administration of treatment. PMID:26867546

  2. Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease

    Changes in plasma von Willebrand factor concentration (VWF:Ag) and ADAMTS-13 activity (the metalloprotease that cleaves VWF physiologically) have been reported in several cardiovascular disorders with prognostic implications. We therefore determined the level of these proteins in the plasma of children with cyanotic congenital heart disease (CCHD) undergoing surgical treatment. Forty-eight children were enrolled (age 0.83 to 7.58 years). Measurements were performed at baseline and 48 h after surgery. ELISA, collagen-binding assays and Western blotting were used to estimate antigenic and biological activities, and proteolysis of VWF multimers. Preoperatively, VWF:Ag and ADAMTS-13 activity were decreased (65 and 71% of normal levels considered as 113 (105-129) U/dL and 91 ± 24% respectively, P < 0.003) and correlated (r = 0.39, P = 0.0064). High molecular weight VWF multimers were not related, suggesting an interaction of VWF with cell membranes, followed by proteolytic cleavage. A low preoperative ADAMTS-13 activity, a longer activated partial thromboplastin time and the need for cardiopulmonary bypass correlated with postoperative bleeding (P < 0.05). Postoperatively, ADAMTS-13 activity increased but less extensively than VWF:Ag (respectively, 2.23 and 2.83 times baseline, P < 0.0001), resulting in an increased VWF:Ag/ADAMTS-13 activity ratio (1.20 to 1.54, respectively, pre- and postoperative median values, P = 0.0029). ADAMTS-13 consumption was further confirmed by decreased ADAMTS-13 antigenic concentration (0.91 ± 0.30 to 0.70 ± 0.25 µg/mL, P < 0.0001) and persistent proteolysis of VWF multimers. We conclude that, in pediatric CCHD, changes in circulating ADAMTS-13 suggest enzyme consumption, associated with abnormal structure and function of VWF

  3. Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease

    Soares, R.P.S. [Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, SP (Brazil); Bydlowski, S.P.; Nascimento, N.M. [Laboratório de Investigação Médica-31, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Thomaz, A.M.; Bastos, E.N.M.; Lopes, A.A. [Faculdade de Medicina, Instituto do Coração, Universidade de São Paulo, São Paulo, SP (Brazil)

    2013-04-05

    Changes in plasma von Willebrand factor concentration (VWF:Ag) and ADAMTS-13 activity (the metalloprotease that cleaves VWF physiologically) have been reported in several cardiovascular disorders with prognostic implications. We therefore determined the level of these proteins in the plasma of children with cyanotic congenital heart disease (CCHD) undergoing surgical treatment. Forty-eight children were enrolled (age 0.83 to 7.58 years). Measurements were performed at baseline and 48 h after surgery. ELISA, collagen-binding assays and Western blotting were used to estimate antigenic and biological activities, and proteolysis of VWF multimers. Preoperatively, VWF:Ag and ADAMTS-13 activity were decreased (65 and 71% of normal levels considered as 113 (105-129) U/dL and 91 ± 24% respectively, P < 0.003) and correlated (r = 0.39, P = 0.0064). High molecular weight VWF multimers were not related, suggesting an interaction of VWF with cell membranes, followed by proteolytic cleavage. A low preoperative ADAMTS-13 activity, a longer activated partial thromboplastin time and the need for cardiopulmonary bypass correlated with postoperative bleeding (P < 0.05). Postoperatively, ADAMTS-13 activity increased but less extensively than VWF:Ag (respectively, 2.23 and 2.83 times baseline, P < 0.0001), resulting in an increased VWF:Ag/ADAMTS-13 activity ratio (1.20 to 1.54, respectively, pre- and postoperative median values, P = 0.0029). ADAMTS-13 consumption was further confirmed by decreased ADAMTS-13 antigenic concentration (0.91 ± 0.30 to 0.70 ± 0.25 µg/mL, P < 0.0001) and persistent proteolysis of VWF multimers. We conclude that, in pediatric CCHD, changes in circulating ADAMTS-13 suggest enzyme consumption, associated with abnormal structure and function of VWF.

  4. Acquired Zinc Deficiency in an Adult Female

    Mohanan Saritha; Divya Gupta; Laxmisha Chandrashekar; Devinder M Thappa; Nachiappa G Rajesh

    2012-01-01

    Acrodermatitis enteropathica is an autosomal recessive inherited disorder of zinc absorption. Acquired cases are reported occasionally in patients with eating disorders or Crohn′s disease. We report a 24-year-old housewife with acquired isolated severe zinc deficiency with no other comorbidities to highlight the rare occurrence of isolated nutritional zinc deficiency in an otherwise normal patient.

  5. PROTEOLYTIC PROCESSING OF VON WILLEBRAND FACTOR BY ADAMTS13 AND LEUKOCYTE PROTEASES

    Stefano Lancellotti

    2013-09-01

    Full Text Available ADAMTS13 is a 190 kDa zinc protease encoded by a gene located on chromosome 9q34.   This protease specifically hydrolyzes von Willebrand factor (VWF multimers, thus causing VWF size reduction. ADAMTS13 belongs to the A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats (ADAMTS family, involved in proteolytic processing of many matrix proteins. ADAMTS13 consists of numerous domains including a metalloprotease domain, a disintegrin domain, several thrombospondin type 1 (TSP1 repeats, a cysteine-rich domain, a spacer domain and 2 CUB (Complement c1r/c1s, sea Urchin epidermal growth factor, and Bone morphogenetic protein domains. ADAMTS13 cleaves a single peptide bond (Tyr1605-Met1606 in the central A2 domain of the VWF molecule. This proteolytic cleavage is essential to reduce the size of ultra-large VWF polymers, which, when exposed to high shear stress in the microcirculation, are prone to form with platelets clumps, which cause severe syndromes called thrombotic microangiopathies (TMAs. In this review, we a discuss the current knowledge of structure-function aspects of ADAMTS13 and its involvement in the pathogenesis of TMAs, b address the recent findings concerning proteolytic processing of VWF multimers by different proteases, such as the leukocyte-derived serine and metallo-proteases and c indicate the direction of future investigations

  6. Increased expressions of ADAMTS-13 and apoptosis contribute to neuropathology during Toxoplasma gondii encephalitis in mice.

    Dincel, Gungor Cagdas; Atmaca, Hasan Tarik

    2016-06-01

    Toxoplasma gondii (T. gondii) is a protozoan parasite with the potential of causing severe encephalitis among immunocompromised humans and animals. Our previous study showed that T. gondii induces high nitric oxide (NO) production, high glial activation (GFAP) and neurofilament expressions, leading to severe neurodegeneration in toxoplasma encephalitis (TE) in the central nervous system (CNS). The aim of this experimental study was to investigate ADAMTS-13 expression and apoptosis in CNS and to identify whether they have any correlation with toxoplasmosis neuropathology and neurodegeneration. Mice were infected with ME49 strain T. gondii and the levels of ADAMTS-13, caspase 3, caspase 8, caspase 9, TNFR1 and Bcl-xL expressions were examined in brain tissues by immunohistochemistry, during the development and establishment of chronic infections at 10, 30 and 60 days post-infection. Results of the study revealed that the levels of ADAMTS-13 (P play an important role in the regulation and protection of the blood-brain barrier integrity and CNS microenvironment in TE. These results also suggest that T. gondii-mediated apoptosis might play a pivotal role and a different type of role in the mechanism of neurodegeneration and neuropathology in the process of TE. Furthermore, expression of ADAMTS-13 might give an idea of the progress and is critical for diagnosis of this disease. To the best of the authors' knowledge, this is the first report on ADAMTS-13 expression in the CNS of T. gondii-infected mice. PMID:26542631

  7. Decreased plasma ADAMTS-13 activity as a predictor of postoperative bleeding in cyanotic congenital heart disease

    Rosangela P.S. Soares

    2013-04-01

    Full Text Available OBJECTIVE: To analyze the preoperative plasma antigenic concentration and activity of von Willebrand factor and its main cleaving protease ADAMTS-13 in pediatric patients with cyanotic congenital heart disease undergoing surgical treatment and investigate possible correlations with postoperative bleeding. METHODS: Plasma antigenic concentrations (von Willebrand factor:Ag and ADAMTS-13:Ag were measured using enzyme-linked immunoassays. Collagen-binding assays were developed to measure biological activities (von Willebrand factor:collagen binding and ADAMTS-13 activity. The multimeric structure of von Willebrand factor was analyzed using Western immunoblotting. Demographic, diagnostic, and general and specific laboratory data and surgery-related variables were subjected to univariate, bivariate, and multivariate analysis for the prediction of postoperative bleeding. RESULTS: Forty-eight patients were enrolled, with ages ranging from 9 months to 7.6 years (median 2.5 years. The plasma concentrations of von Willebrand factor:Ag and ADAMTS-13:Ag were decreased by 65 and 82%, respectively, in the patients compared with the controls (p<0.001. An increased density of low-molecular-weight fractions of von Willebrand factor, which are suggestive of proteolytic degradation (p = 0.0081, was associated with decreased ADAMTS-13 activity, which was likely due to ADAMTS-13 consumption (71% of controls, p = 0.0029 and decreased von Willebrand factor:collagen binding (76% of controls, p = 0.0004. Significant postoperative bleeding occurred in 13 patients. The preoperative ADAMTS-13 activity of <64.6% (mean level for the group, preoperative activated partial thromboplastin time, and the need for cardiopulmonary bypass were characterized as independent risk factors for postoperative bleeding, with respective hazard ratios of 22.35 (95% CI 1.69 to 294.79, 1.096 (95% CI 1.016 to 1.183, and 37.43 (95% CI 1.79 to 782.73. CONCLUSION: Low plasma ADAMTS-13

  8. Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene

    Rank, Cecilie Utke; Kremer Hovinga, Johanna; Taleghani, Magnus Mansouri;

    2014-01-01

    , causing intravascular platelet clumping and thrombotic microangiopathy. Our patient, a 26-year-old man, had attacks of thrombotic thrombocytopenic purpura (TTP) with thrombocytopenia and a urine dipstick positive for hemoglobin (4+), often as the only sign of hemolytic activity. He had ADAMTS13 activity...... of A) leading to p.R1123H. This case report confirms the importance of the analysis of the ADAMTS13 activity and its inhibitor in patients who have episodes of TTP, with a very low platelet count and sometimes without the classic biochemical signs of hemolysis....

  9. The ADAMTS13 metalloprotease domain: roles of subsites in enzyme activity and specificity

    de Groot, Rens; Lane, David A.; James T.B. Crawley

    2010-01-01

    ADAMTS13 modulates von Willebrand factor (VWF) platelet-tethering function by proteolysis of the Tyr1605-Met1606 bond in the VWF A2 domain. To examine the role of the metalloprotease domain of ADAMTS13 in scissile bond specificity, we identified 3 variable regions (VR1, -2, and -3) in the ADAMTS family metalloprotease domain that flank the active site, which might be important for specificity. Eight composite sequence swaps (to residues in ADAMTS1 or ADAMTS2) and 18 single-point mutants were ...

  10. Trombotisk trombocytopenisk purpura hos barn med lavt ADAMTS13 enzymniveau

    Spangenberg, Katrine Bredsdorff; Clasen-Linde, Erik; Poulsen, Anja

    2014-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare condition, but important to consider in case of thrombocytopenia and haemolysis. It is imperative to proceed with the correct treatment, in order to ensure a satisfactory outcome. TTP is either acquired or idiopathic. This case report shows that a...... 14-year-old boy has acquired TTP due to an infection with Campylobacter jejuni and Samonella szentes. Plasma exchange plays an essential role in the treatment of TTP....

  11. The ADAMTS13 metalloprotease domain: roles of subsites in enzyme activity and specificity.

    de Groot, Rens; Lane, David A; Crawley, James T B

    2010-10-21

    ADAMTS13 modulates von Willebrand factor (VWF) platelet-tethering function by proteolysis of the Tyr1605-Met1606 bond in the VWF A2 domain. To examine the role of the metalloprotease domain of ADAMTS13 in scissile bond specificity, we identified 3 variable regions (VR1, -2, and -3) in the ADAMTS family metalloprotease domain that flank the active site, which might be important for specificity. Eight composite sequence swaps (to residues in ADAMTS1 or ADAMTS2) and 18 single-point mutants were generated in these VRs and expressed. Swapping VR1 (E184-R193) of ADAMTS13 with that of ADAMTS1 or ADAMTS2 abolished/severely impaired ADAMTS13 function. Kinetic analysis of VR1 point mutants using VWF115 as a short substrate revealed reduced proteolytic function (k(cat)/K(m) reduced by 2- to 10-fold) as a result of D187A, R190A, and R193A substitutions. Analysis of VR2 (F216-V220) revealed a minor importance of this region. Mutants of VR3 (G236-A261) proteolysed wild-type VWF115 normally. However, using either short or full-length VWF substrates containing the P1' M1606A mutation, we identified residues within VR3 (D252-P256) that influence P1' amino acid specificity, we hypothesize, by shaping the S1' pocket. It is concluded that 2 subsites, D187-R193 and D252-P256, in the metalloprotease domain play an important role in cleavage efficiency and site specificity. PMID:20647566

  12. ADAMTS13解析によるTTP/HUS診断

    松本, 雅則; 石指, 宏通; 八木, 秀男; 藤村, 吉博

    2006-01-01

    ADAMTS13はvon Willebrand因子(VWF)を特異的に切断する酵素で,血栓性血小板減少性紫斑病(TTP)や溶血性尿毒症症候群(HUS)との関連で注目されている。本学輸血部では,同酵素活性が測定可能な本邦の代表的施設として,日本全国の医療機関からの依頼によって,過去7年間にTTP/HUS症例を582例集積した。それらの症例で,ADAMTS13活性と同インヒビター活性測定および同遺伝子解析を行ってきたので,その概要を報告する。また,ADAMTS13活性が著滅する症例において,血小板減少のメカニズムを解説し,血小板輸血が禁忌であるエビデンスを概説する。...

  13. Von Willebrand Factor and ADAMTS13 Expression in Renal Tissue in Chronic Glomerulonephritis Diseases%慢性肾脏病患者肾脏vWF和ADAMTS13表达改变及其意义

    沈蕾; 卢国元; 蒋丽琼; 董宁征; 乔青; 李明; 阮长耿

    2011-01-01

    Objective To observe the expression of von Willebrand factor (vWF) and its cleaving protease-ADAMTS13 in renal tissues of patients with different pathological and clinical types of chronic kidney diseases. Methods Thirty six cases of chronic kidney disease' patients were involved in the study. Expression of vWF and ADAMTS13 in renal tissues was analyzed by immunohistochemistry. Image J analysis software was used to measure vWF and ADAMTS13 expression. The results were expressed as positive staining area percentage of total glomerular. Results (1) The vWF/ADAMTS13 positive staining eara in the NS group was significantly higher than that in the CGN group (P < 0. 05). (2) ADAMTS13 positive staining eara was significantly lower in MN group than that in the non-MN group (P < 0.05). (3) vWF/ADAMTS13 expression level was positively correlated with proteinuria (r = 0. 512,P < 0.01). Conclusion The imbalance of local expression of vWF and ADAMTS13 in chronic kidney di sease is related to the clinical and pathological type in a certain extent, may reflect changes in renal microcirculation in some degree.%目的:观察血管性血友病因子(vWF)及其裂解酶ADAMTS13在慢性肾脏病不同病理类型中的表达以及与临床指标的相关性.方法 采用免疫组织化学分析vWF和ADAMTS13在36例不同病理类型慢性肾炎患者肾脏中的表达,Image J图象分析软件测定vWF和ADAMTS13的阳性染色面积的比例.结果 (1)肾病综合征患者肾小球阳性染色面积vWF/ADAMTS13明显高于慢性肾炎组(P<0.05);(2)膜性肾病组ADAMTS13表达明显低于非膜性肾病组(P<0.05);(3)vWF/ADAMTS13阳性染色面积比与蛋白尿呈正相关(r=0.512,P<0.01).结论 慢性肾脏病局部vWF和ADAMTS13表达间平衡失调与慢性肾脏病的临床和病理类型有关,能在一定程度上反映肾脏微循环的变化.

  14. 人血管性血友病因子裂解蛋白酶pEGFP-N1真核表达载体的构建及其在HeLa细胞中的表达%Construction of ADAMTS13-pEGFP-N1 Vector and Its Expression in HeLa Cells

    凌婧; 马珍妮; 苏建; 阮长耿

    2013-01-01

    This study was aimed to construct a pEGFP-Nl vector of von Willebrand factor cleaving protease (ADAMTS13, a disintegrin and metalloprotease with a thrombospondin type 1 motifs 13) so as to pave the way for further studing its synthesis and secretion. Human full-length cDNA sequence of ADAMTS13 was acquired by polymerase chain reaction (PCR) with Phusion? High-Fidelity(NEB) ,then the PCR product was double digested with EcoR I and Xho I . After digestion, the ADAMTS13 cDNA sequence was purified and recombined with the pEGFP-Nl vector. The DNA sequence analysis showed that ADAMTS13 was ligated to the pEGFP-Nl vector correctly. After transient expression in HeLa cells, the expression of EGFP could be detected by fluorescent microscopy, and the expression of ADAMTS13 protein could be detected by SDS-PAGE and Western blot. It is concluded that the ADAMTS13-pEGFP-N1 vector is successfully constructed, and it can be widely used in further research on the mechanism of the synthesis and secretion of ADAMTS13.%本研究旨在构建人血管性血友病因子裂解蛋白酶(ADAMTS13)的pEGFP-N1真核表达载体,为进一步研究ADAMTS13在细胞内合成及其分泌提供有力工具.通过PCR方法获取目的基因片段,并在目的基因两端加上限制性酶切位点.限制性内切酶酶切后,连接至pEGPF-N1真核表达载体.连接后获得质粒进行酶切鉴定及DNA测序验证,并转染HeLa细胞.通过荧光显微镜检测绿色荧光蛋白表达,Western blot方法鉴定所得蛋白.结果表明,酶切鉴定及DNA测序确认目的基因与载体正确连接,在荧光显微镜下观察到绿色荧光.Western blot显示,转染后HeLa细胞表达ADAMTS13蛋白.结论:成功构建了ADAMTS13-pEGFP-N1真核表达载体,为进一步研究ADAMTS13合成、分泌及其代谢的生理学机制提供了研究工具.

  15. Purpura fulminans due to acquired protein C deficiency

    Devdeep Mukherjee

    2015-01-01

    Full Text Available Purpura fulminans (PF may be the presenting symptom in a patient with protein C (PC deficiency. It is a hematological emergency and presents with extensive areas of hemorrhagic necrosis of the skin. PC deficiency is usually genetically inherited. However, we report a 1 year and 4 months boy, who presented with acquired PC deficiency possibly of postinfectious etiology and developed PF.

  16. Severe Plasmodium falciparum malaria is associated with circulating ultra-large von Willebrand multimers and ADAMTS13 inhibition.

    Larkin, Deirdre

    2009-03-01

    Plasmodium falciparum infection results in adhesion of infected erythrocytes to blood vessel endothelium, and acute endothelial cell activation, together with sequestration of platelets and leucocytes. We have previously shown that patients with severe infection or fulminant cerebral malaria have significantly increased circulatory levels of the adhesive glycoprotein von Willebrand factor (VWF) and its propeptide, both of which are indices of endothelial cell activation. In this prospective study of patients from Ghana with severe (n = 20) and cerebral (n = 13) P. falciparum malaria, we demonstrate that increased plasma VWF antigen (VWF:Ag) level is associated with disproportionately increased VWF function. VWF collagen binding (VWF:CB) was significantly increased in patients with cerebral malaria and severe malaria (medians 7.6 and 7.0 IU\\/ml versus 1.9 IU\\/ml; p<0.005). This increased VWF:CB correlated with the presence of abnormal ultra-large VWF multimers in patient rather than control plasmas. Concomitant with the increase in VWF:Ag and VWF:CB was a significant persistent reduction in the activity of the VWF-specific cleaving protease ADAMTS13 (approximately 55% of normal; p<0.005). Mixing studies were performed using P. falciparum patient plasma and normal pooled plasma, in the presence or absence of exogenous recombinant ADAMTS13. These studies demonstrated that in malarial plasma, ADAMTS13 function was persistently inhibited in a time-dependent manner. Furthermore, this inhibitory effect was not associated with the presence of known inhibitors of ADAMTS13 enzymatic function (interleukin-6, free haemoglobin, factor VIII or thrombospondin-1). These novel findings suggest that severe P. falciparum infection is associated with acute endothelial cell activation, abnormal circulating ULVWF multimers, and a significant reduction in plasma ADAMTS13 function which is mediated at least in part by an unidentified inhibitor.

  17. Severe Plasmodium falciparum malaria is associated with circulating ultra-large von Willebrand multimers and ADAMTS13 inhibition.

    Deirdre Larkin

    2009-03-01

    Full Text Available Plasmodium falciparum infection results in adhesion of infected erythrocytes to blood vessel endothelium, and acute endothelial cell activation, together with sequestration of platelets and leucocytes. We have previously shown that patients with severe infection or fulminant cerebral malaria have significantly increased circulatory levels of the adhesive glycoprotein von Willebrand factor (VWF and its propeptide, both of which are indices of endothelial cell activation. In this prospective study of patients from Ghana with severe (n = 20 and cerebral (n = 13 P. falciparum malaria, we demonstrate that increased plasma VWF antigen (VWF:Ag level is associated with disproportionately increased VWF function. VWF collagen binding (VWF:CB was significantly increased in patients with cerebral malaria and severe malaria (medians 7.6 and 7.0 IU/ml versus 1.9 IU/ml; p<0.005. This increased VWF:CB correlated with the presence of abnormal ultra-large VWF multimers in patient rather than control plasmas. Concomitant with the increase in VWF:Ag and VWF:CB was a significant persistent reduction in the activity of the VWF-specific cleaving protease ADAMTS13 (approximately 55% of normal; p<0.005. Mixing studies were performed using P. falciparum patient plasma and normal pooled plasma, in the presence or absence of exogenous recombinant ADAMTS13. These studies demonstrated that in malarial plasma, ADAMTS13 function was persistently inhibited in a time-dependent manner. Furthermore, this inhibitory effect was not associated with the presence of known inhibitors of ADAMTS13 enzymatic function (interleukin-6, free haemoglobin, factor VIII or thrombospondin-1. These novel findings suggest that severe P. falciparum infection is associated with acute endothelial cell activation, abnormal circulating ULVWF multimers, and a significant reduction in plasma ADAMTS13 function which is mediated at least in part by an unidentified inhibitor.

  18. Production, crystallization and preliminary crystallographic analysis of an exosite-containing fragment of human von Willebrand factor-cleaving proteinase ADAMTS13

    A fragment of the ADAMTS13 ancillary domains (ADAMTS13-DTCS) has been expressed, purified and crystallized and the crystals have been characterized by X-ray diffraction. ADAMTS13 is a reprolysin-type metalloproteinase belonging to the ADAMTS (a disintegrin and metalloproteinase with thrombospondin type 1 motif) family. It specifically cleaves plasma von Willebrand factor (VWF) and regulates platelet adhesion and aggregation. ADAMTS13 is a multi-domain enzyme. In addition to the N-terminal metalloproteinase domain, the ancillary domains, including a disintegrin-like domain, a thrombospondin-1 type 1 repeat, a Cys-rich domain and a spacer domain, are required for VWF recognition and cleavage. In the present study, a fragment of the ADAMTS13 ancillary domains (ADAMTS13-DTCS; residues 287–685) was expressed using CHO Lec cells, purified and crystallized. Diffraction data sets were collected using the SPring-8 beamline. Two ADAMTS13-DTCS crystals with distinct unit-cell parameters generated data sets to 2.6 and 2.8 Å resolution, respectively

  19. Tuberculosis and the acquired immune deficiency syndrome in South Brazil

    Tuberculosis and the acquired immune deficiency syndrome in South Brazil. The authors studied the incidence of tuberculosis in South Brazilian patients with acquired immune deficiency syndrome from January 1985 to June 1988. During this period, tuberculosis occurred in 10.3% of acquired immune deficiency syndrome patients. The socioeconomic conditions and the incidence of disease in the population were not confirmed as a potential risk for tuberculosis infection. Chest radiographs revealed pulmonary infiltrates in six patients, hilar and/or mediastinal adenopathy in three, and pleural effusion in two. The two remaining patients had pulmonary consolidation associated with other features. None of these patients presented pulmonary cavitation or radiographic findings of typical reactivation of pulmonary tuberculosis. (author)

  20. Ethnicity Knowledge and Attitudes toward Acquired Immune Deficiency Syndrome.

    Goh, David S.

    This study examined the effects of race/ethnicity and degree of acculturation on knowledge and attitudes about human immunodeficiency virus/Acquired Immune Deficiency Syndrome (HIV/AIDS). Subjects were 274 college students from 5 racial/ethnic groups (Whites, Blacks, Hispanics, Asian Americans, U.S. born, having an Asian origin with families that…

  1. Acquired thrombotic thrombocytopenic purpura after vascular prosthesis implantation for impending rupture of an abdominal aortic aneurysm.

    Naito, Chiaki; Ogawa, Yoshiyuki; Yanagisawa, Kunio; Ishizaki, Takuma; Mihara, Masahiro; Handa, Hiroshi; Isonishi, Ayami; Hayakawa, Masaki; Matsumoto, Masanori; Nojima, Yoshihisa

    2016-01-01

    Acquired thrombotic thrombocytopenic purpura (TTP) is caused by autoantibodies against ADAMTS13. TTP patients run a rapidly fatal course unless immediate plasma exchange (PEX) is initiated upon diagnosis. Herein, we report a 72-year-old man with TTP, which developed after he underwent artificial blood vessel replacement surgery for an abdominal aneurysm with impending rupture. In the perioperative period, the patient received several platelet transfusions for severe thrombocytopenia (minimum platelet count: 0.6×10(4)/μl). Thereafter, he was admitted to our department for rapidly progressing coma with multiple cerebral infarctions, and was transferred to the ICU. Based on the tentative diagnosis of TTP, we immediately began PEX and steroid pulse therapy. The diagnosis was confirmed thereafter by markedly reduced ADAMTS13 activity (<0.5%) and his being positive for the ADAMTS13 inhibitor. We performed PEX for five consecutive days and administered high-dose prednisolone (PSL). On the second hospital day (HD), his platelet count rose along with improvement of his consciousness level. The ADAMTS13 inhibitor was not detected on the 10th HD. TTP did not relapse and his general condition improved despite tapering of PSL. In this case, by closely monitoring ADAMTS13-related parameters and minimizing the number of plasma exchanges, the patient was able to achieve a remission without the use of boosting inhibitors. PMID:27076251

  2. Acquired zinc deficiency in association with anorexia nervosa: case report and review of the literature.

    Van Voorhees, A S; Riba, M

    1992-09-01

    Zinc deficiency, whether a result of an acquired or inherited abnormality of zinc metabolism, is associated with characteristic cutaneous findings. The inherited variety is known as acrodermatitis enteropathica. We present a case of zinc deficiency secondary to starvation induced by anorexia nervosa. Since the cutaneous stigmata of zinc deficiency and anorexia nervosa can initially be subtle and occasionally overlap, we believe that screening zinc levels in patients with anorexia nervosa with prominent cutaneous findings should be considered. PMID:1488378

  3. Homozygous calreticulin mutations in patients with myelofibrosis lead to acquired myeloperoxidase deficiency.

    Theocharides, Alexandre P A; Lundberg, Pontus; Lakkaraju, Asvin K K; Lysenko, Veronika; Myburgh, Renier; Aguzzi, Adriano; Skoda, Radek C; Manz, Markus G

    2016-06-23

    The pathogenesis of acquired myeloperoxidase (MPO) deficiency, a rare phenomenon observed in patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), is unknown. MPO is a glycoprotein (GP) chaperoned by calreticulin (CALR) in the endoplasmic reticulum. Mutations in CALR are frequently found in patients with myelofibrosis (MF) and essential thrombocythemia (ET) with nonmutated Janus kinase 2 (JAK2). We hypothesized that acquired MPO deficiency in MPN could be associated with the presence of CALR mutations. A cohort of 317 patients with MPN (142 polycythemia vera [PV], 94 ET, and 81 MF) was screened for MPO deficiency. MPO deficiency was observed in 6/81 MF patients (7.4%), but not in PV or ET patients. Susceptibility to infections had been documented in 2/6 (33%) MPO-deficient patients. Five out of 6 patients with MPO deficiency carried a homozygous CALR mutation and were also deficient in eosinophilic peroxidase (EPX). In contrast, 1 patient with MF, a JAK2-V617F mutation, and MPO deficiency, carried 2 previously reported MPO mutations and showed normal EPX activity. Patients with homozygous CALR mutations had reduced MPO protein, but normal MPO messenger RNA (mRNA) levels supporting a posttranscriptional defect in MPO production. Finally, we demonstrate in vitro that in the absence of CALR, immature MPO protein precursors are degraded in the proteasome. Therefore, 4 decades after the first description of acquired MPO deficiency in MPN, we provide the molecular correlate associated with this phenomenon and evidence that CALR mutations can affect the biosynthesis of GPs. PMID:27013444

  4. A case of congenital TTP presenting with microganiopathy in adulthood

    Gallivan, Chris D; Conrad, David M; Kew, Andrea K

    2014-01-01

    Background Congenital thrombotic thrombocytopenic purpura (TTP), also known as Upshaw-Schulman Syndrome is a rare inherited deficiency of ADAMTS13. Unlike the more common acquired TTP which is characterized by an acquired inhibitor of ADAMTS13, patients with congenital TTP have an absolute deficiency of ADAMTS13 without an inhibitor. Congenital TTP generally presents in infancy with repeat episodes of acute hemolysis and evidence of microangiopathy, these episodes are usually triggered by ill...

  5. Meningitis and stridor in advanced Human immunodeficiency virus/acquired immune deficiency syndrome

    Naidoo P

    2013-09-01

    Full Text Available P Naidoo, D Pillay, S SamanDepartment of Internal Medicine, Port Shepstone Regional Hospital, University of KwaZulu-Natal, South AfricaAbstract: A 37-year-old female presented confused with a preceding history of severe headache. After clinical examination and investigations, she was diagnosed with disseminated tuberculosis (including central nervous system involvement, and Human immunodeficiency virus/acquired immune deficiency syndrome. Her hospital stay was complicated. She developed stridor and a cerebrovascular accident with left hemiplegia. She died approximately 2 weeks after admission. The potential causes of her stridor included a mediastinal mass or a central mechanism secondary to tuberculosis meningitis. Limited resources precluded definitive imaging of the chest to rule out a mediastinal mass. Further, an autopsy was not done. Despite these limitations, this case is unique because it reports the presence of both stridor and tuberculosis meningitis in an adult patient.Keywords: Human immunodeficiency virus, acquired immune deficiency syndrome, meningitis, stridor, tuberculosis

  6. Heterotopic ossification (myositis ossificans) in acquired immune deficiency syndrome. Detection by gallium scintigraphy

    A case of heterotopic ossification (myositis ossificans) secondary to the central nervous system complications of acquired immune deficiency syndrome (AIDS) is reported. Because of the overwhelming suspicion of infection in this patient, this diagnosis was not considered until a gallium scan revealed the typical findings of heterotopic ossification. Because of the increasing utilization of gallium imaging in the AIDS population, every imaging specialist should be aware of this potential disorder

  7. Meningitis and stridor in advanced Human immunodeficiency virus/acquired immune deficiency syndrome

    Naidoo P; Pillay D; Saman S

    2013-01-01

    P Naidoo, D Pillay, S SamanDepartment of Internal Medicine, Port Shepstone Regional Hospital, University of KwaZulu-Natal, South AfricaAbstract: A 37-year-old female presented confused with a preceding history of severe headache. After clinical examination and investigations, she was diagnosed with disseminated tuberculosis (including central nervous system involvement), and Human immunodeficiency virus/acquired immune deficiency syndrome. Her hospital stay was complicated. She developed stri...

  8. Chest radiographs in acquired antibody deficiency syndrome with chronic granulomatous inflammation

    Ten cases of acquired antibody deficiency syndrome with chronic granulomatous infection were diagnosed in our hospital during the past 10 years. We were able to perform a retrospective analysis of the initial and follow-up chest radiographs in 8 of these patients. The following pathological findings could be demonstrated: 1. increased bronchovascular markings in the basal lung fields, 2. reticular densities in the middle and basal lung fields, 3. confluent nodular densities of varying size in the periphery of the basal and middle fields, 4. pulmonary infiltrates in the middle and lower lobes, 5. hilar node enlargement of moderate extent. Findings 2, 3 and 5 completely disappeared under steroid therapy whereas 1 showed only partial recovery. If both the radiologic and serologic findings are considered, it is possible to differentiate this disease from sarcoidosis. (orig.)

  9. Social capital of Iranian patients living with acquired immune deficiency syndrome and associated factors.

    Ansari, S K; Nedjat, S; Jabbari, H; Saiepour, N; Heris, M J

    2015-10-01

    This study investigated the social capital of Iranian patients living with acquired immune deficiency syndrome (AIDS) and the associated factors. In a cross-sectional study the Integrated Social Capital Questionnaire was filled by a sequential sample of 300 patients visiting a referral counselling centre in Tehran. The patients' social capital scores were around 50% in the trust, social cohesion, collective action and cooperation and political empowerment domains. The groups and networks membership domain scored the lowest (27.1%). In regression analysis, employment status was significantly associated with groups and networks membership; age, marital status and financial status were associated with collective action and cooperation; period of disease awareness and marital status affected social cohesion and inclusion; and having risky behaviour affected empowerment and political action. Efforts are needed to enhance the social capital of those patients living with AIDS who are younger, unemployed, divorced/widowed, with risky behaviours and shorter disease awareness. PMID:26750165

  10. Meeting Minutes of International Conference on Prevention and Treatment of Acquired Immune Deficiency Syndrome with Chinese Medicine

    LI Yong; WANG Jie; LIN Hong-sheng; WU Xin-fang; LIU Jie; TANG Yan-li; NAN Ji-hong

    2011-01-01

    @@ The International Conference on Prevention and Treatment of Acquired Immune Deficiency Syndrome (AIDS) with Chinese Medicine (CM) was held in Beijing International Hotel from October 16to 17,2010.It was cosponsored by Guang'anmen Hospital,China Academy of Chinese Medical Sciences (CACMS),and the First Affiliated Hospital of Henan University of Traditional Chinese Medicine.

  11. Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome?

    Jose, Sheethal K; Marfatia, Yogesh S

    2016-01-01

    A 47-year-old male with acquired immune deficiency syndrome (AIDS) presented with multiple hyperpigmented papules and nodules on both ankles, dorsum of bilateral feet and soles. It was associated with mild itching and pain. The patient was diagnosed with human immunodeficiency virus (HIV) in 2007. First-line antiretroviral therapy (ART) was started in 2009 to which he responded initially. He was shifted to second-line ART 11 months ago in March 2015 due to treatment failure as suggested by CD4 count of 50 cells/mm(3). The present skin lesions started 2 months after the initiation of second-line ART. Differential diagnoses considered were Kaposi's sarcoma and immune reconstitution inflammatory syndrome (IRIS) related infections, but biopsy was suggestive of erythema elevatum diutinum (EED). Patient was started on oral dapsone 100 mg/day and increased to 200 mg/day to which he is responding gradually. In the present case, appearance of the lesions after initiation of second-line ART coupled with increase in CD4 count and decrease of viral load below undetectable level suggest that EED could be an IRIS. PMID:27190420

  12. Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome?

    Sheethal K Jose

    2016-01-01

    Full Text Available A 47-year-old male with acquired immune deficiency syndrome (AIDS presented with multiple hyperpigmented papules and nodules on both ankles, dorsum of bilateral feet and soles. It was associated with mild itching and pain. The patient was diagnosed with human immunodeficiency virus (HIV in 2007. First-line antiretroviral therapy (ART was started in 2009 to which he responded initially. He was shifted to second-line ART 11 months ago in March 2015 due to treatment failure as suggested by CD4 count of 50 cells/mm3. The present skin lesions started 2 months after the initiation of second-line ART. Differential diagnoses considered were Kaposi's sarcoma and immune reconstitution inflammatory syndrome (IRIS related infections, but biopsy was suggestive of erythema elevatum diutinum (EED. Patient was started on oral dapsone 100 mg/day and increased to 200 mg/day to which he is responding gradually. In the present case, appearance of the lesions after initiation of second-line ART coupled with increase in CD4 count and decrease of viral load below undetectable level suggest that EED could be an IRIS.

  13. Anterior segment manifestations of human immunodeficiency virus/acquired immune deficiency syndrome

    Biswas Jyotirmay

    2008-01-01

    Full Text Available Ocular complications are known to occur as a result of human immunodeficiency virus (HIV disease. They can be severe leading to ocular morbidity and visual handicap. Cytomegalovirus (CMV retinitis is the commonest ocular opportunistic infection seen in acquired immune deficiency syndrome (AIDS. Though posterior segment lesions can be more vision-threatening, there are varied anterior segment manifestations which can also lead to ocular morbidity and more so can affect the quality of life of a HIV-positive person. Effective antiretroviral therapy and improved prophylaxis and treatment of opportunistic infections have led to an increase in the survival of an individual afflicted with AIDS. This in turn has led to an increase in the prevalence of anterior segment and adnexal disorders. Common lesions include relatively benign conditions such as blepharitis and dry eye, to infections such as herpes zoster ophthalmicus and molluscum contagiosum and malignancies such as squamous cell carcinoma and Kaposi′s sarcoma. With the advent of highly active antiretroviral therapy, a new phenomenon known as immune recovery uveitis which presents with increased inflammation, has been noted to be on the rise. Several drugs used in the management of AIDS such as nevirapine or indinavir can themselves lead to severe inflammation in the anterior segment and adnexa of the eye. This article is a comprehensive update of the important anterior segment and adnexal manifestations in HIV-positive patients with special reference to their prevalence in the Indian population.

  14. Correlation of diagnostic imaging and autopsy findings of eight patients with acquired immune deficiency syndrome

    Objective: To investigate the imaging findings with pathologic correlation in patients with acquired immune deficiency syndrome (AIDS). Methods: Imaging findings, autopsy and pathological data were retrospectively analyzed in eight patients with AIDS. Routine CT scanning of different body parts was performed during their hospitalization. CT scanning was performed from the skull to the pelvis immediately following their death. After routine formalin fixing, 7 cadavers were cross sectioned for autopsy in freezing state and 1 for gross autopsy. Tissues were obtained from each sections and organs for pathological examinations. Results: The autopsy data showed parasitic infections (5 cases), bacterial infections (3 cases), fungal infections (2 cases), virus infections (2 cases), lymphoma (1 case) and cerebrovascular diseases (1 case)in eight patients with AIDS. The CT scanning demonstrated symmetrical ground glass liked shadows with pulmonary hilus as the center in 5 cases of pulmonary PCP infection; pulmonary patchy shadows, scattering distribution of nodular shadows, extensive military nodular shadows with even distribution and tuberculous pleurisy; cloudy shadows for 2 cases of fungi infection with multiple foci of chronic inflammation; pulmonary net-like parenchymal changes for 2 cases of pulmonary CMV infection; thickened intestinal wall and narrowed intestinal lumen for 1 case of intestinal tumor; low density shadows of brain tissue for 1 case of CMV encephalitis and MRI findings of high T1 and high T2 signals as well as MRA findings of broken vascular channels in liquefied areas of brain tissues; patchy low density areas inside a cyst of brain for one case of brain toxoplasmosis infection; multiple small patchy low density areas in cerebral basal ganglia for one case of brain cryptococcus infection. Conclusions: In AIDS patients, infection and tumor may occur in various organs resulting in complex symptoms, which makes it more complicated and difficult to make

  15. The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity.

    Wu, Maddalena Alessandra; Castelli, Roberto

    2016-02-01

    Several clinical and biological features of lymphoproliferative diseases have been associated with an increased risk of developing autoimmune manifestations. Acquired deficiency of C1-inhibitor (C1-INH) (AAE) is a rare syndrome clinically similar to hereditary angioedema (HAE) characterized by local increase in vascular permeability (angioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. Bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein (a serine protease controlled by C1-INH), is the mediator of symptoms. In total 46% of AAE patients carry an underlying hematological disorder including monoclonal gammopathy of uncertain significance (MGUS) or B cell malignancies. However, 74% of AAE patients have anti-C1-INH autoantibodies without hematological, clinical or instrumental evidence of lymphoproliferative disease. Unlike HAE patients, AAE patients usually have late-onset symptoms, do not have a family history of angioedema and present variable response to treatment due to the hypercatabolism of C1-INH. Experiments show that C1-INH and/or the classical complement pathway were consumed by the neoplastic lymphatic tissues and/or anti-C1-INH neutralizing autoantibodies. Therapy of AAE follows two directions: 1) prevention/reversal of the symptoms of angioedema; and 2) treatment of the associated disease. Different forms of B cell disorders coexist and/or evolve into each other in AAE and seem to be dominated by an altered control of B cell proliferation, thus AAE represents an example of the strict link between autoimmunity and lymphoproliferation. PMID:26068904

  16. Bilateral Central Retinal Vein Occlusions Combined with Artery Occlusions in A Patient with Acquired Immune Deficiency Syndrome

    Feng Wen; Xuemei Chen; Haitai Li; Ruiduan Liao; Dezheng Wu

    2002-01-01

    Purpose: This is the first report of a bilateral nonischemic central retinal vein occlusionscombined with artery occlusions in a patient with acquired immune deficiency syndrome(AIDS). Methods: Case report. Results: A 22-year-old Chinese(male) with a positive human immunodeficiency virus(HIV) infection developed bilateral nonischemic central retinal vein occlusions combinedwith artery occlusions and severe vision loss. The manifestations of the fundus andfluorescein angiography were similar in both eyes.Conclusion: This case report provides the evidences that central retinal vein and arteryocclusions are probably part of the spectrum of AIDS vascular diseases.

  17. Primary stomal lymphoma. An unusual complication of ileostomy in a patient with transfusion-related acquired immune deficiency syndrome.

    Levecq, H; Hautefeuille, M; Hoang, C; Galian, A; Hautefeuille, P; Rambaud, J C

    1990-02-15

    A 73-year-old heterosexual man developed a high-grade non-Hodgkin's lymphoma at the site of an ileostomy only 2 years after proctectomy for undetermined colitis not cured by previous colectomy. In fact, the early occurrence of this usually very late and rare complication of ileostomy was probably favored by the simultaneous presence of acquired immune deficiency syndrome (AIDS) due to repeated blood transfusions for refractory anemia with excess blasts. The intestinal location of the tumor, its high-grade malignancy and B-cell origin are all features of AIDS-related non-Hodgkin's lymphoma. This case report seems to be one of the rarely identified examples of the cooperation between general predisposing factors and local irritating agents at the origin of a malignant tumor. PMID:2297651

  18. Anaplastic lymphoma kinase negative anaplastic large cell lymphoma of hard palate as first clinical manifestation of acquired immune deficiency syndrome.

    Narwal, Anjali; Yadav, Achla Bharti; Prakash, Sant; Gupta, Shally

    2016-01-01

    Anaplastic large cell lymphoma (ALCL) is an uncommon disease, accounting for <5% of all cases of non-Hodgkin's lymphoma. We report a case of 48-year-old male who presented a clinically benign swelling in the right anterior palatal region since last 2 months. Radiographic evaluation showed no bone loss in palatal area. Histological and radiological examination was in favor of a peripheral reactive lesion like pyogenic granuloma or a benign salivary gland tumor. Immunohistochemistry confirmed the diagnosis of anaplastic lymphoma kinase-negative (ALK(-)) ALCL. Further laboratory tests ELISA for human immunodeficiency virus (HIV) and CD4 cell count was done which showed positivity for HIV. To the best of our knowledge, it is the first case of ALK(-) ALCL in the hard palate presenting as the first clinical manifestation of acquired immune deficiency syndrome. PMID:27041916

  19. Anaplastic lymphoma kinase negative anaplastic large cell lymphoma of hard palate as first clinical manifestation of acquired immune deficiency syndrome

    Anjali Narwal

    2016-01-01

    Full Text Available Anaplastic large cell lymphoma (ALCL is an uncommon disease, accounting for <5% of all cases of non-Hodgkin's lymphoma. We report a case of 48-year-old male who presented a clinically benign swelling in the right anterior palatal region since last 2 months. Radiographic evaluation showed no bone loss in palatal area. Histological and radiological examination was in favor of a peripheral reactive lesion like pyogenic granuloma or a benign salivary gland tumor. Immunohistochemistry confirmed the diagnosis of anaplastic lymphoma kinase-negative (ALK(− ALCL. Further laboratory tests ELISA for human immunodeficiency virus (HIV and CD4 cell count was done which showed positivity for HIV. To the best of our knowledge, it is the first case of ALK(− ALCL in the hard palate presenting as the first clinical manifestation of acquired immune deficiency syndrome.

  20. Wernicke’s Encephalopathy: An Unusual Consequence of the Acquired Immune Deficiency Syndrome—Case Report and Literature Review

    Timothy R. Larsen

    2013-01-01

    Full Text Available Introduction. Wernicke’s encephalopathy is a well-described syndrome characterized by the classic triad of confusion, ataxia, and ophthalmoplegia. Wernicke’s encephalopathy results from thiamine (vitamin B1 deficiency. Common causes include alcoholism and gastric disorders. Wernicke’s has been described in patients with acquired immune deficiency syndrome (AIDS; however, given these patients’ immunosuppressed state, the diagnosis of Wernicke’s encephalopathy is not apparent. Case Presentation. A 31-year-old previously healthy male presented to the ER complaining of progressive dyspnea. Workup revealed HIV/AIDS and PCP pneumonia. He was treated and improved. On day 14 he became confused and developed nystagmus and ataxia. Considering his immunocompromised state, infectious and neoplastic etiologies topped the differential diagnosis. CT head was negative. Lumbar puncture was unremarkable. Brain MRI revealed increased T2 signal in the medial thalamus bilaterally. Intravenous thiamine was administered resulting in resolution of symptoms. Discussion. The classic triad of Wernicke’s encephalopathy occurs in 10% of cases. When immunosuppressed patients develop acute neurologic symptoms infectious or neoplastic etiologies must be excluded. However, given the relative safety of thiamine supplementation, there should be a low threshold for initiating therapy in order to reverse the symptoms and prevent progression to Korsakoff dementia, which is permanent.

  1. Wernicke's Encephalopathy: An Unusual Consequence of the Acquired Immune Deficiency Syndrome-Case Report and Literature Review.

    Larsen, Timothy R; Dragu, Dritan; Williams, Michael

    2013-01-01

    Introduction. Wernicke's encephalopathy is a well-described syndrome characterized by the classic triad of confusion, ataxia, and ophthalmoplegia. Wernicke's encephalopathy results from thiamine (vitamin B1) deficiency. Common causes include alcoholism and gastric disorders. Wernicke's has been described in patients with acquired immune deficiency syndrome (AIDS); however, given these patients' immunosuppressed state, the diagnosis of Wernicke's encephalopathy is not apparent. Case Presentation. A 31-year-old previously healthy male presented to the ER complaining of progressive dyspnea. Workup revealed HIV/AIDS and PCP pneumonia. He was treated and improved. On day 14 he became confused and developed nystagmus and ataxia. Considering his immunocompromised state, infectious and neoplastic etiologies topped the differential diagnosis. CT head was negative. Lumbar puncture was unremarkable. Brain MRI revealed increased T2 signal in the medial thalamus bilaterally. Intravenous thiamine was administered resulting in resolution of symptoms. Discussion. The classic triad of Wernicke's encephalopathy occurs in 10% of cases. When immunosuppressed patients develop acute neurologic symptoms infectious or neoplastic etiologies must be excluded. However, given the relative safety of thiamine supplementation, there should be a low threshold for initiating therapy in order to reverse the symptoms and prevent progression to Korsakoff dementia, which is permanent. PMID:23935638

  2. Serum selenium and skin diseases among Nigerians with human immunodeficiency virus/acquired immune deficiency syndrome

    Akinboro AO

    2013-08-01

    Full Text Available Adeolu Oladayo Akinboro,1 David Ayodele Mejiuni,2 Olaniyi Onayemi,3 Olugbenga Edward Ayodele,4 Adeniran Samuel Atiba,5 Gbenga Micheal Bamimore6 1Dermatology Unit, Department of Internal Medicine, College of Health Sciences, Osogbo, and LAUTECH Teaching Hospital, Ogbomoso, Oyo State, Nigeria; 2Bullsbrook Medical Practice, Perth, WA, Australia; 3Department of Dermatology and Venereology, College of Health Science, Obafemi Awolowo University and OAUTHC, Ile – Ife, Osun State, Nigeria; 4Department of Internal Medicine, College of Health Sciences, Osogbo and LAUTECH Teaching Hospital, Ogbomoso, Oyo State, Nigeria; 5Department of Chemical Pathology, College of Medicine, Ekiti State University, and Ekiti State University Teaching Hospital, Ekiti State, Nigeria; 6Dermatology Unit, Department of Internal Medicine, LAUTECH Teaching Hospital, Ogbomoso, Oyo State, Nigeria Background: The role of selenium as an antioxidant micronutrient has garnered the unprecedented focus of researchers in recent times. No clinical study has related serum selenium concentration to skin diseases in human immunodeficiency virus (HIV/acquired immunodeficiency syndrome (AIDS patients. Methods: In this study, 134 newly diagnosed HIV patients that satisfied the inclusion criteria were included. Skin diseases were clinically diagnosed and fasting venous blood was taken for assessment of serum selenium using an atomic absorption spectrophotometer. Results: The mean age of HIV subjects with and without skin disease were not significantly different: 32.72 ± 9.21 versus 35.86 ± 8.55 years, P = 0.077, respectively. The mean of serum selenium (0.51 ± 0.48 versus 0.81 ± 0.39, CD4+ count (228.06 ± 212.89 versus 446.41 ± 182.87, and body mass index (BMI; 21.09 ± 3.58 versus 23.53 ± 3.35 were significantly lower (P 0.05. Conclusion: serum selenium concentration was lower among HIV subjects with skin diseases than those without skin disease. Pruritic papular eruption, xeroderma

  3. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.

    Agostoni, Angelo; Aygören-Pürsün, Emel; Binkley, Karen E; Blanch, Alvaro; Bork, Konrad; Bouillet, Laurence; Bucher, Christoph; Castaldo, Anthony J; Cicardi, Marco; Davis, Alvin E; De Carolis, Caterina; Drouet, Christian; Duponchel, Christiane; Farkas, Henriette; Fáy, Kálmán; Fekete, Béla; Fischer, Bettina; Fontana, Luigi; Füst, George; Giacomelli, Roberto; Gröner, Albrecht; Hack, C Erik; Harmat, George; Jakenfelds, John; Juers, Mathias; Kalmár, Lajos; Kaposi, Pál N; Karádi, István; Kitzinger, Arianna; Kollár, Tímea; Kreuz, Wolfhart; Lakatos, Peter; Longhurst, Hilary J; Lopez-Trascasa, Margarita; Martinez-Saguer, Inmaculada; Monnier, Nicole; Nagy, István; Németh, Eva; Nielsen, Erik Waage; Nuijens, Jan H; O'grady, Caroline; Pappalardo, Emanuela; Penna, Vincenzo; Perricone, Carlo; Perricone, Roberto; Rauch, Ursula; Roche, Olga; Rusicke, Eva; Späth, Peter J; Szendei, George; Takács, Edit; Tordai, Attila; Truedsson, Lennart; Varga, Lilian; Visy, Beáta; Williams, Kayla; Zanichelli, Andrea; Zingale, Lorenza

    2004-09-01

    Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder. PMID:15356535

  4. Epstein-Barr virus myelitis and Castleman's disease in a patient with acquired immune deficiency syndrome: a case report

    Balderacchi Jasminka

    2011-05-01

    Full Text Available Abstract Introduction Few cases of Epstein-Barr virus myelitis have been described in the literature. Multi-centric Castleman's disease is a lymphoproliferative disorder that is well known for its associations with the human immunodeficiency virus, human herpes virus 8, and Kaposi's sarcoma. The concurrent presentation of these two diseases in a patient at the same time is extremely unusual. Case Presentation We describe the case of a 43-year-old Caucasian man with acquired immune deficiency syndrome who presented with fever, weight loss and diffuse lymphadenopathy, and was diagnosed with multi-centric Castleman's disease. He presented three weeks later with lower extremity weakness and urinary retention, at which time cerebrospinal fluid contained lymphocytic pleocytosis and elevated protein. Magnetic resonance imaging demonstrated abnormal spinal cord signal intensity over several cervical and thoracic segments, suggesting the diagnosis of myelitis. Our patient was ultimately diagnosed with Epstein-Barr virus myelitis, as Epstein-Barr virus DNA was detected by polymerase chain reaction in the cerebrospinal fluid. Conclusion To the best of our knowledge, this is the first case of multi-centric Castleman's disease followed by acute Epstein-Barr virus myelitis in a human immunodeficiency virus-infected patient. Clinicians caring for human immunodeficiency virus-infected patients should be vigilant about monitoring patients with increasing lymphadenopathy, prompting thorough diagnostic investigations when necessary.

  5. Deficiencies

    U.S. Department of Health & Human Services — A list of all deficiencies currently listed on Nursing Home Compare, including the nursing home that received the deficiency, the associated inspection date,...

  6. MHC class I–deficient natural killer cells acquire a licensed phenotype after transfer into an MHC class I–sufficient environment

    Elliott, Julie M.; Wahle, Joseph A.; Yokoyama, Wayne M.

    2010-01-01

    In MHC class I–deficient hosts, natural killer (NK) cells are hyporesponsive to cross-linking of activation receptors. Functional competence requires engagement of a self–major histocompatability complex (MHC) class I–specific inhibitory receptor, a process referred to as “licensing.” We previously suggested that licensing is developmentally determined in the bone marrow. In this study, we find that unlicensed mature MHC class I–deficient splenic NK cells show gain-of-function and acquire a l...

  7. The acquired immune deficiency syndrome and epidemic of infection with human immunodeficiency virus: costs of care and prevention in an inner London district

    1986-01-01

    The epidemic of the acquired immune deficiency syndrome (AIDS) and infection with human immunodeficiency virus (HIV) necessitates early planning of services and allocation of resources. The use of hospital resources by patients with AIDS and the planned additional costs of clinical and preventive services for the epidemic of infection with HIV were calculated for an inner London health district that has treated 18% of the cases in the United Kingdom. Patients with AIDS required on average 50 ...

  8. The WOMED model of benign thyroid disease: Acquired magnesium deficiency due to physical and psychological stressors relates to dysfunction of oxidative phosphorylation

    Roy Moncayo

    2015-06-01

    Conclusions: We interpret the elevated thyroid vascularization and low magnesium levels as signs of an inflammatory process related to the musculoskeletal changes. Improvement of thyroid function and morphology can be achieved after correcting the influence of stressors together with the supplementation regime. We hypothesize that the central biochemical event in thyroid disease is that of an acquired, altered mitochondrial function due to deficiency of magnesium, selenium, and coenzyme Q10.

  9. Clinical features and treatment of hepatitis B virus and hepatitis C virus co-infection among patients with acquired immune deficiency syndrome

    杨蓉蓉

    2014-01-01

    Objective To estimate the clinical features of hepatitis B virus(HBV)and hepatitis C virus(HCV)co-infection among acquired immune deficiency syndrome(AIDS)patients and the interaction of lamivudine(3 TC)contained antiretroviral therapy(ART)with hepatitis virus replication.Methods From 2004 to 2010,199human immunodeficiency virus(HIV)/HBV coinfected patients admitted to Zhongnan Hospital of Wuhan University were enrolled,including 76 cases of HIV/HBV/HCV triple infection and 123 cases of

  10. Vitamin D deficiency is associated with community-acquired clostridium difficile infection: a case–control study

    Sahay, Tanya; Ananthakrishnan, Ashwin N.

    2014-01-01

    Background Clostridium difficile infection (CDI) is increasingly recognized as an important community acquired pathogen causing disease (CA-CDI). Vitamin D [25(OH)D] has immune modulatory effects and plays an important role in intestinal immunity. The role of vitamin D in CA-CDI has not been examined previously. Methods This was a single referral center case–control study. Cases comprised of all patients with CA-CDI who had a serum 25(OH)D measured within 12 months prior to infection. Control...

  11. [Thrombotic microangiopathies

    Nielsen, O.J.; Friis-Hansen, L.

    2009-01-01

    The thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive pathophys......The thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive...

  12. Complex changes in von Willebrand factor-associated parameters are acquired during uncomplicated pregnancy.

    Danielle N Drury-Stewart

    Full Text Available BACKGROUND: The coagulation protein von Willebrand Factor (VWF is known to be elevated in pregnancy. However, the timing and nature of changes in VWF and associated parameters throughout pregnancy are not well understood. OBJECTIVES: To better understand the changes in VWF provoked by pregnancy, we studied VWF-associated parameters in samples collected over the course of healthy pregnancies. METHODS: We measured VWF antigen (VWF:Ag, VWF propeptide (VWFpp, Factor VIII (FVIII, and ADAMTS13 activity in samples collected from 46 women during pregnancy and at non-pregnant baseline. We also characterized pregnant vs. non-pregnant VWF multimer structure in 21 pregnancies, and performed isoelectric focusing (IEF of VWF in two pregnancies which had samples from multiple trimesters. RESULTS: VWF:Ag and FVIII levels were significantly increased during pregnancy. ADAMTS13 activity was unchanged. VWFpp levels increased much later in pregnancy than VWF:Ag, resulting in a progressive decrease in VWFpp:Ag ratios. FVIII:VWF ratios also decreased in pregnancy. Most pregnancies exhibited a clear loss of larger VWF multimers and altered VWF triplet structure. Further evidence of acquired VWF qualitative changes in pregnancy was found in progressive, reversible shifts in VWF IEF patterns over gestation. CONCLUSIONS: These data support a new view of pregnancy in which VWF can acquire qualitative changes associated with advancing gestational age. Modeling supports a scenario in which both increased VWF production and doubling of the VWF half-life would account for the data observed. We propose that gestation induces a prolongation in VWF survival, which likely contributes to increased total VWF levels and altered VWF structure.

  13. The correlation between perceived social support and illness uncertainty in people with human immunodeficiency virus/acquired immune deficiency syndrome in Iran

    Moosa Sajjadi

    2015-01-01

    Full Text Available Background: Illness uncertainty is a source of a chronic and pervasive psychological stress for people living with human immunodeficiency virus (HIV/acquired immune deficiency syndrome (AIDS (PLWH, and largely affects their quality of life and the ability to cope with the disease. Based on the uncertainty in illness theory, the social support is one of the illness uncertainty antecedents, and influences the level of uncertainty perceived by patients. Aim: To examine uncertainty in PLWH and its correlation with social support in Iran. Materials and Methods: This cross-sectional correlational study was conducted with 80 PLWH presenting to AIDS Research Center, Tehran, Iran in 2013. The data collected using illness uncertainty and social support inventories were analyzed through Pearson′s correlation coefficient, Spearman′s correlation coefficient, and regression analysis. Results: The results showed a high level of illness uncertainty in PLWH and a negative significant correlation between perceived social support and illness uncertainty ( P = 0.01, r = -0.29. Conclusion: Uncertainty is a serious aspect of illness experience in Iranian PLWH. Providing adequate, structured information to patients as well as opportunities to discuss their concerns with other PLWH and receive emotional support from their health care providers may be worthwhile.

  14. Effect of traditional Chinese medicine for treating human immunodeficiency virus infections and acquired immune deficiency syndrome: Boosting immune and alleviating symptoms.

    Zou, Wen; Wang, Jian; Liu, Ying

    2016-01-01

    To respond to the human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) epidemic in China, the integration of antiretroviral therapy (ART) and traditional Chinese medicine (TCM) has important implications in health outcomes, especially in China where the use of TCM is widespread. The National Free TCM Pilot Program for HIV Infected People began in 5 provinces (Henan, Hebei, Anhui, Hubei, and Guangdong) in 2004, and quickly scaled up to 19 provinces, autonomous regions, and municipalities in China including some places with high prevalence, 26,276 adults have been treated thus far. Usually, people with HIV infection seek TCM for four main reasons: to enhance immune function, to treat symptoms, to improve quality of life, and to reduce side effects related to medications. Evidences from randomized controlled clinical trials suggested some beneficial effects of use of traditional Chinese herbal medicine for HIV infections and AIDS. More proofs from large, well-designed, rigorous trials is needed to give firm support. Challenges include interaction between herbs and antiretroviral drugs, stigma and discrimination. The Free TCM Program has made considerable progress in providing the necessary alternative care and treatment for HIV-infected people in China, and has strong government support for continued improvement and expansion, establishing and improving a work mechanism integrating Chinese and Western medicines. PMID:26577109

  15. Psychopathology in 90 consecutive human immunodeficiency virus-seropositive and acquired immune deficiency syndrome patients with mostly intravenous drug use history.

    Perretta, P; Nisita, C; Zaccagnini, E; Lorenzetti, C; Nuccorini, A; Cassano, G B; Akiskal, H S

    1996-01-01

    This report presents systematic clinical data regarding psychiatric diagnoses, personal and family psychiatric histories, and symptomatologic aspects of 90 consecutive human immunodeficiency virus (HIV)-seropositive and acquired immune deficiency syndrome (AIDS) patients, of whom slightly less than two thirds were at risk due to intravenous drug abuse. In addition, a comparison was made between the distribution patterns of these variables at various stages of HIV illness and related at-risk behaviors. Eighty-four percent of the patients met criteria for a spectrum of DSM-III-R diagnoses (mostly affective) that were associated with high rates of affective and alcohol abuse disorders among first-degree relatives. Mood disorders did not differ significantly between the two main groups at risk (intravenous drug users [IVDUs] v others) by gender, age, or stage of illness. The overall data from the rating scales show high levels of psychic and somatic anxiety in the early stages of illness, whereas cognitive symptoms, retardation, and disorientation are dominant in later stages. A noteworthy finding in this study is that many depressed patients demonstrated current and/or past hypomanic, hyperthymic, or cyclothymic features with no evidence of brain damage detectable by computed axial tomography (CAT). These temperamental attributes, which preceded HIV infection, may have served as risk factors for both drug abuse and impulsive sexual behavior in all types of at-risk groups. PMID:8826691

  16. Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency

    Levi, M; Choi, G; Picavet, C; Hack, CE

    2006-01-01

    Background: Administration of C1-inhibitor concentrate is effective for prophylaxis and treatment of severe angioedema attacks caused by Cl-inhibitor deficiency. The concentrate should be administered intravenously and hence needs to be administered by health care professionals, which might cause co

  17. Up-Regulation of Mitochondrial Activity and Acquirement of Brown Adipose Tissue-Like Property in the White Adipose Tissue of Fsp27 Deficient Mice

    Toh, Shen Yon; Gong, Jingyi; Du, Guoli; Li, John Zhong; Yang, Shuqun; Ye, Jing; Yao, Huilan; Zhang, Yinxin; Xue, Bofu; Li, Qing; Yang, Hongyuan; Wen, Zilong; Li, Peng

    2008-01-01

    Fsp27, a member of the Cide family proteins, was shown to localize to lipid droplet and promote lipid storage in adipocytes. We aimed to understand the biological role of Fsp27 in regulating adipose tissue differentiation, insulin sensitivity and energy balance. Fsp27 −/− mice and Fsp27/lep double deficient mice were generated and we examined the adiposity, whole body metabolism, BAT and WAT morphology, insulin sensitivity, mitochondrial activity, and gene expression changes in these mouse st...

  18. Up-Regulation of Mitochondrial Activity and Acquirement of Brown Adipose Tissue-Like Property in the White Adipose Tissue of Fsp27 Deficient Mice

    Shen Yon Toh; Jingyi Gong; Guoli Du; John Zhong Li; Shuqun Yang; Jing Ye; Huilan Yao; Yinxin Zhang; Bofu Xue; Qing Li; Hongyuan Yang; Zilong Wen; Peng Li

    2008-01-01

    Fsp27, a member of the Cide family proteins, was shown to localize to lipid droplet and promote lipid storage in adipocytes. We aimed to understand the biological role of Fsp27 in regulating adipose tissue differentiation, insulin sensitivity and energy balance. Fsp27(-/-) mice and Fsp27/lep double deficient mice were generated and we examined the adiposity, whole body metabolism, BAT and WAT morphology, insulin sensitivity, mitochondrial activity, and gene expression changes in these mouse s...

  19. Relative Contribution of Dengue IgG Antibodies Acquired during Gestation or Breastfeeding in Mediating Dengue Disease Enhancement and Protection in Type I Interferon Receptor-Deficient Mice.

    Lee, Pei Xuan; Ong, Li Ching; Libau, Eshele Anak; Alonso, Sylvie

    2016-06-01

    Dengue virus (DENV) causes a spectrum of diseases ranging from self-limiting dengue fever to severe conditions such as haemorrhagic fever and dengue shock syndrome. Antibody-dependent enhancement (ADE) is thought to explain the occurrence of severe dengue whereby pre-existing binding but non-neutralising antibodies enhance DENV infection. The ADE phenomenon is supported by epidemiological findings that infants that born to dengue immune mothers are at greater risk to develop severe dengue upon primary infection. The role of maternally acquired dengue-specific antibodies in disease enhancement was recently recapitulated in a mouse model where mice born to DENV1-immune mothers experienced enhanced disease severity upon DENV2 infection. Here, this study investigates the relative contribution of maternal dengue-specific antibodies acquired during gestation and breastfeeding in dengue disease. Using a surrogate breastfeeding mother experimental approach, we showed that majority of the maternal dengue-specific antibodies were acquired during breastfeeding and conferred an extended enhancement window. On the other hand, in the context of homologous infection, breastfeeding conferred protection. Furthermore, measurement of dengue-specific antibody titres over time in mice born to dengue immune mothers revealed a biphasic pattern of antibody decay as reported in humans. Our work provides evidence of the potential contribution of breast milk-acquired dengue-specific IgG antibodies in enhancement and protection against dengue. Should such contribution be established in humans as well, it may have important implications for the development of guidelines to dengue-immune breastfeeding mothers. PMID:27341339

  20. Relative Contribution of Dengue IgG Antibodies Acquired during Gestation or Breastfeeding in Mediating Dengue Disease Enhancement and Protection in Type I Interferon Receptor-Deficient Mice

    Lee, Pei Xuan; Ong, Li Ching; Libau, Eshele Anak; Alonso, Sylvie

    2016-01-01

    Dengue virus (DENV) causes a spectrum of diseases ranging from self-limiting dengue fever to severe conditions such as haemorrhagic fever and dengue shock syndrome. Antibody-dependent enhancement (ADE) is thought to explain the occurrence of severe dengue whereby pre-existing binding but non-neutralising antibodies enhance DENV infection. The ADE phenomenon is supported by epidemiological findings that infants that born to dengue immune mothers are at greater risk to develop severe dengue upon primary infection. The role of maternally acquired dengue-specific antibodies in disease enhancement was recently recapitulated in a mouse model where mice born to DENV1-immune mothers experienced enhanced disease severity upon DENV2 infection. Here, this study investigates the relative contribution of maternal dengue-specific antibodies acquired during gestation and breastfeeding in dengue disease. Using a surrogate breastfeeding mother experimental approach, we showed that majority of the maternal dengue-specific antibodies were acquired during breastfeeding and conferred an extended enhancement window. On the other hand, in the context of homologous infection, breastfeeding conferred protection. Furthermore, measurement of dengue-specific antibody titres over time in mice born to dengue immune mothers revealed a biphasic pattern of antibody decay as reported in humans. Our work provides evidence of the potential contribution of breast milk-acquired dengue-specific IgG antibodies in enhancement and protection against dengue. Should such contribution be established in humans as well, it may have important implications for the development of guidelines to dengue-immune breastfeeding mothers. PMID:27341339

  1. Up-regulation of mitochondrial activity and acquirement of brown adipose tissue-like property in the white adipose tissue of fsp27 deficient mice.

    Shen Yon Toh

    Full Text Available Fsp27, a member of the Cide family proteins, was shown to localize to lipid droplet and promote lipid storage in adipocytes. We aimed to understand the biological role of Fsp27 in regulating adipose tissue differentiation, insulin sensitivity and energy balance. Fsp27(-/- mice and Fsp27/lep double deficient mice were generated and we examined the adiposity, whole body metabolism, BAT and WAT morphology, insulin sensitivity, mitochondrial activity, and gene expression changes in these mouse strains. Furthermore, we isolated mouse embryonic fibroblasts (MEFs from wildtype and Fsp27(-/- mice, followed by their differentiation into adipocytes in vitro. We found that Fsp27 is expressed in both brown adipose tissue (BAT and white adipose tissue (WAT and its levels were significantly elevated in the WAT and liver of leptin-deficient ob/ob mice. Fsp27(-/- mice had increased energy expenditure, lower levels of plasma triglycerides and free fatty acids. Furthermore, Fsp27(-/-and Fsp27/lep double-deficient mice are resistant to diet-induced obesity and display increased insulin sensitivity. Moreover, white adipocytes in Fsp27(-/- mice have reduced triglycerides accumulation and smaller lipid droplets, while levels of mitochondrial proteins, mitochondrial size and activity are dramatically increased. We further demonstrated that BAT-specific genes and key metabolic controlling factors such as FoxC2, PPAR and PGC1alpha were all markedly upregulated. In contrast, factors inhibiting BAT differentiation such as Rb, p107 and RIP140 were down-regulated in the WAT of Fsp27(-/- mice. Remarkably, Fsp27(-/- MEFs differentiated in vitro show many brown adipocyte characteristics in the presence of the thyroid hormone triiodothyronine (T3. Our data thus suggest that Fsp27 acts as a novel regulator in vivo to control WAT identity, mitochondrial activity and insulin sensitivity.

  2. The Massive Bleeding after the Operation of Hip Joint Surgery with the Acquired Haemorrhagic Coagulation Factor XIII(13) Deficiency: Two Case Reports.

    Kanda, Akio; Kaneko, Kazuo; Obayashi, Osamu; Mogami, Atsuhiko

    2013-01-01

    Two women, aged 81 and 61, became haemorrhagic after surgery. Their previous surgeries were uneventful with no unexpected bleeding observed. Blood tests prior to the current surgeries indicated normal values including those related to coagulation. There were no problems with the current surgeries prior to leaving the operating room. At 3 hours after the surgery, the 81-year-old patient had an outflow of the drain at 1290 grams and her blood pressure decreased. She had disseminated intravascular coagulation (DIC). The 61-year-old woman had repeated haemorrhages after her current surgery for a long time. Their abnormal haemorrhages were caused by a deficiency of coagulation factor XIII(13). The mechanism of haemorrhagic coagulation factor XIII(13) deficiency is not understood, and it is a rare disorder. The only diagnostic method to detect this disorder is to measure factor XIII(13) activity in the blood. In this paper, we used Arabic and Roman numerals at the same time to avoid confusion of coagulation factor XIII(13) with coagulation factor VIII(8) that causes hemophilia A. PMID:23533879

  3. The Massive Bleeding after the Operation of Hip Joint Surgery with the Acquired Haemorrhagic Coagulation Factor XIII(13 Deficiency: Two Case Reports

    Akio Kanda

    2013-01-01

    Full Text Available Two women, aged 81 and 61, became haemorrhagic after surgery. Their previous surgeries were uneventful with no unexpected bleeding observed. Blood tests prior to the current surgeries indicated normal values including those related to coagulation. There were no problems with the current surgeries prior to leaving the operating room. At 3 hours after the surgery, the 81-year-old patient had an outflow of the drain at 1290 grams and her blood pressure decreased. She had disseminated intravascular coagulation (DIC. The 61-year-old woman had repeated haemorrhages after her current surgery for a long time. Their abnormal haemorrhages were caused by a deficiency of coagulation factor XIII(13. The mechanism of haemorrhagic coagulation factor XIII(13 deficiency is not understood, and it is a rare disorder. The only diagnostic method to detect this disorder is to measure factor XIII(13 activity in the blood. In this paper, we used Arabic and Roman numerals at the same time to avoid confusion of coagulation factor XIII(13 with coagulation factor VIII(8 that causes hemophilia A.

  4. The First Report of Multiple Myeloma with Acquired Factor Ⅺ and Ⅸ Deficiency%多发性骨髓瘤并获得性凝血因子Ⅺ及Ⅸ缺乏首例报告

    于阳; 王轩; 包芳; 吴学宾

    2011-01-01

    目的 探讨多发性骨髓瘤并获得性凝血因子Ⅺ及Ⅸ缺乏的诊治要点.方法 回顾性分析我院确诊的多发性骨髓瘤并获得性凝血因子Ⅺ及Ⅸ缺乏1例的临床资料.结果 患者因胸痛1个月,发热3d入院.入院后出现皮肤穿刺处大量出血,检查发现凝血功能异常,凝血因子活性Ⅺ:C 0.18,Ⅸ:C 0.49,血狼疮抗凝物(LA)88.1 s,骨髓及胸腔积液检查可见幼稚浆细胞,确诊为多发性骨髓瘤伴获得性凝血因子Ⅺ及Ⅸ缺乏.采用包括糖皮质激素的化疗方案后,患者病情得到有效控制.结论 对获得性凝血因子缺乏的患者,应警惕为恶性肿瘤产生循环抗凝物所致.积极治疗原发性疾病是彻底纠正获得性凝血功能异常的关键措施.%Objective To explore the main points of diagnosis and treatment of multiple myeloma associated with co agulation disorder. Methods The clinical characteristics and laboratory results of 1 patient with multiple myeloma associated with acquired factor XI and K deficiency was reported,and the literature was reviewed. Results The patient suffered from chest pain for 1 month, had a high temperature for 3 d, who presented heavy bleeding at the pinprick after hospitalization. Ex amination revealed coagulation disorder, the activity of coagulation factor Ⅺ∶ C was 0.18, Ⅸ∶ C was 0.49, blood lupus anti coagulant was 88.1 s. Proplasmacytes were founded in bone marrow and pleural effusion. The patient was diagnosed as having multiple myeloma associated with acquired factor Ⅺ and Ⅸ deficiency. After the treatment of chemotherapy with glucocorti coid,the disease was effectively controlled. Conclusion Acquired coagulation factor deficiency may be caused by malignan cies which may result in circulating anticoagulant. Intensive approach to treat the original disease is the pivotal measure to cor rect the coagulation disorder.

  5. Immunomodulatory and Antioxidant Effects of Purple Sweet Potato Extract in LP-BM5 Murine Leukemia Virus-Induced Murine Acquired Immune Deficiency Syndrome.

    Kim, Ok-Kyung; Nam, Da-Eun; Yoon, Ho-Geun; Baek, Sun Jung; Jun, Woojin; Lee, Jeongmin

    2015-08-01

    The immunomodulatory effects of a dietary supplement of purple sweet potato extract (PSPE) in LP-BM5 murine leukemia virus (MuLV)-induced immune-deficient mice were investigated. Mice were divided into six groups: normal control, infected control (LP-BM5 MuLV infection), positive control (LP-BM5 MuLV infection+dietary supplement of red ginseng 300 mg/kg), purple sweet potato water extract (PSPWE) (LP-BM5 MuLV infection+dietary supplement of PSPE 300 mg/kg), PSP10EE (LP-BM5 MuLV infection+dietary supplement of 10% ethanol PSPE 300 mg/kg), and PSP80EE (LP-BM5 MuLV infection+dietary supplement of 80% ethanol PSPE 300 mg/kg). Dietary supplementation began on the day of LP-BM5 MuLV infection and continued for 12 weeks. Dietary supplementation of PSPE inhibited LP-BM5 MuLV-induced splenomegaly and lymphadenopathy and attenuated the suppression of T- and B-cell proliferation and T helper 1/T helper 2 cytokine imbalance in LP-BM5 MuLV-infected mice. Dietary supplement of PSPE increased the activity of the antioxidant enzymes, superoxide dismutase and glutathione peroxidase. The data suggest that PSPE may ameliorate immune dysfunction due to LP-BM5 MuLV infection by modulating antioxidant defense systems. PMID:26076116

  6. 获得性凝血因子Ⅹ缺乏症三例报告并文献复习%Acquired coagulation factor Ⅹ deficiency: three cases report and literature review

    刘文洁; 宣旻; 薛峰; 杨仁池

    2014-01-01

    目的 加深对获得性凝血因子Ⅹ缺乏症的认识.方法 对3例获得性凝血因子Ⅹ缺乏症患者的临床资料进行分析,并复习相关文献.结果 例1,男,57岁,诊断为多发性骨髓瘤轻链型、继发性淀粉样变、获得性凝血因子Ⅹ缺乏症,表现为自发性皮肤黏膜出血,凝血因子Ⅹ活性(FⅩ∶C)1.8%,予以MP(马法兰+曲安西龙)方案联合沙利度胺及对症治疗,FⅩ∶C未见升高,因原发病进展死亡.例2,男,41岁,以颅内出血入院,FⅩ∶C 26.8%,予以补充叶酸、维生素B12、维生素K,并输注红细胞、血小板及新鲜冰冻血浆治疗,颅内出血好转.例3,女,63岁,因反复发作四肢关节出血4个月入院,FⅩ∶C 6.1%,给予凝血酶原复合物、甲泼尼龙、硫唑嘌呤、利妥昔单抗治疗,FⅩ∶C未见明显升高,关节腔出血仍反复发作.结论 获得性凝血因子Ⅹ缺乏症临床表现具有异质性,诊断依赖病史和实验室检查,治疗包括控制出血和治疗原发病,预后与患者基础疾病相关.%Objective To deepen the understanding of acquired coagulation factor Ⅹ (F Ⅹ) deficiency.Methods The clinical data of 3 patients were analyzed and related literature were reviewed.Results Case 1,a 57-year-old male,secondary to multiple myeloma and amyloidosis,was presented with spontaneous mucous hemorrhage with the level of F Ⅹ ∶ C 1.8%,which kept unchanged after chemotherapy with melphalan,glucocorticoid,and thalidomide,and died of primary disease progression.Case 2,a 41-year-old male with psoriasis,was presented with cerebral and retinal hemorrhage with the level of F Ⅹ ∶ C 26.8%.The signs of hemorrhage were alleviated after the supplement of folic acid,vitamin B12,and vitamin K,and transfusion with red blood cells,platelets,and fresh frozen plasma.Case 3,a 63-year-old female,associated with high level of lupus anticoagulant,was presented with repeated ecchymosis and haemarthrosis with the level of F Ⅹ ∶C 6.1

  7. Acquired Techniques

    Lunde Nielsen, Espen; Halse, Karianne

    2013-01-01

    Acquired Techniques - a Leap into the Archive, at Aarhus School of Architecture. In collaboration with Karianne Halse, James Martin and Mika K. Friis. Following the footsteps of past travelers this is a journey into tools and techniques of the architectural process. The workshop will focus upon...

  8. Acquired blepharoptosis

    Oosterhuis, HJGH

    1996-01-01

    A review is given of the aetiology and possible treatment of acquired (non-congenital) blepharoptosis, which is a common but not specific sign of neurological disease: The diagnostic categories of upper eyelid drooping are scheduled as (a) pseudo-ptosis due to a local process or overactivity of eye

  9. Cerebral toxoplasmosis in acquired immuno deficiency syndrome

    Authors report a case of fatal CNS toxoplasmosis in a young homosexual man suffering from Kaposi angio-sacroma. This paper is principally concerned with CT scan and neuropathological correlations. (orig.)

  10. Behcet's disease in acquired immune deficiency syndrome

    Beenish Siddiqui

    2016-01-01

    Full Text Available HIV/AIDS patients often present with orogenital ulcers. In the immunocompromised patient diagnosis of these ulcers pose a challenge, as there is a myriad of etiologies. We present a case of an HIV/AIDS patient with recurrent orogenital aphthosis that was confirmed to have concomitant diagnosis of Behcet's disease. Proper awareness of the causes of these ulcers is essential for prompt and effective treatment. While rare causes may be at the bottom of a differential list in an immunocompetent host, when HIV/AIDS is involved these rare causes often percolate to the top.

  11. FLASH形式在中学生预防艾滋病教育活动中的作用%Effects of FLASH on health education of acquired immune deficiency syndrome in middle school students

    韩娟; 赵丽娜; 时俊新; 杨森焙; 宋阎超; 陈宏平

    2009-01-01

    目的:探讨FLASH形式在预防艾滋病教育活动中的作用,了解中学生对预防艾滋病的相关知识和态度.方法:在湖北省大冶市和宜昌市秭归县的4所中学采用分层整群抽样的方法抽取在校学生共1 211人,用自制的中学生预防艾滋病生活技能教育的FLASH片进行专题教育,并对其教育前后的预防艾滋病相关知识、态度进行问卷调查.结果:调查对象对艾滋病流行趋势、艾滋病与青少年的关系以及对艾滋病主要传播途径认识,教育后正确回答率提高至80%以上,而对艾滋病的概念以及蚊虫叮咬、同性恋、纹身等认识提高不足80%.结论:中学生仍需加强预防艾滋病知识的教育,教育形式应多样化.FLASH形式生动活泼,教育效果显著,但因其稍纵即逝,会影响教育效果,可作为辅助教材.%Objective: To explore the effects of FLASH on health education of acquired immune deficiency syndrome (AIDS), un-derstand the related knowledge and attitude to AIDS prevention in middle school students. Methods: 1 211 students were selected from 4 middle school by stratified cluster sampling, then health education of AIDS prevention was carried out in the form of self-designed FLASH,the related knowledge and attitude to AIDS prevention of middle school students before and after health education were observed. Results:The accuracy rates of prevailing trend of AIDS, the relationship between AIDS and adolescents, major spread approaches of AIDS after heslth education were increased to over 80%, but the others improved less than 80% such as the meaning of AIDS, mosquito and insect bite, ho-mesexuallty and tattoo and so on. Conclusion: The middle school students still need to strengthen AIDS preventive education which shouldbe multiple modes and be enjoyed by them. FLASH is a lifelike and brisk mode enjoyed by the students which education effect is marked,but it may be an assistant teaching material because it is easy to go in

  12. Acquired causes of intestinal malabsorption.

    van der Heide, F

    2016-04-01

    This review focuses on the acquired causes, diagnosis, and treatment of intestinal malabsorption. Intestinal absorption is a complex process that depends on many variables, including the digestion of nutrients within the intestinal lumen, the absorptive surface of the small intestine, the membrane transport systems, and the epithelial absorptive enzymes. Acquired causes of malabsorption are classified by focussing on the three phases of digestion and absorption: 1) luminal/digestive phase, 2) mucosal/absorptive phase, and 3) transport phase. Most acquired diseases affect the luminal/digestive phase. These include short bowel syndrome, extensive small bowel inflammation, motility disorders, and deficiencies of digestive enzymes or bile salts. Diagnosis depends on symptoms, physical examination, and blood and stool tests. There is no gold standard for the diagnosis of malabsorption. Further testing should be based on the specific clinical context and the suspected underlying disease. Therapy is directed at nutritional support by enteral or parenteral feeding and screening for and supplementation of deficiencies in vitamins and minerals. Early enteral feeding is important for intestinal adaptation in short bowel syndrome. Medicinal treatment options for diarrhoea in malabsorption include loperamide, codeine, cholestyramine, or antibiotics. PMID:27086886

  13. Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy.

    von Auer, Charis; von Krogh, Anne-Sophie; Kremer Hovinga, Johanna A; Lämmle, Bernhard

    2015-02-01

    The complex relation between thrombotic thrombocytopenic purpura (TTP) and pregnancy is concisely reviewed. Pregnancy is a very strong trigger for acute disease manifestation in patients with hereditary TTP caused by double heterozygous or homozygous mutations of ADAMTS13 (ADisintegrin And Metalloprotease with ThromboSpondin type 1 domains, no. 13). In several affected women disease onset during their first pregnancy leads to the diagnosis of hereditary TTP. Without plasma treatment mother and especially fetus are at high risk of dying. The relapse risk during a next pregnancy is almost 100% but regular plasma transfusion starting in early pregnancy will prevent acute TTP flare-up and may result in successful pregnancy outcome. Pregnancy may also constitute a mild risk factor for the onset of acute acquired TTP caused by autoantibody-mediated severe ADAMTS13 deficiency. Women having survived acute acquired TTP may not be at very high risk of TTP relapse during an ensuing next pregnancy but seem to have an elevated risk of preeclampsia. Monitoring of ADAMTS13 activity and inhibitor titre during pregnancy may help to guide management and to avoid disease recurrence. Finally, TTP needs to be distinguished from the much more frequent hypertensive pregnancy complications, preeclampsia and especially HELLP (Hemolysis, Elevated Liver Enzymes, Low Platelet count) syndrome. PMID:25903530

  14. Zinc deficiency and eating disorders.

    Humphries, L; Vivian, B; Stuart, M; McClain, C J

    1989-12-01

    Decreased food intake, a cyclic pattern of eating, and weight loss are major manifestations of zinc deficiency. In this study, zinc status was evaluated in 62 patients with bulimia and 24 patients with anorexia nervosa. Forty percent of patients with bulimia and 54% of those with anorexia nervosa had biochemical evidence of zinc deficiency. The authors suggest that for a variety of reasons, such as lower dietary intake of zinc, impaired zinc absorption, vomiting, diarrhea, and binging on low-zinc foods, patients with eating disorders may develop zinc deficiency. This acquired zinc deficiency could then add to the chronicity of altered eating behavior in those patients. PMID:2600063

  15. Epidemiological Characteristics of Acquired Immune Deficiency Syndrome in Zhoukou, He′nan Province From 1995 to 2014%1995-2014年河南省周口市 AIDS 流行特征分析

    张林

    2015-01-01

    势=1419.0, P<0.01),配偶/固定性伴传播占当年累计报告病例的构成比呈下降趋势(χ2趋势=43.6, P<0.01)。男男同性传播自2007年报告首例后,其占当年累计报告病例的构成比呈上升趋势(χ2趋势=132.1, P<0.01)。结论周口市AIDS疫情近年有所抬头,分布范围广,呈聚集性分布。临床相关检测是HIV感染和AIDS患者的重要来源之一,感染途径已由经血传播向性传播转变,非婚异性传播和男男同性传播上升趋势明显,阻断性传播是当前控制AIDS疫情的重要任务。%Objective To investigate the epidemiological characteristics of acquired immune deficiency syndrome (AIDS) in Zhoukou and to provide scientific evidence for the formulation of prevention and control strategies .Methods We collected the data of HIV-infected patients and AIDS patients in Zhoukou from the Information System for AIDS Prevention and Control of China′s Information System for Disease Prevention and Control , with a time range of 1995 to 2014.Results From 1995 to 2014, the total reported number of HIV -infected people and AIDS patients in Zhoukou was 9 187, among which 8 215 (89.42%) were AIDS patents and 3 254 (35.42%) died.After the exclusion of deceased patients , there were 5 933 HIV-infected patients and AIDS patients surviving , accounted for 67.37/105 in all permanent residents of Zhoukou and of which 5 164 were AIDS patients.From 1995 to 2003, with the HIV/AIDS monitoring strengthened , the detected HIV -infected and AIDS patients had been increasing year by year; 2004 witnessed more reported cases than other years , for HIV census was carried out on FPDs this year; from 2005 to 2012, the number of reported cases was in a decreasing trend; from 2013 to 2014, the disease showed a slight rebound .From 2006, death cases began to decrease year by year .HIV-infected patients and AIDS patients of Zhoukou distributed in 204 villages or towns in 10 districts or

  16. Hospital-acquired pneumonia

    ... this page: //medlineplus.gov/ency/article/000146.htm Hospital-acquired pneumonia To use the sharing features on this page, please enable JavaScript. Hospital-acquired pneumonia is an infection of the lungs ...

  17. A case of thrombotic thrombocytopenic purpura induced by acute pancreatitis

    Arimoto M

    2012-03-01

    Full Text Available Miyoko Arimoto1, Yutaka Komiyama2, Fumiko Okamae1, Akemi Ichibe1, Setsuko Teranishi1, Hirohiko Tokunaga1, Keiko Nakaya3, Michie Fujiwara3, Manabu Yamaoka4, Shuji Onishi4, Rie Miyamoto5, Naoto Nakamichi5, Shosaku Nomura51Blood Transfusion Unit, Kansai Medical University Takii Hospital, 2Department of Clinical Sciences and Laboratory Medicine, Kansai Medical University, 3Clinical Medical Technology Unit, Kansai Medical University Takii Hospital, 4Blood Transfusion Unit, Kansai Medical University Hirakata Hospital, 5First Department of Internal Medicine, Kansai Medical University, Moriguchi, JapanAbstract: Thrombotic thrombocytopenic purpura (TTP is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. In acquired TTP, especially in secondary TTP with various underlying diseases, the diagnosis is difficult because there are many cases that do not exhibit severe deficiency of ADAMTS13 or raised levels of ADAMST13 inhibitors. It is well known that collagen disease, malignancy, and hematopoietic stem cell transplantation can be underlying conditions that induce TTP. However, TTP induced by acute pancreatitis, as experienced by our patient, has rarely been reported. Our patient completely recovered with treatments using steroids and plasma exchange (PE only. In cases where patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia, the possibility of TTP should be considered. Treatments for TTP including PE should be evaluated as soon as a diagnosis is made.Keywords: thrombotic thrombocytopenic purpura, ADAMTS13, acute pancreatitis, plasma exchange

  18. Multispectral Analysis of Color Vision Deficiency Tests

    Sergejs FOMINS; Ozolinsh, Maris

    2011-01-01

    Color deficiency tests are usually produced by means of polygraphy technologies and help to diagnose the type and severity of the color deficiencies. Due to different factors, as lighting conditions or age of the test, standard characteristics of these tests fail, thus not allowing diagnosing unambiguously the degree of different color deficiency. Multispectral camera was used to acquire the spectral images of the Ishihara and Rabkin pseudoisochromatic plates in the visible spectrum. Spectral...

  19. Laboratory-acquired brucellosis

    Fabiansen, C.; Knudsen, J.D.; Lebech, A.M.

    2008-01-01

    Brucellosis is a rare disease in Denmark. We describe one case of laboratory-acquired brucellosis from an index patient to a laboratory technician following exposure to an infected blood culture in a clinical microbiology laboratory Udgivelsesdato: 2008/6/9......Brucellosis is a rare disease in Denmark. We describe one case of laboratory-acquired brucellosis from an index patient to a laboratory technician following exposure to an infected blood culture in a clinical microbiology laboratory Udgivelsesdato: 2008/6/9...

  20. Acquired Immune Deficiency Syndrome, Risky Sexual Behavior and Abortion

    Marshall H. Medoff

    2012-01-01

    Full Text Available Problem statement: incidence of abortion in the United States has steadily declined since 1990. The question is why? Approach: This study, using multiple linear regression, examines whether women’s unprotected sexual activity is deterred by the risk of contracting AIDS as reflected in decreased abortion rates. Results: The empirical evidence consistently finds that the prevalence of AIDS reduces the risky (unprotected sexual activity of women of childbearing ages 15-44 as reflected in their abortion rates. The empirical results remain robust for the abortion rates of teens ages 15-17 and for teens ages 15-19. Conclusion: The empirical results suggest that the behavioral modification induced by the prevalence of AIDS accounted for 21% of the decrease in abortion rates over the time period 1992-2005.

  1. Experimental oral polio vaccines and acquired immune deficiency syndrome.

    Hooper, E

    2001-06-29

    The simian immunodeficiency virus (SIV) of the common chimpanzee is widely acknowledged as the direct ancestor of HIV-1. There is increasing historical evidence that during the late 1950s, kidneys were routinely excised from central African chimpanzees by scientists who were collaborating with the polio vaccine research of Dr Hilary Koprowski, and sent - inter alia - to vaccine-making laboratories in the USA and Africa, and to unspecified destinations in Belgium. While there is no direct evidence that cells from these kidneys were used as a substrate for growing Dr Koprowski's oral polio vaccines, there is a startling coincidence between places in Africa where his CHAT vaccine was fed, and the first appearances in the world of HIV-1 group M and group-M-related AIDS. Because of the enormous implications of the hypothesis that AIDS may be an unintended iatrogenic (physician-caused) disease, it is almost inevitable that this theory will engender heated opposition from many of those in the scientific establishment, and those with vested interests. PMID:11405924

  2. Acquired Immune Deficiency Syndrome, Risky Sexual Behavior and Abortion

    Marshall H. Medoff

    2012-01-01

    Problem statement: incidence of abortion in the United States has steadily declined since 1990. The question is why? Approach: This study, using multiple linear regression, examines whether womens unprotected sexual activity is deterred by the risk of contracting AIDS as reflected in decreased abortion rates. Results: The empirical evidence consistently finds that the prevalence of AIDS reduces the risky (unprotected) sexual activity of women of childbearing ages 15-44 as reflected in their a...

  3. The retinal lesions of the acquired immune deficiency syndrome.

    Friedman, A H

    1984-01-01

    AIDS is a reliably diagnosed disease that is indicative of an underlying cellular immunodeficiency with no other cause for the disorder. To date over 2000 cases have been reported in North America and Europe and the number is rising. Patients fulfilling the definition for AIDS have included male homosexuals, IV drug abusers of both sexes, people from Haiti, heterosexual partners of AIDS patients, hemophiliacs, and some patients who fit no particular pattern. The etiology has been attributed t...

  4. [New marker in thrombotic thrombocytopenic purpura

    Hillarp, A.; Lindblom, A.; Bjork, P.;

    2008-01-01

    Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased...... concentrations of high molecular weight von Willebrand factor forms and increased platelet aggregation. Measurement of ADAMTS-13 activity is useful for the diagnosis of TTP and may also be relevant as a prognostic test for recurrent TTP Udgivelsesdato: 2008/8/11...

  5. Ny markør ved trombotisk trombocytopenisk purpura

    Gøtze, Jens Peter; Hillarp, Andreas; Lindblom, Anders;

    2008-01-01

    Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased...... concentrations of high molecular weight von Willebrand factor forms and increased platelet aggregation. Measurement of ADAMTS-13 activity is useful for the diagnosis of TTP and may also be relevant as a prognostic test for recurrent TTP. Udgivelsesdato: 2008-Aug-11...

  6. Pneumonia - adults (community acquired)

    ... breathing (respiratory) condition in which there is an infection of the lung. This article covers community-acquired pneumonia (CAP). This type of pneumonia is found in persons who have not recently been in the hospital or another health care facility such as a ...

  7. Etiopathology of acquired cholesteatoma

    Prabodh Karnik

    2011-01-01

    Full Text Available The etiopathology of acquired cholesteatoma has undergone numerous changes over the past 150 years. However, certain facts stand out with clarity. The presence of cytokeratins in acquired cholesteatoma, which are akin to those found in the tympanic membrane and external auditory canal, shows that these are probably the site of origin of acquired cholesteatoma. The cholesteatoma sac also shows its greatest growth at its tympanic membrane attachment into the middle ear. Implantations of squamous epithelium due to trauma or surgery could be another originating factor. The basic pathology is the formation of papillary cones from the tympanic membrane or external auditory canal, which progress from microcholesteatoma to frank cholesteatoma with keratin collections. There is an altered matrix metalloproteinase pathway. Tumor necrosis factor activation with altered wound healing process contributes to the collateral destruction of bone. Trisomy and aneuploidy of chromosome 8 predispose to cholesteatoma formation in affected individuals. In this article, we present the etiopathology of acquired cholesteatoma as it stands today.

  8. Iron deficiency

    Schou, Morten; Bosselmann, Helle; Gaborit, Freja;

    2015-01-01

    BACKGROUND: Both iron deficiency (ID) and cardiovascular biomarkers are associated with a poor outcome in heart failure (HF). The relationship between different cardiovascular biomarkers and ID is unknown, and the true prevalence of ID in an outpatient HF clinic is probably overlooked. OBJECTIVES...... understand iron metabolism in elderly HF patients....

  9. VLCAD deficiency

    Boneh, A; Andresen, B S; Gregersen, N; Ibrahim, M; Tzanakos, N; Peters, H; Yaplito-Lee, J; Pitt, J J

    2006-01-01

    We diagnosed six newborn babies with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) through newborn screening in three years in Victoria (prevalence rate: 1:31,500). We identified seven known and two new mutations in our patients (2/6 homozygotes; 4/6 compound heterozygotes). Blood sa...

  10. Successful rituximab treatment in an elderly patient with recurrent thrombotic thrombocytopenic purpura.

    Matsubara, Etsuko; Yamanouchi, Jun; Hato, Takaaki; Takeuchi, Kazuto; Niiya, Toshiyuki; Yasukawa, Masaki

    2016-07-01

    An 81-year-old man presenting with fever, neurological symptoms, thrombocytopenia, and hemolytic anemia was diagnosed with acquired idiopathic thrombotic thrombocytopenic purpura (TTP). His disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) activity was TTP recurrence based on ADAMTS13 activity TTP in Japan, we report the efficacy and safety of rituximab in an elderly patient with recurrent TTP. We suggest that rituximab therapy should be started as soon as possible for recurrent TTP in patients with high titers of ADAMTS13 inhibitor. PMID:27498731

  11. Acquired cystic kidney disease

    Choyke, P.L. [National Institutes of Health, Bethesda, MD (United States). Dept. of Diagnostic Radiology

    2000-11-01

    Acquired cystic kidney disease (ACKD), also known as acquired renal cystic disease (ARCD,) occurs in patients who are on dialysis for end-stage renal disease. It is generally accepted that ACKD develops as a consequence of sustained uremia and can first manifest even before dialysis is initiated while the patient is still in chronic renal failure. The role of immune suppression, particularly in transplant recipients, in the development of ACKD, is still under investigation. The prevalence of ACKD is directly related to the duration of dialysis and the risk of cancer is directly related to the presence of cysts. Herein we review the current understanding of the pathophysiology and imaging implications of ACKD. (orig.)

  12. Acquired cystic kidney disease

    Acquired cystic kidney disease (ACKD), also known as acquired renal cystic disease (ARCD,) occurs in patients who are on dialysis for end-stage renal disease. It is generally accepted that ACKD develops as a consequence of sustained uremia and can first manifest even before dialysis is initiated while the patient is still in chronic renal failure. The role of immune suppression, particularly in transplant recipients, in the development of ACKD, is still under investigation. The prevalence of ACKD is directly related to the duration of dialysis and the risk of cancer is directly related to the presence of cysts. Herein we review the current understanding of the pathophysiology and imaging implications of ACKD. (orig.)

  13. Analysis of 7 Misdiagnosed Cases of Acquired Immune Deficiency Syndrome with Neurological Symptoms at Onset%以神经系统症状首发的获得性免疫缺陷综合征七例误诊原因分析

    叶茂斌; 陈研林; 叶飞; 张临洪

    2015-01-01

    目的:探讨以神经系统症状首发的获得性免疫缺陷综合征( acquired immune deficiency syndrome, AIDS)的发病机制、误诊原因及防范措施。方法回顾性分析我院2007年1月—2013年12月误诊的以神经系统症状首发的AIDS 7例的临床资料。结果本组6例以头痛、头晕就诊,伴记忆力减退2例,伴腹胀、厌食1例;1例以左上肢麻木就诊。均否认毒品接触史、冶游史及输血史。7例首诊考虑后循环缺血5例,躯体化障碍和脑梗死各1例,给予相应治疗,症状均无缓解,进一步行人类免疫缺陷病毒( human immunodeficiency virus, HIV)抗体定量检查及蛋白印迹确认试验阳性,确诊AIDS,转疾病预防控制中心治疗。结论以神经系统症状首发的AIDS易误诊。耐心细致病史询问、发散诊断思维和及时进行特异性医技检查可减少或避免其误诊误治。%Objective To investigate the first symptom of AIDS in nervous system, and analyze the misdiagnosis cause, and enhance awareness of AIDS. Methods Retrospective analysis of 7 misdiagnosed acquired immune deficiency syndrome ( AIDs) cases with neurological symptoms at onset admitted to our hospital during January 2007 and December 2013 was made. Results There were cases of headaches and dizziness in 6 patients, with memory disorder in 2 patients, with abdominal disten-sion anorexia in 1 patient;Body lack of power in 1 patient. All the patients denied drug exposure history, amusement history and blood transfusion history. In the 7 cases, 5 cases were misdiagnosed as posterior circulation ischemia, 1 case was misdiagnosed as somatization disorder, and 1 case of left upper limb weakness patient was misdiagnosed as cerebral infarction. After corre-sponding treatment there was no sings of relief in symptoms. HIV antibody test result was positive, and validation test later con-firmed AIDS, and then the patients were transferred to the center for disease control and prevention for

  14. Acquired epidermolysis bullosa

    Maricel Sucar Batista; Yanier Serrano García; Taimí Miranda Vergara

    2015-01-01

    Epidermolysis bullosa is a group of diseases or skin disorders genetically transmitted and it is characterized by the appearance of bullae, ulcers and skin wounds. It usually appears at birth or in the first months of life. This is a case of a 72-year-old female patient who comes to the dermatology department with skin lesions of 6 months of evolution. A skin biopsy was performed, taking a sample for direct and indirect immunofluorescence. Acquired epidermolysis bullosa of unknown etiology wa...

  15. Acquired hypertrichosis lanuginosa

    Kumar Pramod

    1993-01-01

    Full Text Available Acquired hypertirichosis lanuginose developed rapidly in a patient with no detectable malignancy. Soft, fine, downy hair growth was noticed on the face, ears, limbs and trunk. Bilaterally symmetrical vitiliginous macules were present on the ear and preauricular region. This case is reported because of its rarity, absence of any detectable malignancy and development of vitiligo, which to our knowledge has not been reported earlier.

  16. AIDS: acquired immunodeficiency syndrome

    Gilmore, N. J.; Beaulieu, R.; Steben, M.; Laverdière, M.

    2002-01-01

    Acquired immunodeficiency syndrome, or AIDS, is a new illness that occurs in previously healthy individuals. It is characterized by immunodeficiency, opportunistic infections and unusual malignant diseases. Life-threatening single or multiple infections with viruses, mycobacteria, fungi or protozoa are common. A rare neoplasm, Kaposi's sarcoma, has developed in approximately one third of patients with AIDS. More than 800 cases of AIDS have been reported in North America, over 24 of them in Ca...

  17. Cerebral involvement in acquired immunodeficiency syndrome (AIDS)

    Involvement of the central nervous system in acquired immune deficiency syndrome (AIDS) is usually due to opportunistic infections; these frequently offer a difficult differential diagnostic problem. Imaging methods play an important part in the elucidation of symptoms. CT and MR findings were analysed in 13 patients with AIDS and neurological symptoms. Some infections of the central nervous system (encephalitis of unknown aetiology, cytomegalic encephalitis, meningitis) may show cerebral atrophy or even no morphological changes. Toxoplasmosis and PML are the most common opportunistic infections typical changes on CT and MR may lead to diagnosis. MR offers advantages compared with CT in its higher sensitivity for the demonstration even of small lesions. (orig.)

  18. [Acquired immunodeficiency syndrome in pediatric patients].

    Molina Moguel, J L; Ruiz Illezcas, R; Forsbach Sánchez, S; Carreño Alvarez, S; Picco Díaz, I

    1990-12-01

    The object of this study was to determine how many of the patients treated at the Pediatric Odontology Clinic, a branch of the Maxillo-Facial Surgery Service at the Veinte de Noviembre Regional Hospital, ISSSTE, are VIH-positive of show serious manifestations of Acquired Immuno-Deficiency Syndrome (AIDS). For such purpose, 100 pediatric patients suffering from different systemic or local diseases were evaluated, the most common being hematological alterations. Results evidenced the presence of VIH in the blood of five of the pediatric subjects, all suffering from Hemophilia. PMID:2132469

  19. [Stable expression and characterization of the von Willebrand factor cleaving protease].

    Ma, Zhenni; Dong, Ningzheng; Zhang, Jingyu; Su, Jian; Wang, Anyou; Ruan, Changgeng

    2010-02-01

    This study was to acquire recombinant protein of von Willebrand factor cleaving protease (ADAMTS13, a disintegrin and metalloprotease with a thromboSpondin type 1 motifs 13), for further studies on its biological function in thrombosis and hemostasis. We transfected the Hela cells with the plasmid pSecTag-ADAMTS13 by lipofectamine. A positive cell cloning was selected by hygromycin-B. The recombinant protein was purified with Ni-NTA agarose column by gradient imidazole. The purity and immune activity of purified products were identified with SDS-PAGE and Western blotting respectively. We also measured the enzymatic activity of recombinant protein (rADAMTS13) by GST-His two-site ELISA assay. The results showed that we successfully constructed Hela cells ADAMTS2-4 which expressed high level of rADAMTS13. We received about 5.8 mg recombinant protein in culture supernantants per liter purified with Ni-NTA column. The protein formed a main lane at the position of 190 kDa with SDS-PAGE and reacted with polyclonal antibody against ADAMTS13 by Western blotting. The amount of rADAMTS13 activity was 6.4 U/mL, according to the normal plasma defined as 1 U/mL. In conclusion, rADAMTS13 protein had high purity, immune activity and good enzymatic activity, which could establish the experimental foundation for further research on biological function and mechanism of this unique metalloprotease. PMID:20432945

  20. Learning-By-Being-Acquired

    Colombo, Massimo G.; Moreira, Solon; Rabbiosi, Larissa

    -categorization theories, we argue that R&D team reorganization increases the acquired inventors’ use of the prior stock of technological knowledge of the acquiring firm after the acquisition. Furthermore, this effect is enhanced if acquired inventors have higher innovation ability relative to their acquiring peers but is...

  1. Acquired von Willebrand Syndrome

    郭涛

    2005-01-01

    @@ Acquired von Willebrand syndrome (AvWS) is kind of bleeding disorder with laboratory findings similar to those in congenital yon Willebrand disease (vWD).AvWS doesn's have any personal or family history of bleeding, but is associated with certain diseases or abnormal conditions or drugs. Although AvWS is being stated as a rare disease, it has gained more and more attention during the past years. Not because of the severity of the disease, but it is more common than we thought and most patients don' t have a proper diagnosis.

  2. Acquired epidermolysis bullosa

    Maricel Sucar Batista

    2015-12-01

    Full Text Available Epidermolysis bullosa is a group of diseases or skin disorders genetically transmitted and it is characterized by the appearance of bullae, ulcers and skin wounds. It usually appears at birth or in the first months of life. This is a case of a 72-year-old female patient who comes to the dermatology department with skin lesions of 6 months of evolution. A skin biopsy was performed, taking a sample for direct and indirect immunofluorescence. Acquired epidermolysis bullosa of unknown etiology was diagnosed. Treatment was started with low-dose colchicine to increase it later, according to the patient’s tolerance and disease progression.

  3. Acquired Blaschkoid dermatitis

    Mercy P

    2007-01-01

    Full Text Available Acquired Blaschkoid dermatitis characterised by unilateral relapsing inflammatory disease along the lines of Blaschko. A 40-year-old Indian male presented with unilateral erythematous, itchy grouped papules on the left side of the chest, abdomen, back and left arm of 15 days duration. The eruption stopped abruptly at the midline of the torso, completely sparing the right side of the body. The lesions were arranged in whorls and streaks corresponding to the lines of Blaschko. Skin biopsy showed hyperkeratosis and features suggestive of sub-acute spongiotic dermatitis with lymphocytic infiltrate around the blood vessels in the dermis. Patient was diagnosed as a case of Blaschkoid dermatitis. To the best of our knowledge, this is the first case of this condition being reported from India.

  4. Genetics Home Reference: isolated growth hormone deficiency

    ... deficiency dwarfism, pituitary growth hormone deficiency dwarfism isolated GH deficiency isolated HGH deficiency isolated human growth hormone deficiency isolated somatotropin deficiency isolated somatotropin deficiency disorder ...

  5. Acquired night blindness due to bad eating patterns.

    Parafita-Fernández, A; Escalona-Fermín, M M; Sampil, M; Moraña, N; Viso, E; Fernández-Vila, P C

    2015-06-01

    We report a case of acquired night blindness in a developed country (Spain) without risk factors for nutritional deficiency disease or family history of hereditary retinal disease. A 76-year-old woman presented with acquired night blindness of 6-month progression. After a thorough inquiry about eating patterns she becomes suspicious of vitamin A low dietary intake, which is analytically confirmed and successfully treated. Despite being very uncommon in our environment and even more in patients without digestive problems, in a patient reporting acquired night blindness vitamin A deficiency should not be discarded until eating patterns have been investigated. It might be especially relevant in certain socioeconomic situations and eating disorders such as bulimia or anorexia nervosa. PMID:25804276

  6. Psychological change of patients with human immunodeficiency virus infection or acquired immune deficiency syndrome%艾滋病患者及人类免疫缺陷病毒感染者心理健康状况及其影响因素

    王建宁; 屈文妍

    2010-01-01

    目的 了解艾滋病患者及人类免疫缺陷病毒(HIV)感染者心理健康状况,并探讨其影响因素.方法 确诊艾滋病患者及HIV检测阳性者共94例为研究组,38名健康人作为对照组,对二者进行症状自评量表(SCL-90)、社会支持量表、应付方式问卷(CSQ)及艾森克人格问卷(EPQ)测评.结果 研究组SCL-90的强迫、抑郁、焦虑及精神病因子分显著高于对照组(P<0.05或0.01),研究组内部男性抑郁因子分和偏执因子分均高于女性,其SCL-90多个因子与CSQ和EPQ多个因子之间存在显著相关关系,相关系数在0.21~0.70之间.结论 艾滋病患者及HIV感染者心理健康状况不良,以情绪障碍为主,应进行心理社会支持和治疗.%Objective To investigate the mental health status of adults with human immunodeficiency virus (HIV)infection or acquired immune deficiency syndrome(AIDS).Methods Ninety-four patients with HIV infection or AIDS and 38 healthy subjects were enrolled and filled in the Symptom Checklist 90(SCL-90),Coping Styles Questionnaire(CSQ)and Eysenck Personality Questionnaire(EPQ).Results in SCL-90,the scores of compulsion ,depression,anxiety and psychoticism factor were significantly increased in the patient group(all P<0.05).In the study group,the scores of depression and paranoid ideation factors of male patients were higher than those of female patients.For the participants with HIV infection or AIDS,several factors of SCL-90 were correlative with CSQ and EPQ(r=0.21 to 0.70).Conclusion Emotional disorders may be the most common mental problem of patients with HIV infection or AIDS,and social and psychological supports should be needed.

  7. A case of refractory thrombotic thrombocytopenic purpura treated with plasmapheresis and rituximab.

    Kirui, Nicholas; Sokwala, Ahmed

    2016-07-01

     Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder with no prevalence or incidence studies in sub-Saharan Africa. Acquired TTP has several causes, all of which lead to decreased activity of von Willebrand factor cleaving protease (ADAMTS13) due to autoantibodies that are directed towards ADAMTS13. We report a case of a 46-year-old man who presented with most of the classic clinical manifestations of TTP. PMID:27384362

  8. Intercontrole acquiring by Framatome

    The Framatome group, as the worldwide leader in nuclear power plant construction, has reinforced his competences in nuclear services thanks to the acquiring of the Intercontrole company, specialized in non-destructive testing in nuclear and industrial environments. After a presentation of the functioning principle and of the safety aspects of a PWR reactor, this press dossier presents in a first part the role of nuclear services and in particular of non-destructive testing in nuclear power plants (in-service inspection, regulatory aspects, testing processes). This part is illustrated with some examples of inspection performed on some components of the primary coolant loop (steam generators, reactor vessel, pressurizer, pipes, primary pumps). A second part presents the technical centres and units of Framatome in charge of performing non-destructive inspections, while a third part describes the industrial policy and strategy of the group in this domain (market of nuclear park maintenance in France, in the USA and worldwide, creation of the 'inspection and control' centre of Framatome). A last part presents the activities of the Intercontrole company and of its daughter companies with some examples of actions realized in the nuclear and natural gas domains. (J.S.)

  9. Iron-Deficiency Anemia

    Full Text Available ... Deficiency Anemia Explore Iron-Deficiency Anemia What Is... CAUSES WHO IS AT RISK SIGNS & SYMPTOMS DIAGNOSIS TREATMENTS ... less hemoglobin than normal. Iron-deficiency anemia can cause fatigue (tiredness), shortness of breath, chest pain, and ...

  10. Qualitative study on the protective factors of resilience among children in rural areas in acquired immune deficiency syndrome(HIV/AIDS)-Affected families%我国部分农村地区受艾滋病影响儿童心理弹性的保护性因素研究

    史从戎; 张曼华; 王宇; 来源; 高保兴; 乔晓春

    2011-01-01

    [目的]调查山西省某艾滋病比较集中的地区,探讨该区受艾滋病影响儿童心理弹性的保护性因素.[方法]采用儿童焦虑性情绪障碍筛查表和儿童抑郁障碍自评量表,将受艾滋病影响儿童筛分为心理弹性组与非弹性组,再采用半结构访谈的方法,对弹性组的儿童进行调查. [结果]受艾滋病影响儿童心理弹性的保护性因素可划分为四个因素.家庭因素包括父母的关心和支持、民主的教育方式、父母的榜样力量;学校包括老师的关心、同伴的支持;认知包括对父母的评价、相信自己、对未来的期望;行为包括倾诉、寻求帮助、感恩与回报. [结论]在对受艾滋病影响儿童进行心理干预时,应综合考虑家庭、学校、认知、行为等因素.促进其心理弹性的发展,增进其心理健康.%[Objective] To explore the protective factors of resilience among children in acquired immune deficiency syndrome(HIV/AIDS)-Affected families in Shanxi. [Methods] Children in HIV/AIDS families were allocated into resilience group and non-resilience group by The Screen for Child Anxiety Related Emotional Disorders and Depression Self-rating Scale for Children. Then semi-structured interviews were conducted among the resilience group. [Results] The protective factors of resilience among children include four factors. The factor of family consists of care of parents, democratic family education and a good example of parents. Care of teachers and support of peers were included in the factor of school.Estimate of parents, belief in themselves and expectation for the future were organized into the factors of cognition. Pouring out, searching for help and thanksgiving were packed in the factors of behaviors. [Conclusion] Family, school, cognition and behavior should be considered comprehensively in the psychological intervention for the children in HIV/AIDS family to make them more resilient and more healthy.

  11. Carnitine Deficiency and Pregnancy

    Anouk de Bruyn; Yves Jacquemyn; Kristof Kinget; François Eyskens

    2015-01-01

    We present two cases of carnitine deficiency in pregnancy. In our first case, systematic screening revealed L-carnitine deficiency in the first born of an asymptomatic mother. In the course of her second pregnancy, maternal carnitine levels showed a deficiency as well. In a second case, a mother known with carnitine deficiency under supplementation was followed throughout her pregnancy. Both pregnancies had an uneventful outcome. Because carnitine deficiency can have serious complications, su...

  12. Severe Dermatophytosis and Acquired or Innate Immunodeficiency: A Review

    Claire Rouzaud; Roderick Hay; Olivier Chosidow; Nicolas Dupin; Anne Puel; Olivier Lortholary; Fanny Lanternier

    2015-01-01

    Dermatophytes are keratinophilic fungi responsible for benign and common forms of infection worldwide. However, they can lead to rare and severe diseases in immunocompromised patients. Severe forms include extensive and/or invasive dermatophytosis, i.e., deep dermatophytosis and Majocchi’s granuloma. They are reported in immunocompromised hosts with primary (autosomal recessive CARD9 deficiency) or acquired (solid organ transplantation, autoimmune diseases requiring immunosuppressive treatmen...

  13. High-resolution epitope mapping by HX MS reveals the pathogenic mechanism and a possible therapy for autoimmune TTP syndrome.

    Casina, Veronica C; Hu, Wenbing; Mao, Jian-Hua; Lu, Rui-Nan; Hanby, Hayley A; Pickens, Brandy; Kan, Zhong-Yuan; Lim, Woon K; Mayne, Leland; Ostertag, Eric M; Kacir, Stephen; Siegel, Don L; Englander, S Walter; Zheng, X Long

    2015-08-01

    Acquired thrombotic thrombocytopenic purpura (TTP), a thrombotic disorder that is fatal in almost all cases if not treated promptly, is primarily caused by IgG-type autoantibodies that inhibit the ability of the ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) metalloprotease to cleave von Willebrand factor (VWF). Because the mechanism of autoantibody-mediated inhibition of ADAMTS13 activity is not known, the only effective therapy so far is repeated whole-body plasma exchange. We used hydrogen-deuterium exchange mass spectrometry (HX MS) to determine the ADAMTS13 binding epitope for three representative human monoclonal autoantibodies, isolated from TTP patients by phage display as tethered single-chain fragments of the variable regions (scFvs). All three scFvs bind the same conformationally discontinuous epitopic region on five small solvent-exposed loops in the spacer domain of ADAMTS13. The same epitopic region is also bound by most polyclonal IgG autoantibodies in 23 TTP patients that we tested. The ability of ADAMTS13 to proteolyze VWF is impaired by the binding of autoantibodies at the epitopic loops in the spacer domain, by the deletion of individual epitopic loops, and by some local mutations. Structural considerations and HX MS results rule out any disruptive structure change effect in the distant ADAMTS13 metalloprotease domain. Instead, it appears that the same ADAMTS13 loop segments that bind the autoantibodies are also responsible for correct binding to the VWF substrate. If so, the autoantibodies must prevent VWF proteolysis simply by physically blocking normal ADAMTS13 to VWF interaction. These results point to the mechanism for autoantibody action and an avenue for therapeutic intervention. PMID:26203127

  14. And the Winner is - Acquired

    Henkel, Joachim; Rønde, Thomas; Wagner, Marcus

    2015-01-01

    New entrants to a market tend to be superior to incumbents in originating radical innovations. We provide a new explanation for this phenomenon, based on markets for technology. It applies in industries where successful entrepreneurial firms, or their technologies, are acquired by incumbents that...... lower probability accompanies higher value in case of success—that is, a more radical innovation. In the second stage, successful entrants bid to be acquired by the incumbent. We assume that entrants cannot survive on their own, so being acquired amounts to a prize in a contest. We identify an...

  15. Study of genotypic resistance mutations of protease and reverse transcriptase in non-therapeutic acquired immune deficiency syndrome patients in Heiiongjiang Province%黑龙江省未经治疗的艾滋病患者蛋白酶和反转录酶基因型耐药性检测与分析

    周慧; 王福祥; 周海舟; 马英骥

    2010-01-01

    Objective To study the presence of drug resistant mutations of protease and reverse transcriptase among human immunodeficiency virus (HIV)-1 strains isolated from treatment naive HIV/ acquired immune deficiency syndrome (AIDS) patients in Heilongjiang Province of China and to provide the baseline data for starting antiretroviral therapy in this area. Methods The protease and reverse transcriptase gene sequences were amplified by nested-polymerase chain reaction (PCR) and then sequenced. The results were compared to the subtype B consensus amino acid sequence and analyzed with Stanford HIV-db drug resistance sequence interpretation. Results The results showed that HIV strains from 49 patients were classified as subtype B'. No primary mutations associated with protease inhibitor were detected. Some secondary mutations associated with protease inhibitor were detected, which included V77I(91.5%), L63P(76.6%), I93L(74.5%), E35D(61.7%), R57K (19.1%), R41K(10.6%), A71V(8.5%), M36I(8.5%), L10I(6.4%), D60E(6.4%), L89M (4.2%) and G16E(2. 1%). Only one case had a primary mutation M184I that was associated with resistance to reverse transcriptase inhibitors. However, many secondary mutations associated with resistance to reverse transcriptase inhibitors were found, including I135L/T/R/V(81.8%), V106I(22.7%), V179D/E(11.4%), R211K(9.1%), L214F(4.5%), V189I(4.5%) and V108I(2. 3%).Conclusions The prevalence of genotypic anti-HIV drug resistance is very low in treatment naive HIV/AIDS patients in Heilongjiang Province. However, closely monitoring on drug resistance mutation is very important for preventing the development and prevalence of multi-drug resistant or cross drug resistant HIV.%目的 对黑龙江省流行的HIV-1毒株的蛋白酶基因及反转录酶基因进行基因型耐药分析,为开展大规模临床抗病毒治疗提供本底数据.方法 采用套式-PCR法扩增黑龙江省内49例HIV-1感染者及AIDS患者外周血单个核细胞中前病毒cDNA的部分

  16. Analysis of the pathology of lymph nodes in 151 acquired immune deficiency syndrome patients with superficial lymphadenectasis%浅表淋巴结肿大的151例艾滋病患者淋巴结病理学分析

    卢祥婵; 邓建宁; 黄爱春; 李雪琴; 牟敏红; 欧汝志; 黄磊; 赵敏

    2011-01-01

    目的 探讨浅表淋巴结肿大的HIV/AIDS患者的淋巴结病理类型及其特点.方法 取浅表淋巴结肿大的151例HIV/AIDS患者的淋巴结组织进行病理学检查,通过HE、抗酸、过碘酸雪夫和淀粉酶消化后过碘酸雪夫染色,光学显微镜下观察,描述和分析其病理结果及其与CD4+T淋巴细胞计数的关系.数据行卡方检验.结果 在151例HIV/AIDS患者的淋巴结病理结果中,表现为良性病变的有145例,占96.0%,表现为恶性肿瘤的有6例,占4.0%,其中结核病72例,淋巴结反应性增生34例,淋巴真菌感染23例(其中青霉病19例),AIDS相关淋巴结病14例,非霍奇金淋巴瘤5例,良性纤维组织细胞病1例,结核伴发真菌感染1例,间叶性恶性肿瘤1例.在所有患者中,CD4+T淋巴细胞计数<100×106/L的有83例,占55.0%.青霉病随着CD4+T淋巴细胞计数的减少,发病率明显升高(x2=7.757,P=0.021).结论 HIV/AIDS患者浅表淋巴结肿大的原因大多数为感染性疾病,主要为结核病及真菌感染,且多数发生在CD4+T淋巴细胞计数低下的患者,最常见的恶性肿瘤为非霍奇金淋巴瘤.%Objective To investigate the pathological types and features of lymph nodes in human immunodeficiency virus(HIV)/acquired immune deficiency syndrome(AIDS)patients with superficial lymphadenectasis.Methods The tissues of lymph nodes were obtained from 151 HIV/AIDS patients with superficial lymphadenectasis for pathological examination.The pathological results were observed by light microscope after Hematoxylin-Eosin(HE),acid-fast,periodic acid-Schiff (PAS),and digested-PAS(D-PAS)staining.The pathological results of lymph nodes were described and the correlation between pathological changes and CD4+T lymphocyte count was analyzed.Chisquare test was used for the statistic analysis.Results The benign lesions were found in 145 patients (96.0%),while the malignant tumors were found in 6 patients(4.0%).The pathological findings in the 151 HIV

  17. Folate-deficiency anemia

    ... medlineplus.gov/ency/article/000551.htm Folate-deficiency anemia To use the sharing features on this page, please enable JavaScript. Folate-deficiency anemia is a decrease in red blood cells (anemia) ...

  18. Leukocyte Adhesion Deficiency (LAD)

    ... Content Marketing Share this: Main Content Area Leukocyte Adhesion Deficiency (LAD) LAD is an immune deficiency in ... are slow to heal also may have LAD. Treatment and Research Doctors prescribe antibiotics to prevent and ...

  19. Iron-Deficiency Anemia

    Full Text Available ... Deficiency Anemia What Is... CAUSES WHO IS AT RISK SIGNS & SYMPTOMS DIAGNOSIS TREATMENTS PREVENTION LIVING WITH CLINICAL ... and women are the two groups at highest risk for iron-deficiency anemia. Outlook Doctors usually can ...

  20. Factor V deficiency

    Factor V deficiency is a condition that is passed down through families, which affects the ability of the blood ... These proteins are called blood coagulation factors. Factor V deficiency is caused by a lack of Factor ...

  1. Iron-Deficiency Anemia

    Full Text Available ... and women are the two groups at highest risk for iron-deficiency anemia. Outlook Doctors usually can successfully ... With and Managing Iron-Deficiency Anemia 05/18/2011 This video— ...

  2. Iron-Deficiency Anemia

    Full Text Available ... This Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video— ... treatment. For more information about living with and managing iron-deficiency anemia, go to the Health Topics ...

  3. Familial lipoprotein lipase deficiency

    ... medlineplus.gov/ency/article/000408.htm Familial lipoprotein lipase deficiency To use the sharing features on this page, please enable JavaScript. Familial lipoprotein lipase deficiency is a group of rare genetic disorders ...

  4. Factor VII deficiency

    ... page: //medlineplus.gov/ency/article/000548.htm Factor VII deficiency To use the sharing features on this page, please enable JavaScript. Factor VII (seven) deficiency is a disorder caused by a ...

  5. Iron deficiency and cognition

    Hulthén, Lena

    2003-01-01

    Iron deficiency is the most prevalent nutritional disorder in the world. One of the most worrying consequences of iron deficiency in children is the alteration of behaviour and cognitive performance. In iron-deficient children, striking behavioural changes are observed, such as reduced attention span, reduced emotional responsiveness and low scores on tests of intelligence. Animal studies on nutritional iron deficiency show effects on learning ability that parallel the human studies. Despite ...

  6. Iron-Deficiency Anemia

    Full Text Available ... page from the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a ... Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented ...

  7. Iron-Deficiency Anemia

    Full Text Available ... the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily ... Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the ...

  8. Iron-Deficiency Anemia

    ... the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily ... Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the ...

  9. Acquiring taste in home economics?

    Stenbak Larsen, Christian

    2015-01-01

    that the pupils were encouraged to use their senses: listen to things frying, touch the meat to check if it was done and taste the food in the process of seasoning it. But while some children learned what the teachers expected: to produce well tasting food, others learned to cook very salty and hot food...... appreciated by the group of boys, and others again learned to stick with their idiosyncrasies when pressured by the teacher. Conclusions: Children were acquiring taste in the home economic lessons, but not only the kind of tastes that the teacher had planned for. This leads to reflections on the very complex...... process of taste acquiring and to a call for further research into taste acquiring in complex real life contexts as home economics lessons....

  10. Music-reading deficiencies and the brain

    Cuddy, Lola L.; Sylvie Hébert

    2006-01-01

    This paper reviews the literature on brain damage and music-reading for the past 25 years. Acquired patterns of selective loss and sparing are described, including both the association and dissociation of music and text reading, and association and dissociation among components of music reading. As well, we suggest that developmental music - reading deficiencies may be isolated in a form analogous to developmental dyslexia for text or congenital amusia for auditory music processing. Finally, ...

  11. Multispectral Analysis of Color Vision Deficiency Tests

    Sergejs FOMINS

    2011-03-01

    Full Text Available Color deficiency tests are usually produced by means of polygraphy technologies and help to diagnose the type and severity of the color deficiencies. Due to different factors, as lighting conditions or age of the test, standard characteristics of these tests fail, thus not allowing diagnosing unambiguously the degree of different color deficiency. Multispectral camera was used to acquire the spectral images of the Ishihara and Rabkin pseudoisochromatic plates in the visible spectrum. Spectral data was converted to cone signals, and successive mathematics applied to provide a simple simulation of the test performance. Colorimetric data of the each pixel of the test image can be calculated and distribution of color coordinates is presented.http://dx.doi.org/10.5755/j01.ms.17.1.259

  12. Acquired Aplastic Anemia in Children

    Hartung, Helge D.; Olson, Timothy S.; Bessler, Monica

    2013-01-01

    This article provides a practice-based and concise review of the etiology, diagnosis, and management of acquired aplastic anemia in children. Bone marrow transplantation, immunosuppressive therapy, and supportive care are discussed in detail. The aim is to provide the clinician with a better understanding of the disease and to offer guidelines for the management of children with this uncommon yet serious disorder.

  13. Acquired anhidrosis a case report

    Nair Laxmi

    1992-01-01

    Full Text Available A 30-year -old man was seen for acquired anhidrosis. There was no systemic disease. Vasomotor functions were normal. Biopsy showed normal sweat glands and ducts. Intravenous injection of neostigmine could produce profuse sweating on the face, trunk and arms. The disorder is likely to be due to a peripheral dysautonomia selectively affecting the sudomotor function.

  14. Acquired anhidrosis a case report

    Nair Laxmi; Beena D; Manohar S

    1992-01-01

    A 30-year -old man was seen for acquired anhidrosis. There was no systemic disease. Vasomotor functions were normal. Biopsy showed normal sweat glands and ducts. Intravenous injection of neostigmine could produce profuse sweating on the face, trunk and arms. The disorder is likely to be due to a peripheral dysautonomia selectively affecting the sudomotor function.

  15. Etiology of growth hormone deficiency in children and adolescents

    Mitrović Katarina

    2013-01-01

    Full Text Available Introduction. Growth hormone deficiency (GHD can be isolated or associated with deficiency of other pituitary gland hormones. According to age at diagnosis, causes of GHD are divided into congenital or acquired, and according to etiology into recognized and unknown. Objective. We analyzed etiology and prevalence of GHD, demographic data at birth, age, body height (BH and bone age at diagnosis as well as the frequency of other pituitary hormone deficiencies. Methods. The study involved 164 patients (109 male. The main criterion for the diagnosis of GHD was inadequate response of GH after two stimulation tests. The patients were classified into three groups: idiopathic, congenital and acquired GHD. Results. Idiopathic GHD was confirmed in 57.9% of patients, congenital in 11.6% and acquired in 30.5%. The mean age at diagnosis of GHD was 10.1±4.5 years. The patients with congenital GHD had most severe growth retardation (-3.4±1.4 SDS, while the patients with idiopathic GHD showed most prominent bone delay (-3.6±2.3 SDS. The prevalence of multiple pituitary hormone deficiency was 56.1%, in the group with congenital GHD 73.7%, acquired GHD 54.0% and idiopathic GHD 53.7%. The frequency of thyrotropin deficiency ranged from 88.2-100%, of adrenocorticotrophin 57.1-68.8% and of gonadotrophins deficiency 57.1- 63.0%, while deficiency of antidiuretic hormone was 2.0-25.0%. Conclusion. Although regular BH measurements enable early recognition of growth retardation, patients’ mean age and degree of growth retardation indicate that GHD is still diagnosed relatively late. A high incidence of other pituitary hormone deficiencies requires a detailed investigation of the etiology of disorders and evaluation of all pituitary functions in each child with confirmed GHD.

  16. CNOOC Acquires Oversea Assets Successfully

    Hu Senlin

    2006-01-01

    @@ After last year CNOOC's bidding for buy the US energy company Unocal Corp lost out to the Chevron Corporation, it conducted the crossing-border asset-acquirement again in the beginning of this year. On Jan. 9, 2006,CNOOC Ltd signed a definitive agreement with Nigeria South Atlantic Petroleum Limited (SAPETRO) to acquire a 45 % working interest in an offshore oil developing license OML 130 in Nigeria for US$2.268 billion cash. The purchase will be funded by the internal capital resources of CNOOC Ltd. In which, US$1.75 billion will pay for buying SAPETRO, and the remaining cash will be used to pay for the early operation cost.

  17. Occupationally Acquired American Cutaneous Leishmaniasis

    Maria Edileuza Felinto de Brito

    2012-01-01

    Full Text Available We report two occupationally acquired cases of American cutaneous leishmaniasis (ACL: one accidental laboratory autoinoculation by contaminated needlestick while handling an ACL lesion sample, and one acquired during field studies on bird biology. Polymerase chain reaction (PCR assays of patient lesions were positive for Leishmania, subgenus Viannia. One isolate was obtained by culture (from patient 2 biopsy samples and characterized as Leishmania (Viannia naiffi through an indirect immunofluorescence assay (IFA with species-specific monoclonal antibodies (mAbs and by multilocus enzyme electrophoresis (MLEE. Patients were successfully treated with N-methyl-glucamine. These two cases highlight the potential risks of laboratory and field work and the need to comply with strict biosafety procedures in daily routines. The swab collection method, coupled with PCR detection, has greatly improved ACL laboratory diagnosis.

  18. Cogema acquires TOTAL's uranium assets

    On April 28, France's nuclear fuel cycle conglomerate Cogema and petroleum group TOTAL announced a plan in which Cogema will assume ownership of TOTAL's uranium assets worldwide, and as part of the deal, each firm will acquire shares of the other. On June 2, the agreement will be submitted to shareholders and, assuming it is approved, will go into effect this year. The agreement calls for TOTAL to acquire a 10.8-percent share in Cogema, thus becoming its first private sector shareholder, by underwriting a reserve capital increase of FF1.5 billion (approximately US$283 million). In return, Cogema will pay FF2.52 billion for approximately 4.3 percent of TOTAL, as part of a reserve capital increase totalling FF4.07 billion

  19. Inhibitors against the coagulation factors spontaneously acquired: Acquired B Hemophilia

    Claudia Lucía Sossa Melo; Sara Inés Jiménez Sanguino; Pilar Rodríguez

    2003-01-01

    Spontaneously acquired inhibitors to factor IX, are extremely rare. A 70-year-old male, presented with major continuous post-orthopedic surgery bleeding. His initial APTT was 77.4 s (normal range, 25-36) and normal PT. Expanded APTT corrects, results in favor of deficit of factor IX, confirming the level of dose of IX factor: 52% (NR 70–125%) with normal factor VIII. It was realized with fresh frozen plasma, and by the fifth day of treatment, he presents a bruise in the surgery bed with radic...

  20. Related progress of CT-guided percutaneous biopsy in the thoracic complications of acquired immune deficiency syndrome and occupational exposure prevention%CT引导下经皮穿刺活检术在获得性免疫缺陷综合征胸部并发症中的应用及职业暴露防护相关进展

    王青

    2011-01-01

    CT-guided percutaneous biopsy in the thoracic complications of acquired immunodeficiency syndrome (AIDS)is the main clear diagnosis method.The application of technical solutions, complications and prevention, occupational exposure protection principles and methods of CT-guided percutaneous biopsy in thoracic complications of AIDS were reviewed in this article.%CT引导下经皮穿刺活检术是确诊获得性免疫缺陷综合征(AIDS)胸部并发症的主要方法.本文对CT引导下经皮穿刺活检术在AIDS胸部并发症中应用的技术方案、并发症及预防,职业暴露防护原则及方法进行综述.

  1. Muscle phosphorylase kinase deficiency

    Preisler, N; Orngreen, M C; Echaniz-Laguna, A; Laforet, P; Lonsdorfer-Wolf, E; Doutreleau, S; Geny, B; Akman, H O; Dimauro, S; Vissing, J

    2012-01-01

    To examine metabolism during exercise in 2 patients with muscle phosphorylase kinase (PHK) deficiency and to further define the phenotype of this rare glycogen storage disease (GSD).......To examine metabolism during exercise in 2 patients with muscle phosphorylase kinase (PHK) deficiency and to further define the phenotype of this rare glycogen storage disease (GSD)....

  2. Iron deficiency anemia

    Anemia - iron deficiency ... iron from old red blood cells. Iron deficiency anemia develops when your body's iron stores run low. ... You may have no symptoms if the anemia is mild. Most of the time, ... slowly. Symptoms may include: Feeling weak or tired more often ...

  3. Nutritional iron deficiency

    Zimmermann, M.B.; Hurrell, R.F.

    2007-01-01

    Iron deficiency is one of the leading risk factors for disability and death worldwide, affecting an estimated 2 billion people. Nutritional iron deficiency arises when physiological requirements cannot be met by iron absorption from diet. Dietary iron bioavailability is low in populations consuming

  4. Lipoprotein lipase deficiency.

    Shankar K; Bava H; Shetty J; Joshi M

    1997-01-01

    A rare case of a 3 month old child with lipoprotein lipase deficiency who presented with bronchopneumonia is reported. After noticing lipaemic serum and lipaemia retinalis, a diagnosis of hyperlipoproteinaemia was considered. Lipoprotein lipase deficiency was confirmed with post heparin lipoprotein lipase enzyme activity estimation.

  5. G6PD Deficiency

    Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic disorder that is most common in males. About 1 in 10 African American males in the United States has it. G6PD deficiency mainly affects red blood cells, which carry oxygen ...

  6. Deficiently Extremal Gorenstein Algebras

    Pavinder Singh

    2011-08-01

    The aim of this article is to study the homological properties of deficiently extremal Gorenstein algebras. We prove that if / is an odd deficiently extremal Gorenstein algebra with pure minimal free resolution, then the codimension of / must be odd. As an application, the structure of pure minimal free resolution of a nearly extremal Gorenstein algebra is obtained.

  7. Vitamin deficiencies and excesses

    Vitamins are essential nutrients that must be supplied exogenously either as part of a well balanced diet or as supplements. Deficiency states are uncommon in developed countries except, perhaps, among some food insecure families. In contrast, deficiency states are quite common in many developing ...

  8. Iron-Deficiency Anemia

    Full Text Available ... Blood Tests Blood Transfusion Restless Legs Syndrome Send a link to NHLBI to someone by E-MAIL | ... Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily treated condition that occurs if you ...

  9. Knowledge transfer - Acquiring implicit knowledge

    Many organisations have recognised the problem of experts taking home a huge amount of specific knowledge, which they have gathered in their department, when they leave. The successor is capable only of acquiring explicit expertise because implicit experiences are not documented and therefore no more available. That is why we have started this pilot study in order to try to conserve the above mentioned tacit and implicit knowledge and to make it available to other colleagues. Using a semi-standardised interview we elicit tacit knowledge from the expert and summarise it in a report. This interview-guideline forms the basis of in-depth investigation for implicit knowledge. (author)

  10. Carnitine deficiency disorders in children.

    Stanley, Charles A

    2004-11-01

    Mitochondrial oxidation of long-chain fatty acids provides an important source of energy for the heart as well as for skeletal muscle during prolonged aerobic work and for hepatic ketogenesis during long-term fasting. The carnitine shuttle is responsible for transferring long-chain fatty acids across the barrier of the inner mitochondrial membrane to gain access to the enzymes of beta-oxidation. The shuttle consists of three enzymes (carnitine palmitoyltransferase 1, carnitine acylcarnitine translocase, carnitine palmitoyl-transferase 2) and a small, soluble molecule, carnitine, to transport fatty acids as their long-chain fatty acylcarnitine esters. Carnitine is provided in the diet (animal protein) and also synthesized at low rates from trimethyl-lysine residues generated during protein catabolism. Carnitine turnover rates (300-500 micromol/day) are benefit in genetic or acquired disorders of energy production to improve fatty acid oxidation, to remove accumulated toxic fatty acyl-CoA metabolites, or to restore the balance between free and acyl-CoA. Two disorders have been described in children where the supply of carnitine becomes limiting for fatty acid oxidation: (1) A recessive defect of the muscle/kidney sodium-dependent, plasma membrane carnitine symporter, which presents in infancy with cardiomyopathy or hypoketotic hypoglycemia; treatment with oral carnitine is required for survival. (2) Chronic administration of pivalate-conjugated antibiotics in which excretion of pivaloyl-carnitine can lead to carnitine depletion; tissue levels may become low enough to limit fatty acid oxidation, although no cases of illness due to carnitine deficiency have been described. There is speculation that carnitine supplements might be beneficial in other settings (such as genetic acyl-CoA oxidation defects--"secondary carnitine deficiency", chronic ischemia, hyperalimentation, nutritional carnitine deficiency), but efficacy has not been documented. The formation of abnormal

  11. Thiamine deficiency and delirium.

    Osiezagha, Kenneth; Ali, Shahid; Freeman, C; Barker, Narviar C; Jabeen, Shagufta; Maitra, Sarbani; Olagbemiro, Yetunde; Richie, William; Bailey, Rahn K

    2013-04-01

    Thiamine is an essential vitamin that plays an important role in cellular production of energy from ingested food and enhances normal neuronal actives. Deficiency of this vitamin leads to a very serious clinical condition known as delirium. Studies performed in the United States and other parts of the world have established the link between thiamine deficiency and delirium. This literature review examines the physiology, pathophysiology, predisposing factors, clinical manifestations (e.g., Wernicke's encephalopathy, Wernicke-Korsakoff syndrome, structural and functional brain injuries) and diagnosis of thiamine deficiency and delirium. Current treatment practices are also discussed that may improve patient outcome, which ultimately may result in a reduction in healthcare costs. PMID:23696956

  12. GLUT-1 deficiency without epilepsy - an exceptional case

    Overweg-Plandsoen, WCG; Groener, JEM; Onkenhout, W; Brouwer, OF; Bakker, HD; De Vivo, DC

    2003-01-01

    The GLUT-1 deficiency is a metabolic disorder caused by a defect in glucose transport across the blood-brain barrier as a result of a defect in the glucose-transport protein. Patients present with epileptic seizures, delayed development, ataxia and hypotonia, and in many cases acquired microcephaly.

  13. Complement's participation in acquired immunity

    Nielsen, Claus Henrik; Leslie, Robert Graham Quinton

    2002-01-01

    B cell receptor for antigen (BCR), a complex composed of the iC3b/C3d fragment-binding complement type 2 receptor (CR2, CD21) and its signaling element CD19 and the IgG-binding receptor FcgammaRIIb (CD32). The positive or negative outcome of signaling through this triad is determined by the context...... in which antigen is seen, be it alone or in association with natural or induced antibodies and/or C3-complement fragments. The aim of this review is to describe the present status of our understanding of complement's participation in acquired immunity and the regulation of autoimmune responses.......The preliminary evidence for the involvement of complement in promoting primary humoral responses dates back over a quarter of a century. However, it is only in the course of the past decade or so that the detailed mechanisms underlying complement's influence have been characterized in depth. It is...

  14. Pseudomona pseudomallei community acquired pneumonia

    This is the first published case report en Colombia about pseudomona pseudomallei community acquired pneumonia. This uncommon pathogen is from the epidemiological standpoint a very important one and medical community should be aware to look after it in those patients where no other etiological pathogen is recovered. A brief summary about epidemiology is showed, emphasizing those regions where it can be found. Likewise, comments about the differential diagnosis are important since it should be considered in those patients where tuberculosis is suspected. This is particularly representative for countries with high tuberculosis rates. Furthermore, a microbiological review is shown, emphasizing on isolation techniques, descriptions about therapeutics and other regarding treatment issues according international standards. Finally; a description about the clinical picture, laboratory findings, treatment and evolution of the case reported are shown for discussion

  15. Iron-Deficiency Anemia

    Full Text Available ... Digg. Share this page from the NHLBI on Facebook. Add this link to the NHLBI to my ... such as tiredness, poor skin tone, dizziness, and depression. After her doctor diagnosed her with iron-deficiency ...

  16. Iron-Deficiency Anemia

    Full Text Available ... Topics Anemia Blood Tests Blood Transfusion Restless Legs Syndrome Send a link to NHLBI to someone by ... symptoms. Severe iron-deficiency anemia can lead to heart problems, infections, problems with growth and development in ...

  17. Iron-Deficiency Anemia

    Full Text Available ... symptoms. Severe iron-deficiency anemia can lead to heart problems, infections, problems with growth and development in ... 18/2011 This video—presented by the National Heart, Lung, and Blood Institute, part of the National ...

  18. Iron-Deficiency Anemia

    Full Text Available ... Entire Site Health Topics News & Resources Intramural Research Public Health Topics Education & Awareness Resources Contact The Health ... Severe iron-deficiency anemia can lead to heart problems, infections, problems with growth and development in children, ...

  19. Folate-deficiency anemia

    Folate-deficiency anemia is a decrease in red blood cells (anemia) due to a lack of folate. Folate is a type ... B vitamin. It is also called folic acid. Anemia is a condition in which the body does ...

  20. Sleep Deprivation and Deficiency

    ... page from the NHLBI on Twitter. What Are Sleep Deprivation and Deficiency? Sleep deprivation (DEP-rih-VA- ... Rate This Content: NEXT >> Updated: February 22, 2012 Sleep Infographic Sleep Disorders & Insufficient Sleep: Improving Health through ...

  1. Iron-Deficiency Anemia

    Full Text Available ... Health Topics Education & Awareness Resources Contact The Health Information Center Health Professionals Systematic Evidence Reviews & Clinical Practice ... and see the benefits of treatment. For more information about living with and managing iron-deficiency anemia, ...

  2. Iron-Deficiency Anemia

    Full Text Available ... intravenous iron therapy. Rate This Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the National Heart, Lung, and Blood ...

  3. Iron-Deficiency Anemia

    Full Text Available ... iron-rich protein that carries oxygen from the lungs to the rest of the body. Iron-deficiency ... 2011 This video—presented by the National Heart, Lung, and Blood Institute, part of the National Institutes ...

  4. Iron-Deficiency Anemia

    Full Text Available ... Alerts E-Newsletters About NHLBI Organization NHLBI Director Budget, Planning, & Legislative Advisory Committees Contact Us FAQs Home » ... severity of the condition. Treatments may include dietary changes, medicines, and surgery. Severe iron-deficiency anemia may ...

  5. Iron-Deficiency Anemia

    Full Text Available ... CAUSES WHO IS AT RISK SIGNS & SYMPTOMS DIAGNOSIS TREATMENTS PREVENTION LIVING WITH CLINICAL TRIALS LINKS Related Topics ... Doctors usually can successfully treat iron-deficiency anemia. Treatment will depend on the cause and severity of ...

  6. Acquired vulval lymphangiectases mimicking genital warts

    Sharma Rajeev; Tomar Sudarshan; Chandra Mithilesh

    2002-01-01

    Acquired lymphangiectasia can sometimes occur on the vulva and cause diagnostic difficulties especially if they have a warty appearance. We report a case of acquired vulva I lymphangiectasia which mimicked genital warts.

  7. Acquired vulval lymphangiectases mimicking genital warts

    Sharma Rajeev

    2002-01-01

    Full Text Available Acquired lymphangiectasia can sometimes occur on the vulva and cause diagnostic difficulties especially if they have a warty appearance. We report a case of acquired vulva I lymphangiectasia which mimicked genital warts.

  8. Clinical significance of complement deficiencies.

    Pettigrew, H David; Teuber, Suzanne S; Gershwin, M Eric

    2009-09-01

    The complement system is composed of more than 30 serum and membrane-bound proteins, all of which are needed for normal function of complement in innate and adaptive immunity. Historically, deficiencies within the complement system have been suspected when young children have had recurrent and difficult-to-control infections. As our understanding of the complement system has increased, many other diseases have been attributed to deficiencies within the complement system. Generally, complement deficiencies within the classical pathway lead to increased susceptibility to encapsulated bacterial infections as well as a syndrome resembling systemic lupus erythematosus. Complement deficiencies within the mannose-binding lectin pathway generally lead to increased bacterial infections, and deficiencies within the alternative pathway usually lead to an increased frequency of Neisseria infections. However, factor H deficiency can lead to membranoproliferative glomerulonephritis and hemolytic uremic syndrome. Finally, deficiencies within the terminal complement pathway lead to an increased incidence of Neisseria infections. Two other notable complement-associated deficiencies are complement receptor 3 and 4 deficiency, which result from a deficiency of CD18, a disease known as leukocyte adhesion deficiency type 1, and CD59 deficiency, which causes paroxysmal nocturnal hemoglobinuria. Most inherited deficiencies of the complement system are autosomal recessive, but properidin deficiency is X-linked recessive, deficiency of C1 inhibitor is autosomal dominant, and mannose-binding lectin and factor I deficiencies are autosomal co-dominant. The diversity of clinical manifestations of complement deficiencies reflects the complexity of the complement system. PMID:19758139

  9. Adult growth hormone deficiency

    Vishal Gupta

    2011-01-01

    Adult growth hormone deficiency (AGHD) is being recognized increasingly and has been thought to be associated with premature mortality. Pituitary tumors are the commonest cause for AGHD. Growth hormone deficiency (GHD) has been associated with neuropsychiatric-cognitive, cardiovascular, neuromuscular, metabolic, and skeletal abnormalities. Most of these can be reversed with growth hormone therapy. The insulin tolerance test still remains the gold standard dynamic test to diagnose AGHD. Growth...

  10. Iron deficiency anemia Review

    Yıldız, İnci

    2009-01-01

    Iron deficiency anemia is the most frequent and widespread anemia around the world Its prevalence is increased in infants and adolescent girls The etiologic factors may vary but anemia is essentially related to iron deficient nutrition blood loss and malabsorption Children may have paleness cardiovascular and neurologic impacts of anemia pica epithelial changes as koilonychia glossitis angular stomatitis Treatment is by oral or parenteral supplementation of iron Turk Arch Ped 2009; 44 Suppl: ...

  11. Iron deficiency in Europe.

    Hercberg, S; Preziosi, P; Galan, P

    2001-04-01

    In Europe, iron deficiency is considered to be one of the main nutritional deficiency disorders affecting large fractions of the population, particularly such physiological groups as children, menstruating women and pregnant women. Some factors such as type of contraception in women, blood donation or minor pathological blood loss (haemorrhoids, gynaecological bleeding...) considerably increase the difficulty of covering iron needs. Moreover, women, especially adolescents consuming low-energy diets, vegetarians and vegans are at high risk of iron deficiency. Although there is no evidence that an absence of iron stores has any adverse consequences, it does indicate that iron nutrition is borderline, since any further reduction in body iron is associated with a decrease in the level of functional compounds such as haemoglobin. The prevalence of iron-deficient anaemia has slightly decreased in infants and menstruating women. Some positive factors may have contributed to reducing the prevalence of iron-deficiency anaemia in some groups of population: the use of iron-fortified formulas and iron-fortified cereals; the use of oral contraceptives and increased enrichment of iron in several countries; and the use of iron supplements during pregnancy in some European countries. It is possible to prevent and control iron deficiency by counseling individuals and families about sound iron nutrition during infancy and beyond, and about iron supplementation during pregnancy, by screening persons on the basis of their risk for iron deficiency, and by treating and following up persons with presumptive iron deficiency. This may help to reduce manifestations of iron deficiency and thus improve public health. Evidence linking iron status with risk of cardiovascular disease or cancer is unconvincing and does not justify changes in food fortification or medical practice, particularly because the benefits of assuring adequate iron intake during growth and development are well established

  12. Glucose-6-phosphate dehydrogenase deficiency

    G-6-PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiency ... G6PD deficiency occurs when a person is missing or doesn't have enough of an enzyme called glucose- ...

  13. Infections Acquired in the Garden.

    Cunha, Cheston B; Cunha, Burke A

    2015-10-01

    Gardening is a wonderful pastime, and the garden is a very peaceful place to enjoy one's vacation. However, the garden may be a treacherous place for very young or compromised hosts when one takes into account the infectious potential residing in the soil, as well as the insect vectors on plants and animals. Even normal hosts may acquire a variety of infections from the soil, animals, or animal-related insect bites. The location of the garden, its natural animal and insect inhabitants, and the characteristics of the soil play a part in determining its infectious potential. The most important factor making the garden an infectious and dangerous place is the number and interaction of animals, whether they are pets or wild, that temporarily use the garden for part of their daily activities. The clinician should always ask about garden exposure, which will help in eliminating the diagnostic possibilities for the patient. The diagnostic approach is to use epidemiological principles in concert with clinical clues, which together should suggest a reasonable list of diagnostic possibilities. Organ involvement and specific laboratory tests help further narrow the differential diagnosis and determine the specific tests necessary to make a definitive diagnosis. PMID:26542044

  14. Music-reading deficiencies and the brain

    Lola L. Cuddy

    2006-01-01

    Full Text Available This paper reviews the literature on brain damage and music-reading for the past 25 years. Acquired patterns of selective loss and sparing are described, including both the association and dissociation of music and text reading, and association and dissociation among components of music reading. As well, we suggest that developmental music - reading deficiencies may be isolated in a form analogous to developmental dyslexia for text or congenital amusia for auditory music processing. Finally, we propose that the results of brain damage studies can contribute to the development of a model of normal music reading.

  15. Surface Sampler Arm Acquiring Sample

    1976-01-01

    Operation of the surface sampler in obtaining Martian soil for Viking 2's molecular analysis experiment last Saturday (September 25) was closely monitored by one of the Lander cameras because of the precision required in trenching the small area--8 by 9 inches-surrounded by rocks. Dubbed 'Bonneville Salt Flats,' the exposure of thin crust appeared unique in contrast with surrounding materials and became a prime target for organic analysis in spite of potential hazards. Large rock in foreground is 8 inches high. At left, the sampler scoop has touched the surface, missing the rock at upper left by a comfortable 6 inches, and the backhoe has penetrated the surface about one-half inch. The scoop was then pulled back to sample the desired point and (second photo) the backhoe furrowed the surface pulling a piece of thin crust toward the spacecraft. The initial touchdown and retraction sequence was used to avoid a collision between a rock in the shadow of the arm and a plate joining the arm and scoop. The rock was cleared by 2 to 3 inches. The third picture was taken 8 minutes after the scoop touched the surface and shows that the collector head has acquired a quantity of soil. With surface sampler withdrawn (right), the foot-long trench is seen between the rocks. The trench is three inches wide and about 1 1/2 to 2 inches deep. The scoop reached to within 3 inches of the rock at far end of trench. Penetration appears to have left a cavernous opening roofed by the crust and only about one inch of undisturbed crust separates the deformed surface and the rock.

  16. 17 CFR 210.8-06 - Real estate operations acquired or to be acquired.

    2010-04-01

    ... 17 Commodity and Securities Exchanges 2 2010-04-01 2010-04-01 false Real estate operations acquired or to be acquired. 210.8-06 Section 210.8-06 Commodity and Securities Exchanges SECURITIES AND... Statements of Smaller Reporting Companies § 210.8-06 Real estate operations acquired or to be acquired....

  17. TLR4 drives the pathogenesis of acquired cholesteatoma by promoting local inflammation and bone destruction.

    Si, Yu; Chen, Yu Bin; Chen, Sui Jun; Zheng, Yi Qing; Liu, Xiang; Liu, Yi; Jiang, Huai Li; Xu, Guo; Li, Zhuo Hao; Huang, Qiu Hong; Xiong, Hao; Zhang, Zhi Gang

    2015-01-01

    Acquired cholesteatoma is a chronic inflammatory disease characterized by both hyperkeratinized squamous epithelial overgrowth and bone destruction. Toll-like receptor (TLR) activation and subsequent inflammatory cytokine production are closely associated with inflammatory bone disease. However, the expression and function of TLRs in cholesteatoma remain unclear.We observed inflammatory cell infiltration of the matrix and prematrix of human acquired cholesteatoma, as well as dramatically increased expression of TLR4 and the pro-inflammatory cytokines TNF-α and IL-1β. TLR2 exhibited an up-regulation that was not statistically significant. TLR4 expression in human acquired cholesteatoma correlated with disease severity; the number of TLR4-positive cells increased with an increased degree of cholesteatoma, invasion, bone destruction, and hearing loss. Moreover, TLR4 deficiency was protective against experimental acquired cholesteatoma-driven bone destruction and hearing loss, as it reduced local TNF-α and IL-1β expression and impaired osteoclast formation by decreasing expression of the osteoclast effectors receptor activator of nuclear factor (NF)-κB ligand (RANKL) and tartrate-resistant acid phosphatase (TRAP). TLR2 deficiency did not relieve disease severity, inflammatory responses, or osteoclast formation. Moreover, neither TLR2 nor TLR4 deficiency had an effect on antimicrobial peptides, inducible iNOS,BD-2 expression or bacterial clearance. Therefore, TLR4 may promote cholesteatoma-induced bone destruction and deafness by enhancing inflammatory responses and osteoclastogenesis. PMID:26639190

  18. Antepartum ornithine transcarbamylase deficiency.

    Nakajima, Hitoshi; Sasaki, Yosuke; Maeda, Tadashi; Takeda, Masako; Hara, Noriko; Nakanishi, Kazushige; Urita, Yoshihisa; Hattori, Risa; Miura, Ken; Taniguchi, Tomoko

    2014-01-01

    Ornithine transcarbamylase deficiency (OTCD) is the most common type urea cycle enzyme deficiencies. This syndrome results from a deficiency of the mitochondrial enzyme ornithine transcarbamylase, which catalyzes the conversion of ornithine and carbamoyl phosphate to citrullin. Our case was a 28-year-old female diagnosed with OTCD following neurocognitive deficit during her first pregnancy. Although hyperammonemia was suspected as the cause of the patient's mental changes, there was no evidence of chronic liver disease. Plasma amino acid and urine organic acid analysis revealed OTCD. After combined modality treatment with arginine, sodium benzoate and hemodialysis, the patient's plasma ammonia level stabilized and her mental status returned to normal. At last she recovered without any damage left. PMID:25759629

  19. Metabolic Adaptations of White Lupin Roots and Shoots under Phosphorus Deficiency

    Müller, Julia; Gödde, Victoria; Niehaus, Karsten; Zörb, Christian

    2015-01-01

    White lupin (Lupinus albus L.) is highly adapted to phosphorus-diminished soils. P-deficient white lupin plants modify their root architecture and physiology to acquire sparingly available soil phosphorus. We employed gas chromatography–mass spectrometry (GC-MS) for metabolic profiling of P-deficient white lupins, to investigate biochemical pathways involved in the P-acquiring strategy. After 14 days of P-deficiency, plants showed reduced levels of fructose, glucose, and sucrose in shoots. Phosphorylated metabolites such as glucose-6-phosphate, fructose-6-phosphate, myo-inositol-phosphate and glycerol-3-phosphate were reduced in both shoots and roots. After 22 days of P-deficiency, no effect on shoot or root sugar metabolite levels was found, but the levels of phosphorylated metabolites were further reduced. Organic acids, amino acids and several shikimate pathway products showed enhanced levels in 22-day-old P-deficient roots and shoots. These results indicate that P-deficient white lupins adapt their carbohydrate partitioning between shoot and root in order to supply their growing root system as an early response to P-deficiency. Organic acids are released into the rhizosphere to mobilize phosphorus from soil particles. A longer period of P-deficiency leads to scavenging of Pi from P-containing metabolites and reduced protein anabolism, but enhanced formation of secondary metabolites. The latter can serve as stress protection molecules or actively acquire phosphorus from the soil. PMID:26635840

  20. Pilot study: Computer-based virtual anatomical interactivity for rehabilitation of individuals with chronic acquired brain injury

    C. Douglas Simmons, PhD, OTR/L, FAOTA; Sajay Arthanat, PhD, OTR/L, ATP; Vincent J. Macri, BA, MA

    2014-01-01

    Deficiencies in upper-limb motor function and executive functioning can compromise an affected individual’s ability to complete everyday activities. Impaired motor and executive functioning therefore pose a risk to increasing numbers of veterans who have been diagnosed with acquired brain injury. This article reports on changes in upper-limb motor function and executive functioning of 12 adult participants with chronic acquired brain injury using a novel, computer-based, motor and cognitive r...

  1. Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema

    Kaur R

    2014-12-01

    Full Text Available Ravdeep Kaur, Aerik Anthony Williams, Catherine Baker Swift, Jason W Caldwell Wake Forest University School of Medicine, Wake Forest University, Winston-Salem, NC, USA Abstract: Acquired angioedema is often associated with significant morbidity. An underlying lymphatic malignancy, autoimmune disorder, adenocarcinoma, or other malignancy may be present. Screening for these disorders should occur in all patients with acquired angioedema as treatment may result in resolution of angioedema. Keywords: complement, C1-INH deficiency, ecallantide, hemopathy

  2. Alpha1-antitrypsin deficiency

    Stolk, Jan; Seersholm, Niels; Kalsheker, Noor

    2006-01-01

    biennially to exchange views and research findings. The fourth biennial meeting was held in Copenhagen, Denmark, on 2-3 June 2005. This review covers the wide range of AAT deficiency-related topics that were addressed encompassing advances in genetic characterization, risk factor identification, clinical...... epidemiology, inflammatory and signalling processes, therapeutic advances, and lung imaging techniques....

  3. MCAD deficiency in Denmark

    Andresen, Brage Storstein; Lund, Allan Meldgaard; Hougaard, David Michael; Christensen, Ernst; Gahrn, Birthe; Christensen, Mette; Bross, Peter; Vested, Anne; Simonsen, Henrik; Skogstrand, Kristin; Olpin, Simon; Brandt, Niels Jacob; Skovby, Flemming; Nørgaard-Pedersen, Bent; Gregersen, Niels

    2012-01-01

    Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is the most common defect of fatty acid oxidation. Many countries have introduced newborn screening for MCADD, because characteristic acylcarnitines can easily be identified in filter paper blood spot samples by tandem mass spectrometry (MS/M...

  4. Iron-Deficiency Anemia

    Full Text Available ... the body. Iron-deficiency anemia usually develops over time if your body doesn't have enough iron ... Institutes of Health—shows how Susan, a full-time worker and student, has coped with having iron- ...

  5. Iron-Deficiency Anemia

    Full Text Available ... body. Low iron levels usually are due to blood loss, poor diet, or an inability to absorb enough iron from food. Overview Iron-deficiency anemia is a common type of anemia . The term "anemia" usually refers to ...

  6. Severe Dermatophytosis and Acquired or Innate Immunodeficiency: A Review

    Claire Rouzaud

    2015-12-01

    Full Text Available Dermatophytes are keratinophilic fungi responsible for benign and common forms of infection worldwide. However, they can lead to rare and severe diseases in immunocompromised patients. Severe forms include extensive and/or invasive dermatophytosis, i.e., deep dermatophytosis and Majocchi’s granuloma. They are reported in immunocompromised hosts with primary (autosomal recessive CARD9 deficiency or acquired (solid organ transplantation, autoimmune diseases requiring immunosuppressive treatments, HIV infection immunodeficiencies. The clinical manifestations of the infection are not specific. Lymph node and organ involvement may also occur. Diagnosis requires both mycological and histological findings. There is no consensus on treatment. Systemic antifungal agents such as terbinafine and azoles (itraconazole or posaconazole are effective. However, long-term outcome and treatment management depend on the site and extent of the infection and the nature of the underlying immunodeficiency.

  7. Methylenetetrahydrofolate reductase (MTHFR) deficiency presenting as a rash.

    Crushell, Ellen

    2012-09-01

    We report on the case of a 2-year-old girl recently diagnosed with Methylenetetrahydrofolate reductase (MTHFR) deficiency who originally presented in the neonatal period with a distinctive rash. At 11 weeks of age she developed seizures, she had acquired microcephaly and developmental delay. The rash deteriorated dramatically following commencement of phenobarbitone; both rash and seizures abated following empiric introduction of pyridoxine and folinic acid as treatment of possible vitamin responsive seizures. We postulate that phenobarbitone in combination with MTHFR deficiency may have caused her rash to deteriorate and subsequent folinic acid was helpful in treating the rash and preventing further acute neurological decline as commonly associated with this condition.

  8. Pathophysiology of thrombotic thrombocytopenic purpura : the "two-hit" paradigm

    Lotta, Luca Andrea

    2012-01-01

    Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread thrombosis in capillaries and small arteries. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patie

  9. Active von Willebrand factor in thrombotic thrombocytopenic purpura and malaria

    Groot, E.

    2009-01-01

    Thrombotic thrombocytopenic purpura (TTP) and malaria are two diseases of distinct origin. TTP is a rare disorder caused by a deficiency of the von Willebrand factor (VWF) cleaving protease ADAMTS13. Malaria is a poverty-related disease caused by protozoan parasites from the genus Plasmodium. TTP an

  10. Glucose-6-phosphate dehydrogenase deficiency

    ... this page: //medlineplus.gov/ency/article/000528.htm Glucose-6-phosphate dehydrogenase deficiency To use the sharing features on this page, please enable JavaScript. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a condition ...

  11. Acquired intrathoracic kidney in thoracic kyphosis

    Two cases of acquired intrathoracic kidney associated with thoracic kyphosis are reported, with emphasis on the radiographic manifestations. A search of the scientific literature disclosed that the acquired type of this abnormality is rare. The importance of recognizing this entity from a differential diagnostic standpoint is underscored. (author)

  12. And the Winner is – Acquired

    Henkel, Joachim; Rønde, Thomas; Wagner, Marcus

    value in case of success—that is, a more radical innovation. In the second stage, successful entrants bid to be acquired by the incumbent. We assume that entrants cannot survive on their own, so being acquired amounts to a ‘prize’ in a contest. We identify an equilibrium in which the incumbent chooses...

  13. Transient partial growth hormone deficiency due to zinc deficiency.

    Nishi, Y; Hatano, S; Aihara, K; Fujie, A; Kihara, M

    1989-04-01

    We present here a 13-year-old boy with partial growth hormone deficiency due to chronic mild zinc deficiency. When zinc administration was started, his growth rate, growth hormone levels, and plasma zinc concentrations increased significantly. His poor dietary intake resulted in chronic mild zinc deficiency, which in turn could be the cause of a further loss of appetite and growth retardation. There was also a possibility of renal zinc wasting which may have contributed to zinc deficiency. Zinc deficiency should be carefully ruled out in patients with growth retardation. PMID:2708733

  14. Iron deficiency and cognitive functions

    Jáuregui-Lobera I

    2014-11-01

    Full Text Available Ignacio Jáuregui-Lobera Department of Nutrition and Bromatology, Pablo de Olavide University, Seville, Spain Abstract: Micronutrient deficiencies, especially those related to iodine and iron, are linked to different cognitive impairments, as well as to potential long-term behavioral changes. Among the cognitive impairments caused by iron deficiency, those referring to attention span, intelligence, and sensory perception functions are mainly cited, as well as those associated with emotions and behavior, often directly related to the presence of iron deficiency anemia. In addition, iron deficiency without anemia may cause cognitive disturbances. At present, the prevalence of iron deficiency and iron deficiency anemia is 2%–6% among European children. Given the importance of iron deficiency relative to proper cognitive development and the alterations that can persist through adulthood as a result of this deficiency, the objective of this study was to review the current state of knowledge about this health problem. The relevance of iron deficiency and iron deficiency anemia, the distinction between the cognitive consequences of iron deficiency and those affecting specifically cognitive development, and the debate about the utility of iron supplements are the most relevant and controversial topics. Despite there being methodological differences among studies, there is some evidence that iron supplementation improves cognitive functions. Nevertheless, this must be confirmed by means of adequate follow-up studies among different groups. Keywords: iron deficiency, anemia, cognitive functions, supplementation

  15. The diagnostic value of protein induced by vitamin K absence or antagonist-ii in non-infant patients with acquired deficiency of vitamin K-dependent coagulation factors%维生素K缺乏或拮抗剂诱导的蛋白-Ⅱ在非婴儿获得性维生素K依赖性凝血因子缺乏症中的诊断意义

    王静; 吴天勤; 任传路; 沈红石; 陈海飞; 余自强; 王兆钺

    2014-01-01

    目的 探讨维生素K缺乏或拮抗剂诱导的蛋白-Ⅱ(PIVKA-Ⅱ)在非婴儿获得性维生素K依赖性凝血因子缺乏症(ADVKCF)中的诊断价值.方法 对临床确诊的50例ADVKCF患者采用ELISA法检测治疗0、3、7d的血浆中PIVKA-Ⅱ水平,同时分析不同时间点凝血常规和凝血因子促凝活性,并以20例健康体检者作为对照.结果 患者PIVKA-Ⅱ水平为(3.83±1.40) μg/L,健康对照组为(1.30±0.54) μg/L,差异有统计学意义(P<0.05),患者接受维生素K治疗3d后其值与治疗前相比差异无统计学意义[(3.83±1.40) μg/L比(3.79 ±0.66)μg/L,P>0.05],7d后其值降低但仍高于对照组水平.近期输注血浆组PIVKA-Ⅱ水平为(3.78±1.30)μg/L,同未输注组[(3.91±1.49)μg/L]相比,差异无统计学意义(P>0.05).治疗前患者凝血因子Ⅱ、Ⅶ、Ⅸ及Ⅹ活性明显降低(3.68%~12.28%),而凝血酶原时间(PT)、APTT明显延长(>100 s),治疗1周后基本恢复正常.结论 ADVKCF患者血浆PIVKA-Ⅱ的水平升高,维生素K治疗1周后其值仍高于正常人的水平,且不受血浆输注的影响,PIVKA-Ⅱ较凝血常规、凝血因子活性检测更为灵敏,具有早期辅助诊断本病的价值.%Objective To explore the diagnostic value of protein induced by vitamin K absence or antagonist-Ⅱ (PIVKA-Ⅱ) in non-infant with acquired deficiency of vitamin K-dependent coagulation factors (ADVKCF).Methods PIVKA-Ⅱ levels were measured by ELISA in 50 patients with ADVKCF on day 0,3,7 after vitamin K treatment.Prothrombin time(PT),APTT,FⅡ ∶ C,FⅦ∶ C,FⅨ∶ C,and FⅩ∶ C were analyzed simultaneously.Twenty healthy subjects were enrolled as controls.Results The average level of PIVKA-Ⅱ in ADVKCF group was (3.83 ± 1.40) μg/L,while (1.30 ± 0.54) μg/L in the control group (P < 0.05).The PIVKA-Ⅱ levels on day 0 and 3 did not show significant difference [(3.83 ± 1.40) μg/Lvs (3.79 ± 0.66) μg/L,P > 0.05],but decreasing significantly on day 7

  16. Gallium scans of the thorax in patients with acquired immune deficiency syndrome (AIDS): Description and utilization

    The pattern of distribution of gallium uptake in the thorax was investigated in patients (pts) with AIDS. Eleven pts (ages 18-53), all active homosexual males suspected of having acute pulmonary infection were studied. Ga lung scans were performed at 24-48 and/or 72 hrs. post injection. The diagnosis of AIDS was based on appropriate clinical and laboratory findings. The Ga activity in the lung was graded from zero = background to 4+ which is > liver activity. Eight of eleven pts have positive Ga scan while seven of eleven pts had positive CXR. Six pts had both positive CXR and Ga scan. One pt had a positive Ga scan with negative CXR, and one with positive CXR and negative Ga scan. The positive Ga scans included 3 pts with 4+ diffuse uptake, two pts with 2+ diffuse uptake, two pts with 1+ diffuse uptake, and two with hilar node uptake. Three pts have focal increased uptake superimposed on diffuse uptake. Two pts with 4+ diffuse uptake had mild abnormality on their CXR. One pt with 4+ uptake in the initial scan shows decreased activity on follow-up with clinical improvement after therapy. Thus, all but two pts with positive Ga scans had diffuse lung uptake. These two patients alone had B cell immunoblastic sarcoma and oral candidiasis. The pattern of Ga lung uptake in pts with AIDS reveal that a majority of positive scans are diffuse (6/8) and the intensity may suggest more active disease than CXR (2 normal) and, thus, the study may be useful in detecting changes from atypical pulmonary infection in this population

  17. Gallium scans of the thorax in patients with acquired immune deficiency syndrome (AIDS): Description and utilization

    Le, G.; Chen, D.C.P.; Siegel, M.E.

    1984-01-01

    The pattern of distribution of gallium uptake in the thorax was investigated in patients (pts) with AIDS. Eleven pts (ages 18-53), all active homosexual males suspected of having acute pulmonary infection were studied. Ga lung scans were performed at 24-48 and/or 72 hrs. post injection. The diagnosis of AIDS was based on appropriate clinical and laboratory findings. The Ga activity in the lung was graded from zero = background to 4+ which is > liver activity. Eight of eleven pts have positive Ga scan while seven of eleven pts had positive CXR. Six pts had both positive CXR and Ga scan. One pt had a positive Ga scan with negative CXR, and one with positive CXR and negative Ga scan. The positive Ga scans included 3 pts with 4+ diffuse uptake, two pts with 2+ diffuse uptake, two pts with 1+ diffuse uptake, and two with hilar node uptake. Three pts have focal increased uptake superimposed on diffuse uptake. Two pts with 4+ diffuse uptake had mild abnormality on their CXR. One pt with 4+ uptake in the initial scan shows decreased activity on follow-up with clinical improvement after therapy. Thus, all but two pts with positive Ga scans had diffuse lung uptake. These two patients alone had B cell immunoblastic sarcoma and oral candidiasis. The pattern of Ga lung uptake in pts with AIDS reveal that a majority of positive scans are diffuse (6/8) and the intensity may suggest more active disease than CXR (2 normal) and, thus, the study may be useful in detecting changes from atypical pulmonary infection in this population.

  18. Use of Plasma for Acquired Coagulation Factor Deficiencies in Critical Care.

    Shah, Akshay; McKechnie, Stuart; Stanworth, Simon

    2016-03-01

    Coagulopathy in critically ill patients is common and often multifactorial. Fresh frozen plasma (FFP) is commonly used to correct this either prophylactically or therapeutically. FFP usage is mainly guided by laboratory tests of coagulation, which have been shown to have poor predictive values for bleeding. Viscoelastic tests are an attractive option to guide hemostatic therapy, but require rigorous evaluation. The past few years have seen a gradual reduction in national use of FFP potentially due to an increased awareness of risks such as transfusion-related acute lung injury, patient blood management strategies to reduce transfusion in general, and increased awareness of the lack of high-quality evidence available to support FFP use. Within critical care, FFP is administered before invasive procedures/surgery, to treat major traumatic and nontraumatic hemorrhage, disseminated intravascular coagulation, and for urgent warfarin reversal if first-line agents, such as prothrombin complex concentrate (PCC) are not available. Alternative agents such as fibrinogen concentrate and PCC need further evaluation through large-scale clinical trials. PMID:26716502

  19. Pancytopenia and cutaneous cryptococcosis as an indicator disease of acquired immune deficiency syndrome

    R Khuraijam

    2015-01-01

    Full Text Available We present a case of pancytopenia and cutaneous cryptococcosis in a young girl with no complaints of fever, headache and vomiting. Fine-needle aspiration cytology and further investigation for pancytopenia revealed presence of Cryptococcus in skin and bone marrow aspirates. Fungal cultures of the skin aspirates, blood and bone marrow confirmed cryptococcal infection. Counselling and human immunodeficiency virus (HIV test revealed the status of the patient to be retropositive. Although meningitis is the commonest manifestation of cryptococcosis among HIV-infected patients, rare cutaneous manifestation with pancytopenia but with no meningeal signs indicate the HIV status in an endemic area of penicilliosis, Manipur.

  20. Stroke in an Infant; Its Association with Antiphospholipid Antibody and Acquired Protein C and S Deficiencies

    Soroor Inaloo Mohammad Ghofrani

    2004-06-01

    Full Text Available We present the first reported case of antiphospholipid syndrome with stroke in an Iranian boy (7-month-old who had two ischemic strokes within a period of 2 months. Serum anticardiolipid antibody was positive and the patient had low levels of protein S and C. This case emphasizes the importance of antiphospholipid antibody in children with unexplained ischemic stroke.

  1. A rare radiological manifestation of disseminated tuberculous spondylitisin acquired immune deficiency syndrome patient: A case report

    Kim, Sung Won; Koo, Joon Bum; Kim, Tae Eun [Dept. of of Radiology, Dongguk University Ilsan Hospital, Dongguk University School of Medicine, Goyang (Korea, Republic of)

    2016-04-15

    The spine is the most common site of skeletal involvement in tuberculosis. The radiologic features are reportedly characterized by destruction of the vertebral body, subligamentous extension or subchondral penetration, frequent paravertebral abscess formation and late involvement of the disk space. We experienced a case of a 25-year-old male who was a human immunodeficiency virus carrier without antiretroviral therapy. Incidental findings on abdominal computed tomography included multiple well-demarcated and ovoid osteolytic lesions with hyperdense rims disseminated in the thoracic, lumbar, and sacrum vertebrae, as well as in both ilii. On the lumbar spine magnetic resonance imaging, multiple small round lesions of isointense signal intensity with peripheral hyperintense rims were found on both T1- and T2-weighted imaging. The lesions had peripheral rim enhancement on gadolinium-enhanced T1-weighted imaging. Based on our experience, this rare image finding is one of the manifestations of disseminated tuberculosis.

  2. Features of severe community acquired pneumonia in hiv-infected patients

    Bielosludtseva K.O.

    2014-01-01

    Questions of management of patients with severe community acquired pneumonia (CAP) in HIV-infected are particularly acute. Pneumonia etiology, identification and treatment on the background of immune deficiency, its clinical and diagnostic features and tactics are still not clearly described, this significantly increases mortality from CAP in general clinical practice. That is why the aim of the work was to determine clinical features, etiology, severity of systemic inflammation and cellular ...

  3. The Bile Salt Export Pump: Clinical and Experimental Aspects of Genetic and Acquired Cholestatic Liver Disease

    Lam, Ping; Soroka, Carol J.; Boyer, James L.

    2010-01-01

    The primary transporter responsible for bile salt secretion is the bile salt export pump (BSEP, ABCB11), a member of the ATP-binding cassette (ABC) superfamily, which is located at the bile canalicular apical domain of hepatocytes. In humans, BSEP deficiency results in several different genetic forms of cholestasis, which include progressive familial intrahepatic cholestasis type 2 (PFIC2), benign recurrent intrahepatic cholestasis type 2 (BRIC2), as well as other acquired forms of cholestasi...

  4. Biotin and biotinidase deficiency

    Zempleni, Janos; Hassan, Yousef I.; Wijeratne, Subhashinee SK

    2008-01-01

    Biotin is a water-soluble vitamin that serves as an essential coenzyme for five carboxylases in mammals. Biotin-dependent carboxylases catalyze the fixation of bicarbonate in organic acids and play crucial roles in the metabolism of fatty acids, amino acids and glucose. Carboxylase activities decrease substantially in response to biotin deficiency. Biotin is also covalently attached to histones; biotinylated histones are enriched in repeat regions in the human genome and appear to play a role...

  5. Iron Deficiency Anemia

    Hassan Ahari

    1965-01-01

    Full Text Available The object of this paper is to draw attention to iron deficiency anemia which is the most common nutritional disturbance in infants and children. Iron deficiency anemia constitutes the most prevalent form of anemia in this age group. The records of infants and children admitted to the Pediatric Department of Tehran University Puhlavi Hospital for various ailments during a one year period (Mnrch l!l63 - HHi-t were analyzed. 262 infants and children out of a total number of an5, or 7t•/., showed iron deficiency anemia detect cd by blood film studies and hemoglobin determination, The majority, 123 or 4{.!t•/., of these patients were infants and children between six months and two years of age. The etiology indicates that faulty feeding is the main cause. Infections, parnsitcs, and hemorrhage were among other causes observed. ,'('itll regard to treatment, parenteral iron was preferred because cf its ef., Icctivcncss in short periods of hospital stay. In conclusion, the routine study of blood films and hemoglobin determiualion, especially in the low socio _ economic group of medically less organized countries is advised

  6. Iron-Deficiency Anemia (For Parents)

    ... Things to Know About Zika & Pregnancy Iron-Deficiency Anemia KidsHealth > For Parents > Iron-Deficiency Anemia Print A ... common nutritional deficiency in children. About Iron-Deficiency Anemia Every red blood cell in the body contains ...

  7. Campylobacter fetus Bacteremia Revealed by Cellulitis without Gastrointestinal Symptoms in the Context of Acquired Hypogammaglobulinemia: A Report of Three Cases

    Souleymane Brah

    2011-01-01

    Full Text Available Campylobacter fetus bacteremia is rare and occurs mainly in patients with immunosuppression. This infection, which often involves secondary localizations has already been reported in some primary humoral immune deficiencies. We describe three cases of severe infection due to C. fetus with cellulitis at presentation, but without any gastrointestinal symptoms, occurring in patients with acquired hypogammaglobulinemia.

  8. Enhancing Medicares Hospital Acquired Conditions Policy

    U.S. Department of Health & Human Services — The current Medicare policy of non-payment to hospitals for Hospital Acquired Conditions (HAC) seeks to avoid payment for preventable complications identified...

  9. Acquired Inventors’ Productivity after Horizontal Acquisition

    Colombo, Massimo G.; Moreira, Solon; Rabbiosi, Larissa

    Effective integration of the R&D functions of the acquired and acquiring firms is essential for knowledge recombination after acquisition. However, prior research suggests that the post-acquisition integration process often damages the inventive labor force. We argue that an examination of the mu......Effective integration of the R&D functions of the acquired and acquiring firms is essential for knowledge recombination after acquisition. However, prior research suggests that the post-acquisition integration process often damages the inventive labor force. We argue that an examination...... of the multifaceted nature of the integration process further enhances our understanding of which conditions will be more or less detrimental for corporate inventors. We focus on R&D teams which are the immediate organizational context in which inventors operate and drawing on insights from learning theory...

  10. Hospital-Acquired Condition Reduction Program

    U.S. Department of Health & Human Services — In October 2014, CMS began reducing Medicare payments for subsection (d) hospitals that rank in the worst performing quartile with respect to hospital-acquired...

  11. Infections acquired in clinical laboratories in Utah.

    Jacobson, J.T.; Orlob, R B; Clayton, J L

    1985-01-01

    We reviewed laboratory-acquired infections occurring in Utah from 1978 through 1982. Written and telephone interviews of supervisors of 1,191 laboratorians revealed an estimated annual incidence of 3 laboratory-acquired infections per 1,000 employees. Infections, in order of frequency, included hepatitis B (clinical cases), shigellosis, pharyngitis, cellulitis, tuberculosis (skin test conversion), conjunctivitis, and non-A, non-B hepatitis. One-half of large laboratories (over 25 employees), ...

  12. The Acquired Immunodeficiency Syndrome: current status.

    Quagliarello, V.

    1982-01-01

    A recently recognized syndrome of acquired immunodeficiency (Acquired Immunodeficiency Syndrome-AIDS) has arisen since June 1981. It has received international attention. The clinical spectrum consists of repeated opportunistic infections, rare malignancies, and autoimmune phenomena, occurring in previously healthy adults with no history of an immunologic disorder. The population subset at risk for this syndrome appears to be predominantly homosexual American males and intravenous drug abuser...

  13. Carnitine palmitoyltransferase II deficiency

    Roe, C R.; Yang, B-Z; Brunengraber, H; Roe, D S.; Wallace, M; Garritson, B K.

    2008-01-01

    Background: Carnitine palmitoyltransferase II (CPT II) deficiency is an important cause of recurrent rhabdomyolysis in children and adults. Current treatment includes dietary fat restriction, with increased carbohydrate intake and exercise restriction to avoid muscle pain and rhabdomyolysis. Methods: CPT II enzyme assay, DNA mutation analysis, quantitative analysis of acylcarnitines in blood and cultured fibroblasts, urinary organic acids, the standardized 36-item Short-Form Health Status survey (SF-36) version 2, and bioelectric impedance for body fat composition. Diet treatment with triheptanoin at 30% to 35% of total daily caloric intake was used for all patients. Results: Seven patients with CPT II deficiency were studied from 7 to 61 months on the triheptanoin (anaplerotic) diet. Five had previous episodes of rhabdomyolysis requiring hospitalizations and muscle pain on exertion prior to the diet (two younger patients had not had rhabdomyolysis). While on the diet, only two patients experienced mild muscle pain with exercise. During short periods of noncompliance, two patients experienced rhabdomyolysis with exercise. None experienced rhabdomyolysis or hospitalizations while on the diet. All patients returned to normal physical activities including strenuous sports. Exercise restriction was eliminated. Previously abnormal SF-36 physical composite scores returned to normal levels that persisted for the duration of the therapy in all five symptomatic patients. Conclusions: The triheptanoin diet seems to be an effective therapy for adult-onset carnitine palmitoyltransferase II deficiency. GLOSSARY ALT = alanine aminotransferase; AST = aspartate aminotransferase; ATP = adenosine triphosphate; BHP = β-hydroxypentanoate; BKP = β-ketopentanoate; BKP-CoA = β-ketopentanoyl–coenzyme A; BUN = blood urea nitrogen; CAC = citric acid cycle; CoA = coenzyme A; CPK = creatine phosphokinase; CPT II = carnitine palmitoyltransferase II; LDL = low-density lipoprotein; MCT

  14. Treatment of carnitine deficiency.

    Winter, S C

    2003-01-01

    Carnitine deficiency is a secondary complication of many inborn errors of metabolism. Pharmacological treatment with carnitine not only corrects the deficiency, it facilitates removal of accumulating toxic acyl intermediates and the generation of mitochondrial free coenzyme A (CoA). The United States Food and Drug Administration (US FDA) approved the use of carnitine for the treatment of inborn errors of metabolism in 1992. This approval was based on retrospective chart analysis of 90 patients, with 18 in the untreated cohort and 72 in the treated cohort. Efficacy was evaluated on the basis of clinical and biochemical findings. Compelling data included increased excretion of disease-specific acylcarnitine derivatives in a dose-response relationship, decreased levels of metabolites in the blood, and improved clinical status with decreased hospitalization frequency, improved growth and significantly lower mortality rates as compared to historical controls. Complications of carnitine treatment were few, with gastrointestinal disturbances and odour being the most frequent. No laboratory or clinical safety issues were identified. Intravenous carnitine preparations were also approved for treatment of secondary carnitine deficiency. Since only 25% of enteral carnitine is absorbed and gastrointestinal tolerance of high doses is poor, parenteral carnitine treatment is an appealing alternative therapeutic approach. In 7 patients treated long term with high-dose weekly to daily venous boluses of parenteral carnitine through a subcutaneous venous port, benefits included decreased frequency of decompensations, improved growth, improved muscle strength and decreased reliance on medical foods with liberalization of protein intake. Port infections were the most troubling complication. Theoretical concerns continue to be voiced that carnitine might result in fatal arrhythmias in patients with long-chain fat metabolism defects. No published clinical studies substantiate these

  15. Nasal Tip Deficiency.

    Cerkes, Nazim

    2016-01-01

    Nasal tip deficiency can be congenital or secondary to previous nasal surgeries. Underdeveloped medial crura usually present with underprojected tip and lack of tip definition. Weakness or malposition of lateral crura causes alar rim retraction and lateral nasal wall weakness. Structural grafting of alar cartilages strengthens the tip framework, reinforces the disrupted support mechanisms, and controls the position of the nasal tip. In secondary cases, anatomic reconstruction of the weakened or interrupted alar cartilages and reconstitution of a stable nasal tip tripod must be the goal for a predictable outcome. PMID:26616702

  16. Antithrombin deficiency in pregnancy.

    Durai, Shivani; Tan, Lay Kok; Lim, Serene

    2016-01-01

    We present a case of a 39-year-old, gravida 3 para 2, Chinese female with a history of inherited type 1 Antithrombin deficiency and multiple prior episodes of venous thromboembolism. She presented at 29+4 weeks' gestation with severe pre-eclampsia complicated by haemolysis, elevated liver enzymes and low platelet (HELLP) syndrome. She subsequently underwent an emergency caesarean section for non-reassuring fetal status, which was complicated by postpartum haemorrhage secondary to uterine atony, requiring a B-Lynch suture intraoperatively. PMID:27207982

  17. Zinc-deficiency acrodermatitis in a patient with chronic alcoholism and gastric bypass: a case report

    Dariush Shahsavari

    2014-07-01

    Full Text Available Acquired adult-onset zinc deficiency is occasionally reported in patients with malnutrition states, such as alcoholism, or malabsorptive states, such as post-bariatric surgery. The defining symptoms of hypozincemia include a classic triad of necrolytic dermatitis, diffuse alopecia, and diarrhea. We report a case of zinc deficiency in a 39-year-old man with history of gastric bypass surgery and alcoholism. For this patient, severe hypozincemia confirmed acrodermatitis, and zinc supplementation was met with gradual improvement.

  18. Glucose-6-phosphatase deficiency

    Labrune Philippe

    2011-05-01

    Full Text Available Abstract Glucose-6-phosphatase deficiency (G6P deficiency, or glycogen storage disease type I (GSDI, is a group of inherited metabolic diseases, including types Ia and Ib, characterized by poor tolerance to fasting, growth retardation and hepatomegaly resulting from accumulation of glycogen and fat in the liver. Prevalence is unknown and annual incidence is around 1/100,000 births. GSDIa is the more frequent type, representing about 80% of GSDI patients. The disease commonly manifests, between the ages of 3 to 4 months by symptoms of hypoglycemia (tremors, seizures, cyanosis, apnea. Patients have poor tolerance to fasting, marked hepatomegaly, growth retardation (small stature and delayed puberty, generally improved by an appropriate diet, osteopenia and sometimes osteoporosis, full-cheeked round face, enlarged kydneys and platelet dysfunctions leading to frequent epistaxis. In addition, in GSDIb, neutropenia and neutrophil dysfunction are responsible for tendency towards infections, relapsing aphtous gingivostomatitis, and inflammatory bowel disease. Late complications are hepatic (adenomas with rare but possible transformation into hepatocarcinoma and renal (glomerular hyperfiltration leading to proteinuria and sometimes to renal insufficiency. GSDI is caused by a dysfunction in the G6P system, a key step in the regulation of glycemia. The deficit concerns the catalytic subunit G6P-alpha (type Ia which is restricted to expression in the liver, kidney and intestine, or the ubiquitously expressed G6P transporter (type Ib. Mutations in the genes G6PC (17q21 and SLC37A4 (11q23 respectively cause GSDIa and Ib. Many mutations have been identified in both genes,. Transmission is autosomal recessive. Diagnosis is based on clinical presentation, on abnormal basal values and absence of hyperglycemic response to glucagon. It can be confirmed by demonstrating a deficient activity of a G6P system component in a liver biopsy. To date, the diagnosis is most

  19. Iron Deficiency and Bariatric Surgery

    Ignacio Jáuregui-Lobera

    2013-01-01

    It is estimated that the prevalence of anaemia in patients scheduled for bariatric surgery is higher than in the general population and the prevalence of iron deficiencies (with or without anaemia) may be higher as well. After surgery, iron deficiencies and anaemia may occur in a higher percentage of patients, mainly as a consequence of nutrient deficiencies. In addition, perioperative anaemia has been related with increased postoperative morbidity and mortality and poorer quality of life aft...

  20. Iatrogenic limbal stem cell deficiency.

    Holland, E J; Schwartz, G S

    1997-01-01

    PURPOSE: To describe a group of patients with limbal stem cell (SC) deficiency without prior diagnosis of a specific disease entity known to be causative of SC deficiency. METHODS: We performed a retrospective review of the records of all patients with ocular surface disease presenting to the University of Minnesota between 1987 and 1996. Patients were categorized according to etiology of limbal deficiency. Patients who did not have a specific diagnosis previously described as being causative...

  1. Do Acquirer Capabilities Affect Acquisition Performance? Examining Strategic and Effectiveness Capabilities in Acquirers

    Mudde, Paul A.; Brush, Thomas

    2006-01-01

    This paper examines acquisition performance from the perspective of acquirer capabilities. It argues that the strategic capabilities underpinning a firm’s competitive strategy can be utilized to create economic value in acquisitions. Acquirers with strong cost leadership capabilities are expected to leverage these capabilities to reduce post-acquisition costs as they integrate acquisition targets. Acquirers with strong differentiation capabilities are expected to utilize their strategic capab...

  2. Acquired angioedema secondary to hormone replacement therapy

    Malani Kumar

    2005-10-01

    Full Text Available Angioedema is a potentially life threatening condition and may be either inherited or acquired. The latter is rare with only a handful of cases reported in the world literature. Presenting complaints are often vague. Those most commonly described include swelling in the subcutaneous and submucosal tissues. Patients presenting with laryngeal edema have high mortality, and high clinical suspicion is necessary to avoid instrumentation, which can precipitate laryngeal spasm. We present a review of reported cases of hormonally induced hereditary angioedema, along with a report of a patient with acquired angioedema secondary to hormone replacement therapy. To the best of our knowledge, this case probably represents the first reported case of acquired angioedema secondary to hormone replacement therapy.

  3. Imaging features of copper deficiency myelopathy: a study of 25 cases

    Acquired copper deficiency presents with a spastic gait and sensory ataxia. Spinal cord magnetic resonance imaging (MRI) in patients with copper deficiency myelopathy may show increased T2 signal, most commonly in the dorsal midline cervical and thoracic cord. These imaging findings may be reversible with normalization of serum copper. The clinical and imaging picture is very similar to the subacute combined degeneration seen in patients with vitamin B12 deficiency. Neuroradiologists should consider this possibility when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified. (orig.)

  4. Prolonged QTc interval in association with medium-chain acyl-coenzyme A dehydrogenase deficiency.

    Wiles, Jason R; Leslie, Nancy; Knilans, Timothy K; Akinbi, Henry

    2014-06-01

    Medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency is the most common disorder of mitochondrial fatty acid oxidation. We report a term male infant who presented at 3 days of age with hypoglycemia, compensated metabolic acidosis, hypocalcemia, and prolonged QTc interval. Pregnancy was complicated by maternal premature atrial contractions and premature ventricular contractions. Prolongation of the QTc interval resolved after correction of metabolic derangements. The newborn screen was suggestive for MCAD deficiency, a diagnosis that was confirmed on genetic analysis that showed homozygosity for the disease-associated missense A985G mutation in the ACADM gene. This is the first report of acquired prolonged QTc in a neonate with MCAD deficiency, and it suggests that MCAD deficiency should be considered in the differential diagnoses of acute neonatal illnesses associated with electrocardiographic abnormality. We review the clinical presentation and diagnosis of MCAD deficiency in neonates. PMID:24799540

  5. Cleft palate and gonadotrophin deficiency.

    Gillis, P H; Peeters, R.

    1984-01-01

    A boy who had previously had a cleft lip and palate repaired and bilateral orchiopexies presented at 16 years of age with delayed puberty. Isolated gonadotrophin deficiency and testicular hyporesponsiveness to human chorionic gonadotrophin were found. The possibility of bilateral cryptorchidism due to gonadotrophin deficiency should be considered in boys with either cleft lip or palate, or both.

  6. Iron deficiency anemia in children.

    Subramaniam, Girish; Girish, Meenakshi

    2015-06-01

    Iron deficiency is not just anemia; it can be responsible for a long list of other manifestations. This topic is of great importance, especially in infancy and early childhood, for a variety of reasons. Firstly, iron need is maximum in this period. Secondly, diet in infancy is usually deficient in iron. Thirdly and most importantly, iron deficiency at this age can result in neurodevelopmental and cognitive deficits, which may not be reversible. Hypochromia and microcytosis in a complete blood count (CBC) makes iron deficiency anemia (IDA) most likely diagnosis. Absence of response to iron should make us look for other differential diagnosis like β thalassemia trait and anemia of chronic disease. Celiac disease is the most important cause of true IDA not responding to oral iron therapy. While oral ferrous sulphate is the cheapest and most effective therapy for IDA, simple nonpharmacological and pharmacological measures can go a long way in prevention of iron deficiency. PMID:25636824

  7. Immunomodulation in community-acquired pneumonia

    Remmelts, H.H.F.

    2013-01-01

    Community-acquired pneumonia (CAP) is a common disease with considerable morbidity and mortality, despite effective antibiotic treatment. In this thesis, we showed that the major causative microorganisms in CAP trigger distinct inflammatory response profiles in the host. While an inflammatory respon

  8. Group Treatment in Acquired Brain Injury Rehabilitation

    Bertisch, Hilary; Rath, Joseph F.; Langenbahn, Donna M.; Sherr, Rose Lynn; Diller, Leonard

    2011-01-01

    The current article describes critical issues in adapting traditional group-treatment methods for working with individuals with reduced cognitive capacity secondary to acquired brain injury. Using the classification system based on functional ability developed at the NYU Rusk Institute of Rehabilitation Medicine (RIRM), we delineate the cognitive…

  9. Acquired secondary Grynfeltt's hernia: a case report

    Lumbar hernia is a rare condition whose diagnosis is hardly achieved. The prevalence is higher in elderly men. The present case report describes the case of a male, 78-year-old patient who underwent pleural effusion drainage 17 years before presenting with clinical manifestations and tomographic findings compatible with acquired secondary Grynfeltt's hernia. (author)

  10. Monitoring Agitated Behavior After acquired Brain Injury

    Aadal, Lena; Mortensen, Jesper; Nielsen, Jørgen Feldbaek

    2015-01-01

    Purpose: To describe the onset, duration, intensity, and nursing shift variation of agitated behavior in patients with acquired brain injury (ABI) at a rehabilitation hospital. Design: Prospective descriptive study. Methods: A total of 11 patients with agitated behavior were included. Agitated...